Complications in neonatal surgery

Seminars in Pediatric Surgery 25 (2016) 347–370

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Seminars in Pediatric Surgery journal homepage: www.elsevier.com/locate/sempedsurg

Complications in neonatal surgery Mauricio A. Escobar Jr, MDa,n, Michael G. Caty, MD, MMMb a b

Pediatric Surgery, Mary Bridge Children's Hospital, PO Box 5299, MS: 311-W3-SUR, 311 South, Tacoma, Washington 98415-0299 Section of Pediatric Surgery, Department of Surgery, Yale-New Haven Children's Hospital, New Haven, Connecticut

a r t i c l e in fo

Keywords: Neonatal surgery Surgical complications Atresia Congenital diaphragmatic hernia Necrotizing enterocolitis Abdominal wall defects

a b s t r a c t Neonatal surgery is recognized as an independent discipline in general surgery, requiring the expertise of pediatric surgeons to optimize outcomes in infants with surgical conditions. Survival following neonatal surgery has improved dramatically in the past 60 years. Improvements in pediatric surgical outcomes are in part attributable to improved understanding of neonatal physiology, specialized pediatric anesthesia, neonatal critical care including sophisticated cardiopulmonary support, utilization of parenteral nutrition and adjustments in fluid management, refinement of surgical technique, and advances in surgical technology including minimally invasive options. Nevertheless, short and long-term complications following neonatal surgery continue to have profound and sometimes lasting effects on individual patients, families, and society. & 2016 Elsevier Inc. All rights reserved.

Introduction Surgical conditions of the neonate are often difficult to manage due to their rarity.1 Successful management of neonatal surgical problems requires knowledge of cervical, thoracic, and abdominal anatomy and physiology. A thorough understanding of congenital anomalies and neonatal pathophysiology is essential to the practicing pediatric surgeon. In addition, the surgeon must be aware of functional abnormalities of the esophagus, stomach, intestines, diaphragm, and abdominal wall. Knowledge of potential complications due to technical problems and associated anomalies will allow the pediatric surgeon to anticipate and avoid these problems. Additionally, more information on long-term complications is becoming available due to improved outcomes and longer survival. In the this article, complications related to the treatment of esophageal atresia, intestinal atresias, necrotizing enterocolitis, congenital diaphragmatic hernia, and abdominal wall defects will be discussed.

Esophageal atresia Esophageal atresia (EA) has been described as “the epitome of modern surgery.”2 In 1936, Thomas Lanman performed the first primary extrapleural anastomosis of this anomaly, but the child lived only 3 h.3 In what is now recognized as an incredible feat of n

Corresponding author. E-mail address: [email protected] (M.A. Escobar Jr).

http://dx.doi.org/10.1053/j.sempedsurg.2016.10.005 1055-8586/& 2016 Elsevier Inc. All rights reserved.

surgical courage, he reported his experience with 30 operative cases all of whom died. He stated that “with greater experience, improved technique and good luck,” success would soon be reported. Leven4 and Ladd5 reported the first staged survivors (in 1939) and Cameron Haight6 performed the first successful primary anastomosis in 1941.

Epidemiology Esophageal atresia with varying types of tracheoesophageal fistula (TEF) affects approximately 1:2400 to 1:3000 neonates.6,7 Associated chromosomal abnormalities include trisomy 13 and 18.8 The majority of infants with EA have at least one associated congenital malformation. Cardiovascular anomalies account for approximately 35% of associated defects in patients with EA9 and the majority of deaths associated with EA.10–12 A right-sided aortic arch is found in 5% of neonates with EA13 and its presence may dictate the need for a left sided thoracotomy for the operative approach to the EA repair.13–15 Common gastrointestinal anomalies associated with EA include anorectal malformations, duodenal atresia, and intestinal malrotation.11 Furthermore, these malformations may be clustered in the neonate as the VACTERL-H association, which includes vertebral, anorectal, cardiac, renal, limb abnormalities and, occasionally, congenital hydrocephalus.16,17 Esophageal atresia may be associated with a variety of syndromes including Down syndrome11 and CHARGE syndrome (coloboma, heart defects, choanal atresia, developmental/growth delay, ear anomalies 7 deafness).18 Tracheomalacia is a commonly

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Fig. 1. (A) Right aortic arch with aberrant left subclavian artery, ligamentum artriosum and (B) esophageal lung.

associated anomaly in patients with EA. Tracheomalacia results from a decreased ratio of circumferential cartilaginous trachea to membranous trachea.19 Clinical manifestations of tracheomalacia are noted below (tracheomalacia). A variety of lethal tracheobronchial abnormalities have been reported in association with EA, including tracheal agenesis, laryngeal atresia, congenital tracheal stenosis, and pulmonary agenesis. Other associated tracheobronchial malformations include ectopic bronchi such as tracheal bronchus and trifurcated trachea and absent right upper lobe bronchus. The authors recently reported a combination of a right-sided aortic arch, diverticulum of Kommerell and esophageal lung (Figure 1).20

insufflated through the TEF. Gastric perforation used to be universally fatal; however, survival after gastrointestinal perforation from esophageal atresia has now been reported in low birth weight infants.25 Techniques described to control the TEF in the infant with respiratory compromise include needle decompression of the stomach en route to the operating room, temporary occlusion of the gastroesophageal junction,26 placement of a gastrostomy tube to water seal, cannulating the fistula with a Fogarty catheter placed bronchoscopically,26 transgastric fistula occlusion, passage of a cuffed endotracheal tube distal to the fistula, and high frequency jet ventilation.22,27 Spitz22 advocates emergency ligation of the fistula as the procedure of choice.

Classifications

Operative therapy and complications

There are five basic types of EA 7 TEF. The Gross21 classification is the most widely recognized. In order of prevalence, they are EA with a distal TEF (86% incidence, Gross Type C), EA without TEF (7%, Gross Type A), TEF without EA (“H” Type) (4%, Gross Type E), EA with proximal and distal TEF (2%, Gross Type D), and EA with a proximal TEF (1%, Gross Type B).22 In addition to anatomic classification, several risk stratifications have been utilized. The Waterston criteria stratified neonates based on birth weight, presence of pneumonia, and associated congenital anomalies.23 Spitz24 noted that the two factors with the greatest impact on survival were very low birth weight (o1500 g) and major congenital cardiac malformations. Survival was grouped as follows: group I (birth weight 4 1500 g), 96%; group II (o1500 g or major congenital heart disease), 60%; group III (o1500 g and major congenital heart disease), 18%.22

The operative approach to EA depends on the type of anomaly present and the occurrence of associated anomalies.

Preoperative complications Neonates with EA with distal TEF with severe respiratory distress pose a significant challenge in airway management. They are also at increased risk of gastric perforation as the stomach is

Esophageal atresia with distal tracheoesophageal fistula Esophageal atresia with distal TEF is the classic presentation encountered by the pediatric surgeon. Division of the TEF and primary esophagoesophagostomy is the procedure of choice. Careful coordination with the anesthesia team is critical for optimal operative outcome. The anesthesiologist provides minimal respiratory support by allowing the infant to spontaneously breathe. This minimizes gastric insufflation through the TEF. The anesthesiologist and surgeon work in conjunction to provide the best exposure of the proximal pouch by placing a dilator or sump drain into the proximal pouch to provide gentle tension during the dissection. The operative approach is through a right posterolateral thoracotomy unless a right-sided aortic arch is preoperatively diagnosed. This dictates a left thoracotomy. Many pediatric surgeons prefer an extrapleural approach to avoid the possible complication of an empyema, but in the era of modern antibiotics, a transpleural approach is acceptable. For the extrapleural approach, the chest wall is dissected off the pleura by cotton

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swabs and gauze sponge sticks in a lateral direction. Although primary repair may be performed safely in the premature neonate, a recent review found staged repair of EA/TEF in very low birth weight premature infants resulted in a significantly lower rate of anastomotic complications and overall morbidity.28 Additional technical considerations include reinforcement of the anastomosis, positioning of the drainage tube and maintaining a high degree of suspicion for a second TEF. Postoperative respiratory failure in patients with a missed second TEF has been reported in a patient that did have preoperative bronchoscopy.29 Persistent drainage from an extrapleural chest tube that eroded into the esophageal anastomosis has also been reported.30 Some pediatric surgeons argue against the use of routine postoperative extrapleural drains in uncomplicated cases.31,32 A variety of techniques have been described to reinforce the esophageal anastomosis including using an azygous flap and applying fibrin glue.33 The application of fibrin glue was shown to decrease anastomotic leaks, stenosis, and mortality in a randomized trial of 22 patients compared to 23 patients who did not have fibrin glue applied to the anastomosis.33

Long gap atresia In most instances, neonates who have EA and TEF have a short distance between the esophageal ends allowing for primary repair. Occasionally in infants with EA–TEF and usually in infants with isolated EA, the distance between the atretic esophageal ends limits the ability to easily complete a tension free, end-to-end primary esophageal anastomosis. During the initial procedure the anastomosis may be attempted under tension. Good proximal and possibly distal mobilization34 of the esophageal segments may be necessary to facilitate this. A variety of surgical techniques have been described to lengthen the proximal and distal pouches to achieve primary repair and preserve native esophagus. One such technique is the proximal esophageal circular Livaditis myotomy to lengthen the upper esophageal pouch and facilitate a primary anastomosis.35,36 A second or third proximal pouch myotomy may be performed via a cervical incision37 and even a distal myotomy can be performed.38,39 Complications such as leak, impaction of food particles in the myotomy segment, pseudodiverticulum40 and impaired esophageal motility41 have been reported following the use of myotomies. A spiral upper pouch myotomy with oblique suture closure of the muscles layer may prevent these complications.42,43 An alternative approach is rows of short, horizontal myotomies performed at different levels circumferentially allowing the upper pouch to lengthen and narrow when stretched.44 An anterior flap may be constructed of the upper pouch, which can then be rolled into a tube for anastomosis.45 The stomach may be mobilized via an abdominal incision to achieve a primary esophageal anastomosis46 or a Collis gastroplasty may be used to lengthen the distal end of the esophagus.47 Each of these maneuvers requires a fundoplication. The presence of severe pulmonary disease is the critical factor that might necessitate a staged repair.48 Infants may be prophylactically paralyzed and ventilated for an arbitrary period of 5 days postoperatively.49 The rationale for this is the prevention of disruptive forces at the anastomotic site by paralysis of the striated muscle in the proximal esophagus. A recent controversial study suggested that absolute gap length was irrelevant to the development of postoperative complications.50 However, this finding has led to questions regarding the method of gap measurement51 and subsequent assertion that vertebral body length was meaningless.52 Methods for a reproducible traction system are continually being sought to try to shorten this gap.53 The management of long gap atresia by delayed closure and bouginage may result in a loss of oral use in the neonate and the baby is at risk for constant

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microaspiration because of bouginage of the proximal pouch. These infants experience the same complications as children with short gap atresias, but at somewhat higher rates.11,15,22 However, over time, these problems subside even in children with long gap atresia.54–56 Thoracoscopic repair Thoracoscopic repair of an EA with TEF is a viable alternative for the pediatric surgeon with advanced laparoscopic skills. The baby is placed in the 451 prone position to allow the lung to fall away from the posterior mediastinum. Three ports are used for access to the thoracic cavity. A fourth port may be used for lung retraction. Carbon dioxide insufflation is used to effect lung collapse. The fistula is ligated using a variety of techniques including a suture ligature or a clip placed on the fistula tract with fistula division distal to it. The anastomosis is performed with either 4–0 or 5–0 PDS, polyglactin (vicryl) or silk. The esophageal anastomosis is performed using either extracorporeal knot tying techniques or intracorporeal suturing.57 The incidence of postoperative complications is similar to the open end-to-end technique including the leak rate, stricture rate, rate of fundoplication, rate of recurrent fistula formation, and revision of the anastomosis57–60 although Rothenberg did report a higher stricture rate at the beginning of his experience.61 Interestingly, disappearance of the surgical clips from the site of surgical repair may be a radiologic harbinger of recurrent TEF.62 Thoracoscopic repair requires a transpleural approach. The operation is difficult in babies less than 2 kg and with significant lung disease since it requires the ipsilateral lung to be compressed with the operative pneumothorax to achieve an adequate working space. The primary advantage lies in the potential for a reduction in the musculoskeletal sequelae that may develop following thoracotomy in the newborn61 although current open operative techniques with muscle-sparing incisions minimize these complications.63,64 Advocates of the thoracoscopic repair feel there is superior visualization of the anatomy within the thoracic cavity.57,61 Additionally, the benefits include less postoperative pain, shorter hospital stay, nipple symmetry, better cosmesis, and less longterm musculoskeletal morbidity such as shoulder movement impairment, rib fusions, and scoliosis.59,65,66 Operative time results are contradictory. Three studies showed similar operative times between thoracotomy and thoracoscopy.57,61,67 However, other studies have demonstrated longer operative times,59,68,69 increased intraoperative pCO2,59,68 and bleeding.69 Increased pCO2 levels may have significant consequences (congenital diaphragmatic hernia) and their long-term effect on neurological development is unknown.70 Additionally, decreased cerebral oxygen saturation has been noted during thoracoscopic neonatal EA and CDH repairs.71 A recent report by Mortellaro et al. sought to eliminate the need for CO2 insufflation by repairing neonates with EA and CDH on high-frequency oscillating ventilator. The median operative time was 208 7 72 min. Mean pH, pO2, and pCO2 were within normal limits.72 van der Zee et al.73 recently described the repair of long gap esophageal atresia by thoracoscopic traction of the two esophageal ends and delayed thoracoscopic anastomosis. Currently, there is a lack of evidence that lower weight children, particularly neonates, are good candidates for robot-assisted thoracic surgery.74 Technical complications of the repair of esophageal atresia The most common complications occurring after EA repair are anastomotic leak and stenosis. Esophageal atresia presents a unique surgical challenge because an anatomic gap exists between the esophageal ends that must be repaired. Several anatomic and

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physiologic considerations exist because of this and affect the integrity of the esophageal anastomosis. The submucosa is the strongest layer of the esophageal wall with the highest suture holding capacity. Anastomotic strength during the early postoperative period relies upon the pre-existing mature collagen network in the esophagus. This facilitates suture apposition of the divided ends of the esophagus. Postoperatively, this polymerized collagen is degraded and synthesis of immature collagen occurs, resulting in low mechanical strength. The thin esophageal wall, longitudinal muscle fiber distribution, absence of the serosal layer, and segmental distal esophageal vascularization also contribute to delayed and/or impaired healing.75 In patients with EA, the distance between the esophageal ends, the tension across the suture line and the extent of mobilization of the esophageal segments are additional factors that further impair anastomotic healing.75 Anastomotic leak Anastomotic leak results from a small, friable lower segment, ischemia of the esophageal ends, excess anastomotic tension, myotomy, sepsis, poor suturing technique, type of suture, excessive mobilization of the distal pouch, and increased gap length.33 The incidence is approximately 15%, but has been reported to be as high as 27%.10 Interestingly, one study observed an increased rate of anastomotic leak if the operation was performed “after hours” (between 3:30 pm and 8:00 am or on weekends or holidays).76 Anastomotic leaks after repair of EA are often subclinical and detected only through an esophagram. Most leaks are managed nonoperatively and seal by 4 weeks.10 Approximately, 5% of leaks require reoperation in the setting of uncontrolled sepsis with persistent fluid collection or pneumothorax. The esophageal anastomosis should be revised if the tissues are healthy enough to hold sutures. Various approaches include simple reanastomosis, pleural patch overlay, intercostal muscle flap buttresses, simple debridement, and pericardial flap interposition.77,78 In severe cases, a segmental esophagectomy with cervical esophagostomy may be required. This necessitates esophageal replacement later in life. The mediastinum and thoracic cavity should be widely drained. Anastomotic stricture Predisposing factors implicated in the pathogenesis of esophageal anastomotic stricture are the use of a two-layer anastomosis, increased gap length, anastomotic tension, type of suture, anastomotic leak, and the presence of gastroesophageal reflux (GERD).33,79 Children with an anastomotic stricture have feeding difficulties and vomiting or dysphagia as their major symptoms. They may have chronic microaspiration resulting in chronic lung disease. In babies, the first symptom is often “slow feeding” and regurgitation with or without cyanotic episodes.80 Surgeons define stricture after EA repair differently and up to 50–80% of patients may develop a stricture.10 Chittmittrapap et al.79 defined patients with swallowing problems, recurrent episodes of respiratory infection or foreign body impaction (including food) and narrowing of the esophagus noted on contrast esophagography or esophagoscopy as having a stricture.79 Initial treatment is with dilation by either bougienage10,79–81 or balloon dilation.82,83 The majority of patients ( 4 80%) respond to less than four dilations.79–81 Complications from dilation such as perforation are rare.81–84 Stricture resection is reserved for patients who fail to respond to dilations (require 4 5 dilations).79,80 The earlier the onset of symptoms, the more dilations are required and the higher the likelihood of needing surgery.80 It is extremely important to delineate the role of GERD in stricture formation. Many times, strictures will respond to dilation once the reflux is controlled; conversely, strictures will recur if resected without addressing the GERD first. Mitomycin C has been used in

the past to moderate fibrosis formation at the anastomotic site and help prevent stenosis85; however, a recent study found no benefit in using mitomycin C to treat stricture.86 Additionally, there is no evidence that prophylactic antireflux medication administration prevents strictures.87 Missed and recurrent TEF Missed TEF should be considered in infants with persistent respiratory compromise postoperatively. The diagnosis is established by bronchoscopy or a modified prone esophagram as the nasogastric tube is carefully retracted under fluoroscopic guidance by a skilled pediatric radiologist. Recurrent TEF occurs in 10–15% of EA repairs,80,88 and typically occurs in the first year of life but may occur up to 10–15 years after repair.80 Recurrence of a TEF is more likely to occur if there has been leak from the anastomosis and following an end-to-side anastomosis. Minimal mobilization of the lower esophageal segment helps prevent a recurrent TEF by minimizing ischemia.80 The majority of patients with a recurrent TEF have coughing and sputtering episodes with feeding, usually associated with cyanosis.80 Diagnosis is made by esophagography, esophagoscopy, and bronchoscopy as described above. Spontaneous closure of a recurrent fistula is unlikely and there is little merit in deferring reoperation. The fistula should be cannulated by bronchoscopy with a Fogarty catheter prior to repair as described for H-type fistulas.89 Thoracotomy with fistula ligation and division is the procedure of choice with a high incidence of native esophagus preservation or reconstruction.90 Techniques such as the use of a pleural flap, pericardial flap, fibrin glue, and azygous vein flap interposed between the esophageal and tracheal suture lines have been used to try to prevent recurrence of the fistula and must be used when repairing a recurrent TEF. Novel techniques such as laparoscopically harvested omental flap interposition between the suture lines have also been described.88 Endoscopic therapies have also been advocated including cautery,91 injection of fibrin glue with92 and without cautery,93–96 cyanoacrylate glue (sometimes with polidocanol),97–102 and laser.103 Endoscopic suturing104 and clipping105 have been reported as well. Injury to the vagus nerve and recurrent laryngeal nerve The vagus nerve and its branches, including the recurrent laryngeal nerve, are intimately associated with the trachea and esophagus. These nerves may be damaged during dissection of the proximal esophageal pouch and distal esophagus. Injury to the vagus nerve and recurrent laryngeal nerve manifests as esophageal dysmotility and vocal cord paralysis, respectively. An incidence of 20% recurrent laryngeal nerve palsy has been reported in patients following EA repair,106 and 3% of patients were diagnosed with vocal fold paralysis after EA repair in another study.107 Esophageal motor dysfunction is almost universal in patients with EA. Its role in the pathogenesis of GERD in patients with EA is becoming increasingly clear. It is unclear if vagal nerve and vagal nerve branch dysfunction after EA repair arises from injury to the nerves during dissection or if an underlying nerve abnormality exists preoperatively. It was initially thought that mobilization of the esophageal ends damaged branches of the vagal nerve, including the recurrent laryngeal nerve.108 Meticulous technique during dissection of the esophageal pouch is mandatory to preserve the vagal branches. However, it has been discovered in the Adriamycin-induced EA rats that the vagus and recurrent laryngeal nerves are absent and/or abnormal.109 Extrinsic and intrinsic innervation, including the vagus and laryngeal nerves, is abnormal in patients with EA with and without TEF.110 Injury to the recurrent laryngeal nerve certainly occurs, but recurrent

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laryngeal nerve dysfunction may be due to developmental abnormalities of the esophagus. Laryngoscopy should be performed to document vocal cord function at the time of the first operation.107 Nevertheless, cautery should be utilized judiciously near the vagus nerve and in the tracheoesophageal groove during mobilization of the upper pouch to minimize the risk of injury to the recurrent laryngeal nerve and superior laryngeal nerve. The authors will often use a combination of biopolar cautery and blunt dissection using Q-tips or Kitners (“peanuts”) to minimize thermal injury. Complications related to associated conditions Tracheomalacia The differential diagnosis of respiratory symptoms following EA repair is anastomotic leak, recurrent TEF, GERD, and tracheomalacia. Tracheomalacia describes a generalized or localized weakness of the trachea that allows the anterior and the posterior tracheal walls to collapse together during expiration or coughing. In addition, the flaccid trachea is easily compressed between the aorta anteriorly and the dilated esophagus posteriorly after EA repair. This situation can be exacerbated by esophageal distension during feeding. Presentation of tracheomalacia varies from a typical “brassy” or “barking” cough in mild cases, to lifethreatening apneic spells or recurrent pneumonia in severe cases. Symptoms typically begin 2 months postoperatively.111,112 The incidence of severe tracheomalacia in patients with EA is 10– 25%.112,113 Establishing the diagnosis includes studies to rule out leak, recurrent TEF and GERD. Bronchoscopy with the child breathing spontaneously is diagnostic of severe tracheomalacia when the anteroposterior collapse of the tracheal lumen during coughing and expiration is greater than 75%. Mild-to-moderate tracheomalacia rarely requires operative repair. Aortopexy to the posterior surface of the sternum is the operative treatment of choice in patients with life-threatening anoxic spells.111,112,114 The approach is either via a left anterolateral thoracotomy,111,114 low transverse cervical incision,115 or thoracoscopically.116–119 The tissue plane between the vascular structures and the trachea should not be dissected to facilitate anterior displacement of the tracheal wall as the vessels are pulled forward.111 Sutures are placed carefully into the adventitia to prevent tearing and life-threatening hemorrhage from the aorta. Outcomes for thoracoscopy are generally good and one large case series reported a recurrence rate of tracheomalacia of 31% within 4 weeks of initial operation. All patients were successfully treated thoracoscopically with a redo aortopexy.119 In patients with concomitant GERD and tracheomalacia, the tracheomalacia is treated first if the patient presents with dying spells or recurrent pneumonias.112 Filler et al.112 recommended that an airway stent be considered for children in whom aortopexy fails to relieve tracheal collapse. Failure of the stent necessitates tracheostomy. Right-sided aortic arch The presence of a right-sided aortic arch occurs in 5% of cases with EA.13 A right-sided aortic arch may significantly complicate exposure and repair of the esophagus from the right side of the chest. Furthermore, a right aortic arch is frequently associated with congenital heart disease or a vascular ring that completely encircles the trachea and esophagus. When the aortic arch is on the right, the upper esophageal pouch lies on the left side of the aortic arch and the distal TEF lies to the left of the descending aorta. Harrison et al.13 reported seven patients with right-sided aortic arch associated EA. Six were discovered at right thoracotomy. They encountered extreme difficulty completing the operation from a right thoracotomy resulting in bilateral thoracotomies, staged repair of the EA and TEF or esophageal

Fig. 2. Right aortic arch noted on CT 3D.

replacement.13 One patient developed a postoperative aortoesophageal fistula and exsanguinated. Another patient developed left vocal cord paralysis, tracheobronchitis, subglottic narrowing, and tracheal stenosis requiring tracheostomy after primary esophageal repair through a left thoracotomy. It is important to determine the position of the aorta preoperatively.120 The gold standard for diagnosis is angiography; however, computed tomography angiography and magnetic resonance imaging has recently become a sensitive, less invasive, viable alternative.13–15,121 The authors20 reported a case of an EA–TEF associated with a right-sided aortic arch, diverticulum of Kommerell, and an “esophageal lung.” A 3-D reconstruction of a CT angiogram was utilized to determine the position of the aortic arch (Figure 2). An umbilical artery line noted on x-ray may indicate the side of the aortic arch. Chest x-ray is not sensitive to diagnose a right-sided aortic arch.14 Echocardiography is operator dependent.13,15 Tracheobronchial remnants Spitz122 was the first to demonstrate a congenital basis for distal esophageal stenosis associated with EA by showing the presence of tracheobronchial rests in an esophagectomy specimen. A de novo congenital esophageal stenosis may be encountered or one may present postoperatively after EA repair. Complications arising from tracheobronchial remnants are signs and symptoms similar to those of postoperative anastomotic stenosis. Gittes' model of EA with TEF formation readily explains the presence of tracheobronchial remnants in the distal esophageal pouch by demonstrating foregut trifurcation in Adriamycin-induced esophageal atresia in Sprague-Dawley rats.7 Two of the tracheal branches formed lungs and the third formed the TEF and distal “esophagus.” It is interesting to note the identification of trifurcated tracheas in infants with EA19 and the association of tracheobronchial remnants with EA.123 A tracheobronchial rest, an esophageal membranous diaphragm and segmental hypertrophy of the muscularis and diffuse fibrosis of the submucosa are different etiologies of congenital esophageal stenosis. Esophageal stenosis is typically diagnosed after EA with TEF repair and infants present with symptoms of esophageal stricture. Bouginage or balloon dilation is recommended. If the stricture and symptoms persist after three dilations, then surgical resection of the stenotic segment with end-to-end anastomosis or esophageal replacement is the treatment of choice.123,124 Because the tracheobronchial remnants are limited to a short 1- to 2-cm length, a limited resection rather than a more extensive resection with esophageal substitution is all that is usually required.124

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infections (24.1%), asthma (22.3%), persistent cough (14.6%), and wheezing (34.7%). The prevalence of Barrett's esophagus (6.4%) was 4 and 26 times higher than the adult (1.6%) and pediatric (0.25%) general populations.126 A rare case of achalasia in an adult with a history of EA repair has also been reported.127

Fig. 3. Congenital esophageal stenosis following EA–TEF repair.

If the pediatric surgeon encounters an esophageal stenosis from tracheobronchial remnants during initial repair the authors recommend the following approach. The anastomosis should be completed and a gastrostomy tube place. The stenosis should be stented with a small nasogastric tube to ensure adequate drainage of saliva. The patient should then be allowed to grow and after the anastomosis has healed the stenosis may be evaluated with an esophageal swallow or computed tomography scan (Figure 3). If obstruction persists, the patient should then undergo resection of the stenotic esophageal segment either via thoracotomy, thoracoscopy, or laparotomy. A Foley catheter may be placed through a gastrostomy and passed proximally above the stenosis and the balloon inflated. The surgeon may then gently retract the esophagus into the abdomen and the segment of esophagus with the tracheobronchial remnants may be resected.125 A postoperative esophagram should be performed 5–7 days postoperatively to check for an esophageal leak (Figure 4). Long-term complications A recent systematic review attempted to outline the prevalence of common long-term problems associated with EA repair in patients older than 10 years of age.126 The main active medical conditions identified were the following: dysphagia (50.3%), gastroesophageal reflux disease (GERD) with (40.2%) or without (56.5%) histological esophagitis, recurrent respiratory tract

Fig. 4. Congenital esophageal stenosis p repair with leak.

Pulmonary The most common short-term postoperative complications following a thoracotomy are pulmonary. Atelectasis and pneumonia are seen in up to 57% of patients who have undergone repair of esophageal atresia.10 Aggressive pulmonary support is necessary to minimize these complications. Late pulmonary function in patients with EA includes a variable incidence of restrictive and obstructive pulmonary disease as well as airway hyperreactivity. In one long-term study comparing a cohort of patients with EA and TEF to normal siblings, the incidence of respiratory symptoms was similar in both groups.128 However, pulmonary function tests were significantly abnormal compared to sibling controls. There was no difference in airway hyperreactivity. Pulmonary function abnormalities did not correlate with the presence or severity of current respiratory symptoms, airway hyperreactivity or current gastrointestinal symptoms.128 There was an increased risk of aspiration in patients with EA and TEF. As discussed above and below, abnormal airway epithelium, excessive tracheal pliability, abnormal tracheal innervation, GERD, and abnormal esophageal motility all increase the risk of aspiration and subsequent risk of pneumonia. There also appears to be no difference in functional residual capacity when comparing thoracotomy to thoracoscopy.129 Respiratory problems tend to improve with age.56 There is a recent report of a 19-year old with history of EA–TEF and VACTERL with pulmonary squamous cell carcinoma.130 Gastroesophageal reflux Gastroesophageal reflux disease (GERD) occurs in 30–82% of patients after EA and TEF repair; 30% of these patients require an antireflux operation.10,54,56,131 The bathing of the anastomotic line in stomach acid results in an increased incidence of stricture and leak. Feeding is affected and GERD contributes to aspiration as discussed above. Patients with long gap atresia and delayed primary repair appear to be particularly susceptible to GERD.132 Diagnosis is made by symptoms and pH probe. Tension on the anastomosis causing shortened intra-abdominal esophageal length and blunting of the angle of His,131 intrinsic esophageal dysmotility,108 intrinsic innervation disorders,110 denervation from extensive esophageal pouch dissection and gastric dysmotility133 all contribute to the pathogenesis of GERD in EA. Interestingly, prophylactic use of antireflux medications has not been shown to decrease the incidence of stricture.87,134 Thoracoscopic repair does not appear to impact long-term incidence of esophageal dysmotility and GERD.135 Long-term consequences of GERD in these patients are manifold. It is important to note that the presence of pathologic changes of the esophagus, such as esophagitis and gastric metaplasia, do not always correlate with symptoms.136 Symptoms tend to decrease over time.54 Consequences of GERD in patients with history of EA last well into adulthood,16 including respiratory symptoms (33%), dysphagia (52%), reflux esophagitis (58%), epithelial metaplastic changes (20%), intestinal metaplasia (Barrett's esophagus) (11–33%) and strictures (42%).137 Distal eosinophilic inflammation138 and proximal heterotopic gastric mucosa at the anastomotic line have been reported.139 In patients with history of EA, men older than 35 years of age and individuals with episodes of reflux greater than three times per week have an increased risk of severe esophagitis and Barrett's esophagus.16 There are now eight reported cases of esophageal squamous cell carcinoma in

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patients with EA including one at the anastomosis.16,137 Long-term follow-up is mandatory to screen for these complications of GERD in EA. The management of GERD is beyond the scope of this article. It is important to note that controversy surrounds the proper operative management of GERD in patients with EA. There is no consistent medical management of EA–TEF postoperatively including choice of antireflux medications and length of treatment.140 Fundoplication becomes necessary in more than 40%, particularly in cases of refractory anastomotic stenoses and long gap EA– TEF.141 Nissen-Rossetti fundoplication has traditionally been considered the best option,142,143 but concerns for debilitating dysphagia and incidence of wrap disruption and recurrent GERD (33%)142 have challenged this notion. Some pediatric surgeons have begun to use partial wraps such as the Thal or Toupet procedure to ameliorate these problems. No clear improved outcome has been demonstrated using any of these procedures.142,143 Some surgeons modify their Nissen technique to create a shorter, floppier wrap (1.0–1.5 cm over a larger dilator).143 In addition, some surgeons have used the Collis–Nissen fundoplication to gain intra-abdominal esophageal length144 with good results.145

Dysphagia Dysphagia is a common symptom in individuals who have undergone surgical repair for EA, although in most patients this is mild and simply managed by drinking fluids with meals. The incidence of dysphagia appears to decrease slightly over time with the majority of patients experiencing this during the first 5 postoperative years (40%). Most patients seem to grow accustomed to dysphagia and reflux symptoms but these symptoms do persist well into adulthood.16,54 Impaired esophageal motility appears to be a major contributing factor to dysphagia.16 Esophageal dysmotility patterns include aperistalsis, pressurization, and distal contractions.146 Distal contraction patterns were found exclusively in Type C EA. Gastric dysmotility has also been noted in patients with EA with dysphagia and dyspepsia.133 However, it is important to note that while gastric dysmotility documented by manometry and scintigraphy may persist to adulthood they are not always responsible for symptoms. The much more common cause is GERD and esophageal dysmotility.133

Long-term quality of life Swallowing function greatly affects the quality of life of children and adults with esophageal atresia. Choking occurs in up to 30% of patients54 and contributes to dysphagia and esophageal foreign body obstruction including food boluses. Almost half of the children reviewed by Little et al.54 weighed less than the 25th percentile during the first 5 years; however, the children's weight and height steadily improved. By the time the children had reached 10 years of age, less than one-third of them continued to have weights or heights less than the 25th percentile. Most patients outgrow symptoms of choking, dysphagia, GERD, growth retardation, and respiratory problems after the first few years of life (4 5 years old),54–56 and enjoy a good quality of life and normal psychosocial function. The incidence of food entrapment requiring esophagoscopy or bouginage, which occurs in 38% of patient,55 only lasts a few years. Patients are able to train themselves to avoid certain foods or help themselves swallow appropriately. Importantly, patients do report feeling isolated when making the transition from childhood to adult medical services suggesting that the pediatric surgeon should claim a larger role in preparing the adult physician to care for these patients.55

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Intestinal atresia Intestinal atresias may occur in the duodenum, jejunum, ileum, or colon and each have special considerations when contemplating potential associated complications. Duodenal atresia Duodenal atresia (DA) and stenosis is a frequent cause of congenital intestinal obstruction occurring in 1 per 5000–10,000 live births, affecting boys more commonly than girls. More than 50% of affected patients have associated congenital anomalies including pancreatic anomalies, intestinal malrotation, esophageal atresia, Meckel diverticulum, variants of imperforate anus, congenital heart disease, central nervous system lesions, renal anomalies and, rarely, biliary tract anomalies. Cardiac anomalies are present in 30% of patients with DA and are responsible for 50% of mortality.147–149 Overall patient survival has improved from 60% to over 90% in recent years.147,150 Down syndrome occurs in approximately 30% of patients with DA, polyhydramnios in 33–50% and 45% are premature.147 The most common type of atresia involves a membrane that partially or completely obstructs the duodenal lumen with no interruption of the duodenal wall. This is referred to as Type 1 atresia and occurs in 90% of cases. Type 2 atresia, which occurs in 1% of cases, consists of a fibrous cord connecting two blind loops of duodenum with an intact mesentery. Type 3 atresia, which occurs in 7% of cases, represents two unconnected blind loops with a V shaped mesenteric defect.147 Operative management and complications Initial management of infants with duodenal atresia consists of fluid resuscitation and gastric decompression. The aim of operative management is to resect an obstructing web if possible or bypass a complete obstruction. Intraoperatively, the gastrointestinal tract should be evaluated for malrotation which is known to be associated with duodenal obstruction. The duodenum should be adequately mobilized via a Kocher maneuver in order for it to be fully inspected; this also helps minimize tension on the anastomosis. Duodenal dissection and anastomosis should be performed with great care since proximal duodenal dilation distorts local anatomy. The point of duodenal obstruction is usually located at the transition point between the dilated and decompressed segments of the duodenum although the presence of a windsock deformity may obscure the location of the obstruction. Once the point of obstruction is localized, a duodenotomy is performed and the lumen inspected. In 5% of cases, a thin membrane is all that is obstructing the lumen and when the location of the ampulla can be determined a lateral resection can re-establish continuity.147 More frequently, a duodenoduodenostomy or duodenojejunostomy is required to bypass the obstruction. The proximal duodenum is mobilized then brought down and anastomosed to the duodenum distal to the obstruction. Occasionally, when anastomosis to the duodenum is not possible the proximal duodenum is anastomosed to the jejunum. Many of the complications associated with the management of duodenal atresia are related to this anastomosis (long-term complications).147 An annular pancreas may be encountered intraoperatively. It is unclear if an annular pancreas, when present, is responsible for duodenal obstruction or is merely an associated anomaly. The management, however, does not change and the anastomosis in this case should be performed anterior to the abnormal pancreatic tissue which should not be violated in order to avoid pancreatitis or pancreatic leaks and fistulas. The authors strongly recommend consideration of placement of a gastrostomy tube in infants with

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Down syndrome and associated cardiac disease that may require one or several operations. Intraoperative complications The ampulla of Vater is at risk for injury during dissection or construction of the anastomosis. Boyden et al.151 studied the location of the ampulla of Vater in patients with duodenal atresia and noted that the ampulla is usually present in the immediate vicinity of the point of obstruction. This proximity places the ampulla at risk of injury during surgical repair.148 Gently squeezing the gallbladder and observing bile flow should identify the ampulla in order to minimize the risk of injury both before and after the anastomosis. When a thin membrane is the only source of obstruction and a membrane excision is planned, the excision should be restricted to the lateral aspect of the membrane in order to avoid injuring the ampulla. A tapering duodenoplasty is sometimes considered to improve postoperative bowel function, although this is controversial (see below). Studies have shown that tapering the duodenum prior to anastomosis is associated with an increased risk of injury to the ampulla. The disrupted anatomy of the dilated duodenum places the ampulla at higher risk of injury.152 Persistent mechanical obstruction is another potential postoperative complication of duodenal atresia repair. Obstruction may be a result of a missed second atresia or a windsock membrane. A second distal atresia is present in around 5% of patients. If such a lesion is not identified and corrected, patients will have a persistent obstruction that may jeopardize the proximal anastomosis. A preoperative contrast enema should be performed to identify any associated colonic atresia. Intraoperatively, saline should be injected into the bowel lumen and milked distally in order to check for concomitant small bowel atresias. Another source of persistent obstruction, present in 5% of patients, is a windsock membrane. A windsock membrane is an obstructing membrane that is stretched distally within the lumen of the duodenum. On external examination, the observed point of transition in caliber of the duodenum is actually distal to the origin of obstruction. If such a variant were not recognized, an anastomosis would be ineffective because of its location distal to the site of obstruction. Close inspection usually identifies a small area of constriction of the wall of the duodenum which marks the origin of the membrane and the actual point that needs to be bypassed.153 The presence of a windsock membrane is confirmed by passing an orogastric catheter into the lumen of the duodenum. If a windsock membrane is present, pushing on the catheter results in tenting of the duodenal wall proximal to the transition point. Laparoscopic duodenal atresia repair Laparoscopic repair of duodenal atresia is one of the most demanding pediatric laparoscopic surgical procedures.154 Following initial reports touting successful completion of laparoscopic repairs,155,156 there followed a period of time with a relative lack of new publications describing the outcomes of this technique due to an unacceptable anastomotic leak rate when compared to the open technique.155,157 This leak rate was felt to be directly attributable to intracorporeal suturing technique. Improvements in laparoscopic technique and experience have subsequently led to improved outcomes.154 Alternatively, the use of Nitinol U-clips (Medtronic Surgical, Minneapolis, Minnesota) has demonstrated comparable complication rates when compared to the open technique.157 In a recent series excellent feasibility was reported, with one patient requiring a reoperation. However, three patients were lost to follow-up.158 A specific criticism of the laparoscopic technique regards missing a second intestinal atresia. As noted above, a second

intestinal atresia may be noted in up to 5% of cases,147,148 which if missed may result in postoperative obstruction distal to the anastomosis and threaten the proximal anastomosis. Traditionally, at the time of operative repair, a small, red rubber catheter should be passed distally through the distal enterotomy to evaluate for a second mucosal web.147,148 Laparoscopic surgeons, however, highlight the outstanding visualization of the bowel during this operation and the presence of a second atresia would be obvious externally during intestinal evaluation. The authors note that a mucosal web may not be readily visualized extrinsically. Early postoperative complications Delayed anastomotic function is one of the main complications associated with repair of duodenal atresia. Factors related to both the design of the anastomosis and intrinsic poor function of the proximal dilated duodenum may contribute to this problem. The original management of duodenal atresia consisted of a side-toside duodenojejunostomy. Patients frequently required gastrostomy tubes with transanastomotic feeding because of a delayed return of bowel function. Gastrostomy tubes are associated with their own set of complications such as leaks, skin irritation, dislodgement, mechanical failure, gastroesophageal reflux, and persistent gastrocutaneous fistulae. The advent of the more physiologic duodenoduodenostomy, described by Weitzman et al. in the 1960s159 and then modified to the diamond shaped anastomosis technique described by Kimura et al.,160 have greatly improved the results of this operation. The diamond-shaped duodenoduodenostomy results in quicker resumption of feeds and shorter hospital stay when compared to side-to-side duodenoduodenostomy and side-to-side duodenojejunostomy.161 Because of the quicker return of intestinal function, gastrostomy tubes and transanastomotic feeding tubes are no longer necessary except in special circumstances.162 Intrinsic dysfunction of the proximal dilated duodenum may also contribute to delayed anastomotic function. Dilated segments of intestine have been shown to be ineffective in bolus propulsion regardless of the adequacy of peristaltic function. Even with normal peristalsis, the walls of a sufficiently dilated intestine cannot establish a pressure gradient within the lumen because they cannot coapt well.163 Studies have shown that an anastomosis functions sooner when the proximal dilated intestinal segment is resected or the size of the lumen reduced.148,164 The two main solutions to a dilated duodenum are a tapering duodenoplasty and duodenal plication.165 The tapering duodenoplasty involves the resection of part of the wall of a dilated duodenum followed by primary repair or using a GIA stapler. A duodenal plication entails imbricating the redundant duodenal tissue, which avoids the long suture line associated with a duodenoplasty.166 Although reducing the caliber of the lumen has been shown to be beneficial in patients who present with postoperative functional obstruction from a dilated duodenum, the same cannot be said about the benefit of a prophylactic tapering procedure when a dilated duodenum is encountered during the initial operation.152 Some reports suggest that tapering or plicating the duodenum during the initial operation may prevent late bacterial overgrowth (see below); however, the exact amount of dilation that would benefit from a prophylactic tapering is unclear.167 The extent of duodenal dilation observed at the initial operation has not been shown to predict outcome and recommendations on prophylactic tapering procedures cannot be made based on intraoperative findings.147 Late complications Long-term follow-up into adulthood is mandatory due to a 12% rate of long-term postoperative complications.147 Complications

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include late-onset duodenal dilation, blind loop syndrome, anastomotic stricture, GERD, duodenogastric reflux, and small bowel obstruction (SBO). There are two cases of intussusception related to duodenojejunostomy. The first was a retrograde jejunoduodenal intussusception causing acute pancreatitis in a 33-year-old patient who underwent duodenojejunostomy in infancy for DA.168 The second was a retrograde jejunojejunal intussusception just distal to the anastomosis in a 9-month old.169 Late-onset duodenal dilation (megaduodenum) is a condition that may result in a functional bowel obstruction despite early successful feeding. Progressive dilation of the duodenum following anastomosis may result in a functional obstruction months to years postoperatively.167 Patients with a sufficiently dilated duodenum may experience failure to thrive, vomiting, abdominal pain, and blind loop syndrome.147,170 The dilated stagnant duodenum with blind loop syndrome may cause a functional obstruction and malabsorption secondary to bacterial overgrowth. The functional obstruction is a result of poor propulsion of food through the dilated lumen. Late onset functional obstruction secondary to a dilated duodenum occurs in 5% of patients. Earlier studies reported a 20% rate of duodenal dilation; however, those studies evaluated patients who had undergone a side-to-side anastomosis.164,171 Patients with a symptomatic dilated duodenum can be managed with either duodenoplasty or duodenal plication. The more prevalent use of the diamond shaped anastomosis has decreased the rate of duodenal dilation, probably due to an improved anastomotic patency. Stagnation and bacterial overgrowth may also occur if the distal duodenotomy is not positioned close enough to the site of obstruction leaving a segment of bowel which is not in continuity with flow. This condition was more common when a duodenojejunostomy was performed.162,167,172 Anastomotic stricture or stenosis may be responsible for late-onset obstructive symptoms. The widespread use of the diamond-shaped anastomosis has also made stenosis an uncommon problem.147,162 Gastroesophageal reflux requiring operative intervention occurs in 8% of patients after duodenal atresia repair.147 A certain degree of functional obstruction distal to the stomach, secondary to poor intestinal motility may account for this finding.173,174 Duodenogastric reflux may also be present in some patients. Radiologic evidence of duodenogastric reflux can be seen in 30% of patients after duodenal atresia repair.170 This may cause peptic ulceration and esophagitis from chronic exposure to alkaline biliary fluid. Duodenogastric reflux may be alleviated by prokinetic agents.172

Jejunoileal atresia Jejunoileal atresia (JIA) occurs distal to the ligament of Treitz. Conditions such as abdominal wall defects (gastroschisis), cystic fibrosis, malrotation, and intrauterine volvulus or intussusception may coexist with JIA.149,164 The etiology is believed to be an ischemic insult to the intestines that occurs during the later stages of fetal development. Studies have identified a higher incidence of Factor V Leiden mutations in patients with intestinal atresia when compared to the general population (4% versus 18%).175 Associated conditions in patients with JIA are not as frequent as they are with duodenal atresia and do not impact patient outcome to the same extent. The one exception is coexisting atresias, which should be evaluated for intraoperatively.149 The major causes of mortality in patients with JIA are complications related to short gut syndrome and prolonged use of total parenteral nutrition (TPN) with the vast majority of late mortality resulting from infectious complications of TPN.149 An interesting point to note is that the operative mortality for JIA is less than 1% as compared to a 4% operative mortality in duodenal atresia. This

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lower early mortality is attributed to the less frequent association of JIA with other anomalies, specifically congenital heart disease. The Grosfeld classification system separates jejunoileal atresia into four types.176,177 Type 1 consists of a mucosal defect with intact mesentery. Type 2 defines a lesion where a fibrous cord connects two atretic bowel ends. Type 3a indicates two atretic ends with a V shaped mesenteric defect. Type 3b refers to an apple peal lesion, where a single vessel supplies the distal piece of bowel. Type 4 denotes multiple atretic segments of bowel.149 Small bowel atresia is present in 10% of patients with gastroschisis.149,178,179 Some atresias may not be recognized intraoperatively because of the associated thick peel enveloping the intestines of patients with gastroschisis and are later identified during evaluation for a persistent postoperative obstruction. When an intestinal atresia is discovered during initial evaluation of gastroschisis several options for management are available. The atresia can be repaired primarily, observed and repaired at a later date or fashioned into an ostomy. Recent evidence suggests early operation (r21 days of life) has similar outcomes to later operation (4 21 days of life).180 Primary repair may be considered when the intestines have minimal peel and primary closure of the abdomen is anticipated. This may not be feasible in the setting of a thick peel or if a synthetic material is used because the anastomosis is at an increased risk of breakdown. If the atresia is not addressed at the time of gastroschisis reduction, delayed repair after a period of bowel decompression and parenteral nutrition is usually achievable since the peel softens with time.181 Stoma formation is generally restricted to cases where an intestinal perforation is present or the viability of bowel is in question. Overall, 10% of patients with JIA have associated cystic fibrosis.149 The surgical approach to this group of patient should include steps to evacuate the inspissated meconium, aggressive postoperative respiratory and nutritional support while the workup for cystic fibrosis is underway. Malrotation coexists with JIA in 10% of cases182 and should be addressed when the condition of the intestines permits.

Disease-related complications Patients with JIA may suffer from delayed onset of oral feeding. In one study, meconium peritonitis, luminal discrepancy, number of anastomoses, presence of immature ganglion cells, and short bowel syndrome were factors related to prolonged feeding difficulties. Prematurity increased the duration of hospitalization without affecting time to full oral intake.183 Short bowel syndrome may lead to prolonged feeding difficulties. Short bowel syndrome (SBS) is a condition where not enough intestinal length is available for maintenance of a patient's nutritional needs. It is a quantitative and/or qualitative deficiency in bowel function that results in malnutrition, weight loss, and diarrhea. The length and condition of residual intestinal segments, presence of an ileocecal valve, along with the operation performed are major determinants of outcome in terms of SBS. Patients with an apple peel deformity or multiple atresias are at highest risk for SBS, especially if the small intervening segments of intestine between points of atresia are resected. Innovative surgical techniques such as multiple anastomoses stented by a silicon tube have been developed in order to preserve bowel length and thus avoid SBS.184,185 Short bowel syndrome, which occurs in 25% of patients with JIA, is a major cause of morbidity and mortality.149,186 Management of SBS focuses on promoting adaptation of the intestine. Adaptation is a process that involves morphologic and functional modifications of the residual intestine as it attempts to keep up with nutritional requirements. Exposure of the intestinal cells to nutrients plays a key role in adaptation. The method of administration as well as the composition of enteral feeds affects

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this process. Early, gradual, and continuous feeding with formula containing complex nutrients and low in carbohydrates is advantageous since complex nutrients present more of a “work load” for the intestine and thus are more effective in promoting adaptation than simple nutrients. The advent of parenteral nutrition has made a marked contribution to the outcome of patients with SBS, allowing survival of patients with as little as 20 cm of residual bowel.187 Unfortunately, sepsis and parenteral nutrition-associated liver disease continue to be a significant cause of morbidity and mortality. Liver failure occurs in around 25% of patients with JIA receiving parenteral nutrition, most of whom are either too small for transplant or do not survive long enough to be transplanted. The prevention of catheter-related blood stream infections has also decreased the morbidity of patients with JIA and SBS. In one study, the authors changed from 70% isopropanol alone (Iso-sachets from Griffiths and Nielsen Ltd, Billingshurst, UK) to 2% chlorhexidine in 70% isopropanol (Clinell wipes from Gama Healthcare, London, UK). The wipes were used to clean the central venous catheter (CVC) connectors at the time of accessing the CVC. Chlorhexidine central venous catheter antisepsis significantly reduced the incidence catheter-related sepsis.188 Operative intervention for SBS is usually reserved for patients who reach a plateau with bowel adaptation and continue to require TPN to meet their nutritional needs and is usually not offered until at least 1 year has passed with nonoperative management. Several surgical options aimed at increasing transit time, intestinal length, or improving intestinal motility are available with variable success rates.189,190 Bowel transplantation is reserved for patients who fail all the above attempts at improving bowel function and continue to be dependent on hyperalimentation.

Procedure-related complications Many of the postoperative complications of JIA are related to the intestinal anastomosis, particularly in patients who have multiple atretic segments. Laparoscopy has been described in assisting localization of the atresia with good cosmetic results.191 Postoperative complications include adhesive bowel obstruction, functional anastomotic obstruction, and anastomotic leaks and strictures. With the less frequent use of stomas in these patients, complications of stomal prolapse and strictures are seldom encountered. Adhesive bowel obstruction is a complication of most intraabdominal operations and many patients recover with nonoperative management.149 A more difficult condition to manage is persistent functional obstruction of the anastomosis. This is a situation where there is poor flow across an anastomosis despite it being of adequate caliber and can occur if the proximal dilated intestine is not addressed prior to repair of the atresia. The walls of dilated loops of intestine are incapable of proper apposition which may explain their poor ability to propel food. Resection of dilated bowel, in an attempt to circumvent this problem, is frequently not feasible due to the increased risk of short bowel syndrome. This makes a tapering enteroplasty or intestinal plication more attractive options. A tapering enteroplasty or intestinal plication may help improve intestinal function without compromising bowel length and should be considered when a patent anastomosis associated with dilated proximal intestinal loops fails to function. Another cause of persistent obstruction despite a successful anastomosis is a missed second atresia. Five percent of patients with JIA will have more than one atresia that, if missed, will cause persistent obstruction and may compromise the more proximal anastomosis. In order to avoid such a situation, a preoperative contrast enema must be performed to clear the colon.

Intraoperatively, saline should be injected into the intestinal lumen and milked distally to help identify concomitant atresias as in duodenal atresia. Other anastomotic complications, including leaks and stenosis occur in a small percentage of patients. In a study by Dalla Vecchia et al.,149 the rate of anastomotic leak was 4% in patients treated for JIA. Colonic atresia Colonic atresia (CA) is a rare cause of intestinal obstruction occurring in less than 10% of all intestinal atresias.149,192 Colonic atresia is classified as follows: type 1 consists of a mucosal defect with intact mesentery and bowel wall; type 2 defines a lesion where a fibrous cord connects two atretic bowel ends; and type 3 indicates two atretic ends with a V-shaped mesenteric defect (most common).192 Associated anomalies are rare with CA and include gastroschisis (2.5%),181 Hirschsprung disease (rare),193 complex urologic anomalies, multiple small intestinal atresias, an unfixed mesentery, and skeletal anomalies.194,195 The diagnosis of CA is an indication for urgent operation as the risk for perforation is higher than in JIA.195,196 The coexistence of Hirschsprung disease is assessed by rectal biopsy.193 Initial end colostomy with subsequent resection and anastomosis is typically performed due to high rates of complications with a primary anastomosis.196,197 Survival is typically excellent in the absence of other comorbidities if diagnosed early (4 90%).193 A delayed diagnosis 472 h may result in a mortality 460%.195 This is attributable to a closed loop obstruction that forms between a competent ileocecal valve and the atresia leading to massive colonic distension and perforation. Outcomes are generally worse in the setting of gastroschisis due to a high rate of intestinal necrosis and associated high mortality.198

Necrotizing enterocolitis Necrotizing enterocolitis (NEC) is a condition involving an inflammatory process of the gastrointestinal tract of newborns. It is the most common gastrointestinal emergency seen in the neonatal intensive care unit.199 Patients at highest risk for NEC are preterm low birth weight infants who have received enteral feedings. The pathophysiology of NEC is believed to be related to the combination of ischemic changes to intestinal mucosa, bacterial colonization of the gastrointestinal tract and the presence of a substrate such a formula in the gut lumen.200 Once NEC is diagnosed, patients are started on broad spectrum antibiotics, placed on bowel rest and followed up with serial abdominal exams, plain films, and complete blood counts. Approximately half of the patients with NEC will require operation because of evidence of intestinal perforation, including free intraperitoneal air on an abdominal radiograph, stool, bile, or pus found at paracentesis or clinical evidence of perforation in the joint opinion of the attending surgeon and the neonatologist.201 Disease-related complications Necrotizing enterocolitis may progress to intestinal gangrene and perforation in around 30–50% of patients,201,202 and in this group, mortality may reach 30–40%.202 Intestinal perforation, which is evident by free air on abdominal plain films, is generally considered the only absolute indication for surgical intervention. The extent of intestinal injury in NEC ranges from segmental to massive intestinal involvement (NEC totalis). Intestinal failure and short bowel syndrome (SBS) may develop when residual intestinal

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function or length is insufficient to meet the nutritional demands of the infant (Intestinal Atresia). Short bowel syndrome is the most common long-term complication in NEC patients and occurs in around 10% of survivors.199 Short bowel syndrome from necrotizing enterocolitis is one of the main reasons for intestinal transplantation in infants.203 During operative exploration of patients with extensive intestinal involvement, the surgeon must attempt to preserve as much intestinal length as possible in order to avoid SBS. Deciding on the extent of bowel resection can be difficult (see below). Several operative strategies have been devised to attempt to preserve as much intestinal length as possible. These will be discussed later in the procedure-related complications. Intestinal stenosis may also complicate the course of medically treated NEC. Intestinal stenosis is a narrowing of the intestinal lumen due to either fibrosis (stricture) or edema and ulceration (without fibrosis) and results from the preceding ischemic insult. Schwartz et al.204 performed routine screening contrast enemas on patients after medical management of NEC and noted a 35% rate of stenosis (7 of 28 patients). Three of the seven patients manifested symptoms of intestinal obstruction; two patients had blood in their stools; and two patients were asymptomatic (one went on to develop signs and symptoms of obstruction within 1 month of discharge). Intestinal stenosis was detected an average of 3 weeks after the episode of NEC and tended to occur in the left colon. Schwartz et al.205 then followed up their retrospective study with a prospective observational trial that confirmed a stenosis rate of 36% and observed that half of asymptomatic patients progress to symptoms of intestinal obstruction after hospital discharge. Frequent follow-up of asymptomatic patients with repeat enemas was recommended to follow the progression of the stenosis.204,205 Interestingly, Tonkin et al.206 noted that some patients with asymptomatic stenosis who were re-evaluated with contrast enemas were found to have normal intestinal caliber signifying resolution of the stenosis. Recurrent NEC despite initial successful medical or operative management is rare. Infants may have one or more recurrent episodes of NEC in 6% of cases.207 In a study by Stringer et al.,207 infants with recurrent NEC were noted to be either premature or mature with major associated cardiac anomalies. One patient had an superior mesenteric artery (SMA) thrombosis and experienced four episodes of recurrent NEC. No consistent association was noted between the recurrent episode(s) of NEC and the type or timing of enteral feeds or the anatomical site or method of management (medical versus surgical) of the original attack. The majority of patients with recurrent NEC were successfully medically treated. The mortality rate of 12.5% mirrored that of primary NEC and mortality was due to liver failure related to parenteral nutrition (including the patient with the SMA occlusion).207 Procedure-related complications Patients treated for NEC are a heterogeneous group with variable degrees of illness and comorbidities. This makes comparing the outcomes of different modalities of therapy difficult. This is evident in several studies comparing laparotomy to peritoneal drainage in infants with NEC. These studies are confounded by the preferential treatment of smaller and sicker infants by peritoneal drainage and larger and less sick ones by laparotomy. The two different modalities offer theoretical advantages in terms of preservation of intestinal length. On the one hand, peritoneal drainage avoids potentially unnecessary intestinal resection and gives marginally viable segments of intestine a chance to recover that might have otherwise been resected; on the other hand, laparotomy and intestinal resection may result in more rapid control of the disease process by eliminating the source of sepsis.

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A plethora of surgical options exist at laparotomy and include resection with or without anastomosis, stoma creation, “clip and drop,”208 and “patch, drain, and wait.”202 The heterogeneity of the patient population and the multiple confounding factors related to the management of these patients makes data interpretation difficult. Some infants may be too unstable to safely transport to the operating room. Surgery performed in the NICU has long been an option in these difficult situations. However, Wright et al. reported 67% of neonates dying within 6.5 h of operation and a further 13% after months in hospital. The study posed the question whether surgery is always in the patients' best interests.209

Laparotomy versus drainage Two large, multicenter, prospective, randomized controlled trials (RCTs) have been completed comparing laparotomy to peritoneal drainage for perforated NEC: the North American NECSTEPS trial201 and European NET trial.210 NECSTEPS recruited infants r1500 g and o34 weeks premature. NET limited inclusion to those infants r1000 g. Differences between the two trials included sample sizes, randomization criteria, postoperative care pathways, and techniques of peritoneal drainage. In NECSTEPS, irrigation through the peritoneal drain until effluent cleared and manual compression of the peritoneal cavity to empty stool and pus were encouraged. In the NET trial, irrigation and other maneuvers were discouraged, and investigators in the NET trial were allowed to proceed to “salvage” laparotomy if the patient's clinical status did not improve considerably following drainage.202 In contrast, NECSTEPS investigators rarely performed “salvage” laparotomy.202 Both studies identified survival rates for peritoneal drainage and laparotomy that were not statistically different.210 Overall, 74% of patients assigned to peritoneal drainage ultimately underwent laparotomy in the NET trial210 which was markedly higher than in the NECSTEPS trial.201 Peritoneal drainage was considered an effective definitive treatment in only 11% in the NET trial compared to an overall success rate of 33% in the NECSTEPS trial.202 The NET trial concluded that 74% of neonates treated with primary peritoneal drainage required delayed laparotomy. There were no significant differences in outcomes between the two randomization groups. Primary peritoneal drainage was ineffective as either a temporizing measure or definitive treatment.211 If a peritoneal drain was inserted, a timely “rescue” laparotomy should be considered.210,211 NECSTEPS concluded the type of operation performed for perforated necrotizing enterocolitis did not influence either survival or other clinically important early outcomes in preterm infants.201 A recent Cochrane Review noted evidence from the two RCTs suggested no significant benefits or harms of peritoneal drainage over laparotomy as primary therapy. However, due to the very small sample size, clinically significant differences may have easily been missed. No firm recommendations could be made for clinicians in this review.212 The NEST Trial is currently underway to address this question. The Laparotomy vs. Drainage for Infants With Necrotizing Enterocolitis (NEST) (ClinicalTrials.gov Identifier: NCT01029353) is a randomized controlled trial to compare the effectiveness of laparotomy versus drainage for treating NEC or intestinal perforation (IP) in extremely low birth weight infants.213 The Neonatal Research Network's observational study of 156 ELBW infants with NEC or IP showed comparable outcomes for the two procedures before hospital discharge, but suggested an advantage of laparotomy over drainage at 18–22 months corrected age with lower rates of death or neurodevelopmental impairment.214 However, as noted above, the infants that underwent laparotomy were more mature; infants with drains were smaller and more premature.

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Postoperative complications occur in 50% of patients treated for NEC.201,202 A study by Horwitz et al.199 showed a 63% rate of complications after peritoneal drainage and 44% after laparotomy. The rate of complications in that study was independent of the indication for operation, whether it was failure of medical therapy or perforation, and was higher in patients treated with drainage, possibly because that group of patients tended to be sicker. Complications related to surgical intervention included postoperative sepsis, intestinal stricture formation, short bowel syndrome, wound infections, stoma complications, bowel obstruction, and intra-abdominal abscess formation. Postoperative sepsis occurred in 7% of patients who underwent laparotomy, and 19% of patients who underwent peritoneal drainage. Sepsis was the main postoperative complication in both these two groups and was more frequent in infants weighing less than 1000 g, presumably because of a more immature immune system.199 The significance of postoperative sepsis was an associated 30-day mortality of 50%. The presence and severity of preoperative sepsis were not addressed in that study, so it is unclear if the poor outcome was because the infants were initially sicker. Intestinal strictures Intestinal strictures are a known complication of NEC and result from ischemic changes to the affected intestinal segments. Some studies have shown that patients who undergo laparotomy with bowel resection have a lower incidence of intestinal stenosis than those treated with peritoneal drainage. Horwitz et al.199 noted that intestinal stenosis was twice as common in patients who underwent peritoneal drainage than those who underwent a laparotomy (15% versus 7%). This increased rate of stenosis is believed to be related to the fact that, with peritoneal drainage, the most affected segments of ischemic intestine are left behind and those are more prone to stricture formation.199 Another theory behind the development of strictures in patients with NEC relates to the role of the absence of enteric contents in distal intestinal segments of patients treated with resection and stoma formation. O'Connor et al., in a study of patients after stoma formation, noted a 40% rate of intestinal strictures distal to the site of diversion. Intestinal content may play a role in decreasing the rate of stricture formation, both by a local trophic effect and through mechanical dilation of the intestinal lumen.215 Preservation of intestinal length The two objectives of the surgical management of NEC are to resect nonviable bowel and preserve as much salvageable intestinal length as possible. It is this balance that may mean the difference between insufficient bowel resection and SBS. Deciding on the extent of bowel resection depends on intraoperative judgment of intestinal viability and the procedure planned. Cikrit et al.216 reported on patient outcome after surgical management of NEC and noted a 38% rate of SBS; the majority of these patients underwent intestinal resection and enterostomy. Resection and primary anastomosis, despite its appeal and advantage of avoiding potential complications of stoma formation, may inherently promote excessive intestinal resection as the surgeon attempts to obtain clearly viable intestinal margins suitable for a safe anastomosis. As discussed earlier, procedures have been devised to help preserve as much intestinal length as possible, particularly in cases of massive intestinal involvement (i.e., NEC totalis). Procedures designed to preserve intestinal length include proximal diversion, the “clip and drop”208,217 technique, and “patch, drain, and wait”218 approach.219 When a diverting enterostomy is constructed proximal to the intestinal segments involved with NEC, the aim is to divert enteric content away from ischemic but potentially viable

bowel while it recovers from its injury. The peritoneal cavity may be drained, but intestinal resection is avoided. Once the systemic inflammatory process is controlled, a second operation is performed, usually weeks to months later, and the ostomy is taken down and any stenotic or nonviable bowel is resected. Luzzatto et al.220 reported a decrease in the length of diseased intestinal segments at re-exploration when compared to the initial operation. Unfortunately, the patients are exposed to the risks associated with stoma construction, such as stricture formation, parastomal hernias, wound complications, the need for bowel resection, and anastomotic complications after reversal.221 The stoma also predisposes patients to complications of high ostomy output such as fluid and electrolyte abnormalities. Another operative strategy aimed at reducing the amount of bowel resected is the clip and drop back technique.208,217 With this procedure, only clearly nonviable intestinal segments are resected. Residual intervening segments are dropped back into the abdominal cavity. A second look operation is later performed and based on the viability of residual intestinal segments, they are either anastomosed or further resection performed, and a third look operation planned. This technique has been described in patients with extensive disease or NEC totalis. Two studies were associated with high mortality presumably from the sick patient population but survivors were associated with increased intestinal preservation.208,217 Still another technique aimed at the preservation of intestinal length is the “patch, drain, and wait” approach, where intestinal contents are evacuated through existing intestinal perforations which are then closed using either imbricating sutures or patched with adjacent segments of intestine.218 The basic principle behind this approach is avoiding bowel resection and enterostomies, thus minimizing the chance of SBS. Drains are placed into the peritoneal cavity and no further operative intervention performed for the next 14 days, while the patients receive antibiotics and TPN. Moore218 reported their experience with 23 patients treated in this manner with excellent results. Stoma formation Stoma formation is generally considered the safest approach to the patient who needs intestinal resection for NEC. The combination of unprepped bowel with questionable viability and a patient who is systemically ill makes the option of a stoma appealing. This offers the advantage of avoiding an anastomosis in suboptimal conditions within the peritoneal cavity between intestinal segments that may not have sufficient perfusion, which predisposes to complications such as strictures and anastomotic leaks. Patients are also frequently malnourished and a stoma allows time for optimization of nutritional status prior to formation of an anastomosis. Despite their appeal, enterostomies are not without complications. Potential problems include skin excoriation, strictures, parastomal hernias, prolapse or intussusception, and high output resulting in fluid and electrolyte abnormalities, particularly in more proximally located enterostomies.219–221 Additionally, enterostomies divert luminal contents away from distal bowel. This diversion may be associated with an increased rate of intestinal strictures because luminal contents may prevent stenosis by virtue of their nutritive and mechanical properties.215 A recent systematic review found no quality evidence supporting either early (r8 weeks) or late (4 8 weeks) closure of the ostomy, and no recommendations could be made either way.222 Primary anastomosis In selected patients with NEC, intestinal resection with primary anastomosis may be safely performed. The advantage to primary anastomosis is that it avoids the complications associated with

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enterostomies and the need for a second operation. Additionally, establishing intestinal continuity early may help decrease the rate of distal stricture formation and accelerate distal intestinal adaptation and thus help avoid short bowel syndrome. The argument against primary anastomosis is that performing an anastomosis between segments of bowel with potentially compromised blood flow and in a suboptimal milieu may increase the risk of complications such as anastomotic leaks and strictures. In addition, and in an attempt to reach healthy intestinal segments suitable for anastomosis, surgeons may be forced to resect potentially viable bowel and increase the risk of short bowel syndrome. Studies comparing resection and anastomosis to enterostomy formation report variable results.223–226 Unfortunately, selection bias in these retrospective studies where primary anastomosis might have been more readily performed in healthier patients with less extensive disease makes interpretation of the results difficult. If primary anastomosis is chosen, distal irrigation of the bowel to washout residual stool is vital in order to help avoid anastomotic breakdown. An international multicenter RCT led by Great Ormond Street comparing stoma formation with primary anastomosis following intestinal resection for NEC at laparotomy was completed in 2013 and patients are no longer accruing. The hypothesis to be tested was that primary anastomosis after intestinal resection offered significant advantages to neonates with NEC including more rapid recovery of the intestine and therefore shorter duration of time to full feeding. The results of the STAT Trial have not yet been published to the authors' knowledge.227 Laparoscopy Laparotomy is the gold standard for the management of surgical NEC. However, laparoscopy may be useful in situations when it is uncertain if a patient needs an operation or not.228–230 Laparoscopy in NEC was first reported by Pierro et al.230 to visualize and assess the health of the bowel in suspected NEC, particularly in cases when hard signs to operate were missing. In this series, the bowel was normal in 2 of 11 total patients and NEC confirmed in 9/11. Urgent laparotomy was avoided in eight infants, in one of whom a laparoscopically guided ileostomy was performed. In five infants, a Penrose drain was inserted at laparoscopy; three of these did not require further surgery and the remaining two underwent delayed laparotomy. Three infants, all with intestinal gangrene, died. The presumed benefit of laparoscopy is to identify those patients in whom further operation is not needed avoiding further surgical stress.229 Subsequent reports have been published regarding the use of diagnostic laparoscopy.228,231,232 In patients with perforations or necrotic bowel, the authors would then convert to an open procedure. A recent systematic review revealed laparoscopy was able to exclude NEC in 9% of patients, and confirm NEC in 91% of patients. Eight (18%) infants did not require further surgery following laparoscopy: four (9%) had no evidence of NEC, two (5%) had no evidence of perforation and/or intestinal gangrene, and two (5%) had NEC totalis precluding further surgery.228 One infant (5%) subsequently required laparotomy for a missed perforation.

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are rare (o2%) and associated with other congenital anomalies.237 Associated malformations in CDH range from 10% to 50%238–244 and include skeletal anomalies (32%) such as limb and costovertebral defects241,245; cardiac defects (24%) including cardiac hypoplasia, ventricular septal defects, tetralogy of Fallot, transposition of the great vessels, double outlet right ventricle, and aortic coarctation242,246,247; and tracheobronchial defects (18%) including congenital tracheal stenosis, tracheal bronchus, and trifurcated trachea.245 Puri and Gorman235 found a 100% association of stillborn infants with CDH and associated lethal anomalies including neural tube defects. Pathology The pathology associated with CDH includes anomalies of intestinal rotation and fixation (malrotation), lung hypoplasia, and pulmonary hypertension. Follow normal herniation of the midgut into the yolk sac, the abdominal viscera herniate through the lumbocostal trigone into the intrathoracic cavity if the pleuroperitoneal canal has not closed. This results in abnormal position of the bowel and its improper rotation and fixation.248 The stomach is frequently in the chest resulting in a degree of obstruction causing dilation and ectasia of the esophagus.248 Visceral herniation including liver, spleen, stomach, and bowel results in lung hypoplasia and lung maldevelopment in both sides with a worse severity on the ipsilateral side.249 This results in potentially significant respiratory compromise due to a decreased number of bronchial branches250 and abnormally developed alveoli251 bilaterally. A decreased number of abnormally muscularized pulmonary arterioles may result in fixed and intractable pulmonary hypertension.248,252–254

Right-sided CDH Special mention is made of a right-sided defect. The right lobe of the liver may occupy the majority of the hemithorax and may in fact fuse to the lung parynchema.255 The hepatic veins may drain ectopically into the right atrium resulting in significant complications during attempted repair of the diaphgramatic defect.248,256,257 Right-sided defects (R-CDH) are associated with a higher incidence of congenital anomalies than left and are also associated with a higher mortality rate in at least one European multicenter study.258 In R-CDH, prenatal diagnosis and patch repair correlated with mortality. However, the morbidity following repair of R-CDH was not significantly different from that in L-CDH in survivors.258 A retrospective analysis of the Canadian Pediatric Surgery Network found no difference existed in perinatal risk factors (gestational age, illness severity, and associated anomalies), preoperative treatment intensity (use of vasodilators, inotropes), timing of surgery, ventilation days, need for ECMO, LOS, and overall survival.259 Significantly increased differences were noted for patch repair rate and recurrence of R-CDH compared to L-CDH.259 Outcomes

Congenital diaphragmatic hernia Epidemiology Congenital diaphragmatic hernias (CDH) are estimated to occur from 1:2000 to 5000 births.233–236 The majority of defects occur on the left side (80%) and 20% occur on the right side in the posterolateral position (Bochdalek's hernia). A discussion of Morgagni hernias is beyond the scope of this article. Bilateral defects

CDH is a physiologic emergency, not a surgical one.248 Recognition of the effects of lung hypoplasia and pulmonary hypertension combind with postponing surgery until after clinical stablization have improved survival rates dramatically over time. Treatment modalities such as utilization of gentle ventilation techniques and extracorporeal membrane oxygenation (ECMO) used to amerliorate and correct pulmonary hypertension have resulted in survival of infants with isolated CDH reaching above 90%.236,248,260

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High-quality outcomes are diffiult to determine for CDH due to the single center retrospective nature of available reports. Databases such as the CDH Registry, the Canadian Pediatric Surgery Registry, and the Euro Consortium Group are addressing low patient numbers by combining data from various centers. The VICI-trial suggests that gentle conventional ventilation has equivalent outcomes compared to high frequency oscillatory ventilation (HFOV) and may even be superior regarding shorter ventilation time and lesser need of extracorporeal membrane oxygenation.261 Additionally it is becoming apparent that the existence of a protocolized approach to CDH management262 and treatment of CDH at high volume centers improve survival rates.263 The use of surfactant236,264 and nitric oxide (NO)236,265 have not been shown to improve survival. In fact, surfactant use resulted in a lower survival rate in preterm infants compared to full term infants264 and NO use did not decrease requirements for ECMO.265 Even though the survival rate has increased, long-term morbidity associated with CDH including chronic lung disease and pulmonary hypertension is being increasingly recognized.266 Operative therapy and complications Optimal timing of operative repair of a CDH now favors following physiologic stabilization of the infant267 but no difference in survival is noted between immediate and delayed repair in a Cochrane database study.268 Traditional therapy includes a subcostal incision or thoracotomy.248 Technical considerations include careful dissection of solid organs including the spleen and liver to minimize injury to these structures.248 A hernia sac is encountered in 20% of patients and this must be excised to minimize the risk of recurrence.269 Great care is taken to dissect the posterior rim of the diaphragm to facilitate primary repair if at all possible. If the posterior rim is diminutive or nonexistent a primary repair may be accomplished by suturing a generous anterior rim directly to the ribs. If the defect is too large, or ECMO precludes extensive dissection, a prosthetic non-absorbable patch [GORE-TEXs Soft Tissue Patch (Gore Medical, Flagstaff, Arizona)]) may be inserted which may minimize intra-abdominal closing pressures.270 Complications of patch usage occur in 10–50% of patients and include infection, dislodgement and subsequent reherniation.271 Reherniation may present with bowel obstruction, respiratory distress or may remain asymptomatic.272,273 Omentum has also been described as an autologous patch in animal models.274 Reduction of intrathoracic abdominal contents may result in unacceptably high intra-abdominal pressures with attempted closure of the fascia due to congenital lack of domain. In these circumstances, simple closure of the skin and creation of a ventral hernia that can be closed at a later date may be considered. If skin closure is not possible, placement of a temporary prosthetic silo may be performed to avoid abdominal compartment syndrome. An ipsilateral tube thoracostomy is not indicated unless there is significant bleeding or air leak noted at the time of repair. Additional procedures such as appendectomy or Ladd procedure is not indicated and is, in fact, contraindicated in the possible setting of ECMO.248 A detailed review of ECMO is beyond the scope of this article. Nevertheless, a number of surgical issues are involved in the management of CDH patients while on ECMO which may result in complications. Surgical repair on ECMO, an accepted surgical strategy, is associated with hemorrhagic complications in 60% of patients.275,276 Interestingly, an increased incidence of hemorrhage was noted in nonsurvivors compared to survivors in a review of the ELSO registry.276 Partridge et al. recently reported outcomes were improved in CDH patients undergoing surgical repair following ECMO with significantly increased survival, lower rates of

surgical bleeding and decreased total duration of ECMO therapy compared to patients repaired on ECMO. In patients who can be successfully weaned from ECMO, their study supports a role for delayed repair off ECMO with reduced operative morbidity and increased survival.277 Regardless, the majority of patients still undergo repair of their CDH after their ECMO run.278 Thoracoscopic repair Laparoscopic and thoracoscopic techniques to repair CDH have been described.279,280 Late presentations of Bochdalek hernia may be particularly amenable to laparoscopic or thoracoscopic repair.281–288 The thoracoscopic approach may be superior to laparoscopy for repair of a Bochdalek hernia as the insufflation of the CO2 in the thoracic cavity helps reduce abdominal organs.289 There is a case report of using a biologic mesh during a thoracoscopic repair [Surgisis soft tissue graft (small intestinal submucosa) (Cook Biotech Inc., Lafayette, Indiana)] rather than a prosthetic one with a good result but an excessively high operative time.290 Although a variety of case reports and series touted success and pushed the age of repair to neonates,229 a meta-analysis of thoracoscopic cases by Lansdale et al.291 found higher recurrence rates and longer operation time but similar survival and patch usage compared to open surgery. Similar results were reported by Chan et al.292 and noted a higher recurrence rate when a patch was used. Additionally, a variety of technical and physiologic consequences including high failure rates and increased pCO2 levels have been reported.289 There is increasing evidence that due to absorption of insufflated CO2 thoracoscopy in neonates may have serious neurotoxic side effects such as severe acidosis.229 A pilot randomized controlled trial showed that thoracoscopic repair of CDH was associated with prolonged and severe intraoperative hypercapnia and acidosis compared with open surgery. These findings led to the trial's Data Monitoring and Ethics Committee to not support the use of thoracoscopy with CO2 insufflation and conventional ventilation for the repair of CDH calling into question the safety of this practice.293 As noted above, a recent case series reported successful repair of CDH thoracoscopically on HFOV.72 Success rates for completion of minimally invasive approaches in neonates range around 67%.280 Recurrent diaphragmatic hernia Repair of a recurrent diaphragmatic hernia may “present a formidable surgical challenge.”248 In one study, 46% of patients with patch repairs and 10% of those with primary repairs had a hernia recurrence at a median time of 0.9 (range: 0.1–7.3) years after repair.294 Repair is most commonly attempted through the abdomen but may be facilitated by thoracotomy. The most common organs to reherniate are the small and large intestine and attempt to repair may be hampered by adhesions. A primary repair of the recurrent diaphragmatic defect is preferable if feasible, but several techniques for mesh insertion have been described as well including a prosthetic “plug.”271–273 A laparoscopic approach to repair of a recurrent CDH has been described as well.295 Biologic mesh Controversy continues to surround the use of a biologic mesh to repair a CDH. Repair with biologic absorbables have been studied in animal models with initially promising success,296 and a metaanalysis showed no difference in the incidence of CDH recurrence, SBO, or mortality post-CDH patch repair, when compared to non-absorbable mesh.297,298 However, other studies are

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contradictory.299,300 Use of an absorbable patch was associated with the highest risk of surgical complications294,300 in two studies. An additional study demonstrated unacceptably high rates of SBO, recurrence and second recurrence comparing nonabsorable patches to both SIS and AlloDerms Tissue Matrix (LifeCell, Bridgewater, NJ).298,299 Late complications Associated congenital heart disease continues to be the greatest risk factor for poor short and long-term outcomes.301,302 Late mortality has been noted in 10% of survivors mainly due to persistent pulmonary hypertension or iatrogenic injuries.303–305 In particular, if a patient required ECMO support as a neonate the risk of late mortality is significantly higher.305 Additionally, patients with CDH requiring ECMO have a higher probability of developing late morbidity including respiratory, nutritional and musculoskeletal issues.306 Scoliosis and pectus deformity were common in children with large CDH in one recent study.307 The choice of a muscle flap or prosthetic patch did not appear to affect the incidence of subsequent skeletal deformity. The most common long-term issues faced by CDH survivors are pulmonary. Although the CDH lung continues to mature and demonstrate alveolar division for several years after birth, it ultimately never reaches a normal number of alveoli compared to a healthy lung.306,308 Further, the abnormal alveoli may become emphysematous resulting in compromised function even in the setting of subsequent remodeling of the vascular bed resulting in larger and less muscular arteries.308 Treatment strategies for these patients include oxygen, bronchodilators, corticosteroids, and diuretics.248 Persistent pulmonary hypertension impacts longterm survival as well.248 Gastroesophageal reflux disease (GERD), enteral feed supplementation or dependence and esophageal dysmotility may commonly be seen in patients with history of CDH.309,310 Hiatal hernia in patients with a history of CDH was a significant risk factor for GERD in one study.311 Antireflux procedures may be indicated in cases of medically refractory GERD. Nutritional deficiencies and growth related problems have also been described in CDH survivors.308 Other long-term morbidities associated with CDH include neurodevelopmental delays, neurological complications including visual disturbances, hearing loss, seizures, extra-axial fluid collections, enlarged ventricles and abnormal electroencephalograms, urologic complications including vesicoureteral reflux, cryptorchidism and nephrolithiasis, recurrent diaphragmatic hernia, chest wall deformities such as pectus excavatum and scoliosis, and SBO.311,312 The strongest predictor of SBO was patch repair.294,311

Abdominal wall defects This section will focus on the two most common abdominal wall defects: gastroschisis and omphalocele. However, rarer defects exist. Severe variants of omphalocele result from variations of errors in pleuroperitoneal canal folding including the pentalogy of Cantrell and cloacal exstrophy. A minor variation with the appearance of an omphalocele is an umbilical cord hernia. Ectopia cordis thoracis occurs in the midline sternum and contains the heart. Prune belly syndrome is characterized by the congenital abscess of the abdominal wall musculature in the otherwise present normal layers of the abdominal wall.313 Omphalocele Omphalocele is a large midline abdominal wall defect covered by a sac from which the umbilical cord extends. The sac usually

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contains the liver, intestines and, occasionally, spleen and gonads. The incidence is 1–2.5:5000 live births.313 Babies are often full term. Umbilical cord hernias have a normal rectus compared to an omphalocele and rarely have associated anomalies; thus, this may be seen as a separate entity rather than a mild variant of omphalocele.313 Only midgut is found in the sac. Although most cases have malrotation it is rarely a cause of intestinal obstruction.313 Omphalocele has multiple associated conditions which may impact outcomes including cardiac conditions, chromosomal abnormalities, Down syndrome,314 macrosomia,315 GERD (highly associated with esophagitis),316 undescended testicle (UDT) (at one year of age)317 and musculoskeletal and neural tube defects. Cardiac anomalies include peripheral pulmonary stenosis, persistent pulmonary hypertension, ventricular septal defect, atrial septal defect, ectopia cordis, coarctation of the aorta, dysplasia of the tricuspid valve, and pericardial effusion.318 Chromosomal anomalies include trisomy 21, 18, and 13, deletion of the short arm of chromosome 5, unbalanced translocation involving chromosomes 4 and 15, triploidy, Klinefelter's syndrome, and Beckwith–Wiedemann syndrome with mosaic duplication 11p15.319 Musculoskeletal and neural tube defects include limb deformities (arthrogrypotic in nature), spinal deformities, osteopenia, mild digital deformities, developmental or spondylodysplastic scoliosis, and other congenital spinal deformities.320,321

Treatment All patients require preoperative resuscitation with intravenous fluids, gastric decompression with an orogastric tube, administration of vitamin K, and possibly ventilatory support. An echocardiogram is mandatory given the high incidence of associated congenital cardiac anomalies. There is no clear indication of whether primary closure versus staged repair of the omphalocele is superior. Surgical options include primary repair or staged reduction followed by repair. Primary repair may be accomplished in the operating room after the sac is excised and the fascia is repaired with absorbable suture. The abdominal wall may require manual stretch anteriorly and posteriorly to accommodate the herniated abdominal contents. Complications that may arise include abdominal compartment syndrome if the closure is too tight and ventral hernia if only the midline attenuated fascia is incorporated into the closure.313 If a primary closure is not possible, use of a prosthetic silo that will allow slow reduction of the viscera is indicated. Alternatives include a preformed Silastic bag with a spring-loaded ring322,323 or a hand-sewn sheet of 0.007-in-thick Dacron-reinforced Silastic.313 Another option for moderate sized omphaloceles with a relatively thick sac is to use the sequential ligation of the sac itself for a gradual reduction of the viscera.229 The bowel is typically manually reduced over 7–10 days. It is then secured by a number of ways including tying umbilical tape, suturing the bag over long clamps, mechanical stapling, or applying umbilical cord clamps. The authors have experience using the gastroschisis wringer clamp (GWC). The GWC is an autoclavable, 140-g, aluminum alloy device reminiscent of an old wringer washing machine. It consists of two opposing serrated rollers that pull the Silastic silo through a slotted base plate. This protects the intestine and converts the circular defect into a vertical slit to ease delayed primary closure. The GWC is adjusted daily on the awake newborn in the nursery and the magnitude of each adjustment is gauged by the infant's cardiac and pulmonary status.324 While certainly an elegant method designed to facilitate a more natural closure after the bowel was safely returned into the peritoneal cavity, it is not commercially available and at times can be cumbersome to manage, especially in a premature infant.

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Alternative, manual reduction may not be required and the abdominal contents may spontaneously reduce.325 Most surgeons will close the abdominal wall by 10–14 days due to increased risk of infection and loss of compliance of the abdominal wall creating difficulty in achieving a tension free closure. Giant omphalocele poses a particular challenge. Attempts to repair the defects with absorbable mesh followed by skin closure or graft may be complicated by fistula formation.326 Use of GORE-TEX covered by skin flaps or grafts resulted in universal failure due to infection and sloughing.313 The use of biologic mesh such as AlloDerm has been described in the repair of a giant omphalocele associated with cloacal exstrophy.327 Seromas (4), wound infection (2), and incisional hernias (2) complicated the use of Surgisis in one report of 24 patients.328 Component separation technique (CST) in conjunction with absorbable biologic mesh has also been successfully employed to close giant omphaloceles.329 In these cases CST included dissection of abdominal wall subcutaneous tissue from the muscle and fascia, and an incision of the external oblique aponeurosis 1-cm lateral to the rectus sheath. Nonoperative therapy (painting the sac with an antiseptic) is a useful therapeutic alternative.313 The goal of nonoperative therapy is to promote escharification and epithelialization of the omphalocele sac to facilitate delayed closure of the giant omphalocele. The initial painting agent was mercurochrome330 but toxic side effects were later described.331–333 Silver sulfadiazine painting has subsequently been used as a safe and effective bridge to delayed closure334 and recently use of a silver impregnated hydrofiber dressing was described.335 The side effects of silver sulfadiazine and silver products in general have been meticulously studied in burn care. Side effects include allergic reactions to the sulfadiazine moiety, silver staining, hyperosmolality, methemoglobinemia, and hemolysis due to a congenital lack of glucose-6-phospate dehydrogenase.336 Leukopenia is no longer considered a side effect. Nevertheless, excluding a report of two patients with increased serum levels of silver,337 long-term studies have not demonstrated any complications from silver sulfadiazine therapy in omphalocele.334,338 Topical povidone-iodine is an alternative antiseptic commonly available in the hospital setting. However, because transient hypothyroidism may occur thyroid function studies must guide inpatient therapy.339 Neomycin and polymyxin/bacitracin ointments have also been used.229 Successful negative pressure wound therapy has also been recently described independently340 and in conjunction with a biologic mesh [Strattice reconstructive tissue matrix (LifeCell Corp., Branchburg, NJ)].341 Once the sac has epithelialized gentle pressure may be applied to reduce the ventral hernia. A ventral hernia can usually be repaired at one year of age without the necessary use of a prosthetic patch.313 Gastroschisis Gastroschisis is a smaller defect compared to an omphalocele located to the right of the umbilicus in the vast majority of cases and is not covered by a sack. The intestines are herniated through the defect and occasionally a gonad may be found eviscerated as well. The bowel may have a matted appearance that makes it difficult to distinguish individual loops. The exposed bowel provides a large surface area for heat loss necessitating a more aggressive approach to perioperative fluid management. The incidence is increasing342 and currently between 2–4.9:10000 live births.343,344 It currently surpasses that of omphalocele.313,343,344 There is a higher incidence of prematurity and babies small for gestational age.229,313 Of note, GERD, UDT, and musculoskeletal and neural tube defects are reported in gastroschisis as well as omphalocele. The midgut is not normally rotated by virtue of being congenitally herniated and intestinal atresias are associated with gastroschisis.149

The baby is typically placed in a plastic drawstring bowel bag to prevent heat and fluid loss.345 Primary repair may often be facilitated immediately after delivery346 or bowel may be placed in a silo for sequential reduction either routinely347 or if primary abdominal closure cannot be accomplished. The techniques for closure are similar to that of omphalocele described above. Outcomes comparing primary closure to silo placement with delayed repair (typically using a spring-loaded silo) vary according to studies. In one study, length of stay and ventilator days were higher for the staged closure group; infection and mechanical complications, including necrotizing enterocolitis, were less common in the staged closure group and the time to full feeds did not differ between groups.348 Another study found significantly lower airway pressures, earlier extubation, fewer complications, and decreased length of stay and hospital charges in delayed repair following silo placement.347 Yet another study found decreased ventilator times, shorter times until first postoperative feeding and full feedings, fewer complications, less necrotizing enterocolitis, and a decreased length of hospital stay for patients with staged closure.322,323 A technique in which the bowel is reduced in a delayed fashion without facial closure was first described by Bianchi and Dickson349,350 and has been popularized by Sandler as a “plastic technique.”351 The plastic technique (sometimes referred to as the “Iowa Patch”) has been gaining popularity as the primary repair technique of choice for gastroschisis in some centers.351–354 The technique was popularized with general anesthesia or sedation351 but was initially described and touted to not require anesthesia.349,350 Plastic closure allows nonoperative management without general anesthesia at the patient's bedside in comparison with surgical closure that must be performed under general anesthesia in the operating room.352 The gastroschisis defect is covered with the umbilical cord tailored to fit the opening, and two Tegaderms (3M Healthcare, MN) dressings reinforce the defect (“plastic closure”).351 Outcomes are generally promising: nutritional management (time to start feeds, time to reach goal feeds, and time on parenteral nutrition), length of hospital stay, were similar to conventional surgical closure for patients and there was less likelihood of developing infection or sepsis complications.351–354 Long-term follow-up of patients with the plastic technique revealed normal growth with minimal bowel complications.354 There is a high incidence of umbilical hernia formation following plastic closure but relatively few operative repairs are needed after a year of age.351–354 The cosmetic result is generally pleasing.355 Nevertheless, this technique has been associated with complications including abdominal compartment syndrome, enterocutaneous fistula and technical difficulty reducing the bowel.356 Although the plastic technique is gaining in popularity it may not be the panacea for the treatment of gastroschisis. Surgical management must still be tailored to the needs of the individual patient. Complications Complications in gastroschisis may be related to prematurity and gastrointestinal tract anomalies. Complications in omphalocele are often related to associated anomalies. Patients with gastroschisis and ruptured omphalocele frequently have hypovolemia and require aggressive volume resuscitation guided by urine output. In both gastroschisis and omphalocele, an atresia can be reduced into the abdomen without a stoma and be repaired six weeks later after the abdominal wall has healed.313 Patients with a history of omphalocele or gastroschisis may require additional surgery for adhesive bowel obstruction. As these patients are not typically treated for malrotation at time of abdominal wall repair they could theoretically develop a devastating midgut volvulus

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with subsequent short bowel syndrome.357 A common reoperation for both groups also includes repair of abdominal wall hernias.358 An abdominal compartment syndrome may result from a closure that is too tight necessitating a reopening of the abdomen in the operating room. Symptoms may include respiratory compromise, decreased venous return and low cardiac output, renal failure, sepsis, abdominal wall dehiscence, enterocutaneous fistula, and congested lower extremities. Reduced splanchnic perfusion pressure will lead to oliguria and gut mucosal acidosis and potentially bowel ischemia. Bowel ischemia may develop into NEC which may lead to a loss of bowel resulting in short bowel syndrome.229 In omphalocele, kinking of the hepatic veins may lead to a metabolic acidosis. Operative correction includes opening the fascia and skin closure, or placement of a silo for delayed repair.313 Strategies to avoid abdominal compartment syndrome include intraoperative and postoperative measurement of intraabdominal pressure via a Foley catheter or nasogastric tube. During intraoperative closure it is helpful to observe the inspiratory pressures necessary to achieve a constant tidal volume. Increasing pressures may provide indication of early abdominal compartment syndrome. Special mention is made of preformed spring-loaded silos in gastroschisis. While these prefabricated silos are certainly easy to use and may avoid general anesthesia they do carry risks and complications such as enlargement of the fascial defect (44% of cases), silo displacement (15%), and need of mesh at time of abdominal closure (19%) in one study.359 Additionally, the congenital defect may need to be surgically opened to accommodate the ring. Closure of an ultimately round fascial defect using a ring compared to a vertical slit from a hand-sewn silo may make delayed primary closure more difficult. Outcomes Short term Survival for simple gastroschisis is typically 95%360–362 and omphalocele up to 95%.313 The presence of associated anomalies is the strongest predictor of morbidity and mortality in fetuses or neonates with omphalocele363 and prematurity, intestinal atresia, stenosis, volvulus or perforation for gastroschisis (the latter comprising the so called “complex gastroschisis” as described by Molik).179,313,364 Complex gastroschisis or omphalocele associated with congenital anomalies drop the survival rates to 70– 75%.179,313,360 Infants with gastroschisis and omphalocele almost universally experience a slow return of bowel function with no significant difference between the two in at least one study365 but it is suggested to be slower for gastroschisis than omphalocele in another.313 Small bowel bacterial overgrowth may complicate the tolerance of feeds and patients may require a regimented antibiotic bowel decontamination program during the first year of life.366 Babies with complex gastroschisis may require multiple abdominal operations and long-term use of central venous catheters,178 especially in those who develop short bowel syndrome. It is now increasingly recognized that adhesive SBO is a frequent and serious complication in the first year after treatment of congenital abdominal wall defects particularly in the setting of sepsis and fascial dehiscence.367 It may carry a mortality rate as high as 15%.367 Long term Patients with isolated small omphaloceles have few long-term issues but these patients are in the minority. A number of longterm medical problems have been reported in up to 60% of infants

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with giant omphalocele including GERD, pulmonary insufficiency, recurrent lung infections or asthma, and feeding difficulty with failure to thrive.229 Many children require enteral access for feeding, and severe GERD poses a particularly difficult challenge as fundoplication may be technically difficult or impossible. Nevertheless, as patients with a history of omphalocele pass the toddler years they begin to match their peers for height and weight indicating that feeding difficulties do ultimately resolve.229 A third of patients with omphalocele report intermittent abdominal pain persisting into young adulthood,229,367,368 but lung volumes and oxygen consumption are normal on long-term follow-up.369 Those with abdominal complaints were usually evaluated as nonspecific or functional or related to constipation.367,368 The greatest long-term concern for patients with giant omphalocele was cosmesis.368 This was mirrored in the gastroschisis population as well.360,368 Nearly one half of patients were dissatisfied with the lack of an umbilicus and the associated large abdominal wall scar.229,368–370 However, this did not impact their overall quality of life which was comparable to that of healthy young adults,229,358,368–370 although 12% reported low selfesteem.368 Laparoscopy later in life is feasible in patients with a history of abdominal wall defects who later require abdominal operation.371 The surgeon performing laparoscopy must be aware of the fact that the liver may be in a nonanatomic position and may be directly under the umbilicus. A preoperative ultrasound is helpful to avoid this pitfall. Patients with Beckwith–Wiedemann syndrome (omphalocele, macroglossia, and gigantism) require regular follow-up to screen for associated Wilms tumor and hepatoblastoma.372 Interestingly, rheumatoid arthritis was found more frequently than in the general population (7% of cases).368 The outcome of the majority of patients affected by gastroschisis is considered good in terms of growth in 75% of cases373 but those with complicated gastroschisis generally only reached the 25th percentile for growth.370 Children born with gastroschisis have similar 2-year neurodevelopmental outcomes as nonsurgical, nonsyndromic neonatal intensive care unit children of similar gestational age and birth weight. Both groups of children have a higher rate of enrollment in early intervention than their healthy peers. These data suggest that neurodevelopmental outcomes in gastroschisis children are delayed secondary to prematurity rather than the presence of the surgical disease.374 Nearly one-third of patients with gastroschisis required special classes or being held back a grade but all reported normal physical activity.360 Ultimately, the majority of patients with congenital abdominal wall defects have a quality of life not different from the general population.368,370 The single most significant impactful outcome in gastroschisis is short bowel syndrome. Fortunately, only 13% of patients with gastroschisis had short bowel syndrome and were totally or partially dependent on TPN in one series.364 Two-thirds eventually weaned off TPN but the risk of malabsorption and nutritional deficiencies are potentially present throughout life. Those that remain dependent on TPN represent the main cohort of candidates for subsequent intestinal transplantation. Finally, a few considerations regarding gynecologic and urologic outcomes are worth mentioning. Women with a history of gastroschisis are able to reproduce368 but the presence of adhesions367 may have a deleterious long-term effect regarding readmission rates for gynecological concerns.375 Approximately, 50% of patients with gastroschisis born with an eviscerated testicle will have spontaneous migration of the testicle into the scrotum by one year of age, while o 10% of patients born with omphalocele will.376 One study suggests primary orchiopexy at time of congenital abdominal wall defect repair to improve rates of testicular salvage.377

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Conclusions Improvements in pediatric surgical outcomes have changed the face of short- and long-term complications following neonatal operations in the past 60 years. Rigorous follow-up is mandatory to monitor for the development of these complications. A thorough understanding of potential complications combined with an understanding of embryology, anatomy, and physiology are essential to successfully manage neonates with surgical disease into adulthood.

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