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Hiatal and paraesophageal hernia repair in pediatric patients
Author’s Accepted Manuscript Hiatal and paraesophageal hernia repair in pediatric patients Erin M Garvey, Daniel J Ostlie
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To appear in: Seminars in Pediatric Surgery Cite this article as: Erin M Garvey and Daniel J Ostlie, Hiatal and paraesophageal hernia repair in pediatric patients, Seminars in Pediatric Surgery, http://dx.doi.org/10.1053/j.sempedsurg.2017.02.008 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting galley proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
Hiatal and Paraesophageal Hernia Repair in Pediatric Patients
Erin M Garvey, MD & Daniel J Ostlie, MD Department of Surgery; Phoenix Children’s Hospital
Corresponding Author: Daniel J Ostlie, MD Surgeon in Chief and Chair of Surgery Department of Surgery; Phoenix Children’s Hospital Phoenix Children’s Hospital 1919 E Thomas Road Phoenix, AZ 85016 Phone 602-933-7003 Fax 602-933-0394 [email protected] Abstract Hiatal and paraesophageal hernia (HH/PEH) can be congenital, resulting from embryologic abnormalities/genetic predisposition, or acquired, most commonly after gastro-esophageal surgery such as fundoplication. Minimizing circumferential esophageal dissection at the time of Nissen fundoplication has been shown to decrease the risk of acquired HH/PEH from 36.5 to 12.2%. Gastrointestinal, respiratory and constitutional symptoms, including anemia and failure to thrive, are common with high rates of associated gastroesophageal reflux. Chest x-ray is often abnormal and upper GI confirms the diagnosis. Treatment is surgical with the goal of reducing the hernia contents, excising the hernia sac, closing the crura and performing an antireflux procedure. The laparoscopic approach is safe and effective.
Key Words Hiatal hernia, Paraesophageal hernia, Pediatric, Mesh, Recurrence
Introduction In his 1853 review of 88 postmortem cases of diaphragmatic hernia, Henry Ingersoll Bowditch noted three cases wherein there was dilation of the esophageal opening and described what is thought to be the first report of a type II paraesophageal hernia. (1) A hiatal hernia (HH), also known as a type I or sliding hernia, occurs when the gastroesophageal junction (GEJ) abnormally protrudes cephalad into the chest through the esophageal hiatus. Types II-IVn HH’s are known as paraesophageal hernias (PEH) and are the least common congenital diaphragmatic hernias. A type II PEH occurs when the gastric fundus herniates through the esophageal hiatus alongside the lower thoracic esophagus while the GEJ remains in the appropriate intra-abdominal location. Type III PEH’s represent a combination of types I and II with an intra-thoracic position of the GEJ and herniation of the gastric fundus. The term giant PEH is used when >30% of the stomach is herniated into the chest. Type IV PEH’s involve herniation of other intra-abdominal contents, in addition to the stomach, such as colon, small intestine, spleen, or omentum. (2) This review will detail the historical perspective, epidemiology, presentation, diagnosis, treatment and outcomes of HH/PEH in the pediatric population.
History Well before Bowditch’s review in 1853, there were a number of descriptions of congenital and posttraumatic diaphragmatic hernias including those by Batista Morgagni and Vincent Alexander Bochdalek. However, it was not until 1900 that a HH was diagnosed by means of X-ray, first by Hirsch prior to autopsy and later by Eppinger in live patients. (1, 3) Shortly thereafter, in 1925, Julius Friedenwald and Maurice Feldman correlated the symptom of heartburn to the presence of a HH. (4) The first report on elective surgical repair of HH was published by Angelo Soresi in 1919 detailing an abdominal approach, reduction of the hernia and closure of the hiatus for which he described “the suture has to close in the most perfect manner the opening of the diaphragm especially around the organs that pass through it, esophagus, vena cava, aorta, but without compressing the important organs just mentioned” – surgical principles that are still pertinent today. (5) The current surgical approach to the hiatus is overwhelmingly laparoscopic, a technique first described by Cuschieri in 1992. (6) The present day focus continues to be on the perfect manner by which to protect and close the hiatus with variations in degree of dissection, suture choice and placement, and mesh reinforcement of the crural closure. Epidemiology Etiology and Embryology HH/PEH are either congenital or acquired, the latter of which can be the postoperative consequence of gastro-esophageal surgery or, less commonly, the result of traumatic injury. There are a number of embryological hypotheses as to why these hernias form; however, the true etiology is not fully understood. During development, the pneumoenteric recesses are two small coelomic spaces which evolve on either side of the midline of the mediastinum, and persistence of the right recesses results in a flattened and elongated mesothelial lined space within the esophageal
hiatus. (7, 8) This space has been measured as approximately 1 cm at birth and likely obliterates during normal development into adulthood suggesting persistence of this right recess may predispose to HH/PEH development. Other hypotheses include laxity in gastric attachments, deficient diaphragmatic hiatus and aberrant development of the lumbar component of the diaphragm origination from mesoderm cells around the aorta. (9-11) Any surgical dissection at the esophageal hiatus resulting in disruption of the phrenoesophageal membrane can predispose to the development of HH/PEH. The most common pediatric procedure during which this could occur is fundoplication for gastroesophageal reflux, and the risk of acquired HH/PEH has been shown to be related to the extent of peri-esophageal dissection. A prospective randomized controlled trial during Nissen fundoplication comparing circumferential esophageal mobilization to minimal dissection leaving the phrenoesophageal membrane intact found a postoperative herniation rate at 1 year minimum follow up of 22.7% for the circumferential dissection compared to 2.8% for the minimal dissection group. (12) The five year minimum follow up study to the original trial showed an increase in postoperative hernia rates for both groups (36.5% for the circumferential dissection group versus 12.2% for the minimal dissection group). (13) The importance of minimizing esophageal dissection at the time of initial Nissen fundoplication to decrease the risk of post-operative, acquired HH/PEH cannot be over emphasized. HH/PEH has also been reported after surgical intervention for esophageal atresia and tracheoesophagel fistula. (14) As for post-traumatic HH/PEH, in his review of 216 adult cases, Marchand determined 5% of hiatal hernias were the result of a traumatic incident, most commonly involving a crush injury, but there is no pediatric literature regarding acquired traumatic HH/PEH. (15)
Incidence and Patient Population In 370 patients who underwent laparoscopic Nissen fundoplication over a six year period, 8.1% required a second operation for repair of an acquired HH/PEH with fundoplication transmigration, and the incidence of post-operative herniation at five year follow up from the prospective trial described above ranged from 12.2 to 36.5% depending on the extent of esophageal dissection performed. (13, 16) The true incidence of congenital PEH, on the other hand, is unknown, but this diagnosis is felt to be uncommon. In the Montreal series of 14 patients of congenital PEH, type III PEH’s were the most common at 57% versus 29% for type II and 14% for type IV PEH. (18) There have only been a few case reports of type IV PEH in the pediatric population. (18) A summary of the larger case series examining congenital PEH is shown in Table 1. There was no gender predilection from these studies, and patients were most often diagnosed within the first two years of life. Other congenital abnormalities including malrotation, trisomies, and Marfans syndrome have been associated with PEH. (9, 18-20) In keeping with the embryological hypotheses above, the question of a familial mode of inheritance has also been raised as there have been a number of case series of HH/PEH within families suggesting a genetic predisposition. (9, 20, 21) Clinical Presentation Patients with either congenital or acquired HH/PEH can be asymptomatic or, more commonly, present with a variety of gastrointestinal, respiratory and/or constitutional symptoms. Not surprisingly, symptomatic gastroesophageal reflux was present in 50-68% of patients with PEH. Recurrent respiratory tract infections, emesis, anemia and failure to thrive were other common presenting symptoms. In the Montreal series, emesis was the most common symptom for Type II PEH, while respiratory distress was most commonly seen in type III and IV PEHs, with failure to thrive and anemia also seen in type III. The feared complication of volvulus was reported in three
of five patients in a series from Turkey. In all three of these patients, the volvulus was all organoaxial rotation where the greater curve of the stomach rotates horizontally along the longitudinal axis between the pylorus and the gastroesophageal junction. (2, 19) Given the rarity of HH/PEH, clinicians must maintain an index of suspicion to proceed with radiographic evaluation in pediatric patients who present with these sometimes vague symptoms. Physical exam is often unrevealing, but one may hear borborygmi (bowel sounds) on auscultation of the chest. Diagnostic Evaluation Evaluation often begins with an anteroposterior and lateral chest x-ray (CXR). One series reported seeing a cystic mass on all CXRs corresponding to the hernia diagnosis in all cases, while another series reported a correct presumptive diagnosis was only made in 29% of cases based on clinical symptoms and CXR alone. (11,18) Regardless, CXR is an appropriate first test to evaluate for other pulmonary pathology in addition to HH/PEH, the latter of which can appear as a cystic mass in the posterior mediastinum with an air-fluid level (Figure 1), or demonstrate esophageal dilation or abnormal intrathoracic location of a nasogastric tube. (11, 19) The differential diagnosis of a fluid-filled posterior mediastinal cystic mass would include pulmonary abscess, congenital pulmonary airway malformation, pneumatocele, hydatid disease, mediastinal tumor, pericardial cyst, foregut duplication cyst, epiphrenic diverticulum, contained esophageal perforation and HH/PEH. (11, 19, 20) HH/PEH can be differentiated from Bochdalek and Morgagni hernias on CXR with the finding of a centrally located hernia on anterior view that corresponds to a posterior location on the lateral view. (18) However, CXR can appear normal in the case of HH/PEH owing to the possibility of intermittent reduction. (19)
Upper GI (UGI) can confirm the diagnosis by demonstrating a contrast-filled stomach herniated cephalad into the posterior mediastinum thus confirming the fluid filled cystic structure seen on CXR as a HH/PEH (Figure 2). UGI can also demonstrate volvulus, microgastria, esophageal dilation, and gastroesophageal reflux but has also been reported as normal in the presence of a HH/PEH. (11, 18, 19) Another imaging modality option is computed tomography (CT) which has the advantage of demonstrating the size and contents of the hernia as well as excluding other pulmonary or mediastinal etiologies (Figure 3). (11, 18, 19) Between UGI and CT only one imaging modality should be utilized in order to prevent unnecessary radiation exposure, and some authors feel CT does not offer any advantages over UGI. (11, 18, 22) Interestingly, with advances in fetal MRI there have been case reports of prenatal diagnosis of PEH (18). Endoscopy is used to evaluate for esophageal stricture and esophagitis, especially in those patients who present with dysphagia. (11, 22) Although not necessary, twenty-four hour pH monitoring provides a valid measure of esophageal acid exposure, thus confirming the presence of gastroesophageal reflux. (23) A fundoplication is routinely performed at the time of HH/PEH repair and a pH probe can serve as a measure of outcome in the post-operative period after the fundoplication is complete. Surgical Treatment Given the underlying anatomic abnormality that is HH/PEH, treatment is surgical, and the safety and efficacy of laparoscopy for acquired HH/PEH and pediatric foregut surgery has been well described. (24-26) In his ten year review of more than 1000 fundoplications, Rothenberg had a 0.2% conversion to open rate, a 1.1 day average length of stay and a 4% fundoplication failure rate. (26) With respect specifically to HH/PEH, Yagi published a 2003 series of four patients (one type II and three type III PEHs) managed laparoscopically with no recurrences at a mean follow up
of 30.2 months (range 9 months to 5 years), and Bettolli published a 2008 series of four patients with large PEHs approached laparoscopically with one patient requiring conversion to open. (27, 28) A more recent 2014 study from Korea retrospectively evaluated the outcomes of laparoscopic (n=16) versus open (n=17) HH/PEH repair. In this study, there were 25 type I HHs and 8 PEHs. The laparoscopic procedure took longer (150 vs 120 minutes, p=0.028) but had shorter time to oral intake (1 vs 2 days, p=0.001) and to full feeds (6 vs 10 days, p=0.048). There were no differences in length of stay or complications. There were no mortalities or recurrences. (29) Regardless of the approach, there are four important surgical tenets to consider when treating HH/PEH: 1) reduction of hernia contents 2) excision of the hernia sac 3) crural closure and 4) performing an antireflux procedure. In the initial 20 patients in the South African series, no antireflux procedure was performed and 60% developed significant gastroesophageal reflux requiring further surgical management compared to only 15% in whom an antireflux procedure was performed at the time of initial PEH repair that required further management. (11) Due to the loss of normal anatomy at the GE junction, the antireflux procedure serves to treat the high rate of coexisting gastroesophageal reflux with HH/PEH and may decrease the risk of HH/PEH recurrence. Crural Closure and Mesh Reinforcement There are several approaches to crural closure including the use of pledgeted sutures and hiatal re-approximation with sutures placed both anterior and posterior to the esophagus. (16) Nonabsorbable suture such as silk or ethibond should be utilized. A decreased hernia recurrence rate has been shown with Surgisis mesh (SIS; Cook, Inc, Bloomington, Ind) reinforcement of the crural closure at re-operation for acquired HH/PEH as detailed below in the Outcomes section. (30)
Laparoscopic technique It is the authors’ preference to approach HH/PEH laparoscopically as per the following technique. Infants and small children are placed at the foot of the operating table in the frog leg position. Older children and adolescents are placed in low lithotomy, except in the case of neurologically impaired patients whose condition may preclude them from such positioning. The surgeon stands at the foot of the operating table or between the patient’s legs with the assistant to the patient’s left and the scrub staff to the patient’s right. The abdomen is prepped and draped. A nasogastric tube is inserted to decompress the gastric contents. The patient is placed in reverse Trendelenberg. A 5-mm trocar is introduced through the umbilicus for a 45 degree laparoscope. Four additional 3 or 5-mm instruments are inserted depending on the size of the child: one in the right mid abdomen for placement of a flexible, triangular liver retractor; two surgeon working ports in the upper abdomen; and an assistant port in the left lateral mid abdomen. We prefer stab incisions for reasons previously described but will place a trocar for the surgeon’s right hand if an ultrasonic scalpel is needed for the dissection. (16,17) Atraumatic instruments are used for the surgeon’s left hand and the assistant’s to aid in retraction while dissection is carried out with a Maryland and/or an ultrasonic scalpel. The gastrohepatic ligament is divided with attention to the presence of an accessory left hepatic artery. The right crus is identified, and the degree of the HH/PEH and its contents are evaluated (Figure 4). The hernia contents are reduced back into the abdomen. The hernia sac is grasped and dissected free from the mediastinum and crural attachments being careful not to violate the pleura. The hernia sac is excised to ensure adequate identification of the crura. A retroesophageal window is created, with attention to protecting the posterior vagus nerve, to accommodate passing the fundus through for fundoplication. Depending on the size of the child and the mobility of the fundus, the short gastric vessels are divided. Occasionally,
a bougie, sized according to the patient’s weight (Table 2), is placed into the esophagus to ensure the crural closure is not too tight. (31) Interrupted 2-0 silk sutures are placed posterior to the esophagus to close the crura (Figure 5). If an anterior hiatal hernia defect is present after the posterior closure, it is closed with an interrupted 2-0 silk suture after the anterior vagus is identified and protected. The crura are further secured to the esophagus with interrupted 3-0 silk sutures at the 1, 4, 8 and 11 o’clock positions. If the crural repair is considered “tight” the surgeon has two options. The first is a relaxing incision in the left diaphragm lateral to the crura, which is subsequently closed with a mesh reinforcement. The second option is prophylactic mesh reinforcement of the esophageal hiatus at the time of primary HH/PEH repair. The authors’ preferred technique is mesh reinforcement of the hiatus with an absorbable mesh product. A square piece of mesh is cut to size with the goal of covering the hiatal defect with overlap onto the right crus and caudate lobe of the liver and 2 cm of overlap onto the left crus. The mesh is divided with scissors on one side creating two “pant legs.” The body of the mesh is placed posterior to the esophagus and the pant legs are wrapped around the sides of the esophagus and overlapped on the anterior esophagus. The mesh is secured and the pant legs are approximated with 3-0 interrupted silk sutures. Attention is then turned toward the fundoplication. If the bougie had been passed through the esophageal hiatus for the hiatal repair it is withdrawn into the esophagus and a fold of fundus is grasped and brought from left to right posterior to the esophagus. The bougie is then passed through the hiatus and the remaining fundus is folded anterior to the esophagus. The two folds are sutured together with three 2-0 silk sutures to create a 2 cm fundoplication (Figure 6). At this point the bougie is removed and the fundoplication is inspected to ensure appropriate size and positioning. If a gastrostomy is required it is performed at this time.
Although the authors prefer to perform 360 degree Nissen fundoplication, we understand that the type of fundoplication is surgeon dependent and the surgeon should perform whichever fundoplication they are most proficient with. Re-do HH/PEH repair is also approached laparoscopically, as the authors’ have noted minimal adhesions on re-do laparoscopic procedures for gastroesophageal reflux. (16) Unless the hiatal repair is completely tension free, the authors routinely reinforce the esophageal hiatal repair with mesh at the time of re-do HH/PEH repair. Postoperative Course Absent an injury to the stomach or esophagus during the repair, feeding is initiated within 6 to 12 hours after the operation with advancement to full feeds within 24 to 48 hours. If there was an injury to the esophagus or stomach, we routinely perform an UGI on postoperative day 2 to assess for a leak. If there is no leak the diet is advanced as noted above. Patients that eat a solid diet are maintained on a pureed diet for one month. Regarding hospital length of stay, Rothenberg’s series of more than 1000 laparoscopic fundoplications had a mean length of stay of 1.1 days. It has been our experience that most patients may be discharged on postoperative day two or three, although neurologically impaired patients may require a longer length of stay. Complications Although intra-operative complications are rare they must be a concern for the surgeon, especially in re-do HH/PEH surgery. The most common are esophageal or gastric perforation during re-do surgery, which has been reported to occur in 3-7% of cases. (32, 33) For gastric injuries, the stomach is repaired in a standard fashion with either a single or double layered closure. For esophageal perforation, the authors prefer a single layer closure followed by performing the fundoplication over the repair (if possible), thus buttressing the repair with the fundoplication. If the
esophageal perforation occurs proximal to the location of the fundoplication the esophagus is repaired and a naso-esophageal tube is left in the distal esophagus for 2 days. An UGI is then performed to evaluate for the presence of a leak and prompt emptying of the distal esophagus. Various postoperative complications have been reported after HH/PEH repair including dysphagia, recurrent HH/PEH, pneumonia, pneumothorax, bowel obstruction, intussusception, bleeding, wound infection and gastroparesis (11,18). Management of recurrent HH/PEH has been described above. Patients with dysphagia should be treated conservatively with liquid diet for at least 4-6 weeks before considering dilation of the hiatus/fundoplication. The mortality rate after HH/PEH approaches zero and is primarily associated with other co-morbidities such as congenital heart disease or other congenital or chromosomal abnormalities. Outcomes Recurrence of HH/PEH is perhaps the outcome of most interest. For primary repair of congenital HH/PEH studies, the recurrence rates are shown in Table 1 and range from 0 to 11%. As noted previously, only one study reported recurrence of gastroesophageal reflux which was up to 60% recurrence for reflux symptoms in patients who did not have a fundoplication at the time of initial operation. In one study of acquired HH/PEH over a six year period, 273 patients underwent laparoscopic Nissen fundoplication and 12% presented with emesis and were diagnosed with iatrogenic HH with thoracic herniation of the wrap via UGI and required re-operation. (Figure 7) The mean time interval between the two surgeries was 14.8 +/- 1.9 months. For the first 13 patients, eight operations were performed laparoscopically and only crural closure with 2-0 silk sutures was performed. There were four recurrences, two in the open group and two in the laparoscopic group (31%). Each of these four re-recurrences was repaired in an open fashion with SIS mesh rein-
forcement without evidence of a second recurrence with a minimum of 3 years follow up. For the remaining eight patients from the original 21 patient cohort, crural reinforcement with SIS mesh was performed in addition to crural closure. Seven of the eight procedures were performed open to refine the mesh placement technique, and there were no recurrences in this group at a minimum of 11 months follow up. (30) Mesh placement at the time of re-operation for first or second recurrence of HH/PEH with transthoracic migration of the fundoplication appears to be beneficial in reducing the incidence of re-herniation; however, the use of prophylactic mesh at the time of initial surgery has not been studied in the pediatric population. This may be an area of interest for future study to decrease the rate of acquired post-operative HH/PEH.
References (1) Stylopouls N, Rattner DW. The history of hiatal hernia surgery: from Bowditch to laparoscopy. Ann Surg 2005;241:185-93. (2) DeMeester TR, Banki F. Paraesophageal hiatal hernia, in Cameron JL, Cameron AM (eds): Current Surgical Therapy, Elsevier Inc, 2011, p 33. (3) Hochber LA. Thoracic Surgery before the 20th Century. New York, New York, Vantage Press,1960. (4) Friedenwald J, Feldman M. Report of an interesting type of diaphragmatic hernia of the cardia of the stomach through the esophageal orifice. Am Med Sci 1925;170:263. (5) Soresi AL. Diaphragmatic hernia: its unsuspected frequency: diagnosis and technique for radical cure. Ann Surg 1919;69:254-270. (6) Cuschieri A, Shimi S, Nathanson LK. Laparoscopic reduction, crural repair, and fundoplication of large hiatal hernia. Am J Surg. 1992;163(4):425-30. (7) Skandalkis JE, Gray SW. The diaphragm, in Skandalkis JE, Gray SW (eds): Embryology for Surgeons, Philadelphia, PA, WB Saunders, 1972, pp 359-362. (8) Botha GSM. The gastroesophageal region in infants. Arch Dis Child 1958;33:78-94. (9) Al-Assiri A, Wiseman N, Bunge M. Prenatal diagnosis of intrathoracic stomach (gastric herniation). J Pedatr Surg 2005;40:E15-7. (10) Bradley T, Stephenson J, Drugas G, et al. Laparoscopic management of neonatal paraesophageal hernia with intrathoracic gastric volvulus. J Pediatr Surg 2010;45:E21-3. (11) Karpelowsky JS, Wieselthaler N, Rode H. Primary paraesophageal hernia in children. J Pedatr Surg 2007;42:1298-301.
(12) St. Peter SD, Barnhart DC, Ostlie DJ,et al. Minimal vs. maximal esophageal dissection and mobilization during laparoscopic fundoplication: a prospective, randomized trial. J Pediatr Surg 2011;46:163-8. (13) Desai AA, Alemayehu H, Holcomb III, GW, et al. Minimal vs. maximal esophageal dissection and mobilization during laparoscopic fundoplication: Long-term follow-up from a prospective, randomized trial. J Pediatr Surg 2015;111-114. (14) Al-Salem AH, Congenital paraesophageal hernia in infancy and childhood. Saudi Med J 2000;21:164-7. (15) Marchad P. Traumatic hiatus hernia. Br Med J 1962;1:754-9. (16) Ostlie DJ, Holcomb GW. Reiterative laparoscopic surgery for recurrent gastroesophageal reflux. Semin Pediatr Surg 2007;16:252-8. (17) Ostlie DJ, Holcomb GW III. The use of stab incisions for instrument access in laparoscopic operations. J Pediatr Surg 2003;38:111-5. (18) Yousef Y, Lemoine C, St-Vil D, et al. Congenital paraesophageal hernia: the Montreal experience. J Pedatr Surg 2015;50:1462-66. (19) Imamoglu M, Cay A, Kosucu P, et al. Congenital paraesophageal hiatal hernia: pitafalls in the diagnosis and treatment. J Pedatr Surg 2005;40:1128-33. (20) Jetley NK, Al-Assiri AH, Al Awadi D. Congenital para esophageal hernia: a 10 year experience from Saudia Arabia. Indian J Pediatr 2009;76:489-93. (21) Leung AKC. Familial hiatal hernia. Pediatrics 1987;80:462. (22) Yazici M, Etensel KB, Temir G et al. Paraesophageal hiatal hernias in children. Dis Esophagus 2003;16:210-13.
(23) Vandenplas Y, Rudolph CD, Di Lorenzo C, et al. Pediatric gastroesophageal reflux clinical practice guidelines: joint recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) J Pediatr Gastroenterol Nutr 2009;49:498-547. (24) Chung DH, Georgeson KE. Fundoplication and gastrostomy. Semin Pediatr Surg 1998;7:213-9. (25) Ostlie DJ, Holcomb III GW. Laparoscopic fundoplication and gastrostomy. Semin Pediatr Surg 2002;11:196-204. (26) Rothenberg SS. The first decade’s experience with laparoscopic Nissen fundoplication in infants and children. J Pediatr Surg 2005;40:142-6. (27) Yagi M, Nose K, Yamauchi K, et al. Laparoscopic intervention for intrathoracic stomach in infants. Surg Endosc 2003;17:1636-39. (28) Bettoli M, Rubin SZ, Gutauskas A. Large paraesophageal hernias in children. Early experience with laparoscopic repair. Eur J Pediatr Surg 2008;18:72-4. (29) Namgoon J, Kim D, Kim S. Hiatal hernia in pediatric patients: laparoscopic versus open approaches. Ann Surg Treat Res 2014;86:264-9. (30) St Peter SD, Ostlie DJ, Holcomb GW. The use of biosynthetic mesh to enhance hiatal hernia repair at the time of redo Niseen fundoplication. J Pediatr Surg 2007;42:1298-1301. (31 ) Ostlie DJ, Miller KA, Holcombo GW III. Effective nissen fundoplication length and bougie diameter size in young children undergoing laparoscopic Nissen fundoplication. J Pediatr Surg 2002;37:1664-66. (32) Celik A, Loux TJ, Harmon CM, et al. Revision nissen fundoplication can be completed laparoscopically with a low rate of complications: a single institution experience with 72 children. J Pediatr Surg 2006;41:2081-5.
(33) Rothenberg SS. Laparoscopic redo nissen fundoplication in infants and children. Surg Endosc 2006;20:1518-20.
Table 1. Summary of Congenital Paraesophageal Hernia Studies Study (Author)
Study Years (range)
# of Pa- Mean Age at Repair tients (range)
Surgical Approach Recurrence Lap=laparotomy Thor=thoractomy MIS=laparoscopy
Karpelowsky 19882013
59
23.4 mo (1 day – 11 yrs)
Lap n=56 (95%) Thor n=3 (5%)
12/20 (60%) recurrent reflux Sx (no fundo group)
Yazici
19791999
19
32 mo (8d-14 yrs)
Lap n=12 (63%) Thor n=4 (21%) Thor+Lap n=3 (16%)
n=2 (11%)
Yousef
19882013
14
35 d (0 – 500 d)
Lap n=11 (79%) MIS n=3 (21%)
n=1 (7%)
Jetley
19972007
9
Unknown (8d – 3 mos)
Lap n=8 (89%) Thor n=1 (11%)
n=2 (22%)
Al-Salem
19891997
6
1.3 y (2d-2.5 yrs)
Lap n=6 (100%)
n=0
Imamoglu
19922004
5
7 mos (4-11 mos)
Lap n=5 (100%)
n=0
Table 2. Bougie Size used for calibration of the fundoplication based on body weight Weight (kg)
Bougie Size
2.5-4.0
20-24
4.0-5.5
24-28
5.5-7.0
28-32
7.0-8.5
32-34
8.5-10
34-36
10-15
36-40
Fig. 1 A lateral chest radiograph shows the air fluid level associated with a hiatal hernia(arrow). Fig. 2 This UGI shows a large congenital hiatal hernia in a term infant. Fig. 3 A large hernia (arrows) is shown in this chest CT. Fig. 4 The hiatal dissection after refuction of a hiatal hernia is shown. The arrows identify the right and left crura with the esophagus located posteriorly. Fig. 5 The hiatal repair in this patient required both anterior and posterior placement of permanent sutures. Fig. 6 The completed Nissen fundoplication is shown. A partial fundoplication could also be performed based on surgeon preference. Fig. 7 An acquired paraesophageal hernia is shown in this UGI. The patient had undergone a pervious laparoscopic Nissen with extensive hiatal dissection. The white arrows identifies the hiatal hernia and the red arrow shows the remaining imbrication of the Nisen fundoplication, both are herniated through the esophageal hiatus.
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To appear in: Seminars in Pediatric Surgery Cite this article as: Erin M Garvey and Daniel J Ostlie, Hiatal and paraesophageal hernia repair in pediatric patients, Seminars in Pediatric Surgery, http://dx.doi.org/10.1053/j.sempedsurg.2017.02.008 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting galley proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
Hiatal and Paraesophageal Hernia Repair in Pediatric Patients
Erin M Garvey, MD & Daniel J Ostlie, MD Department of Surgery; Phoenix Children’s Hospital
Corresponding Author: Daniel J Ostlie, MD Surgeon in Chief and Chair of Surgery Department of Surgery; Phoenix Children’s Hospital Phoenix Children’s Hospital 1919 E Thomas Road Phoenix, AZ 85016 Phone 602-933-7003 Fax 602-933-0394 [email protected] Abstract Hiatal and paraesophageal hernia (HH/PEH) can be congenital, resulting from embryologic abnormalities/genetic predisposition, or acquired, most commonly after gastro-esophageal surgery such as fundoplication. Minimizing circumferential esophageal dissection at the time of Nissen fundoplication has been shown to decrease the risk of acquired HH/PEH from 36.5 to 12.2%. Gastrointestinal, respiratory and constitutional symptoms, including anemia and failure to thrive, are common with high rates of associated gastroesophageal reflux. Chest x-ray is often abnormal and upper GI confirms the diagnosis. Treatment is surgical with the goal of reducing the hernia contents, excising the hernia sac, closing the crura and performing an antireflux procedure. The laparoscopic approach is safe and effective.
Key Words Hiatal hernia, Paraesophageal hernia, Pediatric, Mesh, Recurrence
Introduction In his 1853 review of 88 postmortem cases of diaphragmatic hernia, Henry Ingersoll Bowditch noted three cases wherein there was dilation of the esophageal opening and described what is thought to be the first report of a type II paraesophageal hernia. (1) A hiatal hernia (HH), also known as a type I or sliding hernia, occurs when the gastroesophageal junction (GEJ) abnormally protrudes cephalad into the chest through the esophageal hiatus. Types II-IVn HH’s are known as paraesophageal hernias (PEH) and are the least common congenital diaphragmatic hernias. A type II PEH occurs when the gastric fundus herniates through the esophageal hiatus alongside the lower thoracic esophagus while the GEJ remains in the appropriate intra-abdominal location. Type III PEH’s represent a combination of types I and II with an intra-thoracic position of the GEJ and herniation of the gastric fundus. The term giant PEH is used when >30% of the stomach is herniated into the chest. Type IV PEH’s involve herniation of other intra-abdominal contents, in addition to the stomach, such as colon, small intestine, spleen, or omentum. (2) This review will detail the historical perspective, epidemiology, presentation, diagnosis, treatment and outcomes of HH/PEH in the pediatric population.
History Well before Bowditch’s review in 1853, there were a number of descriptions of congenital and posttraumatic diaphragmatic hernias including those by Batista Morgagni and Vincent Alexander Bochdalek. However, it was not until 1900 that a HH was diagnosed by means of X-ray, first by Hirsch prior to autopsy and later by Eppinger in live patients. (1, 3) Shortly thereafter, in 1925, Julius Friedenwald and Maurice Feldman correlated the symptom of heartburn to the presence of a HH. (4) The first report on elective surgical repair of HH was published by Angelo Soresi in 1919 detailing an abdominal approach, reduction of the hernia and closure of the hiatus for which he described “the suture has to close in the most perfect manner the opening of the diaphragm especially around the organs that pass through it, esophagus, vena cava, aorta, but without compressing the important organs just mentioned” – surgical principles that are still pertinent today. (5) The current surgical approach to the hiatus is overwhelmingly laparoscopic, a technique first described by Cuschieri in 1992. (6) The present day focus continues to be on the perfect manner by which to protect and close the hiatus with variations in degree of dissection, suture choice and placement, and mesh reinforcement of the crural closure. Epidemiology Etiology and Embryology HH/PEH are either congenital or acquired, the latter of which can be the postoperative consequence of gastro-esophageal surgery or, less commonly, the result of traumatic injury. There are a number of embryological hypotheses as to why these hernias form; however, the true etiology is not fully understood. During development, the pneumoenteric recesses are two small coelomic spaces which evolve on either side of the midline of the mediastinum, and persistence of the right recesses results in a flattened and elongated mesothelial lined space within the esophageal
hiatus. (7, 8) This space has been measured as approximately 1 cm at birth and likely obliterates during normal development into adulthood suggesting persistence of this right recess may predispose to HH/PEH development. Other hypotheses include laxity in gastric attachments, deficient diaphragmatic hiatus and aberrant development of the lumbar component of the diaphragm origination from mesoderm cells around the aorta. (9-11) Any surgical dissection at the esophageal hiatus resulting in disruption of the phrenoesophageal membrane can predispose to the development of HH/PEH. The most common pediatric procedure during which this could occur is fundoplication for gastroesophageal reflux, and the risk of acquired HH/PEH has been shown to be related to the extent of peri-esophageal dissection. A prospective randomized controlled trial during Nissen fundoplication comparing circumferential esophageal mobilization to minimal dissection leaving the phrenoesophageal membrane intact found a postoperative herniation rate at 1 year minimum follow up of 22.7% for the circumferential dissection compared to 2.8% for the minimal dissection group. (12) The five year minimum follow up study to the original trial showed an increase in postoperative hernia rates for both groups (36.5% for the circumferential dissection group versus 12.2% for the minimal dissection group). (13) The importance of minimizing esophageal dissection at the time of initial Nissen fundoplication to decrease the risk of post-operative, acquired HH/PEH cannot be over emphasized. HH/PEH has also been reported after surgical intervention for esophageal atresia and tracheoesophagel fistula. (14) As for post-traumatic HH/PEH, in his review of 216 adult cases, Marchand determined 5% of hiatal hernias were the result of a traumatic incident, most commonly involving a crush injury, but there is no pediatric literature regarding acquired traumatic HH/PEH. (15)
Incidence and Patient Population In 370 patients who underwent laparoscopic Nissen fundoplication over a six year period, 8.1% required a second operation for repair of an acquired HH/PEH with fundoplication transmigration, and the incidence of post-operative herniation at five year follow up from the prospective trial described above ranged from 12.2 to 36.5% depending on the extent of esophageal dissection performed. (13, 16) The true incidence of congenital PEH, on the other hand, is unknown, but this diagnosis is felt to be uncommon. In the Montreal series of 14 patients of congenital PEH, type III PEH’s were the most common at 57% versus 29% for type II and 14% for type IV PEH. (18) There have only been a few case reports of type IV PEH in the pediatric population. (18) A summary of the larger case series examining congenital PEH is shown in Table 1. There was no gender predilection from these studies, and patients were most often diagnosed within the first two years of life. Other congenital abnormalities including malrotation, trisomies, and Marfans syndrome have been associated with PEH. (9, 18-20) In keeping with the embryological hypotheses above, the question of a familial mode of inheritance has also been raised as there have been a number of case series of HH/PEH within families suggesting a genetic predisposition. (9, 20, 21) Clinical Presentation Patients with either congenital or acquired HH/PEH can be asymptomatic or, more commonly, present with a variety of gastrointestinal, respiratory and/or constitutional symptoms. Not surprisingly, symptomatic gastroesophageal reflux was present in 50-68% of patients with PEH. Recurrent respiratory tract infections, emesis, anemia and failure to thrive were other common presenting symptoms. In the Montreal series, emesis was the most common symptom for Type II PEH, while respiratory distress was most commonly seen in type III and IV PEHs, with failure to thrive and anemia also seen in type III. The feared complication of volvulus was reported in three
of five patients in a series from Turkey. In all three of these patients, the volvulus was all organoaxial rotation where the greater curve of the stomach rotates horizontally along the longitudinal axis between the pylorus and the gastroesophageal junction. (2, 19) Given the rarity of HH/PEH, clinicians must maintain an index of suspicion to proceed with radiographic evaluation in pediatric patients who present with these sometimes vague symptoms. Physical exam is often unrevealing, but one may hear borborygmi (bowel sounds) on auscultation of the chest. Diagnostic Evaluation Evaluation often begins with an anteroposterior and lateral chest x-ray (CXR). One series reported seeing a cystic mass on all CXRs corresponding to the hernia diagnosis in all cases, while another series reported a correct presumptive diagnosis was only made in 29% of cases based on clinical symptoms and CXR alone. (11,18) Regardless, CXR is an appropriate first test to evaluate for other pulmonary pathology in addition to HH/PEH, the latter of which can appear as a cystic mass in the posterior mediastinum with an air-fluid level (Figure 1), or demonstrate esophageal dilation or abnormal intrathoracic location of a nasogastric tube. (11, 19) The differential diagnosis of a fluid-filled posterior mediastinal cystic mass would include pulmonary abscess, congenital pulmonary airway malformation, pneumatocele, hydatid disease, mediastinal tumor, pericardial cyst, foregut duplication cyst, epiphrenic diverticulum, contained esophageal perforation and HH/PEH. (11, 19, 20) HH/PEH can be differentiated from Bochdalek and Morgagni hernias on CXR with the finding of a centrally located hernia on anterior view that corresponds to a posterior location on the lateral view. (18) However, CXR can appear normal in the case of HH/PEH owing to the possibility of intermittent reduction. (19)
Upper GI (UGI) can confirm the diagnosis by demonstrating a contrast-filled stomach herniated cephalad into the posterior mediastinum thus confirming the fluid filled cystic structure seen on CXR as a HH/PEH (Figure 2). UGI can also demonstrate volvulus, microgastria, esophageal dilation, and gastroesophageal reflux but has also been reported as normal in the presence of a HH/PEH. (11, 18, 19) Another imaging modality option is computed tomography (CT) which has the advantage of demonstrating the size and contents of the hernia as well as excluding other pulmonary or mediastinal etiologies (Figure 3). (11, 18, 19) Between UGI and CT only one imaging modality should be utilized in order to prevent unnecessary radiation exposure, and some authors feel CT does not offer any advantages over UGI. (11, 18, 22) Interestingly, with advances in fetal MRI there have been case reports of prenatal diagnosis of PEH (18). Endoscopy is used to evaluate for esophageal stricture and esophagitis, especially in those patients who present with dysphagia. (11, 22) Although not necessary, twenty-four hour pH monitoring provides a valid measure of esophageal acid exposure, thus confirming the presence of gastroesophageal reflux. (23) A fundoplication is routinely performed at the time of HH/PEH repair and a pH probe can serve as a measure of outcome in the post-operative period after the fundoplication is complete. Surgical Treatment Given the underlying anatomic abnormality that is HH/PEH, treatment is surgical, and the safety and efficacy of laparoscopy for acquired HH/PEH and pediatric foregut surgery has been well described. (24-26) In his ten year review of more than 1000 fundoplications, Rothenberg had a 0.2% conversion to open rate, a 1.1 day average length of stay and a 4% fundoplication failure rate. (26) With respect specifically to HH/PEH, Yagi published a 2003 series of four patients (one type II and three type III PEHs) managed laparoscopically with no recurrences at a mean follow up
of 30.2 months (range 9 months to 5 years), and Bettolli published a 2008 series of four patients with large PEHs approached laparoscopically with one patient requiring conversion to open. (27, 28) A more recent 2014 study from Korea retrospectively evaluated the outcomes of laparoscopic (n=16) versus open (n=17) HH/PEH repair. In this study, there were 25 type I HHs and 8 PEHs. The laparoscopic procedure took longer (150 vs 120 minutes, p=0.028) but had shorter time to oral intake (1 vs 2 days, p=0.001) and to full feeds (6 vs 10 days, p=0.048). There were no differences in length of stay or complications. There were no mortalities or recurrences. (29) Regardless of the approach, there are four important surgical tenets to consider when treating HH/PEH: 1) reduction of hernia contents 2) excision of the hernia sac 3) crural closure and 4) performing an antireflux procedure. In the initial 20 patients in the South African series, no antireflux procedure was performed and 60% developed significant gastroesophageal reflux requiring further surgical management compared to only 15% in whom an antireflux procedure was performed at the time of initial PEH repair that required further management. (11) Due to the loss of normal anatomy at the GE junction, the antireflux procedure serves to treat the high rate of coexisting gastroesophageal reflux with HH/PEH and may decrease the risk of HH/PEH recurrence. Crural Closure and Mesh Reinforcement There are several approaches to crural closure including the use of pledgeted sutures and hiatal re-approximation with sutures placed both anterior and posterior to the esophagus. (16) Nonabsorbable suture such as silk or ethibond should be utilized. A decreased hernia recurrence rate has been shown with Surgisis mesh (SIS; Cook, Inc, Bloomington, Ind) reinforcement of the crural closure at re-operation for acquired HH/PEH as detailed below in the Outcomes section. (30)
Laparoscopic technique It is the authors’ preference to approach HH/PEH laparoscopically as per the following technique. Infants and small children are placed at the foot of the operating table in the frog leg position. Older children and adolescents are placed in low lithotomy, except in the case of neurologically impaired patients whose condition may preclude them from such positioning. The surgeon stands at the foot of the operating table or between the patient’s legs with the assistant to the patient’s left and the scrub staff to the patient’s right. The abdomen is prepped and draped. A nasogastric tube is inserted to decompress the gastric contents. The patient is placed in reverse Trendelenberg. A 5-mm trocar is introduced through the umbilicus for a 45 degree laparoscope. Four additional 3 or 5-mm instruments are inserted depending on the size of the child: one in the right mid abdomen for placement of a flexible, triangular liver retractor; two surgeon working ports in the upper abdomen; and an assistant port in the left lateral mid abdomen. We prefer stab incisions for reasons previously described but will place a trocar for the surgeon’s right hand if an ultrasonic scalpel is needed for the dissection. (16,17) Atraumatic instruments are used for the surgeon’s left hand and the assistant’s to aid in retraction while dissection is carried out with a Maryland and/or an ultrasonic scalpel. The gastrohepatic ligament is divided with attention to the presence of an accessory left hepatic artery. The right crus is identified, and the degree of the HH/PEH and its contents are evaluated (Figure 4). The hernia contents are reduced back into the abdomen. The hernia sac is grasped and dissected free from the mediastinum and crural attachments being careful not to violate the pleura. The hernia sac is excised to ensure adequate identification of the crura. A retroesophageal window is created, with attention to protecting the posterior vagus nerve, to accommodate passing the fundus through for fundoplication. Depending on the size of the child and the mobility of the fundus, the short gastric vessels are divided. Occasionally,
a bougie, sized according to the patient’s weight (Table 2), is placed into the esophagus to ensure the crural closure is not too tight. (31) Interrupted 2-0 silk sutures are placed posterior to the esophagus to close the crura (Figure 5). If an anterior hiatal hernia defect is present after the posterior closure, it is closed with an interrupted 2-0 silk suture after the anterior vagus is identified and protected. The crura are further secured to the esophagus with interrupted 3-0 silk sutures at the 1, 4, 8 and 11 o’clock positions. If the crural repair is considered “tight” the surgeon has two options. The first is a relaxing incision in the left diaphragm lateral to the crura, which is subsequently closed with a mesh reinforcement. The second option is prophylactic mesh reinforcement of the esophageal hiatus at the time of primary HH/PEH repair. The authors’ preferred technique is mesh reinforcement of the hiatus with an absorbable mesh product. A square piece of mesh is cut to size with the goal of covering the hiatal defect with overlap onto the right crus and caudate lobe of the liver and 2 cm of overlap onto the left crus. The mesh is divided with scissors on one side creating two “pant legs.” The body of the mesh is placed posterior to the esophagus and the pant legs are wrapped around the sides of the esophagus and overlapped on the anterior esophagus. The mesh is secured and the pant legs are approximated with 3-0 interrupted silk sutures. Attention is then turned toward the fundoplication. If the bougie had been passed through the esophageal hiatus for the hiatal repair it is withdrawn into the esophagus and a fold of fundus is grasped and brought from left to right posterior to the esophagus. The bougie is then passed through the hiatus and the remaining fundus is folded anterior to the esophagus. The two folds are sutured together with three 2-0 silk sutures to create a 2 cm fundoplication (Figure 6). At this point the bougie is removed and the fundoplication is inspected to ensure appropriate size and positioning. If a gastrostomy is required it is performed at this time.
Although the authors prefer to perform 360 degree Nissen fundoplication, we understand that the type of fundoplication is surgeon dependent and the surgeon should perform whichever fundoplication they are most proficient with. Re-do HH/PEH repair is also approached laparoscopically, as the authors’ have noted minimal adhesions on re-do laparoscopic procedures for gastroesophageal reflux. (16) Unless the hiatal repair is completely tension free, the authors routinely reinforce the esophageal hiatal repair with mesh at the time of re-do HH/PEH repair. Postoperative Course Absent an injury to the stomach or esophagus during the repair, feeding is initiated within 6 to 12 hours after the operation with advancement to full feeds within 24 to 48 hours. If there was an injury to the esophagus or stomach, we routinely perform an UGI on postoperative day 2 to assess for a leak. If there is no leak the diet is advanced as noted above. Patients that eat a solid diet are maintained on a pureed diet for one month. Regarding hospital length of stay, Rothenberg’s series of more than 1000 laparoscopic fundoplications had a mean length of stay of 1.1 days. It has been our experience that most patients may be discharged on postoperative day two or three, although neurologically impaired patients may require a longer length of stay. Complications Although intra-operative complications are rare they must be a concern for the surgeon, especially in re-do HH/PEH surgery. The most common are esophageal or gastric perforation during re-do surgery, which has been reported to occur in 3-7% of cases. (32, 33) For gastric injuries, the stomach is repaired in a standard fashion with either a single or double layered closure. For esophageal perforation, the authors prefer a single layer closure followed by performing the fundoplication over the repair (if possible), thus buttressing the repair with the fundoplication. If the
esophageal perforation occurs proximal to the location of the fundoplication the esophagus is repaired and a naso-esophageal tube is left in the distal esophagus for 2 days. An UGI is then performed to evaluate for the presence of a leak and prompt emptying of the distal esophagus. Various postoperative complications have been reported after HH/PEH repair including dysphagia, recurrent HH/PEH, pneumonia, pneumothorax, bowel obstruction, intussusception, bleeding, wound infection and gastroparesis (11,18). Management of recurrent HH/PEH has been described above. Patients with dysphagia should be treated conservatively with liquid diet for at least 4-6 weeks before considering dilation of the hiatus/fundoplication. The mortality rate after HH/PEH approaches zero and is primarily associated with other co-morbidities such as congenital heart disease or other congenital or chromosomal abnormalities. Outcomes Recurrence of HH/PEH is perhaps the outcome of most interest. For primary repair of congenital HH/PEH studies, the recurrence rates are shown in Table 1 and range from 0 to 11%. As noted previously, only one study reported recurrence of gastroesophageal reflux which was up to 60% recurrence for reflux symptoms in patients who did not have a fundoplication at the time of initial operation. In one study of acquired HH/PEH over a six year period, 273 patients underwent laparoscopic Nissen fundoplication and 12% presented with emesis and were diagnosed with iatrogenic HH with thoracic herniation of the wrap via UGI and required re-operation. (Figure 7) The mean time interval between the two surgeries was 14.8 +/- 1.9 months. For the first 13 patients, eight operations were performed laparoscopically and only crural closure with 2-0 silk sutures was performed. There were four recurrences, two in the open group and two in the laparoscopic group (31%). Each of these four re-recurrences was repaired in an open fashion with SIS mesh rein-
forcement without evidence of a second recurrence with a minimum of 3 years follow up. For the remaining eight patients from the original 21 patient cohort, crural reinforcement with SIS mesh was performed in addition to crural closure. Seven of the eight procedures were performed open to refine the mesh placement technique, and there were no recurrences in this group at a minimum of 11 months follow up. (30) Mesh placement at the time of re-operation for first or second recurrence of HH/PEH with transthoracic migration of the fundoplication appears to be beneficial in reducing the incidence of re-herniation; however, the use of prophylactic mesh at the time of initial surgery has not been studied in the pediatric population. This may be an area of interest for future study to decrease the rate of acquired post-operative HH/PEH.
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(12) St. Peter SD, Barnhart DC, Ostlie DJ,et al. Minimal vs. maximal esophageal dissection and mobilization during laparoscopic fundoplication: a prospective, randomized trial. J Pediatr Surg 2011;46:163-8. (13) Desai AA, Alemayehu H, Holcomb III, GW, et al. Minimal vs. maximal esophageal dissection and mobilization during laparoscopic fundoplication: Long-term follow-up from a prospective, randomized trial. J Pediatr Surg 2015;111-114. (14) Al-Salem AH, Congenital paraesophageal hernia in infancy and childhood. Saudi Med J 2000;21:164-7. (15) Marchad P. Traumatic hiatus hernia. Br Med J 1962;1:754-9. (16) Ostlie DJ, Holcomb GW. Reiterative laparoscopic surgery for recurrent gastroesophageal reflux. Semin Pediatr Surg 2007;16:252-8. (17) Ostlie DJ, Holcomb GW III. The use of stab incisions for instrument access in laparoscopic operations. J Pediatr Surg 2003;38:111-5. (18) Yousef Y, Lemoine C, St-Vil D, et al. Congenital paraesophageal hernia: the Montreal experience. J Pedatr Surg 2015;50:1462-66. (19) Imamoglu M, Cay A, Kosucu P, et al. Congenital paraesophageal hiatal hernia: pitafalls in the diagnosis and treatment. J Pedatr Surg 2005;40:1128-33. (20) Jetley NK, Al-Assiri AH, Al Awadi D. Congenital para esophageal hernia: a 10 year experience from Saudia Arabia. Indian J Pediatr 2009;76:489-93. (21) Leung AKC. Familial hiatal hernia. Pediatrics 1987;80:462. (22) Yazici M, Etensel KB, Temir G et al. Paraesophageal hiatal hernias in children. Dis Esophagus 2003;16:210-13.
(23) Vandenplas Y, Rudolph CD, Di Lorenzo C, et al. Pediatric gastroesophageal reflux clinical practice guidelines: joint recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) J Pediatr Gastroenterol Nutr 2009;49:498-547. (24) Chung DH, Georgeson KE. Fundoplication and gastrostomy. Semin Pediatr Surg 1998;7:213-9. (25) Ostlie DJ, Holcomb III GW. Laparoscopic fundoplication and gastrostomy. Semin Pediatr Surg 2002;11:196-204. (26) Rothenberg SS. The first decade’s experience with laparoscopic Nissen fundoplication in infants and children. J Pediatr Surg 2005;40:142-6. (27) Yagi M, Nose K, Yamauchi K, et al. Laparoscopic intervention for intrathoracic stomach in infants. Surg Endosc 2003;17:1636-39. (28) Bettoli M, Rubin SZ, Gutauskas A. Large paraesophageal hernias in children. Early experience with laparoscopic repair. Eur J Pediatr Surg 2008;18:72-4. (29) Namgoon J, Kim D, Kim S. Hiatal hernia in pediatric patients: laparoscopic versus open approaches. Ann Surg Treat Res 2014;86:264-9. (30) St Peter SD, Ostlie DJ, Holcomb GW. The use of biosynthetic mesh to enhance hiatal hernia repair at the time of redo Niseen fundoplication. J Pediatr Surg 2007;42:1298-1301. (31 ) Ostlie DJ, Miller KA, Holcombo GW III. Effective nissen fundoplication length and bougie diameter size in young children undergoing laparoscopic Nissen fundoplication. J Pediatr Surg 2002;37:1664-66. (32) Celik A, Loux TJ, Harmon CM, et al. Revision nissen fundoplication can be completed laparoscopically with a low rate of complications: a single institution experience with 72 children. J Pediatr Surg 2006;41:2081-5.
(33) Rothenberg SS. Laparoscopic redo nissen fundoplication in infants and children. Surg Endosc 2006;20:1518-20.
Table 1. Summary of Congenital Paraesophageal Hernia Studies Study (Author)
Study Years (range)
# of Pa- Mean Age at Repair tients (range)
Surgical Approach Recurrence Lap=laparotomy Thor=thoractomy MIS=laparoscopy
Karpelowsky 19882013
59
23.4 mo (1 day – 11 yrs)
Lap n=56 (95%) Thor n=3 (5%)
12/20 (60%) recurrent reflux Sx (no fundo group)
Yazici
19791999
19
32 mo (8d-14 yrs)
Lap n=12 (63%) Thor n=4 (21%) Thor+Lap n=3 (16%)
n=2 (11%)
Yousef
19882013
14
35 d (0 – 500 d)
Lap n=11 (79%) MIS n=3 (21%)
n=1 (7%)
Jetley
19972007
9
Unknown (8d – 3 mos)
Lap n=8 (89%) Thor n=1 (11%)
n=2 (22%)
Al-Salem
19891997
6
1.3 y (2d-2.5 yrs)
Lap n=6 (100%)
n=0
Imamoglu
19922004
5
7 mos (4-11 mos)
Lap n=5 (100%)
n=0
Table 2. Bougie Size used for calibration of the fundoplication based on body weight Weight (kg)
Bougie Size
2.5-4.0
20-24
4.0-5.5
24-28
5.5-7.0
28-32
7.0-8.5
32-34
8.5-10
34-36
10-15
36-40
Fig. 1 A lateral chest radiograph shows the air fluid level associated with a hiatal hernia(arrow). Fig. 2 This UGI shows a large congenital hiatal hernia in a term infant. Fig. 3 A large hernia (arrows) is shown in this chest CT. Fig. 4 The hiatal dissection after refuction of a hiatal hernia is shown. The arrows identify the right and left crura with the esophagus located posteriorly. Fig. 5 The hiatal repair in this patient required both anterior and posterior placement of permanent sutures. Fig. 6 The completed Nissen fundoplication is shown. A partial fundoplication could also be performed based on surgeon preference. Fig. 7 An acquired paraesophageal hernia is shown in this UGI. The patient had undergone a pervious laparoscopic Nissen with extensive hiatal dissection. The white arrows identifies the hiatal hernia and the red arrow shows the remaining imbrication of the Nisen fundoplication, both are herniated through the esophageal hiatus.