Oesophageal atresia and tracheo-oesophageal fistula: current management strategies and complications

Paediatric Respiratory Reviews 11 (2010) 100–107

Contents lists available at ScienceDirect

Paediatric Respiratory Reviews

CME Review

Oesophageal atresia and tracheo-oesophageal fistula: current management strategies and complications Andrew J.A. Holland 1,2, Dominic A. Fitzgerald 2,3,* 1

Douglas Cohen Department of Paediatric Surgery, The Children’s Hospital at Westmead, Locked Bag 4001, Westmead, NSW 2145, Australia The Children’s Hospital at Westmead, Sydney Medical School, The Children’s Hospital at Westmead, Locked Bag 4001, Westmead, NSW 2145, Australia 3 The University of Sydney, Department of Respiratory Medicine, The Children’s Hospital at Westmead, Locked Bag 4001, Westmead, NSW 2145, Australia 2

EDUCATIONAL AIMS    

IN READING THIS ARTICLE, THE READER WILL:

Develop familiarity with the peri-operative management issues if the care of infants with OA/TOF. Come to appreciate the gastro-intestinal complications of OA/TOF. Develop an understanding of the respiratory complications of OA/TOF. Come to realise that the problems associated with OA/TOF have life-long implications.

A R T I C L E I N F O

S U M M A R Y

Keywords: Oesophageal atresia tracheo-oesophageal fistula complications oesophageal stricture gastro-oesophageal reflux tracheomalacia dysphagia

The successful operative management of oesophageal atresia and tracheo-oesophageal atresia has been available for approximately 70 years. During this time neonatal intensive care has evolved, surgical techniques have improved and consequently near 100% survival for this condition may now be achieved. In keeping with promising results, the co-morbidities of the condition have gained increasing recognition. In this article, the clinical course from antenatal assessments, neonatal surgery and comorbidities from infancy to adulthood are reviewed to provide a broad overview of the condition. Crown Copyright ß 2010 Published by Ltd. All rights reserved.

INTRODUCTION Oesophageal atresia (OA) represents a congenital developmental anomaly characterised by anatomical discontinuity of the oesophagus such that the lumen is not patent.1 In approximately 85-88% of cases OA will be associated with an abnormal communication between the trachea and oesophagus, in 6-7% there will be no fistula and in the remainder a fistula but no true OA.1–3 In those patients with OA and an associated tracheooesophageal fistula (TOF), the most common arrangement would be a blind-ending upper oesophageal pouch with a distal fistula: isolated proximal or the presence of both proximal and distal fistulas occurs much less commonly.1–3 An understanding of these

* Corresponding author. Department of Respiratory Medicine, The Children’s Hospital at Westmead, Locked Bag 4001, Westmead, NSW, Australia, 2145. Tel.: +61 2 9845 3397; Fax: +61 2 9845 3396. E-mail address: [email protected] (D.A. Fitzgerald).

anatomical variants remains important to facilitate optimal medical and surgical management.4 The frequency of OA appears fairly consistent in most populations, occurring between 1 in 2,500 to 4,500 live births.1,2,5–7 The great majority of cases occur as a sporadic phenomenon, although the incidence is higher in twins.5,8 Perhaps of greater clinical importance remains the high frequency of associated anomalies, at over 50%, which may greatly impact on both treatment and outcome.2,5,9 In addition to their frequency, anomalies remain unequally distributed between patients: thus those with ‘pure’ or ‘isolated’ OA without fistula have anomalies in up to 65% of cases, with a much lower frequency in those with a fistula but no atresia, perhaps contributing to diagnostic delay.1,9,10 Cardiac defects consistently account for the greatest number of anomalies and may be a feature of the VACTERL (Vertebral, Atesia – duodenal and anorectal, Cardiac, Tracheo-oesphageal, Renal, Limb) and CHARGE (Coloboma, Heart, Atresia choanal, Retarded growth, Genital hyposplasia, Ear deformities) syndromes in addition to OA associated with Trisomy 18 and 21.1,7,9,11

1526-0542/$ – see front matter . Crown Copyright ß 2010 Published by Ltd. All rights reserved. doi:10.1016/j.prrv.2010.01.007

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Whilst atresia remains the most common congenital anomaly of the oesophagus, much has changed in the management of this condition since the first reported primary repair in 1941 by Haight.12–14 Following this description, successful repairs were performed in 1948 at both Great Ormond Street Hospital for Children in London and at the Royal Children’s Hospital in Melbourne.15,16 Whereas these early procedures were associated with mortality rates of 30-50%, improvements in surgical, anaesthetic and neonatal care over the last 50 years have resulted in the present situation where survival following surgery may generally be expected, even for infants with long-gap defects, prematurity and some associated anomalies.15–18 With these improvements, the focus of the critical lens has moved from mortality to morbidity and quality of life issues.3,17,19–24 DIAGNOSIS The diagnosis of OA most commonly occurs in the first 24 hours of life, but may occur either antenatally or may be delayed.1,25–27 Antenatal diagnosis confers the advantages of facilitating consultation with the parents prior to delivery to discuss the clinical management and planning of the delivery close to or within a tertiary paediatric facility.3,18,25 These potential advantages must be offset against the level of anxiety generated in the parents and reluctance to initiate changes in obstetric care, whilst acknowledging the false positive, negative and diagnostic error rate of antenatal Ultrasound (US) alone.18 Nevertheless, once the diagnosis has been made, the opportunity should be used to exclude associated defects, including chromosomal anomalies, in order to assist in providing accurate antenatal counselling.3,18 This may include an antenatal echocardiogram (ECHO) and amniocentesis for foetal karyotyping.26 Antenatal diagnosis Antenatal diagnosis of OA was first reported in 1980 by Farrant in a single foetus at 26 weeks of gestation, based on the presence of polyhydramnios in association with an absent stomach.28 Both remain non-specific features which may be associated with other, unrelated anomalies including myopathies and neurological defect.18,29 Polyhydramnios may also be associated with other thoracic and gastrointestinal anomalies, including congenital diaphragmatic hernia (CDH), Congenital Cystic Adenomatoid Malformation (CCAM) and duodenal atresia.18,29,30 Whilst the criteria for US diagnosis of polyhydramnios remain specific, an Amniotic Fluid Index of greater than the 97.5 percentile, in practical terms this will only rarely be recognised at the routine 18 to 20 week abnormality scan.30,31 Further, assessment of what constitutes a ‘small’ stomach remains subjective.26,32 Whereas the positive predictive value (PPV) of antenatal diagnosis increases to between 44% and 56% with the findings of polyhydramnios in association with a small or absent stomach, usually after 24 weeks gestation, figures as low as 8.9% to 14.6% have been reported.17,26,32,32 Typically the accuracy of antenatal US appears to be greater with isolated OA, with a PPV of between 75% to 91%, although in part this may reflect a higher incidence of associated anomalies.32,33 The relative inaccuracy of polyhydramnios and absent stomach has prompted the search for other predictive parameters for the antenatal diagnosis of OA. US detection of the blind-ending upper oesophageal pouch, the ‘Upper pouch sign’, was first reported in 1983.34 Typically this may only be detected relatively late in gestation, however, at a median 32 weeks.18 Moreover, in infants found to have OA at birth, even when both polyhydramnios and an absent stomach have been reported antenatally, an upper pouch

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sign may not be detected despite specific examination.35 Whilst data remains limited, the presence of the upper pouch sign does seem to correlate with a higher risk of Trisomy 18.18 As with congenital diaphragmatic hernia [CDH], the value of antenatal Magnetic Resonance Imaging (MRI), whilst intuitively an attractive and logical modality for diagnosis and assessment of OA, remains limited in practice, with a false positive rate of up to 64% when used in isolation.36,37 Comprehensive analysis of oesophageal anatomy and physiology using high-resolution US would seem to offer greater scope for improved antenatal diagnosis of OA.38 Diagnosis at birth The majority of infants with OA will be diagnosed at birth on the basis of drooling whilst unable to swallow saliva, choking with attempted feeding, mild to moderate respiratory distress and inability to pass a 10F gastric tube beyond 10 cm from the lips.1,16,18 Use of a thinner tube, which may curl in the posterior pharynx or pass through a fistula, should be discouraged, whilst recognising that appropriate care should be taken to avoid an inadvertent oesophageal perforation, particularly in the premature infant.39,40 Whilst up to 91% of infants with OA may not be diagnosed antenatally, it is the inability of the infant to feed that typically prompts correct diagnosis. Whilst a twin pregnancy and the rare positive family history may serve as diagnostic ‘red flags’, it may conversely be the presence of a more obvious associated anomaly, present in up to 50% of cases, that prompts diagnosis of the OA.1,3,16 Cardiovascular defects remain the most common, accounting for around one third of all anomalies, followed by approximately equal numbers of genitourinary, gastrointestinal, vertebroskeletal and anorectal defects.1,3,9 Just as the finding of one of these anomalies should prompt the clinician to exclude OA, the diagnosis of OA apparently in isolation should also stimulate a detailed review to identify any associated defects. Although several authors have suggested a higher frequency of associated anomalies in OA without fistula, up to 65%, this has not been a feature of all series with the exception of a higher frequency of chromosomal abnormalities, particularly Trisomy 21 and 18.3,33,41 Several well-recognised syndromes or associations should be specifically excluded if two or more anomalies have been identified: VACTERL, CHARGE, Potter’s (renal agenesis, pulmonary hypoplasia, dysmorphic facies) and SCHISIS (neural tube defects, oral clefts, CDH, genital hypoplasia).1,11,42 Delayed diagnosis Although rare, delayed diagnosis of OA with tracheo-oesophageal fistula [TOF] at 25 days of life with subsequent survival has been reported.43 Much more commonly delay in the diagnosis of an associated proximal fistula or ‘H or N-type’ TOF may occur, with at least one case diagnosed in an adolescent.3,4,27,44,45 In the former group, clinicians should consider the possibility prior to surgery and consider performing endoscopy of both the trachea and upper oesophageal pouch immediately prior to surgical repair. If necessary, the assistance of an experienced paediatric ENT surgeon may be required.46 As the oesophagus remains in continuity in a patient with an Htype TOF, diagnostic delay often occurs.27,44,45 Typically patients present with coughing episodes during feeds, recurrent pneumonia, cyanotic spells and intermittent abdominal distension with excessive flatulence.2,27,44,45 Generally located in the lower cervical region/upper thoracic region, the fistula may be missed at endoscopy or during an inexpertly performed contrast study.2,27,47

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INVESTIGATON Following clinical examination, investigations serve one of three purposes in the infant with OA: first, to confirm the diagnosis, secondly to provide additional information pertaining to the OA to guide optimal surgical management and thirdly to determine the presence of associated anomalies. Thus, just as it might be inappropriate to offer surgical intervention for OA in an infant with Trisomy 18, the optimal time to diagnose a right-sided aortic arch would not appear to be during a right-sided thoracotomy for repair of OA.48,49 Diagnostic investigations for OA and TOF In the majority of cases a chest radiograph with the ‘gastric’ tube in place will provide sufficient confirmation of the clinical diagnosis. Several features may assist in estimating the likely surgical difficulty: the presence of 13 ribs or what appears to be a short upper pouch despite gentle pressure of the ‘gastric’ tube suggests a long-gap.50 Similarly, a ‘gasless’ abdomen suggests the diagnosis of OA without fistula, which typically will be associated with a long-gap but may occur as a result of mucus plugging of a distal fistula.51 Whilst not the primary purpose of this investigation, additional clues of important associated anomalies should be sought, including vertebral or rib anomalies, the ‘double-bubble’ of duodenal atresia or an abnormal cardiac shadow.1 Institutional expertise will guide investigation of more complex cases in which the diagnosis remains unclear. Whilst advocated in some centres, prone oesophagograms require a high degree of radiological expertise, may be associated with a false negative result and complications including aspiration pneumonia.52 Other centres favour endoscopy prior to surgical repair, with the use of contrast studies reserved for those patients in whom the diagnosis remains unclear or to monitor oesophageal growth in a patient with a long-gap.41,46 Endoscopy, in addition to assessment of any degree of associated tracheomalacia, allows easier identification of any fistula by placement of a stent immediately prior to surgical repair.2 Diagnostic investigations for associated anomalies Whilst important, these investigations should be tailored to the history and clinical findings. Those patients diagnosed antenatally may have had a foetal ECHO and karyotyping performed.1,18 These results should be reviewed in the context of the patient’s clinical examination and may need to be repeated. In the infant that has passed urine, has no dysmorphic features and appears clinically normal, a chest radiograph [identifies most vertebral anomalies] and ECHO may be all that is required. The majority of centres, including ours, would include a renal US as a routine investigation.2,52 Conversely, those infants with obvious associated anomalies should be very carefully assessed, with a formal cardiology and genetic assessments as part of their examination for cardiac, genitourinary, gastrointestinal and musculoskeletal abnormalities.1,2 MANAGEMENT Resuscitation and stabilisation Following diagnosis, the upper pouch should be suctioned via a 10F Replogle tube to reduce the risk of aspiration.1,2 No further attempts should be made to feed the infant, who should be nursed in the Neonatal Intensive Care Unit (NICU) with the head slightly elevated to assist drainage of the upper pouch and decrease the risk of regurgitation.2 Intravenous access should be established and

maintenance fluids commenced.2 Some infants will require respiratory support, especially those that are premature, have associated cardiac anomalies or in whom the diagnosis has initially been missed and diaphragm splinted secondary to gastric distension.1,2 Handling should be reduced to a minimum and an experienced neonatal nurse allocated to the patient.2 In infants requiring respiratory support, great care should be taken over intubation and the supervision of ventilation. The risk of excessive gastric distension can be reduced by careful positioning of the tip of the endotracheal tube (ETT) just beyond any distal fistula and the use of low-pressure ventilation.1 A rare but well recognised complication remains increasing gastric distension, subsequent respiratory embarrassment and ultimately perforation leading to a tension pneumoperitoneum.1,52 This challenging and potentially rapidly fatal situation appears best managed by an emergency ligation of the distal fistula via a transpleural approach.52,53 Endoscopic placement of a Fogarty balloon catheter, whilst conceptually an elegant temporising manoeuvre, requires a high degree of expertise and has not proven a successful approach in most centres.1,2 Surgical repair Primary repair may now be achieved in the majority of cases.1,16,54–57 The standard approach remains a right posterolateral, muscle-sparing thoracotomy in the 4th interspace adopting an extrapleural approach.1,2 Following its identification, the fistula should be divided completely and the resultant cuff on the trachea oversewn.52 The upper pouch, more readily identified once gentle pressure has been applied to the Replogle tube, should then be mobilised and a primary repair performed.1 There appears limited evidence to support the use of a trans-anastomotic tube and the majority of surgeons would not currently routinely use an intercostal catheter providing the repair remains extrapleural.1,56 In most centres the infant would return to the NICU ventilated and with the neck flexed to reduce anastomotic tension. A routine contrast study appears unnecessary and oral feeding may be commenced once the infant is able to swallow their saliva.1,58 The use of Minimally Invasive Surgery (MIS) has increased dramatically in children in parallel with advances in equipment and surgical experience with these techniques.59,60 Following the first reports of OA repair without fistula in 1999, and with TOF in 2000, MIS has been supported by a number of centres reporting equivalent initial results to open surgery.61–63 There remain no medium to long-term outcome data, however, with suggestions of a higher incidence of early anastomotic structure.61,63 Certainly this procedure requires a highly skilled surgeon with extensive experience in MIS.3,60 Long-gap techniques A wide variety of strategies have been adopted to deal with a long-gap and problematic anastomotic tension, including circular or spiral myotomies, extensive mobilisation of the distal oesophagus and tubularisation of an upper pouch flap.64–67 The relative success of these different approaches, each with differing complications, remains difficult to determine; due in part to subjectivity in the measurement of any gap, its subsequent tension and any reduction as a result.33 Whilst the literature would now suggest that a distance of four or more vertebral bodies whilst under tension be regarded as long-gap, the optimal surgical repair for this group of patients remains controversial with no clear consensus.33,68 In general, retention of the native oesophagus would seem the best approach, at least initially, with a number of series advocating the benefits of delayed primary repair on the basis of differential growth of the oesophagus.55,69

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The benefits of exerting some tension on the oesophagus to stimulate growth to enable primary repair has been revived by the use of traction sutures, reported by Foker et al in 1997.70 Despite some technical difficulties, this approach has gained increasing acceptance although other centres remain proponents of oesophageal replacement in this setting.1,33,71–74 Whether any replacement should involve the stomach, colon or small bowel generally reflects the individual centres experience with each conduit, with supporters for each and no clear evidence of significant differences in long-term function.33,73–77

divided, with an oesophageal anastomotic leak and close apposition of the suture line of the posterior wall of the trachea and the sutures in the oesophagus.82–84 The diagnosis of a recurrent TOF may be suspected in a child with repeated lower respiratory tract infections who under goes a chest radiograph and mediastinal air is seen outlining the oesophagus.1 A routine contrast oesophagram has a low yield and bronchoscopy will be needed to confirm the diagnosis. At this time a catheter is placed through the fistula and its distal end viewed in the oesophagus as a lead up to corrective surgery.

COMPLICATIONS

Oesophageal Stricture

Short term complications, including anastomotic leak and sepsis, remain relatively common, reflecting the prematurity of many of these infants and the complexity of their surgery, but may usually be managed non-operatively. Other complications such as feeding difficulties, fistula recurrence, oesophageal strictures. gastro-oesophageal reflux (GOR) and pulmonary aspiration are common in the medium to longer term with implications for management into adulthood. Many of these problems presenting in the early years persist into adult life and reflect the ability of adaptive behaviours, such as with GOR and swallowing, rather than resolution of co-morbidities.78

Whilst 95% of early anatomotic leaks will resolve spontaneously, up to 50% may be complicated by an oesophageal stricture.83 Oesophageal strictures have been reported to occur in 6% to 40% of cases of oesophageal atresia.81 An anastomotic stricture is more common when the anastomotic gap is greater than 2.5 cm and this is thought to result from the fact that the repair is under tension.85 Additionally, vascular compromise of the lower oesophagus may occur with mobilisation as a result of devascularisation from its more precarious blood supply via segmental vessels originating from the aorta or intercostal arteries, in contrast to the more reliable arterial supply to the upper oesophagus from the inferior thyroid artery.41 The type of suture material, as well as the suture pattern employed, has also been suggested, but not proven, to influence stricture formation.83 Oesophageal strictures occur much more frequently in association with GOR.83 The presence of an oesophageal stricture has been reported in 52% of patients with GOR as opposed to 22% of patients with no reflux.86 The management of oesophageal stricture involves serial dilatations, but these become less frequent with increasing age, decreasing from one per year in the first 10 years of life to once every five years in the third decade in one small series of 12 patients followed longitudinally.87 Certainly, the presence of gastro-oesophageal reflux is common and when present is treated pharmacologically to reduce the risk of stricture.83 Approximately 70% of cases complicated by oesophageal strictures will require dilatation.81,86.

Feeding difficulties These are near universal in the months following repair of OA with or without fistula. They relate to the disruption of oesophageal motility which predisposes to gastro-oesophageal reflux, dysphagia, and poor co-ordination of swallowing and pulmonary aspiration. In the early post-operative period swallowing will be compromised and many children will be dependent upon nasogastric or transpyloric feeding because of impaired swallow. Anastomotic Leak This is a relatively common complication, reportedly occurring in 15% to 20% of cases, with there being a significant disruption to recovery in less than a third of cases.16,79,80 The major leaks occur early in the post-operative course and can present with a tension pneumothorax, necessitating chest drain placement and potentially surgery to oversew the leak, or in extreme cases, the need to undo the repair and opt for a cervical oesophagostomy and oversewing of the distal oesophagus pending oesophageal replacement surgery.1 In contrast, minor leaks are common and may be noted on a ‘‘routine’’ contrast study performed five to seven days after the primary repair. A conservative approach is warranted as these leaks will heal spontaneously, but are nonetheless associated with a greater incidence of later stricture development or recurrence of the fistula.1,81 Fistula recurrence The incidence of recurrent TOF has been suggested to be 5%-14% and to occur within 18 months of the initial surgery from series reported more than 20 years ago.46,82 This number is likely to be lower currently with the advent of better surgical instruments and imaging as well as improved surgical techniques. Nonetheless, it is important to differentiate a recurrent fistula from a second congenital fistula located nearby, typically in the pouch of the original fistula.This can usually be achieved by performing a bronchoscopy prior to the original TOF repair. A recurrent fistula is more common after a primary oesophageal repair under tension, with an original fistula that was ligated rather than completely

Gastro–oesophageal reflux and Barrett’s Oesophagus It has been suggested that around 40% of infants will have GOR necessitating treatment following OA/TOF repair.41,81 Half of these children will be managed successfully with medical therapy to suppress gastric acid production and the remainder may require a fundoplication.88,89 The failure rate for fundoplication carried out in the first three months of life is high.90 If untreated, GOR has the potential to result in oesophagitis and intestinal metaplasia [Barrett’s Oesophagus], which may predispose to adenocarcinoma.91 As reviewed by Deurloo et al. [2003],78 only three studies have prospectively looked at the prevalence of GOR in patients who underwent repair of OA with endoscopy and histological biopsies.92–94 These found esophagitis in the adults up to 26 years after surgery, but only Krug et al [1999]94 found Barrett’s oesophagus. The questionnaire based study by Deurloo et al [2003]78 surveyed symptoms of GOR in 38 adults operated on for OA/TOF between 1947 and 1972 which represented 95% of the survivors [n = 40, aged 28 to 45 years] that could be traced from a single surgical centre in the Netherlands. Of the 38 patients, a subset of 23 [61%] agreed to undergo endoscopy and biopsy of their oesophagus. Ten patients [26%] had no complaints, thirteen patients [34%] experienced trouble swallowing solid foods, two [5%] had trouble swallowing mashed food and fifteen [40%] needed to wash down

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their food with drinks. None of the patients were under medical care for their complaints and none used anti-reflux medications at the time of completing the questionnaire. Of the 23 patients, 19 [81%] had a normal appearance of their oesophagus on direct inspection, 13 [56%] had a hiatus hernia demonstrated, 2 [9%] had grade 1 oesophagitis and 2 [9%] had macroscopic Barrett’s oesophagus [Grade V oesophagitis]. There was no correlation between reported symptoms and abnormalities on oesophageal biopsy, nor between symptoms and the presence of a hiatus hernia. The authors could not recommend endoscopy screening for oesophagitis in adults who underwent repair of OA/TOF in infancy. Pulmonary aspiration Recurrent lower respiratory tract infections, bronchitis and aspiration pneumonia are more common in infancy and the preschool years but become much less common by middle childhood. The prevalence may decrease from 85% at 12 months to 25% by the age of eight years.95 In this study, a history of dysphagia was associated with developing bronchitis or pneumonia. Recurrent pulmonary aspiration and impaired muco-ciliary clearance predisposes bronchiectasis, although from the limited data available, this occurrence has been reported in up to 22% of adults.96 The same large series from Melbourne in 1988 reported that the rates of hospital admission decreased from 38% of patient in the first decade to 1.5% in adulthood. Tracheomalacia Tracheomalacia is a structural and functional weakness in the wall of the trachea which results in partial and occasionally complete obstruction of the lumen of the trachea. It occurs as a consequence of abnormal tracheal rings, deficient in cartilage, and an increase in length of the transverse muscle.97 This will result in collapse of the airway during expiration and will give rise to a brassy, ‘‘TOF’’ cough. It is also associated with impaired mucociliary clearance, recurrent bronchitis and, in extreme cases, life threatening apnoea.1 Tracheomalacia is a consistent finding in most if not all children with TOF, but is reported to be clinically significant in only 10-20% of patients.98 Tracheomalacia appears much less frequent in children with isolated OA, occurring in only 17%.99,100. Severe tracheomalacia, associated with ‘dying spells’ or Apparent Life Threatening Events [‘‘ALTEs’’] will necessitate intervention with an aortopexy being the most common operative procedure.101 In this procedure, the ascending aorta and the arch of the aorta are elevated anteriorly by being attached to the posterior aspect of the sternum. This procedure is useful as the posterior aspect of the aorta is adherent to the anterior aspect of the trachea and by pulling the anterior wall of the trachea forwards, the lumen [crosssectional area] of the trachea will increase in size.83 The operation is needed uncommonly in children with TOF, possibly in 5-10% cases.1 The placement of airway stents for various types of tracheomalacia has been used in recent years with mixed results. Although the placement of the stent is minimally invasive, life threatening complications have occurred when the stents were removed.102 Whether tracheomalacia requires treatment or not, the natural history is one of improvement with age.95

for %FEV1 and %FVC either in the normal range95,99,104 or reduced.103,104 The mean TLC percent predicted was reported in four of the five series and was normal.95,99,104,105 More recently, Malmstrom and colleagues [2008]106 reported on a longitudinal follow-up of bronchial inflammation, respiratory symptoms and lung function in a cohort of 31 adolescents managed at a single centre in Sweden. Responses to a questionnaire completed by 27/31 [87%] patients were correlated with serial bronchial biopsy findings [ages <3 yrs, 3-7 years and > 7 years], spirometry and exhaled nitric oxide values. Approximately 40% of subjects aged 9 to 19 years had current respiratory [cough and wheeze] or oesophageal symptoms [dysphagia and ‘heartburn’]. Spirometry showed approximately a normal pattern in a third, a mildly obstructed pattern in a third and a restrictive pattern in a third. Increased bronchial responsiveness was weakly associated with respiratory symptoms and a low vital capacity, but the mean exhaled nitric oxide was within the predicted normal range. The changes on biopsy of the reticular basement membrane from the airways, even in the presence of atopy, did not reflect the changes that one would see in asthma. The largest series of patients followed into adulthood was reported by Chetcuti et al. [1992],107 which showed generally reassuring results. In this series of 155 patients [74 adults] only minor lung function abnormalities were seen, with only 10% having evidence of severely deranged lung function. Airways disease was more pronounced in younger patients. Between a third and a half of subjects had an increase in their peak inspiratory flow to peak expiratory flow ratio and VE50/VI50, suggestive of obstruction at the extrathoracic level. Scoliosis and chest wall deformities Chest wall deformities are common in patients with OA/TOF. In the large Melbourne series reported by Chetcuti et al. [1989],107 19% [58/302] had a spinal deformity: the later was three times more likely [47% vs 14%] in patients with vertebral anomalies compared to those without vertebral anomalies. Scoliosis associated with fixed vertebral anomalies in the lower thoracic spine had the worst prognosis. A congenital scoliosis was apparent in 15% of cases. Only one of 26 patients with an isolated tracheooesophageal fistula had a vertebral anomaly. Patients with anorectal and rectal abnormalities were more likely to have vertebral anomalies, as would be consistent with VACTERL syndrome. Nonetheless, a thoracic scoliosis was present in 14% of patients with normal vertebrae. A series from Cudmore [1990]108 reported 16% of patients with anterior chest wall asymmetry, a further 4% with anterior chest wall deformity and scoliosis and 6% with scoliosis in isolation. If there was an anterior chest wall deformity in isolation, the spirometry was normal. Scoliosis was associated with a lower value for total lung capacity, but remained within the normal range. In addition to vertebral anomalies, an open thoracotomy can lead to significant musculoskeletal morbidity.3 Atrophy of serratus anterior muscle leading to chest wall asymmetry was noted in 20% and a winged scapula in 24% resulting from partial paralysis of the latissimus dorsi muscle in a cohort of TOF patients.109 Similarly, pectoral muscle and breast maldevelopment may result from surgical incisions.110

Lung function and symptoms Improving outcomes: Role of multi-disciplinary clinics Pulmonary function tests conducted in infants soon after the repair of OA/TOF have demonstrated abnormal airflow with increased airway resistance.103 A series of studies have measured lung function in older children and adolescents following surgery for OA/TOF and have demonstrated mean percent predicted values

Whilst the outcomes for children with OA and TOF have been generally favourable, there remains the need for better established multi-disciplinary clinics to follow these children in a prospective manner. This would undoubtedly generate expertise, allow for

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better documentation of outcomes and ultimately improve the care for these children.

References 1. Spitz L. Oesophageal atresia. Orphanet J Rare Dis 2007;2:24. 2. Beasley SW. Esophageal atresia and tracheoesophageal fistula. In: Oldham KT, Colombani PM, Foglia RP, Skinner MA, editors, (Eds.), Principles and practice of pediatric surgery, Philadelphia: Lippincott Williams & Wilkins, p. 1039–52. 3. Goyal A, Jones MO, Couriel JM, Losty PD. Oesophageal atresia and tracheooesophageal fistula. Arch Dis Child Fetal Neonatal Ed 2006;91:F381–4. 4. Bax KN, Roskott AM, van dZ. Esophageal atresia without distal tracheoesophageal fistula: high incidence of proximal fistula. J Pediatr Surg 2008;43:522– 5. 5. Ioannides AS, Copp AJ. Embryology of oesophageal atresia. Semin Pediatr Surg 2009;18:2–11. 6. DePaepe A, Dolk H, Lechat MF, EUROCAT Working Group. The epidemiology of tracheo-oesophageal fistula and oesophageal atresia in Europe. Arch Dis Child 1993;68:743–8. 7. Torfs CP, Curry CJ, Bateson TF. Population-based study of tracheoesophageal fistula and esophageal atresia. Teratology 1995;52:220–32. 8. Orford J, Glasson M, Beasley S, Shi E, Myers N, Cass D. Oesophageal atresia in twins. Pediatr Surg Int 2000;16:541–5. 9. Chittmittrapap S, Spitz L, Kiely EM, Brereton RJ. Oesophageal atresia and associated anomalies. Arch Dis Child 1989;64:364–8. 10. Scharli AF. Esophageal atresia. Pediatr Surg Int 1997;12:549. 11. Keckler SJ, St Peter SD, Valusek PA, et al. VACTERL anomalies in patients with esophageal atresia: an updated delineation of the spectrum and review of the literature. Pediatr Surg Int 2007;23:309–13. 12. Myers NA. The history of oesophageal atresia and tracheo-oesophageal fistula–1670-1984. Prog Pediatr Surg 1986;20:106–57. 13. Said M, Mekki M, Golli M, et al. Balloon dilatation of anastomotic strictures secondary to surgical repair of oesophageal atresia. Br J Radiol 2003;76:26–31. 14. Haight C. Congenital Atresia of the Esophagus With Tracheoesophageal Fistula: Reconstruction of Esophageal Continuity by Primary Anastomosis. Ann Surg 1944;120:623–52. 15. Myers NA. Oesophageal atresia. Pediatr Surg Int 1992;7:83–5. 16. Spitz L. Esophageal atresia: past, present, and future. J Pediatr Surg 1996;31:19–25. 17. Calisti A, Oriolo L, Nanni L, Molle P, Briganti V, D’Urzo C. Mortality and long term morbidity in esophageal atresia: the reduced impact of low birth weight and maturity on surgical outcome. J Perinat Med 2004;32:171–5. 18. Houben CH, Curry JI. Current status of prenatal diagnosis, operative management and outcome of esophageal atresia/tracheo-esophageal fistula. Prenat Diagn 2008;28:667–75. 19. Ahmed A, Spitz L. The outcome of colonic replacement of the esophagus in children. Prog Pediatr Surg 1986;19:37–54. 20. Ure BM, Slany E, Eypasch EP, Weiler K, Troidl H, Holschneider AM. Quality of life more than 20 years after repair of esophageal atresia. J Pediatr Surg 1998;33:511–5. 21. Bouman NH, Koot HM, Hazebroek FW. Long-term physical, psychological, and social functioning of children with esophageal atresia. J Pediatr Surg 1999;34:399–404. 22. Schier F, Korn S, Michel E. Experiences of a parent support group with the longterm consequences of esophageal atresia. J Pediatr Surg 2001;36:605–10. 23. Ludman L, Spitz L. Quality of life after gastric transposition for oesophageal atresia. J Pediatr Surg 2003;38:53–7. 24. Little DC, Rescorla FJ, Grosfeld JL, West KW, Scherer LR, Engum SA. Long-term analysis of children with esophageal atresia and tracheoesophageal fistula. J Pediatr Surg 2003;38:852–6. 25. Choudhry M, Boyd PA, Chamberlain PF, Lakhoo K. Prenatal diagnosis of tracheo-oesophageal fistula and oesophageal atresia. Prenat Diagn 2007; 27:608–10. 26. Stringer MD, McKenna KM, Goldstein RB, Filly RA, Adzick NS, Harrison MR. Prenatal diagnosis of esophageal atresia. J Pediatr Surg 1995;30:1258–63. 27. Brookes JT, Smith MC, Smith RJ, Bauman NM, Manaligod JM, Sandler AD. Htype congenital tracheoesophageal fistula: University Of Iowa experience 1985 to 2005. Ann Otol Rhinol Laryngol 2007;116:363–8. 28. Farrant P. The antenatal diagnosis of oesophageal atresia by ultrasound. Br J Radiol 1980;53:1202–3. 29. Havard AC, MacDonald LM. Oesophageal atresia and other disorders with a similar antenatal presentation. Br J Radiol 1991;64:557–8. 30. Barnhard Y, Bar-Hava I, Divon MY. Is polyhydramnios in an ultrasonographically normal fetus an indication for genetic evaluation? Am J Obstet Gynecol 1995;173:1523–7. 31. Pretorius DH, Drose JA, Dennis MA, Manchester DK, Manco-Johnson ML. Tracheoesophageal fistula in utero. Twenty-two cases. J Ultrasound Med 1987;6:509–13. 32. Sparey C, Robson SC. Oesophageal atresia. Prenat Diagn 2000;20:251–3. 33. Holland AJ, Ron O, Pierro A, et al. Surgical outcomes of esophageal atresia without fistula for 24 years at a single institution. J Pediatr Surg 2009;44:1928– 32. 34. Eyheremendy E, Pfister M. Antenatal real-time diagnosis of esophageal atresias. J Clin Ultrasound 1983;11:395–7.

105

35. D’Elia A, Pighetti M, Nappi C. Prenatal ultrasonographic appearance of esophageal atresia. Eur J Obstet Gynecol Reprod Biol 2002;105:77–8. 36. Levine D. Magnetic resonance imaging in prenatal diagnosis. Curr Opin Pediatr 2001;13:572–8. 37. Levine D, Barnewolt CE, Mehta TS, Trop I, Estroff J, Wong G. Fetal thoracic abnormalities: MR imaging. Radiology 2003;228:379–88. 38. Malinger G, Levine A, Rotmensch S. The fetal esophagus: anatomical and physiological ultrasonographic characterization using a high-resolution linear transducer. Ultrasound Obstet Gynecol 2004;24:500–5. 39. Gopal M, Woodward M. Potential hazards of contrast study diagnosis of esophageal atresia. J Pediatr Surg 2007;42:E9–10. 40. Seefelder C, Elango S, Rosbe KW, Jennings RW. Oesophageal perforation presenting as oesophageal atresia in a premature neonate following difficult intubation. Paediatr Anaesth 2001;11:112–8. 41. Spitz L, Kiely E, Brereton RJ. Esophageal atresia: five year experience with 148 cases. J Pediatr Surg 1987;22:103–8. 42. Spitz L, Kiely E, Brereton RJ, Drake D. Management of esophageal atresia. World J Surg 1993;17:296–300. 43. Gupta A, Narasimhan KL. Unusually delayed presentation of esophageal atresia. Pediatr Surg Int 2004;20:906. 44. Karnak I, Senocak ME, Hicsonmez A, Buyukpamukcu N. The diagnosis and treatment of H-type tracheoesophageal fistula. J Pediatr Surg 1997;32:1670–4. 45. Ng J, Antao B, Bartram J, Raghavan A, Shawis R. Diagnostic difficulties in the management of H-type tracheoesophageal fistula. Acta Radiol 2006;47:801–5. 46. Benjamin B. Endoscopy in esophageal atresia and tracheoesophageal fistula. Ann Otol Rhinol Laryngol 1981;90:376–82. 47. Benjamin B, Pham T. Diagnosis of H-type tracheoesophageal fistula. J Pediatr Surg 1991;26:667–71. 48. Bowkett B, Beasley SW, Myers NA. The frequency, significance, and management of a right aortic arch in association with esophageal atresia. Pediatr Surg Int 1999;15:28–31. 49. Okamoto T, Takamizawa S, Arai H, et al. Esophageal atresia: prognostic classification revisited. Surgery 2009;145:675–81. 50. Kulkarni B, Rao RS, Oak S, Upadhyaya MA. 13 pairs of ribs–a predictor of long gap atresia in tracheoesophageal fistula. J Pediatr Surg 1997;32:1453–4. 51. Gedicke MM, Gopal M, Spicer R. A gasless abdomen does not exclude distal tracheoesophageal fistula: the value of a repeat x-ray. J Pediatr Surg 2007;42:576–7. 52. Morrow SE, Ashcraft KW. Esophageal atresia. In: Ziegler MM, Azizkhan RG, Weber TR, editors (Eds.), Operative Pediatric Surgery, New York: McGraw-Hill, p. 349–54. 53. Malone PS, Kiely EM, Brain AJ, Spitz L, Brereton RJ. Tracheo-oesophageal fistula and pre-operative mechanical ventilation. Aust N Z J Surg 1990;60:525–7. 54. Tonz M, Kohli S, Kaiser G. Oesophageal atresia: what has changed in the last 3 decades? Pediatr Surg Int 2004;20:768–72. 55. Orford J, Cass DT, Glasson MJ. Advances in the treatment of oesophageal atresia over three decades: the 1970s and the 1990s. Pediatr Surg Int 2004;20:402–7. 56. Patel SB, Ade-Ajayi N, Kiely EM. Oesophageal atresia: a simplified approach to early management. Pediatr Surg Int 2002;18:87–9. 57. Mortell AE, Azizkhan RG. Esophageal atresia repair with thoracotomy: the Cincinnati contemporary experience. Semin Pediatr Surg 2009;18:12–9. 58. Yanchar NL, Gordon R, Cooper M, Dunlap H, Soucy P. Significance of the clinical course and early upper gastrointestinal studies in predicting complications associated with repair of esophageal atresia. J Pediatr Surg 2001;36:815–22. 59. Rothenberg SS, Chang JH, Bealer JF. Experience with minimally invasive surgery in infants. Am J Surg 1998;176:654–8. 60. Ponsky TA, Rothenberg SS. Minimally invasive surgery in infants less than 5 kg: experience of 649 cases. Surg Endosc 2008;22:2214–9. 61. Rothenberg SS. Thoracoscopic repair of esophageal atresia and tracheo-esophageal fistula. Semin Pediatr Surg 2005;14:2–7. 62. Lugo B, Malhotra A, Guner Y, Nguyen T, Ford H, Nguyen NX. Thoracoscopic versus open repair of tracheoesophageal fistula and esophageal atresia. J Laparoendosc Adv Surg Tech A 2008;18:753–6. 63. Holcomb III GW, Rothenberg SS, Bax KM, et al. Thoracoscopic repair of esophageal atresia and tracheoesophageal fistula: a multi-institutional analysis. Ann Surg 2005;242:422–8. 64. Livaditis A, Radberg L, Odensjo G. Esophageal end-to-end anastomosis. Reduction of anastomotic tension by circular myotomy. Scand J Thorac Cardiovasc Surg 1972;6:206–14. 65. Kimura K, Nishijima E, Tsugawa C, Matsumoto Y. A new approach for the salvage of unsuccessful esophageal atresia repair: a spiral myotomy and delayed definitive operation. J Pediatr Surg 1987;22:981–3. 66. Gough MH. Esophageal atresia–use of an anterior flap in the difficult anastomosis. J Pediatr Surg 1980;15:310–1. 67. Lessin MS, Wesselhoeft CW, Luks FI, DeLuca FG. Primary repair of long-gap esophageal atresia by mobilization of the distal esophagus. Eur J Pediatr Surg 1999;9:369–72. 68. Ron O, De Coppi P, Pierro A. The surgical approach to esophageal atresia repair and the management of long-gap atresia: results of a survey. Semin Pediatr Surg 2009;18:44–9. 69. Sri Paran T, Decaluwe D, Corbally M, Puri P. Long-Term results of delayed primary anastomsis for pure oesophageal atresia: a 27 year follow up. Paediatr Surg Int 2007;23:647–51. 70. Foker JE, Linden BC, Boyle Jr EM, Marquardt C. Development of a true primary repair for the full spectrum of esophageal atresia. Ann Surg 1997;226:533–41.

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A.J.A. Holland, D.A. Fitzgerald / Paediatric Respiratory Reviews 11 (2010) 100–107

71. Foker JE, Kendall Krosch TC, Catton K, Munro F, Khan KM. Long-gap esophageal atresia treated by growth induction: the biological potential and early followup results. Semin Pediatr Surg 2009;18:23–9. 72. Lopes MF, Reis A, Coutinho S, Pires A. Very long gap esophageal atresia successfully treated by esophageal lengthening using external traction sutures. J Pediatr Surg 2004;39:1286–7. 73. Spitz L, Kiely E, Pierro A. Gastric transposition in children–a 21-year experience. J Pediatr Surg 2004;39:276–81. 74. Spitz L. Gastric transposition in children. Semin Pediatr Surg 2009;18:30–3. 75. Anderson KD, Noblett H, Belsey R, Randolph JG. Long-term follow-up of children with colon and gastric tube interposition for esophageal atresia. Surgery 1992;111:131–6. 76. Lindahl H, Louhimo I, Virkola K. Colon interposition or gastric tube? Follow-up study of colon-esophagus and gastric tube-esophagus patients. J Pediatr Surg 1983;18:58–63. 77. Arul GS, Parikh D. Oesophageal replacement in children. Ann R Coll Surg Engl 2008;90:7–12. 78. Deurloo JA, Ekkelkamp S, Bartelsman JFWM, et al. Gastroesophageal Reflux: Prevalence in Adults Older than 28 years After Correction of Esophageal Atresia. Ann Surg 2003;238:686–9. 79. Harmon CM, Coran AG. Congenital anomalies of the esophagus. In: O’Neill Jr JA, Rowe MI, Grosfeld JL, editors. Pediatric surgery. St. Louis, MO: Mosby; 1998. p. 941–67. 80. Chittmittrapap S, Spitz L, Kiely EM, Brerton RJ. Anastomotic Leakage following surgery for esophageal atresia. J Pediatr Surg 1992;27:29–32. 81. Engun SA, Grosfeld JL, West KW, et al. Analysis of morbidity and mortality in 227 cases of esophageal atresia and/or tracheoesophageal fistula over two decades. Arch Surg 1995;130:502–8. 82. Ghandour KE, Spitz L, Brereton RJ, Kiely EM. Recurrent tracheo-esophageal fistula: experience with 24 patients. J Paediatr Child Health 1990;26:89–91. 83. Kovesi T, Rubin S. Long Term complications of Esophageal Atresia and/or Tracheoesophageal Fistula. Chest 2004;126:915–25. 84. Vos A, Ekkelkamp S. Congenital tracheo-esophageal fistula: preventing recurrence. J Pediatr Surg 1996;31:936–8. 85. McKinnon LJ, Kosloske AM. Prediction and prevention of anastomotic complications of esophageal atresia and tracheoesophageal fistula. J Pediatr Surg 1990;25:778–81. 86. Chittmittrapap S, Spitz L, Kiely EM, et al. Anastomotic stricture following repair of esophageal atresia. J Pediatr Surg 1990;25:508–11. 87. Biller JA, Allen JL, Schuster SR, et al. Long-term evaluation of esophageal and pulmonary function in patients with repaired esophageal atresia and tracheoesophageal fistula. Dig Dis Sci 1987;32:985–90. 88. Parker AF, Christie DL, Cahill JL. Incidence and significance of gastroesophageal reflux following repair of esophageal atresia and tracheoesophageal fistula and the need for antireflux procedures. J Pediatr Surg 1979;14(1):5–8. 89. Wheatley MJ, Coran AG, Wesley JR. Efficacy of the Nissen fundoplication in the management of gastroesophageal reflux following esophageal atresia repair. J Pediatr Surg 1993;28(1):53–5. 90. Kubiak R, Spitz L, Kiely EM, Drake D, Pierro A. Effectiveness of fundoplication in early infancy. J Pediatr Surg 1999;34(2):295–9. 91. Hameeteman W, Tytgat GNJ, Houthoff HJ, et al. Barrett’s esophagus: development of dysplasia and adenocarcinoma. Gastroenterology 1989;96:1249–56.

CME SECTION This article has been accredited for CME learning by the European Board for Accreditation in Pneumology (EBAP). You can receive 1 CME credit by successfully answering these questions online. (A) Visit the journal CME site at http://www.prrjournal.com. (B) Complete the answers online, and receive your final score upon completion of the test. (C) Should you successfully complete the test, you may download your accreditation certificate (subject to an administrative charge). Educational questions Answer true or false to the following questions: 1. In relation to the incidence and diagnosis of OA: a. OA appears more common in Asian populations b. Antenatal diagnosis may now be routinely expected as a result of improvements in maternal US screening c. An upper pouch sign on antenatal US in association with polyhydramnios confirms the diagnosis of OA d. A long-gap may be expected if the chest radiograph reveals 13 ribs e. A gas-less abdomen is typically associated with an isolated OA

92. Lindahl H, Rintala R, Sariola H. Chronic esophagitis and gastric metaplasia are frequent late complications of esophageal atresia. J Pediatr Surg 1993;28:1178–80. 93. Somppi E, Tammela O, Ruuska T, et al. Outcome of patients operated on for esophageal atresia: 30 years’ experience. J Pedaitr Surg 1998;33:1341–6. 94. Krug E, Bergmeijer JH, Dees J, et al. Gastroesophageal reflux and Barrett’s esophagus in adults born with esophageal atresia. Am J Gastroenterol 1999;94:2825–8. 95. Couriel JM, Hibbert M, Olinsky A, et al. Long term pulmonary consequences of oesophageal atresia with tracheo-eosophageal fistula. Acta paediatr Scand 1982;71:973–8. 96. Chetcuti P, Myers NA, Phelan PD, et al. Adults who survived repair of congenital oesophageal atresia and tracheo-oesophageal fistula. BMJ 1988; 297:344–6. 97. Wailoo MP, Emery JL. The trachea in children with tracheo-oesophageal fistula. Histopathology 1979;3:329–38. 98. Spitz L. Esophageal atresia and tracheoesophageal fistula in children. Curr Opin Pediatr 1993;5:347–52. 99. Robertson DF, Mobaireek K, Davis GM, et al. Late pulmonary function following repair of tracheoeosophageal fistula or oesophageal atresia. Pediatr Pulmonol 1995;20:21–6. 100. Rideout DT, Hayashi AH, Gillis DA, et al. The absence of significant tracheomalacia in patients having oesophagela atresia without tracheoeosophageal atresia. J Pediatr Surg 1991;26:1303–5. 101. Filler RM, Messineo A, Vinograd I. Severe tracheomalacia associated with esophageal atresia: results of surgical treatment. J Pediatr Surg 1992;27:1136– 41. 102. Nicolai T. Airway stents in children. Pediatr Pulmonol 2008;43:330–44. 103. Agrawal L, Beardsmore CS, MacFadyen UM. Respiratory function in childhood following repair of tracheoesophageal fistula. Arch Dis Child 1999;81: 404–8. 104. Van Gysel D, De Boeck Lerut T, et al. Pulmonary status during childhood after corrected oesophageal atresia [abstract]. Eur Respir J 1992;15(Supp):103s. 105. LeSouef P, Myers NA, Landau LI. Etiologic factors in long-term respiratory function abnormalities following oesophageal atresia repair. J Pediatr Surg 1987;22:918–22. 106. Malmstrom K, Lohi J, Lindahl H, et al. Longitudinal Follow-up of Bronchial Inflammation. Respiratory Symptoms and Pulmonary Function in Adolescents after Repair of Esophageal Atresia with Tracheoesophageal Fistula. J Pediatr 2008;153:396–401. 107. Chetcuti P, Dickens DRV, Phelan PD. Spinal deformity in patients born with oesophageal atresia and tracheo-oesophageal fistula. Arch Dis Child 1989;64:1427–30. 108. Cudmore RE. Oesophageal atresia and tracheo-eosophageal fistula. In: Lister J, Irving IM, editors. Neonatal Surgery. 3rd Ed., London, UK: Butterworths; 1990. p. 231–58. 109. Jaureguizar E, Vazquez J, Murcia J, et al. Morbid musculoskeletal sequelae of thoracotomy for tracheoesophageal fistula. J Pediatr Surg 1985;20:511–4. 110. Cherup LL, Siewers RD, Futrell JW. Breast and pectoral muscle maldevelopment after anterolateral and posterolateral thoracotomies in children. Ann Thorac Surg 1986;41:492–7.

There are no obvious racial variations in the prevalence of OA. Antenatal imaging is yet to provide definitive assistance in making the diagnosis of OA. The presence of 13 ribs is associated with a higher likelihood of a long gap in OA. Similarly a gas-less abdomen suggests an isolated OA because of a lack of communication of the lower pouch with the tracheo-bronchial tree. 2. In the management of TOF: a. Minimally Invasive Surgical repair is now the standard of care b. A chest drain is not required if the repair has been performed using a extrapleural approach c. A contrast study should be performed following the repair before commencing oral feeds d. The optimal approach to the management of long-gap OA appears to be oesophageal replacement e. The presence of Trisomy 18 remains an absolute contraindication to OA repair Minimally invasive surgery is not considered the standard of care for OA repair. A standard extrapleural approach will not require a chest drain to be placed. Oesophageal replacement is not the preferred option even with long

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gap OA. A contrast study is no longer considered a routine test before oral feeds are introduced post-operatively. A syndromal diagnosis of trisomy 18 is not considered a contra-indication for surgery. 3. In relation to complications following OA and TOF repair a. GOR remains a frequent association and generally requires the routine use of an H2 antagonist b. An anastomotic leak may lead to subsequent development of a stricture and therefore requires surgical revision for optimal results c. Fistula recurrence may represent a missed second fistula d. A recurrent fistula may often settle with time and so may be treated non-operatively e. Tracheomalacia appears more commonly associated with isolated OA due to anastomotic tension resulting from the long-gap Fistula recurrence may include a pre-existing fistula that was missed at presentation as well as a complication of the repair. Anastomotic leaks are relatively common but usually do not require surgical intervention as most settle with conservative management. A degree of tracheomalacia is probably universal in OA and TOF patients but only a minority, perhaps 10%, will need surgical intervention. 4. With regard to the risk of a tracheo-oesopphageal fistula recurring: a. The risk is reported to be <1%. b. A primary anastomosis achieved under significant tension will increase the risk of fistula recurrence? c. The risk is greater with a ligated rather than divided original fistula. d. The appearance of mediastinal air on a chest radiograph in a child with recurrent lower respiratory tract infections should raise the suspicion of a recurrent fistula.

e. An oesophagram is the gold standard test for diagnosing a fistula recurrence. The risk of a recurrent fistula may be as high as 18% according to the literature but is probably lower than this if multiple fistulae present at birth are properly excluded. Nonetheless, the figure for fistula recurrence is higher than 1%. It should be suspected in a child with recurrent chest infections and mediastinal air on the chest radiograph. Factors believed to increase the risk of recurrence include a primary repair under tension and ligating rather than dividing the original fistula. An oesophagram is not a reliable method for diagnosing a recurrence. A bronchoscopy is a superior, but not perfect, diagnostic test. 5. Longer term issues to consider in children with TOF include: a. Tracheomalacia is a significant problem in 80% of patients. b. Lung function tends to be considerably reduced in studies of older children and adults. c. A brassy cough continues into adulthood. d. Barrett’s oesophagus is a common problem in adulthood. e. Spinal abnormalities [eg scoliosis] are three times more likely in patients with vertebral anomalies. Tracheomalacia, giving rise to a brassy cough, is a near uniform occurrence but a significant problem requiring intervention in a minority, perhaps 10%, of patients. The cough continues into adulthood. Lung function tends to be normal or only slightly reduced in follow-up studies. Recurrent gastro-oesophageal reflux is common and predisposes to pre-malignant change [Barrett’s oesophagus] in a small minority of patients. Spinal abnormalities predispose to scoliosis and occur 3 times more commonly in patients with vertebral anomalies.

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