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Newborn babies presenting with difficulties related to the aerodigestive tract (ADT) are often provisionally diagnosed and managed as having oesophageal atresia +/- tracheo-oesophageal fistula. Continuing difficulties with management and abnormal findings on investigations should lead to the consideration of other congenital anomalies of the ADT, including complete larnygo-tracheo-oesophageal cleft (LTOC). We present two patients who were eventually diagnosed with complete LTOC and care was withdrawn. We discuss the inherent difficulties in reaching this diagnosis and present an algorithm to help manage these rare and challenging situations.

Background The role of simulation training in paediatric surgery is expanding as more simulation devices are designed and validated. We aimed to conduct a training needs assessment of UK paediatric surgical trainees to prioritise procedures for simulation, and to validate a novel 3D-printed simulation model for oesophageal atresia and tracheo-oesophageal fistula (OA-TOF) repair. Methods A questionnaire was sent to UK trainee paediatric surgeons surveying the availability and utility of simulation. The operation ranked as most useful to simulate was OA-TOF repair. 3D-printing techniques were used to build an OA-TOF model. Content, face and construct validity was assessed by 40 paediatric surgeons of varying experience. Results Thirty-four paediatric surgeons completed the survey; 79% had access to surgical simulation at least monthly, and 47% had access to paediatric-specific resources. Perceived utility of simulation was 4.1/5. Validation of open OA-TOF repair was conducted by 40 surgeons. Participants rated the model as useful 4.9/5. Anatomical realism was scored 4.2/5 and surgical realism 3.9/5. The model was able to discriminate between experienced and inexperienced surgeons. Conclusion UK paediatric surgeons voted OA-TOF repair as the most useful procedure to simulate. In response we have developed and validated an affordable 3D-printed simulation model for open OA-TOF repair.

This study is aimed to evaluate the effectiveness of osteopathic manipulative treatment (OMT) in dysphagia symptoms and aspiration risk. This is a clinical case report on a 40-day-old infant with a diagnosis of oesophageal atresia and tracheo-oesophageal fistula. The patient received one OMT every 15 days for 7 times. The clinical outcome was the clinical changes in dysphagia symptoms assessed by the Paediatric Eating Assessment Tool-10 (PEDI-EAT-10). At the first osteopathic evaluation (T0), the total score of the PEDI-EAT-10 was 7, at the fourth osteopathic evaluation (T1), the total score was 3, and at the seventh osteopathic evaluation (T2), the total score was 1. OMT was effective and safe in reducing dysphagia symptoms and aspiration risk.

Oesophageal atresia and tracheo-oesophageal fistula is a congenital structural abnormality that affects 1:4500 live infants. It is due to failure of the primitive foregut tube to separate correctly into oesophagus and trachea. About 50% have associated abnormalities, of which the VACTERL (Vertebral, Anorectal, Cardiac, Tracheo-oEsophageal, Renal and Limb) association is the most common. Prematurity is common and all have some degree of tracheomalacia. Surgery of the common type can be performed through a fourth interspace thoracotomy or by thoracoscopy. It involves division of the distal tracheo-oesophageal fistula and anastomosing together the two ends of the oesophagus. The absence of a distal fistula reveals itself as a ‘gasless abdomen’ on plain radiology, and usually indicates a long gap between the blind oesophageal ends: this sometimes necessitates an oesophageal replacement if extensive oesophageal mobilization fails to achieve an end-to-end anastomosis of the oesophagus. Potential post-operative problems include anastomotic leak, anastomotic stricture, recurrence of the fistula, gastro-oesophageal reflux, oesophageal dysmotility and food impaction. Survival is determined mainly by coexisting congenital abnormalities. The long-term risk of oesophageal malignancy is yet to be established. Isolated tracheo-oesophageal fistula (‘H fistula’) can occur without atresia, and often presents after feeding has commenced. It is divided through a cervical incision.

Oesophageal atresia and tracheo-oesophageal fistula are congenital anomalies of the oesophagus requiring surgical repair in infancy, either by open or thoracoscopic approach. Although mortality rates associated with this procedure are low, children may go on to have complications throughout childhood and into adulthood, most commonly related to ongoing gastrointestinal and respiratory symptoms. This review outlines the early, mid and long-term outcomes for these children in terms of quality of life and incidence of symptoms.

Development of hypertrophic pyloric stenosis (HPS) after a few weeks of repair of an oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF) is a rare condition in early infancy. Although vomiting or feeding intolerance in operated cases of OA+TOF are attributed to oesophageal stricture, gastro-oesophageal reflux and oesophageal dysmotility, it may also be caused by HPS. Herein, we report a newborn infant who had OA and TOF operation on day 2 of life and diagnosed to have HPS at 15th day of age. Even though it is a rare anomaly, HPS should be kept on mind in the presence of persistent vomiting following repair of OA.

Congenital oesophageal atresia and tracheo-oesophageal fistula are one of the anomalies that require expert surgical intervention and commitment for correction to achieve a good long-term outcome. Traditionally, the repair is achieved by an open thoracotomy with an extrapleural approach to repair the tracheo-oesophageal fistula and anastomose the oesophagus that has over the years produced good long-term outcomes. However, recent minimally invasive thoracoscopic approach to achieve the same goal is being practised by many skilled surgeons, and they are improving their results by sharing their technical tips. Both these techniques are discussed in this chapter. The complications of the surgery include anastomotic leakage and/or complete dehiscence with resultant mediastinal and pleural infection, pneumothorax, oesophageal stricture, gastro-oesophageal reflux and recurrent trachea-oesophageal fistula. The infants require long-term follow-up by a committed Pediatric surgeon to regularly monitor their growth and developments, swallowing, reflux and acquired spinal abnormalities.

Aim A recent international study reported a higher prevalence of oesophageal atresia with or without tracheo-oesophageal fistula (OA±TOF) in Western Australia (WA). The aim of this study was to examine the prevalence and trends of OA and/or TOF in WA, determine the proportion of cases with associated anomalies and explore the impact of time of diagnosis. Methods The study population comprised all infants born in WA, 1980–2009, and registered with OA and/or TOF on the WA Register of Developmental Anomalies (WARDA). Results OA±TOF and TOF alone affect, on average, one in every 2927 births in WA, with a total prevalence of 3.00 and 0.42 per 10 000 births, respectively. The prevalence of OA±TOF increased by 2.0% per annum, with only cases with associated anomalies (64% of cases) demonstrating an increase. TOF rates were stable. Among OA±TOF infants, the proportion of live births, stillbirths and elective terminations of pregnancy for fetal anomaly (TOPFA) was 79%, 6% and 15%, respectively, whereas the majority (94%) of TOF only cases were live births. In 2000–2009, there was 30% fall in OA±TOF live births with 61 (58%) cases diagnosed in first week of life, 10 (9%) prenatally and 34 (32%) at post-mortem only. Conclusions A higher prevalence of OA±TOF in WA was observed with increase over time attributable to increase with associated anomalies. Consistent reporting, availability of prenatal diagnosis and ascertainment of cases following TOPFA or post-mortem examinations can significantly affect prevalence of OA and/or TOF.

Oesophageal atresia with tracheo-oesophageal fistula is a relatively common congenital anomaly occurring in around 1:2500 births. The aetiology and embryology of the condition remain unclear, whilst associations with other significant anomalies are common. Studies in rodent models are contributing to our understanding of the condition. Advances in surgical care and neonatal management have improved survival considerably to around 90%. Long-gap and isolated oesophageal atresia present significant management challenges. Post-operative and long-term complications including oesophageal stricture, gastro-oesophageal reflux and respiratory compromise however remain relatively common and continue to pose a challenge for the ongoing management of patients.

Tracheo-oesophageal fistula (TEF)/oesophageal atresia is one of the most common and serious congenital malformation. Despite progresses made in the field of early diagnosis, surgical techniques, ventilatory support and control of chest infections; morbidity and mortality still remains quite high and differs a lot from one to another centre particularly in the developing countries; as the availability and the level of neonatal care facilities are different. Associated anomalies play a significant role in dictating the outcome, timing of intervention and even the approach to management. The objectives of this review article is to outline the spectrum of associated anomalies, emphasise need of standardised system of documentation of anomalies, prognosis and management issues that would influence timing and approach of TEF repair.

Oesphageal atresia is a relatively common congenital anomaly that requires urgent diagnosis, transfer to a neonatal surgical centre and management by a multidisciplinary team. Peri-operative management requires vigilant monitoring for the many possible associated morbidities. There are unique anaesthetic, airway and ventilatory considerations for this group of patients. Beyond the perinatal period, systematic neurodevelopmental follow-up is recommended to better understand the longer term outcomes for these children.

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 co-morbidities from infancy to adulthood are reviewed to provide a broad overview of the condition.

This contribution outlines the knowledge, skills and controversies that trainees in paediatric surgery must understand to manage oesophageal atresia. The commonest anomaly and its surgical repair are discussed.

The aetiology of OA/TOF is poorly understood. The birth of an infant with OA/TOF in a family without a previous history of the condition is associated with a recurrence risk of about 1%. The twin concordance rate for OA/ TOF is about 2.5%. These data suggest that genetic factors do not play a major role in the pathogenesis of OA/TOF. However, there are specific chromosomal anomalies that predispose to this condition, such as trisomy 18 and 21. Three separate genes associated with OA/ TOF in humans have recently been identified. Analysis of anomalous tracheo-oesophageal development in adriamycin-exposed rat embryos suggests that defective sonic hedgehog (Shh) gene expression, signalling, or both, leads to disruption of the normal development of the foregut. Animal models also suggest that the embryological origin of the upper oesophagus is different from that of the distal oesophagus. Human studies suggest that the fistula develops from a trifurcation of the embryonic lung bud. Associated anomalies

Oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF) are severe congenital anomalies of which the aetiology is largely unknown. Several chromosomal anomalies have been described in patients presenting with these anatomical malformations, but until now none of these has led to the identification of a single aetiological factor. This paper reviews the chromosomal abnormalities reported in cases of OA/TOF and serves as a starting point to identify chromosomal regions harbouring genes involved in the aetiology of OA/TOF.

The most common major birth defects affecting the oesophagus and trachea are oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF). Epidemiological information on OA and/or TOF in the USA has been derived from only a few populations. Sixty-one cases of OA or TOF were identified through a population-based birth defects registry in Hawaii during 1986-2000. The relationship between various clinical and demographic factors and OA or TOF were examined. The rate of OA or TOF was 2.2 per 10,000 live births. The cases were 67.2% OA with TOF, 21.3% TOF alone, and 11.5% OA alone. Risk of OA or TOF was significantly higher among multiple births and live births with low birth weight and gestational age. The risk of OA or TOF was significantly higher for whites. The rate of OA or TOF was consistent with that reported elsewhere. The risk of the defects differed significantly between the major racial/ethnic groups in Hawaii. The impact of many factors on OA or TOF in Hawaii was similar to that reported elsewhere.

Oesophageal atresia and tracheo-oesophageal fistula are relatively frequently occurring foregut malformations of which the aetiology and pathogenesis are poorly understood. Recent results of molecular genetic studies, in particular the use of single and compound mutant mice, have yielded a tremendous increase in the understanding of the molecular mechanisms involved in normal and abnormal foregut morphogenesis. In the introduction of this paper, we review the very early stages of normal and abnormal embryology of the foregut derivatives and the separation of the foregut into a ventral respiratory part and a dorsal digestive part. After that, we describe the genes that have been demonstrated to play an important role in these processes.

Gastro-oesophageal reflux (GOR) is a major cause of morbidity in children who undergo surgical repair for oesophageal atresia with tracheo-oesophageal fistula (OA/TOF). We performed a retrospective analysis to determine the incidence of GOR on radionuclide scintigraphy in symptomatic and asymptomatic OA/TOF patients in the first post-operative year. A total of 124 patients (74 males, 50 females), with a mean age of 3.5 months (range, 20 days to 12 months), were studied. Of these 124 patients, 67 were symptomatic and 57 were asymptomatic. On radionuclide scintigraphy, 73 patients (48 symptomatic and 25 asymptomatic) had reflux. Of the 48 symptomatic patients with scintigraphic studies positive for reflux, 79.2% (38) had proximal reflux and 20.8% (10) had distal reflux, whereas, of the 57 asymptomatic patients, 48% (12) had proximal reflux and 52% (13) had distal reflux. There was a significantly higher incidence of GOR in symptomatic children than in asymptomatic children (P

After surgical management of their oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF), most patients exhibit evidence of gastro-oesophageal reflux (GOR) and many have oesophagitis. However, the aetiology of the GOR is still controversial. This study was undertaken to document whether there are congenital abnormalities in the intra-abdominal oesophagus and the hiatus in the fetal rat with OA and TOF following exposure to adriamycin (ADR). Time-pregnant rats were injected daily with either saline or 2 mg/kg ADR intraperitoneally on gestational days (GD) 6-9. The fetuses (n = 56) from 8 litters were harvested on GD 21 for examination. The length of the oesophagus between the diaphragmatic crura and the gastro-oesophageal junction (GOJ) and the sizes of the stomach and the oesophageal hiatus were measured under a dissecting microscope. The length of the oesophagus between the diaphragmatic crura and the GOJ in the ADR-treated fetuses (0.85 +/- 0.37 mm) was significantly shorter than in control fetuses (2.41 +/- 0.32 mm) (P0.0001). The size of the stomach in ADR-treated fetuses (5.30 +/- 1.01 mm) was significantly smaller than in the controls (8.07 +/- 0.49 mm) (P0.001). Moreover, the size of the oesophageal hiatus in ADR-treated fetuses (1.16 +/- 0.43 mm) was markedly larger than in the controls (0.32 +/- 0.1 mm) (P0.0001). These results showed that the congenital abnormalities in ADR-treated rat fetuses may account for the oesophageal functional disorders seen after surgical correction in patients who have OA and TOF.

Associated congenital anomalies have emerged as the most significant prognostic factor in babies born with oesophageal atresia and/or tracheo-oesophageal fistula (OA-TOF). The most frequently encountered groups of anomalies are cardiovascular (CV) and gastrointestinal, the former being more significant from a prognostic point of view. Some, such as a right-sided aortic arch (RAA), vascular ring, or major heart defects, may alter the timing and surgical approach for the repair of OA-TOF or adversely affect the prognosis. The rat fetal OA model induced by adriamycin (Adr) has been described previously. In the present experiments, information was sought regarding the incidence and type of CV abnormalities in fetal rats obtained from Adr-treated dams. OA-TOF was induced in 24 of 36 fetal rats from Adr-treated dams. DV abnormalities were found in 18 (75%) OA-TOF fetuses and 10 (83%) Adr-treated fetuses without OA-TOF. The difference was not significant (P >0.05). The most frequently found anomalies were ventricular and atrial septal defects. A RAA was present in 8/36 fetuses and a double aortic arch in 2/36. A patent ductus arteriosus was present in all treated fetuses compared with two-thirds of controls. The findings in the present study emphasise the importance of CV anomalies in association with OA, and reinforce the value of the Adr-induced rat fetal OA model by adding to our knowledge of the basic embryogenesis of both OA and CV anomalies.

Clinically significant gastro-oesophageal reflux (GOR) following oesophageal atresia and tracheo-oesophageal fistula (OA/TOF) repair is commonly considered normal sequela after repair. A retrospective review of patients operated on by two consultants was undertaken. All patients underwent oesophageal tailoring and augmentation for reconstruction of their oesophagus. The presence of clinically significant GOR was confirmed by contrast swallows, 24 hour pH study and endoscopy. Clinically significant GOR occurred in 7 (13%) of the 54 patients operated for OA and TOF. Two patients responded to non-surgical management. Four children (one with extensive tracheo-bronchomalacia and one with CHARGE association) had anti-reflux surgery (three Nissen and one Thal). We believe that oesophageal tailoring and augmentation for reconstruction of the oesophagus has the advantage of creating a more uniform oesophagus thus avoiding swallowing difficulty, bolus obstruction and the need of oesophageal dilatations too often accepted as integral to the problem following OA repair.

The cooccurrence of congenital diaphragmatic hernia and oesophageal atresia with distal tracheo-oesophageal fistula is very rare and carries high mortality. Very few anecdotal case reports and one case series have been reported in the literature. We report a case of a late preterm, low birth weight infant with this rare association who was successfully managed by staged surgical approach and had good outcome.

Aim A right-sided aortic arch (RAA) occurs in around 5% of patients with oesophageal atresia and tracheo-oesophageal fistula (OA/TOF). This anatomical variation can complicate the operative management of these patients, as it is often not diagnosed preoperatively but only discovered at thoracotomy, and it remains unproven as to whether a right or left thoracotomy is the best operative approach. This retrospective study aimed to determine the prevalence of RAA in OA/TOF, review the accuracy of preoperative investigations, and investigate the best operative approach, by reviewing the literature and our own patient series. Methods The case notes of all infants with OA/TOF over a 15 year period (1994–2008) were retrospectively analysed to identify those with a RAA. Birth weight, gestational age, associated anomalies, preoperative investigations, surgical management, postoperative complications and long-term prognosis were all extracted. Main Results A total of 107 case notes of OA/TOF infants were reviewed, identifying 4 with a RAA. Preoperative echocardiography was performed in all of the 4 RAA infants, but RAA was only identified in one. All 4 infants were managed surgically via a right thoracotomy, regardless of the echocardiography result, with primary anastomosis achieved successfully in all. A laryngeal cleft repair was performed in 1 infant due to an interarytenoid cleft. Laparoscopic fundoplication was performed in 1 patient, because of severe gastro-oesophageal reflux. There were no postoperative anastomotic leaks, bleeding, or deaths in this group. Conclusion In our study, the incidence of RAA in OA/TOF was 3.7%. Preoperative echocardiography identified the RAA in only 1 of 4 cases. However, echocardiography was helpful for diagnosing other cardiac anomalies, which might have potentially affected the management of these patients. Previous studies have cited the operative difficulties associated with RAA and OA/TOF. However, in this series of 4 infants, primary anastomosis was achieved via conventional right thoracotomy without complication, and with no effect on outcome or prognosis. Therefore, we conclude that, where possible, a conventional right-sided thoracotomy should be performed in OA/TOF patients with a RAA.

BACKGROUND: Respiratory complications are common after neonatal repair of oesophageal atresia and tracheo-oesophageal fistula. The prevalence of lung function abnormalities and the relation between gastrointestinal complications and lung function has not been studied in a large number of patients. METHODS: Lung volumes and flow-volume loops were measured in 155 patients without spinal curvature aged 6-37 years who had undergone surgery for oesophageal atresia and tracheo-oesophageal fistula. RESULTS: Sixty four of the 155 patients had evidence of mild lower airways disease, with values for FEV1 more than two standardised scores below the predicted value in 39 (25%) and above 2 standardised scores for the residual volume (RV)/total lung capacity (TLC) ratio in 64 (41%). Restrictive lung disease (TLC more than 2 standardised scores below predicted) was present in 28 (18%). Severe lung function abnormalities were present in under 10% of the 155. Half the subjects had some evidence of extra-thoracic tracheal obstruction, with a high ratio of expiratory to inspiratory flow for peak flow in 76 (50%) and at 50% of vital capacity in 59 (38%). Patients with radiological gastro-oesophageal reflux in early childhood had more airways obstruction and smaller lung volumes. Patients with current gastrointestinal symptoms were similar in their lung function to symptom free patients. CONCLUSIONS: Minor lung function abnormalities are common in patients after repair of oesophageal atresia. Early diagnosis and management of gastro-oesophageal reflux may help to minimise these lung function abnormalities.

Oesophageal atresia-tracheo-oesophageal fistula has featured in paediatric surgery since its beginnings. The first successful primary repair was in 1941. With overall survival now exceeding 90% in dedicated centres, the emphasis has changed to reducing morbidity and achieving improvements in the quality of life. An overview of current and emerging strategies in managing patients with this condition is presented. Advances in developmental biology and molecular genetics reflecting improved understanding of the pathogenesis are highlighted.

Background: Oesophageal atresia and tracheo-oesophageal fistula (TOF) in neonates and young infants is routinely diagnosed using frontal and lateral chest radiographs in the former and fluoroscopic studies in the latter. Objective: The aim of the study was to assess whether a combination of mediastinal and abdominal sonography can be used for the diagnosis of these anomalies in paediatric patients. Material and methods: Sonography was performed in 16 neonates (age range 1–20 days; mean 4 days) with radiologically confirmed oesophageal atresia or isolated TOF. A small volume of saline solution was instilled into the blind upper oesophageal pouch to document its extension. Results: Sonography identified 11 neonates with the most common type of oesophageal atresia (type IIIb), one patient with type II and one with type IIIa anomaly. The length of the upper pouch and the features of its wall were clearly documented in all cases. In one case, two upper TOF were first diagnosed by mediastinal sonography and later confirmed by fluoroscopy. In two of three cases with isolated TOF, the fistula could be located sonographically by detecting moving air bubbles. In all cases the position of the aortic arch, as well as associated malformations, could be documented during a single US examination. Conclusions: These results indicate that mediastinal sonography is a useful tool for the diagnosis of oesophageal atresia and, if air bubbles can be detected, isolated TOF.

Two neonates developed sialorrhoea and feeds intolerance. A nasogastric tube (NGT) was passed with difficulty …

The management of oesophageal atresia and tracheo-oesophageal atresia (OATOF) is very challenging. While in developed countries survival of patients with this condition has improved, the outcome in many developing countries has been poor. Primary repair through a thoracotomy (or video-assisted thoracoscopic surgery where available) is the gold standard treatment of OATOF. However, in our setting where patients typically present late and with minimum support resources such as Neonatal Intensive Care Unit and total parenteral nutrition; staged repair may be the only hope of survival of these patients and this communication highlights the essential steps of this mode of treatment.

Department of Social Medicine, University of Birmingham, and the Department of Child Health, University of Durham, at King's College, Newcastle upon Tyne. There is a common superstition among hospital staffs that rare diseases tend to come in groups. One possible explanation for this belief may be found in the character of the frequency distribution of lengths of intervals between suc cessive events occurring at random. In contrast with the distributions of everyday experience, where, for example, a very short man is also an uncommon man, the distribution of random intervals is exponential, and the intervals be come more frequent as they get shorter and further from the mean. The unfamiliarity of this pattern could easily produce impressions of grouping, and our chief difficulty is not in detecting groups of illnesses but in dis tinguishing random groups from those which could not reasonably be attributed to chance. The present paper is an exercise in such distinction. Oesophageal atresia and tracheo oesophageal fistula commonly occur in the same child and may be considered together as a relatively uncommon congenital malformation. Yet Gross (1953) was able to show on a single photograph no fewer than nine children who were patients at the Children's Hospital, Bos ton, for the treatment of this condition. At least six appear to be very young infants, presumably having a primary repair, and other statistics given suggest that this represents a remarkable coincidence of events. Indeed, it supplies a concise statement of the specific problem with which this paper is concerned.

A term baby girl had respiratory distress on first feed. She had cardiac impulse and heart sounds on the right side. Liver was palpable below the left costal margin with liver dullness on the left side. An orogastric tube got arrested at 10 cm. Chest x-ray showed the arrest of a replogal tube with its tip at T4 in keeping with oesophageal atresia (OA) and dextrocardia (figure 1A). X-ray …

Three cases of upper gastrointestinal perforation affecting the stomach, duodenum, and jejunum, respectively, are described. Each occurred in premature infant with oesophageal atresia and tracheo-oesophageal fistula on ventilatory support. In each case there was a delay in diagnosis. All infants survived. The management options and current opinion on the treatment of this difficult problem are discussed.

Upper gastrointestinal morbidity and anthropometric data in 334 patients aged 1 to 37 years with repaired oesophageal atresia and tracheo-oesophageal fistula are reported. Two thirds were subsequently hospitalised with oesophageal complications and half underwent one or more surgical procedures. Thirteen percent were hospitalised for more than 50 days. Anastomotic strictures were present in one third and just under half had gastro-oesophageal reflux. Dysphagia was present in about 65% in all age groups but symptom severity and surgical intervention decreased significantly after 5 years of age. Gastro-oesophageal reflux symptoms were commoner in the older patients increasing from 18% in the group under 5 years of age to 52% in those over 15 years. Height centile distribution was normal, but the median weight centile was the 25th. Weight for height values greater than 2 SD below the mean were present in 13%, and two thirds of these patients were under 5 years of age. The majority of adults enjoyed a normal lifestyle and were comparable with their peers.

The history of oesophageal atresia extends over three centuries and contains many fascinating accounts of the anomaly. Initially, these accounts described the clinical presentation and pathology, but as time passed, an increasing interest in the surgical management developed. The critical year was 1888, the year in which Charles Steele made the first surgical attempt at correction of the anomaly. Significant events in the understanding and management of oesophageal atresia before and after that date are summarized in Tables 1.1 and 1.2.

Spinal deformity was present in 58 (19%) of 302 patients born with oesophageal atresia and fistula when examined at review. This was present in 24 (47%) of 51 patients with vertebral anomalies and 34 (14%) of 251 patients with normal vertebrae. Scoliosis was present in 21 patients with vertebral anomalies, torticollis in two, and lordosis in one. Two thirds of the patients with congenital scoliosis have had or are likely to require operation, compared with five patients with non-congenital scoliosis. Scoliosis associated with mixed vertebral anomalies in the lower thoracic spine had the worst prognosis. The medical records of a further 64 patients who had survived operation but who could not be traced were reviewed, and indicated that four had had congenital vertebral anomalies but none had a spinal deformity. We recommend early detection of vertebral anomalies in this group and careful follow up of patients with these abnormalities.

A series of 86 infants (54 boys and 32 girls) with congenital oesophageal atresia and tracheo-oesophageal fistula underwent operation during the years 1952--76. The operative technique is described. The average survival rate was 45%, increasing to 56% during the last 10 years. With correction for low birth weight and associated congenital anomalies, the survival rate is considerably increased, in our series to 73%. In most of the fatal cases, the causes of death were suture leakage, pulmonary complications and associated anomalies. Among the 36 survivors, 19 became free from symptoms and 19 had a radiographic stricture, but in the latter group dysphagia was present in only 13, including 10 who required repeated dilatation with a Fogarthy balloon catheter. It is emphasized that correct and early diagnosis and meticulous pre- and postoperative care are of the greatest importance if the cure rate is to be improved further.

There appears to be no question about the advantages of endotracheal intubation in the administration of anaesthesia for the repair of oesophageal atresia and tracheo-oesophageal fistula. Controlled respirations provide ideal working conditions when every advantage is needed for working in a small space. Ventilation is maintained even though there may be severe pulmonary disease. Minimal anaesthesia is required. Laryngeal oedema cannot be held as a deterrent with all the advantages to be gained. Finally, the anaesthesiologist is in a position to aid in the early diagnosis.

As this is the history of oesophageal atresia, it is appropriate and virtually obligatory to commence with reference to two classical contributions — that of Durston [31] in 1670 and that of Gibson [46] in 1697. However, at the outset it is equally appropriate to give credit to those who have previously researched the history, none more thoroughly than Ashcraft and Holder [7] and, in more recent times, Chang [24]. With many of the contributions in the literature, introductory and later paragraphs frequently describe events of historical significance, and these contributions are also acknowledged.

One hundred and twenty five adults who were born before 1969 with oesophageal atresia or tracheo-oesophageal fistula or both and were managed at the Royal Children's Hospital, Melbourne, were reviewed. Most enjoyed a normal life. Though over half had difficulties in swallowing and symptoms of gastro-oesophageal reflux, the symptoms occurred only occasionally and were regarded as inconsequential by most. One third of the patients had wheeze and a quarter had at least one episode of bronchitis a year, but these interfered little with daily activities. Overall, these results are encouraging for young patients with oesophageal atresia and their families.

The importance of starting milk-feeding as soon as possible after major operations on newly born children is now widely appreciated. In babies with oesophageal atresia and tracheo oesophageal fistula after division of the fistula and primary anastomosis, few surgeons have such confidence in the oeso phageal anastomosis that they will permit even the swallowing of water within 48 hours of operation. In order to avoid continuous intravenous therapy and yet achieve a satisfactory intake of water and milk from 24 to 36 hours after operation, some form of feeding by tube is necessary, either through a fine tube passed through the nose and across the oesophageal anastomosis into the stomach at operation or by gastrostomy. For a transoesophageal tube it is usual at this hospital to use translucent polyvinyl chloride tubing (2D Portex), which is soft and limp. More than 400 children have been treated here for oesophageal atresia ; in over 300 a primary anastomosis of the oesophagus has been possible, and in most of these a Portex tube was passed across the anastomosis without complications related to the use of the tubing. This report concerns two children in whom the use of a Stifter type of tubing made of nylon resulted in perforation of the anterior gastric wall by the end of the tubing. In both cases generalized peritonitis resulted and one child died.