Distal Tracheoesophageal Fistula Research Articles

Anesthesia Management of Patients with Esophageal Atresia/Traceoesophageal Fistula undergoing Thoracostomy and Esophagostomy Procedures

Esophageal atresia is a congenital disorder characterized by a disruption in the continuity of the esophageal lumen. Esophageal atresia may be accompanied by tracheoesophageal fistula, which is a lumen connection between the proximal and or distal part of the esophagus and the airway (trachea). Type C atresia is the most prevalent, which is approximately 88.5% to 90% of cases, where there is proximal esophageal atresia with distal tracheoesophageal fistula. In this case, a 9-day-old infant was admitted to Arifin Ahmad Hospital for thoracostomy and esophagostomy surgery due to continuous mucus discharge. The patient underwent awake intubation and was positioned intraoperatively, with a tilt to the right when an orogastric tube was placed. During surgery, the patient maintained stable hemodynamic and was subsequently admitted to the NICU using a ventilator. Infants with esophageal atresia often show symptoms of hypersalivation and shortness of breath caused by aspiration pneumonia. When the nasogastric tube cannot pass through the esophagus, atresia can be suspected. Radiology studies play an important role in diagnosing esophageal atresia, confirming esophageal atresia with or without fistula and diagnosing other anomalies associated with VACTERL.

Thoracoscopic Repair for Kluth Type Ⅲb3 Esophageal Atresia and Distal TracheoesophagealFistula

IntroductionThis report aims to present our initial miniseries of successful thoracoscopic repair for esophageal atresia (EA) and distal tracheoesophageal fistula (TEF) of Kluth type Ⅲb3 in accordance with Kluth's classification. MethodsFrom January 2012 to January 2024, ten patients with Kluth type Ⅲb3 EA-TEF were treated by thoracoscopic surgery. The therapeutic methods and surgical outcomes were retrospectively reviewed. ResultsAll procedures were completed thoracoscopically without conversions. A preoperative bronchoscopy assessment was conducted in four of the cases, revealing that the fistula from the distal segment was located high on the trachea at the level of T2 vertebral. The mean age of the patients at the time of operation was 2.0 ± 0.7 d (range, 1-3 d), and the mean weight at operation was 2.6 ± 0.4 kg (range, 1.8-3.0 kg). The mean operative time (skin to skin) for the entire series was 137.0 ± 8.9 min (range, 120-150 min). Oral feeding was initiated on the postoperative day 8.0 ± 1.9 (range, 6-12 d), and the mean duration for patients after surgery was 14.0 ± 2.4 d (range, 12-20 d). The postoperative period has been uneventful with no occurrences of mortality or morbidity to date. Three cases of formatted anastomotic stricture required at least one esophageal dilation after surgery. ConclusionsPediatric surgeons should be aware of the rare variants of EA-TEF to avoid the diagnostic and management pitfalls. Patients with Kluth type Ⅲb3 EA-TEF were amenable to repair by thoracoscopic surgery.

Esophageal atresia type III? Intraoperative surprise and different approaches.

A newborn was referred due to clinical and radiological suspicion of esophageal atresia (EA) type III. Surgery revealed an esophagus without evident interruptions; however, intraoperative advancement of the nasogastric tube was unsuccessful, and the distal esophagus inflated with each ventilation, indicating the presence of a distal fistula. An intraoperative esophago-tracheobronchoscopy showed a proximal esophageal pouch with a tiny tracheoesophageal fistula and a large distal tracheoesophageal fistula. The esophageal ends were blind but overlapping, with no external discontinuity observed. With the diagnosis of Krediet type IIIc2 esophageal atresia, we performed a meticulous esophago-tracheal dissection, distal fistula closure, and end-to-end anastomosis. Due to hemodynamic instability, the proximal fistula was closed two weeks later via cervicotomy without incidents.

Cardiovascular malformation associated with esophageal atresia

Abstract Background Congenital heart disease (CHD) is the commonest abnormality associated with esophageal atresia (EA). EA is a rare congenital malformation occurring in 3-4 in 10,000 births, in 87% occurs along with a distal tracheoesophageal fistula (TEF). The coexistence of a cardiac malformation and in addition, prematurity can be challenging and affects the further treatment and quality of life (QOL) of the child and family. Knowledge of the anatomical type and physiological consequences of CHD allows a well-coordinated treatment plan. Purpose The aim of this paper is to investigate whether the presence of underlying associated malformations, disease-specific problems and prematurity would have a considerable influence on the family of a child after surgical repair of EA. Methods We used a crossed-sectional questionnaire study. The impact of EA was assessed using an Authors-Designed Questionnaire (ADQ) to collect medical and sociodemographic background data (gestational week (HBD), comorbidities, type of EA, according to the Gross classification) as well as standardized questionnaire: the PedsQL™ Family Impact Module (PedsQL-FIM). Results Of the 76 children born with EA/TEF included in this study, 26% had a vascular malformation. Mean age was 5.3 years (SD = 3.7 years). Cardiovascular malformation associated with EA significantly (p=0.037) affects the functioning of the family in the emotional domain (FIM – ED). The QOL (p < 0,034) of children born before 37th HBD is lower that children born in term The presence of concomitant anomalies does affect the overall generic QoL. No statistical correlation was found between the type of anomaly (with/without TEF) and patients' QoL. Conclusions Cardiovascular malformation and prematurity significantly affect child’s QOL and the functioning of the family with EA. Patients who have undergone EA treatment require specialist outpatient care, not only in the first years of life, but also in adolescence and adulthood.

A case of accidental transtracheal gastric passage of feeding tube in esophageal atresia with distal tracheo esophageal fistula: a rare occurrence

A case of accidental transtracheal gastric passage of feeding tube in esophageal atresia with distal tracheo esophageal fistula: a rare occurrence

Thoracoscopic external traction suture elongation for the management of long-gap oesophageal atresia: a two-centre experience.

Van der Zee (VdZ) described a technique to elongate the oesophagus in long-gap oesophageal atresia (LGOA) by thoracoscopic placement of external traction sutures (TPETS). Here, we describe our experience of using this technique. Retrospective review of all LGOA + / - distal tracheo-oesophageal fistula (dTOF) cases where TPETS was used in our institutions. Data are given as medians (IQR). From 01/05/2019 to 01/03/2023, ten LGOA patients were treated by the VdZ technique. Five had oesophageal atresia (Gross type A or B, Group 1) and five had OA with a dTOF (type C, Group 2) but with a long gap precluding primary anastomosis. Age of first traction procedure was Group 1 = 53 (29-55) days and Group 2 = 3 (1-49) days. Median number of traction procedures = 3; time between first procedure and final anastomosis was 6days (4-7). Four cases were converted to thoracotomy at the third procedure. Three had anastomotic leaks managed conservatively. Follow-up was 12-52months. All patients achieved oesophageal continuity and were orally fed; no patient required an oesophagostomy. In this series, TPETS in LGOA facilitated delayed primary anastomoses and replicated the good results previously described but, in addition, was successful in cases with dTOF. We believe traction suture placement and tensioning benefit from being performed thoracoscopically because of excellent visualisation and the fact that the tension does not change when the chest is closed. Surgical and anaesthetic planning and expertise are crucial. It is now our management of choice in OA patients with a long gap with or without a distal TOF.

Esophago-gastric junction findings on high resolution impedance manometry in children with esophageal atresia.

Using high resolution impedance manometry (HRIM), this study characterized the esophago-gastric junction (EGJ) dynamics in children with esophageal atresia (EA). Esophageal HRIM was performed in patients with EA aged less than 18 years. Objective motility patterns were analyzed, and EGJ data reported. Controls were pediatric patients without EA undergoing investigations for consideration of fundoplication surgery. Seventy-five patients (M:F = 43:32, median age 1 year 3 months [3 months-17 years 4 months]) completed 133 HRIM studies. The majority (64/75, 85.3%) had EA with distal tracheo-esophageal fistula. Compared with controls, liquid swallows were poorer in patients with EA, as evident by significant differences in distension pressure emptying and bolus flow time (BFT). The integrated relaxation pressure for thin liquid swallows was significantly different between EA types, as well as when comparing patients with EA with and without previous esophageal dilatations. The BFT for solid swallows was significantly different when compared with EA types. We have utilized HRIM in patients with EA to demonstrate abnormalities in their long-term EGJ function. These abnormalities correlate with poorer esophageal compliance and reduced esophageal peristalsis across the EGJ. Understanding the EGJ function in patients with EA will allow us to tailor long-term management to specific patients.

Diagnose and treatment for Type D congenital esophageal atresia with tracheoesophageal fistula.

Type D esophageal atresia (EA) with tracheoesophageal fistula (TEF) is characterized by EA with both proximal and distal TEFs. It is a rare congenital anomaly with a very low incidence. To investigate diagnostic and treatment strategies for this rare condition. We retrospectively reviewed the clinicopathological features of patients with EA/TEF treated at our institution between January 2007 and September 2021. Among 386 patients with EA/TEF, 14 (3.6%) had type D EA/TEF. Only two patients were diagnosed with proximal TEF preoperatively. Seven patients were diagnosed intraoperatively. Five patients were missed for diagnosis during the initial surgery but was later confirmed by bronchoscopy. During the neonatal period, seven patients underwent a one-stage repair of proximal and distal TEF via thoracoscopy or thoracotomy. Due to missed diagnosis and other reasons, the other 7 patients underwent two-stage surgery for repair of the proximal TEF, including cervical incision and thoracoscopy. Ten of the 14 patients experienced postoperative complications including anastomotic leakage, pneumothorax, esophageal stricture, and recurrence. Patients who underwent one-stage repair of distal and proximal TEF during the neonatal period showed a higher incidence of anastomotic leak (4/7). In contrast, only one of seven patients with two-stage repair of the proximal TEF developed an anastomotic leak. Type D EA/TEF is a rare condition, and proximal TEFs are easily missed. Bronchoscopy may aim to diagnose and determine the correct surgical approach. A cervical approach may be more suitable for repairing the proximal TEF.

Glycopyrrolate as an Adjunct in the Management of Anastomotic Leak Following Repair of Esophageal Atresia: A Clinicoradiological Perspective.

Anastomotic leak after primary repair of esophageal atresia (EA) with tracheoesophageal fistula (TEF) is a well-known complication and can represent a challenging clinical scenario. The present study aimed to evaluate the role of glycopyrrolate as an adjunct in the treatment of anastomotic leak after primary repair of EA Vogt type 3b. A retrospective study was carried out in our tertiary care teaching institute from January 2015 to December 2022. Neonates with EA with distal TEF with primary repair who had developed anastomotic leak, managed by the author(s), were studied. The study included patients with major, minor, and radiological leaks. Glycopyrrolate was administered in the dose of 4 μg/kg 8 hourly. The outcomes of the study were either resolution or progression of the leak. There were 21 patients who were managed with glycopyrrolate in addition to the classical treatment of the anastomotic leak following repair of EA with distal TEF. The male: female ratio was 1:1.1. All the cases had anastomotic leaks with either clinically detectable in the chest tube (15) or radiological leak (6). The leaks were detected early in patients with major leak (mean = 3.2 ± 0.84 days) compared to minor leak (mean =4.9 ± 1.29 days). Radiological leaks were detected in all the neonates on postoperative day 7. In five patients with major leak, there was a negligible reduction in the amount of chest tube output, and were subjected to diversion procedures. There were a total of three deaths out of five in this group. In 10 patients with minor leak, there was complete resolution of anastomotic leak in eight patients (80%); there was one patient each with mortality and diversion procedure. The patients with a radiological leak (6) did not show any deterioration, and they were fed 1-5 days after the esophagogram. Glycopyrrolate may be an advantageous postoperative adjunct in the management of minor and radiological leak after tracheoesophageal repair.

A Minimally Interventional Approach to Oesophageal Atresia Repair With Early Enteral Feeding is Safe, Optimises Neonatal Outcomes, and Reduces Resource use

PurposeRecent series of newborn Oesophageal Atresia (OA) repair continue to report widespread use of chest drains, gastrostomy, routine contrast studies and parenteral nutrition (PN) despite evidence suggesting these are superfluous. We report outcomes using a minimally interventional approach to post-operative recovery. MethodsEthically approved (15/WA/0153), single-centre, retrospective case-note review of consecutive infants with OA 2000–2022. Infants with OA and distal trache-oesophageal fistula undergoing primary oesophageal anastomosis at initial surgery were included (including those with comorbidities such as duodenal atresia, anorectal malformation and cardiac lesions). Our practice includes routine use of a trans-anastomotic tube (TAT), no routine chest drain nor gastrostomy, early enteral and oral feeding, no routine PN and no routine contrast study. Data are median (IQR). ResultsOf total 186 cases of OA treated during the time period, 157 met the inclusion criteria of which 2 were excluded as casenotes unavailable. TAT was used in 150 infants. A chest drain was required in 13 (8%) and two infants had a neonatal gastrostomy. Enteral feeds were started on postoperative day 2 (2–3), full enteral feeds established by day 4 (4–6) and oral feeds started on day 5 (4–8). PN was required in 15%. Median postoperative length of stay was 10 days (8–17). Progress was quicker in term infants than preterm. One infant died of cardiac disease prior to neonatal discharge. Two planned post-operative contrast studies were performed (surgeon preference) and a further 7 due to clinical suspicion of anastomotic leak. Contrast study was therefore avoided in 94%. There were 2 anastomotic leaks; both presented clinically at day 4 and day 8 after oral feeds had been started. ConclusionOur minimally interventional approach is safe. It facilitates prompt recovery with lower resource use, reduced demand on nursing staff, reduced radiation burden, and early discharge home compared to published series without adversely affecting outcomes. Level of EvidenceLevel 4.

First successful neonatal primary repair of esophageal atresia with distal tracheoesophageal fistula in Belize

Esophageal atresia repair represents the epitome of Pediatric Surgery and it is considered as an indicator of the level of quality of any Surgical Neonatal Service. This article describes the first case completely and successfully treated in Belize, and it is focused on its management strategies. In the midst of COVID-19 pandemic, a full-term male neonate, adequate for gestational age and withnormal birth weight was referred to Karl Heusner Memorial Hospital’s Neonatal Intensive Care Unit and diagnosed with esophageal atresia with distal tracheoesophageal fistula. Primary repair was performed through open thoracotomy, closing the tracheoesophageal fistula and completing end to end esophageal anastomosis with minimal tension. A minor anastomotic leak was identified on postoperative day 9, which was initially managed conservatively but required surgical repair 5 days after, using a pedicled intercostal muscle flap over the re-sutured esophagus. The patient recovered completely with no midterm complications. It is feasible to perform complex neonatal surgical repair such as the AE/TEF in small countries like Belize, when both an experienced surgical team and adequate neonatal intensive care are available. Proper management of major surgical complications is essentialto achieve good results.

Are Low Weight or Cardiopathy Contraindications for Thoracoscopic Repair of Esophageal Atresia with Tracheoesophageal Fistula?

Thoracoscopic repair of esophageal atresia (EA) with tracheoesophageal fistula (TEF) is becoming an increasingly widespread technique; there is still controversy about its indication in certain patients. Our objective is to analyze if potential risk factors such as major congenital heart disease (CHD) or low birth weight (LBW) are a limitation to this approach. Retrospective study (2017-2021) of patients with EA and distal TEF who underwent thoracoscopic repair were included. Patients with LBW less than 2,000 g or major CHD were compared with the rest. Twenty-five patients underwent thoracoscopic surgery. Nine patients (36%) had major CHD. Five of them (20%) were LBW less than 2,000 g, and only 8% (2/25) presented both risk factors. There were no differences in terms of operative time, conversion rate, tolerance evaluated with gasometric parameters (pO2, pCO2, pH) or complications (anastomotic leak and stricture, both early or during follow-up) in patients with major CHD and LBW (1,473 ± 319 vs. 2,664 ± 402 g). One conversion to thoracotomy was performed in a neonate weighing 1,050 g due to anesthetic intolerance. There was no recurrence of TEF. One patient died at the age of 9 months, due to major uncorrectable heart disease. Thoracoscopic repair of EA/TEF is feasible technique in patients with CHD or LBW, with similar results to other patients. The complexity of this technique warrants individualizing the indication in each case. IV.

Routine Fogarty Catheter Occlusion of Fistula in Esophageal Atresia with Tracheoesophageal Fistula Surgery: A Retrospective Study

Objective: We aimed to analyze the outcomes of patients who underwent surgical repair of congenital esophageal atresia (EA) with a distal tracheoesophageal fistula (EA/TEF) or a Gross type C with successful routine Fogarty catheter occlusion of TEF. Materials and Methods: We retrospectively reviewed the medical records of patients who underwent surgical repair of Gross type C with successful routine Fogarty catheter occlusion of fistula between April 2010 and November 2016. Results: Nineteen patients were enrolled and included for analysis. Mean gestational age was 38.7 (1.9) weeks with 2 (10.5%) neonates born prematurely. Mean birthweight was 2569.3 (425.3) g. Five (26.3%) patients required mechanical ventilation (MV) before surgical repair of TEF. Median post-operative required MV after TEF surgery was 4 (3-6) days. The most common of post-operative complications were wound dehiscence (10.5%) and pneumothorax (10.5%). Long-term complications were gastroesophageal reflux disease (36.8%) and tracheomalacia (31.6%). Conclusion: The success rate of routine TEF occlusion with a Fogarty catheter was 86.4%. Routine Fogarty catheter occlusion of TEF can be used safely with experienced personnel, low incidence of aspiration and satisfied ventilation. There was no serious complication associated with placement of Fogarty catheter or catheter dislodgement, and it did not occur during any of the procedures.

Intraoperative Bronchoscopic Inspection Facilitates Thoracoscopic Repair of Esophageal Atresia with Tracheoesophageal Fistula.

Aim: The value of intraoperative bronchoscopic inspection (IBI) for accurate confirmation of the location and distance between the distal tracheoesophageal fistula (TEF) and the proximal blind end of the esophagus (GAP) was evaluated in Type C esophageal atresia (EA)+TEF. Methods: IBI involved inserting the tip of a bronchoscope into the TEF and a nasogastric tube into the blind end of the EA and measuring GAP with fluoroscopy. EA+TEF patients (n = 23) treated thoracoscopically between 2007 and 2020 were classified according to IBI as IBI+ (n = 16) and IBI- (n = 7) to compare demographics, operative time, and time taken for TEF division. Results: Demographics were similar. Mean time for TEF division (15.4 ± 4.6 minutes for IBI+ versus 38.6 ± 20.9 minutes for IBI-; p < .05) and mean operative time (215.3 ± 48.9 minutes for IBI+ versus 286.4 ± 51.7 minutes for IBI+; p < .05) were significantly shorter. Mean GAP measured radiographically was 0.5 cm (range: 0-1.2 cm); mean GAP measured with IBI was 0.9 cm (range: 0-2.2 cm). Postoperative complications were 3 anastomotic leakages (1/16 in IBI+ and 2/7 in IBI-) that resolved without surgery and 8 strictures (3/16 in IBI+ and 5/7 in IBI-) treated by dilatation. Conclusions: IBI was effective for measuring GAP and is recommended for improving the efficiency of thoracoscopic repair.

Scimitar syndrome and distal tracheoesophageal fistula with esophageal atresia (type III b): a case report of diagnostic and therapeutic approach

Scimitar syndrome is a rare congenital heart defect (CHD) manifested by a partial abnormal inflow of pulmonary veins of the right lung into the suprahepatic segment of the inferior vena cava (VCI), making an angiographic image with the right heart edge similar to a Turkish saber (“scimitar”). It is found in only 1 to 3 per 100,000 births. Here we are presenting a patient who, in addition to the basic finding and presentation of a special partial anomalous inflow of pulmonary veins, also had other features of the Scimitar syndrome; dextroposition of the heart, without signs of heterotaxy, hypoplasia of the right lung, aberrant arterial supply of the right lung from the descending aorta (lung sequestration) with all hemodynamic signs of left-right flow (dilated right heart cavity and pulmonary artery), but without pulmonary hypertension. In addition, the patient had esophageal atresia with distal tracheoesophageal fistula (TEF). Treatment included operative occlusion of TEF with termino-terminal esophageal anastomosis. In the further course, esophagography revealed circular esophageal stenosis at the anastomosis site, but without the need for dilatation, which resolved spontaneously. Tracheotomy was needed due to the inability to separate from mechanical ventilation. Considering cardiopulmonary stability and the absence of pulmonary hypertension, a complete cardiosurgical correction was postponed to after the first year of life. The review is exceptional due to the concomitant occurrence of a TEF Vogt type IIIb, because the unusual combination of Scimitar syndrome with such type of TEF has not been described in the literature so far. To our knowledge, there is only one described case report with an H-type of TEF.

Left Nostril Agenesis in a Fetus with VACTERL Association: A Case Report

VACTERL association represents a rare condition with a broad spectrum of coexisting congenital abnormalities. Although a multifactorial origin has been described, the etiology is still unclear. Prenatal diagnosis can be challenging, as specific characteristics may be difficult to be determined before birth. This case report describes the finding of multiple congenital abnormalities in a male fetus at 30 weeks of gestation. The most notable findings were the presence of duodenal stenosis, esophageal atresia with distal tracheoesophageal fistula (type 3), interatrial septal communication foramen ovale, and left nostril and choana agenesis. After birth and making a broad differential diagnosis, all these characteristics oriented the diagnosis towards the VACTERL association. To the best of our knowledge, no cases of VACTERL association and unilateral nostril and choana agenesis have been previously described.

Atresia de esôfago com história familiar positiva: um relato de caso

INTRODUCTION: Esophageal atresia is a malformation marked by discontinuity of the esophagus, where the proximal part ends in a cul-de-sac, with or without the presence of a fistula with the trachea. An incidence pathology of 1 in every 2500 to 4500 live births, predominantly in individuals of white race and males, whose diagnosis can be made in the prenatal period by ultrasound or in the post-natal period when the clinical suspicion is present. The diagnosis must be early for treatment success, avoiding complications such as the evolution from pneumonia to respiratory failure. In view of the investigation, family history can be an alert for early suspicion. OBJECTIVES: To describe a case of esophageal atresia with distal tracheoesophageal fistula associated with heart disease, diagnosed after birth, in a female child, who had a positive family history for this condition, in the absence of a diagnosis of known genetic syndromes. METHOD: This is a descriptive observational study with data collection from the patients record at the Maternity Hospital. Literature reviews were carried out on platforms such as Lilacs, Scielo, Pubmed and research in Pediatric and Pediatric Surgery books on the subject. CONCLUSIONS: In the reported case, the diagnosis occurred after birth, with the newborn being referred early for surgical evaluation, which presented a successful approach, without complications. We hope that with the report, it will be possible to establish the family history as a point of the anamnesis to be valued in the investigation of the newborn.

Determining the factors that predict the feasibility of one-stage primary esophageal repair versus staged repair in neonates with type C esophageal atresia: Developing some new criteria

Background: Several factors can predict the feasibility of primary repair or a staged repair should be done from the start in patients with esophageal atresia with distal tracheoesophageal fistula (Type C TEF) to avoid unnecessary lengthy operations which may result in high morbidity and mortality in such condition.

Muscle-sparing posterolateral thoracotomy for esophageal atresia Vogt Type 3b

Background: Posterolateral muscle cutting thoracotomy is the gold standard approach to repair esophageal atresia with distal tracheoesophageal fistula. This technique is associated with morbidities in terms of poor motor and aesthetic outcomes. We aim to share our experience with muscle-sparing skin crease incision posterolateral thoracotomy for esophageal atresia Vogt type 3b.&#x0D; Methods: It was a retrospective observational study conducted over a period of 3 years and 6 months from January 2016 to June 2019 at two tertiary care teaching institutes. All patients with esophageal atresia having distal tracheoesophageal fistula were included.&#x0D; Results: Fifty-nine neonates underwent muscle-sparing thoracotomy, with 23 males and 36 females. The 34 (58%) neonates had low birth weight. Anorectal malformation (ARM) was the most common (6) associated major malformation(18). Intraoperative findings included long gap EA (6), right aortic arch (RAA, 3), aberrant vessels (1), and long upper pouch (1). Conversion to muscle cutting approach (during early learning curve) was performed in 8 cases i.e. long gap EA (3), RAA (2), Subglottic stenosis (2), others (1). No intraoperative complication was encountered; postoperative seroma formation (related to the approach) was observed in 2 (3.4%) neonates. Most of the patients achieved satisfactory functional and aesthetic outcomes.&#x0D; Conclusions: Muscle-sparing skin crease incision posterolateral thoracotomy is a viable approach for repair of esophageal atresia with distal tracheoesophageal fistula. The technique is easy to perform with adequate exposure and provides satisfactory functional and aesthetic outcomes with relatively minimum morbidity.

Long proximal pouch oesophageal atresia with tracheo-oesophageal fistula, a diagnostic dilemma: case report

BackgroundOesophageal atresia is a congenital abnormality commonly encountered in neonates. Classification proposed by Gross is the most frequently used. It divides the oesophageal anomalies into five types. Type C—proximal oesophageal atresia and distal tracheo-oesophageal fistula—is the most common. Dafoe and Ross described a rare subtype with a long proximal pouch. Only 11 cases of a long proximal pouch have been reported in the English literature so far. We describe a neonate with long proximal pouch oesophageal atresia with distal tracheo-oesophageal fistula emphasizing the difficulty in arriving at the diagnosis.Case presentationA 1-week-old neonate presented with feed intolerance and failure to advance orogastric tube into the stomach. Water soluble upper gastrointestinal tract contrast revealed a blind ending proximal oesophagus at the level of the diaphragm. Gastric volvulus was considered as a diagnosis. Patient underwent a laparotomy where a normal stomach was found. Bubbles were seen coming from the fashioned gastrostomy with each inspiration. This prompted us to consider the possibility of a missed oesophageal atresia with distal tracheo-oesophageal fistula. Diagnosis was confirmed on bronchoscopy and right thoracotomy. The tracheo-oesophageal fistula was ligated and a cervical oesophagostomy and Stamm gastrostomy were performed due to irreparable tear in the long upper pouch.ConclusionThis rare subtype of long upper pouch oesophageal atresia poses a diagnostic dilemma that can result in delay in the diagnosis and management. This diagnosis should be suspected if the tip of the orogastric/feeding tube is seen to be far below the level of the carina.