Acute gastric volvulus secondary to H-type tracheo-oesophageal fistula.

H-type tracheo-oesophageal fistula is an uncommon type of tracheo-oesophageal malformation. Acute gastric volvulus is another infrequent pathology in children. They rarely present together.We report the case of a toddler with acute gastric volvulus possibly secondary to an undiagnosed H-type tracheo-oesophageal fistula. The fistula was suspected due to persistent gastric distention observed during volvulus detorsion. This kind of tracheo-oesophageal fistula often presents with subtle symptoms making early diagnosis difficult.Acute gastric volvulus is a life-threatening condition. Gastric distension caused by the passage of air into the stomach through the fistula could be a triggering factor for gastric volvulus.

Thoracoscopic primary repair is useful for esophageal atresia with tracheoesophageal fistula in neonates with low body weight.

PURPOSE: The surgical indication of thoracoscopic primary repair for esophageal atresia with tracheoesophageal fistula is under debate. The current study aimed to investigate the outcome of thoracoscopic primary repair for esophageal atresia with tracheoesophageal fistula in patients weighing < 2000 g and those who underwent emergency surgery at the age of 0 day. METHODS: The surgical outcomes were compared between patients weighing < 2000 g and those weighing > 2000 g at surgery and between patients who underwent surgery at the age of 0 day and those who underwent surgery at age ≥ 1 day. RESULTS: In total, 43 patients underwent thoracoscopic primary repair for esophageal atresia with tracheoesophageal fistula. The surgical outcomes according to body weight were similar. Patients who underwent surgery at the age of 0 day were more likely to develop anastomotic leakage than those who underwent surgery at the age of ≥ 1 day (2 vs. 0 case, p = 0.02). Anastomotic leakage was treated with conservative therapy. CONCLUSION: Thoracoscopic primary repair is safe and useful for esophageal atresia with tracheoesophageal fistula even in newborns weighing < 2000 g. However, emergency surgery at the age of 0 day should be cautiously performed due to the risk of anastomotic leakage.

Rare type of tracheal agenesis: Unexpected presentation and immediate consideration of emergent esophageal intubation in neonatal resuscitation program. Case reports and review of the literature.

BACKGROUND: Tracheal agenesis, or tracheal atresia, is a rare congenital anomaly. The presence of a tracheoesophageal fistula (TEF) can help with breathing for newborns with tracheal agenesis. In this article, we presented three unique cases and outcomes of neonates with tracheal agenesis along with a review of the literature. METHODS: This study consisted of a single center case series followed by a review of literature. Case reports were generated using both written and electronic medical records from a single hospital. We summarized three unique cases and outcomes of neonates with tracheal agenesis and performed a review of the literature. RESULTS: We identified three cases of tracheal agenesis presented with severe cyanosis without spontaneous crying upon birth. Experienced pediatricians attempted to intubate the babies but were unsuccessful. Endotracheal tubes were subsequently either accidentally or purposely placed into the esophagus, and oxygen saturation levels improved. This suggested tracheal agenesis with TEF. Two cases underwent surgical intervention after resuscitation with esophageal intubation. CONCLUSION: Esophageal intubation may be a life-sustaining ventilation support for patients with tracheal agenesis and TEF at initial resuscitation. Clinicians should suspect tracheal agenesis when a newborn presents with severe cyanosis and voiceless crying upon birth, and esophageal intubation should be immediately attempted.

Analysis of gap length as a predictor of surgical outcomes in esophageal atresia with distal fistula: a single center experience.

PURPOSE: Long-gap esophageal atresia (LGEA) is still a challenge for pediatric surgery. No consensus exists as to what constitutes a long gap, and few studies have investigated the maximum gap length safely repairable by primary anastomosis. Based on surgical outcomes at a single institution, we aimed to determine the gap length in LGEA with a high risk of complications. METHODS: The medical records of 51, consecutive patients with esophageal atresia (EA) with primary repair in the early neonatal period between 2001 and 2021 were retrospectively reviewed. Three, major complications were found in the surgical outcomes: (1) anastomotic leakage, (2) esophageal stricture requiring dilatation, and (3) GERD requiring fundoplication. The predictive power of the postsurgical complications was assessed using receiver operating characteristic analysis, and the area under the curve (AUC) and the cutoff value with a specificity of > 90% were calculated. RESULTS: Sixteen patients (31.4%) experienced a complication. The AUC of gap length was0.90 (p < 0.001), and the gap length cutoff value was ≥ 2.0 cm for predicting any complication (sensitivity: 62.5%, specificity: 91.4%). CONCLUSION: A gap length ≥ 2.0 cm was considered as defining LGEA and was associated with an extremely high complication rate after primary repair.

Long-term Airway Outcomes and Interventions in Children With Oesophageal Atresia With Tracheoesophageal Fistula: A 20-year Single Centre Observational Study.

BACKGROUND: Airway anomalies, symptoms and interventions are commonly reported in children with oesophageal atresia with tracheoesophageal fistula (OA/TOF). The purpose of this study was to assess the incidence of these airway pathologies and those requiring interventions in the long-term. METHODS: A retrospective case note review of all patients admitted to the Neonatal Unit at the Royal Hospital for Children, Glasgow between January 2000 and December 2015 diagnosed with OA/TOF. Included patients had a minimum of 5 years follow-up. RESULTS: 121 patients were identified. 118 proceeded to OA/TOF repair. 115 patients had long-term follow-up data. Ninety-five (83%) children had one or more airway symptom recorded. Thirty-six (31%) neonates underwent airway endoscopy at the time of their initial OA/TOF repair. Forty-six (40%) children underwent airway endoscopy at a later date due to airway symptoms. Airway pathologies identified included airway malacia, thirty-two (28%), subglottic stenosis, eleven (10%), tracheal pouch, twenty-five (22%), laryngeal cleft, seven (6%) and recurrent fistula, five (4%). Airway interventions included endoscopic division of tracheal pouch, ten (9%), tracheostomy, seven (6%), aortopexy, six (5%), repair of recurrent fistula, five (4%), endoscopic repair of laryngeal cleft, three (3%) and four (3%) required open airway reconstruction for subglottic stenosis. One child (1%) remains tracheostomy dependent. CONCLUSIONS: Long-term airway pathologies are common in children with OA/TOF. Many of these are remediable with surgical intervention. Clinicians should be cognisant of this and refer to Airway Services appropriately.

Local injection of platelet-rich plasma offers a new therapeutic option for the treatment of esophagotracheal fistula: a case report.

Esophagotracheal fistula (ETF), one of the most serious complications in the treatment of esophageal cancer, presents a complex management challenge. Early diagnosis and treatment are crucial to alleviate clinical symptoms and improve the quality of life of patients with ETF. The most commonly used method for treating ETF is esophageal stenting. However, because of the variable location and size of the fistula, stent placement alone sometimes fails to completely close the fistula, and complications such as fracture and displacement of the esophageal stent may occur. Therefore, safer and more effective methods for the treatment of ETF are required. In recent years, the application of bioactive factors to promote human tissue repair and wound healing has increased and achieved good therapeutic results. We herein describe a case in which we performed endoscopic injection of platelet-rich plasma directly into the ETF site and achieved a favorable outcome. This case suggests that local injection of platelet-rich plasma is a novel treatment modality for ETF.

Endoscopic Chemocauterization with Trichloroacetic Acid for Congenital or Recurrent Tracheoesophageal Fistula in Children with Esophageal Atresia: Experience from a Tertiary Center.

BACKGROUND: Recurrent tracheoesophageal fistula (rTEF) is a well-known complication after surgery of EA, occurring in roughly 3-10% of the patients. Recent studies have highlighted safety and efficacy of endoscopic management of recurrent TEF. The aim of this study was to evaluate the efficacy of chemocauterization with trichloroacetic acid (TCA) in rTEF and congenital tracheoesophageal fistula (cTEF). METHODS: Retrospective chart review of consecutive patients with recurrent or congenital TEF who underwent endoscopic chemo-cauterization between 2018 and 2022 at a tertiary center. Children diagnosed with TEF who underwent primary or secondary endoscopic treatment were included. Median follow up time was 19 months for rTEF and 33 months for cTEF. RESULTS: During the study period, 18 patients were treated endoscopically by chemocatuerization with TCA at our institution. Treatment of recurrent TEF was successful in 13 of 14 patients (93%) Treatment of congenital TEF was successful in 2 of 4 patients (50%). In 14 patients, closure was seen after 1-2 treatments. There were no serious adverse reactions or complications to the endoscopic treatment of TEF. CONCLUSION: Endoscopic chemocauterization is a minimal invasive technique with low morbidity and high success rate and may be considered as primary treatment for recurrent TEF. LEVEL OF EVIDENCE: III.

A case of antenatally undiagnosed laryngeal atresia with tracheo-esophageal fistula with airway obstruction at birth and its management.

Laryngeal atresia is a rare congenital anomaly that is usually diagnosed by antenatal ultrasound, however, if undiagnosed presents with desaturation after birth. A term neonate presented with airway obstruction after birth with multiple failed attempts at intubation and was rescued by proseal laryngeal mask airway (LMA). An esophagoscopy using an Ambuscope utilizing a modified connector assembly revealed an opening on the anterior wall of the esophagus with no esophageal atresia, leading to a diagnosis of H-type tracheo-esophageal fistula (TEF) with laryngeal atresia. The ability to ventilate the neonate via LMA with an absent glottic opening raised the possibility of TEF.

What Proportion of Children With Complex Oesophageal Atresia Require Oesophageal Lengthening Procedures?

INTRODUCTION: Babies with oesophageal atresia (OA) who cannot achieve a primary anastomosis (complex OA) may be treated by attempted oesophageal lengthening. We contrast reported outcomes of lengthening with our experience of managing complex OA. PATIENTS AND METHODS: A consecutive series treated in an English regional centre was completed. Outcomes of interest were the rate of retention of the native oesophagus, complications requiring thoracotomy, rates of anastomotic leak, stricture, fundoplication, and mortality. Possible explanatory variables were the surgical techniques applied. RESULTS: 29/215 (13%) OA were complex, and 25/207 survived to repair. 14/25 (56%) had no distal fistula, pure OA, while 11/25 (44%) had a long gap with distal fistula. 18/25 (72%) had delayed primary anastomosis, while 7/25 (28%) required oesophageal replacement. However, 2 of the replacements were salvage procedures following failed traction. Only 4/207 (2%) of OA were potentially treatable by traction. Salvage surgery was required in 2/23 (9%) complex OA not subjected to lengthening. The native oesophagus was retained without utilising lengthening in 200/207 (97%). Amongst complex OA where traction techniques had not been attempted, the native oesophagus was retained in 18/23 (78%) of cases, with median time to oesophageal continuity of 77 days. There was no in hospital mortality following treatment of complex OA, and overall survival was identical to non-complex OA among cases surviving to anastomosis. DISCUSSION: Management of complex OA without lengthening procedures leads a similar rate of retention of the native oesophagus as reports describing lengthening, but with significantly less morbidity. We see little need for oesophageal lengthening in the management of complex OA. LEVEL OF EVIDENCE: IV.

Primary Posterior Tracheopexy at Time of Esophageal Atresia Repair Significantly Reduces Respiratory Morbidity.

PURPOSE: Esophageal atresia with tracheoesophageal fistula (EA/TEF) is often associated with tracheobronchomalacia (TBM), which contributes to respiratory morbidity. Posterior tracheopexy (PT) is an established technique to treat TBM that develops after EA/TEF repair. This study evaluates the impact of primary PT at the time of initial EA/TEF repair. METHODS: Review of all newborn primary EA/TEF repairs (2016-2021) at two institutions. Long-gap EA and reoperative cases were excluded. Based on surgeon preference and preoperative bronchoscopy, neonates underwent primary PT (EA + PT Group) or not (EA Group). Perioperative, respiratory and nutritional outcomes within the first year of life were evaluated. RESULTS: Among 63 neonates, 21 (33%) underwent PT during EA/TEF repair. Groups were similar in terms of demographics, approach, and complications. Neonates in the EA + PT Group were significantly less likely to have respiratory infections requiring hospitalization within the first year of life (0% vs 26%, p = 0.01) or blue spells (0% vs 19%, p = 0.04). Also, they demonstrated improved weight-for-age z scores at 12 months of age (0.24 vs -1.02, p < 0.001). Of the infants who did not undergo primary PT, 10 (24%) developed severe TBM symptoms and underwent tracheopexy during the first year of life, whereas no infant in the EA + PT Group needed additional airway surgery (p = 0.01). CONCLUSION: Incorporation of posterior tracheopexy during newborn EA/TEF repair is associated with significantly reduced respiratory morbidity within the first year of life. LEVEL OF EVIDENCE: Level III.

Comparison of robotic versus thoracoscopic repair for congenital esophageal atresia: a propensity score matching analysis.

BACKGROUND: Despite the rapid advancement of robotic surgery across various surgical domains, only cases of robotic repair (RR) for neonates with esophageal atresia (EA) have been reported. Comprehensive studies comparing RR and thoracoscopic repair (TR) are lacking. The authors aimed to compare the safety and efficacy of RR and TR for EA. METHODS: A retrospective multicenter study was conducted on 155 EA neonates undergoing RR (79 patients) or TR (76 patients) between August 2020 and February 2023 using propensity score matching. Asymmetric port distribution and step-trocar insertion techniques were applied during RR. Demographics and surgical outcomes were compared. RESULTS: After matching, 63 patients (out of 79) in RR group and 63 patients (out of 76) in TR group were included. There were no significant differences in short-term outcomes between two groups, except for longer total operative time (173.81 vs. 160.54 min; P <0.001) and shorter anastomotic time (29.52 vs. 40.21 min; P <0.001) in RR group. Compared with TR group, the RR group had older age at surgery (8.00 vs. 3.00 days; P <0.001), but a comparable pneumonia rate. More importantly, the incidence of anastomotic leakage (4.76 vs. 19.05%, P =0.013), anastomotic stricture (15.87 vs. 31.74%, P =0.036) within 1 year postoperatively, and unplanned readmission (32.26 vs. 60.00%, P =0.030) within 2 years postoperatively were lower in RR group than in TR group. CONCLUSIONS: RR is a technically safe and effective option for EA patients. This approach delays the age of surgery without increasing respiratory complication rates while reducing the incidence of postoperative anastomotic complications and unplanned readmission.

Esophageal atresia with tracheoesophageal fistula: two case reports.

BACKGROUND: The incidence of esophageal atresia with tracheoesophageal fistula is 1 out of 3000-5000 live births. Its incidence in lower middle income countries is not known. The infants usually present with excessive secretions or choking while feeding and are at risk for aspiration. The outcome of these infants in lower middle income countries is not encouraging due to delays in referral, sepsis at presentation requiring preoperative stabilization, postoperative complications such as anastomosis leaks, pneumonia, and pneumothorax. CASE PRESENTATION: We present two African babies who were term infants at age 2 days (male) and 5 days (female) with diagnosis of esophageal atresia and tracheoesophageal fistula. The 5-day-old infant required preoperative stabilization due to sepsis and delayed surgery with a poor postoperative outcome. The 2-day-old infant was preoperatively stable and had a good postoperative outcome. The challenges faced in management of these two cases have been highlighted. CONCLUSION: Outcome of infants with esophageal atresia and tracheoesophageal fistula in lower middle income countries is not encouraging due to delays in referral and poor postoperative healing attributed to sepsis and recurrent pneumothorax. Timely referral, preoperative condition of the infant, and timely management has shown to be a contributory factor for an improved outcome.

Mortality and neurodevelopmental outcomes in very low birth weight infants with esophageal atresia.

PURPOSE: Very low birth weight infants (VLBWIs) have been thought as risk of bad outcomes in the patients with esophageal atresia (EA). However, detailed outcomes of EA within VLBWIs were not fully understood. We aimed to reveal short- and long-term outcomes in VLBWIs with EA. METHODS: Clinical data regarding VLBWIs with EA registered in Neonatal Research Network Japan, a multicenter research database in Japan, were collected. Patients with chromosomal abnormality were excluded. Short term outcome was survival discharge from NICU and long-term outcome was neurodevelopmental impairment (NDI) at 3 years. RESULTS: A total of 103 patients were analyzed. the overall survival discharge rate from NICU was 68.0% (70/103). The risk of death was increased as the birth weight got reduced. The presence of associated anomaly increased the risk of death. Three-year neurodevelopmental information was available in 32.9% (23/70) of patients. Of the 23 included patients for 3-year follow-up, 34.8% had NDI. The risk of NDI was increased as the birth weight reduced. CONCLUSIONS: In VLBWIs with EA, survival discharge from NICU was still not high. More immature patients and patients with an associated anomaly had worse outcomes. Among patients who survived, NDI was confirmed in a certain number of patients.

Challenging management of an enlarged tracheoesophageal fistula in an irradiated patient.

Speech restoration after total laryngectomy may be achieved in different ways, the gold standard being tracheoesophageal puncture (TPE) with the positioning of a speech prosthesis. TPE is not immune to complications, the most common of which being leakage through or around the prosthesis. When dealing with an enlarged tracheoesophageal fistula, the management can be either conservative or surgical. In the following case report, we present a particularly challenging case, in which every conservative strategies failed and a major surgery was required to close the fistula.

Azygos vein preservation revisited: impact on early outcomes after repair of esophageal atresia/tracheoesophageal fistula in newborns.

Since the first successful repair of esophageal atresia/tracheoesophageal fistula (EA-TEF) was performed approximately 8 decades ago, surgeons have made considerable technical advances in solving intraoperative surgical challenges and reducing postoperative complications. According to some surgeons, preserving the Azygos vein makes this modification attractive. This study aimed to evaluate the benefits of preserving the Azygos vein during surgery for esophageal atresia with tracheoesophageal fistula and to highlight its advantages in reducing anastomotic leak, stricture, and other postoperative outcomes. This prospective comparative series was conducted between April 2020 and April 2023. The study included all newborns with EA-TEF eligible for primary repair. Patients were randomized to either Group A or B. Group A underwent Azygos vein preservation, whereas the remaining patients (Group B) underwent Azygos vein disconnection. Sixty-four patients were included in this study. Thirty-two patients (Group A) underwent Azygos vein preservation during EA-TEF repair, and the remaining thirty-two patients (Group B) underwent Azygos vein ligation and disconnection. Both groups were comparable in terms of demographics, clinical data, and operative findings (P > 0.05). Pneumonitis occurred in 4 patients in Group A and 16 patients in Group B. Anastomotic leaks occurred in two (6.2%) patients in Group A and six (18.7%) patients in Group B. There were two deaths in Group A and six deaths in Group B, with a significant difference between the two groups (P = 0.0485). Preserving the Azygos vein during esophageal atresia repair reduces the occurrence of postoperative pneumonia, leakage, and stenosis, and decreases postoperative mortality. Therefore, we declare that this modification is a significant and valuable addition to the current surgical procedures.

Diagnosis and management of congenital type D esophageal atresia.

This study was performed to describe the current clinical practice and outcomes of type D esophageal atresia. We retrospectively analyzed 10 patients who were diagnosed with type D esophageal atresia and underwent esophageal atresia and tracheoesophageal fistula repair in the Capital Institute of Pediatrics and Beijing Children's Hospital from January 2017 to May 2022. Ten patients include three newborns and seven non-newborns. Seven (70%) cases were misdiagnosed as type C esophageal atresia before the first operation. Three neonatal children underwent thoracoscopic distal tracheoesophageal fistula ligation and esophageal anastomosis: the proximal tracheoesophageal fistula was simultaneously repaired with thoracoscopy in one of these children, and the proximal tracheoesophageal fistula was not detected under thoracoscopy in the other two children. Among the seven non-neonatal children, one underwent repair of the proximal tracheoesophageal fistula through the chest and the other six underwent repair through the neck. Nine patients were cured, and one died of complications of severe congenital heart disease. Type D esophageal atresia lacks specific clinical manifestations. Misdiagnosis as type C esophageal atresia is the main cause of an unplanned reoperation. Patients without severe malformations have a good prognosis.

Analysis of mortality and long-term outcomes of pediatric patients with tracheoesophageal fistula/esophageal atresia.

BACKGROUND/PURPOSE: This study analyzed factors that affect mortality in patients surgically treated for EA, then explored the long-term problems encountered by these patients. MATERIALS AND METHODS: Among 252 patients with EA who were either diagnosed, treated, and subsequently followed at the Department of Pediatric Surgery, Dicle University Medical Faculty Hospital, or were treated surgically elsewhere but followed at our center, between January 2010 and January 2020,214 patients were included in the study. RESULTS: Early complications:One or more early complications were observed in 132(62.5%) of the 211 patients who underwent surgical EA repair. The most common early complication was AS, which developed in 106(50.2%) patients. Anastomosis leakage. Was detected in 48 (22.7%) patients. Late complications: The late findings of 161 patients who underwent definitive surgery and had a mean follow-up period of 68 months (range, 6-120 months) were evaluated. The most common late complication was GER. MORTALITY: Of the 214 patients in our study, 63(29.4%) died, including 48(76.2%) during the early period after the first surgery. Of the 10(15.9%) patients who died during the late period. CONCLUSION: In our study, the most common early complication was anastomotic stenosis in patients with esophageal atresia. We recommend balloon dilation in the treatment of anastomotic stenosis.Gastroesophageal reflux is the most common long-term complication. Most of these can be treated conservatively and medically. The association of low birth weight, cardiac anomalies, prematurity and VACTERL increased mortality in patients with esophageal atresia in our study. KEY WORDS: Esophageal Atresia, Morbidity, Late Period, Mortality, Tracheoesophageal Fistula.

Morbidity and mortality among neonates with esophageal atresia and/or tracheoesophageal fistula in the United States.

BACKGROUND: Although rare, esophageal atresia (EA) and/or tracheoesophageal fistula (TEF) can lead to severe morbidity and mortality. A known complication of EA and/or TEF repair is vocal fold motion impairment (VFMI). OBJECTIVE: To characterize the prevalence of VFMI among repaired EA and/or TEF at a national level and to identify factors associated with in-patient mortality and common clinical endpoints in this population (tracheostomy and gastrostomy tube dependence). METHODS: This is a retrospective cohort study of inpatient neonate admissions with EA and/or TEF in the 2016 Kids' Inpatient Database (KID). Patients with a history of EA and/or TEF were identified with ICD-10 codes. VFMI prevalence was assessed by observed frequencies and percentages. Clinical endpoints included gastrostomy (G-)tube placement, tracheostomy status, and inpatient mortality. Potential predictors of these three primary outcomes included cardiac anomalies, airway disorders, chromosomal anomalies, surgery within 24 h of admission, prematurity, and low birthweight (LBW). Chi-squared analysis was performed between each parameter and the primary outcomes. Significant associations (p-value<0.05) were further assessed with multivariate logistic regression. RESULTS: Of 1062 neonates with history of EA and/or TEF, 24 (2.3%) had VFMI. Among this population, 27 patients underwent tracheostomies and 63 required G-tubes. There was a significantly higher likelihood of tracheostomy in patients with VFMI compared to patients without (16.7% versus 2.2%; p < 0.01). After multivariate analysis, birthweight less than 1.5 kg (BW < 1.5 kg; OR = 2.5, 95% CI: 1.03-6.10), extreme prematurity (OR = 7.5, 95% CI: 2.00-28.07), cardiac anomalies (OR = 1.8, 95% CI: 1.01-3.04) and chromosomal anomalies (OR = 4.2, 95% CI: 2.39-7.44) were significantly associated with in-hospital mortality. Bronchopulmonary dysplasia (BPD; OR = 4.7, 95% CI: 1.73-12.78) and BW < 1.5 kg (OR = 2.4, 95% CI: 1.05-5.49) were significant predictors for G-tube status. For tracheostomy, BPD (OR = 15.8, 95% CI: 4.70-52.83) and subglottic stenosis (SS, OR = 11.2, 95% CI: 1.85-68.02) remained significant predictors after multivariate regression. CONCLUSION: The national prevalence of VFMI among neonates following EA and/or TEF repair was 2.3% and was associated with an increased likelihood of tracheostomy in the KID 2016. Significant predictors of in-hospital mortality were cardiac anomalies, chromosomal anomalies, extreme prematurity, and BW < 1.5 kg. BPD and SS were significant predictors of tracheostomy, and BPD and BW < 1.5 kg were significant predictors for G-tube requirements.

Type III congenital pulmonary airway malformation associated with oesophageal atresia and tracheoesophageal fistula. A case report and review of literature.

Congenital pulmonary airway malformation (CPAM) together with oesophageal atresia and tracheoesophageal fistula (TOF) is a very rare condition in neonates. We presented a case of an infant with Gross type C oesophageal atresia with TOF coexisting with Stocker Type III CPAM in our centre. It is interesting to know that TOF associated with type III CPAM has never been reported in the literature. The child was delivered through caesarean section, and because of respiratory distress post-delivery, endotracheal intubation was carried out immediately. CPAM was diagnosed by a suspicious finding from the initial chest X-ray and the diagnosis was confirmed through computed tomography scan of the chest. The patient was initially stabilised in a neonatal intensive care unit (NICU), and after the successful ligation of fistula and surgical repair of TOF, lung recruitment was started by high flow oscillatory ventilation. The patient recovered well without complications and able to maintain good saturation without oxygen support through the stay in the neonatal unit. Early recognition of this rare association is essential for immediate transfer to NICU, the intervention of any early life-threatening complications, and for vigilant monitoring in the postoperative period.