Oesophageal atresia and tracheo-oesophageal fistula

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.     

Successful management of late presentation neonatal oesophageal atresia with distal tracheo-oesophageal fistula and significant pulmonary complications

The successful management of a neonate presenting with oesophageal atresia and a distal tracheo-oesophageal fistula on the twelfth day of life is described. This case is the most delayed diagnosis associated with survival known to have been reported. There were significant pulmonary complications at presentation and initial treatment was by gastrostomy under local anaesthesia, followed by a delayed primary repair.     

Unilateral pulmonary agenesis associated with oesophageal atresia and tracheoesophageal fistula: A case report with prenatal diagnosis

We describe herein a case of unilateral pulmonary agenesis (PA) with oesophageal atresia (EA)/tracheoesophageal fistula (TEF) that was diagnosed prenatally and repaired by esophagoesophagostomy with stable postoperative course. The patient was born at 34 weeks gestation, after ultrasonography at 22 weeks gestation showed possible right-sided diaphragmatic eventration or PA and EA was subsequently suspected due to hydramnios. The initial X-ray showed mediastinal shift to the right, and coil up sign of the nasogastric tube, without intracardiac anomaly. Immediately after the diagnosis of EA/TEF and unilateral PA on day 0, the patient was intubated in the operating room, and a gastrostomy tube was placed. After pulmonary status stabilized, at 4 days old, EA/TEF was repaired through a thoracotomy in the right 4^th intercostal space. The right main bronchus was noted to continue into the distal oesophagus; this fistula was ligated and divided, and a single-layer esophagoesophagostomy was performed under mild tension with one vertebral gap. The neonate was maintained on mechanical ventilation and gradually weaned to extubation at 7 days old. The postoperative course was uneventful, with the exception of prolonged jaundice that emerged at 3 months old. Laparoscopic cholangiography at that time excluded biliary atresia, and jaundice resolved spontaneously. The patient has not shown any respiratory symptoms or feeding difficulties as of the 12-month follow-up.     

Long-gap oesophageal atresia

The various options for the management of long-gap oesophageal atresia are discussed. Of 89 infants treated, 27 had isolated atresia, 6 distal atresia with proximal fistula, and 56 atresia with distal fistula. The preferred approach for the former two groups was oesophageal replacement via gastric transposition. Recently, delayed primary anastomosis has been attempted after 6–12 weeks. For wide-gap atresia with distal fistula, primary anastomosis under marked or extreme tension with elective paralysis and mechanical ventilation for 5 days postoperatively achieved highly successful results in 39 infants. There were no major anastomotic disruptions and only 7 minor leaks. Strictures developed in 72% of cases and gastro-oesophageal reflux in 54%, 66% of whom required antireflux surgery. There was only 1 death in the 43 patients undergoing oesophageal replacement (none after gastric transposition, n = 34). A highly satisfactory outcome was achieved in 85%–90% of infants undergoing a replacement procedure.     

Clinically significant gastro-oesophageal reflux following oesophageal flap repair for oesophageal atresia and tracheo-oesophageal fistula

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.     

Esophageal atresia with proximal pouch tracheoesophageal fistula and noncommunicating esophageal duplication

This report describes a case of esophageal atresia with proximal-pouch tracheoesophageal fistula and a noncommunicating esophageal duplication. Until now this variation has not appeared in the literature.     

Right oesophageal bronchus associated with oesophageal atresia,tracheo-oesophageal fistula,tracheal stenosis,and left hypoplastic lung

We report a neonate with a rare communicating broncho-pulmonary foregut malformation associated with oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF). The malformation consisted of a right unilobed lung arising from a lower oesophageal bronchus, atresia of the right main tracheal bronchus with severe complete tracheal stenosis, hypoplastic left lung, high OA, and an unusual TOF. The malformation and association are both considered extremely rare, with only four similar cases previously reported. The anomaly was not correctable. Correspondence to: B. Currie     

Anterior flap repair of oesophageal atresia: a 16-year evaluation

The aim of the study was to evaluate the outcome of primary repair of oesophageal atresia (OA)/tracheo-oesophageal (TOF) using an anterior flap from the upper pouch to relieve tension on the anastomosis. Of 66 patients with OA/TOF managed in this institution in the period 1977–1993, only 1 (1.5%) had a colonic interposition. There were 15 (10 long-gap OA) primary repairs with an anterior flap. Median follow-up was 2 years (range 11 months – 16 years). Complications included anastomotic leaks in 4 (27%), anastomotic strictures in 13 (87%), of which 2 (13.3%) required resection, gastro-oesophageal reflux in 9 (60%), of which 3 (20%) required fundoplication, recurrent TOF in 2 (14%), and oesophageal incoordination in 6 (40%). Median hospital stay was 77 days (range 18–240 days). There were 3 late deaths (12, 12, 14 months). Of 12 survivors, 8 (66.7%) achieved normal growth. In addition, 3 patients had a myotomy as well as a flap (anastomotic leak 2, stricture 3). We conclude that the anterior flap technique for repair of OA/TOF reduces the need for oesophageal replacement. There is considerable morbidity associated with its use in these high-risk patients, but a satisfactory outcome can be achieved in long-term survivors.     

H-type tracheo-oesophageal fistula with congenital oesophageal stenosis

H-type tracheo-oesophageal fistula (H-TOF) and congenital oesophageal stenosis (COS) are both rare anomalies. Only six cases of H-TOF in association with COS have been described in the literature. Characteristically, diagnosis is delayed, often well beyond the neonatal period. We report a patient who was accurately diagnosed on day 12 of life, and outline several of the key clinical dilemmas encountered in managing a neonate with this combination of rare congenital anomalies.     

胸腔镜与开胸手术治疗先天性食管闭锁并食管气管瘘的对比研究

目的 比较胸腔镜手术与开胸手术治疗先天性食管闭锁并食管气管瘘的疗效差异.方法 回顾性分析50例Ⅲ型先天性食管闭锁并食管气管瘘的临床资料,其中25例为江西省儿童医院2012年12月至2014年1月收治,采用胸腔镜治疗;另25例为首都儿科研究所2008年6月至2014年4月收治,采用开胸治疗.男27例,女23例,术前均明确诊断并完成手术治疗.胸腔镜组采用3孔法,经胸行食管气管瘘结扎,食管吻合术.开胸组采用经胸膜外完成手术.结果 胸腔镜组25例均一期完成食管吻合术,无中转,术后2例死亡.开胸组25例中23例完成经胸膜外食管吻合术,2例因食管盲端距离过长行食管气管瘘结扎,胃造瘘术,术后放弃治疗.手术时间胸腔镜组平均为127min(110～160min),开放组平均133min(105～170min);呼吸机使用时间胸腔镜组平均为2.6 d(1～13 d),开放组平均2.3 d(1～11 d),两组对比差异均无统计学意义.胸腔镜组25例中2例吻合口漏(8％),7例狭窄(28％),2例食管气食管瘘复发(8％).开放组25例中2例吻合口漏(8％),4例狭窄(16％),无食管气管瘘复发,两组比较差异均无统计学意义.结论 胸腔镜手术治疗先天性食管闭锁并食管气管瘘是安全可行的,但要求术者具备熟练的微创手术技术和相关的手术技巧.     

Extrapleural thoracoscopic repair of esophageal atresia with tracheoesophageal fistula

Traditionally, open repair of esophageal atresia (EA) with tracheoesophageal fistula (TEF) required thoracotomy. Innovations in minimal access surgery have created a thoracoscopic technique resulting in violation of the pleural space. Most pediatric surgeons favor an extrapleural approach for open repair. We present a novel minimal access, extrapleural technique for repairing EA with TEF. A 2-day-old infant with EA and distal TEF underwent thoracoscopic extrapleural repair that utilized three ports. Initial creation of the extrapleural space was achieved through one of the port sites and was completed thoracoscopically. A thoracoscopic repair of EA with distal TEF was achieved within the extrapleural space. A small tear in the pleura was inadvertently created during the dissection. The child began feeding normally. At 1 year of age, the patient had dysphagia requiring a single esophageal dilatation. This is the first known report of an extrapleural thoracoscopic repair of EA with TEF. Although thoracoscopic repairs of EA/TEF have been previously reported, these were all done transpleurally. Many pediatric surgeons favor the extrapleural approach for two reasons: (1) containment of a potential leak within the extrapleural space, avoiding an empyema, and (2) easier transpleural access for future thoracic procedures.     

Proposal of a novel method to evaluate anastomotic tension in esophageal atresia with a distal tracheoesophageal fistula

Anastomotic tension with the potential to lead to post-operative complication is usually evaluated using gap length before anastomosis in patients with esophageal atresia and a distal tracheoesophageal fistula (EA with a TEF). However a uniform, accurate measurement of gap length is not possible and estimation of the length the delicate distal esophageal stump is stretched by the anastomosis may have greater utility. The aim of this paper was to propose a novel method to evaluate the anastomotic tension in EA with a TEF. Forty consecutive patients having EA with a TEF were studied. Primary anastomosis without gastrostomy was performed in all cases. When the TEF was cut off, the most proximal site of the tracheal side was marked using a tiny metallic clip. When anastomosis was completed, the distance from the clip to the anastomotic site was measured as the stretched length. On the esophagram taken subsequently, the same distance was measured, together with the distance from the clip to the esophago-cardiac junction as the original distal esophageal length. The stretching ratio was calculated by dividing the former by the latter. The stretched length on esophagram (median: 3.0 mm, range: −12 to 21) was significantly correlated with that measured during surgery (median: 2.3 mm, range; −14 to 15) (r=0.96, P<0.0001). The median of original distal esophageal lengths was 60.0 mm (range: 35–80). The stretching ratio was significantly correlated with the stretched length, and the number of the stretching ratio as a percentage corresponded to about double the number of the stretched length on esophagram in millimeters (y=1.91x+0.58, r=0.98, P<0.0001). Anastomotic leakage and recurrence of TEF were not experienced. In patients complicated with gastroesophageal reflux (GER), the site of TEF was significantly more distal as compared with the other cases [median (range): 5.0th (4.0–6.0) vs 3.5th (1.5–5.0) thoracic vertebral level, P<0.009]. The stretched length and the stretching ratio were also longer and larger, respectively [median (range): 10.0 mm (6–21) vs 2.0 (−12 to 14) mm, P<0.008, 17.3% (12.7–47.7) vs 2.9% (−16.4 to 29.8)%, P<0.018). Similar tendencies were observed for patients complicated with stricture. Estimation of the stretched length of the distal esophageal stump is useful to evaluate the anastomotic tension. If the stretched length is more than 10 mm, it will be necessary to consider the possibility that stricture or GER may arise afterwards.     

Gastric perforation in infants with oesophageal atresia and distal tracheo-oesophageal fistula

Gastric perforation (GP) is a well-recognised complication of oesophageal atresia (OA) with distal tracheo-oesophageal fistula (TOF), and is usually associated with extreme prematurity, hyaline membrane disease, and the requirement for assisted ventilation. The presentation is sudden, and leads to further deterioration in respiratory function because of increasing abdominal distension from pneumoperitoneum and splinting of the diaphragm. Unrelieved, the infant becomes increasingly hypoxic and may die. A review of six infants with OA and distal TOF in whom GP occurred has enabled us to develop the following guidelines for the appropriate initial surgical management of this complication: (1) Needle paracentesis of the abdomen en route to surgery if the infant continues to deteriorate; (2) Urgent laparotomy to decompress the abdomen and to occlude the lower oesophagus with a catheter introduced through the GP; (3) Thoracotomy and division of the fistula; (4) Oesophageal anastomosis if the infant's condition improves sufficiently and the anatomy is favourable; and (5) Repair of the GP and formation of a gastrostomy.     

Health‐related quality of life and its determinants in children and adolescents born with oesophageal atresia

Aim: Following surgical correction in the neonatal period, patients born with oesophageal atresia have significant co‐morbidity, particularly in childhood. This study evaluates health‐related quality of life and its determinants such as concomitant anomalies and the presence of respiratory and/or gastro‐intestinal symptoms 6–18 years after repair of oesophageal atresia. Methods: Parents of 24 patients with oesophageal atresia completed the child health questionnaire for parents and 37 patients completed the child form. Gastro‐intestinal symptoms were assessed by a validated standardized reflux questionnaire. Results were compared with a healthy reference population. Results: Parents as well as patients themselves scored significantly lower on the domain general health perception. According to parents, general health perception was negatively affected by age at follow‐up and concomitant anomalies. Patients reported that reflux symptoms reduced general health perception. Conclusion: In this first study describing health‐related quality of life in children and adolescents born with oesophageal atresia, we demonstrated that general health remains impaired because of a high incidence of concomitant anomalies and gastrointestinal symptoms in patients with oesophageal atresia when compared with the healthy reference population.