Erudition after neonatal gastric transposition for esophageal atresia at 10 years of follow-up

Background: Gastric transposition (GT) is a well-established procedure of esophageal re­placement (ER) for children with esophageal atresia. ER in the neonatal period is sparsely reported. We report characteristics of children who have undergone neonatal GT and have completed ten years of follow-up. Methods: The cross-sectional study was conducted which included all children who under­went neonatal GT at the institute and have completed a follow-up of at least 10-years. An­thropometry, oral contrast study, Hepatobiliary Scintigraphy, Gastroesophageal reflux study, Gastric emptying test, spirometry, and blood investigations were done for all the children. Results: Four children (three male and one female) were included in the study. The mean age at ER was 5.3 ±2.2 days with a mean birth-weight of 2.43 ±0.13 kg. Two children had prima­ry GT, while the other two had GT following a leak in primary anastomosis. During the mean follow-up of 180.25 ±43.5 months, none of the children required esophageal dilatation or other surgical intervention or procedures. All children were below 3rd centile for weight-for-age while all except one were below 50th centile for height-for-age. There was no stricture on oral contrast study, however, one child had grade III reflux on GER scan. Persistent duode­nogastric reflux on HIDA scan was seen in one child. Three children had restrictive parame­ters on spirometry. Symptomatically, all reported poor weight gain, one had left vocal cord palsy with hoarseness, and one had chest-wall protuberance. Conclusions: Neonatal gastric transposition is a feasible alternative to delayed esophageal replacement for neonates with esophageal atresia with limited complications.

Post-esophageal atresia repair double acquired tracheoesophageal fistulas treated successfully by gastric transposition: a case report

Background Postoperative recurrence of tracheoesophageal fistula (TEF) is a frequent complication in the repair of esophageal atresia (EA). Based on the recent etiologic classification, a TEF that develops in a different new pathway from the original one is categorized as an acquired TEF. The TEFs that fall into this category have been reported to be refractory and their mechanisms have not been fully understood. Here, we report the complicated case of an acquired TEF derived from mediastinitis after the original TEF repair developed an anastomotic stricture. The TEF contained double fistulas, both towards the right lobe bronchi, and was repaired by gastric transposition through a retrosternal route. Case presentation The patient was diagnosed with Gross C esophageal atresia after birth and underwent tracheoesophageal fistula banding during the neonatal period. He experienced an intractable anastomotic stenosis after surgery which was treated with repeated balloon dilation therapy. By the age of 11 months, he developed a mediastinal abscess that improved with conservative treatment. At 18 months old, a fistula from the esophagus to the right superior lobe bronchus was identified. The patient underwent a right upper lobectomy to resect the fistula. However, at 21 months old, another fistula to the right lower lobe was revealed. An esophageal banding was done to relieve the respiratory symptoms. This was followed by esophagectomy and gastric transposition through the retrosternal route at 26 months old. The patient started rehabilitation and oral intake gradually after surgery. By 3 years after gastric transposition, he could already take blended food orally with the support of small amounts of enteral feeding. Conclusion Cases of TEF derived from severe inflammation have the potential to form a complicated network and lead to recurrence. Surgeons should consider the possibility of multiple tiny fistulas in cases of severe acquired TEF. These may be repaired successfully by gastric transposition through the retrosternal route.

DOZ047.99: A unique case of ‘esophageal trachea’ requiring a multistep sequential surgical reconstruction

Introduction Communicating bronchopulmonary foregut malformations (CBPFMs) have been defined by Srikanth et al. in 1992 as a fistula between a portion of respiratory tissue and esophagus/stomach. Four types of CBPFMs have been described, none of those contemplating a complete communication between the distal trachea and esophagus. Case Report This study reports a case of a full-term neonate presenting with a VACTERL association and a tracheoesophageal malformation characterized by the presence of a long common duct including both the middle esophagus and distal trachea, which consists in esophageal tissue, without any cartilaginous rings 3–4 cm above the carina. A few days after birth, the esophagus and trachea have been surgically separated, keeping the common duct on the tracheal side and creating a residual long-gap esophageal atresia (LGEA). The resulting severe tracheomalacia has been simultaneously treated by posterior splinting using an autologous pericardium patch, as well as by anterior aortopexy. A terminal esophagostomy and a gastrostomy have been created to postpone esophageal reconstruction. Since the age of 18 months, delayed repair of LGEA has been performed by using a multistep strategy consisting of a combination of Kimura extrathoracic esophageal elongations and distal stump Foker external traction. Since no cartilaginous rings were present, refractory tracheomalacia has been further treated by a tracheoplasty through a combined median sternotomy and tracheoscopy approach at the age of 3 years by using a semitubular rigid Gore-Tex® prosthesis for an anterior external stenting. Today, 9 months after last surgery, complete weaning from respiratory support has been stably achieved, as well as full oral feeding. Conclusions This is the first description of an ‘esophageal trachea’, which may be considered as a new anatomic variant of CBPFMs. Multidisciplinary medical and surgical expertise was needed to manage this particular case, in order to properly plan multiple sequential surgical approaches. Native esophageal reconstruction should always be considered, even because colonic or gastric transposition might further affect severe tracheomalacia, resulting in more challenging treatment.

Diffuse Esophageal Leiomyomatosis in Pediatric Patients: A Systematic Review and Quality of Evidence Assessment

Background Diffuse esophageal leiomyomatosis (DEL) is a rare disorder characterized by benign hypertrophy of esophageal smooth muscle cells. No rigorous summary of available evidence on how to best manage these patients exists. Objective To define the clinical features and outcomes of pediatric patients with DEL. Materials and Methods A systematic literature search of the PubMed and Cochrane databases was performed with respect to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement (end-of-search date: October 6, 2018). The algorithm: “esophageal leiomyomatosis AND (children OR pediatric*)” was implemented. Results Thirty-five studies including a total of 58 patients were analyzed. The female:male ratio was 1.45:1. Mean patient age was 8.54 ± 4.67 years. The most common disease manifestations were dysphagia and gastrointestinal symptoms (90.0%, 95% confidence interval [CI]: 78.2–96.1), followed by failure to thrive (57.9%, 95% CI: 36.2–76.9) and pulmonary symptoms (56.4%, 95% CI: 41.0–70.7). Alport syndrome (AS) was seen in 57.7% (95% CI: 44.2–70.1) of the patients. The most commonly implemented procedure was esophagectomy (85.2%; n = 46/54; 95% CI: 73.1–92.6) with gastric transposition (37.8%; n = 17/45; 95% CI: 25.1–52.4). Postoperative complications developed in 33.3% (n = 15/45; 95% CI: 21.3–48) of the patients. All-cause mortality was 7.0% (95% CI: 2.3–17.2) and disease-specific mortality was 3.5% (95% CI: 0.3–12.6). Conclusion DEL is an uncommon condition that typically occurs in the setting of AS. Esophagectomy with gastric transposition is the mainstay of treatment. Although complications develop in one-third of the patients, mortality rates are low.