Long-term outcomes of oesophageal atresia without or with proximal tracheooesophageal fistula - Gross types A and B.

PURPOSE: Because of an extended gap between esophageal pouches a variety of methods are employed to treat oesophageal atresia (OA) without (type A) or with (type B) proximal tracheooesophageal fistula. This retrospective observational study describes their single centre long-term outcomes from 1947 to 2014. METHODS: Of 693 patients treated for OA 68 (9.7%) had type A (n=58, 8.3%) or B (n=10, 1.4%). Hospital records were reviewed. Main outcome measures were survival and oral intake. RESULTS: Nine (13%) patients had early and 10 (15%) delayed primary anastomosis, 30 (44%) underwent reconstruction including colonic interposition (n=13), reversed gastric tube (n=11) and jejunum interposition (n=6), whereas19 (28%) had died without a definite repair. Median follow up was 35 (interquartile range, 7.4-40) years. Thirty-one (63%) of 49 patients with definitive repair survived long term. Survival was 22% for early and 80% for delayed primary anastomosis, 57% for colon interposition, 82% for gastric tube and 84% for jejunum interposition. Gastrooesophageal reflux was most common after gastric tube (80%), dysphagia after colon interposition (50%), and 3 (60%) of 5 survivors with jejunum interposition had permanent feeding ostomy because of neurological disorder. Endoscopic follow-up disclosed no oesophageal cancer or dysplasia. Repair in the most recent patients from 1985 to 2014 (n=14) included delayed primary anastomosis (n=7), jejunum interposition (n=6) and gastric tube (n=1) with 93% long-term survival. CONCLUSION: Morbidity among long-term survivors of type A or B OA is high. With modern management survival is, however, excellent and patients without neurological disorder achieve full oral intake either after primary anastomosis or reconstruction. LEVELS OF EVIDENCE: IV.

Endoscopic Surveillance After Repair of Oesophageal Atresia: Longitudinal Study in 209 Patients.

AIM: After repair of oesophageal atresia (OA), the need for endoscopic follow-up (EFU) remains unclear. To end this, we assessed the trends of oesophageal mucosal changes in successive follow-up biopsies. METHODS: EFU records of 264 patients including histological grades of oesophagitis (from 0 to III), gastric (GM) or intestinal (IM) metaplasia and dysplasia (mild to severe) at 1, 3, 5 10, 15, and >15 years after repair of OA were reviewed. RESULTS: Included were 209 patients with 616 biopsies. A total of 60 patients had undergone antireflux surgery and 24 had long-gap OA (LG). Median follow-up was 12 (range 1-17) years with 3 (1-6) endoscopies per patient. Highest grade of oesophagitis was Gr 0 (no oesophagitis) in 47%, Gr I in 37%, and Gr II or III in 16%. Metaplasia, GM (n = 31), IM (n = 4), occurred in 17% of patients and reached 15% prevalence by 15 years. Dysplasia and cancer were not found. From 1 to 15 years after repair grade of histological oesophagitis often fluctuated between Gr 0 and Gr I, but further progression was unlikely, hazard ratio = 0.2-3.4 (95% confidence interval 0.0-29), P = 0.06-0.87. LG and antireflux surgery predicted early detection of metaplasia (P < 0.001). Only 9% of patients with metaplasia and 32% with Gr II oesophagitis were symptomatic. A total of 6 (3%) patients had a symptomatic anastomotic stenosis at 1 year. CONCLUSIONS: EFU revealed frequent oesophagitis and metaplasia, but no dysplasia or cancer. Routine endoscopic surveillance had limited benefit and seems unnecessary during childhood after repair of OA.

A population-based, complete follow-up of 146 consecutive patients after transanal mucosectomy for Hirschsprung disease.

OBJECTIVE: The objective of the study is to define the population-based bowel functional outcomes and enterocolitis following transanal endorectal pull-through (TEPT) in patients with Hirschsprung disease (HD) treated at our institution between 1986 and 2011. METHODS: 146 consecutive patients who had undergone primary surgical treatment for HD were included. The median follow-up time was 15 (3-33) years. The clinical details and prevalence of enterocolitis were evaluated in all patients, and bowel function in patients >3 years of age with functional intestino-anal continuity. RESULTS: No patients were lost to follow-up. Overall survival was 98%. The level of disease was rectosigmoid in 83%, long segment in 7%, total colonic in 4%, and extending up to the small bowel in 6%. 29% had an associated syndrome. 22% had a preoperative stoma. Operations included TEPT (89%), proctocolectomy with ileoanal anastomosis in 9%, and 3% had a permanent endostomy owing to small intestinal aganglionosis. One patient underwent intestinal transplantation. At the latest follow-up, 42% had occasional soiling, 12% had frequent soiling and 46% had no soiling. Constipation occurred in 9%. An associated syndrome was the only predictor for soiling or constipation (OR 4.3, 95% CI 1.5-12). 44% developed recurrent postoperative enterocolitis, which was predicted by extended aganglionosis (OR 6.9, 95% CI 2.4-20) and syndromatic disease (OR 2.4, 95% CI 1.2-5.0). CONCLUSION: The major functional sequelae following TEPT were recurrent enterocolitis and fecal soiling, which was mostly occasional. An associated syndrome was a predictor of a reduced bowel functional outcome, and alongside extended aganglionosis were significant risk factors for recurrent postoperative enterocolitis.

Revisional surgery for recurrent tracheoesophageal fistula and anastomotic complications after repair of esophageal atresia in 258 infants.

AIM: We assessed the occurrence and outcome of major reoperations following repair of esophageal atresia with or without tracheoesophageal fistula (TOF). Major outcome measures were survival, preservation of native esophagus, and long-term esophageal function. METHODS: Hospital charts of 258 consecutive patients treated for esophageal atresia from 1980 to 2013 were reviewed. RESULTS: Forty-two (16%) patients required a total of 57 reoperations after primary repair (n=37) or esophageal reconstruction (n=5). The indications were anastomotic leakage (n=17), anastomotic rupture after endoscopic dilatation (n=5), recurrent tracheoesophageal fistula (TOF) (n=12), undiagnosed proximal TOF (n=3), recalcitrant anastomotic stricture (n=11, primary anastomosis 9, reconstruction 2), undetected proximal fistula (n=3), and inadvertently perforated jejunal graft (n=1). Anastomotic leakage and rupture after dilatation were treated with rethoracotomy and suture and recurrent or undetected TOF by open repair. Strictures not manageable with repeated dilatations were resected and esophageal ends reanastomosed (n=10) or bridged with jejunum graft (n=1). Five (12%) patients required further reoperations, two after recurrent TEF (reocclusion n=1, reconstruction with gastric tube n=1), two after stricture operations (re-resection n=1, resuture after leakage n=1), and one after recurrent dilatation-related rupture. Mortality was 4/42 (10%). Two patients died of recurred leakage or TOF and two of unrelated cause. Of 38 survivors, 35 retained their native or initially reconstructed esophagus, and 3 had secondary reconstruction. After a median follow-up of 23 (range 0.6-32) years, 35 (95%) patients have acceptable esophageal function. Three patients remained dependent on gastrostomy feedings. CONCLUSION: Anastomotic and TOF complications required a substantial number of reoperations, including esophageal reconstructions. Over 90% of the patients survived with a functioning native or reconstructed esophagus.

The cumulative incidence of significant gastroesophageal reflux in patients with congenital diaphragmatic hernia-a systematic clinical, pH-metric, and endoscopic follow-up study.

BACKGROUND: Gastroesophageal reflux (GER) is common in patients with congenital diaphragmatic hernia (CDH). Gastroesophageal reflux may occur early after the primary repair of CDH and require antireflux surgery (ARS). It is unknown how many patients will be severely affected later on. We conducted an objective long-term follow-up for the cumulative incidence of CDH-associated GER based on symptoms, pH-metry, and histology. MATERIALS AND METHODS: From March 1990 to July 2006, we admitted 33 newborn patients with CDH. Twenty-six patients (79%) (male 13, left-sided 21) survived. Extracorporeal membrane oxygenation was required in one patient and patch closure in 10 patients. The follow-up consisted of assessment of GER symptoms at 6 months, 1 year, 3 years, 5 years, and 10 years; endoscopy; and pH-metry for all patients at 1 year and to selected patients (with symptoms or complications of GER) from 3 to 10 years after the primary closure of CDH. Gastroesophageal reflux was considered significant (sGER) when a symptomatic patient required ARS, had endoscopic biopsies showing at least moderate esophagitis, or total and preprandial reflux index of more than 10% and 5%, respectively. MAIN RESULTS: The incidence of sGER (patients with sGER/total amount assessed) at 6 months, 1 year, 3 years, 5 years, and 10 years was 27% (7/26), 42% (11/26), 53% (8/15), 53% (8/15), and 55% (5/9), respectively. During a median follow-up of 60 months (range, 12-195 months), 12 (46%) of 26 patients had sGER and 4 (15%) required ARS. After the assessment at 1 year, only one new case of sGER appeared. Endoscopic and/or pH-metric assessment covered 100% of the patients at 1 year follow-up, but later on only 70%. CONCLUSION: One year after the primary closure of CDH, the incidence of sGER was 42%. After 1 year follow-up, only one new case of sGER was found, and ARS was not required. In patients who required ARS manifested before 6 months.