Outcomes of fundoplication in oesophageal atresia associated gastrooesophageal reflux disease

Aim of the study: Conservative management of gastrooesophageal reflux (GORD) in oesophageal atresia (OA) is sometimes inefficient, and fundoplication is required. We assessed the outcomes of fundoplication among OA patients from 1980 to 2016. Methods: After ethical consent, hospital records of 290 patients, including 22 referred patients, were reviewed. Included were 262 patients with end-to-end repair. Excluded were patients who underwent oesophageal reconstruction (n = 23) or no repair (n = 5). Primary outcome measures included survival, retaining the native oesophagus, resolution of GGORD symptoms, failure of fundoplication, and long-term endoscopic results. Main results: Gross types of OA in 262 patients were A (n = 12), B (n = 2), C (n = 217), D (n = 10), E (n = 19), and F (n = 2). Eighty-six (33%) patients, type A (n = 12, 100%), B (n = 2, 100%), C (n = 69, 31%), D (n = 3, 30%), and F (n = 1, 50%), underwent fundoplication at the median age of 5.4 (IQR 3.1-16) months. Main indications included recalcitrant anastomotic stenosis (RAS) in 41 (48%), respiratory symptoms in 16 (19%), and acute life threatening events (ALTE) in 15 (17%) of patients. Associated tracheomalacia in 25 (29%) patients were treated with aortopexy. Median follow-up was 7.5 (IQR 1.8-15) years. RAS resolved in 30 (73%) patients, whereas 11 (27%) with unresolved RAS underwent oesophageal resection (n = 8) or replacement (n = 3). Six (7%) patients died of heart failure (n = 4), bolus impaction (n = 1), and ALTE (n = 1). Fundoplication failed in 27 (31%) patients, and 13 (15%) underwent redo fundoplication. Fundoplication failure was predicted by long-gap OA RR = 3.8 (95% CI = 1.1-13), P = 0.04. In total GORD associated symptoms persisted in 7 (8%) patients, including one with permanent feeding jejunostomy. Latest endoscopy showed moderate or severe oesophagitis in 7% of fundoplicated and in 3% nonfundoplicated patients and intestinal metaplasia in 3% and 1% (p = 0.20-0.29). Conclusion: Fundoplication provided a safe and relatively effective control of OA associated symptomatic GORD and oesophagitis. The failure rate of fundoplication was high in those with long-gap OA. Type of study: Treatment study. Level of evidence: IV (C) 2017 Elsevier Inc. All rights reserved.Peer reviewe

Combined management of perianal rhabdomyosarcoma with chemotherapy, radical surgery, and irradiation : A series of three consecutive children

Background and aim: We describe a series of three successive patients with perianal rhabdomyosarcoma (PRMS) from 2014 to 2017 managed with combined chemotherapy, radical surgery, and radiotherapy. Methods: Ethical consent was obtained. Data including tumor presentation, treatment, and survival was collected from hospital reports. Results: Two girls aged 15 and 16 years (patient #1 and #2) and one boy aged five years (patient #3) were referred because of a suspected perianal abscess. MRI showed large perianal tumors from 7 to 12 cm in diameter that surrounded or infiltrated the anal sphincters and were inconsistent with abscess. Tumor biopsies showed RMS of alveolar (#1 and #2) and embryonal (#3) types. Patient #1 had lymph node and bone metastases, patient #2 lymph node metastases, and patient # 3 no metastases. Pretreatment staging, IRS Clinical Group, and Risk Groups were: Stage 4, II, high; Stage 3, GII, intermediate; and Stage 3, I, low, respectively. All underwent colostomy before neoadjuvant chemotherapy (CWS-RMS 2009 program). Neoadjuvant chemotherapy failed to clear the tumors from anal sphincters preventing anus-saving surgery, and all patients underwent abdominoperineal excision. All removed specimens had freemargins with negative lymph nodes. After adjuvant chemotherapy and local radiation, the patients were tumor free after 48, 13, and 18 months. Conclusion: In PRMS local surgical control required abdominoperineal excision. Confusion between PRMS and abscess may cause unnecessary delay in management. (C) 2018 Elsevier Inc. All rights reserved.Peer reviewe

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. (C) 2017 Elsevier Inc. All rights reserved.Peer reviewe

Recovery after open versus laparoscopic pyloromyotomy for pyloric stenosis: a double-blind multicentre randomised controlled trial

BackgroundA laparoscopic approach to pyloromyotomy for infantile pyloric stenosis has gained popularity but its effectiveness remains unproven. We aimed to compare outcomes after open or laparoscopic pyloromyotomy for the treatment of pyloric stenosis.MethodsWe did a multicentre international, double-blind, randomised, controlled trial between June, 2004, and May, 2007, across six tertiary paediatric surgical centres. 180 infants were randomly assigned to open (n=93) or laparoscopic pyloromyotomy (n=87) with minimisation for age, weight, gestational age at birth, bicarbonate at initial presentation, feeding type, preoperative duration of symptoms, and trial centre. Infants with a diagnosis of pyloric stenosis were eligible. Primary outcomes were time to achieve full enteral feed and duration of postoperative recovery. We aimed to recruit 200 infants (100 per group); however, the data monitoring and ethics committee recommended halting the trial before full recruitment because of significant treatment benefit in one group at interim analysis. Participants, parents, and nursing staff were unaware of treatment. Data were analysed on an intention-to-treat basis with regression analysis. The trial is registered with ClinicalTrials.gov, number NCT00144924.FindingsTime to achieve full enteral feeding in the open pyloromyotomy group was (median [IQR]) 23·9 h (16·0–41·0) versus 18·5 h (12·3–24·0; p=0·002) in the laparoscopic group; postoperative length of stay was 43·8 h (25·3–55·6) versus 33·6 h (22·9–48·1; p=0·027). Postoperative vomiting, and intra-operative and postoperative complications were similar between the two groups.InterpretationBoth open and laparoscopic pyloromyotomy are safe procedures for the management of pyloric stenosis. However, laparoscopy has advantages over open pyloromyotomy, and we recommend its use in centres with suitable laparoscopic experience.FundingSir Arthur Halley Stewart Trust