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Objective The aim of this study was to assess incidence, risk factors, and outcomes of distal recurrent tracheoesophageal fistula (RTEF) after repair of esophageal atresia (EA). Methods Ethical consent was obtained. Data collection was done by review of hospital records of 286 patients (268 in-house and 18 referred) who underwent repair of type C or D EA from 1980 to 2021. Spitz class, long-gap (tracheoesophageal fistula at carina), fundoplication, leakage, and stricture were assessed as RTEF risk factors. Outcome measures were long-term closure of RTEF, retainment of native esophagus, and survival. Results RTEF occurred in 23 patients (19 in-house) with type C (n = 22) or type D (n = 1) EA with median 4.4 (interquartile range [IQR]: 1.7–13) months after repair. Five patients had late RTEF 3.5 to 16 years after repair. Nineteen (7.3%) in-house patients developed RTEF. Presenting symptoms, age at diagnosis, and presence of anastomotic stricture (AS) are listed. No statistically significant risk factors were found, risk ratio of 0.5 to 2.7 (IQR: 0.1–8.7), p-value of 0.25 to 0.75. Detached closure clip and esophageal foreign body contributed to RTEF in two patients. Sixteen patients underwent rethoracotomy for closure of RTEF, 5 (22%) with AS eventually underwent esophageal reconstruction after a period with cervical esophagostomy. Two patients with late-manifested RTEFs underwent closure with laser cauterization. In 22 patients, treatment of RTEF succeeded, whereas 1 (4%) premature patient died of instant re-RTEF. Conclusion RTEF had an incidence of 7% with diverse patterns of manifestation and predicting factors. Closure rate and patient survival were excellent, but RTEF with AS predicted loss of native esophagus.

An assessment of the clinical data and outcome of patients with oesophageal atresia (OA) with very low birth weight (VLBW) was completed.With ethical consent, we reviewed the records of 327 successive patients with OA from 1980 to 2020. Main outcome measures survival and oesophageal repair were compared between patients with VLBW(≤1500 g) and with BW1500 g.Thirty-four (10%) patients had VLBW. Gross types of OA in VLBW were similar as in other patients: A (15%/7%), B (3%/3%), C (78%/82%), D (3%/4%), E (0%/7%), F (0%/1%) (p = 0.16-0.99). In VLBW the incidence of congenital heart disease (CHD) (47%) and trisomy 13/18 and Cri du Chat (15%) were higher than in BW1500 g (23% and 1%), (p = 0.001 both). In VLBW one-month mortality was 35% vs 4% in patients with BW1500 g (p 0.001), overall mortality 56% and 8% (p 0.001), respectively. Cause of one-month mortality in VLBW (12 patients) were CHD w/wo chromosomal abnormality (n = 7), cerebral hemorrhage (n = 2), gastric perforation (n = 1), anastomotic leakage (n = 1) and pulmonary hemorrhage (n = 1). Of VLBW patients 79% and of other patients 99% underwent oesophageal repair (p 0.001). Repair in VLBW patients included early (n = 18) or delayed (n = 5) end-to-end anastomosis and reconstruction (n = 4). Anastomotic complications occurred in 24% of patients with VLBW and in 17% with BW1500 g, (p = 0.31). From 1980-2000 to 2001-2020 survival in VLBW changed from 11% to 81% (p = 0.002). During 2001 - 2020 all VLBW patients underwent repair.OA with VLBW had high incidence of life-threatening associated anomalies and decreased survival. Recently survival and rate of oesophageal repair have improved significantly.

OBJECTIVE The aim of the study was to assess long-term morbidity in children operated for choledochal malformation (CM) by relating clinical complications to liver histopathology, follow-up imaging, liver stiffness, and biochemistry. METHODS A single-center retrospective follow-up study including all CM patients (n = 55, 71% girls) treated during 1976 to 2018 was performed. Mann-Whitney U test and Spearman rank correlation were used for statistical analyses. RESULTS During median follow-up of 5.8 (interquartile range, 2.5-12) years, 1 patient was lost to follow-up whereas all survived. Intraoperative liver biopsies showed fibrosis in 32%, and patients with Metavir stage ≥2 were younger at surgery (0.36 [0.11-1.9] vs 3.8 [0.72-10.5] years, P = 0.024) than those without fibrosis. Overall, 21% had long-term complications including cholangitis in 9 (>2 episodes in 5) patients, anastomotic stricture in 2 referred patients and adhesive volvulus or hepatocellular carcinoma in 1 each. Anastomotic strictures were successfully managed nonoperatively and hepatocellular carcinoma with thermoablation. In postoperative magnetic resonance cholangiography (MRCP) performed 6.4 (3.6-16) years after hepaticojejunostomy, diameters of both main intrahepatic ducts had decreased significantly to 3.0 (2.5-3.5) mm (P = 0.0001) but a distal cyst stump was remaining in 30% with a length of 6.0 (4.0-20) mm that associated with operation age (r = 0.71, P = 0.015) and fusiform CM type. Follow-up ultrasound revealed mild dilation of intrahepatic bile ducts in 6.3% and mildly to moderately elevated liver biochemistry in 23%, and liver stiffness (>7 kPa) in 22%. CONCLUSIONS Whilst cholangitis was the most common postoperative problem, individual patients experienced other more significant complications and one quarter of patients showed evidence of underlying liver dysfunction.

Introduction In necrotizing enterocolitis (NEC), blood transfusions (BTs) are a disputed factor concerning the etiology and the outcome. We assessed retrospectively the effects of preonset BT on the outcome of NEC and spontaneous intestinal perforation (SIP). Materials and Methods With ethical consent, we reviewed the hospital records of 113 neonates from 2002 to 2019 with surgical NEC (NECs) (n = 57), conservatively treated NEC (NECc) (n = 20), and SIP (n = 36). The onset was defined as the day of surgery (NECs and SIP) or diagnosis (NECc). The effects of preonset BT (total, beyond 48 hours, and within 48 hours) were compared with the effects of birth weight (BW), gestational age (GA), intracerebral hemorrhage, respiratory distress syndrome, septicemia, and patent ductus arteriosus. Main outcome measure was 2-week mortality, and secondary measures were small intestinal loss and days on parenteral nutrition (PN). Results Overall mortality (8.9%) was predicted independently by BT total and BT beyond 48 hours, risk ratio (RR) = 1.1 to 1.2 (95% confidence interval [CI] = 1.0–1.4), p = 0.01 to 0.02, and BW and GA, RR = 0.7 to 1.0 (95% CI = 0.5–1.0), p = 0.02 to 0.04. Mortality in NECs (12%) was predicted by BT total and BT beyond 48 hours only, RR = 1.1 to 1.2 (95% CI = 1.0–1.5), p = 0.03 to 0.04. BT within 48 hours of onset did not predict mortality. No factors were related with secondary outcome measures or with mortality in patients with SIP or NECc. Conclusion In NECs, preoperative BT and BT given more than 48 hours before surgery were correlated with slightly increased 2-week mortality but not with small intestinal loss or duration of PN.

Introduction Several surgical techniques are available for pediatric esophageal reconstruction. We started to use pedicled jejunum interposition graft (PJIG) because other techniques had significant long-term complications. In this retrospective study, the indications, surgical complications, and long-term outcomes were assessed in patients with PJIG. Materials and Methods With ethical consent, we reviewed the hospital records of 14 patients (7 females) who from 2005 to 2019 underwent a total of 16 esophageal reconstructions with PJIG. Results Median age at PJIG was 1.6 (range: 0.2–15) years. Underlying conditions were esophageal atresia (EA) (n = 11) or native esophagus lost by trauma or infection (n = 3). Eight patients with EA underwent PJIG as primary reconstruction and three as a rescue operation after complications in primary repair. Significant surgical complications occurred in 43% of patients. Major reoperations in six (43%) patients included resection and reanastomosis of strictured proximal PJIG (n = 1) and redo PJIG after failure of the first operation (n = 2). Surgical mortality was nil. After a median follow-up of 6.5 (range: 0.7–14) years, 13 (93%) patients survived, and 1 died of congenital heart disease. PJIG failed in three (23%) survivors of whom two underwent graft removal because of life-threatening aspiration and one did not start oral feeds at all. Ten survivors (77%) have full enteral feeds. Respiratory function in the survivors is satisfactory. Two patients have moderate and three mild gastroesophageal reflux symptoms. Conclusion PJIG was a functional option for a variety of conditions that required esophageal reconstruction. However, significant early and late complications required major surgical revisions.

Simple Summary We investigated the increased prevalence of Barrett's esophagus in adults with esophageal atresia. A higher polygenic risk score and disturbances in inflammatory, stress response and oncological pathways upon acid exposure suggest a genetic susceptibility and increased induction of inflammatory processes. Although further research is required to explore this hypothesis, this could be a first-step into selecting patients that are more at risk to develop Barrett's esophagus and/or esophageal carcinoma. Currently, an endoscopic screening and surveillance program is in practice in our institution for patients born with esophageal atresia, to early detect (pre)malignant lesions. Since recurrent endoscopies can be a burden for the patient, selecting patients by for example genetic susceptibility would allow to only include those at risk in future practice. The prevalence of Barrett's esophagus (BE) in adults born with esophageal atresia (EA) is four times higher than in the general population and presents at a younger age (34 vs. 60 years). This is (partly) a consequence of chronic gastroesophageal reflux. Given the overlap between genes and pathways involved in foregut and BE development, we hypothesized that EA patients have an intrinsic predisposition to develop BE. Transcriptomes of Esophageal biopsies of EA patients with BE (n = 19, EA/BE); EA patients without BE (n = 44, EA-only) and BE patients without EA (n = 10, BE-only) were compared by RNA expression profiling. Subsequently, we simulated a reflux episode by exposing fibroblasts of 3 EA patients and 3 controls to acidic conditions. Transcriptome responses were compared to the differential expressed transcripts in the biopsies. Predisposing single nucleotide polymorphisms, associated with BE, were slightly increased in EA/BE versus BE-only patients. RNA expression profiling and pathway enrichment analysis revealed differences in retinoic acid metabolism and downstream signaling pathways and inflammatory, stress response and oncological processes. There was a similar effect on retinoic acid signaling and immune response in EA patients upon acid exposure. These results indicate that epithelial tissue homeostasis in EA patients is more prone to acidic disturbances.

Aim To assess the risk factors for anastomotic stricture (AS) in end-to-end anastomosis (EEA) in patients with esophageal atresia (EA). Methods With ethical consent, hospital records of 341 EA patients from 1980 to 2020 were reviewed. Patients with less than 3 months survival (n = 30) with Gross type E EA (n = 24) and with primary reconstruction (n = 21) were excluded. Outcome measures were revisional surgery for anastomotic stricture (RSAS) and number of dilatations required for anastomotic patency without RSAS. The factors that were tested for risk of RSAS or dilatations were distal tracheoesophageal fistula (TEF) at the carina in C-type EA (congenital TEF [CTEF]), type A/B EA, antireflux surgery (ARS), anastomotic leakage, recurrent TEF, and Spitz group and congenital heart disease. Main Results A total of 266 patients, Gross type A (n = 17), B (n = 3), C (n = 237), or D (n = 9) underwent EEA (early n = 240, delayed n = 26). Early anastomotic breakdown required secondary reconstruction in five patients. Of the remaining 261 patients, 17 (6.1%) had RSAS, whereas 244 patients with intact end to end required a median of five (interquartile range: 2–8) dilatations for anastomotic patency. Main risk factors for RSAS or (> 8) dilatations were CTEF, type A/B, ARS, and anastomotic leakage that increased the risk of RSAS or dilatations from 4.6- to 11-fold. Conclusion The risk of severe AS is associated with long-gap EA, significant gastroesophageal reflux, and anastomotic leakage.

Introduction The purpose of this study was to investigate the epidemiology and characteristics of surgically treated ovarian lesions in preadolescent girls. Material and methods This was a retrospective cohort study including all 0- to 11-year-old girls operated at a single center from 1999 to 2016 for ovarian cysts, neoplasms or torsions. Patient charts were reviewed for symptoms, preoperative radiological imaging, operative details and histopathology. Results We identified 78 girls, resulting in a population-based incidence of 4.2/100 000. Infants (n = 44) presented with benign cysts (42/44, 95%, one bilateral), a benign neoplasm (1/44, 2%) and a torsion without other pathology (1/44, 2%). Torsion was found in 25/29 (86%) ovaries with complex cysts and in 3/15 (21%) ovaries with simple cysts in preoperative imaging (P

Introduction The aim of this study was retrospective assessment of late major reoperations after surgery for necrotizing enterocolitis (NEC) and spontaneous intestinal perforation (SIP) in neonates. Materials and Methods Appropriate ethical consent was obtained. Data collection was by review of hospital records of 165 successive prematures who underwent surgery for NEC (n = 99) or SIP (n = 66) from 1986 to 2019. Outcome measures were late major abdominal surgeries or endoscopies that occurred after the primary surgical treatment, closure of enterostomies, and weaning from parenteral nutrition (PN). We assessed also risk factors for late abdominal surgeries. Results Hundred and twenty-six (76%) patients (NEC, n = 70, 71% and in SIP, n = 56, 85%) survived. Median follow-up was 13 (interquartile range: 5.9–23) years. Nineteen (15%) patients underwent late abdominal surgery with 0% mortality. Most common was surgery for small bowel obstruction (SBO) in 12(9.5%) patients (NEC, n = 5; 7.0%, SIP, n = 7; 13%, p = 0.36) with 10-year cumulative risk of 8.7 (95% confidence interval [CI]: 3.5–14). Long duration of PN and development of intestinal failure associated cholestasis (IFAC) increased the risk of SBO surgery, relative risk: 1.0–3.2 (95% CI: 1.0–9.1), p = 0.02–0.03. Other surgeries included serial transverse enteroplasty (NEC, n = 2), incisional hernia repair (n = 3), cholecystectomy (n = 1), and ovarian torsion (n = 1). Twenty (29%) NEC and four (7%) SIP patients required endoscopic assessment of postoperative symptoms (p = 0.003). Conclusion Late abdominal reoperations occurred in 15% of patients with NEC and SIP with nil mortality. SBO was the main indication. Risk of SBO was similar between NEC and SIP. Long PN and IFAC increased the risk of SBO.

Aim:Controversies exist on the optimal diagnostic workup for neonates with esophageal atresia (EA) with/without tracheoesophageal fistula (TEF). Aim of this study was to describe the current diagnostic policies in EA/TEF patients enrolled in an International multicenter registry. Methods:All patients consecutively registered from July 2014 to December 2017 in the EUPSA Esophageal Atresia Registry (EUPSA-EAR) were included in the study. Data related to diagnostic investigations among Centers forming the EUPSA-EAR were analyzed. Main Results:During the study period, 374 consecutive patients were recorded by 23 Centers. The majority of patients underwent chest X-rays, echocardiography, abdominal ultrasound, and abdominal X-rays. Preoperative bronchoscopy and esophageal gap measurement were performed in one third of the patients. Conclusions:Present data from a large cohort of patients from the EUPSA-EAR show both inter-institutional and intra-institutional variability in diagnostic workup of patients with EA/TEF. Efforts should be made to develop guidelines on the diagnostic workup for EA/TEF patients.

Introduction Many aspects of the management of esophageal atresia (EA) and tracheoesophageal fistula (TEF) are controversial and the evidence for decision making is limited. Members of the European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) conducted a consensus conference on the surgical management of EA/TEF based on expert opinions referring to the latest literature. Materials and Methods Nineteen ERNICA representatives from nine European countries participated in the conference. The conference was prepared by item generation, item prioritization by online survey, formulation of a final list containing the domains diagnostics, preoperative, operative, and postoperative management, and literature review. The 2-day conference was held in Berlin in October 2018. Anonymous voting was conducted via an internet-based system. Consensus was defined when 75% of the votes scored 6 to 9. Results Fifty-two items were generated with 116 relevant articles of which five studies (4.3%) were assigned as level-1evidence. Complete consensus (100%) was achieved on 20 items (38%), such as TEF closure by transfixing suture, esophageal anastomosis by interrupted sutures, and initiation of feeding 24 hours postoperatively. Consensus ≥75% was achieved on 37 items (71%), such as routine insertion of transanastomotic tube or maximum duration of thoracoscopy of 3 hours. Thirteen items (25%) were controversial (range of scores, 1–9). Eight of these (62%) did not reach consensus. Conclusion Participants of the conference reached significant consensus on the management of patients with EA/TEF. The consensus may facilitate standardization and development of generally accepted guidelines. The conference methodology may serve as a blueprint for further conferences on the management of congenital malformations in pediatric surgery.

Background & Aims: Diagnostic criteria, progression risk and optimal monitoring for intestinal failure (IF)-associated liver disease (IFALD) remain undefined. We assessed predictors, noninvasive markers and progression of histopathological liver disease in patients with IF. Methods: In total, 77 children with IF and median age of 1.7 years underwent diagnostic liver biopsy, which was repeated in 48 patients after 2.9 years with simultaneous evaluation of liver biochemistry, liver stiffness, serum citrulline (a surrogate for viable enterocyte mass), spleen size, esophageal varices and clinical data. Patients were staged according to histopathological liver disease activity: active IFALD (cholestasis and/or inflammation), chronic IFALD (significant fibrosis and/or steatosis), or no IFALD (none of these features). Results: Diagnostic liver biopsy revealed active, chronic or no IFALD in 48%, 21% and 31% of patients. Active IFALD was segregated by low serum citrulline, parenteral nutrition (PN) dependency and young age, while weaning off PN and older age predicted chronic IFALD. Although the liver histopathology in most patients either normalized (52%) or transformed to a less reactive (chronic) disease stage (23%), 19% of patients retained and 6.3% progressed to an active cholestatic/inflammatory IFALD phenotype. Decreased serum citrulline and PN-dependency also predicted active IFALD in follow-up biopsies. Increased median liver biochemistry values and liver stiffness only associated with active IFALD, which was accurately identified by gammaglutamyltransferase (GGT), citrulline and liver stiffness, their combinations reaching diagnostic and follow-up AUROC values above 0.90. Conclusions: Active IFALD, essentially predicted by intestinal disruption and PN-dependency, was accurately detected by GGT, liver stiffness and citrulline, which together with recent advances in clinical management options, provides new avenues for monitoring and targeted liver protection in patients with IF. Lay summary: Liver disease is a common and critical complication in patients with intestinal failure, who require intravenous nutrition for survival due to severe intestinal dysfunction. We showed that both intravenous nutrition dependency and intestinal disruption essentially predicted development of active histopathological liver disease, which persisted in 25% of patients during long-term follow-up and could be accurately detected without the need for liver biopsy. Identification of the active and potentially progressive histopathology offers new possibilities for monitoring and targeted liver protection in patients with intestinal failure. (C) 2020 European Association for the Study of the Liver. Published by Elsevier B.V.

Objectives: Outcomes of pediatric-onset portal hypertension are poorly defined. We aimed to assess population-based long-term outcomes of pediatric-onset portal hypertension complicated by varices. Methods: All children with esophageal varices (n = 126) were identified from 14,144 single nationwide referral center endoscopy reports during 1987 to 2013, and followed up through national health care and death registers. A questionnaire was sent to survivors (n = 94) of whom 65 (69%) responded. Results: Nineteen underlying disorders included biliary atresia (35%), extrahepatic portal vein obstruction (35%), autosomal recessive polycystic kidney disease (7%), and other disorders (23%). During median follow-up of 15.2 (range 0.5-43.1) years patients underwent median 9 (1-74) upper gastrointestinal endoscopies. Esophageal varices were first observed at a median age of 4.0 (0.3-18.2) years, 112 (89%) patients underwent median 6 (1-56) sclerotherapy/banding sessions, and 61 (48%) experienced median 2 (range 1-20) variceal bleeding episodes. Forty-eight surgical shunt procedures were performed to 41 (36%) patients and 38% underwent liver transplantation. Portal hypertensive biliopathy was diagnosed in 4 patients. Hepatopulmonary syndrome necessitated liver transplantation in 2 patients, hepatic encephalopathy in 2, and hepatorenal syndrome in 1. No patient died of variceal bleeding. Patient-reported perception of health on a scale of 1 to 10 was 9 (range 4-10), and 86% reported no current symptoms attributable to esophageal varices. Conclusions: Pediatric-onset portal hypertension is a heterogeneous disease with significant long-term morbidity, requiring multimodal approach with considerable resources and continuation of follow-up in adulthood. Although mortality to variceal bleeding was avoided, bleeding episodes recurred also in adulthood, while patient-reported health of long-term survivors was encouraging.

BACKGROUND AND AIMS Childhood-onset ulcerative colitis [UC] requires total colectomy in one-quarter of patients at some point of their disease. The study objective was to evaluate long-term outcomes after proctocolectomy with ileoanal anastomosis [IAA] for paediatric UC. METHODS Medical records of all children undergoing proctocolectomy with IAA for UC during 1985-2016 in Helsinki University Hospital were retrospectively assessed. Data on disease history, diagnostic and operative details, occurrence of surgical complications, functional outcome, postoperative diagnosis of Crohn's disease [CD] and pouch failure were collected. Risk factors for IAA failure were analysed with Cox regression. RESULTS Of 87 patients, 85 [98%] had UC and 2 [2%] inflammatory bowel disease unclassified [IBD-U] preoperatively. Altogether 66% underwent two-stage and 34% underwent three-stage procedures. During 7.8 [4.1-14.5] years' follow-up, nine [10%] patients were diagnosed with postoperative CD. Postoperative leakages [n = 8, 9%] and strictures [n = 10, 11%] were equally common, whereas fistulas [78% vs 9%, p

Background: Necrotizing enterocolitis (NEC) and spontaneous intestinal perforation (SIP) are the most common abdominal surgical conditions in preemies. Associated mortality remains high and long periods of parenteral nutrition (PN) may be required. We assessed the developments in the outcomes of surgically treated NEC and SIP in the two largest Finnish neonatal intensive care units (NICU). Methods: Retrospective observational study based on hospital records during 1986-2014. Main outcome measures were three-month survival during 1986-2000 compared with 2001-2014 and predictors of mortality. Results: Included were 225 patients (NICU A 131 and NICU B 94) with NEC in 142 (63%) and SIP 83 (37%). The median birth weight (BW) (870 vs 900 g) and gestation age (GA) (27 vs 27 weeks, p = 0.96) were similar in NEC and SIP. Small intestine was affected in 85% of NEC and 76% of SIP patients (p = 0.12). In 5% of patients NEC was panintestinal. Median small intestinal loss was 25% in NEC and 4.0% in SIP (p

Background/purpose: Biliary atresia is the most common reason for newborn cholestasis and pediatric liver transplantation. Even after normalization of serum bilirubin after portoenterostomy, most patients require liver transplantation by adulthood due to expanding fibrosis. We addressed contemporary outcomes of biliary atresia in the Nordic countries. Methods: Data on center and patients characteristics, diagnostic practices, surgical treatment, adjuvant medical therapy after portoenterostomy, follow-up and outcomes were collected from all the Nordic centers involved with biliary atresia care during 2005-2016. Results: Of the 154 patients, 148 underwent portoenterostomy mostly by assigned surgical teams at median age of 64 (interquartile range 37-79) days, and 95 patients (64%) normalized their serum bilirubin concentration while living with native liver. Postoperative adjuvant medical therapy, including steroids, ursodeoxycholic acid and antibiotics was given to 137 (93%) patients. Clearance of jaundice associated with young age at surgery and favorable anatomic type of biliary atresia, whereas annual center caseload >3 patients and diagnostic protocol without routine liver biopsy predicted early performance of portoenterostomy. The cumulative 5-year native liver and overall survival estimate was 53% (95% CI 45-62) and 88% (95% CI 83-94), respectively. Portoenterostomy age 3 patients were predictive for long-term native liver survival, while normalization of serum bilirubin after portoenterostomy was the major predictor of both native liver and overall 5-year survival. Conclusions: The outcomes of biliary atresia in the Nordic countries compared well with previous European studies. Further improvement should be pursued by active measures to reduce patient age at portoenterostomy. Retrospective prognosis study: Level II. (C) 2017 Elsevier Inc. All rights reserved.

Background Pediatric-onset intestinal failure (IF) remains a severe illness with life-threatening consequences. In this study, we analyzed a single center's outcomes of IF over 3 decades. Methods All children with IF who required parenteral nutrition (PN) >2 months or small-intestinal resection ≥50% managed since 1984 were included for retrospective outcome analyses. Results In total, 100 patients with median PN duration of 1.2 (interquartile range, 0.4-3.5) years were identified. Causes of IF were short bowel syndrome (SBS; n = 78), primary intestinal motility disorders (n = 14), and congenital intestinopathies (n = 8). Patients with SBS had median 40 (25-60) cm of small bowel remaining. Overall, Kaplan-Meier 5- and 10-year weaning-off estimates were 67% (95% CI, 57-77) and 73% (95% CI, 63-84), respectively. Weaning off PN was predicted by remaining bowel anatomy, multidisciplinary treatment era, and absence of immune deficiency. Catheter-related bloodstream infections decreased from 1.4 to 0.6/1000 PN days (P = .0003) with systematic use of taurolidine locks. None had progressive liver disease. Thirty-one percent of patients with SBS underwent autologous intestinal reconstructive surgery. Five patients received and 2 were listed for isolated intestinal transplantation. Eight patients died, and overall 15-year survival rate estimate was 91% (95% CI, 85-98). Conclusions Despite reassuring rates of survival and weaning off PN, long-term PN failed in 14% of patients solely because of catheter complications in the recent era. Achievement of enteral autonomy in those with the shortest remaining small bowel and functional cause of IF remains challenging.

Study Design: A retrospective cohort study of consecutively operated neuromuscular scoliosis patients. Background and Aim: Surgical correction of neuromuscular scoliosis can be complicated by early gastrointestinal complications, but data on the extent and severity of them is scarce. The aim of the study was to determine the incidence, course, and risk factors of gastrointestinal complications after neuromuscular scoliosis correction. Material and Methods: Ninety-one patients (Results: The average age at surgery was 14.5 (SD 2.9) and follow-up time was 4.9 (SD 2.3) years. Gastrointestinal complications occurred in 12 (13%) patients and included prolonged paralytic ileus (7%, 6/91), dysphagia (7%, 6/91), and gastroparesis (1%, 1/91). Hospital stay was 22 (SD 11) days in patients with gastrointestinal complications and 16 (SD 20) days in non-complicated patients (p = 0.005). Dysphagia required permanent feeding gastrostomy in one patient whereas other complications were transient and none caused death. The risk factors for postoperative gastrointestinal complications were preoperative main curve correction 90° (RR = 5.5 (95% CI 1.3–23); p = 0.020), disturbance in intraoperative spinal cord monitoring (RR = 6.0 (95% CI 1.1–34); p = 0.043), and intravenous opioid medication over 5 days postoperatively (RR = 7.9 (95% CI 1.8–35), p = 0.006). Conclusion: Gastrointestinal complications occurred in 13% of patients after neuromuscular scoliosis correction. Marked gastrointestinal complications extended postoperative hospitalization period, but they were transient in majority (92%) of cases and none caused death. Rigid scoliosis was the most significant risk factor for gastrointestinal complications. Gastrointestinal complications appear to be less frequent after posterior only spinal fusion with total pedicle screw instrumentation and Ponte osteotomies.

Recurrent pediatric inguinal hernia is relatively rare. In a large series, the recurrence rates vary from 0.3 to 1.2%. In a university hospital with surgical service for a wide spectrum of sick pediatric patients, the real recurrence rate may be 3 to 6%. Recurrence manifests typically 6 months after first repair. Current evidence indicates that recurrence rates in open and laparoscopic repair are similar. Laparoscopic repairs are, however, still performed by a minority of skilled pediatric surgeons, whereas the majority of pediatric surgeons rely on open repair not least because of the assumed lower risk of recurrence. After repair of an indirect hernia, the majority of recurrences (97%) are indirect, whereas in rare cases the recurrence turns out to be a formerly undiagnosed direct or femoral hernia. In Children's Hospital of Helsinki, Finland, recurrence rate from 2012 to 2016 was 2.8% and operations for recurrent hernias comprised 3.3% of the total. From 1991 to 2017, a total of 130 operations for recurrent hernias were performed with open inguinal approach (n = 75, 58%), laparoscopy (n = 49, 38%), and laparotomy (n = 4, 4%). One teenage patient underwent a Lichtenstein Prolene net repair. A recurrent inguinal hernia can be repaired either with an open or laparoscopic approach. In laparoscopy, the nature of the recurrent hernia can be clarified and an immediate repair performed either by laparoscopic or open technique. In expert hands new laparoscopic techniques using muscular arch, lateral umbilical ligament, iliopubic tract, and division of the sac may be an alternative to a simple purse-string closure.

Aim The study aimed to compare outcomes and intestinal morbidity among complicated gastroschisis, isolated gastroschisis, and intestinal atresia. Methods In this retrospective observational single institution study, outcomes and intestinal morbidity were compared among gastroschisis complicated by intestinal atresia or perinatal bowel perforation, isolated gastroschisis, and isolated intestinal atresia. We included two cohorts; the first cohort included 68 consecutive patients with complicated gastroschisis (n = 9), isolated gastroschisis (n = 34), and intestinal atresia (n = 25) managed in our center. The second cohort included 20 patients (12 referred) with intestinal failure due to these underlying etiologies managed by our intestinal rehabilitation team. Results Patients with complicated gastroschisis had a significantly longer need for mechanical ventilation, primary hospital stay, and duration of parenteral nutrition (PN) and developed intestinal failure more often compared with other groups (p Conclusion Complicated gastroschisis is associated with markedly increased intestinal morbidity, reflected by prolonged duration of PN, more frequent reoperations for intestinal complications, and bowel re-dilatation after AIR surgery, when compared with patients with isolated gastroschisis or intestinal atresia.

Background Although adaptive mucosal growth of the remaining small intestine is an essential compensatory mechanism to bowel resection in experimental short-bowel syndrome (SBS), only scarce clinical data are available. We studied structural and molecular mechanisms of intestinal adaptation in children with SBS. Methods Fourteen patients, who had been dependent on parenteral nutrition (PN) since neonatal period for a median (interquartile range)1.4 (0.7-6.5) years, were studied at the age of 1.5 (1.0-6.5) years. Median length of remaining small bowel was 33 (12-60) cm, and 6 patients had their ileocecal valve preserved. Six children without gastrointestinal disorders served as age-matched and gender-matched controls. All patients underwent duodenal biopsies. Mucosal microarchitecture, proliferation, apoptosis, inflammation, and epithelial-barrier function were addressed using histology, immunohistochemistry, and quantitative real-time polymerase chain reaction. Results Villus height, crypt depth, enterocyte proliferation, and apoptosis were similar in patients and matched controls. Messenger RNA (mRNA) expression of numerous genes regulating gut epithelial-barrier function (TGFB2, CAV1, CLDN1, MUC2, and NLRC4) was significantly altered. Of various nutrient transporters studied, only expression of SLC2A1 encoding facilitative glucose transporter GLUT1 was increased among patients, whereas RNA expression of genes encoding sodium-dependent glucose, sterol, fatty-acid, and peptide transport remained unchanged. Conclusion Duodenal mucosal hyperplasia has a limited role in mediating physiological adaptation following intestinal resection among PN-dependent children with SBS. Further clinical studies addressing functional significance of the observed alterations in mucosal RNA expression are warranted.

OBJECTIVES We elucidated pathophysiology of pediatric gallstone disease by assessing liver expression of bile transporters in relation to bile acids and surrogates of cholesterol absorption and synthesis in serum and gallstones. METHODS RNA expression of canalicular bile transporters in liver biopsies from 32 pediatric gallstone patients and from 6 liver donors (controls) was measured by qRT-PCR (quantitative real-time reverse transcription polymerase chain reaction). Concentrations of cholesterol and precursors, plant sterols and bile acids in gallstones, and in serum of the patients and 82 healthy children were measured. Primary outcomes were the difference in RNA expressions and serum sterol profiles between patients and controls. RESULTS Cholesterol stones (CS; n = 15) contained cholesterol >42% and pigment stones (PS; n = 17)

Background: Surgery for necrotizing enterocolitis (NEC) and spontaneous intestinal perforation (SIP) is often complicated by intestinal failure (IF) and intestinal failure associated cholestasis (IFAC). Objective: Assessment of incidence, predictors, and mortality associated with IFAC in surgically treated NEC and SIP. Methods: A retrospective observational study based on hospital records during 1986-2014 in the two largest Finnish neonatal intensive care units was performed. IFAC was defined as conjugated bilirubin >34 mu mo1/1 (2.0 mg/dl) for >= two postoperative weeks while receiving parenteral nutrition (PN). Results: In total 225 patients underwent surgery for NEC (n 142; 63%) or SIP (n = 83; 37%). Included were 57 survivors with >= two weeks PN. Sixty-five (42%) patients developed IFAC. Two-year survival with IFAC was 80% and without IFAC 89% (p = 0.13). Of the 65 patients with IFAC, all eight with unresolved IFAC died in comparison to six of 57 (11%) whose IFAC resolved (p

Aim To define patterns in the management of pediatric appendicitis. Methods A total of 169 delegates from 42 (24 European) countries completed a validated survey administered at the EUPSA 2017 annual congress. Results In the work-up of children with suspected acute appendicitis, most surgeons rely on full blood count (92%), C-reactive protein (82%), and abdominal ultrasonography (76%), but rarely on computed tomography scans or magnetic resonance imaging. In suspected simple appendicitis, most surgeons (76%) do not perform appendectomy at night in clinically stable patients and start antibiotic preoperatively (64%), but only 15% offer antibiotic therapy alone (no appendectomy). In suspected perforated appendicitis, 96% start antibiotic preoperatively, and 92% perform an appendectomy. Presence of phlegmon/abscess is the main contraindication to immediate surgery. In case of appendix mass, most responders (75%) favor a conservative approach and perform interval appendectomy always (56%) or in selected cases (38%) between 2 and 6 months from the first episode (81%). Children with large intraperitoneal abscesses are managed by percutaneous drainage (59% responders) and by surgery (37% responders). Laparoscopy is the preferred surgical approach for both simple (89%) and perforated appendicitis (81%). Most surgeons send the appendix for histology (96%) and pus for microbiology, if present (78%). At the end of the operation, 58% irrigate the abdominal cavity only if contaminated using saline solution (93%). In selected cases, 52% leave a drain in situ. Conclusion Some aspects of appendicitis management lack consensus, particularly appendix mass and intraperitoneal abscess. Evidence-based guidelines should be developed, which may help standardize care and improve clinical outcomes.

Introduction Improvements in care of patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) have shifted the focus from mortality to morbidity and quality-of-life. Long-term follow-up is essential, but evidence is limited and standardized protocols are scarce. Nineteen representatives of the European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) from nine European countries conducted a consensus conference on the surgical management of EA/TEF. Materials and Methods The conference was prepared by item generation (including items of surgical relevance from the European Society for Pediatric Gastroenterology Hepatology and Nutrition (ESPGHAN)-The North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) guidelines on follow-up after EA repair), item prioritization, formulation of a final list containing the domains Follow-up and Framework, and literature review. Anonymous voting was conducted via an internet-based system. Consensus was defined as ≥75% of those voting with scores of 6 to 9. Results Twenty-five items were generated in the domain Follow-up of which 17 (68%) matched with corresponding ESPGHAN-NASPGHAN statements. Complete consensus (100%) was achieved on seven items (28%), such as the necessity of an interdisciplinary follow-up program. Consensus ≥75% was achieved on 18 items (72%), such as potential indications for fundoplication. There was an 82% concordance with the ESPGHAN-NASPGHAN recommendations. Four items were generated in the domain Framework, and complete consensus was achieved on all these items. Conclusion Participants of the first ERNICA conference reached significant consensus on the follow-up of patients with EA/TEF who undergo primary anastomosis. Fundamental statements regarding centralization, multidisciplinary approach, and involvement of patient organizations were formulated. These consensus statements will provide the cornerstone for uniform treatment protocols and resultant optimized patient care.

Gastroschisis and omphalocele are the most common congenital abdominal wall defects. Very commonly encountered associated chromosomal abnormalities and structural malformations significantly modify treatment strategies in the patients with omphalocele, while congenital cardiac defects are currently observed more frequently in gastroschisis than previously recognized. The incidence of gastroschisis is continuously increasing in the Western world. Despite recent developments of novel abdominal closure methods, including sutureless-staged closure with preformed silo in gastroschisis, and primary skin graft followed by expansion of the abdominal cavity and delayed abdominal wall closure with compartment separation in omphalocele, both conditions are still associated with significant morbidity. Dysmotile intestine in gastroschisis, especially when complicated by intestinal atresia, perforation, or necrosis, predisposes to prolonged dependence of parenteral nutrition and complications of intestinal failure. Mortality in omphalocele is mainly caused by associated congenital malformations and lung hypoplasia occasionally observed with a giant defect. With carefully planned, individualized multidisciplinary management, well over 90% of gastroschisis patients currently survive, while mortality in omphalocele remains higher mainly due to associated disorders.

BACKGROUND: The surgical repair of long-gap esophageal atresia (LGEA) is still a challenge and there is no consensus on the preferred method of reconstruction. We performed a systematic review of the surgical treatment of LGEA Gross type A and B with the primary aim to compare the postoperative complications related to the different methods within the first postoperative year.METHODS: Systematic literature review on the surgical repair of LGEA Gross type A and B within the first year of life published from January 01, 1996 to November 01, 2016.RESULTS: We included 57 articles involving a total of 326 patients of whom 289 had a Gross type A LGEA. Delayed primary anastomosis (DPA) was the most applied surgical method (68.4%) in both types, followed by gastric pull-up (GPU) (8.3%). Anastomotic stricture (53.7%), gastro-esophageal reflux (GER) (32.2%) and anastomotic leakage (22.7%) were the most common postoperative complications, with stricture and GER occurring more often after DPA (61.9% and 40.8% respectively) compared to other methods (p CONCLUSION: The majority of patients in this review were managed by DPA and postoperative complications were common despite the surgical method, with anastomotic stricture and GER being most common after DPA.LEVEL OF EVIDENCE: Systematic review of case series and case reports with no comparison group (level IV).

Introduction Esophageal atresia (EA) is associated with significant respiratory mortality. We aimed to assess incidence and predictive factors of EA associated pneumonia during the first 5 years of life. Materials and Methods Institutional ethical consent was obtained. Hospital records of patients with EA from 2002 to 2017 were reviewed. Episodes of pneumonia that were diagnosed in university or regional hospitals were included. For instance, respiratory infections other than pneumonia, anastomotic complications, aortopexy, fundoplication, major EA associated diseases, types of EA, and EA anastomosis dilatations were tested as potential risk factors for pneumonia. Results A total of 104 patients (56 males; type A 7, B 3, C 83, D 3, E 6, F 2) was included. Thirty-five (34%) patients had 94 episodes of pneumonia corresponding to median 2 (IQR [interquartile range]: 1–4) and 609 episodes per thousand patient years. Majority of pneumonias occurred before the age of 3 years. The cause of pneumonias could be identified as RS (respiratory syncytial) virus in 15(16%) and aspiration in seven (7.4%) episodes. In univariate analysis, pneumonia was predicted by occurrence and number of nonpneumonia respiratory infections, anastomotic reoperations, fundoplication, and number of EA anastomosis dilatations. In multivariate logistic regression analysis, significant risk factors for pneumonia were occurrence of nonpneumonia respiratory infections and number of anastomotic dilatations. Conclusion Episodes of pneumonia occurred in one-third of patients with EA. After the final repair, the incidence of pneumonia was highest during the first 3 years of life. Patients with other acute respiratory infections and high number of dilatations were at the greatest risk.

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 free margins 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. Level of Evidence IV (Treatment Study, Case Series with no Comparison Group).

Background Previous studies have reported an association among esophageal atresia (EA), Barrett's esophagus, and esophageal adenocarcinoma later in life. Objective The objective of the article is to evaluate KRAS and BRAF mutations as potential genetic markers for early detection of malignant transformation, we used an ultrasensitive technique to detect tissue expression of KRAS and BRAF mutations in endoscopic biopsies from 61 adult patients under follow-up after treatment for EA. Materials and Methods RNA was extracted from 112 fresh-frozen endoscopic tissue biopsies from 61 adult patients treated for EA in early childhood. RNA was reverse transcribed using the extendable blocking probe reverse transcription method. KRAS codons 12 and 13, as well as BRAF mutations were detected by quantitative polymerase chain reaction. Results No mutations of KRAS codon 12, KRAS codon 13, or BRAF were found in 112 endoscopic biopsy samples from 61 patients. Conclusion Despite the presence of histological findings indicating long-standing gastroesophageal reflux in 25%, as well as symptomatic gastroesophageal reflux in more than 40%, there was no detectable tissue expression of KRAS or BRAF mutations in this cohort of patients.

We analyzed retrospectively the outcomes in long gap Gross type C esophageal atresia. We hypothesized that outcomes in type C (long gap) atresia differ from type C (normal gap) and be similar with outcomes in Gross type A and B esophageal atresia. Location of the distal tracheoesophageal fistula (TEF) at the carina was chosen as the hallmark of type C atresia (long gap). We compared the type of esophageal repair, major reoperations for anastomotic complications and gastroesophageal reflux, and long-term mucosal changes between type C (normal gap), type C (long gap), and type A/B. We analyzed the hospital charts of 247 successive patients from 1984 to 2014 who either underwent repair of esophageal atresia in our institution (n = 232) or were referred from elsewhere because of anastomotic complications (n = 15). Among the 232 patients of our institution, 181 had type C and 21 type A or B esophageal atresia. Twenty-two (12%) of type C patients had TEF at the carina and were considered as type C (long gap). The referred patients included a disproportionately high number (42%) of patients with type C (long gap). We attempted primary anastomosis in 98% of patients with type C (normal gap), in 95% with type C (long gap), and 53% with type A/B underwent delayed primary anastomosis. Leakage after primary anastomosis occurred in 40% of patients with type A/B and in 23% with type C (long gap) compared with 6% in patients with type C (normal gap) (P < 0.05). Recalcitrant anastomotic stricture that eventually required esophageal resection occurred in 30% of patients with type A/B and in 18% with type C (long gap) compared with 3% in patients with type C (normal gap) (P < 0.05). The overall rate of major reoperations for anastomotic complications after primary anastomosis, type A/B (36%), type C (long gap) (27%), and antireflux surgery, type A/B (100%) and type C (long gap) (61%) were higher than in type C (normal gap), (9% and 24%), (P < 0.05 in both). Ten (47%) patients with type A/B esophageal atresia (primary anastomosis not possible n = 10), three (14%) with type C (long gap) (primary anastomosis not possible n = 1, significant loss of esophageal length after complications n = 2) and two (1%) with type C (normal gap) (significant loss of esophageal length after complications n = 2) underwent esophageal reconstruction. Endoscopic follow-up, median length 7.0 (IQR: 3.0-14) years, disclosed gastric metaplasia in 31% and 33% of patients with type A/B and type C (long gap) compared with 11% in type C (normal gap) (P < 0.05). Intestinal metaplasia was found in one patient type C (normal gap) (0.7%) and one with type C (long gap) (5.6%), (P = 0.21), only. The outcomes of type C (long gap) esophageal atresia are associated with more frequent complications, gastroesophageal reflux and esophageal mucosal changes than outcomes in type C (normal gap). Outcomes in type C (long gap) esophageal atresia resemble those in type A/B. The percentage of patients who remain with their native esophagus is, however, higher in type C (long gap) atresia (86%) than in type A/B (53%).

Purpose: Clinical and endoscopic assessment of the outcome after fundoplication for pediatric gastroesophageal reflux. Basic procedures: Hospital records of 279 consecutive patients who underwent fundoplication for gastroesophageal reflux from 1991 to 2014 were reviewed. Underlying disorders, clinical and endoscopic findings, imaging studies, pH monitoring, and surgical technique were assessed. Main outcome measures were patency of fundoplication, control of symptoms and esophagitis, complications, redo operations, and predictive factors of failures. Main results: A total of 279 patients underwent 300 fundoplications (277 primaries and 23 redos). Underlying disorders in 217 (72%) patients included neurological impairment (28%) and esophageal atresia (22%). Indications for fundoplication included recalcitrant gastroesophageal reflux symptoms (44%), failure to thrive (22%), respiratory symptoms (15%), esophageal anastomotic stricture (4%), apneic spells (2%), and regurgitation (2%). Preoperative endoscopy was performed in 92% and pH monitoring in 49% of patients. Median age at primary fundoplication was 2.2 ((IQR = 0.5–7.5)) years. Fundoplication was open in 205 (74%; Nissen n = 63, Boix-Ochoa n = 97, Toupet n = 39, and other n = 6), laparoscopic in 72 (24%; Nissen n = 67 and Toupet n = 5), and included hiatoplasty in 73%. Clinical follow-up was a median of 3.9 (IQR = 1.2–9.9) years. Mortality related to surgery was 0.3%. Symptom control was achieved in 87% of patients, and esophagitis rate decreased from 65% to 29% (p Conclusion: The majority of patients who underwent fundoplication had an underlying disorder. Primary fundoplication provided control of symptoms in almost 90% of patients and also reduced the rate of esophagitis. Failure of primary fundoplication occurred in 15% of patients, and an underlying disorder, esophageal atresia, and hiatoplasty increased the risk of failure.

Pediatric-onset intestinal failure (IF) remains a severe illness with life-threatening consequences. In this study, we analyzed a single center's outcomes of IF over 3 decades.All children with IF who required parenteral nutrition (PN)2 months or small-intestinal resection ≥50% managed since 1984 were included for retrospective outcome analyses.In total, 100 patients with median PN duration of 1.2 (interquartile range, 0.4-3.5) years were identified. Causes of IF were short bowel syndrome (SBS; n = 78), primary intestinal motility disorders (n = 14), and congenital intestinopathies (n = 8). Patients with SBS had median 40 (25-60) cm of small bowel remaining. Overall, Kaplan-Meier 5- and 10-year weaning-off estimates were 67% (95% CI, 57-77) and 73% (95% CI, 63-84), respectively. Weaning off PN was predicted by remaining bowel anatomy, multidisciplinary treatment era, and absence of immune deficiency. Catheter-related bloodstream infections decreased from 1.4 to 0.6/1000 PN days (P = .0003) with systematic use of taurolidine locks. None had progressive liver disease. Thirty-one percent of patients with SBS underwent autologous intestinal reconstructive surgery. Five patients received and 2 were listed for isolated intestinal transplantation. Eight patients died, and overall 15-year survival rate estimate was 91% (95% CI, 85-98).Despite reassuring rates of survival and weaning off PN, long-term PN failed in 14% of patients solely because of catheter complications in the recent era. Achievement of enteral autonomy in those with the shortest remaining small bowel and functional cause of IF remains challenging.

Duplications of the alimentary tract are cystic or tubular structures most often in close proximity with a section of the alimentary tract. The most common location is abdomen and the most common location is small intestine. Duplications possess a surrounding muscular layer, an inside mucosal layer, intrinsic nerves and peristalsis. Approximately 10% of duplications are multiple and there is a high prevalence of coexisting malformations of vertebral column, intestines and urinary tract and genitals. A diagnosed duplication should always warrant for search of others and the coexisting malformations. Duplications may contain heterotropic gastric or other kind of mucosa.

BACKGROUND: Different treatment policies can influence biliary atresia outcomes, but the pathophysiology of expanding fibrosis occurring even after successful portoenterostomy remains unclear. STUDY DESIGN: Clearance of jaundice (COJ) (bilirubin

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 aim of this study was to evaluate treatment and outcomes of infantile very short bowel syndrome (SBS).A retrospective review of 42 consecutive children treated for infantile onset SBS defined as remaining small bowel length less than 30% of predicted or more than 3 months of parenteral nutrition (PN) was performed. Surgical treatment and outcomes were compared between very SBS (VSBS, small bowel length less than 25 cm, n=12) and SBS (more than 25 cm, n=30).Median follow-up was 5.7 years (IQR, 2.8 to 11). Absolute initial small bowel length (cm), presence of ileocecal valve (%), and proportion of remaining colon (%) was 15 (10 to 21) vs. 48 (32 to 60) (P0.0001), 58 vs. 50 (P=0.74), and 95 (76 to 100) vs. 78 (60 to 100) (P=0.27) in VSBS and SBS, respectively. More autologous intestinal reconstruction procedures per patient were performed in SBS group (27/30 vs. 5/12; P=0.002) leading to intestinal autonomy in 2 of 4 VSBS patients in relation to 9 of 11 SBS patients (P=0.52). Cumulative 5-year probability of weaning from PN was 46% (95% CI, 16 to 77) in VSBS and 92% (95% CI, 81 to 100) in SBS (P0.01). Five-year cumulative survival was 80% (95% CI, 54 to 100) in VSBS and 93% (95% CI, 83 to 100) in SBS (P0.30). No patients were transplanted. At final follow-up, plasma alanine aminotransferase (29 U/L [21 to 47]), bilirubin (6.0 μmol/L [3.0 to 8.0]), height (-1.4 SD [-2.5 to 0.1]), and relative weight (-5% [-12 to -2]) were similar between the groups.Although survival, well-preserved biochemical liver function, and growth in VSBS patients are comparable to their counterparts with longer remaining bowel, regaining intestinal autonomy remains challenging in children with the shortest small intestinal remnant.

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.

Background/purpose Posterior sagittal anorectoplasty (PSARP) has become the standard surgical treatment for males rectourethral fistula (RUF) at most surgical centers worldwide. We aimed to define the long-term bowel functional outcomes following PSARP for RUF at our institution between 1983 and 2006, with comparison to age- and gender-matched controls. Methods Patients were invited to answer a detailed, previously validated Bowel Function Score (BFS) questionnaire by post. Respondents were matched by age and gender to three controls from the general population who had answered identical questionnaires. Case records were reviewed retrospectively for operative details. Ethical approval was obtained. Social continence was defined as soiling or fecal accidents Results Of 34 (79%) respondents (median age 19 (range, 4–29) years), 74% had voluntary bowel movements (VBMs), 24% were reliant on anterograde continence enema (ACE) washouts, and 1 patient had a colostomy. Impairment of bowel function was significantly higher in all aspects of fecal control among patients than controls ( p p ≤0.03). Thirty-one percent of patients with VBMs had constipation managed with diet or laxatives (vs 2% of controls, p =0.0002). Of patients with VBMs followed up for >12years (n=20), 50% were completely continent (vs 73% of controls; p =NS). Overall, 76% of respondents were socially continent with or without artificial means in the form of ACE washouts. By BFS score, 39% had a good functional outcome, 27% had a moderate outcome, 9% had a clearly poor score and 24% were living with an ACE. Conclusions Our results suggest that in the long-term, functional symptoms remain highly prevalent among patients treated for RUF with PSARP. However, the majority can be expected to achieve social continence, although for some this will require intervention with ACE bowel management. Approximately one third may report VBMs and complete continence.

Few reports on choledochal malformations (CMs) in European populations exist.The medical records of pediatric CM patients managed in our nationwide referral center for pediatric hepatobiliary surgery between 1976 and 2013 (n = 38; 71% females) were reviewed.Over follow-up time, the relative proportion of fusiform CMs increased significantly (p = 0.007) and the estimated total incidence rose from 1:128,000 to 1:38,000 (p = 0.017). Cystic CMs (42%) presented at younger age than fusiform CMs (47%) (0.8 vs. 4.6 years, p = 0.001). Two-thirds had abdominal pain and half were cholestatic at presentation. Pancreatitis had occurred in 16%. In addition to ultrasound, 71% underwent magnetic resonance cholangiopancreatography and 39% underwent endoscopic retrograde cholangiopancreatography. Median CM dilatation was 15 (10-28) mm and a 12 (9-13) mm long common pancreaticobiliary channel was confirmed in 61%, increasingly during recent years. Intrahepatic biliary tree was dilated in 19%, whereas main ducts were dilated in 50%. Apart from two operated in the 1970s and one with choledochocele, patients underwent resection of extrahepatic bile ducts with Roux-en-Y hepaticojejunostomy at age of 4.2 (0.8-9.2) years. Postoperative bile leakage and hemorrhage required reoperations in two. At last follow-up 4.8 (1.3-13.2) years postoperatively, none had dilated intrahepatic biliary ducts or elevated plasma bilirubin (5 [3-7] µmol/l). Single cholangitis episodes had occurred in two, whereas others were asymptomatic.CM incidence has increased significantly in Finland, being currently over threefold higher than previous estimates in the Western world would suggest. Removal of the extrahepatic biliary tree with hepaticojejunostomy for type I and IV CMs yielded excellent results.

Rectopexy is a simple treatment of persisting complete rectal prolapse (RP) or related functional disorders in children. The results of rectopexy have been encouraging with few complications. We describe the postoperative complications and outcome of rectopexy in our institution from 2002 to 2013. Ethical committee accepted the study. Hospital records of 27 successive patients (16 males), median age 7.2 (range 2.8–17) years, who underwent rectopexy (25 laparoscopic, 2 open) were reviewed. Indication for rectopexy included RP (n = 24), solitary rectal ulcer with enterocele (n = 2) and rectocele (n = 1). Nine patients (39 %) were healthy. In the remaining 14 patients, RP was secondary to anorectal malformation (n = 2), bladder exstrophy (n = 1), sacrococcygeal teratoma (n = 1) and myelomeningocele (n = 1) or associated with mental retardation (n = 8) and Asperger’s syndrome (n = 1). Five (18 %) patients had constipation. Unexpected postoperative events and complications were rated by Clavien–Dindo classification (Grades I–V). Seventeen (61 %) patients had postoperative complications (Grade I n = 5, II n = 2 and III n = 7). Readmission was required in 11 (41 %) and reoperation, endoscopy or other surgical procedure in 9 (33 %) patients. Complications included severe faecal obstruction (n = 2), constipation (n = 3), faecal soiling (n = 1) urinary retention (n = 2), enuresis (n = 1), infection (n = 2), residual mucosal prolapse (n = 5), discomfort at defecation (n = 1) and recurrent RP (n = 2). Reoperations included sigmoid resection with re-rectopexy (n = 1), resection of mucosal prolapse (n = 1), suprapubic urinary catheter (n = 2), evacuation of faecal impaction (n = 2), colonoscopy (n = 3), appendicostomy for antegrade continence enema (n = 1). Mental retardation or behavioural disorder increased the risk of postoperative faecal obstruction and constipation RR = 84 (95 % CI 4.3–1600), p = 0.0035. After median follow-up of 4.1 (range 0.6–11) years RP or related condition was cured in 26 patients. Constipation and faecal soiling require management in a total of seven patients. Long-term results of rectopexy were good. Postoperative complications from mild to moderate grade were unexpectedly frequent. Preoperative neurobehavioural disorder and constipation increase the risk of postoperative problems and should be mentioned in patient counselling.