Bowel Control, Bladder Function, and Quality of Life in Children with Cloacal Malformations.

Introduction: Long-term outcomes of cloacal malformations remain unclear. We evaluated postoperative bowel control, bladder function and quality of life in patients under 18 years of age with cloaca.

Materials And Methods: This was a multi-center cross-sectional observational study accomplished by the Nordic Pediatric Surgery Research Consortium.

Long-term outcomes in newborn surgery.

This article describes the common methods to study long-term outcomes in patients who have undergone major surgery in newborn period. It also sums up today's knowledge on the long-term outcome of some classic newborn surgical conditions. The analysis of long-term outcomes is important to pediatric surgeons.

Lessons learned from lower urinary tract complications of anorectoplasty for imperforate anus with rectourethral/rectovesical fistula: Laparoscopy-assisted versus posterior sagittal approaches.

Purpose: To report the sequelae of and preventive strategies for selected lower urinary tract (LUT) complications, i.e., posterior urethral diverticulum (PUD), intraoperative LUT injuries, postoperative dysuria, and fistula recurrence in male imperforate anus (IA) with rectourethral/rectovesical (RU/RV) fistula after laparoscopy-assisted anorectoplasty (LAARP) or posterior sagittal anorectoplasty (PSARP).

Intra-articular venous malformation of the knee in children: magnetic resonance imaging findings and significance of synovial involvement.

Background: Intra-articular venous malformations of the knee are an uncommon cause of unilateral knee pain in children. Timely diagnosis is important because lesions with intrasynovial involvement can lead to joint space hemorrhage and secondary cartilage damage.

Objective: To describe our tertiary center's experience of diagnostics and typical magnetic resonance imaging (MRI) findings.

Upper endoscopy for non-acute non-specific symptoms is seldom beneficial for children under the age of seven.

Aim: This study estimated the diagnostic yield of oesophagogastroduodenoscopy (OGD) in young children with non-acute, non-specific gastrointestinal or respiratory symptoms who were treated by a Finnish tertiary level referral centre.

Methods: A retrospective chart analysis was performed on 1850 Finnish children under 7 years of age who underwent their first diagnostic OGDs at Helsinki University Hospital during 2006-2016. We noted the endoscopy indications, macroscopic findings, the histology of the mucosal biopsies and the follow-up data.

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.

Medium and long-term outcomes of gastroschisis.

As survival of gastroschisis patients has improved significantly, it has become apparent that longitudinal follow up strategies need to be developed. Problems concerning patients with gastroschisis are usually associated with gastrointestinal morbidity, but there is mounting evidence that also neurodevelopmental, cognitive, behavioral and late-onset auditory sequelae exist. The presence of associated anomalies, as well as complex features (bowel atresia, necrosis, volvulus, perforation) increase morbidity and impact long-term outcomes.

Long-term Single-centre Outcomes After Proctocolectomy With Ileoanal Anastomosis for Paediatric Ulcerative Colitis.

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.

Noncoding RET variants explain the strong association with Hirschsprung disease in patients without rare coding sequence variant.

The pathogenesis of Hirschsprung disease is complex. Although the RET proto-oncogene is the most frequently affected gene in Hirschsprung disease, rare coding sequence variants explain only a small part of Hirschsprung disease cases. We aimed to assess the genetic background of Hirschsprung disease using a genome-wide association analysis combined with sequencing all RET exons in samples from 105 Hirschsprung disease cases (30 familial and 75 sporadic) and 386 controls.

Congenital diaphragmatic hernia: a scientometric analysis of the global research activity and collaborative networks.

Despite a growing interest to clinicians and scientists, there is no comprehensive study that examines the global research activity on congenital diaphragmatic hernia (CDH). A search strategy for the Web of Science™ database was designed to identify scientific CDH publications. Research output of countries, institutions, individual authors, and collaborative networks was analyzed.

Location of TEF at the carina as an indicator of long-gap C-type esophageal atresia.

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).

Pediatric Intestinal Failure: The Key Outcomes for the First 100 Patients Treated in a National Tertiary Referral Center During 1984-2017.

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.

Intestinal Microbiota in Hirschsprung Disease.

Objectives: The aim of the study was to characterize the microbiota profiles of patients with Hirschsprung disease (HD) and to evaluate this in relation to postoperative bowel function and the incidence of Hirschsprung-associated enterocolitis (HAEC).

Methods: All patients operated on for HD at our center between 1987 and 2011 were invited to answer questionnaires on bowel function and to participate in a clinical follow-up for laboratory investigations, including fecal DNA extraction, fecal calprotectin (FC), and brush border lactase (LCT) genotyping. The microbiota compositions of patients with HD were compared with those of healthy controls aged between 2 and 7 years.

Appendectomy versus non-operative treatment for acute uncomplicated appendicitis in children: study protocol for a multicentre, open-label, non-inferiority, randomised controlled trial.

Background: Appendectomy is considered the gold standard treatment for acute appendicitis. Recently the need for surgery has been challenged in both adults and children. In children there is growing clinician, patient and parental interest in non-operative treatment of acute appendicitis with antibiotics as opposed to surgery.

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.

Congenital diaphragmatic hernia with heart defect has a high risk for hypoplastic left heart syndrome and major extra-cardiac malformations: 10-year national cohort from Finland.

Introduction: Congenital diaphragmatic hernia (CDH) has a well-known risk of congenital heart defects with poor prognosis. This study was conducted to determine the national total prevalence and prenatal detection rates of CDH with heart defects and its association with major extra-cardiac malformations and to further evaluate the impact of the heart defect severity on survival.

Material And Methods: A 10-year national cohort was derived from four national registries, including live births, stillbirths, and terminations of pregnancy for fetal anomalies.

Long-term bowel function, quality of life and sexual function in patients with anorectal malformations treated during the PSARP era.

Anorectal malformations are an important group of congenital anomalies that vary widely in their anatomical characteristics and complexity. Understanding the long-term functional outcomes after modern treatments, and how these compare to the general population, are essential for ensuring that patients receive optimal, evidence-based care. With increasing appreciation of the wider impact of the illness on patients and their families, minimizing social disability from fecal incontinence and enabling normal social integration from the outset are key management concerns.

Social Morbidity in Relation to Bowel Functional Outcomes and Quality of Life in Anorectal Malformations and Hirschsprung's Disease.

Background:  Anorectal malformations (ARMs) and Hirschsprung's disease (HD) are chronic bowel conditions associated with varying degrees of fecal incontinence. We aimed to discuss the contemporary status of social morbidity associated with ARMs and HD in the long term after contemporary treatments.

Materials And Methods:  The bowel functional outcomes of our recent institutional series up to adulthood were reviewed and compared for ARMs and HD.

No Tissue Expression of KRAS or BRAF Mutations in 61 Adult Patients Treated for Esophageal Atresia in Early Childhood.

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.

Current Management of Congenital Pulmonary Airway Malformations: A "European Pediatric Surgeons' Association" Survey.

Aim:  To define current management of congenital pulmonary airway malformation (CPAM).

Methods:  A total of 181 European Pediatric Surgeons' Association members (91% senior) from 48 countries completed an online questionnaire.

Main Results:  Prenatal: 93% respondents work in centers with prenatal diagnosis facilities, and 27% in centers offering in utero surgery.

Fundoplication in Patients with Esophageal Atresia: Patient Selection, Indications, and Outcomes.

Patients with esophageal atresia (EA) suffer from abnormal and permanent esophageal intrinsic and extrinsic innervation that affects severely esophageal motility. The repair of EA also results in esophageal shortening that affects distal esophageal sphincter mechanism. Consequently, gastroesophageal reflux (GER) is common in these patients, overall approximately half of them suffer from symptomatic reflux.

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.