Variation in Glycemic Outcomes in Focal Forms of Congenital Hyperinsulinism-The UK Perspective.

Context: In focal congenital hyperinsulinism (CHI), localized clonal expansion of pancreatic β-cells causes excess insulin secretion and severe hypoglycemia. Surgery is curative, but not all lesions are amenable to surgery.

Objective: We describe surgical and nonsurgical outcomes of focal CHI in a national cohort.

Standardizing the surgical management of benign ovarian tumors in children and adolescents: A best practice Delphi consensus statement.

Aim: No widely agreed consensus protocols exist for the management of benign ovarian tumors (BOT) in children. This presents a substantial risk for suboptimal management. We aimed to generate multispecialty consensus guidance to standardize surgical management and provide a clear follow-up protocol for children with BOTs.

Case report: contradictory genetics and imaging in focal congenital hyperinsulinism reinforces the need for pancreatic biopsy.

Background: Congenital Hyperinsulinism (CHI) is an important cause of severe hypoglycaemia in infancy due to excessive, dysregulated insulin secretion. In focal CHI, a localised lesion within the pancreas hypersecretes insulin and, importantly, hypoglycaemia resolution is possible through limited surgical resection of the lesion. Diagnosis of focal CHI is based on a crucial combination of compatible genetics and specialised imaging.

Operative management of pediatric ovarian tumors and the challenge of fertility-preservation: Results from the UK CCLG Surgeons Cancer Group Nationwide Study.

Background: Ovarian tumors in the pediatric age group are rare. A significant number of children with ovarian mass lesions present "out of hours "as surgical emergencies, and surgical management does not always involve a surgical oncologist. This multicenter study reports how the mode of clinical presentation may influence (i) operation (conventional open vs minimally invasive surgery (MIS)) and (ii) examines if young females presenting as surgical emergency(s) are more likely to undergo total oophorectomy or ovarian sparing surgery.

Outcome and prognostic factors in pediatric malignant peripheral nerve sheath tumors: An analysis of the European Pediatric Soft Tissue Sarcoma Group (EpSSG) NRSTS-2005 prospective study.

Background: Malignant peripheral nerve sheath tumors (MPNST) are rare tumors of childhood. The role of standard chemotherapy in unresectable MPNST is still unclear. We report the outcome and prognostic factors in the EpSSG risk-adapted prospective study for localized pediatric MPNST.

Paediatric Horner's syndrome: is investigation for underlying malignancy always required?

Objective: Horner's syndrome (HS) is characterised by a triad of ocular miosis, ptosis and anhidrosis. HS may be a subtle sign of occult pathology in otherwise asymptomatic children, neuroblastoma (NBL) being the the most common associated malignant tumour. Despite such knowledge, the incidence of underlying malignancy in children with HS remains unclear and robust evidence to guide best clinical practice is sparse.

Transanastomotic tubes reduce the cost of nutritional support in neonates with congenital duodenal obstruction.

Purpose: To determine the impact of intra-operative Trans-anastomotic Tube (TAT) placement on the cost of post-operative nutrition in infants with congenital duodenal obstruction (CDO).

Methods: A retrospective analysis of patients undergoing corrective surgery for CDO, with birth-weight over 1.5 kg over a 10-year period.

Clinical Diversity in Focal Congenital Hyperinsulinism in Infancy Correlates With Histological Heterogeneity of Islet Cell Lesions.

Congenital Hyperinsulinism (CHI) is an important cause of severe and persistent hypoglycaemia in infancy and childhood. The focal form (CHI-F) of CHI can be potentially cured by pancreatic lesionectomy. While diagnostic characteristics of CHI-F pancreatic histopathology are well-recognized, correlation with clinical phenotype has not been established.

Manchester Arena bombing: lessons learnt from a mass casualty incident.

On 22 May 2017 Salman Abedi detonated an improvised explosive device in the Manchester Arena resulting in 23 deaths (including the attacker). This was the deadliest terrorist attack on UK soil since the 2005 London bombings, but was only one of five mass casualty terrorist attacks in the UK in 2017. Preparation for mass casualty incidents (MCI) is obligatory, involving such methods as multiagency tabletop exercises, mock hospital exercises, as well as simulation and training for clinicians in managing the injuries that would be anticipated in such an event.

Controversies in the management of ovarian tumours in prepubertal children - A BAPS and UK CCLG Surgeons Cancer Group National Survey.

Background: No clear treatment and follow-up protocols have been established for prepubertal patients with ovarian tumours. The lack of adequate prospective data in the literature includes all aspects of their management. A significant number of children with ovarian masses present out of hours as a surgical emergency and are initially managed by paediatric surgeons without special interest in surgical oncology.

Mortality and morbidity in oesophageal atresia.

Background: Several classification systems exist to predict mortality in oesophageal atresia, the most widely quoted of these being over 20 years old. No classification system exists to predict morbidity. We sought to test whether these classification systems remain relevant and to determine whether they can be useful to predict morbidity.

Atypical Forms of Congenital Hyperinsulinism in Infancy Are Associated With Mosaic Patterns of Immature Islet Cells.

Objectives: We aimed to characterize mosaic populations of pancreatic islet cells from patients with atypical congenital hyperinsulinism in infancy (CHI-A) and the expression profile of NKX2.2, a key transcription factor expressed in β-cells but suppressed in δ-cells in the mature pancreas.

Patients/methods: Tissue was isolated from three patients with CHI-A following subtotal pancreatectomy.

Enhanced Islet Cell Nucleomegaly Defines Diffuse Congenital Hyperinsulinism in Infancy but Not Other Forms of the Disease.

Objectives: To quantify islet cell nucleomegaly in controls and tissues obtained from patients with congenital hyperinsulinism in infancy (CHI) and to examine the association of nucleomegaly with proliferation.

Methods: High-content analysis of histologic sections and serial block-face scanning electron microscopy were used to quantify nucleomegaly.

Results: Enlarged islet cell nuclear areas were 4.

Non-cross-linked porcine acellular dermal matrix (Strattice Tissue Matrix) in pediatric reconstructive surgery.

Background: A variety of prosthetic materials are used in the pediatric population for abdominal and chest wall reconstruction. Pediatric experience of non-cross-linked porcine acellular dermal matrix is limited to patients following liver transplantation. We review our outcomes in patients in whom this matrix was used.

Nematode infection mimicking paratesticular malignancy.

Paratesticular swellings pose a diagnostic dilemma due to concerns over malignancy. We present a case of paratesticular swelling in a 13-year-old boy as a result of Dirofilaria immitis infection. The boy presented with a 2-month history of right testicular discomfort associated with an irregular mass within the scrotum.

Granular hemostat deposits mimicking disseminated malignancy.

Hemostatic matrices are a part of the surgeon's armamentarium against profuse intraoperative bleeding. Granular deposits may form after the use of a liquid hemostat which can be mistaken for tumor recurrence or metastatic disease in the setting of neoplasia. We present two cases that highlight the importance of full knowledge of product usage during previous operations and the need for histological examination of these lesions.

Laparoscopic orchidopexy in boys with prune belly syndrome--outcome and technical considerations.

Background And Purpose: Cryptorchidism is an ubiquitous feature in prune belly syndrome (PBS). Laparoscopic orchidopexy allows dissection of the spermatic cord with minimal morbidity. We discuss the technical difficulties and outcome of three boys with PBS who underwent two-stage laparoscopic Fowler-Stephens orchidopexy (F-SO).

Laparostomy closure in stage 4S neuroblastoma.

Laparostomy in children is a rare procedure, and there is little in the literature as to the best method for closure in children with an open abdomen. We describe 2 cases with stage 4S neuroblastoma that required emergency decompressive laparostomy. Both cases highlight advantages and disadvantages with known techniques for achieving skin and fascia closure.

Negative exploration for pyloric stenosis--is it preventable?

Background: The diagnosis of infantile hypertrophic pyloric stenosis (IHPS), although traditionally clinical, is now increasingly dependent on radiological corroboration. The rate of negative exploration in IHPS has been reported as 4%. The purpose of our study was to look at elements of supportive clinical evidence leading to positive diagnosis, and to review these with respect to misdiagnosed cases undergoing negative exploration.

Primary pull-through for Hirschsprung's disease: comparison of open and laparoscopic-assisted procedures.

Rectosigmoid Hirschsprung's disease is usually amenable to minimally invasive primary neonatal pull-through. This may be performed either entirely transanally or with laparoscopic assistance for biopsies with or without colonic mobilization. In our center, all dissection is performed transanally; laparoscopy is used for obtaining colonic biopsies and orientation of the pulled-through bowel segment.

Early adult outcome of the Duhamel procedure for left-sided Hirschsprung disease--a prospective serial assessment study.

Purpose: To assess both early adult functional outcome and change in long-term functional outcome over time after the Duhamel procedure (DP) for left-sided Hirschsprung disease (HSCR).

Methods: The study population consisted of 78 children (aged 19.9 +/- 3.

Umbilical pyloromyotomy: comparison of vertical linea alba and transverse muscle cutting incisions.

Background: Pyloromyotomy is commonly performed through a supraumbilical skinfold incision. Entry into the peritoneal cavity can be achieved via a vertical linea alba incision or a transverse muscle cutting approach. The aim of this study was to compare the morbidity associated with these 2 operative techniques.