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Background: Ganglioneuroblastoma intermixed (GNBi) and ganglioneuroma (GN) represent benign variants of peripheral neuroblastic tumours. While historically surgical resection was recommended, watchful active observation has become the accepted management for a subset of patients., Objectives: To review clinical features, biology, natural history and management of a retrospective UK CCLG study cohort of GN and GNBi, and compare outcomes of patients treated with surgical resection or watchful active observation., Methods: Retrospective review of histologically confirmed non-metastatic GN and GNBi diagnosed between 1990 and 2020., Results: A total of 259 patients were identified (163 GN, 93 GNBi, median age 62 months). In all 201 (78%) had initial surgery and 58 (22%) were observed. Overall survival was 98%. Twenty-one of 58 observed (36%) required subsequent surgery due to progressive tumour growth (52%). Gross total resection (GTR) was achieved in 79% of patients with a 19% complication rate. GTR was obtained in a similar proportion of patients having initial surgery (65%) and delayed surgery (76%). Patients obtaining GTR were more likely to have complete symptom(s) control (43% vs. 24%). In 45 cases (39%), surgical pathology was different from pathology at biopsy, and in 14 (12%) the pathology changed from GN/GNBi to neuroblastoma/GNB nodular., Conclusion: Watchful active observation can be a safe approach, with surgical resection reserved for symptomatic patients. However, a small proportion of patients in the observation group had their diagnosis revised to malignant at surgery. Careful assessment of surgical risk and expected benefits, after considering an initial period of observation, will allow clinicians to make optimal decisions for patients and their families., (© 2024 The Author(s). Pediatric Blood & Cancer published by Wiley Periodicals LLC.)

Aim  Pneumatosis intestinalis (PI) is a condition in which multiple gas-filled cysts form within the wall of the gastrointestinal tract in either the subserosa or submucosa. The presence of pneumoperitoneum in the presence of PI can present a therapeutic dilemma. The aim of our study was to review our experience and management of this condition. Methods  A single-center retrospective study of consecutive children (> 1 year) presenting with a pneumoperitoneum and evidence of PI (2009-2015). Demographics, case notes, microbiology, and imaging were reviewed. Results  Seven patients were identified (four males; age range 5-14 years). Four children had global developmental delay and were percutaneous endoscopic gastrostomy or jejunostomy fed, one was immunocompromised (acute lymphoblastic leukemia). The others had encephalitis and eosinophilic gastroenteritis. One patient proceeded to an exploratory laparotomy; no perforation was identified, pneumatosis of the colon was observed, and a loop ileostomy was formed. The remaining six were managed conservatively and made nil by mouth with intravenous antibiotics commenced. Five of the six had a computed tomography (CT) scan which revealed PI and free air with no other worrying signs. One died from nongastrointestinal causes, while the remaining five had feeds reintroduced uneventfully. Conclusion  Free air in the setting of PI may represent rupture of the gas cysts and not always transmural perforation. Surgery may not always be indicated and conservative management may suffice. A CT scan can be useful for excluding other intra-abdominal pathological findings and continued clinical assessment is essential., (Georg Thieme Verlag KG Stuttgart · New York.)

Purpose: In the UK, air enema reduction of intussusception is predominantly performed by consultant radiologists. At our institution, it is usually performed by a team involving radiology and surgical registrars. The purpose of this study was to assess who performs air enemas in the UK and the efficacy and safety of our registrar-led service., Methods: A telephone survey of paediatric surgical centres in the UK was performed asking who performs air enema in these institutions. Following this, a retrospective review of all air enemas at our institution between January 2005 and 2011 was performed. Cases were identified from radiology databases and reviewed for grade of radiologist, perforation and outcome., Results: At all 25 centres, consultant radiologists performed air enemas. At our institution 145 enemas were performed in 6 years. 141 were analysed (54 girls, 87 boys). Median age was 9 months (range 3-107 months). 82 % were performed by the registrar-only team. Over 6 years the registrar-led reduction rate was 77.5 % and in the last 3 years 84 %. The perforation rate was 0.9 %., Conclusion: A paediatric surgical registrar-led service for air enema reduction can be safe and effective ensuring a team approach is adopted, equipment is efficient and a strict protocol is applied.

The authors report a case of a neonate with gastroschisis, which, after repair, was further complicated by the diagnosis of Hirschsprung's disease. The authors discuss the diagnostic and management dilemmas posed by the coexistence of these two conditions for the clinician., (Copyright 2010 Elsevier Inc. All rights reserved.)

Perianal abscess (PA) is a common condition encountered in childhood, but its optimal primary treatment is uncertain. Treatment of PA by incision and drainage (I & D) alone is associated with an unacceptably high recurrence rate, either as fistula-in-ano (FIA) or as PA. To identify possible causes of recurrence and assess the value of concomitant laying open of a fistulous tract at the time of primary incision and drainage, the case notes of all children who presented to our institution with a PA between January 1992 and January 1997 were reviewed retrospectively. Thirty-three cases were identified (29 boys and 4 girls). A fistulous tract was identified and laid open at the time of primary drainage in 20 cases, whilst 13 were treated by I & D alone. Following primary drainage, there were 7 recurrences (21.2%) (FIA 5 and PA 2). All recurrences had been primarily treated by drainage alone, whilst there were no recurrences in patients who had also undergone fistulotomy at the time of primary drainage. Thus the primary treatment of PA in childhood should involve a careful search for a coexisting fistula and treatment of this by fistulotomy.

Background/purpose: With the improvement in long-term outcome from childhood malignancy, the expected quality of life is important. This study was to assess the value of reverse orchidopexy in patients requiring radiotherapy to the groin or pelvic area., Methods: The details of 6 boys (age 2 to 13 years) who had malignancy necessitating groin or pelvic radiotherapy were reviewed. Each had reverse orchidopexy of the contralateral testis for the course of the radiotherapy treatment. Brief case summaries are presented. The testis was replaced in the scrotum 4 to 12 months after the reverse orchidopexy., Results: The protected testis continued to grow and was considered normal at 1 to 7 years later. One individual had fathered a child., Conclusion: Reverse orchidopexy is a promising method of preserving testicular function in boys who require radiotherapy to the groin or pelvis area.

Hypercalcemia is an extremely rare paraneoplastic manifestation of malignancy in children. The authors report a case of dysgerminoma in a 14-year-old girl associated with severe hypercalcemia that only resolved after excision of the tumor. There are only 6 previously reported cases of dysgerminoma associated with hypercalcemia. Serum calcium levels should be checked in all children with solid ovarian tumors. Hypercalcemia in such circumstances may be resistant to medical management, and surgical resection of the tumor results in the normalization of calcium levels., (Copyright 2001 by W.B. Saunders Company.)

Background/purpose: Pyloric atresia is an uncommon condition occurring in 1 of 100,000 live births. When occurring in isolation, the clinical course usually is uncomplicated after surgical treatment. However, it may occur in association with other congenital abnormalities. The authors present 5 new cases, 3 of associated abnormalities including 1 of esophageal atresia and 2 of agenesis of the gall bladder and malrotation. Agenesis of the gall bladder has not been described previously in combination with pyloric atresia. The literature has been reviewed and guidelines are suggested for the management., Methods: The case records of 4 neonates who presented to the author's institution between January 1998 and June 1999 and 1 who presented at another center in 1991 were reviewed. A Medline literature search was performed, and guidelines were developed for the management of this condition based on our cases and the literature review., Results: Patients 1 and 5 had no associated anomalies. Patient 2 had associated esophageal atresia, tracheoesophageal fistula, atrial septal defect, crossed renal ectopia, malrotation, and absent gall bladder. Patient 3 had a rectovestibular fistula, vaginal atresia, atrial septal defect, malrotation absent gallbladder, and absent extrahepatic portal vein. Patient 4 had epidermolysis bullosa. Patients 2 and 5 had unremarkable recoveries, patients 2 and 3 had markedly delayed gastric emptying that responded to cisapride. Patient 3 has portal hypertension and remains under close follow-up. Patient 4 died at 22 days of age of pseudomonas sepsis., Conclusions: Based on our cases and literature review, we have adopted the following guidelines: (1) All children with pyloric atresia should be screened for multiple anomalies. (2) Delayed gastric emptying should be considered early and may respond to prokinetic agents. (3) Association with Epidermolysis bullosa should not preclude surgical treatment. (4) A skin biopsy specimen should be taken at the time of surgery for electron microscopy if there is a family history of epidermolysis bullosa.

Background/purpose: Newborns with congenital diaphragmatic hernia (CDH) frequently have associated anomalies that have a major impact on survival rate independent of pulmonary hypoplasia and pulmonary hypertension. Cardiovascular malformations (CVM) represent a major group of lethal extrapulmonary abnormalities that often assume greatest prognostic significance in most CDH studies. Animal models resembling human CDH may aid knowledge of the basic embryology that leads to the coexpression of CDH and CVM. This study, therefore, analyzed the incidence and spectrum of CVM in fetal rats with CDH., Methods: Left-sided CDH (LCDH) was induced in fetal rats by the maternal administration of 100 mg of nitrofen by gavage on day 9.5 gestation (term, day 22). Control animals received olive oil (OO) and were used for comparative analysis. Fetal rats were harvested by cesarean section on day 21.5 or day 22, histologically processed and examined for CVM., Results: A significant number of CVM were observed in 15 of 60 (25%) LCDH rats compared with 4 of 60 (6.7%) nitrofen non-CDH rats (P = .01). The spectrum of abnormalities in CDH included ventricular septal (VSD) defects (n = 6), vascular rings (n = 4), anomalous subclavian arteries (n = 3), atrioventricular septal defects (n = 1) and Fallot's tetralogy (n = 1). A VSD (n = 1), double-outlet right ventricle VSD (n = 1) and Fallot's tetralogy (n = 2) were noted in nitrofen non-CDH rats. Control (OO) fetal rats (n = 60) displayed no malformations., Conclusions: These results confirm a significant incidence and spectrum of CVM in a teratogenic CDH model similar to that seen in humans with CDH. The findings of this study reinforce the validity of the nitrofen model as a research tool to uncover the genetic and molecular mechanisms responsible for the genesis of CDH and allied malformations.

The high mortality associated with congenital diaphragmatic hernia (CDH) is due to pulmonary hypoplasia and hypertension, structural and functional abnormalities which can to some extent be ameliorated by prenatal administration of glucocorticoids. In the hypoplastic, hypertensive lungs of neonatal rats in which CDH has been induced by nitrofen, those pulmonary neuroendocrine cells (PNCs) containing calcitonin gene-related peptide (CGRP) increase in number, and it has been suggested that this might be due to inhibition of secretion of the peptide, the consequent decrease in its vasodilatory effects contributing to the hypertension. Whether this increase affects the entire population of PNCs, however, and how these cells are affected by administration of prenatal glucocorticoids, is unknown. As revealed by immunolabelling for protein gene product (PGP) 9.5, a general marker of NCs and expressed per cm2 tissue section, the total PNC population in rats with nitrofen-induced CDH was significantly greater than in controls receiving only olive oil (672 vs 375/cm2, P = 0.03) and was further increased (824 per cm2) in animals treated prenatally with dexamethasone (n = 8 in all groups). The increase in the total PNC population in rats with CDH is similar in magnitude to that described for the CGRP-containing subpopulation. Since the major role of the products of PNCs is now thought to be the regulation of development of pulmonary tissues and their response to injury, it is probable that the expansion of their population in the abnormal lungs associated with CDH is an adaptive response to pulmonary maldevelopment, a response possibly augmented by exogenous corticosteroids.

Background: Intra-abdominal abscess remains a significant cause of morbidity following appendicectomy. In children, little emphasis has been placed on the non-invasive management of this complication using antibiotic therapy alone. This study reviews the experience of a paediatric surgical department in managing abscess arising after appendicectomy., Methods: Hospital records of all children undergoing appendicectomy between January 1992 and January 1997 were reviewed retrospectively., Results: Some 1024 children underwent appendicectomy over a 5-year period. Twenty three patients (2.2 per cent) developed abscesses after appendicectomy. Non-operative management with intravenous or oral antibiotics alone was successful in 21 patients, with complete clinical and radiological resolution of the abscess. Drainage of the septic collection was performed in only two patients: by laparotomy (n=1) and by the transrectal route (n=1)., Conclusion: Antibiotic therapy alone is an efficacious and safe first-line treatment modality in children who develop this complication. Drainage of an abscess developing after appendicectomy is rarely necessary in children.

Aims: To study the effect of prenatal glucocorticoid treatment on endothelial nitric oxide synthase (eNOS) expression in rats with congenital diaphragmatic hernia (CDH)., Methods: CDH was induced in fetal rats by the maternal administration of nitrofen on day 9.5 of gestation. Dexamethasone was administered on days 18.5 and 19.5 before delivery of the fetuses on days 20.5 and 21.5. Pulmonary eNOS protein expression was studied by western immunoblotting and immunohistochemistry., Results: On day 20.5, eNOS expression was significantly reduced in CDH pups compared with normal control rats. Dexamethasone treated CDH pups had eNOS concentrations equivalent to those of normal animals. By day 21.5, however, there was no detectable difference in eNOS expression between the experimental groups., Conclusions: eNOS is deficient in near term (day 20.5) CDH rats. Dexamethasone restores eNOS expression in these animals to that seen in normal rat lungs. At term, the precise role of eNOS in the pathophysiology of CDH remains uncertain.

Background/purpose: Neonates with congenital diaphragmatic hernia (CDH) have a high morbidity and mortality rate caused by pulmonary hypoplasia associated with pulmonary hypertension (PH). In experimental CDH, antenatal glucocorticoid therapy improves surfactant biochemical immaturity, enhances lung compliance, and induces morphological maturation in CDH rats. The effects of steroid therapy on preventing or treating PH in this condition have not been studied. Angiotensin converting enzyme (ACE), which is produced by the vascular endothelium, is implicated in the pathogenesis of pulmonary hypertension. The aim of this study was to evaluate the effect of antenatal glucocorticoid therapy on ACE activity and expression in CDH rat lungs., Methods: CDH was induced in fetal rats by the maternal administration of 100 mg nitrofen on day 9.5 of gestation (term, day 22). Dexamethasone (Dex) (0.25 mg/kg) was given by intraperitoneal injection on days 18.5 and 19.5 before delivery of the fetuses by cesarean section on day 21.5. Control animals received olive oil (OO) by gavage and normal saline (NS) as vehicle injection. ACE activity was measured spectrophotometrically in the lungs of rats from four treatment groups: CDH-NS, non-CDH-NS, CDH-Dex, and OO-NS controls. Total lung ACE activity (mU per lung) was significantly lower in CDH-NS (P = .002) and CDH-Dex (P = .004) rats compared with non-CDH-NS and OO-NS controls (9.1 +/- 1.0 and 10.7 +/- 1.3 v 16.2 +/- 1.6 and 15.4 +/- 1.7). When specific ACE activity (mU/mg protein) was derived by expressing ACE activity per milligram of lung protein, CDH-NS animals showed elevated specific ACE activity (P = .05) compared with OO-NS controls (6.31 +/- 1.1 v 4.4 +/- 0.4). CDH-Dex animals had a significantly lower specific ACE activity (P = .01) compared with CDH-NS and Non-CDH-NS rats (4.0 +/- 0.4 v 6.31 +/- 1.1 and 5.83 +/- 0.54). The specific ACE activity levels of CDH-Dex rats were equivalent to those seen in the lungs of OO-NS controls (P = .24)., Conclusion: Antenatal steroid therapy, by suppressing pulmonary ACE activity, may reduce the risk of pulmonary hypertension developing in human newborns with antenatally diagnosed CDH.

Background/purpose: Pulmonary hypertension (PH) contributes significantly to the mortality of congenital diaphragmatic hernia (CDH). Pulmonary vascular changes in CDH include a reduced vascular bed with increased arterial medial wall thickness and peripheral extension of muscle into intraacinar vessels. Antenatal steroids improve biochemical immaturity, lung compliance, and morphology in experimental CDH animals. The aim of this study was to examine the effects of prenatal glucocorticoid therapy on pulmonary artery muscularisation in CDH rats., Methods: CDH was induced in fetal rats by the maternal administration of 100 mg of nitrofen by gavage on day 9.5 of gestation (term, day 22). Control animals received olive oil (OO). Dexamethasone (Dex, 0.25 mg/kg) or normal saline (NS) was given by intraperitoneal injection on days 18.5 and 19.5, and fetuses were delivered by caesarean section on day 21.5. Lung sections from five fetuses in each of four experimental groups were studied by a blinded investigator- OO-NS controls, CDH-NS, CDH-Dex, and non-CDH-NS. The external diameter (ED), medial wall thickness (MT), percent of medial wall thickness, and wall structure were evaluated from preacinar arteries accompanying conducting airways, and the intraacinar arterioles associated with the respiratory bronchi and saccules., Results: In the preacinar arteries, CDH-NS animals had a significantly increased MT percentage compared with OO-NS controls (21.2+/-8.8 v 17.8+/-10.3, P = .0001). CDH-Dex rats had a lower MT percentage than CDH-NS rats (15.5+/-6.7 v 21.2+/-8.8, P = .0001). In the intraacinar region, CDH-Dex fetuses had a reduced percentage of muscularised intraacinar blood vessels compared with CDH-NS and OO-NS controls (10% v 24% and 28%, respectively, P = .01). Dexamethasone-treated CDH pups also displayed a significantly lower MT percentage of the intraacinar arteries compared with CDH-NS and OO-NS animals (15.7+/-13 v 23.4+/-9 and 25.4+/-12, P = .003)., Conclusions: Medial hypertrophy is present in the preacinar but not the intraacinar blood vessels of CDH rats before birth. Dexamethasone inhibits medial hypertrophy and reduces the number of muscularised intraacinar vessels. Antenatal glucocorticoids may reduce the risk of PH developing in human newborns with antenatally diagnosed CDH.

Air guns are commonly used for sporting entertainment. The inappropriate use of these weapons often leads to injury. In general, trauma inflicted by air weapons is trivial. However, the potential for more serious and fatal injuries is significant. We report two cases of serious air gun injury in children and a review of the relevant legislation covering the use of air weapons in the UK. Enhanced public education and awareness as well as coordinated policies by organizations involved in the care of children is desirable to prevent future calamities involving air weapons.

Introduction: Management of liver trauma in childhood represents a rare but formidable challenge., Methods: Clinical presentation, grade of liver injury and Injury Severity Score (ISS) were studied in 11 cases of blunt liver trauma to examine factors influencing outcome., Results: Seven of the 11 children were injured severely and had an ISS greater than 16. Seven who were haemodynamically stable were treated without operation, but four required surgery for grade III, IV and V liver injuries. Two children had primary repair of hepatic lacerations. Perihepatic packing was employed in two other cases (grade IV and V injury) for uncontrollable haemorrhage. Delayed debridement and thrombectomy plus vena cava repair with suturing of liver lacerations in these patients obviated heroic efforts at primary repair. Nine children survived. There were two deaths from head and neck trauma., Discussion: Selected children with liver trauma can be managed non-operatively using established trauma guidelines. Perihepatic packing is recommended in unstable patients with complex injuries, followed by delayed definitive repair.

The asplenia [Ivemark] syndrome (AS) is the association of congenital absence of the spleen with a variety of visceral abnormalities, predominantly of the cardiovascular system. Varying degrees of malrotation and malfixation of the bowel are common in this condition, and the occurrence of catastrophic gastric volvulus due to malfixation of the bowel has been reported. With the improvement in long-term outlook for these patients with modern cardiac surgery and prophylactic antibiotics, the intra-abdominal anomalies have become increasingly significant. This paper draws attention to the prophylactic treatment of gastric malfixation in the AS through the presentation of two cases in which gastropexy was performed.