John Gillick

Balanitis xerotica obliterans in children and adolescents: A literature review and clinical series

OBJECTIVE Balanitis xerotica obliterans (BXO) is a chronic inflammatory disease that is considered as male genital variant lichen sclerosis. The incidence varies greatly in different series; diagnosis is mostly clinical but histopathological confirmation is mandatory. Various treatments are described, but there is no consensus that one is the best. MATERIALS AND METHODS A literature review was made of BXO and lichen sclerosis in boys under 18 years of age, between 1995 and 2013, analyzing demographic dates, treatments and outcomes. In addition to that, we reviewed BXO cases treated in our centers in the last 10 years. RESULTS After literature review, only 13 articles matched the inclusion criteria. Analyzing those selected, the global incidence of BXO is nearly 35% among circumcised children. Described symptoms are diverse and the low index of clinical suspicion is highlighted. The main treatment is circumcision, with use of topical and intralesional steroids and immunosuppressive agents. CONCLUSION BXO is a condition more common than we believe and we must be vigilant to find greater number of diagnoses to avoid future complications. The main treatment for BXO is circumcision, but as topical or intralesional treatments are now available with potentially good outcomes, they may be considered as coadjuvants.

The Incidence of Symptomatic Malrotation Post Gastroschisis Repair

UNLABELLED Gastroschisis is known to be associated with abnormal bowel rotation. Currently, the broadly accepted practice is not to perform Ladds procedure routinely at the time of closure of gastroschisis defects. However the incidence of symptomatic malrotation and volvulus post gastroschisis repair is unknown; this incidence is important in view of the current practice of bedside gastroschisis closure. This study examined the incidence of symptomatic malrotation and volvulus following gastroschisis repair. METHOD Patients who had undergone gastroschisis repair between 1999 and 2009 in any of 2 tertiary centers were identified using the Hospital Inpatient Enquiry system. The medical records were reviewed to obtain demographic data and postoperative outcomes. Patients were contacted for follow-up. RESULTS 128 patients were identified with a median postoperative follow-up of 4 years (range: 6 weeks to 12 years). Upper gastrointestinal (GI) contrast studies were performed in 30 patients (23.4%), 21 (16.4%) of whom showed evidence of malrotation. Malrotation was documented during the primary repair in 12 patients (9.4%); however Ladds procedure was performed primarily in only 3 patients. 7 patients underwent Ladds procedure in a second laparotomy for mechanical obstruction secondary to causes not related to malrotation and volvulus. A total of 29 patients (22.7%) had either operative or radiological evidence of malrotation. None of these patients developed volvulus after being followed for a median period of 4 years. CONCLUSION Bedside gastroschisis closure without concomitant Ladds procedure is a safe practice. None of the patients with documented malrotation developed volvulus post gastroschisis repair.

Radiological Versus Clinical Evidence of Malrotation, a Tortuous Tale—10-Year Review

INTRODUCTION Malrotation is a common abnormality, often diagnosed in the neonatal period. Symptoms may be nonspecific and clinical signs of volvulus are often only seen in the late stages when there has been significant ischemic insult to the bowel. The gold standard diagnostic investigation is the upper gastrointestinal (UGI) contrast study. This study was designed to assess the incidence of negative laparotomy in patients with malrotation diagnosed on UGI contrast study and to identify the clinical signs and symptoms at presentation. METHODS A retrospective review of patients who underwent laparotomy for malrotation, over a 10-year period (2001 to 2010) was performed. Inclusion criteria were patients<16 years of age with a diagnosis of malrotation on preoperative UGI contrast study. RESULTS A total of 72 patients were reviewed. UGI contrast study diagnosed 43(60%) cases of malrotation without volvulus, and 29(40%) cases of malrotation with volvulus. Laparotomy revealed that 39(54.2%) patients had malrotation without volvulus, 27(37.5%) had malrotation with volvulus, and 6(8.3%) had no evidence of malrotation. A total of 13(18%) patients had an incorrect diagnosis on UGI contrast study; 6(8.3%) had normal anatomy, 3(4.2%) diagnosed with malrotation without volvulus, had volvulus at laparotomy, and 4(5.5%) diagnosed with malrotation and volvulus had no volvulus at laparotomy. CONCLUSION UGI contrast study can occasionally be misleading. In the above study, we have shown that there is a significant rate of negative laparotomy following diagnosis of malrotation on UGI contrast study. Therefore, we would advocate that all parents of patients undergoing laparotomy for malrotation should be informed of the risk of negative laparotomy as part of the consent process.

Altered neurotransmitter expression profile in the ganglionic bowel in Hirschsprung's disease

PURPOSE Despite having optimal pull-through (PT) surgery for Hirschsprungs disease (HSCR), many patients experience persistent bowel symptoms with no mechanical/histopathological cause. Murine models of HSCR suggest that expression of key neurotransmitters is unbalanced proximal to the aganglionic colonic segment. We aimed to investigate expression of key enteric neurotransmitters in the colon of children with HSCR. METHODS Full-length PT specimens were collected fresh from children with HSCR (n=10). Control specimens were collected at colostomy closure from children with anorectal malformation (n=8). The distributions of neuronal nitric oxide synthase (nNOS), choline acetyltransferase (ChAT), vasoactive intestinal peptide (VIP), and substance P (SP) were evaluated using immunofluorescence and confocal microscopy. Neurotransmitter quantification was with Western blot analysis. RESULTS ChAT expression was high in aganglionic bowel and transition zone but reduced in ganglionic bowel in HSCR relative to controls. Conversely, nNOS expression was markedly reduced in aganglionic bowel but high in ganglionic bowel in HSCR relative to controls. VIP expression was similar in ganglionic HSCR and control colon. SP expression was similar in all tissue types. CONCLUSION Imbalance of key excitatory and inhibitory neurotransmitters in the ganglionic bowel in HSCR may explain the basis of bowel dysmotility after an optimal pull-through operation in some patients.

Altered tryptophan hydroxylase 2 expression in enteric serotonergic nerves in Hirschsprung’s-associated enterocolitis

AIM To determine if expression of colonic tryptophan hydroxylase-2 (TPH2), a surrogate marker of neuronal 5-hydroxytryptamine, is altered in Hirschsprungs-associated enterocolitis. METHODS Entire resected colonic specimens were collected at the time of pull-through operation in children with Hirschsprungs disease (HSCR, n = 12). Five of these patients had a history of pre-operative Hirschsprungs-associated enterocolitis (HAEC). Controls were collected at colostomy closure in children with anorectal malformation (n = 10). The distribution of expression of TPH2 was evaluated using immunofluorescence and confocal microscopy. Protein expression of TPH2 was quantified using western blot analysis in the deep smooth muscle layers. RESULTS TPH2 was co-expressed in nitrergic and cholinergic ganglia in the myenteric and submucosal plexuses in ganglionic colon in HSCR and healthy controls. Co-expression was also seen in submucosal interstitial cells of Cajal and PDGFRα(+) cells. The density of TPH2 immuno-positive fibers decreased incrementally from ganglionic bowel to transition zone bowel to aganglionic bowel in the myenteric plexus. Expression of TPH2 was reduced in ganglionic bowel in those affected by pre-operative HAEC compared to those without HAEC and healthy controls. However, expression of TPH2 was similar or high compared to controls in the colons of children who had undergone diverting colostomy for medically refractory HAEC. CONCLUSION Altered TPH2 expression in colonic serotonergic nerves of patients with HSCR complicated by HAEC may contribute to intestinal secretory and motor disturbances, including recurrent HAEC.

Unusual childhood presentations of abdominal non‐Hodgkin's lymphoma

Non‐Hodgkins lymphoma (NHL) is a relatively common childhood cancer that can present in a myriad of ways. It is essential that NHL is included in the differential diagnosis of children presenting with an abdominal complaint, especially those with unexplained or prolonged symptoms. We describe three acute pediatric presentations of abdominal NHL, two of which presented as acute abdomen (the first mimicking intussusception and the second appendicitis), and the third involving lower limb edema. This case series illustrates the array of presentations of abdominal NHL and the diagnostic challenges that they can provide.

Pre-operative Management and Vascular Access

The surgical procedure performed by the paediatric surgeon forms only a part of the continuum of care provided by paediatric surgeons to their patients. The operation is preceded by pre-operative management involving the assessment and optimisation of each individual patient for the surgical procedure. The operation itself is then followed by a post-operative course where the patient’s recovery is followed and managed appropriately. This chapter deals with preoperative management in the paediatric patient with specifi c reference to vascular access.

Adolescent de novo hydroceles – should they be dealt with by inguinal or scrotal approach?

AIM The objective of this study was to investigate the optimal approach for the treatment of hydroceles in adolescents. MATERIALS AND METHODS A retrospective chart review of all adolescents (10-16 years old) diagnosed with a de-novo hydrocele in 2 tertiary care institutions over a 10 year period (2007-2016) was performed comparing the inguinal and trans-scrotal (Jaboulay) approaches. RESULTS Fifty-three boys with a mean age of 13.4 years (range 10-16 years) were diagnosed with hydrocele. The inguinal approach was used in 31 (59%) patients for treatment of their hydrocele. In 19 (61%) of these cases a patent processus vaginalis (PPV) ligation was performed. In the other 12 (39%) patients the PPV was closed or not found and a further repair of the hydrocele through the same inguinal incision was performed. A transcrotal Jaboulay procedure was performed in 22 (41%) of the patients. There was no difference in the complications rate between inguinal and trans-scrotal approaches (p = 0.71). Age of presentation less than 12 years was associated with the presence of a PPV (p < 0.05). CONCLUSIONS A trans-scrotal approach should be considered as first-line in adolescents when the history is not suggestive of a communicating hydrocele. Children less than 12 years of age are more likely to have a PPV and an inguinal approach may be more appropriate. TYPE OF STUDY Retrospective comparative study LEVEL OF EVIDENCE: Level II.

A novel association of gastric ischaemia and aortic coarctation

We report a newborn who presented with acute abdominal distension secondary to gastric ischaemia with an associated undiagnosed coarctation of the descending aorta. This is the first description of such a previously unrecognised association, which is discussed further with reference to the current literature.