13 ribs as a predictor of long gap esophageal atresia: myth or reality? Analysis of associated findings of esophageal atresia and abnormal rib count.

BACKGROUND: The presence of 13 pairs of ribs on pre-operative chest x-ray has been postulated to be an indicator for long gap esophageal atresia (EA). This study sought to determine the validity of this theory and identify associated pathological conditions in patients with EA and abnormal rib count. METHODS: Babies with EA from January 2005 - December 2012 were retrospectively analyzed. Information was gathered from neonatal health records and operation notes. Chest x-rays were reviewed to determine rib count. Long gap EA was defined as failure to achieve primary esophageal anastomosis. Statistical analysis performed with Fisher's exact test. RESULTS: Seventy-six patients were identified. Eight patients had long gap EA, with none of these patients having 13 pairs of ribs. Paradoxically, 10 patients with esophageal atresia +/- trachea-esophageal atresia (EA +/- TEF) and supernumerary ribs underwent primary repair. Nine patients had 11 pairs of ribs, of which 2 had pure EA and a long gap. Using Fisher's exact test to compare the groups of supernumary ribs and non-supernumary ribs there is a p value of 0.587. VACTERL association was identified in 40% of those with supernumerary ribs. Various associated syndromes and concomitant abnormalities were identified. CONCLUSION: We found no association between 13 pairs of ribs and long gap in esophageal atresia. Those with 13 pairs of ribs were more likely to have associated anomalies, although this was not statistically significant. Our cohort of patients was found to have a range of pathology related to genetic syndromes, further atresias, and malformations, which is well known to be associated with children born with EA +/- TEF. LEVEL OF EVIDENCE: Prognosis study - level IV.

Pediatric Benign Cystic Peritoneal Mesothelioma.

BACKGROUND: Benign cystic peritoneal mesothelioma is a rare tumor comprising multiple cysts typically found within the abdominal cavity. Although the literature has numerous accounts of this entity in adults, only a handful of cases have been published regarding its presence in the pediatric population. CASE: We present the case of a prepubertal 10-year-old girl with the finding of benign cystic peritoneal mesothelioma at laparoscopy for suspected appendicitis, the management, and outcome. SUMMARY AND CONCLUSION: Although benign cystic peritoneal mesothelioma can be treated with primary excision, the high recurrence rate in adult cases of this disease necessitates the need for long-term follow-up of these patients and provides a challenge to surgeons in providing continuing care.

Laparoscopic management of impalpable testes: comparison of different techniques.

PURPOSE: Laparoscopy is an important modality for management of impalpable testes. We present long-term outcomes of intra-abdominal testes managed by either single stage orchidopexy or two-stage Fowler Stephen's orchidopexy (FSO) over 12 years. METHODS: Data were prospectively collected and retrospectively analyzed on patients who underwent laparoscopy for impalpable testes between 1998 and 2010. Demographic data, intra-operative findings, management, histology and follow-up findings were collected and analyzed. Fisher's Exact test was used for statistical analysis. RESULTS: Laparoscopy was performed for 168 impalpable testes (78 left, 58 right and 16 bilateral). Patients were between 8 months and 15 years of age (median 1 year 10 months). Ninety-three testes were found to have cord structures entering the inguinal ring (canalicular), 65 were intra-abdominal and 10 had blind ending vas and/or vessel. Fifty-seven (34%) testes were atrophic and underwent orchidectomy; 100 (60%) testes underwent orchidopexy: either two-stage FSO (48) or single stage orchidopexy (52) and 10 (7%) had findings consistent with 'vanishing testes'. Histopathologically, the excised remnants (34%) showed no viable testicular tissue. The follow-up was a median of 8 months (3 months to 6 years). Four patients were lost to follow-up (two each after FSO and single stage orchidopexy) while, two FSO are awaiting follow-up. At follow-up, 36/44 testes (FSO) and 13/13 testes (single stage orchidopexy) are in the scrotum and of good size. Eight testes had atrophied after two-staged FSO. CONCLUSION: Canalicular testes are often difficult to palpate (55%). Laparoscopy allows direct visualization and definitive management. There is no statistically significant difference between the results following single stage orchidopexy or two-stage FSO for impalpable testes.

Left paramesocolic hernia presenting as post appendicectomy abdominal cocoon.

We describe a rare case of left mesocolic hernia presenting as post appendicectomy intestinal obstruction in a girl. Laparotomy confirmed partial peritoneal encapsulation of upper small bowel due to herniation of jejunal loops into the left mesocolic hernia sac. Reduction of contents, resection of the sac and repair of the defect concluded the procedure uneventfully.

Coeliac disease in a child with anorectal malformation: The importance of considering other causes of diarrhea.

We present the case of an Indian child with a high anorectal malformation (ARM) who postoperatively had troublesome fecal incontinence. Based on a dietary history, weight loss, and diarrhea, a duodenal biopsy was performed that revealed coeliac disease. Since being on a gluten-free diet, her symptoms have improved dramatically. To the best of our knowledge this is the first report in the English literature of such an association between ARMs and coeliac disease. Dietary modification alone can dramatically improve symptoms in these children.

Gastrostomy complications in infants and children: a comparative study.

PURPOSE: Gastrostomy is a commonly undertaken procedure in children. Methods for gastrostomy insertion have evolved and laparoscopy is commonly used to aid its insertion. The aim of this study is to review the outcome and complications of laparoscopic-assisted gastrostomy insertion. METHODS: A retrospective case note review of children undergoing gastrostomy insertion by a single surgeon from 1999 to 2007. RESULTS: 114 children underwent gastrostomy insertion, 68 males and 46 females, with a median age of 55 months (IQR 28-149 months). The majority were neurologically impaired. 98 underwent laparoscopic-assisted PEG (86%), 1 was converted to open procedure (0.9%) and 16 had standard PEG (14%). Median follow-up was 10.5 months with 90 children having their original gastrostomy changed to a button at a median interval of 7.4 months. Complications observed included new or deteriorating gastro-oesophageal reflux (GOR) (n = 16), infection (n = 9), granulation tissue (n = 11), tube dislodgement (n = 2) and intra-abdominal leakage of feed (n = 2). There were two gastrocolic fistulae which occurred in the standard PEG group. CONCLUSION: The inadvertent formation of a gastrocolic fistula was avoided with the use of laparoscopy to aid PEG insertion.

A ganglioneuroma of the sigmoid colon presenting as leading point of intussusception in a child: a case report.

We present a case of intestinal ganglioneuroma (GN) of the sigmoid colon in a 5-year-old girl, which caused intermittent colocolic intussusception. Ganglioneuromas are rare benign tumors of the autonomic nervous system composed of mature ganglion cells and satellite cells. Colonic GNs are uncommon. The unusual intramural proliferation of neural elements in this case resembled the diffuse intestinal ganglioneuromatosis, which is known to be associated with multiple endocrine neoplasia type 2B. However, the specific mutations of multiple endocrine neoplasia type 2B were not found by genetic sequencing. This is the first pediatric case described in the literature of a solitary polypoid GN presenting as a colocolic intussusception. We present a brief overview of intestinal ganglioneuromatous lesions and associated conditions.

Infantile myofibromatosis.

Despite being the most common fibrous tumour of infancy, infantile myofibromatosis is still sufficiently rare for the diagnosis not to be apparent to many clinicians. We present the data from the 12 cases seen in our institute over the last 14 years and highlight three cases, the first a "typical" case, then a retroperitoneal myofibroma that presented with duodenal obstruction and finally one that presented as an isolated scrotal mass. We have also reviewed the literature on the subject.

Midgut malrotation and associated Hirschsprung's disease: a diagnostic dilemma.

Malrotation and Hirschsprung's disease (HSD) are rarely reported together. We report our experience with three patients who presented during the neonatal period in whom the association resulted in diagnostic difficulty. In this report, we focus on the clinical presentation, diagnosis and appropriate management.

Candida peritonitis: a rare complication following early dislodgement of percutaneous endoscopic gastrostomy tube.

UNLABELLED: Candida peritonitis is a rare but potentially fatal complication of early dislodgement of percutaneous endoscopic gastrostomy (PEG) feeding tube. We report the case of 12-year-old boy who developed Candida peritonitis subsequent to early dislodgement of PEG tube. PEG tubes may be prone to accidental dislodgement or removal by patients or carers. This complication has to be recognized early in order to avoid the risk of peritonitis. In our case the patient initially developed coliform peritonitis followed by peritoneal and systemic candidiasis. The patient needed ventilatory support, inotropic support, broad-spectrum antibiotics, total parenteral nutrition and antifungal agents liposomal amphotericin and flucytosine. CONCLUSION: Early dislodged PEG tubes should be recognized early in order to avoid the risk of peritonitis and managed by endoscopic or surgical replacement rather than blind replacement by the appropriately skilled personnel.