Complete anal membrane in a female: a rare malformation and indication for prone cross-table lateral radiograph.

Magnetic resonance imaging (MRI) of a 27 week gestation female fetus suggested an abnormal rectum. Subsequent examination of the term newborn confirmed an imperforate anus present in the normal position. No meconium was seen on the perineum by 18 hours. A prone cross-table lateral radiograph confirmed that rectal gas was present 3 mm beneath the membrane leading to the diagnosis of complete anal membrane, and an anoplasty rather than a colostomy was performed.

Laparoscopic repair of anorectal malformations at the Red Cross War Memorial Children's Hospital: taking stock.

BACKGROUND: The current standard repair for anorectal malformations in children is a posterior sagittal anorectoplasty. Recently, laparoscopic-assisted anorectoplasty (LAARP) was performed at the Red Cross Children's Hospital. METHODS: A detailed case note review was conducted. Patient outcome was prospectively evaluated by colorectal nurse specialists using the Krickenbeck standardized questionnaire. Comparison among patients undergoing posterior sagittal anorectoplasty was performed. RESULTS: Between September 2005 and June 2009, 24 children underwent LAARP. Sixteen had associated anomalies, including 7 children with renal and 4 children with cardiac abnormalities. Median age at surgery was 7.5 months (range, 2.6-15.0 months). Subtypes of anorectal malformation were as follows: vestibular, 2; bulbar, 9; prostatic, 7; vesical, 3; and with no fistula, 3. There was a 16% early complication rate. Redo-anoplasty was required in 9 patients. Eleven children had difficulties with follow-up. Thirteen children had regular follow-up and were analyzed further. Toilet training had been completed in 7 children (median age, 4.3 years; range, 3.5-6 years). Six children developed voluntary bowel motions. Six children are awaiting toilet training or are unable to train because of incontinence. CONCLUSIONS: Anal stenosis was the most common complication post-LAARP. Etiology appeared to be multifactorial, but poor compliance with dilatations was a leading cause.

Proximal colonic atresia: is right hemicolectomy inevitable?

PURPOSE: Proximal colonic atresia often results in a marked discrepancy of the atretic ends presenting a technical challenge for restoration of bowel continuity. We review our series of colonic atresia patients with particular reference to the techniques for restoring bowel continuity. METHODS: Case notes of all patients diagnosed with colonic atresia and admitted to our unit between 1997 and 2011 were reviewed. RESULTS: There were 13 patients with a median gestational age of 39 weeks. Location of the atresia was ascending colon (n = 1), hepatic flexure (n = 9), proximal transverse colon (n = 2) and sigmoid colon (n = 1). The primary procedure was: primary anastomosis (n = 1), right hemicolectomy followed by ileo-colic anastomosis (n = 2), right hemicolectomy and stoma formation (n = 3). Six patients underwent stoma formation, of which five subsequently had a right hemicolectomy at a later procedure. In total, 10/12 patients required right hemicolectomy to facilitate restoration of bowel continuity. Median time to full feeds was 11 days (4-46). Median follow up time was 11 months (22 days-5.6 years). CONCLUSION: A temporising stoma does not reduce the discrepancy in the calibre of the atretic ends in proximal colonic atresia. Right hemicolectomy and ileo-colic anastomosis should therefore be considered at the initial surgery.

Mesoblastic nephroma: a report of the United Kingdom Children's Cancer and Leukaemia Group (CCLG).

BACKGROUND: Mesoblastic nephroma (MN) is a rare tumour which occurs mainly in early infancy and for which primary nephrectomy is the treatment of choice. This study aimed to assess surgical complications and outcomes in this patient group and to re-evaluate the age threshold of 6 months for recommending primary nephrectomy. PROCEDURE: A retrospective file review of all cases of MN registered in UK Children's Cancer and Leukaemia Group renal tumour trials between October 1991 and March 2008. Data from the trials were compared with data held by the National Registry of Childhood Tumours, Oxford. RESULTS: Forty-seven (3.5%) confirmed cases of MN were found among 1346 registered renal tumours. Median age at diagnosis was 30 days (range birth-3.8 years). MN was significantly more common in the first 3 months of life compared to between 3 and 6 months (33 vs. 2 cases). Seven cases occurred between 6 months and 1 year and only five cases occurred beyond 1 year of age. There was a significant difference in the age of diagnosis by histological subtype. There were 11 complications in the series; no registered patient developed a recurrent tumour; and all were alive at last follow-up. CONCLUSIONS: Outcome for children with MN is excellent at all ages, with little indication for adjuvant chemotherapy. Children presenting at <3 months of age, should be treated by primary nephrectomy. In those presenting aged >3 months, alternative diagnoses should be considered, especially in the presence of surgical risk factors.

Cystoscopy assisted transvesical biopsy of prostatic rhabdomyosarcoma.

The treatment of prostatic rhabdomyosarcoma (RMS) depends on tumour stratification based on site and histology. An increasing range of cytogenetic, molecular, and immunohistochemistry studies are required. This is difficult to achieve using standard cystoscopic biopsies alone. We present a 5-year-old male, diagnosed with a prostatic RMS. He underwent cystoscopy to confirm the diagnosis and at the same time tissue was obtained for histology using laparoscopic graspers via a STEP Port inserted percutaneously into the apex of his bladder. Histology and cytogenetics confirmed an embryonal botryoid RMS for which he received chemotherapy followed by a radical prostatectomy for residual disease.

Functional and cosmetic outcome of the VQ plasty for Mitrofanoff stomas.

PURPOSE: A skin lined Mitrofanoff stoma in the iliac fossa is traditionally achieved using a VQZ technique, providing a channel for intermittent catheterization. We simplified our approach by using a VQ flap at the distal end of the Pfannenstiel incision. We present a comparison of the functional and cosmetic outcome of these 2 approaches. MATERIALS AND METHODS: Records of all patients requiring a Mitrofanoff stoma performed by a single surgeon (RS) were reviewed. Data were collected from hospital notes, patient followup clinics and urology nurse specialist records. All VQ type stomas were performed since March 2004. RESULTS: A total of 21 patients had 23 stomas formed (11 VQZ and 12 VQ plasty). Each group was composed mainly of appendiceal conduits. Three ileal (Monti) conduits were formed. Mean followup was 18.3 months for VQZ and 15 months for VQ cases. Removing the Z flap leaves behind a neat skin lined stoma unlike a VQZ plasty, which results in a more prominent and irregular scar. One VQ stoma prolapsed slightly. Three stomas were revised in the VQZ group for stenosis (1 traumatic) or leakage, 2 using VQ plasty. CONCLUSIONS: Formation of a skin lined stoma by VQ plasty as opposed to VQZ plasty reduces scarring around the conduit without affecting the complication rate or stoma continence. Patients continue to be positive about the cosmetic appearance of the stoma.

Pediatric pancreatic hemangioma: a case report and literature review.

CONTEXT: The pancreas is an unusual site for a hemangioma in an infant. A child with obstructive jaundice caused by a pancreatic hemangioma is presented and management strategies for this benign tumor are discussed. CASE REPORT: A 5-month-old girl presented with a 2-week history of jaundice, pale stools and dark urine. Liver function tests confirmed obstructive jaundice. An abdominal ultrasound scan and magnetic resonance imaging showed an enhancing mass in the head of the pancreas. At laparotomy, a wedge biopsy of the pancreatic tumor was taken and a tube cholecystostomy inserted. Histological examination of the specimen revealed a pancreatic hemangioma with sclerotic features. The high volume of bile loss from the cholecystostomy proved problematic and biliary diversion with a Roux-en-y hepaticojejunostomy was therefore performed. The tumor subsequently regressed spontaneously and was no longer visible on follow-up imaging two years later. The child has since thrived. CONCLUSIONS: Pancreatic hemangiomas are rare and may cause diagnostic confusion. Pancreatic resection should be avoided since the natural history of these benign tumors is that of spontaneous involution. Various strategies can be used to manage any associated obstructive jaundice.