Laparoscopic management of colonic complications in Ehlers-Danlos syndrome type IV.

Ehlers-Danlos syndrome type IV, also known as the vascular type, is the most severe form of the disease. It may cause spontaneous bowel perforation and rupture of major arteries. The treatment of bowel perforation in this population remains controversial because they are at risk for major wound complications owing to poor healing. We report the first use of laparoscopy in children with Ehlers-Danlos syndrome.

The risks of minimal access surgery in children: an aid to consent.

AIM: The aim of this study was to determine the risk of complications and conversions for minimally invasive procedures in children, thus allowing properly informed consent. METHODS: Data were retrieved for all minimally invasive surgical procedures performed between 1995 and 2009. RESULTS: There were 2352 cases performed in 2288 (1428 were male) patients. Of these, 2210 cases (94%) were laparoscopic, and 143 (6%), thoracoscopic. The median age at operation was 6 years and 4 months. The overall complication rate was 3.6%, with the risk of early reoperation at 1.7%. The risk was highest for fundoplication and pyloromyotomy at 3.2% and 4%, respectively. The risk of an infective complication was 0.5% and was highest for appendicectomy and nephrectomy. The risk of visceral injury overall in this series was 0.4%. Visceral injury, explicable only by port insertion, occurred in just under 1 in 1000 cases. The conversion rate was 2.3%. The lowest rates were observed with appendicectomy, fundoplication, and pyloromyotomy. Thoracoscopic cases, nephrectomies, and procedures for an underlying oncological diagnosis had a higher conversion rate. CONCLUSION: Informed consent requires knowledge of the risks of surgery. This series may serve as an aid for other units in obtaining consent for minimally invasive surgery in the pediatric population.

Introduction of sodium pentosan polysulfate and avoidance of urethral catheterisation: improved outcomes in children with haemorrhagic cystitis post stem cell transplant/chemotherapy.

PURPOSE: Haemorrhagic cystitis (HC) is an uncommon but potentially devastating complication of chemotherapy and bone marrow transplantation in children. We aimed to test the hypothesis that early recognition, sodium pentosan polysulfate (SPP), and avoidance of urethral catheterisation improve outcomes in children with HC. METHODS: A retrospective case note review was performed of all patients treated for HC in our hospital from 2002 to 2010. A protocol for the management of HC was introduced in 2007 advocating early detection, use of SPP, and avoidance of urethral catheterisation. Data collected on each patient included primary condition, medications at onset, blood transfusions, duration of symptoms, catheter usage, and outcome. Statistical analysis was performed using the Mann-Whitney U test, and Fisher's Exact test as appropriate, P < .05 being significant. RESULTS: Five patients were treated using protocol with 5 historical controls. There was no significant difference between the ages of the group, diagnosis, and treatment at onset of HC. In the historical group, 4 of 5 died with HC, but all recovered in the protocol group (P < .05). Blood transfusion requirements were also significantly reduced after protocol introduction (P < .05). CONCLUSION: Early identification, avoidance of urethral catheterisation, and use of SPP significantly reduces blood transfusion requirements and mortality from HC.

Obturator internus pyomyositis presenting as a pararectal abscess.

This report describes two children who presented with fever, hip pain and a limp, and were subsequently found to have a primary pyomyositis of the obturator internus muscle. A clinical diagnosis of septic arthritis of the hip was made initially, but in both children MRI showed a pararectal abscess, which required incision and drainage. Staphylococcus aureus was cultured from pus from the abscesses and both children made a full recovery subsequently. This report highlights the main features of this unusual entity and emphasises the need for early imaging in the child with an unexplained limp.