Simultaneous occurrence of right adrenocortical tumor and left adrenal neuroblastoma in an infant with Beckwith-Wiedemann syndrome.

Children with Beckwith-Wiedemann syndrome (BWS) have increased risk for development of embryonal tumors. We present the case of an infant with BWS who has hypomethylation of LIT1 gene in the 11p15.5 chromosomal region and at 6 months of age presented with simultaneous occurrence of neuroblastoma arising from the left adrenal gland and a right adrenocortical tumor. She underwent surgical resection of both tumors and remains tumor free 18 months after surgery.

Trisomy 21 and the pediatric surgeon.

PURPOSE OF REVIEW: Trisomy 21 (Down syndrome) is a common condition encountered by the pediatric surgeon. The surgical anomalies associated with Down syndrome now have treatment options that were previously considered futile. An effective surgical team approach to these children involves knowledge of the unique pathophysiology of trisomy 21 and an appreciation for the desires of the family and the needs of the child. RECENT FINDINGS: Surgical techniques benefit children with Down syndrome equally as typical children. Pessimism regarding the outcome of surgical treatment of colorectal anomalies is fading, with evidence of nearly equivalent outcomes. Several autoimmune disorders impact children with Down syndrome. Idiosyncratic susceptibility to infections and malignancy is noted. Unique anesthetic complications and aerodigestive tract disorders have been identified. Many anomalies have surgical options that enhance the quality of life. SUMMARY: There is optimism in the surgical treatment of children with Down syndrome. Improved operations, better anesthetic management and recognition of problems specific to the child with trisomy 21 make it safer and ethical to offer surgical solutions. Societal acceptance and mainstream participation are on the rise. The astute, compassionate pediatric surgeon does much to enhance the quality of life of these children and of the families who love them.

Laparoscopic suture rectopexy for full-thickness anorectal prolapse in children: an effective outpatient procedure.

BACKGROUND/PURPOSE: Our approach to full-thickness anorectal prolapse has transitioned to laparoscopic suture rectopexy (LSRP). The purpose of this study was to describe the indications, technique, and postoperative outcomes for LSRP. METHODS: Rectopexy was performed using 3 or 4 laparoscopic ports. Redundant rectum was retracted from the pelvis, and the posterior rectal wall was secured to the sacral promontory using 3 permanent sutures. RESULTS: Nineteen children (7 girls) underwent LSRP from March 2003 to January 2008. Mean age was 6.2 ± 3.6 years. Three patients had prior perineal operations: 2 sacrococcygeal teratoma resections and 1 pull-through for Hirschsprung disease. One patient had cystic fibrosis, and another had Prader-Willi syndrome. The remaining children had either chronic constipation or idiopathic prolapse. All patients were treated preoperatively with laxatives. Two patients received antegrade continent enemas. Length of stay was 1 ± 0.8 days, with only the first 5 patients admitted to the hospital. The patient with Prader-Willi syndrome had a full-thickness recurrence (5%) owing to obsessive-compulsive behavior. Partial mucosal prolapse occurred in 2 patients. There were no other complications. CONCLUSIONS: Laparoscopic suture rectopexy is an effective minimally invasive method to treat full-thickness rectal prolapse in children from various etiologies. It can be performed as an outpatient procedure with minimal morbidity and low recurrence rate (5%).

Complete duplication of bladder and urethra in a sagittal plane in a male infant: case report and literature review.

Complete duplication of the bladder and urethra is a rare entity. It may occur in the coronal and sagittal planes, and is often associated with other organ system anomalies, in particular of the gastrointestinal tract. We report an unusual variant of sagittal duplication of the bladder, in a male, associated with rudimentary hindgut duplication, and review the literature pertaining to this unusual anomaly.

Complicated appendicitis in children: a clear role for drainage and delayed appendectomy.

INTRODUCTION: Children presenting with complicated appendicitis represent a common and challenging problem. Conflicting data exist concerning optimal treatment of these patients with primary versus delayed appendectomy. METHODS: A retrospective review of all children undergoing appendectomy over a 5-year period was performed. RESULTS: We identified 1,106 children: 360 had evidence of perforation and 92 had an intra-abdominal abscess or right lower quadrant phlegmon. Of these 92, 60 underwent primary appendectomy and 32 underwent drainage and/or antibiotic therapy with delayed appendectomy. Children undergoing delayed appendectomy had a longer prodrome of symptoms (6.9 vs 4.6 days, P = .002), slightly higher presenting white blood cell count (19.3 vs 16.6, P = .08), and had the same hospital length of stay, yet had a lower complication rate requiring readmission to the hospital (0% vs 10%) compared to those undergoing immediate appendectomy. CONCLUSION: In children presenting with prolonged symptoms and a discrete appendiceal abscess or phlegmon, drainage and delayed appendectomy should be the treatment of choice.