Primary intrarenal neuroblastoma in a 13-month-old girl presenting as a Wilms' tumor with pulmonary metastasis.

We report a 13-month-old girl with primary intrarenal neuroblastoma initially diagnosed as Wilms' tumor. Intrarenal neuroblastoma is exceedingly rare in pediatric age that may masquerade as Wilms' tumor clinically and radiographically and it is important to differentiate for management.

Necrotizing fasciitis in children: diagnostic and therapeutic aspects.

BACKGROUND: Necrotizing fasciitis (NF) is a severe life-threatening soft tissue infection characterized by rapidly spreading necrosis of the fascia and the subcutaneous tissue. Its incidence owing to invasive Streptococcus pyogenes has significantly increased in children recently. Our experience with NF in children to describe diagnostic and therapeutic aspects is hence presented herein. METHODS: Records of children who were treated for NF in our unit from 1999 to 2006, inclusive, were reviewed retrospectively. Information recorded for each patient included medical history, clinical characteristics, diagnostic procedures, treatment methods, and the outcome. RESULTS: Thirteen patients with a mean age of 35 months were treated for NF during the study period. All of the 13 children had no previous immunosuppression. The predisposing factors were composed of varicella lesions, intramuscular injections, application of a cream containing menthol to the cervical region, penetrant gluteal trauma, omphalitis, dental abscess, and streptococcal toxic shock syndrome. The most common site of the initial involvement was the abdominal wall, followed by the gluteal region and thigh, head and neck, and upper and lower extremities. The initial skin presentations were induration or cellulitis and erythema and edema with progression to skin discoloration and bullae formation. Fever and tachycardia were the most common clinical features. S. pyogenes was the most common causative microorganism, followed by Staphylococcus epidermidis and Pseudomonas aeruginosa. All patients underwent extensive surgical debridement and received appropriate antibiotics and supportive therapy. Twelve patients survived, and 1 patient with delayed diagnosis of NF died of septic shock. CONCLUSION: Although these infections are rare in children, their lethal potential and early diagnostic signs must be recognized. All children with NF should undergo early surgical debridement to prevent delay in treatment. The mortality and morbidity associated with NF in children can be decreased with clinical awareness, early diagnosis, and adequate and urgent surgical debridement followed by intensive supportive care and early wound resurfacing.

Ambiguous genitalia: an overview of 17 years' experience.

AIM: The newborn with abnormal genital development presents a difficult diagnostic and treatment challenge for the pediatric surgeon providing care. The purpose of this study was to evaluate the results of surgical treatment for children with ambiguous genitalia. PATIENTS AND METHODS: The records of 85 children managed surgically for ambiguous genitalia in our unit from 1988 to 2005 were reviewed retrospectively. Age at surgery, operative procedures, sex of rearing, and outcome were recorded. RESULTS: The intersex committee's decision concerning sex assignment was female for 62 children (75%) and male for 23 children (25%). The etiologies of children reared as female were congenital adrenal hyperplasia (n = 37), male pseudohermaphroditism (n = 12), mixed gonadal dysgenesis (n = 6), true hermaphroditism (n = 4), and Mayer-Rokitansky syndrome (n = 3). Fifteen children with male pseudohermaphroditism, 5 children with congenital adrenal hyperplasia, and 3 children with true hermaphroditism were reared as male. The mean age at surgery was 4.4 years and follow-up period averaged 7 years. Eighteen (29%) patients with feminization procedures and 8 (34%) of 23 patients with masculinization procedures experienced complications and required redo operations. Vaginal stenosis was the most common complication. CONCLUSION: The surgical management of ambiguous genitalia has always been difficult, and it must be performed by skilled pediatric surgeon. Genital surgery in infancy needs to be reassessed in the light of literature findings revealing poor outcome. In patients who underwent feminizing genitoplasty, vaginal reconstruction should be delayed until adolescence to achieve better cosmetic and functional results.