Normal adrenal function in an infant following a pregnancy complicated by maternal adrenal cortical carcinoma and mitotane exposure.

Maternal adrenal cortical carcinoma in pregnancy is rare. We report a case of an infant born to a mother with a history of adrenal cortical carcinoma. The pregnancy was complicated by fetal exposure to mitotane and dexamethasone. Despite the potential teratogenic exposures, there was no evidence of adrenal dysfunction in the infant. Growth and development at 12 months of age are normal and prognosis appears favorable. The long-term impact of fetal exposure to mitotane and glucocorticoid requires further investigation.

Scrotoschisis associated with meconium periorchitis.

Scrotoschisis is a rare congenital defect in the scrotal wall resulting in testicular exposure or exstrophy. We report the case of a newborn presenting with a large scrotal wall defect exposing both testicles. The tunica vaginalis appeared to be intact on both sides. Although the pathogenesis of scrotoschisis remains unclear, a proposed mechanism for its occurrence and the management in this case are presented in detail.

Outcomes of mitral valve replacement in children: a competing-risks analysis.

OBJECTIVE: We sought to define patient characteristics, outcomes, and associated factors after mitral valve replacement in children. METHODS: We included 104 children undergoing at least one mitral valve replacement between 1980 and 2003 and reviewed clinical records. Competing-risks methodology was used to determine time-related prevalence and associated risk factors after initial mitral valve replacement for death and repeat replacement. RESULTS: The underlying mitral valve disease was congenital in 83%, rheumatic in 13%, Marfan syndrome in 3%, and isolated endocarditis in 1%, with 64% having primarily regurgitation, 16% having stenosis, 20% having both, and 32% having undergone previous valvotomy, valvuloplasty, or repair. There were 137 valve replacements, with 26 patients having more than one. Valve prosthesis type was St Jude Medical in 37%, Bjork-Shiley in 25%, Carbomedics in 20%, Ionescu-Shiley in 10%, and other types in 8%. Both early and late complications were common. Median age at the initial replacement was 5.9 years (range, birth to 19 years). Competing-risks analysis predicted 19% to have died at 15 years after initial replacement, with risk factors including noncongenital valve morphology, lower weight, and longer duration of cardiopulmonary bypass. A repeat replacement was predicted for 71%, with risk factors including the presence of multiple left-heart obstructive lesions and Ionescu-Shiley valve prosthesis. CONCLUSIONS: Mitral valve replacement might be necessary in children with extremely dysplastic valves and severe hemodynamic impairment or after failed repair. However, with the appropriate selection of the prosthetic valve and reduction of cardiopulmonary bypass time, surgeons might decrease mortality and increase prosthesis longevity.