Neurofibromatosis in Infancy Presenting With Congenital Stridor

Abstract

THE OCCURRENCE of von Recklinghausen's disease in an infant is not in itself remarkable, nor is involvement of the larynx in generalized neurofibromatosis noteworthy. A neurofibroma of the larynx causing respiratory distress in an infant with cafe-au-lait spots is, however, unusual. The Department of Pediatrics of the Mount Sinai Hospital recently had the opportunity to study an infant with von Recklinghausen's disease and persistent stridor caused by a plexiform neurofibroma of the larynx. The child died as a result of complications arising from the laryngeal lesion and came to postmortem examination. Report of a Case A 6½-month-old white girl was admitted to the Mount Sinai Hospital for the first and only time in January 1966, with a three-day history of increasing respiratory distress. The infant's mother was a 20-year-old gravida 1 who had an uncomplicated pregnancy and delivery. The birthweight was approximately 3,500 gm (7 lb 12 oz). Noisy respirations