Molecular and immunocytochemical studies of neurofibromas and related cell types.
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Laryngeal obstruction due to neurofibromatosis in a girl with von Recklinghausen's disease first occurred in the neonatal period, and recurred by age seven years. She has done well for four years following laryngofissure surgery. Pharyngeal and laryngeal neurofibromatosis are rare manifestations of von Recklinghausen's disease. The dominant hereditary pattern of this disease requires that the condition be considered when respiratory distress occurs in the child of an affected parent, whether or not skin tumors or other features are present.
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We describe a five-year-old child with neurofibromatosis type I who developed a Charcot knee. Infiltration of the joint by tissue associated with the disease caused damage to the proprioceptive mechanism and resulted in severe joint instability, accelerated destruction and development of neuropathic arthropathy.
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