NEUROFIBROMATOSIS PRESENTING AS A SEVERE SYSTEMIC VASCULOPATHY

In a boy with neurofibromatosis type 1 (NF-1), hypertension, septic in fection of an aneurysm in the deltoid muscle, bowel infarction, multip le arterial aneurysms and venous thrombosis occured within a period of 6 weeks. Histologically, vascular neurofibromatosis of the small vess els of the gut was found. This unusual occurrence of a multitude of cl inical features within a few weeks was caused by vascular neurofibroma tosis. Awareness of this condition in patients with NF-1 may help the paediatrician to avoid un-necessary diagnostic procedures and to initi ate appropriate symptomatic therapy.