Neurofibromatosis presenting as a severe systemic vasculopathy.
In a boy with neurofibromatosis type 1 (NF-1), hypertension, septic infection of an aneurysm in the deltoid muscle, bowel infarction, multiple arterial aneurysms and venous thrombosis occurred within a period of 6 weeks. Histologically, vascular neurofibromatosis of the small vessels of the gut was found. This unusual occurrence of a multitude of clinical features within a few weeks was caused by vascular neurofibromatosis. Awareness of this condition in patients with NF-1 may help the paediatrician to avoid unnecessary diagnostic procedures and to initiate appropriate symptomatic therapy.
Lehrnbecher
T
T
Universitätskinderklinik und Poliklinik, Würzburg, Germany.
Gassel
A M
AM
Rauh
V
V
Kirchner
T
T
Huppertz
H I
HI
D001158:Arteries / Q000473:pathology; D002675:Child, Preschool; D006801:Humans; D008297:Male; D009456:Neurofibromatosis 1 / Q000175:diagnosis* / Q000473:pathology; D014652:Vascular Diseases / Q000175:diagnosis* / Q000473:pathology