PMID: 3762859Jun 1, 1986Paper

Cerebral and pulmonary arteriovenous fistula with possible hereditary hemorrhagic telangiectasia: case report

No shinkei geka. Neurological surgery
S NakaoH Sakamoto

Abstract

Hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease) is a syndrome characterized by the presence of mucocutaneous telangiectasia, recurrent hemorrhage (epistaxis from nasal telangiectasia is by far the most common form), and hereditary occurrence. Hereditary hemorrhagic telangiectasia is thought not to be a simple mucocutaneous disease but a generalized vascular dysplasia, because multiple visceral organs and systems, including central nervous system, are involved. But, an involvement of the central nervous system in this disease is rare. We experienced a case with cerebral arteriovenous fistula that was also diagnosed as possible hereditary hemorrhagic telangiectasia. In this communication this case is reported and the relevant literature is reviewed. A 30-year-old male, who was diagnosed as having possible hereditary hemorrhagic telangiectasia, was found to have abnormal findings on head CT scan during the evaluation of his disease. So he was admitted to the neurosurgical department for further examination. He was also known to have pulmonary arteriovenous fistula (11.5% arteriovenous shunt) and secondary polycythemia (RBC 533 X 10(4)/mm3, Hb 17.6 g/dl, Ht 51%). His past medical history was noteworthy for several...Continue Reading

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