Klippel-Trenaunay syndromeにおける頭蓋の非対称について

CRANIAL HEMIHYPERTROPHY IN KLIPPEL-TRENAUNAY SYNDROME

I．はじめに

　Klippel-Trenaunay syndromeに対してはphacoma—tosis,hemihypertrophy，血管外科の3分野よりしばしば関心がよせられる。しかしこの症候群における頭蓋の非対称に触れられることは少なく，わずかに剖検報告^7）で知られるのみである。著者らは著明な頭蓋を含むhemihypertrophyとmacrodactylyを伴つた本症候群を経験したので報告し，若干の考察を加える。

Klippel-Trenaunay Syndrome with hemicranial hypertrophy and macrodactyly is reported.

The patient was a 3-year-old Japanese woman with an extensive nevus flammeus which extended over the right side of her neck, axilla, upper arm, and leg. Her right extremities were longer and thicker than left, and showed apparent macro-dactyly in the right hand. Her skin of right side body was irregular, rough surface and was pig-mented prominently. Hyperthermia was present in the hypertrophic limbs, but hyperhidrosis was none.

She had moderate mental retardation, delayed speech. Her hemicranial hypertrophy (same side as hypertrophic limbs) was examined by CT scan. Her right lateral ventricle was dilatated and corti-cal sulci was well demonstrated. Her right hemicranial volume was enlarged but rt-cerebral hemisphere was atrophic, and her infratentorial structures were symmetrical.

Presented case, Klippel-Trenaunay Syndrome with hemicranial hypertrophy, suggested the em-bryological theory affecting on trilaminar disc in embryonal stage as the genesis of this rare syndrome.