[Cranial hemihypertrophy in Klippel-Trenaunay syndrome (author's transl)]

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 the neck, axilla, upper arm, and leg. Her right extremities were longer and thicker than left, and showed apparent macrodactyly in the right hand. Her skin of right side body was irregular, rough surface and was pigmented 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 cortical 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 embryological theory affecting on trilaminar disc in embryonal stage as the genesis of this rare syndrome.