家族性大腸腺腫症にみられる網膜色素上皮異常

Anomalous retinal pigment epithelium in familial adenomatosis coli

　家族性大腸腺腫症16例の臨床的検討を行い，13例に網膜色素上皮肥大を認めた。また他に，脱色素斑，色素上皮剥離，眼底色素のむらなどの網膜色素上皮の異常が多くみられた。さらに25歳女性の剖検眼球において，組織学的に広範な網膜色素上皮の異常がみられた。

　家族性大腸腺腫症では，網膜色素上皮肥大のほかに，広範な網膜色素上皮の異常が随伴する可能性が示された。

In a consecutive series of 16 cases with familial adenomatosis coli, we detected hypertrophy of the retinal pigment epithelium in 13 cases. The lesion appeared as black or brown pigment patches of variable sizes surrounded, occasionally, by a depig-mented halo. We also detected depigmented patches in 4 cases and detachment of retinal pig-ment epithelium in one. Mottled appearance of the fundus was a prevalent feature in the series.

Postmortem examinations of eyes in a 25-year-old female with familial adenomatosis coli showed vacuoles in the retinal pigment epithelial cells in the equatorial and peripheral retina.

The clinical and histological studies seemed to suggest that diffuse abnormalities of the retinal pigment epithelial cells were associated with famil-ial adenomatosis coli.