一側停留睾丸，反対側睾丸無形成であつたPrader-Willi症候群の1例

A CASE OF PRADER-WILLI SYNDROME WITH CRYPTORCHIDISM OF ONE SIDE AND TESTICULAR APLASIA OF THE OTHER SIDE

緒言

　1956年Prader,Labhart,Willi^1）は精神発達遅延，低身長，停留睾丸および新生児期の筋緊張低下を主症状とする9例を新しい症候群として報告した。その後，本症候群はPrader-（Labhart）-Willi症候群またはその主症状である筋緊張低下（Hypo-tonia），精神発達遅延（Hypomentia），停留睾丸をはじめとする外陰部発育不全（Hypogonadism），肥満（Obesity）の頭文字をとつたHHHO症候群とも呼ばれている。われわれは両側陰嚢内容の欠如を主訴とした本症候群に一側は睾丸固定術を施行したが，反対側は睾丸無形成であつた1例を経験したので報告する。

A-6-year-old boy was admitted to Yokohama Municipal Citizen's Hospital with chief complaints of bilateral scrotal vacancy and maldevelopment of external genitalia. He had a history of hypotonia in the neonatal period and early infancy. Hyperphagia and obesity became apparent after about 2 years and 6 months of age. He was markedly mentally retarded. Right orchidopexy was performed and from the left groin the tissue was removed which looked like left testicle. Histologically the right testicle was hypoplastic and the left testicle was not identified.

Some problems on orchidopexy with Prader-Willi syndrome were discussed.