Poland's Syndrome

概要

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In 1841, Alfred Poland first described a cadaver with symbrachydactyly associated with lack of the ipsilateral pectoral muscles. Afterward, many observation, as "Poland's syndactyly", "pectoral aplasia-dysplasia syndrome", or "hand and ipsilateral thorax syndrome", etc., were reported. In 1976, Sugiura described the details of 45 cases of Poland's syndrome, and advocated a clear definition and classification. That was 1) Unilateral brachymesophalangy of the indix, middle and ring fingers (or occasionally more severe reduction malformation of the hand). 2) Cutaneous syndactyly in the affected digits (symbrachydactyly). 3) Moderate microhand. 4) Absence of the sterno-costal portion of the ipsilateral pectoralis major muscle. And classification was 1) Brachymesophalangeal triphalangy type. 2) Diphalangy type. 3) Monophalangy type. 4) Ectrodactyly type.24 cases of Poland's syndrome (14: definitely diagnosed, 10: possible) were picked out from the outpatients in the past 20 years. They were 0.02% of the outpatients and 1.92% of those with congenital anomalies of the upper limb. 17 of them were male, 15 affected right side. Diphalangy type (using Sugiura's classification) was seen in 15 cases, the most numerous.
西日本整形・災害外科学会の論文