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[法国报道的一例新手部巨指症病例。一项统计学研究尝试(作者译)]

[A new case of macrodactyly of the hand reported in France. An attempt at a statistical study (author's transl)].

作者信息

Wangermez J, Normandin C

出版信息

J Radiol Electrol Med Nucl. 1978 Feb;59(2):99-105.

PMID:641886
Abstract

A boy of 4 years presented with hypertrophy of the last three fingers of the right hand associated with syndactyly of the middle and ring fingers. The fingers were of adult length and wider. Arteriography did not show any evidence of an aneurism but demonstrated the existence of an extensive collateral circulation. Thermography confirmed the hypervascularization with the presence of hot points. The authors found reports on 118 cases in the literature published during the last century which could be analyzed statistically. There is a slight predominance in boys (55%) and on the right side. Two fingers are usually involved, the index and middle finger being those affected. Discussions as to aetiology (GOURNET) usually vary between a neurological cause with the possibility of a fibrolipoma of the middle finger being present, and it being due to a phacomatosis. Effective therapy in adults is amputation. In the young there are several possibilities; ablation of the neurovascular masses at the base of the fingers (even though the metacarpals are often involved), destruction of proliferating cartilage, etc.

摘要

一名4岁男孩,右手末三指肥大,伴有中指和环指并指。手指已达成人长度且更宽。动脉造影未显示动脉瘤迹象,但显示存在广泛的侧支循环。热成像证实有热点存在,即血管增生。作者发现上个世纪发表的文献中有118例相关病例报道,可进行统计学分析。男孩略占优势(55%),且多发生于右侧。通常累及两指,即示指和中指。关于病因(古尔内)的讨论通常在神经病因(可能存在中指纤维脂肪瘤)和因错构瘤病之间存在差异。成人的有效治疗方法是截肢。对于儿童则有多种可能性;切除手指根部的神经血管团(尽管掌骨常受累)、破坏增生的软骨等。

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