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巨指(趾)畸形

Macrodactyly.

作者信息

Dell P C

出版信息

Hand Clin. 1985 Aug;1(3):511-24.

PMID:3938454
Abstract

Digital gigantism is an unusual congenital anomaly that is present at birth or recognized in early infancy and is progressive during the period of normal skeletal maturation. It involves cell types that are predominately affected by neurogenic growth control and follow a distribution similar to the sensory supply of a major peripheral nerve, usually the median nerve. There seem to be three variants that overlap somewhat in clinical presentation. They are type I digital gigantism with lipofibromatous hamartoma of a peripheral nerve, type II digital gigantism associated with neurofibromatosis, and type III hyperostotic digital gigantism. Treatment should be individualized and initiated early in life. Epiphysiodeses, osteotomies, and division of branches of the supplying digital nerves may be indicated during childhood. In the adult, arthrodesis of the interphalangeal joints with bony shortening or ray resection may be indicated. Skin flap necrosis is a common complication postoperatively.

摘要

指巨大症是一种罕见的先天性异常,出生时即存在或在婴儿早期被发现,在正常骨骼成熟期间呈进行性发展。它涉及的细胞类型主要受神经源性生长控制影响,其分布类似于主要外周神经(通常是正中神经)的感觉支配。临床表现似乎有三种变体,它们在某种程度上相互重叠。它们是伴有周围神经脂肪纤维瘤性错构瘤的I型指巨大症、与神经纤维瘤病相关的II型指巨大症和III型骨肥厚性指巨大症。治疗应个体化并在生命早期开始。儿童期可能需要进行骨骺阻滞、截骨术以及供应指神经分支的切断术。对于成年人,可能需要进行指间关节融合术并伴有骨质缩短或射线切除。皮瓣坏死是术后常见的并发症。

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