Surgical correction of contractural arachnodactyly deformity of a child with Marfan syndrome
10.3760/cma.j.issn.1009-2587.2018.10.015
- VernacularTitle: 手术矫治马方综合征蜘蛛样指畸形挛缩一例
- Author:
Zhou XIA
1
;
Wei QIU
;
Youxin XU
;
Ya′nan ZHAO
Author Information
1. Department of Plastic Surgery, the Second Affiliated Hospital of Kunming Medical University, Kunming 650101, China
- Publication Type:Case Reports
- Keywords:
Marfan syndrome;
Surgical procedures, operative;
Hand deformities, congenital;
Contracture;
Arachnodactyly
- From:
Chinese Journal of Burns
2018;34(10):728-730
- CountryChina
- Language:Chinese
-
Abstract:
A Marfan syndrome child with contractural arachnodactyly deformity of both hands was admitted to our unit in June 2015. The left and right middle fingers and ring fingers of the child received the continuous " Z" skin flap and " V-Y" plasty to reduce the palmar tension on the skin and release the contracture. Due to the improper therapeutic strategy and the underestimation of effect of underlying lesions of Marfan syndrome on the therapeutic strategy, the right middle finger of child was with ischemic necrosis and amputated, and the finger tip was accompanied with distension and paroxysmal neuropathic pain at a specified future date. The skin flaps of the other three surgical fingers were with local necrosis and scar healing. The child was complicated by distal interphalangeal joints stiffness and some serious complications post-discharge. Combined with this case, the similar disorders are not recommended to carry out one-stage operation.
