Hadjimichael Argyris C, Kaspiris Angelos, Spyridonos Sarantis
Medical School, National and Kapodistrian University of Athens School of Medicine, Athens, GRC.
Upper Limb, Hand, and Microsurgery Department, "KAT" General Hospital, Athens, GRC.
Cureus. 2022 Mar 21;14(3):e23357. doi: 10.7759/cureus.23357. eCollection 2022 Mar.
Hand macrodactyly is a very scarce deformity. It was first described over 200 years ago and was characterized as "local gigantism" of one or multiple digits. Benign bone overgrowth, massive increase of soft tissue volume, and nerve involvement are associated with hand macrodactyly have been consistently reported in the literature. Often, macrodactyly affects one or more digits and is further classified as static or progressive, depending on the growth pattern, and as sporadic or syndromic, according to its genetic predisposition. Surgical treatment for hand macrodactyly remains a complex issue even for expert hand surgeons. In most of the cases, macrodactyly is diagnosed during early childhood and can be appropriately managed with minimal and well affordable surgical approaches that stabilize its fast progression. However, adults with progressive hand macrodactyly develop advanced deformities leading to severe functional deterioration and aesthetic hand dysmorphia. The purpose of this report is to document the management and surgical approach of the oldest published case, a 60-year-old adult patient with neglected progressive hand macrodactyly despite previous surgical attempts for disease stabilization. A personalized preoperative planning was created, which included ray resection involving the fourth metacarpal and fourthfinger along with extensive debulking of the overgrown fatty soft tissue and carpal tunnel release. At six months' follow-up, the patient reported an excellent aesthetic and functional outcome.
手部巨指症是一种非常罕见的畸形。它在200多年前首次被描述,其特征为一个或多个手指的“局部巨大症”。文献中一直报道,良性骨过度生长、软组织体积大量增加以及神经受累与手部巨指症相关。通常,巨指症会影响一个或多个手指,并根据生长模式进一步分为静止型或进展型,根据遗传易感性分为散发性或综合征性。即使对于手部外科专家而言,手部巨指症的手术治疗仍然是一个复杂的问题。在大多数情况下,巨指症在儿童早期被诊断出来,并且可以通过最小且费用合理的手术方法进行适当治疗,以稳定其快速进展。然而,患有进展性手部巨指症的成年人会出现严重畸形,导致严重的功能恶化和手部美观畸形。本报告的目的是记录已发表的年龄最大病例的治疗和手术方法,该病例为一名60岁成年患者,尽管之前曾尝试通过手术稳定病情,但仍患有被忽视的进展性手部巨指症。制定了个性化的术前规划,包括切除涉及第四掌骨和第四指的射线,同时广泛切除过度生长的脂肪软组织并进行腕管松解。在六个月的随访中,患者报告了极佳的美观和功能结果。