Management of Children with Megafoot Secondary to Proteus Syndrome: A Report of Three Cases with Long-Term Follow-Up.

IF 0.5 4区 医学 Q4 ORTHOPEDICS
Mehmet Demirel, Dağhan Koyuncu, Yavuz Sağlam, Fuat Bilgili, Önder İsmet Kılıçoğlu, Hayati Durmaz
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引用次数: 0

Abstract

Background: Proteus syndrome is a rare disorder characterized by overgrowth of limbs and organs and neurocutaneous findings.

Methods: We examined three Proteus syndrome cases with unilateral foot enlargement, megafoot. The patients had ambulatory and cosmetic difficulties. Debulking surgery was performed.

Results: After the surgeries, patient mobility and functional scores were analyzed and found to be improved; only one patient had recurrence after 5 years. In the literature, there is no consensus about the treatment and the timing of treatment of Proteus syndrome. The literature suggests that early surgery for megafoot leads to better functional results. Debulking surgery and amputation are the two options mentioned. In the present cases, soft-tissue debulking was performed.

Conclusions: In patients with megafoot caused by Proteus syndrome, the functional results were better for those with early debulking surgery compared with those who did not undergo surgery. Patients were able to fit into shoes and walk without difficulty.

继发于变形虫综合征的巨足症患儿的治疗:三例病例的长期随访报告。
背景:普罗特斯综合征是一种罕见的疾病,以肢体和器官过度生长以及神经皮肤症状为特征:普罗特斯综合征是一种罕见的疾病,其特征是肢体和器官过度生长,并伴有神经皮肤症状:我们研究了三例普特斯综合征病例,他们都患有单侧足部肿大(巨足)。方法:我们对三例普特斯综合征患者进行了研究,他们都患有单侧足部肿大(巨足),患者行动不便,外观难看。结果:手术后,患者活动自如:手术后,对患者的活动能力和功能评分进行了分析,结果发现患者的情况有所改善;5 年后,只有一名患者复发。在文献中,关于 Proteus 综合征的治疗方法和治疗时机尚未达成共识。文献表明,巨足早期手术可获得更好的功能效果。去骨手术和截肢是提到的两种选择。本病例采用的是软组织剥脱术:结论:对于由变形虫综合征引起的巨足患者,与未接受手术的患者相比,早期接受去骨赘手术的患者功能效果更好。患者能够穿上鞋子,行走自如。
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来源期刊
CiteScore
1.10
自引率
0.00%
发文量
128
审稿时长
6-12 weeks
期刊介绍: The Journal of the American Podiatric Medical Association, the official journal of the Association, is the oldest and most frequently cited peer-reviewed journal in the profession of foot and ankle medicine. Founded in 1907 and appearing 6 times per year, it publishes research studies, case reports, literature reviews, special communications, clinical correspondence, letters to the editor, book reviews, and various other types of submissions. The Journal is included in major indexing and abstracting services for biomedical literature.
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