手术治疗一名 3 岁儿童的先天性甲状腺发育不全。

IF 1.3 Q3 SURGERY
Archives of Plastic Surgery-APS Pub Date : 2023-12-21 eCollection Date: 2023-11-01 DOI:10.1055/s-0043-1771520
Jack D Sudduth, Christopher Clinker, Matthew Holdaway, Jessica L Marquez, Jacob Veith, Thomas Wright, W Bradford Rockwell
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引用次数: 0

摘要

先天性甲沟炎是一种罕见的遗传性疾病,其特征是甲板肥厚、甲床角化过度、手指和脚趾的下趾骨增厚,从而影响手的灵活性,并导致美观不佳。现有文献介绍了各种治疗方法,但没有一种方法被定义为黄金标准。在本病例中,作者采用了不同的手术技术来治疗先天性趾骨粗大症,以评估最有效的方法。一名 3 岁的男孩出现了肥厚性指甲增生,涉及双手和双脚的所有手指。对患者的手指和脚趾进行了三种手术,分别是单纯胚芽基质切除术(GME)、胚芽基质切除术加部分无菌基质切除术(pSME)或胚芽基质切除术加完全无菌基质切除术(cSME)。接受 GME + cSME 治疗的手指指甲生长没有复发。而只接受 GME 治疗的患者,虽然生长速度减慢,但增生的指甲仍会复发。切除 GME + cSME 可防止增生性指甲复发,而单独切除 GME 或同时切除 pSME 则会导致增生性指甲生长缓慢。完全切除生发基质和无菌基质并植皮闭合可能是治疗先天性甲沟炎的最终方法,但还需要进一步的研究来验证这些发现。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Surgical Management of Pachyonychia Congenita in a 3-Year-Old.

Pachyonychia congenita is a rare genetic disorder characterized by hypertrophic nail plates, hyperkeratotic nail beds, and thickened hyponychium of the fingers and toes, impairing manual dexterity and resulting in poor aesthetics. The current body of literature describes various treatment modalities, but no singular approach has been defined as the gold standard. In this case, the authors employed different surgical techniques for treating pachyonychia congenita to evaluate the most effective approach. A 3-year-old boy presented with hypertrophic nail growth involving all digits of both hands and feet. Three surgical procedures were performed on the patient's fingers and toes using germinal matrix excision (GME) alone, GME plus partial sterile matrix excision (pSME), or GME plus complete sterile matrix excision (cSME). The digits treated with GME + cSME exhibited no recurrence of nail growth. Those treated with GME alone exhibited recurrence of hypertrophic nail growth, although their growth slowed. Excision of GME + cSME prevented recurrence of hypertrophic nails, while GME alone or with pSME led to slower-growing hypertrophic nails. Complete excision of the germinal and sterile matrices with skin graft closure may be a definitive treatment for pachyonychia congenita, but further studies are needed to validate these findings.

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来源期刊
CiteScore
2.10
自引率
6.70%
发文量
131
审稿时长
10 weeks
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