- 期刊
Surgical Reconstruction in Apert Hand -- Planning and Execution
亞伯氏手部畸形重建－計畫及執行
摘要
Background: Apert syndrome is a rare genetic disease featuring multiple body malformations, including acrocephalosyndactyly of the craniofacial and hands. Three different types of Apert hand which range in severity, have been described in literature. Multiple staged surgeries are often needed in order to achieve a five-digit hand. Aim and Objectives: This retrospective study analyzed the surgical plan and flap design of patients with Apert hand in Taichung Veterans General Hospital. The comprehensive experience has been reviewed and discussed in literature, with the hopes this will provide appropriate treatment options for patients with Apert hand in the future. Materials and Methods: From January 1996 to December 2019 a total of four patients were identified who fit our study criteria and were subsequently selected for retrospective analysis. The type of Apert hand classification, timing of each surgery, details of surgical design and its relation to the type of Apert hand classifications were all recorded. Results: A total of one Upton type I, two Upton type II, and one Upton type III Apert hand patients were evaluated. The Upton type I and a Upton type II patient received a total of two surgeries prior to achieving bilateral five digit hands. The remaining Upton type II and the Upton type III patient received four surgeries. No digits were sacrificed. All patients received first web space revision surgery except the Upton type I patient. No major complications which required surgical room revision occurred. Surgical design included a dorsal rectangular flap at the proximal phalanx base for division of the web, followed by a straight line incision to the finger tip where a Buck-Gramcko pulp flap was used. An additional straight line incision followed the flap to an inverted T at the metacarpophalangeal crease. A full thickness skin graft was always necessary on the lateral side of the divided fingers after separation of syndactyly. Conclusion: From our study, we determined that for all types of Apert hand a five-digit hand is achievable using the timeline and procedure design provided.
並列摘要
背景：亞伯氏症是一種罕見的先天性遺傳疾病合併多種身體畸形，包括顱顏部及手部的骨骼變異合併併指。文獻描述了三種不同類型的亞伯氏症手畸形，其嚴重程度範圍不一。為了獲得完整五隻手指，多階段重建手術通常是必須的。目的及目標：這項回顧性研究分析了台中榮民總醫院中亞伯氏症手畸形病患的手術計劃和皮瓣設計。將我們整體的手術經驗與文獻進行回顧和探討，希望將來能為亞伯氏症手患者提供更合理的治療選擇。材料及方法：自1996年1月至2019年12月，總共四名符合我們研究標準的患者進行回顧性分析。分析方法包含探討手術記錄中亞伯氏症手畸形分類的類型，每次手術的時間，手術設計的細節及其與亞伯氏症手畸形分類類型的關係。結果：四名患者分別為一名Upton I型，二名Upton II型和一名Upton III型亞伯氏症手部患者。一名Upton I型和一名Upton II型患者總共接受了兩次手術之後達到雙側完整五隻手指。另一位Upton II型和Upton III型患者接受了四次手術。除一名Upton I型患者外，所有患者均接受了後續第一指縫加深手術。術中沒有任何手指被犧牲且並未發現重大併發症。外科手術設計包括在指骨近端底部創造一個矩形背側皮瓣以隔開指縫，然後於指間沿直線切開至指尖，並使用Buck gramcko皮瓣包覆指尖。之後順著兩手指中線再次沿直線切開並於掌指摺痕處創造一個倒T傷口。分指後之指縫兩側需進行全層植皮手術以覆蓋皮膚不足處。結論：從我們的研究中可以看出，按照我們所曾進行的手術計劃和皮瓣設計，對於所有類型的亞伯氏症手畸形，都可以達到完整五隻手指的結果。