Punjabi Ayesha, Kurlander David E, Wee Corinne, Long Tobias C, Gillis Joshua A, Khouri Joseph S
Plastic Surgery, Case Western Reserve University, Cleveland, Ohio.
Plastic Surgery, Western University, London, Ontario, Canada.
Plast Reconstr Surg Glob Open. 2020 Nov 25;8(11):e3146. doi: 10.1097/GOX.0000000000003146. eCollection 2020 Nov.
Patients with the alpha actin 2 genetic mutation suffer early onset aneurismal and vascular-occlusive conditions due to dysfunctional smooth muscle contractility. Outcomes of free flap reconstruction in this patient population are unknown. Here we report the case of a 21-year-old woman with alpha actin 2 mutation who required decompressive hemicraniectomy following an acute stroke. The entire Cushing flap underwent necrosis, requiring debridement and exposing dura. This condition was treated with a free latissimus myocutaneous flap. The patient's post-operative course was complicated by venous thrombosis, requiring intra-flap tPA and revision of the venous anastomosis with a saphenous vein graft. Ultimately the distal 75% of the flap was lost, leaving the dura exposed. The patient's course was further complicated by multiple wound healing complications: large areas of necrosis of the latissimus and saphenous vein donor sites, the neck vessel recipient site, and the right hand after IV infiltration. She ultimately healed with a regenerative tissue matrix strategy. Reconstructive options with no or minimal donor site morbidity should be considered in patients with the alpha actin 2 mutation. We encourage further reporting of outcomes in these patients.
携带α-肌动蛋白2基因突变的患者由于平滑肌收缩功能障碍，会出现早期发作的动脉瘤和血管闭塞性疾病。该患者群体中游离皮瓣重建的结果尚不清楚。在此，我们报告一例21岁携带α-肌动蛋白2基因突变的女性病例，该患者在急性中风后需要进行减压性颅骨切除术。整个库欣皮瓣发生坏死，需要清创并暴露硬脑膜。这种情况采用游离背阔肌肌皮瓣进行治疗。患者术后出现静脉血栓形成并发症，需要在皮瓣内使用组织型纤溶酶原激活剂（tPA）并采用大隐静脉移植对静脉吻合进行修复。最终，皮瓣远端75%坏死，硬脑膜暴露。患者的病程因多种伤口愈合并发症而进一步复杂化：背阔肌和大隐静脉供区、颈部血管受区以及静脉输液渗漏后右手出现大面积坏死。她最终采用再生组织基质策略愈合。对于携带α-肌动蛋白2基因突变的患者，应考虑选择供区发病率低或无供区发病率的重建方案。我们鼓励进一步报告这些患者的治疗结果。
