ACTA2 Mutation: Microsurgeon Beware!

Summary. 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...

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Bibliographic Details
Main Authors: Ayesha Punjabi, MD, David E. Kurlander, MD, Corinne Wee, MD, Tobias C. Long, MD, Joshua A. Gillis, MD, FRCSC, Joseph S. Khouri, MD
Format: Article
Language:English
Published: Wolters Kluwer 2020-11-01
Series:Plastic and Reconstructive Surgery, Global Open
Online Access:http://journals.lww.com/prsgo/fulltext/10.1097/GOX.0000000000003146
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Summary:Summary. 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.
ISSN:2169-7574