Surgical Management of Sirenomelia: A Case Study.

Autor:	Bhagat ND; From the Indiana University School of Medicine, Indianapolis, Ind., Patel A; From the Indiana University School of Medicine, Indianapolis, Ind., Gross JN; Division of Plastic Surgery, Indiana University School of Medicine, Indianapolis, Ind., Borschel GH; Division of Plastic Surgery, Indiana University School of Medicine, Indianapolis, Ind.; Division of Plastic Surgery, Riley Hospital for Children, Indianapolis, Ind.
Jazyk:	angličtina
Zdroj:	Plastic and reconstructive surgery. Global open [Plast Reconstr Surg Glob Open] 2023 Sep 15; Vol. 11 (9), pp. e5275. Date of Electronic Publication: 2023 Sep 15 (Print Publication: 2023).
DOI:	10.1097/GOX.0000000000005275
Abstrakt:	Background: Sirenomelia is a rare congenital condition characterized by fusion of the lower limbs. Patients with sirenomelia generally do not survive long after birth because the condition is associated with multisystem organ dysfunction due to developmental anomalies. Considering the low incidence and few cases surviving the neonatal period, there is minimal understanding regarding the surgical management of sirenomelia. We present a unique case of an infant born with type 1 sirenomelia, absence of external genitalia, presence of a cloaca, absence of the bladder, and presence of an imperforate and vestigial anus, who not only survived the birth process, but, at the age of 11 months, was determined to be a candidate for surgical separation of the lower extremities. Methods: This case was approached much like a dorsal rectangular flap syndactyly release. Large Z-plasty flaps were designed and raised, and the soft tissue within the skin bridge was meticulously dissected to preserve anatomy and to provide adequate skin flaps without perineal skin grafting. A quadrangular flap was designed to reconstruct the perineum and produce a neo-vulva using de-epithelialization. Results: Successful lower extremity separation was achieved. There were no major postoperative complications. The patient progressed with lower extremity function, and eventually achieved independent ambulation. Conclusions: Management of sirenomelia is incredibly challenging, and data to guide surgical management are limited. This report details our approach to a successful lower extremity separation, repair, and neo-vulvar reconstruction in a case of type I sirenomelia. Competing Interests: The authors have no financial interest to declare in relation to the content of this article. Disclosure statements are at the end of this article, following the correspondence information. (Copyright © 2023 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of The American Society of Plastic Surgeons.)
Databáze:	MEDLINE
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