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Surgical Management of Sirenomelia: A Case Study
Neel Bhagat BS, Aadarsh Patel BS, Jeffrey Gross MD, Gregory Borschel MD
Indiana University School of Medicine
2023-01-29
Presenter: Aadarsh Patel
Affidavit:
I certify that the material proposed for presentation in this abstract has not been published in any scientific journal or previously presented at a major meeting. All work on this project represents the original work of the students, residents, and faculty involved with the project.
Director Name: William A Wooden
Author Category: Medical Student
Presentation Category: Clinical
Abstract Category: General Reconstruction
Introduction:
Sirenomelia is a rare congenital condition characterized by fusion of the lower limbs. Patients generally do not survive long after birth, as the condition is associated with multi-system organ dysfunction due to developmental anomalies. Considering the low incidence and few cases surviving the neonate period, there is minimal understanding surrounding the surgical management of Sirenomelia. We present a unique case of an infant born with Sirenomelia, who not only survived the birth process, but at age 11 months, was determined to be a candidate for surgical separation of the lower extremities.
Report of Case:
The patient is an 11-month-old female born with simple (non-bony) fusion of the lower limbs, absence of external genitalia, presence of a cloaca and no bladder, and presence of an imperforate and vestigial anus. CT imaging revealed no bony involvement of the fusion and adequate vasculature, indicative of type I Sirenomelia. 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 provide adequate skin flaps without perineal skin grafting. A quadrangular flap was designed to reconstruct the perinium and produce a neo-vulva using de-epithelization.
Conclusions
Management of Sirenomelia is incredibly challenging. There is limited data to guide surgical management of affected patients. This report details our approach to a successful lower extremity separation, repair, and neo-vulvar reconstruction in a case of type I Sirenomelia.
