Surgical Management of Sirenomelia: A Case Study
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...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Wolters Kluwer
2023-09-01
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| Series: | Plastic and Reconstructive Surgery, Global Open |
| Online Access: | http://journals.lww.com/prsgo/fulltext/10.1097/GOX.0000000000005275 |
| _version_ | 1797375049369387008 |
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| author | Neel D. Bhagat, MD Aadarsh Patel, BS Jeffrey N. Gross, MD Gregory H. Borschel, MD |
| author_facet | Neel D. Bhagat, MD Aadarsh Patel, BS Jeffrey N. Gross, MD Gregory H. Borschel, MD |
| author_sort | Neel D. Bhagat, MD |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-08T19:16:36Z |
| format | Article |
| id | doaj.art-83a1b368e2e24311a47f73c611117880 |
| institution | Directory Open Access Journal |
| issn | 2169-7574 |
| language | English |
| last_indexed | 2024-03-08T19:16:36Z |
| publishDate | 2023-09-01 |
| publisher | Wolters Kluwer |
| record_format | Article |
| series | Plastic and Reconstructive Surgery, Global Open |
| spelling | doaj.art-83a1b368e2e24311a47f73c6111178802023-12-27T06:49:45ZengWolters KluwerPlastic and Reconstructive Surgery, Global Open2169-75742023-09-01119e527510.1097/GOX.0000000000005275202309000-00037Surgical Management of Sirenomelia: A Case StudyNeel D. Bhagat, MD0Aadarsh Patel, BS1Jeffrey N. Gross, MD2Gregory H. Borschel, MD3From the * Indiana University School of Medicine, Indianapolis, Ind.From the * Indiana University School of Medicine, Indianapolis, Ind.† Division of Plastic Surgery, Indiana University School of Medicine, Indianapolis, Ind.† Division of Plastic Surgery, Indiana University School of Medicine, Indianapolis, Ind.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.http://journals.lww.com/prsgo/fulltext/10.1097/GOX.0000000000005275 |
| spellingShingle | Neel D. Bhagat, MD Aadarsh Patel, BS Jeffrey N. Gross, MD Gregory H. Borschel, MD Surgical Management of Sirenomelia: A Case Study Plastic and Reconstructive Surgery, Global Open |
| title | Surgical Management of Sirenomelia: A Case Study |
| title_full | Surgical Management of Sirenomelia: A Case Study |
| title_fullStr | Surgical Management of Sirenomelia: A Case Study |
| title_full_unstemmed | Surgical Management of Sirenomelia: A Case Study |
| title_short | Surgical Management of Sirenomelia: A Case Study |
| title_sort | surgical management of sirenomelia a case study |
| url | http://journals.lww.com/prsgo/fulltext/10.1097/GOX.0000000000005275 |
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