Multidisciplinary Surgical Approach to a Surviving Infant With Sirenomelia

Sirenomelia is an extremely complex and rare malformation with different degrees of lower-extremities fusion associated with gastrointestinal, musculoskeletal, vascular, cardiopulmonary, and central nervous system malformations. In the English literature, there are only 5 reports of infants survivin...

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Bibliographic Details
Published in:Pediatrics (Evanston) 2006-07, Vol.118 (1), p.e220-e223
Main Authors: Messineo, Antonio, Innocenti, Marco, Gelli, Riccardo, Pancani, Simone, Lo Piccolo, Roberto, Martin, Alessandra
Format: Article
Language:English
Subjects:
Abnormalities, Multiple - diagnostic imaging
Abnormalities, Multiple - surgery
Anal Canal - abnormalities
Calcaneus - abnormalities
Chondroitin Sulfates - therapeutic use
Collagen - therapeutic use
Colon - abnormalities
Ectromelia - embryology
Ectromelia - surgery
Female
Hip - abnormalities
Humans
Ileostomy
Infant, Newborn
Intestinal Atresia - surgery
Leg - abnormalities
Leg - surgery
Patient Care Team
Reconstructive Surgical Procedures
Skin Transplantation
Skin, Artificial
Spine - abnormalities
Tissue Expansion
Tomography, X-Ray Computed
Vagina - abnormalities
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Summary:Sirenomelia is an extremely complex and rare malformation with different degrees of lower-extremities fusion associated with gastrointestinal, musculoskeletal, vascular, cardiopulmonary, and central nervous system malformations. In the English literature, there are only 5 reports of infants surviving with this condition. In our case, a 2540-g female infant was born with normal vital signs, no facial dysmorphism, and a complete soft tissue fusion of the lower limbs, from perineum to ankles. Radiologic examinations revealed an intestinal atresia and a single pelvic kidney, with a unique ureter, 2 femurs, 2 tibias, 2 fibulas, and 2 feet (simpus dipus). At 7 months of age, a multidisciplinary surgical team achieved complete separation of the lower limbs, with independent vascular and nerve supplies. At the time of this writing, the infant was 28 months old and had a regular growth curve. Many future reconstructive surgeries have been planned to achieve an acceptable quality of life for this infant.
ISSN:0031-4005
1098-4275
DOI:10.1542/peds.2005-3001