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 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.
Messineo
Antonio
A
Department of Pediatric Surgery and Burn Unit, Anna Meyer Children's Hospital, Via Luca Giordano 13, 50132 Florence, Italy. a.messineo@meyer.it
Innocenti
Marco
M
Gelli
Riccardo
R
Pancani
Simone
S
Lo Piccolo
Roberto
R
Martin
Alessandra
A
D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging / Q000601:surgery*; D001003:Anal Canal / Q000002:abnormalities; D002111:Calcaneus / Q000002:abnormalities; D002809:Chondroitin Sulfates / Q000627:therapeutic use; D003094:Collagen / Q000627:therapeutic use; D003106:Colon / Q000002:abnormalities; D004480:Ectromelia / Q000196:embryology / Q000601:surgery*; D005260:Female; D006615:Hip / Q000002:abnormalities; D006801:Humans; D007081:Ileostomy; D007231:Infant, Newborn; D007409:Intestinal Atresia / Q000601:surgery; D007866:Leg / Q000002:abnormalities* / Q000601:surgery; D010348:Patient Care Team; D019651:Plastic Surgery Procedures; D016038:Skin Transplantation; D017768:Skin, Artificial; D013131:Spine / Q000002:abnormalities; D015626:Tissue Expansion; D014057:Tomography, X-Ray Computed; D014621:Vagina / Q000002:abnormalities
C114835:integra artificial skin; D002809:Chondroitin Sulfates; D003094:Collagen