07 September 2024 : Case report
[In Press] Anesthetic Strategies and Challenges in the Separation of Pygopagus Conjoined Twins: A Case Report
Congenital defects / diseases
Roland N. Kaddoum1AEF, Sahar M. Siddik1AEF, Gloria M. Al Karaki1AEF, Samar J. Mkhayel1AEF, Marie T. Aouad1AEFDOI: 10.12659/AJCR.944776
Am J Case Rep In Press; DOI: 10.12659/AJCR.944776
Available online: 2024-09-07, In Press, Corrected Proof
Publication in the "In-Press" formula aims at speeding up the public availability of the pending manuscript while waiting for the final publication. The assigned DOI number is active and citable. The availability of the article in the Medline, PubMed and PMC databases as well as Web of Science will be obtained after the final publication according to the journal schedule
Abstract
BACKGROUND
Conjoined twins are a rare congenital anomaly with various types, depending on the connection site. Managing these cases requires a structured approach leading to separation surgery. This report provides a detailed description of the anesthetic strategies and challenges of pygopagous conjoined female twins who underwent 2 surgeries before separation at 17 weeks of age.
CASE REPORT
The female twins were conjoined at the sacral level (S4), with fused thecal sacs at L5 and cord tethering, with the conus terminating below L3 in both patients. They had separate rectal ampullae that fused into a single anal canal in the midline in a Y formation. The babies underwent 2 surgeries prior to separation: meningocele repair for twin B and anal canal dilation and sphincter mapping for both twins, followed by a ventriculoperitoneal shunt insertion for twin B. The separation surgery included dividing the bony vertebral fusions and dural sacs, untethering the spinal cords and nerves, and correctly allocating the intrapelvic muscles, guided by sphincter muscle mapping. Anesthesia was managed by 2 distinct teams each time, with duplicated equipment and color-coded medications to prevent errors. Due to the critical condition of twin B, general anesthesia was administered to her first. The report also addresses the challenges faced during the 3 surgical procedures.
CONCLUSIONS
Anesthetic management for pygopagus twins presents complex challenges. Despite limited experience with similar cases, successful management was achieved through planning, effective communication, and rehearsal of unfamiliar setups. Attention to detail and involvement of highly experienced teams were crucial to the success of the procedures.
Keywords: Anesthesia; Anesthetics; Case Reports; Operating Rooms; Pediatrics; Twins, Conjoined
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