Anesthetic Strategies and Challenges in the Separation of Pygopagus Conjoined Twins: A Case Report

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.

Figure 1.

Magnetic resonance imaging of pelvis of the conjoined twins at 5 days of life, the arrow shows the tethering of both spinal cords terminating below the level of L3, separate rectal arches with combined single anal canal. In twin B (left side), note is made of syrinx at the level of the cervical and upper thoracic spine, as well as the lumbar spine.

Figure 2.

The conjoined twins at birth. The arrows show the skin communication at the sacral level and retrognathia.

Figure 3.

Caudal view of the conjoined twins showing a single anal opening (white arrow) and fusion of the posterior aspect of the external genitalia.