Emergency neurosurgical separation of pygopagus conjoined twins in a triplet pregnancy: illustrative case.

BACKGROUND

Conjoined twins are a rare type of congenital malformation. The point of attachment is the primary factor used to classify conjoined twins; typically, this is front to front, with thoracopagus and omphalopagus twins accounting for about 75% of cases and pygopagus twins only between 6% and 19%.

OBSERVATIONS

This is a case report of 46-day-old female triplets born to a 37-year-old para 4 mother. Triplets A and B were conjoined at the lumbosacrococcygeal area dorsally. Triplet C was not conjoined. The conjoined triplets had a low birth weight; hence, it was decided to start nutritional management and achieve adequate weight gain before surgical separation. While on nutritional management, at 46 days of age, triplet B developed cardiorespiratory failure. Therefore, the multidisciplinary team decided to proceed with emergency neurosurgical separation surgery, with the primary aim of saving triplet A. Triplet B died 7 hours after the surgery due to irreversible cardiorespiratory failure, and triplet A remained in the hospital for 14 days and was discharged with intact neurological function.

LESSONS

Most cases of conjoined twins do not require emergency separation surgery and need nutritional support before the separation procedure. Separation surgery requires a multidisciplinary team meeting, planning ahead of time, and preparation. https://thejns.org/doi/10.3171/CASE24818.