Case Study of "Once in a Lifetime" Patients- Pygopagus Conjoined Twins

Background: Seven-hour-old pygopagus conjoined male twins were admitted to our NICU. Initial examination revealed imperforate anus. Twin A was found to have situs inversus totalis, single kidney and  heart defect. Twin B was diagnosed with hydrocephalus, Dandy-Walker syndrome and severe heart defect. They also had a shared lumbar spinal cord and column and common rectum.

Problems: Colostomy location would be very important due to the limited positioning options for these babies. Special positioning aids would be needed to prevent pressure ulcers and to help maintain near-normal body alignment. Continence would likely be a future problem because of their imperforate anus and the likelihood of neurogenic bowel and bladder.

Clinical Course: On day 1 the twins were marked for their colostomies, taking into account anatomical landmarks, positioning limitations and Twin A’s situs inversus. On day 2, they had their first surgeries, for colostomies and gastrostomy tubes. Initial stoma pouching was done using preemie pouches with ports, which allowed the pouches to be emptied even when the babies were positioned "stoma down". Because of the frequent pressure and tension on the feeding tubes, stomal erosion and granulation tissue were problems. Silicone-adhesive foam dressings were used to stabilize the tubes and absorb drainage. Fluidized positioners and gel pillows were used for pressure redistribution and body alignment. Positioning became more important after tissue expanders were implanted. Skin breakdown over one expander was treated with a silver-impregnated foam dressing. Anal reconstruction was done as part of the separation surgery. After separation surgery there was some necrosis of the muscle flaps. Twin B required NPWT and a skin graft to heal his remaining wound.

Outcome: Both twins have "near-perfect" stoma sites. Neither has had a pressure ulcer. All surgical wounds have now healed. Ostomy closure is pending.