5-year follow up after successful craniopagus separation: Review on hydrocephalus and venous system re-arrangement

Craniopagus is one of the rarest congenital abnormalities. Separation of craniopagus twin is associated with high morbidity and mortality, especially in total type, where the twin had shared dural venous sinuses. One of the complications after separation surgery is hydrocephalus. While detailed cerebral vasculature imaging is needed pre-operatively to ensure most optimal surgical approach, post-operative vasculature imaging is no less important to assess changes in cerebral venous system after separation surgery.

Separation surgery was successfully accomplished in a total vertical craniopagus twin with shared dural venous sinuses. One twin experienced hydrocephalus after surgery, while the other twin had CSF leakage from the wound. LP shunt was placed in both twin and they had good recovery. We then compared the cerebral venous structure in both twins, before and after separation surgery using reconstruction of CT venography.

Successful separation of total vertical craniopagus twin where both twin developed well without any neurological deficit is a very rare occurrence. Based on follow up CTV, cerebral venous system underwent re-arrangement to accommodate changing hemodynamic needs after separation surgery. This might give us new insight about cerebral venous system that favors good prognosis for craniopagus twin.