5-year follow up after successful craniopagus separation: Review on hydrocephalus and venous system re-arrangement - 06/09/25

Doi : 10.1016/j.neuchi.2025.101717

Samsul Ashari ^a,⁎ , Alvi Aulia ^b, Affan Priyambodo ^a, Setyo Widi Nugroho ^a, Amanda Aldilla ^a
^a Division of Paediatrics, Department of Neurosurgery, Faculty of Medicine Universitas Indonesia, Cipto Mangunkusumo General Hospital, Jakarta, Indonesia
^b Division of Neurosurgery, Department of Surgery, Harapan Kita Mother and Child Hospital, Jakarta, Indonesia

^⁎Corresponding author.

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Highlights

•	Craniopagus twin is very rare, found in only 1 per 2,5 million live births.
•	The presence of shared dural venous sinuses is among the most challenging issues faced in any craniopagus twin separation attempt.
•	Due to venous system anomaly in total vertical craniopagus twin, hydrocephalus is not an uncommon complication after separation.
•	We review craniopagus cases which developed hydrocephalus post-separation surgery and their related venous and arterial system abnormalities. We found all cases with hydrocephalus happened in total type of craniopagus.
•	This is the first paper that reports long-term follow-up after separation surgery accompanied by CT Venography examination. Thus, we could evaluate the venous system re-arrangement after craniopagus separation.

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Abstract

Introduction

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.

Case presentation

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.

Conclusion

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.

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Keywords : Craniopagus, Conjoined twin, Hydrocephalus, Sagittal sinus, Separation surgery