Successful staged separation of total angular craniopagus

Area of Science:

• Neurosurgery
• Pediatric Surgery
• Congenital Anomalies

Background:

• Total craniopagus is a rare conjoined twin condition with high surgical risks.
• Multistep surgical approaches improve outcomes by developing venous collateral circulation.
• Angular craniopagus is less common and more challenging than vertical craniopagus.

Observation:

• A case of total angular craniopagus twins presented with occipital fusion and complex dural venous sinuses.
• Each twin's superior sagittal sinus drained preferentially to a single shared transverse sinus.
• The twins had a large occipital fusion area with a bone defect and separated cerebral vasculature.

Findings:

• A multidisciplinary team designed a detailed multistep surgical plan utilizing advanced imaging and modeling.
• The surgical strategy leveraged the preferential venous drainage of each twin's superior sagittal sinus.
• Separation of the transverse sinuses was performed based on the identified venous anatomy.

Implications:

• This case demonstrates the feasibility of successful surgical separation for total angular craniopagus.
• The multistep approach, focusing on venous system management, offers a viable strategy for complex craniopagus cases.
• Achieving excellent outcomes highlights the importance of meticulous planning and multidisciplinary collaboration in separating conjoined twins.