Successful staged separation of total angular craniopagus.

Abstract

Total craniopagus is an exceedingly rare condition in which surgical treatment is complex and potentially fatal. Over the last decades, a multistep surgical approach, which allows development of venous collateral circulation, has fostered a dramatic improvement of successful separation rates and neurological outcomes. Most of the experience derives from management of vertical craniopagus, the angular form being rarer and less amenable to successful surgical separation. We present a case of total angular craniopagus twins observed at our Institution. Specific features included a large occipital fusion area with a bone defect, complete separation of brain and arterial vessels and a complex configuration of dural venous sinuses. The superior sagittal sinus of each twin preferentially drained to a single transverse sinus through a shared torcular. After an extensive diagnostic phase, including neuroimaging, tridimensional and virtual reality modelling, neurological, neurophysiological and rehabilitation assessment, a detailed multistep surgical plan, was proposed to a wide multidisciplinary team. The venous system was managed by taking advantage of the fact each twin's superior sagittal sinus was drained preferentially by the transverse sinus on the twin's left. The transverse sinuses were thus separated accordingly. Successful separation was achieved in three surgical steps over one year, with an excellent outcome for both twins.