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Brain Advance Access published online on April 5, 2006
Brain, doi:10.1093/brain/awl065
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© The Author 2006. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org. The online version of this article has been published under an open access model. Users are entitled to use, reproduce, disseminate, or display the open access version of this article for non-commercial purposes provided that: the original authorship is properly and fully attributed; the Journal and Oxford University Press are attributed as the original place of publication with the correct citation details given; if an article is subsequently reproduced or disseminated not in its entirety but only in part or as a derivative work this must be clearly indicated. For commercial re-use, please contact journals.permissions@oxfordjournals.org
Received December 15, 2005
Revised February 20, 2006
Accepted February 21, 2006

Review Article

The craniopagus malformation: classification and implications for surgical separation

James L. Stone 1 * and James T. Goodrich 2

1 Departments of Neurological Surgery and Neurology, University of Illinois at Chicago, and Division of Neurosurgery, Cook County Hospital, Chicago, IL, USA
2 Division of Pediatric Neurological Surgery, Montefiore Children's Hospital, Albert Einstein College of Medicine, Bronx, New York, NY, USA

* To whom correspondence should be addressed.
James L. Stone, E-mail: jlstone4{at}aol.com


  Abstract

Craniopagus twins (CPT) are an uncommon, highly fascinating accident of nature. The clinical pathology of this complex entity is reviewed and placed in perspective. A logical classification aids understanding of the anomaly, and is essential to gauge outcome from separation attempts. ‘Partial forms’ lack significant shared dural venous sinuses (SDVS) and ‘Total forms’ with SDVS also exhibit more severe compressional brain distortion. Our classification consists of Partial Angular (PA), Partial Vertical (PV), Total Angular (TA) and Total Vertical (TV, formerly O'Connell Types I-III). Total vertical has a continuous cranium, and inter-twin axial facial rotation <40° (Type I), 140-180° (Type II) or intermediate (Type III). The term ‘Angular’ denotes an inter-twin longitudinal angle below 140°, regardless of axial rotation. Our review categorized 64 well-delineated CPT, including 41 operative separation attempts in small children since initial success in 1952. Just over one-half were TV, almost one-third TA, and partial forms accounted for the remaining one-sixth. About 30% of CPT had shared or fused brain tissue, and a similar percentage of TA twins shared a posterior fossa. Partial forms had significantly higher birth weights, were separated at an earlier age (6 versus 11 months) and had lower mortality and better outcome compared with Total forms. A multi-staged surgical separation for Total CPT had a significantly better mortality than single-staged separation. Discussion emphasizes embryological, anatomical and clinical aspects of the malformation, with emphasis upon obstacles to a successful outcome.

Keywords: craniopagus twins; dural venous sinuses; surgery of craniopagus; embryology; pediatric neurosurgery.
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