Abstract
Case report
Twin boys joined at the head in a total vertex configuration were born in Egypt in June 2001. At 12 months, they were transported to Dallas for evaluation and eventual surgical separation. In Dallas, a large multidisciplinary team of health care providers from two pediatric hospitals was assembled to care for the boys. Extensive radiographic evaluation revealed that the twins had essentially separate, well-formed brains, each with regions of schizencephaly. Each child’s left cerebral hemisphere drained most of the venous blood to the right jugular system of the other. A detailed assessment of the foreseeable risks of surgical separation was then estimated and presented to the parents, as well as to the ethics committee of the two institutions. The decision was then made to proceed with separation. Surgical planning included the construction of multiple polymer models, and the design and construction of customized head holders and an operating table. Prior to separation a series of preparatory operations were performed to expand the scalp, as well as the fascia lata for dural grafting. At the age of 28 months, the twins were successfully separated during a 33-h operation. No attempt was made to reconstruct the dural venous sinuses. Scalp closure was adequate, requiring a split-thickness skin graft on one boy.
Outcome
Postoperatively each child demonstrated an incomplete right hemiparesis. One twin required cerebral spinal fluid shunting. Neither child had a CSF leak or a CSF infection. At 6 months follow-up, both boys are rapidly acquiring speech in both English and Arabic, motor function is improving, and both are progressing toward independent ambulation.
Similar content being viewed by others
Notes
The word craniopagus is singular and refers to a set of twins conjoined at the head. Since the term refers to both children as one entity its use is limited in this article
The authors would like to acknowledge the generous expert advice provided by Drs’ Keith Goh, John Frazee, Jorge Lazareff, Barbara Van de Wiele, Duke Samson, Marion Walker, Jonathan Peter, Dachling Pang, Ken Winston, and others
References
Winston KR (1987) Craniopagi: anatomical characteristics and classification. Neurosurgery 21:769–781
Christensen AM, Humphries SM, Goh KYC, Swift DM (2004) Advanced “tactile” medical imaging for separation surgeries of conjoined twins. Childs Nerv Syst http://dx.doi.org/10.1007/s00381-004-0982-7
Winston KR, Rockoff MA, Mulliken JB, Strand RD, Murray JE (1987) Surgical division of craniopagi. Neurosurgery 21:782–791
Khan ZH, Tabatabai SA, Saberi H (1999) Anesthetic and surgical experience in a case of total vertical craniopagus. Surg Neurol 52:62–66
Todorov AG, Cohen KL, Spilotro V, Landau E (1974) Craniopagus twins. J Neurol Neurosurg Psychiatry 37:1291–1298
Dallas Morning News, Sisters were joyful in 43 years together, 8/24/2003 Page 8W
Goh KYC (2004) Separation surgery for total vertex craniopagus twins. Childs Nerv Syst http://dx.doi.org/10.1007/s00381-004-0978-3
Krauthammer C (2003) A doctor’s duty. Time 162:80
Acknowledgments
Although the success of this case is attributed to the aforementioned concepts and techniques, in the end the undertaking would have been impossible without the massive team effort incorporating the combined resources of Dallas’ two pediatric hospitals. The authors would like to acknowledge the support of the Children’s Medical Center of Dallas, and the North Texas Hospital for Children at Medical City Dallas in this endeavor.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Swift, D.M., Weprin, B., Sklar, F. et al. Total vertex craniopagus with crossed venous drainage: case report of successful surgical separation. Childs Nerv Syst 20, 607–617 (2004). https://doi.org/10.1007/s00381-004-1011-6
Received:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-004-1011-6