Emergency separation of craniopagus twins: case report

Abstract

<jats:p>Craniopagus is a very rare congenital anomaly that tends to affect females more often than males. It is classified as partial or total. Most affected twins are either stillborn or die during the perinatal period. Those who survive birth should undergo detailed radiological evaluations soon after their condition becomes stable so that the precise anatomy of the conjoined part can be defined and surgery can be planned in detail by a multidisciplinary team. Recommendations for decreasing the risk of unsuccessful surgery include performing surgery on an elective basis after extensive preoperative radiological evaluations as well as when the twins are at an acceptable age and weight for a complex surgical separation, generally as staged procedures. In addition, the operation should be performed by a well-equipped expert multidisciplinary team.</jats:p><jats:p>When one of the conjoined twins dies, however, surgical separation cannot be postponed because the shared circulatory system predisposes the alive child to life-threatening complications, including coagulopathy. The authors report on the successful separation of craniopagus twins performed on an emergency basis at 32 weeks of gestational age because of the sudden death of one of them. At the time of separation surgery, the twins each weighed 1250 g. To the best of the authors’ knowledge, this is the youngest age and lowest weight yet reported for successful surgical separation. The surviving twin developed a pseudomeningocele, which required a second operation and placement of a cystoperitoneal shunt 4 months after the operation. Additional surgery is planned to repair a cranial defect that resulted from the pseudomeningocele, but his general physical and mental condition was otherwise good at latest follow-up (12 months after separation surgery).</jats:p>

Preoperative Evaluation of Craniopagus Twins: Anatomy, Imaging Techniques, and Surgical Management

“…9,41 There have been some reported cases of shared cerebral arterial supply, such as the shared middle cerebral artery circulation, which increases the complexity of a separation and may preclude safe separation. 8,42 There are a variety of cerebral venous abnormalities associated with total CPT. Because total CPT lack 2 complete dural envelopes, the peripheral dural shelf at the conjoined cerebral hemispheric zone encloses a circumferential venous sinus (CVS) (Fig 4).…”

“…3D reconstruction of the CT data is helpful to evaluate the extent of bone fusion (Fig 7A). 8 These data can then be reconstructed into a 3D model, which can be used by the surgeon and radiologist in preoperative planning and intraoperative reference. 42 CT angiography and venography provide adequate information related to vascularity in the conjoined area (Fig 7B , -7C), with CT venography of paramount importance in presurgical planning before each stage of separation.…”

“…4 Although there is high prenatal and postnatal mortality for CPT, successful separation has become more common due to advances in neuroimaging, neuroanesthesia, and neurosurgical techniques. [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] Joined brain tissue, shared arteries and veins, and defects in the skull and dura make surgery technically challenging. 21 Separation can take place in single or multistage procedures and has evolved as the understanding of the physiology, surgical techniques, and technology of CPT have improved.…”

Editorial. Separation of craniopagus twins

“…he authors have detailed a very rare case of a set of craniopagus twins who were born at 28 weeks' gestational age and shortly after birth required an emergency surgical separation due to the rapid and unexpected death of one twin. 1 The authors provide a comprehensive literature review and point out that this appears to be a case with the youngest known age for separation where one twin survived the surgery and at 12 months' follow-up appears to be doing well. The medical details and surgical planning are well detailed along with the complexity of this type of frontal angular craniopagus type.…”

Multiparametric Imaging for Presurgical Planning of Craniopagus Twins: The Experience of Two Tertiary Pediatric Hospitals with Six Sets of Twins

“…This planning phase is typically an elective and prolonged process unless one of the twins dies. If this occurs, then expedient separation is necessary to prevent coagulopathy and related lethal complications in the surviving twin (7).…”