Total Craniopagus Twins

Villarejo, F.; Soto, Mirtha G.; Amaya, C.; Pascual-Castroviejo, I; Morales, Carlos Enrique Díaz

doi:10.1159/000119977

Cited by 7 publications

(5 citation statements)

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“…Careful case selection for surgery, routine use of 3D print brain and skull models, simulation software, neuronavigation assistance, intraoperative adjuncts/tools, endovascular interventions, innovative surgical methods (distraction osteogenesis), multistage surgeries, and advanced perioperative intensive care availability enable better outcomes. Over the past 20 years, both twins survived in 21 of 32 separation attempts (65.6%) compared with 12 of 30 separation earlier (40%) 16,28-53 (Table 1).…”

Section: Discussionmentioning

confidence: 99%

“…Death of one of twins during craniopagus twin separation surgery: Of 30 CPT separation attempts in the past century, one twin died in 4 twin set surgeries. Of 32 CPT separation attempts in the past 20 years, one twin died in 9 CPT cases (in 4 cases, death resulted from intraoperative massive blood loss and directional venous drainage/shunting, in 3 cases, one twin died before surgery because of other congenital cardiac/renal anomalies, and in 2 cases, cause of death not reported) 16,28-53 (Table 1).…”

Section: Discussionmentioning

confidence: 99%

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A Century With Craniopagus Twin Separation Surgeries: Nihilism to Optimism

et al. 2022

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Craniopagus conjoined twins are extremely rare, reported 1 in 2.5 million live births. To date, 62 separation attempts in 69 well-documented cases of craniopagus twins have been made. Of these, 34 were performed in a single-stage approach, and 28 were attempted in a multistage approach. One or both twins died of massive intraoperative blood loss and cardiac arrest in 14 cases. We report our surgical experience with conjoined craniopagus twins (JB) with type III total vertical joining and shared circumferential/circular sinus with left-sided dominance. A brief review of the literature is also provided. In our twins, the meticulous preoperative study and planning by the multidisciplinary team consisting of 125-member, first-staged surgical separation consisted of creation of venous conduit to bypass part of shared circumferential sinus and partial hemispheric disconnection. Six weeks later, twin J manifested acute cardiac overload because of one-way fistula development from blocked venous bypass graft necessitating emergency final separation surgery. Unique perioperative issues were abnormal anatomy, hemodynamic sequelae from one-way fistula development after venous bypass graft thrombosis, cardiac arrest after massive venous air embolism requiring prolonged cardiopulmonary resuscitation, and return of spontaneous circulation at 15 minutes immediately after separation. This is the first Indian craniopagus separation surgery in a complex total vertical craniopagus twin reported by a single-center multidisciplinary team. Both twins could be sent home, but one remained severely handicapped. Adequate perioperative planning and multidisciplinary team approach are vital in craniopagus twin separation surgeries.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

A Century With Craniopagus Twin Separation Surgeries: Nihilism to Optimism

et al. 2022

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“…9,29,47 Total Angular CPT Total angular (TA) CPT have more acute intertwin longitudinal angulation and SDVS accompanied by complex, interconnecting venous channels (or CVS) and markedly distorted cerebral hemispheres. [48][49][50][51][52] One twin's brain can be inside the other twin's calvaria, adding additional to the complexity of the surgical separation. Cerebral compaction, distortion, and displacement may result in skull base deformity.…”

Section: Partial Cptmentioning

confidence: 99%

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

Goldman‐Yassen

Goodrich

Miller

et al. 2020

AJNR Am J Neuroradiol

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Craniopagus twins are a rare congenital malformation in which twins are conjoined at the head. Although there is high prenatal and postnatal mortality for craniopagus twins, successful separation has become more common due to advances in neuroimaging, neuroanesthesia, and neurosurgical techniques. Joined brain tissue, shared arteries and veins, and defects in the skull and dura make surgery technically challenging, and neuroimaging plays an important role in preoperative planning. Drawing on our experience from consultation for multiple successful separations of craniopagus twins, we discuss what radiologists need to know about the anatomy, classification, imaging techniques, and surgical management of craniopagus twins. ABBREVIATIONS: CPT ¼ craniopagus twins; CVS ¼ circumferential venous sinus; SDVS ¼ shared dural venous sinuses; TA ¼ total angular; TV ¼ total vertical C raniopagus twins (CPT) are a rare congenital malformation in which twins are conjoined at the head. It accounts for only 2%-6% of conjoined twins, with an incidence of approximately 1 in 2.5 million lives births. 1,2 The skulls are most often joined at homologous regions on each twin in both vertical and angular orientations, with the face and foramen magnum not primarily involved. 3 CPT can manifest as total, the twins share dural venous sinuses (SDVS), and partial forms, with separate venous anatomy. 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. 16 Neuroimaging, including CT, MR imaging, and conventional angiography, plays an important role in mapping the shared arterial and venous structures, brain parenchyma, calvaria, and dura. [22][23][24] Understanding the shared vascular anatomy is important for surgical planning because separating common vessels is associated with complications such as thrombosis, air embolism, infarction, and hemorrhage. 25 Digital and physical 3D models generated from CT and MR imaging data are important tools for operative planning and as a guide in the operating room. 21 We will review the preoperative radiologic evaluation of CPT, including the anatomy, classification systems, and surgical management, that is important for the radiologist to understand. We draw on our experience in consultation for multiple successful CPT separations with an experienced neurosurgical specialist in CPT.

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“…Craniopagus twinning is the least common form of conjoined twins. The incidence ranges from 0.6 (1) to 2.5 (2) per million live births.…”

Section: Introductionmentioning

confidence: 99%

Perioperative management of face‐to‐face craniopagus twins separation

Pietrini¹,

Valenti²,

Pusateri³

et al. 2005

Pediatric Anesthesia

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Craniopagus conjoining represents a complex and challenging issue for neurosurgeons as well as for anesthesiologists. A rare face-to-face case of conjoined twins underwent surgical separation and presented peculiar differences compared with those already reported in the literature. Even in cases lacking large cerebrovascular sinus connections, the impending risk of large blood loss and hemorrhagic shock in the infant requires a high level of surveillance and the institution of invasive monitoring.

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Total Craniopagus Twins

Cited by 7 publications

References 1 publication

A Century With Craniopagus Twin Separation Surgeries: Nihilism to Optimism

A Century With Craniopagus Twin Separation Surgeries: Nihilism to Optimism

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

Perioperative management of face‐to‐face craniopagus twins separation

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