Craniopagus conjoined twins represent a rare phenomenon of congenital malformation/ dysmorphism. The clinical pathology of this complex entity is reviewed and placed in perspective. Confusion surrounds the severity of craniopagus conjoined twins especially in relation to the difficulty of separation and subsequent outcome. Successful separation of craniopagus twins remains a rarity, however modern neurosurgical techniques have created opportunities for successful separation and brought hope for a normal survival of these children who in the past were often left as historical footnotes or put on display as oddities of nature. We report on a craniopagus conjoined twins from Sudan who had been successfully separated.
Introduction
Cerebellar pilocytic astrocytomas (cPAs) in childhood have long been recognized to have a good prognosis after total resection but the final outcome after incomplete surgery remains largely unpredictable with the incidence of symptomatic progressive disease ranging from 18-100%. Traditionally thought that GTR was required for long-term survival, small residuals were classically resected in a subsequent operation.
Results
Using our Pediatric Low-Grade Glioma (PLGG) database, 283 patients were identified to have a histopathological diagnosis of intracranial pilocytic astrocytoma between 1985-2020 of which 202 were within the cerebellum (68.2%). The majority of patients with cerebellar PA were between 1-10 years of age (n=147, 72.8%) without gender predominance (M:F=101:101), usually presenting with 1 lesion (n=195, 96.5%) and rarely with disseminated disease at diagnosis (N=7, 3.4%). The majority of initial surgeries for cerebellar PA achieved GTR (n=149, 73.8%). In patients with NTR or STR, the mean largest diameter of the post-operative residual was 1.1cm (0.2-3.7cm). Sixty-eight patients experienced relapse of their disease, 4 in which progression was multifocal. In 31 patients the neuro-oncology MDT recommended a second surgical resection at a mean time interval of 22.9 months (0.13-81.6 months) from the initial surgery. The children in the multiple operation cohort experienced more complications of therapy including ongoing endocrinopathy, treatment-associated hearing deficit, and neurocognitive deficiencies.
Conclusion
Residual disease in cPA should be maintained under clinocoradiological surveillance post-operatively with a more conservative approach when residual disease is not significantly changing over time.
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