Intratumoral hemorrhage and deadly upward transtentorial herniation after endoscopic third ventriculostomy in the management of posterior fossa tumor-related hydrocephalus - case report and literature review

The management of hydrocephalus associated with posterior fossa tumors in children has always been controversial. Studies show that 71%-90% of children with posterior fossa tumors already presented hydrocephalus on admission. Of these cases, 10%-62% will have persistent hydrocephalus after surgical treatment of the tumor. The shunt, although it is an important alternative for preoperative treatment in these cases is not without complications. Among the numerous complications identified by the literature, we highlight the most rare, intratumoral hemorrhage, upward transtentorial herniation and dissemination of neoplastic cells by the peritoneum. Medulloblastoma is considered the most common malignant pediatric tumor, comprising 15%-20% of all intracranial tumors in childhood. In addition, 5%-6% of primary or recurrent medulloblastomas may be associated with spontaneous bleeding and rapid deterioration. The bleeding presents with tumor swelling, extending to the ventricular system through the tumor capsule, increased intracranial pressure and upward transtentorial herniation. Tumoral hemorrhage associated with ventricular drainage for treatment of hydrocephalus in patients with medulloblastoma represents an extremely rare phenomenon. The endoscopy third-ventriculostomy (ETV) is known to be very useful in the treatment of intracranial hypertension preoperatively and prevent persistent postoperative hydrocephalus in cases associated with posterior fossa tumors, with efficacy superior to traditional ventricular shunts. We describe a case of intratumoral hemorrhage and upward transtentorial herniation associated with endoscopic third-ventriculostomy (ETV). O manejo da hidrocefalia associada a tumores de fossa posterior em criancas sempre foi controverso. Trabalhos mostram que 71%-90% das criancas com tumores de fossa posterior ja se apresentam hidrocefalicos na admissao. Desses casos, 10%-62% terao hidrocefalia persistente apos o tratamento cirurgico do tumor. A derivacao ventricular, embora consista em importante alternativa de tratamento pre-operatorio nesses casos, nao esta isenta de complicacoes. Dentre as inumeras complicacoes apontadas pela literatura atual, particularmente nos casos de meduloblastoma que necessitaram de derivacao ventricular por hidrocefalia, destacamos as mais raras: hemorragia tumoral, herniacao transtentorial ascendente e disseminacao de celulas neoplasicas pelo peritonio. O meduloblastoma e considerado o tumor pediatrico maligno mais comum, compreendendo 15%-20% de todas as neoplasias intracranianas na infância. Alem disso, 5%-6% dos meduloblastomas primarios ou recorrentes podem estar associados a hemorragia espontânea e rapida deterioracao. A hemorragia cursa com aumento de volume do tumor, extravasamento para o sistema ventricular atraves da capsula tumoral, aumento da pressao intracraniana e herniacao transtentorial ascendente. Hemorragia tumoral associada a drenagem ventricular, para tratamento de hidrocefalia em portadores de meduloblastoma, representa um fenomeno extremamente raro. A terceiroventriculostomia endoscopica (TVE) e reconhecida por ser bastante util no tratamento da hipertensao intracraniana no pre-operatorio e por prevenir a hidrocefalia persistente pos-operatoria nos casos associados a tumores de fossa posterior, com eficacia superior as derivacoes ventriculares tradicionais. Descrevemos um caso de hemorragia tumoral e herniacao transtentorial ascendente associados a terceiro-ventriculostomia endoscopica (TVE).