Brainstem cavernous malformations: a review with two case reports

Abstract

-Central nervous system (CNS) cavernous malformations (CMs) are developmental malformations of the vascular bed with a highly variable clinical course due to their dynamic nature. We present one case of "de novo" brainstem cavernous malformation after radiation therapy adding to the increasing number of reported cases in the medical literature, and the case of a pregnant patient with symptomatic intracranial hemorrhage related to brainstem CMs to illustrate the complex nature in management of these patients, followed by a review of clinical and radiographic characteristics. CMs account for 8-15% of all intracranial and intraspinal vascular malformations. Although traditionally thought to be congenital in origin, CMs may present as acquired lesions particularly after intracranial radiation therapy. Clinical manifestations are protean and surgical treatment should be considered for patients with progressive neurologic deficits.Key WordS: cavernous malformations, epilepsy, "de novo". Malformações cavernosas do tronco cerebral: uma revisão com relato de dois casosResumo -Malformações cavernosas (MFC) do sistema nervoso central são malformações do desenvolvimento do leito vascular com múltiplas apresentações clínicas devido a sua natureza dinâmica. Apresentamos dois casos de malformações cavernosas do tronco cerebral: o primeiro após radioterapia e o segundo em paciente grávida com hemorragia intracraniana sintomática. MFC são responsáveis por cerca de 8-15% de todas as malformações vasculares. embora tradicionalmente sejam genéticas, as MFC podem também ser adquiridas, particularmente após radioterapia. As manifestações clínicas são variáveis e o tratamento cirúrgico deve ser considerado para pacientes com quadros neurológicos progressivos.PAlAvrAS-ChAve: malformações cavernosas, epilepsia, "de novo". department of Neurology, loyola University Chicago, USA.

Surgical Treatment of Cavernous Angiomas in the Pineal Gland – Case Report

“…Of the total cavernous malformations, 9 to 35% occur in the brainstem, 9 and the probability of involvement of the pineal gland region is < 1%. 5 The risk of hemorrhage in cavernous angiomas varies according to the location: those lesions located more deeply, such as those in the brainstem, thalamus or basal ganglia, present a risk of 4.1%; in the cases of superficial craniomas, 0.4%, 5,9 whereas the risk of hemorrhage in the region of the pineal gland is quite small. 3 Regardless of the area affected by the cavernous angioma, an initial hemorrhage significantly increases a new episode of hemorrhage.…”

Pregnancy Outcomes Complicated with Cavernous Angioma: a Systematic Review

“…Neurosurgical resection of CCMs was performed in 4 of the 13 patients who underwent cesarean delivery. Two of these patients had symptomatic CMMs in the pons, one of whom underwent surgery at 27 weeks of gestation and another at 29 weeks of gestation [11][12][13][14][15][16][17][18][19][20][21][22]. Of the other two patients, one patient with intramedullary CCMs underwent laminectomy 2 weeks after her uneventful cesarean delivery [6], and the other patient with a CCM in the pons underwent neurosurgical resection 6 years postpartum during the fourth haemorrhage [8].…”

Malformación cavernomatosa: Revisión de una patología clásica

“…Cohortes seguidas prospectivamente demostraron que el mayor porcentaje de los sujetos es asintomático, siendo estas lesiones un hallazgo incidental. Dentro de aquella minoría sintomática, los hallazgos son heterogéneos, dependiendo de su localización y de si se asocian o no a hemorragia, encontrándose manifestaciones como crisis convulsivas (40-50%), focalización neurológica, cefaleas (30%) y hemorragia intracraneal (10-25%) (24,25) .…”

“…Habitualmente diagnosticadas en la quinta década de la vida y sin predominio de género, con una tasa de sangrado estimado en 1.4% por lesión/año (0.4-2% por año). Se presume que las eventuales hemorragias ocurren a baja presión y por ello las manifestaciones clínicas no son tan dramáticas como las provenientes de lesiones arteriales (25,35,36) . La historia natural de las MC medulares es confusa.…”

“…Generalmente el manejo es conservador, interviniéndose en casos seleccionados, ya sea con cirugía tradicional o radiocirugía (Gamma knife). En los casos en que estas opciones no sean posibles, como en lesiones de tronco con sangrado recurrente, se ha descrito el manejo con factor VI Ia recombinante endovenoso, con lo que se obtiene buenos resultados (25,40) .…”