Cavernous malformations of the brain stem

Abstract

A retrospective analysis of 139 patients with brain stem cavernous malformations is presented. The material consists of 41 cases from Bern and Phoenix and 98 further well-documented cases from the literature. Sixty-eight patients were male, 70 were female. The average age was 31.8 + 11.8 years. Sixty-two percent of the cavernous malformations were in the pons, 14% were in the mesencephalon, 12% were in the pontomesencephalic and in the pontomedullary junction, and 5% were in the medulla. Eighty-eight percent of the patients showed evidence of recent or previous hemorrhage, 55% had one hemorrhage. 17% had two hemorrhages, and 17% had three or more hemorrhages. Twelve patients died from a hemorrhage, 5 with the first bleeding and 7 with a rebleeding. The minimum bleeding rate was 2.7% per year and the average rebleeding rate 21% per year and per lesion. Most lesions had a diameter between 10 and 30 mm. Increase in size was observed in 12 of the patients; this corresponds to about 21% when only patients with a follow-up of at least one year are considered. In 93 patients the cavernous malformation was removed operatively while in 30 patients the lesion was not removed. In the group with conservative management at the end of the observation period (up to 25 and 32 years), 66.6% had no or only a slight neurological deficit, 6.7% were moderately disabled, 6.7% were completely dependent, and 20% had died. In the group treated surgically 83.9% had no or only a slight neurological deficit, and 15% were moderately disabled. One patient remained severely disabled, no patient died. The limitations of the retrospective nature of this study are stressed.

Hemorrhage risks and functional outcomes of untreated brainstem cavernous malformations

“…20 The percentage of improved or unchanged functional outcomes was retrospectively reported in relatively small cohorts and ranged from 46.7% to 95.7%. 10,16,23 This study demonstrated that an initial small lesion size, no prospective hemorrhage, and younger age contributed to promote complete recovery. These findings were interpreted as implying that a significant space-occupying effect, cumulative impairment caused by repeated hemorrhages, and advanced age with decreasing potential of rehabilitation or functional compensation would delay or even inhibit functional improvement.…”

Clinical course of untreated pediatric brainstem cavernous malformations: hemorrhage risk and functional recovery

“…10,11,14,19,22,30,34,37,38,42,47,51 Among these studies, Tarnaris et al 47 reported fairly poor outcomes, with 53.3% of patients exhibiting worsening conditions, and cited a neurological deterioration of 10% per patient year. An extremely malignant prognosis was reported with a mortality rate of 20%.…”

Intramedullary spinal cord cavernous angioma in children: case report and literature review

“…In 2002, Nagib and O'Fallon [14] summarized nine cases including their two personal cases, and in 2006, Bakir et al [3] reported another pediatric patient with ImCA: Although the number of reported pediatric cases with ImCA is small, we can outline some differences from that of the While ImCA in the adult occur more frequently in women than in men [12,17], with a female/male ratio of approximately 2:1, seven of ten cases of symptomatic ImCA reported in children were male, thus accounting for a male/ female ratio of 2:1. The hypothesis of a hormonal effect leading to a greater risk of bleeding in female adults does not seem to be verified in children, but the mean age at bleeding is 13.2 years (range [8][9][10][11][12][13][14][15][16][17] and can also suggest the hormonal influence of puberty.…”

“…Differences between intracerebral and intraspinal CA A higher risk of bleeding has been identified for ImCA: The actuarial risk of bleeding of cerebral sustentorial lesions is 0.2-0.7% per year [5,13,16] against 1.6% per year in ImCA [11,14] and 2.7% for brainstem CAs [10]. Moreover, the potential for a devastating hemorrhage with significant morbidity is higher for ImCA than for their cerebral counterpart because of the exiguous parenchymatous space where these lesions are located.…”