Cerebral Cavernous Malformations: Patient-Reported Outcome Validates Conservative Management.

BACKGROUND: Cerebral cavernous malformations (CCM) are clusters of dilated sinusoidal channels lined by a single layer of endothelium. In contradistinction to arteriovenous malformations, these lesions do not have smooth muscle or elastin in their lining and they are angiographically occult, and the MRI is the most sensitive test for CCM detection. CCM are one of the most prevalent vascular malformations of the central nervous system, affecting about 0.4-0.6% of the general population. The main complication of this malformation is the risk of bleeding, which may cause neurological deficits that affect the quality of life (QoL) in patients. When symtomatic, they may be surgically treated for relieving the mass effect and seizures refractory to drug uses, hemorrhage and drug-refractory epilepsy. Patient-reported outcome (PRO) may be a strategy that can be used to evaluate QoL of CCM population and was used in a sample of non-operated patients. METHODS: An observational, cross-sectional analysis to evaluate the PRO using the SF-36 and EuroQol 5 dimensions (EQ-5D) questionnaires of QoL added to functional metrics using the Karnofsky Performance Status (KPS) in 49 patients not submitted to intervention and with long-term follow-up. RESULTS: During the 364 person-years of follow-up, there was an average of individual follow-up of 7.42 years. The mean age was 46.8 years (18-84) - 57% of them were female, 71% had superficial lesions, and 65% had the familial form. Comparisons of SF-36 dimensions with KPS graded <100 had a worse score only in terms of the pain (p = 0.04), vitality (p = 0.001), and general state of health (p = 0.03) domains. The domain mental health was worse in patients without surgical indication (p = 0.032). The functional capacity domain had the highest overall grading in the group. The EQ-5D dimensions of mobility (p = 0.03) and pain/discomfort (p = 0.001) were the ones with lower score compared to KPS <100. CONCLUSION: The study is the first to evaluate, with validated tools, the PRO of non-operated CCM patients and has demonstrated in a selected group of patients that it was possible to achieve long-term clinical stability, thereby maintaining QoL and functional neurological outcome.

Cavernomas del neuroeje: presentación de 4 casos / Neuraxis cavernous malformations: a four cases report

Los cavernomas son tumores vasculares benignos con morfología similar a una frambuesa, comúnmente descritas dentro de las llamadas malformaciones crípticas vasculares, patologías neurológicas poco frecuentes. Constituyen un capítulo polémico dentro de las neurociencias, debido a la variabilidad clínico-topográfica de su presentación y su manejo continúa siendo controversial. Se presentan cuatro pacientes con cavernomas del neuroeje y su caracterización desde el punto vista clínico e imagenológico, hasta su desenlace final. La detección precoz, la comprensión, y el mejor manejo de los cavernomas del neuroeje son fruto del avance tecnológico contemporáneo. La revisión histórica del tema muestra la diferencia entre los pocos casos que se lograban diagnosticar clínicamente y/o por radiología convencional, y el mayor número de lesiones que se detectaban en autopsias o durante la cirugía(AU)

Cavernous malformations are benign vascular tumors, with a raspberry-like morphology, commonly described as part of the group of the so called cryptic vascular malformations, which are rare neurological presentations. They are considered to be a controversial chapter in neuroscience due to the clinical-topographic variability of their presentation. Their management remains controversial. We present the cases of four patients with neuraxis cavernomas who are characterization from clinical and imaging standpoint, up to their final outcome. Early detection, understanding, and better management of the neuraxis cavernous malformations are the result of contemporary technological advances. The historical review on the subject shows the difference between the few cases that could be clinically diagnosed through conventional radiology and the largest number of lesions detected at autopsy or during surgery(AU)

Neurosurgical management of cerebellar cavernous malformations.

OBJECT: The aim of this study was to analyze cerebellar cavernous malformations (CMs) with respect to epidemiological, clinical, radiological, and therapeutic aspects. METHODS: Between 1984 and 2004, 100 patients were surgically treated for intracranial CMs at the Division of Neurosurgery of Federal University of Sao Paulo. The authors reviewed the records of 10 patients whose lesions were located in the cerebellum. There were four male and six female patients (ratio 1:1.5) whose ages ranged from 14 to 45 years (mean age 33 years). Clinical presentation was sudden or acute in all cases, and neuroimaging examinations performed in all patients demonstrated signs of bleeding. The mean size of the malformations was 4.6 cm, and in all but one patient the lesions were totally removed without complications. After a mean follow-up period of 70 months, all patients were considered to be in good or excellent clinical condition. CONCLUSIONS: Cerebellar CMs should be analyzed separately from other posterior fossa CMs. These lesions can reach large sizes and cause massive hemorrhages, resulting in acute or sudden presentation. Surgery is a safe and effective option that provides a curative treatment when a complete removal is achieved.