BACKGROUND AND PURPOSE: Much has been written about the initial radiologic features of cavernous malformations, but less known are the radiologic natural history and evolution of the lesions, particularly when they initially present with hemorrhage. We aimed to describe the typical evolution of a sporadic, hemorrhagic brain cavernous malformation with time. MATERIALS AND METHODS: From our institutional review board-approved cavernous malformation registry, we assessed initial clinical and radiologic features and the subsequent MR imaging evolution of 51 patients with sporadic, hemorrhagic brain cavernous malformations (with follow-up brain MRIs available for review). RESULTS: The initial MR imaging demonstrated mostly Zabramski type I lesions (94.2%) with T1 hyperintensity (94.2%) and associated edema (76.5%). Eight patients (15.6%) rebled in the first year with lesions characterized by new T1 hyperintensity (100%), edema (61.5%), and growth (median, 4 mm). By 90 days, most lesions had changed from Zabramski type I to type II or III (65.2%). While 76.5% had edema associated with the acute hemorrhage, none had persistent edema beyond 90 days unless rebleeding occurred. Across time, the frequency of T1 hyperintensity decreased from 94.2% at baseline to 73.9%, 57.1%, and 50.0% at Ͻ90, 91-365, and Ͼ365 days. DWI intensity at baseline and Ͻ90, 91-365, and Ͼ365 days was hypointense at 53.1%, 56.5%, 70.1%, and 81.2%, respectively. CONCLUSIONS: Hemorrhagic cavernous malformation demonstrates a characteristic pattern of evolution on follow-up imaging. Knowing this evolution helps to analyze the timing of imaging in relation to the clinical presentation and may help distinguish true cavernous malformation hemorrhagic evolution from mimics and guide appropriate timing of interval-imaging follow-up after symptomatic bleeds in untreated patients.
Background and Purpose:
Pregnancy as a hemorrhage risk factor in women with cavernous malformations (CMs) is controversial. We describe prospective hemorrhage risk in women who become pregnant after an established CM diagnosis.
Methods:
Beginning in 2015, we recruited consecutive patients with radiologically confirmed CM of the brain or spinal cord to participate in a prospective registry. Participants underwent a baseline and annual medical record review, imaging review, and annual surveys to ascertain pregnancies, deliveries, and neurological complications. We collected prospective hemorrhage data on reproductive age women. We calculated prospective hemorrhage rates on childbearing age patients based on the number of hemorrhages occurring post-CM diagnosis during pregnancy and nonpregnancy, divided by the years of follow-up and censored at last follow-up, age 46, or surgery in sporadic-form CM. The hemorrhage rates were compared and the 95% CI presented with a
P
value of <0.05.
Results:
Of 160 women, 90 presented with CM under the age of 46 (average age 31.6 years; 25.6% familial form; 46.7% with hemorrhage; 24.4% brain stem location). These 90 patients had 136 pregnancies before CM diagnosis; 36 pregnancies occurred at or after the diagnosis of CM. Four patients had a hemorrhage while pregnant or postpartum leading to the first CM diagnosis. During 402.6 years of follow-up while not pregnant, 42 hemorrhages occurred prospectively yielding a 10.4% per year (95% CI, 7.5–14.0) risk of hemorrhage while not pregnant. No hemorrhages occurred during 32 prospective pregnancies (26 live births including 1 twinset and 7 nonviable fetuses) after CM diagnosis during 26.9 years of pregnancy time yielding a rate of 0% per year (95% CI, 0–13.6). We found no statistical difference in the rates of hemorrhage during pregnant and nonpregnant time (
P
=0.09). No hemorrhage occurred during delivery.
Conclusions:
Our prospective data suggest that pregnancy does not increase the risk of hemorrhage in women with a known brain or spinal cord CM and that vaginal delivery is safe in appropriate candidates.
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