Priority Clinical Recommendations for HHT CAVMs



Background: The full report would have a section outlining the evidence, including the evidence tables, for all topics considered in the CAVM section. This text would include all those factors which become “clinical considerations”. The text would end with the recommendations and the short paragraph for each, which would then be pasted into the executive summary.

1. The expert panel recommends screening of all patients with suspected or confirmed HHT for cerebral AVMs.

Clinical considerations: Approximately 10% of the HHT population will harbor a CAVM. The natural history of CAVMs in patients with suspected or confirmed HHT remains unknown. The annual rate of rupture of CAVMs in patients without HHT is between 3-7%/year with an associated 10-80% rate of morbidity and mortality per hemorrhage. Based on the non-HHT literature, most physicians consider the obliteration of cerebral AVMs to effectively eliminate the future risk of hemorrhagic sequelae associated with their presence. Effective treatment strategies including: embolization, surgery and stereotactic radiation, or a combination of these modalities, appears to provide effective treatment strategies for the obliteration of CAVMs. Screening with non-invasive MRI/MRA cranial imaging remains the safest and most sensitive non-invasive screening tool available. The expert panel feels that screening for CAVMs in patients with suspected or confirmed HHT should be performed in both the adult and pediatric age group. The expert panel feels that screening should be repeated every five years in both adults and children.

2. The expert panel recommends the use of MRI/MRA for CAVM screening in patients with suspected or confirmed HHT using a protocol that includes gadolinium administration and gradient echo sequences to maximize the sensitivity of non-invasive imaging in the detection of CAVMs.

Clinical Considerations: While the gold standard for the detection of CAVMs remains cerebral catheter angiography, unfortunately such invasive testing continues to carry a 0.5-1% risk of stroke. Gadolinium enhanced MRI/MRA with gradient echo sequences possesses the most sensitive, non-invasive technique currently available for the detection of both HHT and non-HHT CAVMs. The expert panel recognizes the challenges in the detection of micro-CAVMs with non-invasive imaging and the difficulties associated with delineating brain capillary telangiectasia from CAVM, but feel it to be the safest, most sensitive screening test currently available for both HHT and non-HHT CAVM detection. The expert panel also feels that by including gradient echo sequences that this modality will increase the yield of screening by detecting blood products suggestive of occult hemorrhage.

3. The expert panel recommends that physicians strongly consider treatment in both children and adults discovered to have a ruptured CAVM with suspected or confirmed HHT. The expert panel recognizes that asymptomatic CAVMs discovered during screening in patients with suspected or confirmed HHT may carry a more favorable natural history and therefore be managed on a case by case basis.

Clinical Considerations: The natural history of CAVMs in patients with suspected or confirmed HHT remains unknown. The annual rate of rupture of CAVMs in patients without HHT is between 3-7%/year with an associated 10-80% rate of morbidity and mortality per hemorrhage. The expert panel recommends that all patients with suspected or confirmed HHT with a ruptured CAVM be strongly considered for treatment. The expert panel recognizes that asymptomatic CAVMs discovered during screening in patients with suspected or confirmed HHT may carry a more favorable natural history. These patients should be managed on a case by case basis. No Class I evidence exists regarding the optimum management of CAVMs in either the non-HHT or HHT population. The expert panel recommends that when treatment is prescribed for CAVMs in patients with suspected or confirmed HHT, that microsurgical excision be reserved for CAVMs in non-eloquent areas of the brain and embolization and stereotactic radiation reserved for CAVMs that occur in eloquent or surgically difficult areas of the brain. The expert panel recommends that the management of patients with suspected or confirmed HHT with a CAVM occur in a center where a multidisciplinary approach (interventional neuroradiology, stereotactic radiosurgery, neurovascular surgery) is available.

4. The expert panel recommends that mothers with suspected or confirmed HHT harboring an asymptomatic CAVM during pregnancy have definitive treatment of their CAVM deferred until after delivery of their fetus. The expert panel recommends that the delivery of the fetus follow obstetrical principles.

The natural history of CAVMs during pregnancy in both HHT and non-HHT patients remains unknown. The optimal management of CAVMs in both the HHT and the non-HHT population during pregnancy also remains controversial however, the expert panel feels that it is acceptable to defer definitive management of a known or discovered asymptomatic CAVMs during pregnancy in a patient with suspected or confirmed HHT until after delivery of the fetus. Method of delivery of the unborn to a mother with suspected or confirmed HHT should follow general obstetrical principles. The expert panel feels that the management of ruptured CAVMs in patients with suspected or confirmed HHT follow general neurosurgical principles.

5. The expert panel recommends that physicians provide long-term follow-up for patients who have CAVMs (treated or untreated), in order to detect a change or development of more CAVMs in patients with suspected or confirmed HHT every 5 years using the same MRI/MRA protocol as used for screening.

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