Benefits of Treating Arteriovenous Malformations in Hereditary Hemorrhagic Telangiectasia: A Retrospective Analysis of 14 Patients

Abstract

Background Arteriovenous malformations (AVMs) are a cardinal feature of hereditary hemorrhagic telangiectasia (HHT). However, whether to treat brain AVMs in patients with HHT remains questionable because of the possible risks. Methods We performed a retrospective study of patients with HHT who had been treated for brain AVMs at our institution from January 1, 2003, to December 31, 2016. An institutional database was queried for the phrases “hereditary hemorrhagic telangiectasia” and “HHT,” and those patients who had been treated during the study period were identified. Data were extracted regarding presentation, AVM characteristics, treatment modality, and treatment outcomes. Results We identified 14 patients (10 males, 4 females) with HHT who had had AVMs ( n = 27) from the institutional database. The mean age of the patients was 43 years (range, 2–64). Of the 27 brain AVMs, 13 were Spetzler-Martin grade I, 12 were grade II, and 2 were grade III; none were grade IV or V. Treatment was by microsurgery only (11 AVMs in 10 patients), embolization followed by microsurgery (2 AVMs in 2 patients), and radiosurgery only (12 AVMs in 2 patients). AVM obliteration was achieved in 100% of the patients. No new fixed neurologic deficits developed after treatment of unruptured HHT AVMs. Conclusions The risk of treatment of brain AVMs in patients with HHT is quite low for appropriately selected patients with treatment individualized to radiosurgery, microsurgery, or a combination of embolization and microsurgery.

Surgical treatment of brain arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia: a single-center experience

“…Other series show similar results; Woodall and colleagues also reported a 100% obliteration rate with no fixed complications related to surgery. 7 Likewise, Meybodi and colleagues reported complete obliteration and only transient postoperative neurological deficits. 13 Additionally, consistent with our study, these series documented low-grade SM bAVMs in their patients, and a younger age at presentation and operation on average, compared with patients with sporadic bAVMs.…”

“…6,11 These studies have significant methodological flaws, which have been discussed by Woodall and colleagues. 7 The most important error in these studies is the assumption that patients with HHT are born with bAVMs, thereby inflating the patient-years used as a denominator and leading to a biased lower annual bleeding risk. The second argument for conservative treatment of bAVMs in patients with HHT is that the multiplicity of bAVMs (42% of these patients had > 1 bAVM) translates to an increased risk of treatment.…”

“…Thirty-seven percent of patients reported in a series by Meybodi et al and 10% in another study by Woodall et al underwent adjuvant treatment in the form of embolization or radiosurgery. 7,13 Only scarce evidence exists on the role of radiosurgery in the treatment of bAVMs in patients with HHT. Haettel et al 14 reported a single case of a 34-yearold female patient who had successful obliteration of a cerebellar AVM, and unexpectedly, spontaneous regression of 3 other supratentorial AVMs after radiosurgery.…”

“…Kuo et al 15 reported 2 successfully treated cases of pediatric bAVMs using radiosurgery, whereas an older series by Maarouf et al 16 also reported successful obliteration in 2 patients who underwent LINAC radiosurgery. Finally, in the Woodall et al series, 7 the authors treated patients who had multiple bAVMs (a total of 12 bAVMs in 2 patients) with radiosurgery, reporting successful obliteration of the lesions with no permanent deficits. The limitations of these studies are their small sample sizes and selection bias.…”

“…Previ-ous studies [4][5][6] reported a lower risk of bleeding of these bAVMs compared with sporadic bAVMs, but this is likely an underestimation of the prevalence and lifetime risk of bleeding. 7 It has been argued that the risks of surgery are lower compared with those for sporadic bAVMs, which is related to the angioarchitectural characteristics of these bAVMs, their generally small size, and the young age of the patients. Our institution has traditionally pursued surgical management of these bAVMs when possible.…”