Available online 5 August 2022

AbstractCase illustration

We report the case of a 34 years old woman with HHT family history. After radiosurgical treatment of a symptomatic evolving cerebellar AVM, late control subtracted digital angiography (DSA) demonstrated the complete obliteration of this AVM but also spontaneous obliteration of 3 fronto-parietal AVMs without any hemorrhagic sign on MRI.

Conclusion

To our knowledge, this is the first report of spontaneous obliteration of multiple and unruptured AVMs in a HTT case.

Section snippetsAbbreviationsHHT

Hereditary Hemorrhagic Telangiectasia

AVM

Arteriovenous Malformation

DSA

Digital Subtraction Angiography

GKS

Gamma-Knife Surgery

MCA

Middle Cerebral Artery

ACA

Anterior Cerbral Artery

PICA

Postero-Inferior Cerbellar Arery

Case report

This 34-years-old woman initially presented in infancy with recurrent epistaxis leading to nasal angioma diagnosis. Personal history revealed smoking and contraceptive pill taking since the age of 15. Family history revealed intracranial aneurysm rupture causing death of her grandmother at age of 66 and late HHT diagnosis in her mother. HHT diagnosis was also confirmed some years later in the patient (heterozygote ENG mutation). The clinical disease expression was recurrent epistaxis and

Discussion

No previous case of cerebral unruptured and untreated AVM obliteration has been described in HHT. In sporadic AVMs, spontaneous obliteration rate varies between 0.014% and 3% depending on series [1], [9], [11]. Several factors have been described as able to favor spontaneous obliteration: small nidus size, single feeding arteries and few draining veins. In the literature, spontaneous obliteration is related in most of cases to a hemorrhagic event [1], [9], [11]. The resulting hematoma favors

Conclusion

To our knowledge, this is the first report of multiple spontaneous obliteration of unruptured AVMs in a patient with HHT. Multidisciplinary discussion concerning AVMs in HTT is mandatory due the potential hemodynamics modifications conducting to appearance and disappearance of these cerebrovascular lesions.

Ethics approval

Ethical approval was waived by the local Ethics Committee of Lille University in view of the retrospective nature of the study and all the procedures being performed were part of the routine care.

Consent to participate

Informed consent was obtained from all individual participants included in the study.

Consent for publication

The authors affirm that human research participants provided informed consent for publication of the images in Fig. 1.

Availability of data and material

The datasets generated during and/or analysed during the current study are available from the corresponding author on reasonable request.

Funding

None.

Authors’ contributions

All authors contributed to the study conception and design. Material preparation, data collection and analysis were performed by Antoine Devalckeneer, Rabih Aboukais and Jean-Paul Lejeune. The first draft of the manuscript was written by Antoine Devalckeneer and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Disclosure of interest

The authors declare that they have no competing interest.

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