Conservative management of a palatal ischaemic area following embolization for a cranio-facial arteriovenous malformation (AVM) in a patient with Wyburn-Mason Syndrome

We read with interest the paper from Santos et al. that was published recently (1). Here we present a similar patient with a necrotic mucosal area in the midline of the maxilla, following embolization, in a patient with Wyburn-Mason Syndrome. Wyburn-Mason Syndrome is an exceedingly rare, non-hereditary congenital neurocutaneous disorder leading to arteriovenous malformations (AVMs) (2). It is also known as Congenital Unilateral Retinocephalic Vascular Malformation Syndrome and is characterised by arteries that directly connect to veins without intervening capillaries and leads to a fragile mass of abnormal blood vessels found in the midbrain, eyes, orbit and rarely cutaneous nevi.

Our patient was a 58-old female patient with a slow flow vascular malformation lying in the suprasellar/hypothalamic region and extending into the right orbit, face and nose. The AVM consisted of a small and relatively stable intracranial component and an orbital/facial component, supplied by the bilateral ophthalmic arteries, bilateral external carotid system (internal maxillary and facial) and the mandibulovidian branch of the right internal carotid artery (ICA).

The magnetic resonance angiography (MRA) demonstrated multiple small aneurysms in the facial component of the AVM, particularly in the right nasal cavity; this area has shown clinical progression, resulting in the patient suffering several significant episodes of epistaxis. Our patient underwent radiologically guided embolisation for management of the epistaxis. Specifically, Sceptre Mini advanced into left IMA and PHIL 25 was injected until no further devascularisation of the arterial pedicles could be performed with excellent penetration of the arterial pedicles supplying the AVM. Further embolisation was then performed via the right ophthalmic artery via the Sceptre Mini using a mixture of PHIL 25 and 30 with again very good devascularisation of the arterial pedicles supplying the AVM. Shortly after this, she presented to the maxillofacial clinic with a necrotic area of mucosa in the midline of her palate (Figure 1). A CT scan showed no bone involvement. In the absence of infective signs and symptoms she was managed conservatively with analgesia and an antimicrobial mouthwash. At review the necrotic area had spontaneously healed, and no further interventions were deemed necessary (Figure 2).

Figure 1 Palatal necrosis at presentation.

Figure 2 Palatal epithelialization following conservative management.

In general, embolization can potentially cause stroke, ischaemia, skin necrosis, bleeding, blindness, adverse haemodynamic changes and pulmonary embolism (3). We recommend, in specific cases of necrosis in the maxilla following embolization, adopting a cautious initial management in order to allow necrosis to self-demarcate and heal.

Acknowledgments

We would like to thank Leeds Teaching Hospitals NHS Trust for their support.

Funding: None.

Footnote

Provenance and Peer Review: This article was a standard submission to the journal. The article has undergone external peer review.

Conflict of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://fomm.amegroups.org/article/view/10.21037/fomm-20-85/rc). AK serves as an unpaid editorial board member of Frontiers of Oral and Maxillofacial Medicine from Oct 2020 to Sept 2022. The other authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). Informed consent was obtained from the patient.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

References

1. Santos AMS, Barbosa S, Lima-Neto TJ, et al. Aseptic necrosis of the maxilla on embolized patient: a challenger condition. Front Oral Maxillofac Med 2020;2:28. [Crossref]
2. So JM, Mishra C, Holman RE. Wyburn-Mason Syndrome. [Updated 2020 Jul 2]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020. Available online: https://www.ncbi.nlm.nih.gov/books/NBK493218/
3. Jayaraman MV, Marcellus ML, Hamilton S, et al. Neurologic complications of arteriovenous malformation embolization using liquid embolic agents. AJNR Am J Neuroradiol 2008;29:242-6. [Crossref] [PubMed]