Endoscopic surgical treatment of epistaxis in hereditary haemorrhagic telangiectasia: our experience

Abstract

SUMMARY Objectives Hereditary haemorrhagic telangiectasia (HHT) is a rare autosomal dominant disease characterised by epistaxis. Surgical procedures for epistaxis vary from diathermocoagulation to nasal closure. The aim of this paper is to report our experience in endoscopic surgical management of epistaxis in HHT patients. Methods This is a descriptive, longitudinal study carried out at the Otorhinolaryngology Department of IRCCS Policlinico San Matteo in Pavia, a reference centre for the treatment and diagnosis of HHT. We retrospectively evaluated HHT patients who underwent surgery for epistaxis from 1996 to 2015, including only those treated with endoscopic surgery. Results Among the 591 patients hospitalised and screened for HHT, 323 (54.7%) underwent endoscopic surgery for epistaxis, for a total of 679 procedures. General anaesthesia was used in 77.2% of procedures; argon plasma coagulation was the instrument of choice in the majority of patients, followed by lasers and quantum molecular resonance technology. Conclusions We report one of the largest cohorts undergoing endoscopic treatment of epistaxis in HHT patients. This mini-invasive surgical treatment allowed us to control epistaxis without major complications and nasal packaging and can be repeated over time. For these reasons, we recommend it as first choice in case of epistaxis in HHT patients.

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“…Hence, clinical hallmarks of HHT disease are multiple arterio-venous shunts, or arterio-venous malformations (AVMs), affecting several organs, such as nasal and oral mucosa, skin, lung, liver, brain, and GI tract [ 15 , 16 ]. Although the commonest symptom is represented by spontaneous, repeated nosebleeds, affecting over 90% of patients [ 17 ], visceral involvement may entail sudden AVM-related complications [ 18 , 19 ].…”

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“…SWS patients carry mutations of the RAS p21 protein activator 1 gene, which contributes to cell proliferation [ 20 ]. HHT is a rare disease that causes systemic fibrovascular endothelial dysplasia developing telangiectasias and aneurysms as VMs [ 21 ]. The prevalence of HHT is about 1:5000–1:8000 cases and is an autosomal dominant disease, although 20% of cases occur because of spontaneous mutations.…”

“…In the current study, color and power Doppler HD intraoral US was also the elected imaging technique for the diagnosis of multiple intraoral and perioral CVMs with a diameter <1 cm, which would not be depicted by MRI [ 1 , 6 , 7 , 23 ]. In addition, as the ISSVA classification highlights, the presence of multiple syndromic CVMs implies the need for more than one treatment session and close follow-up because the syndrome causes the appearance of new primary lesions as manifestations of abnormal angiogenesis [ 3 , 4 , 19 , 21 , 22 , 23 ]. Voluminous CVMs require a long-term treatment plan, too [ 5 ].…”

“…Among all the lasers acting on the hemoglobin chromophore (e.g., Neodymium YAG, KTP), the diode laser is the one studied in the literature for the photocoagulation of intraoral and perioral CVMs as well as for the excision of benign and malignant neoformations of the oral cavity [ 16 ]. Diode laser induces photocoagulation of the lesions and consequent healing without adverse events such as intraoperative bleeding, postoperative bleeding, recurrences, and scars, which are the main risks associated with the treatment of those malformations [ 6 , 16 , 21 , 23 ]. The findings of the current study agree with the literature because all sites healed without severe adverse events, scars, or recurrences.…”

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“…Surgical management of HHT-related epistaxis strongly depends on bleeding severity. As first-line approach, several forms of mini-invasive surgical techniques including argon plasma coagulation (APC), lasers and coblation can be used, while more invasive procedures, such dermoplasty and modified Young’s procedure, might be evoked in patients with severe and refractory epistaxis 2 , 24-30 .…”