Sclerotherapy for the Management of Epistaxis in HHT Telangiectasia

Michael J. Marino, MD, and Devyani Lal, MD

Hereditary hemorrhagic telangiectasia (HHT) is a rare and typically autosomal dominant genetic condition that may result in vascular malformations at multiple sites and organs, along with recurrent epistaxis. Many patients experience frequent nosebleeds that substantially affect quality of life and in some cases can be life-threatening. Clinical criteria for the diagnosis of HHT include recurrent epistaxis; telangiectasia of the skin and mucosa (oral cavity, nose, and fingers); vascular lesions in the gut, lungs, liver, or brain; and a first-degree relative with a known diagnosis of HHT.

Patients with three or more of these criteria have a probable diagnosis of HHT, while two criteria indicate a possible diagnosis.¹ The disease is characterized by variable penetrance and expression. Genetic testing can also identify known mutations, although not all patients meeting the clinical criteria will have an identifiable mutation. Given the underlying genetic cause for these vascular malformations, a curative intervention is not yet available, and patients often undergo repeated treatment for recurrent symptomatic telangiectases.

Managing recurrent epistaxis satisfactorily in the HHT patient is challenging for the otolaryngologist. Various surgical techniques have been used to improve epistaxis, but differ in the degree of efficacy, morbidity, complications, sequalae, and durability of results. These procedures include electrocautery, laser photocoagulation, coblation, septodermoplasty, local injection of bevacizumab, and nasal closure (Young’s procedure). 

Dating back to at least 2004, but gaining popularity more recently, sclerotherapy of nasal telangiectasia has been investigated as a possible treatment for epistaxis in HHT.²

Generally, sclerotherapy involves the injection of a sclerosing agent into a vascular or lymphatic malformation and has been used in the treatment of varicose leg veins, esophageal varices, and hemorrhoids in addition to nasal telangiectasia. 

Several sclerosing agents have been used for the treatment of nasal lesions,² although sodium tetradecyl sulfate (STS) has become increasingly popular for this application.^3-6 A foamed solution is created by mixing 3% STS with air in a 1:4 ratio. A three-way stopcock facilitates mixing of the 3% STS with air and is connected to tubing and a butterfly needle with the wings removed (Figure 1). Practical preparation of the foamed solution involves drawing up 0.5 mL of STS into one 3 mL syringe and then mixing with 2 mL of air in a second syringe. The procedure can be performed in the operating room or office setting depending on the number of lesions to be treated, the patient’s comfort, and surgeon experience.

Figure 1. A three-way stopcock facilitates mixing of the 3% STS with air and is connected to tubing and a butterfly needle with the wings removed.

A telangiectasia is treated by inserting the tip of the butterfly needle into the lesion. Next, draw back on the syringe to ensure that blood is not drawn into the tubing and prevent injection of the sclerosing agent into a larger vessel. After performing this safety check, the foamed STS solution is then injected until blanching of the lesion is seen. (See video below.) The procedure can then be repeated for multiple telangiectases throughout the nasal cavities, up to a total of 2 mL of 3% STS solution.

Sclerotherapy has been shown to decrease epistaxis severity scores² and may result in decreased emergency department visits and hospital admissions in HHT patients. Use of sclerotherapy for management of epistaxis also resulted in a decreased number of treatments at both 12 and 24 months of follow-up when compared to electrocautery and/or laser photocoagulation.⁶ Patients may report more general improvements in nasal quality of life related to effective and durable control of epistaxis.

The sclerotherapy treatments have been generally well tolerated, although some minor adverse effects do occur, and consideration should be given to prevention of major complications. A systematic review² and our own series found the most frequently reported complications to be typically minor, such as bleeding, nasal crusting, nasal congestion/obstruction, and facial pain. Septal perforation has been reported in one case, and avoiding treatment of the bilateral nasal septum in the same session may prevent this occurrence. Prior septoplasty may increase the risk for septal perforation. Orbital injury and blindness are potentially serious complications that deserve proactive prevention. Drawing back on the syringe before injection can prevent accidental injection into a larger nasal vessel with subsequent sclerosing of the ophthalmic vasculature. There has not been a reported case of blindness secondary to nasal sclerotherapy, although ongoing vigilance is indicated given the seriousness of the potential complication.

Epistaxis in HHT has long posed an ongoing challenge for otolaryngologists. Intralesional sclerotherapy of nasal telangiectasia has emerged as a reliable and viable option for the management of problematic epistaxis in HHT with durable results. Clinicians should be familiar with possible complications such as septal perforation and the rare but catastrophic risk of blindness. Building expertise with sclerotherapy may offer an effective and well-tolerated procedure for this difficult condition. 

References

1. Shovlin CL, Guttmacher AE, Buscarini E, et al. Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome). Am J Med Genet. Mar 6 2000;91(1):66-67. doi:10.1002/(sici)1096-8628(20000306)91:1<66::aid-ajmg12>3.0.co;2-p
2. Thiele B, Abdel-Aty Y, Marks L, Lal D, Marino M. Sclerotherapy for hereditary hemorrhagic telangiectasia-related epistaxis: a systematic review. Ann Otol Rhinol Laryngol. Jan 2023;132(1):82-90. doi:10.1177/00034894221078075
3. Boyer H, Fernandes P, Duran O, Hunter D, Goding G. Office-based sclerotherapy for recurrent epistaxis due to hereditary hemorrhagic telangiectasia: a pilot study. Int Forum Allergy Rhinol. Jul-Aug 2011;1(4):319-323. doi:10.1002/alr.20053
4. Boyer H, Fernandes P, Le C, Yueh B. Prospective randomized trial of sclerotherapy vs standard treatment for epistaxis due to hereditary hemorrhagic telangiectasia. Int Forum Allergy Rhinol. May 2015;5(5):435-440. doi:10.1002/alr.21484
5. Hanks JE, Hunter D, Goding GS, Jr., Boyer HC. Complications from office sclerotherapy for epistaxis due to hereditary hemorrhagic telangiectasia (HHT or Osler-Weber-Rendu). Int Forum Allergy Rhinol. May 2014;4(5):422-427. doi:10.1002/alr.21287
6. Woodard TD, Yappel-Sinkko KB, Wang X, McCrae KR, Parambil JG. Sclerotherapy versus cautery/laser treatment for epistaxis in hereditary hemorrhagic telangiectasia. Laryngoscope. May 2022;132(5):920-925. doi:10.1002/lary.29701