Sclerotherapy for Hereditary Hemorrhagic Telangiectasia-Related Epistaxis: A Systematic Review

Thiele, Brittney; Abdel-Aty, Yassmeen; Marks, Lisa; Lal, Devyani; Marino, Michael J.

doi:10.1177/00034894221078075

Cited by 2 publications

(1 citation statement)

References 33 publications

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“…Sclerotherapy, most commonly with the agent sodium tetradecyl sulfate (STS), can be effective as an alternative, although this technique can have serious risks and must be implemented with caution. 22 For all patients requiring operative procedures, we also offer the option of intranasal injection of 0.5 mL of bevacizumab 25 mg/mL in divided doses in the sphenopalatine foramen, posterior bony nasal septum, upper lateral nasal wall, and nasal floor bilaterally. 23 A recent prospective, double-blind RCT comparing this protocol with bipolar cauterization alone showed a clinically significant benefit 4 months after treatment.…”

Section: Epistaxismentioning

confidence: 99%

Multidisciplinary coordinated care of hereditary hemorrhagic telangiectasia (Osler–Weber–Rendu disease)

Alkhalid,

Darji,

Shenkar

et al. 2023

Vasc Med

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Hereditary hemorrhagic telangiectasia (HHT), also known as Osler–Weber–Rendu disease, is a rare disorder with a case prevalence as high as one in 5000, causing arteriovenous malformations in multiple organ systems. HHT is familial with autosomal dominant inheritance, with genetic testing allowing confirmation of the diagnosis in asymptomatic kindreds. Common clinical manifestations are epistaxis and intestinal lesions causing anemia and requiring transfusions. Pulmonary vascular malformations predispose to ischemic stroke and brain abscess and may cause dyspnea and cardiac failure. Brain vascular malformations can cause hemorrhagic stroke and seizures. Rarely, liver arteriovenous malformations can cause hepatic failure. A form of HHT can cause juvenile polyposis syndrome and colon cancer. Specialists in multiple fields may be called to care for one or more aspects of HHT, but few are familiar with evidence-based guidelines for HHT management or see a sufficient number of patients to gain experience with the unique characteristics of the disease. Primary care physicians and specialists are often unaware of the important manifestations of HHT in multiple systems and the thresholds for their screening and appropriate management. To improve familiarity, experience, and coordinated multisystem care for patients with HHT, the Cure HHT Foundation, which advocates for patients and families with this disease, has accredited 29 centers in North America with designated specialists for the evaluation and care of patients with HHT. Team assembly and current screening and management protocols are described as a model for evidence-based, multidisciplinary care in this disease.

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Section: Epistaxismentioning

confidence: 99%

Multidisciplinary coordinated care of hereditary hemorrhagic telangiectasia (Osler–Weber–Rendu disease)

Alkhalid,

Darji,

Shenkar

et al. 2023

Vasc Med

View full text Add to dashboard Cite

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Innovations in the management of epistaxis secondary to hereditary hemorrhagic telangiectasia: our evolution to injection sclerotherapy as the treatment of choice

Kumar,

Lança Gomes,

Marino

et al. 2024

Front. Allergy

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IntroductionWe compared the efficacy of intralesional sclerotherapy using 3% sodium tetradecyl sulfate with non-sclerotherapy-based treatments for Hereditary Hemorrhagic Telangiectasia-associated epistaxis management.MethodologyThis is a retrospective study of patients who underwent surgical intervention for HHT-associated epistaxis management from 01/2010–02/2024. Patients undergoing sclerotherapy with intralesional 3% sodium tetradecyl sulfate were included in the sclerotherapy group and others undergoing conventional non-sclerotherapy-based procedures in the non-sclerotherapy group. Outcomes like breakthrough epistaxis, emergency visits, intra-op blood loss, blood transfusions, and procedure complications in the 3-month perioperative period were compared.ResultsTwenty-three patients who underwent 74 intranasal procedures were identified. In the sclerotherapy group, 17 patients underwent 47 procedures. In the non-sclerotherapy group, 10 patients underwent 27 procedures. Till the 3rd post-treatment month, fewer breakthrough epistaxis episodes were observed after sclerotherapy procedures (13/47) vs. non-sclerotherapy procedures (14/27); (p = 0.037). Intraoperative blood loss was significantly lower during sclerotherapy (median: 10 ml) vs. non-sclerotherapy procedures (median: 50 ml); p < 0.001. The time interval between successive procedures was not significantly different in the sclerotherapy (median 6.5 months) vs. the non-sclerotherapy group (median 3.5 months); p = 0.13. Nasal crusting was the most common complication in the sclerotherapy group (36.9%). Two patients in each group had new onset septal perforation, none of the patients had vision loss or cerebrovascular accident. One emergency department visit was reported in the sclerotherapy group vs. 7 (in 3 patients) in the non-sclerotherapy group.ConclusionsCompared to non-sclerotherapy treatments, intralesional sclerotherapy for epistaxis in HHT is more effective in decreasing breakthrough epistaxis, and has lower intraoperative blood loss.

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Sclerotherapy for Hereditary Hemorrhagic Telangiectasia-Related Epistaxis: A Systematic Review

Cited by 2 publications

References 33 publications

Multidisciplinary coordinated care of hereditary hemorrhagic telangiectasia (Osler–Weber–Rendu disease)

Multidisciplinary coordinated care of hereditary hemorrhagic telangiectasia (Osler–Weber–Rendu disease)

Innovations in the management of epistaxis secondary to hereditary hemorrhagic telangiectasia: our evolution to injection sclerotherapy as the treatment of choice

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