Hereditary Hemorrhagic Telangiectasia

About Dr. Jason Hamilton

Jason S. Hamilton, M.D. is the Director of Plastic and Reconstructive Surgery for the Osborne Head and Neck Institute based at Cedar-Sinai Medical Towers. Dr. Hamilton has advanced training in plastic and reconstructive surgical techniques involving the face, head, and neck, and limits his practice to the treatment of these areas exclusively.

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About Dr. Ryan Osborne

Ryan F. Osborne, M.D. is the Director of Head and Neck Surgery at OHNI and is an internationally renowned expert in head and neck oncology. He has developed a special interest for the treatment of parotid gland tumors and focuses on the use of minimally-invasive techniques in the care of patients needing parotid surgery.

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Early Diagnosis of Nasal Mucosal Telangiectasias

HHT Overview

Hereditary Hemorrhagic Telangiectasia (HHT) is a hereditary disorder characterized by abnormal clusters of fragile capillaries that bleed with minimal agitation. Telangiectasias, commonly involve the nasal mucosa causing recurrent nosebleeds that may be severe enough to require hospitalization, surgical cauterization as well as blood and/or iron transfusions.

Although HHT is a well know disease and is studied in medical training programs, there are few illustrations or pathologic photographs that depict nasal mucosal telangiectasias for clinicians to use as a reference. When working-up the recurrent nosebleed patient, clinicians need to be knowledgeable of the physical exam findings of patients with HHT. For the HHT patient, the best chance of being diagnosed early is if the treating physician is knowledgeable about the disease process including simple identification of what telangiectasias look like in the nose, skin, and mouth.

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Figures: HHT associated nasal telangiectasias. Note the dilated nest of capillaries surround by normal pink mucosa of the floor of the nose (above) and nasal septum (below).

Clinical Considerations

For ideal visualization of nasal telangiectasias the clinician will need to use a nasal endoscope for complete examination of the nasal cavity. The nasal septum, floor of the nose, and turbinates must all be thoroughly examined as each location may harbor telangiectasias, the common source of recurrent nosebleeds in HHT patients. Other nasal pathologies that may cause nasal irritation and symptoms include a deviated septum, turbinate hypertrophy, and nasal polyps. These pathologies can all be easily diagnosed during the HHT exam for nasal telangiectasias. Treatment usually will include some form of cauterization of the abnormal vessels. Care should be taken to not treat aggressively as over-cauterization of the nasal septum can lead to a septal perforation (HHT septal perforation).

Surgeon Comments

Dr. Jason Hamilton, Director of Plastic and Reconstructive Surgery at the Osborne Head and Neck Institute, is double board certified by the American Board of Facial Plastic and Reconstructive Surgery and the American Board of Otolaryngology/Head and Neck Surgery. His extensive training and expertise provide him with a unique perspective to address both functional and aesthetic concerns of the nose.

For more information on hereditary hemorrhagic telangiectasia or septal perforation repair, please contact the Osborne Head and Neck Institute or visit www.perforatedseptum.com.

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