Morphology and Distribution of Nasal Telangiectasia in HHT-Patients with Epistaxis

Abstract

Background

Epistaxis in hereditary hemorrhagic telangiectasia (HHT; Rendu-Osler-Weber syndrome) is a frequent symptom that may be caused by a multitude of different genetic and epigenetic phenomena. This investigation analyzes the distribution of nasal telangiectasia in 21 patients with HHT.

Methods

The patients were examined for endonasal telangiectasia by videoendoscopy with rigid endoscopes; in addition, the anterior portion of the nose was examined under the operating microscope. The endonasal findings were recorded on videotape and then evaluated in the media laboratory.

Results

Morphology of the nasal telangiectasia showed wide variations: the vessels were shaped like spots, loops, or spiders or they clustered and resembled raspberries. Gender did not have an influence on the phenotype of telangiectasia, whereas advancing age correlated with a higher density of telangiectasia. Patients with an intact nasal septum exhibited the bulk of telangiectasia in the anterior nasal cavity but also on the middle turbinates, the floor of the nose, and within the valve area. Patients with septal perforations displayed the majority of telangiectasia around the edge of the perforations, on the floor of the nose, and on the turbinates. Scattered telangiectasia also could be found in the profound parts of the nasal cavity and in the nasopharynx, especially in patients with septal perforations.

Conclusion

The shapes of endonasal telangiectasia in HHT patients are very heterogeneous; predilection sites could first of all be found within the anterior portion of the nose. Morphology and distribution of endonasal telangiectasia change as a result of therapeutic interventions, development of septal perforations, and with advancing age. Therefore, repeat endoscopies are recommended to assess the actual stage of the disease before epistaxis therapy.