Classification of endonasal HHT lesions using digital microscopy.

Area of Science:

• Otorhinolaryngology
• Genetics
• Medical Imaging

Background:

• Recurrent epistaxis is a primary debilitating symptom in hereditary hemorrhagic telangiectasia (HHT).
• Current HHT classification focuses on genetic mutations, lacking a standard for endonasal manifestations.
• A need exists for classifying mucocutaneous endonasal lesions in HHT patients.

Purpose of the Study:

• To document endonasal HHT lesions using digital microscopy.
• To propose a novel clinical classification for endonasal HHT manifestations.
• To correlate lesion classification with clinical outcomes like epistaxis severity and quality of life.

Main Methods:

• Digital microscopy was used to record endonasal HHT lesions in 28 patients.
• 3D images and videos were reconstructed for lesion classification into Grade A (flat telangiectasias) and Grade B (raised telangiectasias).
• Epistaxis severity (ESS), quality of life (VAS), hemoglobin, and plasma VEGF levels were assessed.

Main Results:

• Grade A patients exhibited significantly lower epistaxis severity and higher quality of life compared to Grade B patients.
• No significant differences in hemoglobin or plasma VEGF levels were found between Grade A and Grade B.
• Plasma VEGF levels showed no gender specificity or correlation with extranasal manifestations.

Conclusions:

• The proposed classification for endonasal HHT lesions effectively indicates epistaxis severity and quality of life.
• Digital microscopy with 3D reconstruction is a valuable tool for classifying and monitoring HHT lesions over time.
• This classification aids in evaluating treatment efficacy and is beneficial for academic teaching of rare diseases.