Rendu-Osler-Weber Syndrome: Chronic Epistaxis

Case Report

Rendu-Osler-Weber Syndrome: Chronic Epistaxis

Larhrabli Ibtissam ^*
Olia Reda
Loudghyri Myriam
Bijou Walid
Youssef Oukessou
Sami Rouadi
Redallah larbi Abada
Mohamed Roubal
Mohamed Mahtar

ENT Head and Neck Surgery Department, Ibn Rochd University Hospital, Faculty of Medicine and Pharmacy, Hassan II University, Casablanca, Morocco.

*Corresponding Author: Larhrabli Ibtissam, ENT Head and Neck Surgery Department, Ibn Rochd University Hospital, Faculty of Medicine and Pharmacy, Hassan II University, Casablanca, Morocco.

Citation: Ibtissam L, Reda O, Myriam L, Walid B, Oukessou Y, et al. (2024). Rendu-Osler-Weber Syndrome: Chronic Epistaxis, International Clinical Case Reports and Reviews, BioRes Scientia publishers. 2(2):1-2. DOI: 10.59657/2993-0855.brs.24.013

Copyright: © 2024 Larhrabli ibtissam, this is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Received: June 11, 2024 | Accepted: June 28, 2024 | Published: August 23, 2024

Abstract

Rendu-Osler-Weber Disease, also known as Hereditary Hemorrhagic Telangiectasia, is an uncommon fibrovascular dysplasia that renders vascular walls susceptible to damage and rupture, resulting in bleeding from the skin and mucous membranes. Visceral arteriovenous malformations, recurring epistaxis and telangiectasia on the face, hands, and oral cavity, as well as a positive family history, are the hallmarks of this dominant autosomal inheritance condition. Epistaxis is frequently the primary manifestation. It is linked to several organ arteriovenous malformations. Hematologic, neurological, pulmonary, dermatological, and gastrointestinal problems are all possible outcomes. Supportive care is provided to assist avoid problems.

Keywords: hematologic; pulmonary; chest

Case Presentation

A 67-year-old woman has been experiencing minor recurrent epistaxis for 20 years. Her clinical picture has gotten worse over the last two months, and she has been bleeding for more than an hour three times a day, always following physical activity or sneezes. Although the patient required a blood transfusion multiple times each week, nasal packing has not yet been necessary. He had hemangiomas all over her body for around 45 years, with the most prominent ones being in her lower and upper lip, tongue, face, fingers, chest, and cheek mucosa. She had diabetes and hypertension for five years. We discovered telangiectasia lesions all over her face (figure 1), lower and upper lip (figure 2), oropharynx, tongue (figure 3-4), nails, fingers, and chest during the physical examination. Hematic spots were observed on his septal mucosa and left middle turbinate during anterior rhinoscopy. Additionally, the patient's right septal mucosa had hematic crusts, and a fibroscopy of the posterior oropharynx revealed telangiectasia lesions throughout the oesophagus.

There were no signs of arteriovenous malformations on her chest x-ray, abdomen echography, or skull and abdomen CT scan. A blood test revealed 5,4g/dl of Hb. We first packed his nostrils and admitted him to the hospital so that they could give him blood. The epistaxis episodes returned after the nasal packing was removed, therefore we chose to cauterize the nasal lesions no need for a maxillary artery embolization. After being referred to Hematology, Gastroenterology, and Thoracic Surgery for evaluation, the patient is still being followed up on an outpatient basis. The evaluation revealed no gastrointestinal or pulmonary anomalies. He is still receiving anemic treatment at the hematology ward. Hb 10,6g/dL was found in her most recent blood test.

Figure 1: Face Telangiectasia

Figure 2: Upper lip and tongue telangiectasia

Figure 3-4: Tongue Telangiectasia

Declarations

Patient Consent

Consent to publish the case report was obtained.

Funding
No funding or grant support.

Authorship
All authors at test that they meet the current ICCRR criteria for Authorship.

References

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