MyMedR

Leung AKC, Leong KF, Barankin B

J Pediatr, 2019 07;210:232.

PMID: 31003749 DOI: 10.1016/j.jpeds.2019.03.013

Matched MeSH terms: Telangiectasia, Hereditary Hemorrhagic/pathology*

Education and Imaging. Gastrointestinal: massive intraperitoneal haemorrhage in a young woman with hereditary hemorrhagic telangiectasia

Sivarani S, Chan YK, Liam CK, Kumar G, Abdullah BJ, Mahadeva S

J Gastroenterol Hepatol, 2010 Sep;25(9):1587.

PMID: 20796159 DOI: 10.1111/j.1440-1746.2010.06469.x

Matched MeSH terms: Telangiectasia, Hereditary Hemorrhagic/complications*

Nasal closure for severe hereditary haemorrhagic telangiectasia in 100 patients. The Lund modification of the Young's procedure: a 22-year experience

Lund VJ, Darby Y, Rimmer J, Amin M, Husain S

Rhinology, 2017 Jun 01;55(2):135-141.

PMID: 28064338 DOI: 10.4193/Rhin16.315

INTRODUCTION: Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disease characterized by recurrent epistaxis, mucocutaneous telangiectasia and visceral arteriovenous malformations. The nosebleeds can be life-threatening and in these circumstances, radical treatment is required.

METHODS: Since 1994, closure of the nose has been undertaken to prevent severe nasal bleeding in patients meeting specific selection criteria. Outcome data collected on this cohort pre- and post-operatively is available for analysis.

RESULTS: From a cohort of 515 HHT patients, 100 have undergone nasal closure, bilateral ab initio in 81%. Fifty patients completed pre- and post-operative epistaxis severity questionnaires and provided information on post-operative problems and 28 completed a Glasgow Benefit Inventory (GBI). Overall most patients derived significant benefit from the procedure with complete cessation of nasal bleeding in 94%, a highly significant improvement in the epistaxis score and a mean GBI score of 53.4. Loss of smell and taste was the most frequent post-operative complaint.

CONCLUSION: HHT can be associated with life-threatening epistaxis significantly affecting patients quality of life. Permanent prevention of airflow is associated with complete or near-total cessation of epistaxis in the majority of patients.

Matched MeSH terms: Telangiectasia, Hereditary Hemorrhagic/surgery*

A Massive Hemothorax in a Pregnant Woman: Role of Interventional Radiology

Md Noh MSF, Abdul Rashid AM, Abdul Rahim E

J Bronchology Interv Pulmonol, 2018 07;25(3):e30-e32.

PMID: 29944591 DOI: 10.1097/LBR.0000000000000456

Matched MeSH terms: Telangiectasia, Hereditary Hemorrhagic*

Fulltext Hereditary hemorrhagic telangiectasia or Osler-Weber-Rendu syndrome: management of epistaxis in 4 cases

Salina H., Lim P.S., Gendeh B.S.

International Medical Journal Malaysia, 2018;17(2):129-134.

MyJurnal

Hereditary Hemorrhagic Telangiectasia, also known as Osler-Weber-Rendu Syndrome is an autosomal
dominant disorder causing systemic abnormalities of the vascular structure. There are multiple arteriovenous malformations present in the skin and mucosal surface of the nail beds, nose, gastrointestinal tract, lungs and brain. Epistaxis is the common presentation symptom, which may require multiple hospital admissions and blood transfusions. It is extremely rare disease in our population. We report 4 cases of HHT who presented to us with moderate to severe epistaxis and how we managed these patients.

Matched MeSH terms: Telangiectasia, Hereditary Hemorrhagic

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