Curaçao Criteria for hereditary hemorrhagic telangiectasia

Calculator

When to use

Spontaneous, recurrent nosebleeds

Yes

Multiple telangiectasias at characteristic sites including lips, oral cavity, fingers and nose

Yes

Visceral lesions, such as gastrointestinal telangiectasia, or pulmonary, hepatic, cerebral or spinal arteriovenous malformations

Yes

First degree relative meeting diagnostic criteria for HHT

Yes

Calculation

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The Curaçao Criteria is a diagnostic scoring system specifically designed for Hereditary Hemorrhagic Telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome. This calculator is used in the clinical setting to help diagnose HHT, a genetic disorder that leads to abnormal blood vessel formation in the skin, mucous membranes, and often in organs such as the lungs, liver, and brain. The Curaçao Criteria includes four components: spontaneous, recurrent nosebleeds; multiple telangiectasias in typical locations (lips, oral cavity, fingers, nose); a direct line first-degree relative with HHT; and visceral lesions such as gastrointestinal telangiectasia or arteriovenous malformations in the lungs, liver, brain, spine, or gastrointestinal tract. A diagnosis of HHT is considered "definite" if three or more criteria are present, "possible" or "suspected" if two criteria are present, and "unlikely" if fewer than two criteria are present. There are no specific exclusion criteria for the use of the Curaçao Criteria, but it should be noted that it is designed for use in patients with a suspected diagnosis of HHT. It may not be applicable or

Reference

C L Shovlin, A E Guttmacher, E Buscarini et al. Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome). Am J Med Genet. 2000 Mar 6;91(1):66-7.

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