Screening and diagnosis

Classification and risk stratification

Perioperative care

Quality improvement

Patient education

Nonpharmacologic interventions

Therapeutic procedures

Respiratory support

Follow-up and surveillance

Specific circumstances

Preventative measures

Medical management

Surgical interventions

Inpatient care

Diagnostic procedures

Diagnostic investigations

Insufficient evidence to make a recommendation.

Strongly favor this test or intervention.

The Curaçao Criteria for Hereditary Hemorrhagic Telangiectasia (HHT) is a tool used to assess the likelihood that an individual has HHT based on specific clinical and familial indicators. The evaluation involves four key questions about symptoms and family history. Each question can be answered with a simple yes or no, and each affirmative answer contributes 1 point to the total score.

Here's how the scoring works:

- Spontaneous, recurrent nosebleeds: 1 point
- Multiple telangiectasias at characteristic sites (lips, oral cavity, fingers, and nose): 1 point
- Visceral lesions such as gastrointestinal telangiectasia, or arteriovenous malformations in the lungs, liver, brain, or spine: 1 point
- A first-degree relative meeting the diagnostic criteria for HHT: 1 point

The total score ranges from 0 to 4 based on the user's responses. This score determines the interpretation of the likelihood of having HHT:

- A score of less than 2 means the criteria are not met, suggesting it's unlikely the person has HHT.
- A score of exactly 2 suggests the criteria for possible HHT are met, indicating a potential but uncertain diagnosis needing further evaluation.
- A score of 3 or higher indicates the criteria for definite HHT are met, strongly suggesting the presence of HHT in the individual.

This systematic approach guides medical professionals in identifying individuals who may require further investigation or treatment for HHT.

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

Curaçao Criteria for hereditary hemorrhagic telangiectasia

Hereditary hemorrhagic telangiectasia

Prognostic assessment

Pediatric patients

Genetic testing

Diagnosis

Red blood cell transfusion

RBC transfusion

Topical therapies for epistaxis

Patients with vascular malformations

Patients with VMs, liver

Evaluation for vascular malformations

Evaluation for VMs (pulmonary)

Surgical management of epistaxis

Surgical management of epistaxis (ablative therapies)

Liver transplantation

Evaluation for iron deficiency and anemia

Diagnostic imaging

Liver biopsy

Iron replacement

Antifibrinolytic therapy

Evaluation for VMs (brain)

Patients with VMs (brain)

Evaluation for VMs, liver

Indications for screening

Patients with VMs (pulmonary)

Pregnant patients

Antiangiogenic therapy

Preconception and prenatal counseling

Severity grading

Anticoagulation and antiplatelet therapy

Endoscopic argon plasma coagulation

Surgical management of epistaxis (nasal closure)

Surgical management of epistaxis (septodermoplasty)

Transcatheter embolotherapy

Upper and lower gastrointestinal endoscopy

Hereditary hemorrhagic telangiectasia

Guidelines

Key sources