Juvenile Polyposis Hereditary Hemorrhagic Telangiectasia Syndrome C1832942
A rare genetic disorder combining gastrointestinal polyps with abnormal blood vessel formation. Patients need coordinated care for multiple organ systems.
Key facts at a glance
- ICD-10
- K76.9
- ICD-11
- DA00.0
- Body system
- gastrointestinal
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Gastrointestinal bleeding
Rectal bleeding
Intestinal obstruction
Abdominal pain or nausea
(gastrointestinal)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Abdominal pain
(gastrointestinal)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Comprehensive AVM Surveillance
Systematic screening and monitoring of arteriovenous malformations across multiple organ systems including brain, lungs, liver, and GI tract using imaging studies appropriate to each location.
GI disease management
Active treatment with regular monitoring by a healthcare provider is recommended.
GI Polypectomy
Endoscopic removal of GI polyps to control bleeding and anemia, similar to juvenile polyposis syndrome management. May need to be coordinated with embolization procedures.
Additional treatment options
AVM Embolization
Catheter-based embolization of symptomatic arteriovenous malformations, particularly pulmonary AVMs causing right-to-left shunting or hepatic AVMs causing high-output cardiac failure.
surgicalAntifibrinolytic Therapy
Agents such as tranexamic acid to reduce bleeding from GI telangiectasias and polyps. Particularly useful for chronic GI bleeding not controlled by endoscopic therapy.
medicationSystemic Antiangiogenic Therapy
Targeted therapy with agents such as bevacizumab for diffuse hepatic AVMs causing high-output cardiac failure or for recurrent bleeding unresponsive to other treatments.
medicationWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Juvenile Polyposis Hereditary Hemorrhagic Telangiectasia Syndrome C1832942?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
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If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy