2022 ICD-10-CM Code I78.0

Hereditary hemorrhagic telangiectasia

Version 2021

Valid for Submission

ICD-10:I78.0
Short Description:Hereditary hemorrhagic telangiectasia
Long Description:Hereditary hemorrhagic telangiectasia

Code Classification

  • Diseases of the circulatory system (I00–I99)
    • Diseases of arteries, arterioles and capillaries (I70-I79)
      • Diseases of capillaries (I78)

I78.0 is a billable diagnosis code used to specify a medical diagnosis of hereditary hemorrhagic telangiectasia. The code I78.0 is valid during the fiscal year 2022 from October 01, 2021 through September 30, 2022 for the submission of HIPAA-covered transactions.

The ICD-10-CM code I78.0 might also be used to specify conditions or terms like brachydactyly of hand, conjunctival telangiectasis, epilepsy telangiectasia syndrome, familial cutaneous telangiectasia and oropharyngeal cancer predisposition syndrome, hereditary benign telangiectasia , hereditary dysplasia of blood vessel, etc.

Tabular List of Diseases and Injuries

The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with coding notes and guidance for inclusions, exclusions, descriptions and more. The following references are applicable to the code I78.0:


Inclusion Terms

Inclusion Terms
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.

Index to Diseases and Injuries

The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for the code I78.0 are found in the index:

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

Clinical Information

Convert I78.0 to ICD-9 Code

Information for Patients


Bleeding Disorders

Normally, if you get hurt, your body forms a blood clot to stop the bleeding. For blood to clot, your body needs cells called platelets and proteins known as clotting factors. If you have a bleeding disorder, you either do not have enough platelets or clotting factors or they don't work the way they should.

Bleeding disorders can be the result of other diseases, such as severe liver disease or a lack of vitamin K. They can also be inherited. Hemophilia is an inherited bleeding disorder. Bleeding disorders can also be a side effect of medicines such as blood thinners.

Various blood tests can check for a bleeding disorder. You will also have a physical exam and history. Treatments depend on the cause. They may include medicines and transfusions of blood, platelets, or clotting factor.


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Vascular Diseases

What are vascular diseases?

Your vascular system is your body's network of blood vessels. It includes your

Vascular diseases are conditions which affect your vascular system. They are common and can be serious. Some types include

What causes vascular diseases?

The causes of vascular diseases depend on the specific disease. These causes include

Sometimes the cause is unknown.

Who is at risk for vascular diseases?

The risk factors for vascular diseases can vary, depending on the specific disease. But some of the more common risk factors include

What are the symptoms of vascular diseases?

The symptoms for each disease are different.

How are vascular diseases diagnosed?

To make a diagnosis, your health care provider will do a physical exam and ask about your symptoms and medical history. You may have imaging tests and/or blood tests.

How are vascular diseases treated?

Which treatment you get depends on which vascular disease you have and how severe it is. Types of treatments for vascular diseases include

Can vascular diseases be prevented?

There are steps you can take to help prevent vascular diseases:


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Hereditary hemorrhagic telangiectasia

Hereditary hemorrhagic telangiectasia is a disorder that results in the development of multiple abnormalities in the blood vessels.

In the circulatory system, blood carrying oxygen from the lungs is normally pumped by the heart into the arteries at high pressure. The pressure allows the blood to make its way through the arteries to the smaller vessels (arterioles and capillaries) that supply oxygen to the body's tissues. By the time blood reaches the capillaries, the pressure is much lower. The blood then proceeds from the capillaries into veins, through which it eventually returns to the heart.

In hereditary hemorrhagic telangiectasia, some arterial vessels flow directly into veins rather than into the capillaries. These abnormalities are called arteriovenous malformations. When they occur in vessels near the surface of the skin, where they are visible as red markings, they are known as telangiectases (the singular is telangiectasia).

Without the normal buffer of the capillaries, the blood moves from the arteries at high pressure into the thinner walled, less elastic veins. The extra pressure tends to strain and enlarge these blood vessels, and may result in compression or irritation of adjacent tissues and frequent episodes of severe bleeding (hemorrhage). Nosebleeds are very common in people with hereditary hemorrhagic telangiectasia, and more serious problems may arise from hemorrhages in the brain, liver, lungs, or other organs.

There are several forms of hereditary hemorrhagic telangiectasia, distinguished mainly by their genetic cause but with some differences in patterns of signs and symptoms. People with type 1 tend to develop symptoms earlier than those with type 2, and are more likely to have blood vessel malformations in the lungs and brain. Type 2 and type 3 may be associated with a higher risk of liver involvement. Women are more likely than men to develop blood vessel malformations in the lungs with type 1, and are also at higher risk of liver involvement with both type 1 and type 2. Individuals with any form of hereditary hemorrhagic telangiectasia, however, can have any of these problems.

Juvenile polyposis/hereditary hemorrhagic telangiectasia syndrome is a condition that involves both arteriovenous malformations and a tendency to develop growths (polyps) in the gastrointestinal tract. Hereditary hemorrhagic telangiectasia types 1, 2 and 3 do not appear to increase the likelihood of such polyps.


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Code History

  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016 (First year ICD-10-CM implemented into the HIPAA code set)