Valid for Submission
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 TermsInclusion 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.
- Rendu-Osler-Weber disease
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:
- - Disease, diseased - See Also: Syndrome;
- - Osler (-Weber)-Rendu disease - I78.0
- - Rendu-Osler-Weber disease or syndrome - I78.0
- - Telangiectasia, telangiectasis (verrucous) - I78.1
- - Weber-Osler syndrome - I78.0
The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:
- Brachydactyly of hand
- Conjunctival telangiectasis
- Epilepsy telangiectasia syndrome
- Familial cutaneous telangiectasia and oropharyngeal cancer predisposition syndrome
- Hereditary benign telangiectasia
- Hereditary dysplasia of blood vessel
- Hypotrichosis, lymphedema, telangiectasia, renal defect syndrome
- Osler hemorrhagic telangiectasia syndrome
- Vascular abnormality of conjunctiva
- TELANGIECTASIA HEREDITARY HEMORRHAGIC-. an autosomal dominant vascular anomaly characterized by telangiectases of the skin and mucous membranes and by recurrent gastrointestinal bleeding. this disorder is caused by mutations of a gene on chromosome 9q3 which encodes endoglin a membrane glycoprotein that binds transforming growth factor beta.
Diagnostic Related Groups - MS-DRG Mapping
|MS-DRG||MS-DRG Title||MCD||Relative Weight|
|299||PERIPHERAL VASCULAR DISORDERS WITH MCC||05||1.5314|
|300||PERIPHERAL VASCULAR DISORDERS WITH CC||05||1.0422|
|301||PERIPHERAL VASCULAR DISORDERS WITHOUT CC/MCC||05||0.7425|
The relative weight of a diagnostic related group determines the reimbursement rate based on the severity of a patient's illness and the associated cost of care during hospitalization.
Convert I78.0 to ICD-9 Code
Information for Patients
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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What are vascular diseases?
Your vascular system is your body's network of blood vessels. It includes your
- Arteries, which carry oxygen-rich blood from your heart to your tissues and organs
- Veins, which carry the blood and waste products back to your heart
- Capillaries, which are tiny blood vessels that connect your small arteries to your small veins. The walls of the capillaries are thin and leaky, to allow for an exchange of materials between your tissues and blood.
Vascular diseases are conditions which affect your vascular system. They are common and can be serious. Some types include
- Aneurysm - a bulge or "ballooning" in the wall of an artery
- Atherosclerosis - a disease in which plaque builds up inside your arteries. Plaque is made up of fat, cholesterol, calcium, and other substances found in the blood.
- Blood clots, including deep vein thrombosis and pulmonary embolism
- Coronary artery disease and carotid artery disease, diseases that involve the narrowing or blockage of an artery. The cause is usually a buildup of plaque.
- Raynaud's disease - a disorder that causes the blood vessels to narrow when you are cold or feeling stressed
- Stroke - a serious condition that happens when blood flow to your brain stops.
- Varicose veins - swollen, twisted veins that you can see just under the skin
- Vasculitis - inflammation of the blood vessels
What causes vascular diseases?
The causes of vascular diseases depend on the specific disease. These causes include
- Heart diseases such as high cholesterol and high blood pressure
- Medicines, including hormones
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
- Age - your risk of some diseases goes up as you get older
- Conditions that can affect the heart and blood vessels, such as diabetes or high cholesterol
- Family history of vascular or heart diseases
- Infection or injury that damages your veins
- Lack of exercise
- Sitting or standing still for long periods of time
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
- Lifestyle changes, such as eating a heart-healthy diet and getting more exercise
- Medicines, such as blood pressure medicines, blood thinners, cholesterol medicines, and clot-dissolving drugs. In some cases, providers use a catheter to send medicine directly to a blood vessel.
- Non-surgical procedures, such as angioplasty, stenting, and vein ablation
Can vascular diseases be prevented?
There are steps you can take to help prevent vascular diseases:
- Make healthy lifestyle changes, such as eating a heart-healthy diet and getting more exercise
- Don't smoke. If you are already a smoker, talk to your health care provider for help in finding the best way for you to quit.
- Keep your blood pressure and cholesterol in check
- If you have diabetes, control your blood sugar
- Try not to sit or stand for up long periods of time. If you do need to sit all day, get up and move around every hour or so. If you traveling on a long trip, you can also wear compression stockings and regularly stretch your legs.
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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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