ICD-10: I77.3

Arterial fibromuscular dysplasia

Clinical Information

Inclusion Terms

  • Fibromuscular hyperplasia (of) carotid artery
  • Fibromuscular hyperplasia (of) renal artery

Additional Information

Treatment Guidelines

Arterial fibromuscular dysplasia (FMD), classified under ICD-10 code I77.3, is a vascular condition characterized by abnormal growth of cells in the arterial wall, leading to stenosis, aneurysms, or dissections, primarily affecting the renal and carotid arteries. The management of FMD is multifaceted, focusing on symptom relief, prevention of complications, and addressing the underlying vascular abnormalities. Here’s a detailed overview of standard treatment approaches for this condition.

Diagnosis and Assessment

Before treatment can begin, a thorough diagnostic process is essential. This typically includes:

  • Imaging Studies: Magnetic Resonance Angiography (MRA) is often utilized to visualize the arterial structure and identify areas of stenosis or aneurysm[6]. Other imaging modalities may include CT angiography or conventional angiography.
  • Clinical Evaluation: A comprehensive assessment of symptoms, which may include hypertension, headaches, or neurological deficits, is crucial for tailoring treatment strategies.

Medical Management

1. Pharmacological Treatment

  • Antihypertensive Medications: Since FMD often leads to secondary hypertension, controlling blood pressure is a primary goal. Commonly prescribed medications include:
  • ACE Inhibitors: Such as lisinopril, which help relax blood vessels.
  • Angiotensin II Receptor Blockers (ARBs): Like losartan, which also aid in blood pressure control.
  • Calcium Channel Blockers: Such as amlodipine, which can be effective in managing hypertension associated with FMD[1].

  • Antiplatelet Therapy: In some cases, low-dose aspirin may be recommended to reduce the risk of thromboembolic events, especially if there are signs of vascular compromise[1].

2. Lifestyle Modifications

Patients are often advised to adopt lifestyle changes that can help manage symptoms and improve overall vascular health:

  • Dietary Changes: A heart-healthy diet low in sodium and saturated fats can help control blood pressure.
  • Regular Exercise: Engaging in regular physical activity can improve cardiovascular health and aid in weight management.
  • Smoking Cessation: Quitting smoking is crucial, as tobacco use can exacerbate vascular issues.

Interventional Procedures

In cases where medical management is insufficient or complications arise, interventional procedures may be necessary:

1. Endovascular Treatments

  • Angioplasty and Stenting: This minimally invasive procedure involves the insertion of a balloon catheter to widen the narrowed artery, often followed by the placement of a stent to maintain patency. This is particularly effective for renal and carotid artery stenosis due to FMD[1][2].

2. Surgical Options

  • Surgical Resection: In rare cases where there are significant aneurysms or if endovascular approaches are not feasible, surgical resection of the affected segment of the artery may be performed. This is typically considered a last resort due to the risks involved[1].

Monitoring and Follow-Up

Regular follow-up is essential for patients with FMD to monitor for disease progression and manage any complications. This may include:

  • Routine Imaging: Periodic MRA or ultrasound examinations to assess the status of the arteries.
  • Blood Pressure Monitoring: Regular checks to ensure that hypertension remains controlled.

Conclusion

The management of arterial fibromuscular dysplasia (ICD-10 code I77.3) requires a comprehensive approach that includes medical therapy, lifestyle modifications, and possibly interventional procedures. Early diagnosis and tailored treatment strategies are crucial for preventing complications and improving patient outcomes. Continuous monitoring and follow-up care are essential to adapt treatment plans as needed and ensure optimal management of this vascular condition.

Description

Arterial fibromuscular dysplasia (AFMD) is a rare vascular condition characterized by abnormal growth and development of the cells in the arterial wall, leading to stenosis (narrowing), aneurysms, or dissections of the affected arteries. The ICD-10 code for this condition is I77.3.

Clinical Description

Pathophysiology

AFMD primarily affects the medium-sized arteries, particularly the renal and carotid arteries. The condition is thought to result from a combination of genetic predisposition and environmental factors, although the exact cause remains unclear. The abnormal growth of smooth muscle cells and fibrous tissue leads to a characteristic "string of beads" appearance on imaging studies, which is a hallmark of the disease.

Symptoms

Patients with arterial fibromuscular dysplasia may present with a variety of symptoms, often depending on the arteries involved:

  • Renal Artery Involvement: This can lead to hypertension due to renal artery stenosis, which may manifest as resistant hypertension or sudden onset hypertension in younger individuals.
  • Cerebrovascular Involvement: Patients may experience transient ischemic attacks (TIAs) or strokes due to compromised blood flow in the carotid arteries.
  • Peripheral Artery Involvement: Symptoms may include claudication or limb ischemia if peripheral arteries are affected.

Diagnosis

Diagnosis of AFMD typically involves imaging studies, including:

  • Doppler Ultrasound: This non-invasive test can help visualize blood flow and detect stenosis.
  • CT Angiography or MR Angiography: These imaging modalities provide detailed images of the blood vessels and can reveal the characteristic "string of beads" appearance.
  • Angiography: In some cases, traditional angiography may be performed for a definitive diagnosis.

Treatment

Management of arterial fibromuscular dysplasia focuses on controlling symptoms and preventing complications:

  • Medications: Antihypertensive medications are often prescribed to manage hypertension. In some cases, antiplatelet therapy may be indicated.
  • Surgical Interventions: In severe cases, procedures such as angioplasty or stenting may be necessary to restore normal blood flow.
  • Monitoring: Regular follow-up with imaging studies is essential to monitor the progression of the disease and the effectiveness of treatment.

Conclusion

Arterial fibromuscular dysplasia, classified under ICD-10 code I77.3, is a significant vascular disorder that can lead to serious complications if not diagnosed and managed appropriately. Early recognition and a multidisciplinary approach to treatment are crucial for improving patient outcomes and quality of life. Regular monitoring and tailored therapeutic strategies can help mitigate the risks associated with this condition.

Clinical Information

Arterial fibromuscular dysplasia (AFMD) is a vascular condition characterized by abnormal growth and development of the cells in the arterial wall, leading to stenosis, aneurysms, or dissections. The ICD-10-CM code for arterial fibromuscular dysplasia is I77.3. Understanding the clinical presentation, signs, symptoms, and patient characteristics associated with this condition is crucial for timely diagnosis and management.

Clinical Presentation

Demographics

AFMD predominantly affects women, particularly those between the ages of 15 and 50. The condition is relatively rare, and its exact prevalence is not well established, but it is recognized as a significant cause of secondary hypertension in young women[1].

Symptoms

The symptoms of AFMD can vary widely depending on the arteries involved and the severity of the condition. Common symptoms include:

  • Hypertension: Often the first sign, especially in younger women, due to renal artery involvement.
  • Headaches: Patients may experience recurrent headaches, which can be attributed to cerebral artery involvement.
  • Dizziness or Lightheadedness: This may occur due to reduced blood flow to the brain.
  • Visual Disturbances: These can arise from carotid artery involvement, leading to transient ischemic attacks (TIAs) or strokes.
  • Neck Pain: Particularly if the cervical arteries are affected.
  • Abdominal Pain: This may occur if the mesenteric arteries are involved, leading to ischemia.

Signs

Upon physical examination, healthcare providers may note:

  • Bruits: Abnormal sounds over the arteries, particularly in the neck or abdomen, indicating turbulent blood flow.
  • Elevated Blood Pressure: Often significantly higher than expected for the patient's age and health status.
  • Neurological Deficits: In cases of cerebral involvement, patients may exhibit signs of stroke or TIA.

Patient Characteristics

Risk Factors

While the exact cause of AFMD remains unclear, several risk factors have been identified:

  • Gender: Female sex is a significant risk factor, with a higher incidence in women compared to men.
  • Age: Most commonly diagnosed in individuals aged 15 to 50 years.
  • Family History: A familial predisposition may exist, although the genetic basis is not well understood.

Associated Conditions

AFMD can be associated with other vascular conditions, including:

  • Aneurysms: Particularly in the renal and carotid arteries.
  • Dissections: Arterial dissections can occur, leading to acute complications.
  • Other Vascular Dysplasias: Patients may have other forms of vascular dysplasia or connective tissue disorders.

Conclusion

Arterial fibromuscular dysplasia is a complex condition that primarily affects young women and can lead to significant cardiovascular complications if not diagnosed and managed appropriately. Awareness of its clinical presentation, including common symptoms like hypertension and headaches, as well as the characteristic signs observed during physical examinations, is essential for healthcare providers. Early recognition and intervention can help mitigate the risks associated with this vascular disorder, improving patient outcomes and quality of life.

Approximate Synonyms

Arterial fibromuscular dysplasia (AFMD), classified under ICD-10 code I77.3, is a vascular condition characterized by abnormal growth or development of the cells in the arterial wall, leading to stenosis or aneurysm formation. This condition primarily affects the medium-sized arteries and is most commonly seen in the renal and carotid arteries. Below are alternative names and related terms associated with this condition.

Alternative Names for Arterial Fibromuscular Dysplasia

  1. Fibromuscular Dysplasia (FMD): This is the most common term used interchangeably with arterial fibromuscular dysplasia. It encompasses various forms of dysplasia affecting different vascular territories.

  2. Renal Fibromuscular Dysplasia: Specifically refers to fibromuscular dysplasia affecting the renal arteries, which can lead to hypertension and renal impairment.

  3. Cervical Fibromuscular Dysplasia: This term is used when the condition affects the arteries in the neck, particularly the carotid arteries.

  4. Medial Fibromuscular Dysplasia: A subtype of fibromuscular dysplasia that describes the specific histological changes in the media layer of the arterial wall.

  5. Multifocal Fibromuscular Dysplasia: This term indicates the presence of multiple lesions or areas of dysplasia within the arterial system.

  1. Vascular Dysplasia: A broader term that includes various abnormalities in the development of blood vessels, including fibromuscular dysplasia.

  2. Aneurysm: A potential complication of fibromuscular dysplasia, where a localized enlargement of an artery occurs due to the weakening of the arterial wall.

  3. Stenosis: Refers to the narrowing of the artery, which can occur due to fibromuscular dysplasia, leading to reduced blood flow.

  4. Hypertension: Often associated with renal fibromuscular dysplasia, where the narrowing of renal arteries can lead to secondary hypertension.

  5. Ischemia: A condition that may arise from reduced blood flow due to arterial stenosis, potentially leading to tissue damage.

  6. Endovascular Intervention: A treatment approach that may be employed for managing complications of fibromuscular dysplasia, such as stenting or angioplasty.

Understanding these alternative names and related terms can enhance communication among healthcare professionals and improve patient education regarding arterial fibromuscular dysplasia and its implications. If you have further questions or need more specific information, feel free to ask!

Diagnostic Criteria

Arterial fibromuscular dysplasia (AFMD) is a vascular condition characterized by abnormal growth and development of the arterial wall, leading to stenosis, aneurysms, or dissections. The ICD-10-CM code for this condition is I77.3. Diagnosing AFMD involves a combination of clinical evaluation, imaging studies, and specific diagnostic criteria. Below are the key criteria and methods used for diagnosis.

Clinical Evaluation

Symptoms

Patients with AFMD may present with a variety of symptoms, which can include:
- Hypertension: Often due to renal artery involvement.
- Headaches: Particularly if cerebral arteries are affected.
- Transient ischemic attacks (TIAs) or strokes: Resulting from compromised blood flow.
- Abdominal pain: If mesenteric arteries are involved.

Medical History

A thorough medical history is essential, including:
- Family history of vascular diseases.
- Previous episodes of vascular complications.
- Any known risk factors such as smoking or connective tissue disorders.

Imaging Studies

Non-Invasive Imaging

  1. Ultrasound: Doppler ultrasound can assess blood flow and detect abnormalities in the arteries.
  2. Magnetic Resonance Angiography (MRA): This imaging technique provides detailed images of blood vessels and can reveal characteristic "string of beads" appearance associated with AFMD.
  3. Computed Tomography Angiography (CTA): Similar to MRA, CTA can visualize arterial structures and identify stenosis or aneurysms.

Invasive Imaging

  • Digital Subtraction Angiography (DSA): Considered the gold standard for diagnosing AFMD, DSA allows for direct visualization of the arterial lumen and can confirm the presence of fibromuscular dysplasia.

Diagnostic Criteria

Radiological Findings

The diagnosis of AFMD is often supported by specific radiological findings, including:
- "String of beads" appearance: This is a classic finding on angiography, indicating alternating stenosis and aneurysms in the affected arteries.
- Segmental stenosis: Narrowing of the artery that can be visualized through imaging studies.

Exclusion of Other Conditions

It is crucial to rule out other potential causes of arterial abnormalities, such as:
- Atherosclerosis
- Vasculitis
- Other connective tissue disorders

Conclusion

The diagnosis of arterial fibromuscular dysplasia (ICD-10 code I77.3) relies on a combination of clinical symptoms, imaging studies, and exclusion of other vascular conditions. Non-invasive imaging techniques like ultrasound, MRA, and CTA, along with invasive DSA, play a critical role in confirming the diagnosis. Understanding these criteria is essential for healthcare providers to ensure accurate diagnosis and appropriate management of patients with AFMD.

Related Information

Treatment Guidelines

  • Use ACE inhibitors or ARBs for hypertension
  • Prescribe antiplatelet therapy in some cases
  • Adopt heart-healthy diet low in sodium and fats
  • Engage in regular physical activity
  • Quit smoking to prevent exacerbation
  • Consider angioplasty and stenting for stenosis
  • Reserve surgical resection for severe aneurysms

Description

  • Rare vascular condition causing arterial narrowing
  • Abnormal growth in arterial wall cells
  • Stenosis, aneurysms, or artery dissections
  • Medium-sized arteries affected primarily
  • Renal and carotid arteries involved commonly
  • Genetic predisposition and environmental factors
  • Transient ischemic attacks (TIAs) or strokes
  • Resistant hypertension or sudden onset hypertension
  • Claudication or limb ischemia in peripheral artery involvement

Clinical Information

  • Affects predominantly women
  • 15-50 years age group most common
  • Rare condition exact prevalence unknown
  • Hypertension often first sign
  • Headaches and dizziness common symptoms
  • Visual disturbances due to carotid involvement
  • Neck pain from cervical artery issues
  • Abdominal pain from mesenteric artery problems
  • Bruit sounds over affected arteries
  • Elevated blood pressure a key finding
  • Familial predisposition possible
  • Associated with aneurysms and dissections

Approximate Synonyms

  • Fibromuscular Dysplasia (FMD)
  • Renal Fibromuscular Dysplasia
  • Cervical Fibromuscular Dysplasia
  • Medial Fibromuscular Dysplasia
  • Multifocal Fibromuscular Dysplasia
  • Vascular Dysplasia

Diagnostic Criteria

  • Hypertension often due to renal artery involvement
  • Headaches if cerebral arteries are affected
  • Transient ischemic attacks or strokes from compromised blood flow
  • Abdominal pain if mesenteric arteries are involved
  • Family history of vascular diseases is essential
  • Previous episodes of vascular complications are considered
  • Smoking and connective tissue disorders are risk factors
  • String of beads appearance on angiography indicates AFMD
  • Segmental stenosis narrowing of the artery can be visualized
  • Atherosclerosis, vasculitis and other conditions must be ruled out

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