ICD-10: H35.33

Angioid streaks of the macula, classified under ICD-10 code H35.33, are a significant ocular condition characterized by the presence of irregular, jagged lines in the retina, particularly in the macular region. These streaks are often associated with various systemic conditions and can lead to serious visual impairment.

Clinical Description

Definition and Characteristics

Angioid streaks are described as thin, grayish or brownish lines that radiate from the optic disc and can be seen on fundoscopic examination. They are typically a result of breaks in Bruch's membrane, which is a layer of tissue in the eye that supports the retina. The presence of these streaks can indicate underlying conditions such as pseudoxanthoma elasticum, Ehlers-Danlos syndrome, or other connective tissue disorders[1].

Pathophysiology

The pathophysiology of angioid streaks involves the calcification and fragmentation of elastic fibers in Bruch's membrane. This structural alteration can lead to the development of these streaks, which may predispose individuals to complications such as choroidal neovascularization (CNV) and subsequent vision loss. The streaks themselves do not typically cause symptoms unless they are associated with complications[2].

Symptoms

Patients with angioid streaks may not experience any symptoms initially. However, as the condition progresses or if complications arise, symptoms can include:
- Blurred vision
- Distorted vision (metamorphopsia)
- Central scotomas (blind spots in the central vision)
- Decreased visual acuity[3].

Diagnosis

Clinical Examination

Diagnosis of angioid streaks is primarily made through a comprehensive eye examination, including:
- Fundoscopy: Direct visualization of the retina to identify the characteristic streaks.
- Fluorescein Angiography: This imaging technique can help assess the extent of any associated choroidal neovascularization and the overall health of the retinal vasculature[4].

Differential Diagnosis

It is essential to differentiate angioid streaks from other retinal conditions that may present similarly, such as:
- Choroidal rupture
- Retinal tears
- Other forms of retinal degeneration[5].

Management and Treatment

Monitoring

Management of angioid streaks typically involves regular monitoring of the condition, especially for patients at risk of developing complications like CNV. This may include:
- Routine eye exams
- Visual field testing
- Optical coherence tomography (OCT) to monitor retinal changes[6].

Treatment Options

If complications arise, such as CNV, treatment options may include:
- Anti-VEGF Therapy: Injections of anti-vascular endothelial growth factor (VEGF) medications can help reduce neovascularization and preserve vision.
- Photodynamic Therapy: This treatment involves the use of light-activated drugs to target abnormal blood vessels in the retina[7].

Conclusion

Angioid streaks of the macula, represented by ICD-10 code H35.33, are a notable ocular condition that can lead to significant visual impairment if not monitored and managed appropriately. Regular eye examinations and prompt treatment of any complications are crucial for maintaining visual health in affected individuals. Understanding the underlying systemic associations and potential complications is essential for effective management and patient education.

References

1. Clinical Policy: Extended Ophthalmoscopy.
2. Disorders of choroid and retina (H30-H36).
3. Local Coverage Article: Billing and Coding: Ophthalmology.
4. Billing and Coding: Ophthalmology: Posterior Segment.
5. Common ICD-10 Diagnosis Codes that support the noted conditions.
6. Contractor Information.
7. Clinical Policy: Destruction of Localized Lesion of Choroid.

Angioid streaks of the macula, classified under ICD-10 code H35.33, are a significant ocular condition characterized by the presence of irregular, jagged lines in the retina, particularly in the macular region. These streaks are often associated with various systemic conditions and can lead to serious visual impairment. Below is a detailed overview of the clinical presentation, signs, symptoms, and patient characteristics associated with angioid streaks of the macula.

Clinical Presentation

Definition and Etiology

Angioid streaks are breaks in Bruch's membrane, which is a thin layer of tissue that supports the retina. They can be associated with several systemic diseases, including:
- Pseudoxanthoma elasticum: A genetic disorder affecting elastic fibers in connective tissue.
- Ehlers-Danlos syndrome: A group of disorders affecting connective tissues.
- Paget's disease of bone: A chronic disorder that can result in enlarged and deformed bones.
- Sickle cell disease: A genetic blood disorder that can affect blood flow to the eyes.

Patient Characteristics

Patients with angioid streaks often present with a history of systemic conditions that predispose them to this retinal finding. They may be of varying ages, but conditions like pseudoxanthoma elasticum typically manifest in young adults.

Signs and Symptoms

Visual Symptoms

Patients with angioid streaks may experience:
- Blurred vision: This can occur due to the involvement of the macula, where the streaks are located.
- Scotomas: Patients may report blind spots in their vision, particularly if the streaks lead to complications such as choroidal neovascularization.
- Metamorphopsia: Distortion of visual images can occur, especially if the macula is affected.

Ophthalmic Signs

During a comprehensive eye examination, the following signs may be observed:
- Angioid streaks: These appear as dark, irregular lines radiating from the optic disc towards the periphery of the retina.
- RPE changes: Retinal pigment epithelium (RPE) alterations may be noted, including atrophy or hyperplasia.
- Choroidal neovascularization: This is a potential complication where new, abnormal blood vessels grow under the retina, leading to further vision loss.

Systemic Associations

Given the systemic nature of conditions associated with angioid streaks, patients may also present with:
- Skin changes: Such as the characteristic yellowish papules seen in pseudoxanthoma elasticum.
- Bone deformities: In cases related to Paget's disease, patients may exhibit skeletal abnormalities.

Conclusion

Angioid streaks of the macula (ICD-10 code H35.33) represent a complex interplay between ocular and systemic health. The clinical presentation is marked by specific visual symptoms and distinct ophthalmic signs, often linked to underlying systemic conditions. Early detection and management are crucial to prevent complications such as vision loss, making awareness of the associated patient characteristics essential for healthcare providers. Regular monitoring and comprehensive eye examinations are recommended for individuals at risk, particularly those with known systemic diseases.

Angioid streaks of the macula, classified under the ICD-10-CM code H35.33, are associated with various conditions and terminologies in the field of ophthalmology. Understanding these alternative names and related terms can enhance clarity in diagnosis and treatment discussions. Below are some of the key terms associated with this condition.

Alternative Names for Angioid Streaks of Macula

1. Angioid Streaks: This is the most common term used to describe the condition, referring to the irregular, streak-like changes in the retina that can be observed during an eye examination.

2. Choroidal Ruptures: Although not identical, this term is sometimes used interchangeably in discussions about retinal changes, particularly when discussing the consequences of trauma or other conditions that may lead to similar appearances.

3. Pseudoxanthoma Elasticum (PXE): Angioid streaks are often associated with PXE, a genetic disorder that affects elastic fibers in the body, leading to skin, vascular, and ocular manifestations.

4. Ehlers-Danlos Syndrome: This connective tissue disorder can also present with angioid streaks, making it a related term in clinical discussions.

5. Streaks of the Retina: A more general term that may be used to describe the appearance of angioid streaks, though it lacks the specificity of the term "angioid."

Related Terms and Conditions

1. Macular Degeneration: While not directly synonymous, macular degeneration can coexist with angioid streaks, particularly in older patients, and is often discussed in the context of retinal health.

2. Choroidal Neovascularization: This condition can occur as a complication of angioid streaks, leading to vision loss and is often a focus in treatment discussions.

3. Retinal Pigment Epithelium (RPE) Changes: Changes in the RPE can be associated with angioid streaks and are important in the assessment of retinal health.

4. Fundus Flavimaculatus: This term refers to a condition that can present with similar retinal findings and may be relevant in differential diagnoses.

5. Retinal Hemorrhage: This term may be used in conjunction with angioid streaks, especially if there is associated bleeding in the retina.

Conclusion

Understanding the alternative names and related terms for ICD-10 code H35.33 is crucial for healthcare professionals involved in the diagnosis and treatment of retinal conditions. These terms not only facilitate clearer communication among specialists but also enhance patient education regarding their condition. If you have further questions or need more specific information about angioid streaks or related conditions, feel free to ask!

The diagnosis of angioid streaks of the macula, classified under ICD-10 code H35.33, involves specific clinical criteria and diagnostic evaluations. Angioid streaks are irregular, jagged lines that appear in the retina, often associated with various systemic conditions. Here’s a detailed overview of the criteria used for diagnosis:

Clinical Criteria for Diagnosis

1. Fundoscopic Examination:
   - The primary method for diagnosing angioid streaks is through a comprehensive eye examination, particularly a fundoscopic examination. During this examination, the presence of streaks radiating from the optic disc towards the periphery of the retina is noted. These streaks are typically brownish or grayish in color and can be mistaken for other retinal conditions if not carefully evaluated[1].

2. Patient History:
   - A thorough patient history is essential. Clinicians will inquire about any systemic diseases that may be associated with angioid streaks, such as pseudoxanthoma elasticum, Ehlers-Danlos syndrome, or Paget's disease of bone. A family history of these conditions may also be relevant[2].

3. Visual Acuity Assessment:
   - Patients may undergo visual acuity tests to determine the impact of angioid streaks on vision. While the streaks themselves may not always cause vision loss, associated complications, such as choroidal neovascularization, can lead to significant visual impairment[3].

4. Additional Imaging:
   - Optical coherence tomography (OCT) and fluorescein angiography may be utilized to assess the extent of retinal damage and to visualize any associated complications. These imaging techniques help in identifying any leakage or neovascularization that may occur due to the presence of angioid streaks[4].

5. Exclusion of Other Conditions:
   - It is crucial to differentiate angioid streaks from other retinal pathologies. This may involve additional tests to rule out conditions such as choroidal rupture, retinal tears, or other forms of retinal degeneration[5].

Conclusion

The diagnosis of angioid streaks of the macula (ICD-10 code H35.33) relies on a combination of clinical examination, patient history, visual assessments, and advanced imaging techniques. Proper diagnosis is essential not only for treatment planning but also for monitoring potential complications that can arise from this condition. If you suspect the presence of angioid streaks, it is advisable to consult an eye care professional for a comprehensive evaluation.

Angioid streaks of the macula, classified under ICD-10 code H35.33, are associated with various underlying conditions and can lead to significant visual impairment. Understanding the standard treatment approaches for this condition is crucial for effective management. Below, we explore the treatment options, their indications, and the underlying considerations.

Understanding Angioid Streaks

Angioid streaks are irregular, jagged lines that appear in the retina, typically radiating from the optic disc. They are often associated with conditions such as pseudoxanthoma elasticum, Ehlers-Danlos syndrome, and other systemic diseases. The presence of these streaks can lead to complications, including choroidal neovascularization (CNV), which can severely affect vision.

Standard Treatment Approaches

1. Observation and Monitoring

In cases where angioid streaks are asymptomatic and not associated with CNV, a conservative approach involving regular monitoring may be appropriate. This includes:

• Routine Eye Examinations: Regular dilated fundus examinations to monitor for changes in the retina.
• Visual Acuity Tests: Periodic assessments to track any changes in vision.

2. Management of Underlying Conditions

Since angioid streaks can be associated with systemic diseases, managing these underlying conditions is essential. This may involve:

• Genetic Counseling: For patients with hereditary conditions like pseudoxanthoma elasticum.
• Systemic Treatment: Addressing any systemic issues that may contribute to the progression of retinal changes.

3. Treatment of Choroidal Neovascularization (CNV)

If CNV develops as a complication of angioid streaks, more aggressive treatment is warranted. Standard approaches include:

• Anti-VEGF Therapy: Intravitreal injections of vascular endothelial growth factor (VEGF) inhibitors, such as ranibizumab or aflibercept, are commonly used to reduce CNV and improve visual outcomes. These treatments aim to inhibit the abnormal blood vessel growth associated with CNV[5][9].

• Photodynamic Therapy (PDT): This involves the use of a light-activated drug to target and destroy abnormal blood vessels. PDT can be effective in managing CNV associated with angioid streaks, particularly in cases where anti-VEGF therapy is insufficient[7].

4. Laser Photocoagulation

In certain cases, laser photocoagulation may be employed to treat CNV. This technique involves using a laser to seal off leaking blood vessels, although it is less commonly used now due to the effectiveness of anti-VEGF therapies.

5. Supportive Therapies

Patients may benefit from supportive therapies, including:

• Low Vision Rehabilitation: For those with significant vision loss, low vision aids and rehabilitation services can help maximize remaining vision.
• Patient Education: Informing patients about their condition, potential complications, and the importance of regular follow-up care.

Conclusion

The management of angioid streaks of the macula (ICD-10 code H35.33) requires a multifaceted approach that includes monitoring, treatment of underlying conditions, and intervention for complications like CNV. Anti-VEGF therapy has become a cornerstone of treatment, significantly improving outcomes for affected patients. Regular follow-up and patient education are essential components of care to ensure optimal management and support for individuals with this condition.