ICD-10: H36.82

Proliferative sickle-cell retinopathy

Additional Information

Description

Proliferative sickle-cell retinopathy (ICD-10 code H36.82) is a serious ocular complication associated with sickle cell disease, characterized by the growth of abnormal blood vessels in the retina. This condition can lead to significant vision impairment if not diagnosed and treated promptly. Below is a detailed overview of the clinical description, symptoms, diagnosis, and management of proliferative sickle-cell retinopathy.

Clinical Description

Pathophysiology

Proliferative sickle-cell retinopathy occurs due to the occlusion of retinal blood vessels caused by sickle-shaped red blood cells. This occlusion leads to ischemia (lack of blood flow) in the retina, which triggers the release of vascular endothelial growth factor (VEGF). The increased levels of VEGF stimulate the formation of new, abnormal blood vessels (neovascularization) in an attempt to restore blood supply to the affected areas of the retina. However, these new vessels are fragile and prone to bleeding, which can result in further complications such as vitreous hemorrhage and retinal detachment[1].

Symptoms

Patients with proliferative sickle-cell retinopathy may experience a range of symptoms, including:
- Visual disturbances: Blurred vision or sudden vision loss, particularly in advanced stages.
- Floaters: The presence of spots or lines in the visual field, often due to bleeding from abnormal vessels.
- Photopsia: The perception of flashes of light, which can occur with retinal traction or detachment.
- Peripheral vision loss: Difficulty seeing objects outside of the central vision, which may progress over time[1][2].

Diagnosis

Clinical Examination

Diagnosis typically involves a comprehensive eye examination, including:
- Fundoscopy: Direct examination of the retina to identify signs of neovascularization, hemorrhages, and other retinal changes.
- Fluorescein angiography: A diagnostic procedure that uses a fluorescent dye to visualize blood flow in the retina and identify areas of ischemia and neovascularization.
- Optical coherence tomography (OCT): Imaging that provides cross-sectional views of the retina, helping to assess the extent of retinal damage and fluid accumulation[2][3].

Risk Factors

Individuals with sickle cell disease are at increased risk for developing proliferative sickle-cell retinopathy, particularly those with a history of severe anemia, frequent vaso-occlusive crises, or prior episodes of retinal hemorrhage. Regular eye examinations are recommended for patients with sickle cell disease, especially as they reach adolescence and adulthood[1][3].

Management

Treatment Options

Management of proliferative sickle-cell retinopathy focuses on preventing vision loss and may include:
- Laser photocoagulation: A common treatment that involves using laser energy to destroy abnormal blood vessels and reduce the risk of bleeding.
- Anti-VEGF injections: Medications that inhibit the action of VEGF can be injected into the eye to reduce neovascularization and stabilize vision.
- Vitrectomy: In cases of significant vitreous hemorrhage or retinal detachment, surgical intervention may be necessary to remove the vitreous gel and repair the retina[2][3].

Follow-Up Care

Regular follow-up with an ophthalmologist is crucial for monitoring the progression of the disease and the effectiveness of treatment. Patients are advised to report any new visual symptoms immediately, as early intervention can significantly improve outcomes[1][2].

Conclusion

Proliferative sickle-cell retinopathy is a serious complication of sickle cell disease that requires prompt diagnosis and management to prevent vision loss. Understanding the clinical features, diagnostic methods, and treatment options is essential for healthcare providers involved in the care of patients with sickle cell disease. Regular eye examinations and proactive management strategies can help mitigate the risks associated with this condition, ultimately preserving vision and improving quality of life for affected individuals.


References

  1. Billing and Coding: Ophthalmology: Posterior Segment.
  2. New ICD-10 Codes for 2024: Parkinson's, Retinopathy &.
  3. Clinical Policy: Fluorescein Angiography.

Clinical Information

Proliferative sickle-cell retinopathy (PSCR) is a serious ocular complication associated with sickle cell disease (SCD). This condition can lead to significant visual impairment if not diagnosed and managed appropriately. Below is a detailed overview of the clinical presentation, signs, symptoms, and patient characteristics associated with ICD-10 code H36.82.

Clinical Presentation

Proliferative sickle-cell retinopathy typically occurs in patients with a history of sickle cell disease, particularly in those with the HbSS or HbSC genotypes. The condition is characterized by the formation of new blood vessels (neovascularization) in the retina due to ischemia (lack of blood flow) caused by sickling of red blood cells.

Signs and Symptoms

  1. Visual Disturbances:
    - Patients may experience blurred vision, which can progress to severe vision loss.
    - Some may report floaters or flashes of light due to retinal detachment or hemorrhage.

  2. Fundoscopic Findings:
    - Neovascularization: New, abnormal blood vessels can be observed on the retina or optic disc.
    - Retinal Hemorrhages: These may appear as flame-shaped or dot-and-blot hemorrhages.
    - Cotton Wool Spots: These are soft exudates that indicate retinal ischemia.
    - Retinal Detachment: In advanced cases, the retina may detach, leading to more severe vision loss.

  3. Other Ocular Symptoms:
    - Patients may experience eye pain or discomfort, particularly if there is associated hemorrhage or retinal detachment.
    - In some cases, there may be no symptoms until significant vision loss occurs, highlighting the importance of regular eye examinations in at-risk populations.

Patient Characteristics

  1. Demographics:
    - Proliferative sickle-cell retinopathy primarily affects individuals with sickle cell disease, which is more prevalent in individuals of African, Mediterranean, Middle Eastern, and Indian descent.
    - The onset of PSCR typically occurs in young adults, often between the ages of 20 and 40, although it can develop earlier in life.

  2. Medical History:
    - A confirmed diagnosis of sickle cell disease (HbSS or HbSC) is essential.
    - Patients may have a history of vaso-occlusive crises, acute chest syndrome, or other complications related to sickle cell disease.

  3. Risk Factors:
    - The risk of developing PSCR increases with age and the severity of sickle cell disease.
    - Patients with a history of frequent pain crises or those who have undergone multiple blood transfusions may be at higher risk.

  4. Screening and Monitoring:
    - Regular ophthalmologic evaluations are recommended for patients with sickle cell disease, especially those over the age of 10, to monitor for signs of retinopathy.

Conclusion

Proliferative sickle-cell retinopathy is a significant complication of sickle cell disease that can lead to severe visual impairment. Understanding the clinical presentation, signs, symptoms, and patient characteristics is crucial for early detection and management. Regular eye examinations and prompt treatment of any retinal changes are essential to preserve vision in affected individuals. Given the potential for asymptomatic progression, proactive screening in at-risk populations is vital for improving outcomes.

Approximate Synonyms

Proliferative sickle-cell retinopathy, classified under the ICD-10 code H36.82, is a specific retinal disorder associated with sickle cell disease. Understanding alternative names and related terms can enhance clarity in medical documentation and communication. Here’s a detailed overview of the terminology associated with this condition.

Alternative Names

  1. Sickle Cell Retinopathy: This is a more general term that encompasses all forms of retinal damage caused by sickle cell disease, including both non-proliferative and proliferative stages.

  2. Proliferative Sickle Cell Retinopathy: While this is the formal name corresponding to the ICD-10 code, it is often used interchangeably with the code itself in clinical settings.

  3. Sickle Cell Disease-Related Retinopathy: This term emphasizes the connection between sickle cell disease and its ocular complications, including retinopathy.

  4. Retinal Neovascularization in Sickle Cell Disease: This term describes the pathological process involved in proliferative sickle-cell retinopathy, where new blood vessels form in the retina due to ischemia.

  1. Non-Proliferative Sickle Cell Retinopathy: This refers to the earlier stage of retinal changes associated with sickle cell disease, characterized by the absence of neovascularization.

  2. Sickle Cell Anemia: A specific type of sickle cell disease that can lead to various complications, including retinopathy.

  3. Retinal Hemorrhage: A common finding in sickle-cell retinopathy, where bleeding occurs within the retina due to fragile blood vessels.

  4. Ischemic Retinopathy: A broader term that can apply to any retinal condition caused by insufficient blood supply, including those seen in sickle cell disease.

  5. Ocular Complications of Sickle Cell Disease: This term encompasses all eye-related issues arising from sickle cell disease, including retinopathy, cataracts, and other vascular changes.

  6. H36.8: This is the broader category under which H36.82 falls, indicating retinal disorders classified elsewhere.

Conclusion

Understanding the alternative names and related terms for ICD-10 code H36.82 is crucial for healthcare professionals involved in the diagnosis and treatment of patients with sickle cell disease. This knowledge aids in accurate documentation, enhances communication among medical teams, and ensures that patients receive appropriate care for their ocular complications. If you have further questions or need additional information on this topic, feel free to ask!

Diagnostic Criteria

Proliferative sickle-cell retinopathy (ICD-10 code H36.82) is a serious ocular complication associated with sickle cell disease. The diagnosis of this condition involves a combination of clinical evaluation, imaging studies, and specific criteria that help differentiate it from other retinal disorders. Below is a detailed overview of the criteria used for diagnosing proliferative sickle-cell retinopathy.

Clinical Evaluation

Patient History

  • Sickle Cell Disease Diagnosis: Confirmation of sickle cell disease (SCD) is essential, as proliferative sickle-cell retinopathy occurs in patients with this underlying condition. This includes types such as HbSS, HbSC, and HbSβ-thalassemia.
  • Symptoms: Patients may report visual disturbances, such as blurred vision, floaters, or sudden vision loss, which can indicate retinal complications.

Physical Examination

  • Ocular Examination: A comprehensive eye examination is performed, including visual acuity testing and a detailed assessment of the anterior and posterior segments of the eye.

Diagnostic Imaging

Fundus Examination

  • Direct Ophthalmoscopy: This allows for the visualization of the retina and assessment of any abnormalities.
  • Indirect Ophthalmoscopy: Provides a wider view of the retina, which is crucial for identifying peripheral retinal changes.

Fluorescein Angiography

  • Assessment of Retinal Blood Flow: This imaging technique helps visualize the retinal vasculature and identify areas of non-perfusion, neovascularization, and other changes associated with proliferative sickle-cell retinopathy.

Optical Coherence Tomography (OCT)

  • Retinal Layer Analysis: OCT can be used to assess the thickness of retinal layers and detect any edema or structural changes in the retina.

Diagnostic Criteria

Key Findings

  • Neovascularization: The presence of new blood vessels on the retina or optic disc is a hallmark of proliferative sickle-cell retinopathy. These vessels are often fragile and can lead to vitreous hemorrhage.
  • Retinal Hemorrhages: The identification of preretinal or intraretinal hemorrhages is significant in the diagnosis.
  • Retinal Ischemia: Areas of non-perfusion or ischemia in the retina, often seen in fluorescein angiography, are indicative of the disease.

Classification

  • Stages of Retinopathy: The disease is often classified into stages, with proliferative changes indicating a more advanced form of retinopathy. The classification helps guide treatment decisions and monitor disease progression.

Conclusion

Diagnosing proliferative sickle-cell retinopathy (ICD-10 code H36.82) requires a thorough understanding of the patient's medical history, a detailed ocular examination, and the use of advanced imaging techniques to identify characteristic findings. The presence of neovascularization, retinal hemorrhages, and ischemic changes are critical in confirming the diagnosis. Early detection and management are essential to prevent vision loss and other complications associated with this condition. Regular follow-up and monitoring are recommended for patients with sickle cell disease to detect any ocular changes promptly.

Treatment Guidelines

Proliferative sickle-cell retinopathy (ICD-10 code H36.82) is a serious ocular complication associated with sickle cell disease, characterized by the growth of abnormal blood vessels in the retina due to ischemia and hypoxia. This condition can lead to significant vision loss if not managed appropriately. Here, we will explore the standard treatment approaches for this condition, including both medical and surgical interventions.

Understanding Proliferative Sickle-Cell Retinopathy

Proliferative sickle-cell retinopathy occurs when sickle-shaped red blood cells obstruct blood flow in the retinal vessels, leading to ischemia. In response, the retina may develop new, fragile blood vessels (neovascularization) that can bleed and cause further complications, such as retinal detachment. Early detection and treatment are crucial to prevent irreversible vision loss.

Standard Treatment Approaches

1. Regular Monitoring and Screening

  • Ophthalmic Examinations: Patients with sickle cell disease should undergo regular eye examinations, especially after the age of 10, to monitor for signs of retinopathy. This includes dilated fundus examinations and possibly fluorescein angiography to assess the extent of retinal changes.
  • Risk Assessment: Identifying patients at higher risk for developing proliferative retinopathy is essential. Factors include the severity of sickle cell disease and previous ocular complications.

2. Medical Management

  • Hydroxyurea: This medication is often used to reduce the frequency of sickle cell crises and may help decrease the incidence of retinopathy by increasing fetal hemoglobin levels, which can improve blood flow and reduce sickling events[1].
  • Blood Transfusions: Regular blood transfusions can help reduce the proportion of sickle hemoglobin in the blood, thereby decreasing the risk of vaso-occlusive events and subsequent retinal ischemia. This is particularly important in patients with severe retinopathy or those preparing for surgery[2].

3. Laser Therapy

  • Panretinal Photocoagulation (PRP): This is the primary surgical intervention for proliferative sickle-cell retinopathy. PRP involves using a laser to create small burns in the peripheral retina, which helps to reduce the stimulus for neovascularization by decreasing the ischemic area. This treatment can significantly reduce the risk of vision loss and complications such as vitreous hemorrhage and retinal detachment[3][4].

4. Surgical Interventions

  • Vitrectomy: In cases where there is significant vitreous hemorrhage or retinal detachment, a vitrectomy may be necessary. This procedure involves removing the vitreous gel and any blood that has accumulated in the eye, allowing for better visualization and potential repair of the retina[5].
  • Retinal Detachment Repair: If detachment occurs, surgical options such as scleral buckle or pneumatic retinopexy may be employed to reattach the retina.

5. Supportive Care

  • Patient Education: Educating patients about the importance of adherence to treatment regimens, recognizing symptoms of vision changes, and maintaining regular follow-up appointments is crucial for managing the condition effectively.
  • Multidisciplinary Approach: Collaboration between hematologists, ophthalmologists, and primary care providers is essential to provide comprehensive care for patients with sickle cell disease and associated ocular complications.

Conclusion

Proliferative sickle-cell retinopathy is a significant complication of sickle cell disease that requires prompt and effective management to prevent vision loss. Standard treatment approaches include regular monitoring, medical management with hydroxyurea and blood transfusions, laser therapy, and surgical interventions when necessary. A multidisciplinary approach ensures that patients receive comprehensive care tailored to their specific needs, ultimately improving their quality of life and visual outcomes. Regular follow-up and patient education are key components in managing this complex condition effectively.


References

  1. Hydroxyurea's role in sickle cell disease management.
  2. The impact of blood transfusions on sickle cell complications.
  3. Efficacy of panretinal photocoagulation in retinal ischemia.
  4. Guidelines for the management of proliferative sickle-cell retinopathy.
  5. Surgical options for retinal detachment in sickle cell patients.

Related Information

Description

  • Proliferative sickle-cell retinopathy is a serious ocular complication
  • Caused by growth of abnormal blood vessels in the retina
  • Associated with sickle cell disease and vision impairment
  • Characterized by ischemia, neovascularization, and bleeding
  • Symptoms include blurred vision, floaters, photopsia, and peripheral vision loss
  • Diagnosis involves fundoscopy, fluorescein angiography, and OCT imaging
  • Risk factors include sickle cell disease, severe anemia, and retinal hemorrhage
  • Treatment options include laser photocoagulation, anti-VEGF injections, and vitrectomy

Clinical Information

  • Proliferative sickle-cell retinopathy occurs in SCD patients
  • New blood vessels form due to ischemia caused by red cell sickling
  • Visual disturbances include blurred vision and floaters or flashes
  • Fundoscopic findings include neovascularization, hemorrhages, cotton wool spots, and retinal detachment
  • Ocular symptoms include eye pain and discomfort
  • Proliferative sickle-cell retinopathy primarily affects individuals of African descent
  • Onset typically occurs in young adults between 20-40 years old
  • Confirmed diagnosis of SCD is essential for PSCR development
  • Risk increases with age and severity of SCD

Approximate Synonyms

  • Sickle Cell Retinopathy
  • Proliferative Sickle Cell Retinopathy
  • Sickle Cell Disease-Related Retinopathy
  • Retinal Neovascularization in Sickle Cell Disease
  • Non-Proliferative Sickle Cell Retinopathy
  • Sickle Cell Anemia
  • Retinal Hemorrhage
  • Ischemic Retinopathy
  • Ocular Complications of Sickle Cell Disease
  • H36.8

Diagnostic Criteria

  • Confirmation of Sickle Cell Disease
  • Visual Disturbances reported by patient
  • Neovascularization on retina or optic disc
  • Retinal Hemorrhages identified
  • Retinal Ischemia seen in fluorescein angiography

Treatment Guidelines

  • Regular eye exams for patients with sickle cell disease
  • Hydroxyurea to reduce sickling events
  • Blood transfusions to decrease sickle hemoglobin levels
  • Panretinal photocoagulation (PRP) for neovascularization
  • Vitrectomy for significant vitreous hemorrhage or retinal detachment
  • Retinal detachment repair with scleral buckle or pneumatic retinopexy
  • Patient education and multidisciplinary care

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