Basal Laminar Drusen

Basal Laminar Drusen

Article
Eye & Vision
Women's Health
+3
Contributed byKrish Tangella MD, MBAJan 29, 2024

What are the other Names for this Condition? (Also known as/Synonyms)

  • Cuticular Drusen
  • Diffuse Drusen
  • Early-Adult Onset Grouped Drusen

What is Basal Laminar Drusen? (Definition/Background Information)

  • Basal Laminar Drusen (BLD) is a rare disorder, which is considered to be part of disorders causing age-related macular degeneration (AMD). It is a subtype of early-onset Drusen
  • The characteristic findings of the condition are the presence of small yellow Drusen detected during fundic autofluorescence study. Fluorescent angiography shows the presence of punctate areas of staining, creating a “stars in the sky” pattern
  • Drusens are small yellowish deposits that accumulate around the retina. The presence of Drusen without any associated complications does not require any treatment. However, Drusens may be indicative of an increased risk for AMD
  • Understanding the characteristics of Basal Laminar Drusen is vital for early detection and management, as their presence may indicate an increased risk of advanced AMD. Management strategies may involve lifestyle modifications, nutritional interventions, and regular monitoring by an eye care professional

Who gets Basal Laminar Drusen? (Age and Sex Distribution)

  • Basal Laminar Drusen (BLD) is primarily observed in adults; the onset of BLD may take place during young adulthood
  • Both males and females may be affected; some studies indicate a strong preference for females (between 60-90% of the cases are observed in them)
  • Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Basal Laminar Drusen? (Predisposing Factors)

The following risk factors are noted for Basal Laminar Drusen:

  • Use of tobacco (cigarette smoking)
  • Individuals with certain specific genotypes, such as the complement factor H Y402H variant
  • A positive family history of age-related macular degeneration
  • Female gender

It is important to note that having a risk factor does not mean one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Basal Laminar Drusen? (Etiology)

  • Currently, the cause of the development of Basal Laminar Drusen (BLD) is not well understood. It is a subtype of early-onset drusen
  • The name Basal Laminar Drusen is given due to imaging study findings of thickening of the basal membrane of retinal pigment epithelium (RPE)
  • There is evidence to suggest a genetic component in the development of BLD, with certain genetic variants associated with an increased risk

If inherited, BLD can be autosomal dominant or autosomal recessive. In the case of autosomal recessive inheritance, the condition is also known as Fleck Retina Disease.

What are the Signs and Symptoms of Basal Laminar Drusen?

In many cases, Basal Laminar Drusen (BLD) is typically asymptomatic; and by itself, drusen findings are insignificant.

  • These Drusen are found between the retinal pigment epithelium (RPE) and Bruch's membrane, which are layers within the retina
  • BLD may appear as small, round deposits with a characteristic distribution pattern.
  • Patients with vitelliform pigment epithelial detachment, choroidal neovascularization (CNV), or geographic atrophy may present with decreased visual acuity and visual distortion

In some individuals, certain symptoms may start appearing with age (gradually over time).

How is Basal Laminar Drusen Diagnosed?

A diagnosis of Basal Laminar Drusen is based on the clinical findings of a fundoscopic exam. Additionally, the following observations and tests may be performed:

  • Physical examination and medical history evaluation
  • Assessment of signs and symptoms
  • General eye exam
  • Fundoscopic (ophthalmoscopic) examination by an eye specialist who examines the back part of the eye (or the fundus). During the fundic exam, the following findings may also be noted:
    • Vitelliform pigment epithelial detachment that is often bilateral
    • Choroidal neovascularization (CNV)
    • Geographic atrophy (GA)
  • Visual acuity test using a unique and standardized test chart (Snellen chart)
  • Slit-lamp examination
  • Gonioscopy, a painless eye exam using special devices
  • Visual evoked potential (VEP) test

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Basal Laminar Drusen?

The complications of Basal Laminar Drusen may include:

  • Emotional stress
  • Bilateral vitelliform pigment epithelial detachment
  • Choroidal neovascularization
  • Geographic atrophy
  • Reduced quality of life if vision is affected

How is Basal Laminar Drusen Treated?

Basal Laminar Drusen (BLD) management mainly includes closely monitoring the patient to detect the development of complications early (if any).

  • Bilateral vitelliform pigment epithelial detachment, choroidal neovascularization (CNV), and geographic atrophy should be diagnosed early and treated promptly
  • In individuals who develop CNV, photodynamic therapy is helpful

In the case of isolated BLD (when no associated eye condition is observed), the healthcare provider may not recommend any treatment.

How can Basal Laminar Drusen be Prevented?

No specific methods or guidelines are presently available to prevent Basal Laminar Drusen (BLD).

  • Lifestyle modifications, dietary changes, and regular checkups are recommended for early detection of eye conditions
  • In some cases, analyzing genetics for specific mutations can assist in guiding patients who possess identified alleles linked to BLD progression

What is the Prognosis of Basal Laminar Drusen? (Outcomes/Resolutions)

The prognosis of Basal Laminar Drusen depends on the severity of the condition and one’s response to treatment.

  • An early diagnosis and prompt medical intervention can help improve the outcomes
  • The development of complications may worsen the prognosis

Additional and Relevant Useful Information for Basal Laminar Drusen:

Please visit our Eye & Vision Health Center for more physician-approved health information:

https://www.dovemed.com/health-topics/vision-center/

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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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