Ocular Manifestations of Albinism Treatment & Management

Updated: Dec 11, 2019
  • Author: Mohammed O Peracha, MD; Chief Editor: Hampton Roy, Sr, MD  more...
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Treatment

Medical Care

No specific medical treatment is available. Refractive errors should be corrected, and some patients benefit from bifocal lenses. If visual acuity is severely impaired, these patients can be helped with telescopic and other low-vision devices.

Schulze Schwering et al reported improvement of visual acuity with proper refraction in patients with oculocutaneous albinism in Malawi. They found the most improvement in patients with mild to moderate myopia, while the patients with greater than +1.5 D hyperopia did not improve much. [18]

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Surgical Care

Surgical management in patients with ocular involvement is limited to ocular muscle surgery.

Strabismus surgery for esotropia or exotropia can be considered for better ocular alignment.

Large 4 muscle horizontal rectus recessions have been reported to improve visual acuity in some patients with nystagmus.

If coexistent retinal disease is present and laser photocoagulation is required (eg, for patients with diabetic retinopathy or retinal tears), laser treatment may be ineffective. Laser photocoagulation requires pigment in the retinal pigment epithelial cells for adequate absorption of laser energy. [19] If the pigment is insufficient for adequate absorption of energy, cryopexy may be used as an alternative to treat peripheral retinal lesions. For posterior retinal pathology, the cryopexy probe is not accessible and no alternative treatment is available.

Sinha et al reported on the surgical challenges and outcomes of retinal detachment repair in 10 patients with albinism. Four of their patients underwent scleral buckling procedure, and six underwent pars plana vitrectomy with silicone oil. They found that patients in both groups did well, and only two patients in the vitrectomy group developed recurrent retinal detachment after three months. They also noted that identifying the retinal breaks, creating a posterior vitreous detachment, and applying endolaser were challenging in these patients. [20]

Farahi et al reported good outcomes of cataract surgery combined with aniridia ring in patients with oculocutaneous albinism. They found that these patients had not only improved visual acuity but also significant reduction in glare and photophobia after surgery. [21]

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Consultations

Depending on patient presentation and historical or physical findings, appropriate consultations from various subspecialties should be obtained, as follows:

  • Hematology

    • For patients with a history of easy bruising and bleeding diathesis (to rule out Hermansky-Pudlak syndrome, which is more common in Puerto Ricans)

    • For patients with a history of recurrent infections (to rule out Chediak-Higashi syndrome)

  • Genetics - For additional genetic analysis or for genetic counseling for patients and their families

  • Dermatology - For patients with any skin lesions or any signs suggestive of skin cancer, or for dermatologic counseling (to avoid excessive sun exposure)

  • Low-vision specialist - Patients may benefit from low-vision aids to help with their daily visual activities.

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Prevention

Since there is an increased risk of skin cancer in these patients, they should be advised to use skin tanning lotion and proper clothing for protection against exposure to sunlight.

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Long-Term Monitoring

Examine patients with oculocutaneous albinism and ocular albinism periodically to monitor their visual development and to assess the status of their refractive error and/or strabismus.

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