41

QUESTION

WHAT SYSTEMIC CONDITIONS ARE ASSOCIATED WITH AN INCREASED RISK OF RETINAL DETACHMENT? WHAT SHOULD I DO ABOUT IT?

Joseph N. Martel, MD
Mallika Doss, MD

The risk of rhegmatogenous retinal detachment (RRD) is increased in systemic diseases (Table 41-1) that fundamentally result in abnormal vitreous, atypical vitreoretinal traction, predisposing vitreoretinal lesions, or other anatomical ocular abnormalities. While there are systemic disorders associated with exudative retinal detachment (eg, eclampsia, Vogt-Koyanagi-Harada syndrome, metastasis, vascular malformations), the focus of this chapter will be on systemic conditions that increase the risk of RRD.

Inherent Vitreoretinal Abnormalities

Stickler syndrome (hereditary progressive arthro-ophthalmopathy) is perhaps the most important hereditary systemic disorder associated with RRD. This condition portends about a 50% lifetime risk of RRD. It is a connective tissue disorder caused by defect in Type II procollagen¹ and characterized as a hyaloideoretinopathy, whose hallmark is vitreous liquefaction (optically empty vitreous) and adherent cortical vitreous remnants behind the lens.² In addition to an anomalous vitreous, patients with Stickler syndrome often have ocular anatomical predispositions to retinal detachment, such as high axial myopia and perivascular radial lattice degeneration with strong vitreous adhesions along the borders of these lesions.³ Retinal breaks are often numerous with variable distances from the vitreous base region.⁴ Systemic findings in Stickler syndrome may include hearing impairment, arthritis, skeletal abnormalities, cleft palate, bifid uvula, and flattening of the mid-face.4 Additionally, about 12% of patients with Pierre Robin sequence (cleft palate, micrognathia, and glossoptosis) suffer from Stickler syndrome.

Table 41-1

Systemic Conditions Associated With Rhegmatogenous Retinal Detachment

Inherent Vitreoretinal Abnormalities	Stickler syndrome Marfan syndrome Ehlers-Danlos syndrome Homocystinuria
Acquired Vitreoretinal Abnormalities	Proliferative Retinopathies Diabetes mellitus Sickling hemoglobinopathies Hypertension Infectious Uveitis Necrotizing herpetic retinitis Traumatic/Self-induced Atopic dermatitis4,5 Cognitive disability

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