Schwartz-Matsuo Syndrome

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Article initiated by:

Nhi Nguyen, MD

All contributors:

Mark L.Helm, MD, Nhi Nguyen, MD, Christina Y. Weng, MD, MBA, Jennifer I Lim MD

Assigned editor:

Review:

Assigned status Update Pending

 by Christina Y. Weng, MD, MBA on June 16, 2024.

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Schwartz-Matsuo syndrome

Disease Entity

Disease

Schwartz-Matsuo syndrome is a condition characterized by elevated intraocular pressure (IOP) and aqueous cells and is associated with rhegmatogenous retinal detachment. The disorder was first described by Schwartz in 1973.

Risk Factors

• Retinal dialysis at the ora serrata, retinal tears, or rhegmatogenous retinal detachment.

Pathophysiology

Typically, retinal detachment is associated with low IOP, as a result of increased outflow by active pumping of fluid through the exposed retinal pigment epithelium . In Schwartz-Matsuo syndrome (also referred to as "Schwartz's syndrome") the opposite is observed with elevated IOP. Several theories have been postulated to explain this condition. Schwartz hypothesized that iridocyclitis could cause a reduction in outflow facility, which could contribute to an elevated intraocular pressure in the presence of normal or reduced aqueous production. Davidorf suggested another possible mechanism where pigment granules released from the retinal pigment epithelium could migrate anteriorly within the aqueous humor and obstruct the trabecular meshwork . Many separate reports on patients with retinal detachment appeared in the Japanese literature and the authors recognized that the angle in the eyes of those patients had: 1) the same level of pigmentation as in the fellow eye, 2) the cornea had no pigment deposits, and 3) the anterior chamber showed no floating pigment. These findings were inconsistent with Davidorf's suggestion that pigment in the subretinal space and vitreous flowed into the aqueous humor . Recently, Matsuo and colleagues isolated photoreceptor outer segments and inflammatory cells in aqueous humor aspirates of patients who met the criteria for Schwartz's syndrome. He has hypothesized that photoreceptor outer segments pass through the retinal break and gain access to aqueous outflow pathways, leading to outflow obstruction. In Schwartz-Matsuo syndrome, the IOP typically returns to normal following the surgical repair of a retinal detachment. .

Diagnosis

Possible associated history

• Blunt ocular trauma and subsequent retinal detachment
• Postoperative status
• Atopic dermatitis

Physical examination

• Systemic examination (Marfan's syndrome or atopic dermatitis may help identify patients who are at risk of a retinal break around the ora serrata).
• Visual acuity, intraocular pressure measurement
• Slit lamp examination (with dilated pupil)
• Gonioscopy to rule out other etiologies of increased IOP: angle recession, angle closure, peripheral anterior synechiae, neovascularization, etc.
• Dilated fundus examination ( to look for retinal detachment)

Three major signs are:

• Aqueous cells
• Elevated IOP with fluctuation
• Rhegmatogenous retinal detachment with tears around the ora serrata
• The angle is often open in this condition. Angle recession may indicate prior blunt ocular trauma

Symptoms

• Symptoms of high IOP may include eye pain, blurred vision, headache, nausea, or vomiting
• Symptoms of retinal detachment may include visual field loss, photopsia, floaters, decreased visiual acuity, scotoma

Clinical diagnosis

Schwartz-Matsuo syndrome is diagnosed by the combination of three clinical signs:

• Aqueous cells in varying number, but no other signs of uveitis
• The retinal detachment is often shallow and involves a wide area, including the macula. Tears are located mostly at the ora serrata or in the nonpigmented epithelium of the pars plana or pars plicata.
• High IOP with marked fluctuation

Diagnostic procedures

Optical coherence tomography can help identify the presence of subretinal fluid. B-scan ultrasonography can assist in identification of tears or retinal detachment if the view is suboptimal. Gonioscopy can assist in evaluation the status of the angle.

Laboratory test

Electron microscopy may identify photoreceptor outer segments .

Differential diagnosis

1. Iritis. The presence of anterior synechiae and keratic precipitates indicates an inflammatory condition which is not consistent with Schwartz-Matsuo syndrome. It should also be noted that the aqueous cells in Schwartz-Matsuo syndrome are unresponsive to corticosteroid treatment.
2. Open-angle glaucoma should be considered, especially if it occurs in a patient who has a history of blunt trauma.
3. Posner-Schlossman syndrome shows very mild anterior chamber inflammation with few cells and little flare, few fine keratic precipitates, and responds to steroid treatment.

Management

Medical therapy

IOP-lowering topical drops or oral carbonic anhydrase inhibitor (acetazolamide 500 mg sequel p.o. bid) can be used to reduce the IOP while awaiting surgery.

Although unapproved, pilocarpine may help to open the trabecular meshwork pores and aid in lowering IOP.

Surgery

Reattach the retina by standard methods including scleral buckle or pars plana vitrectomy.

Prognosis

The elevated intraocular pressure tends to normalize and the aqueous cells disappear after a successful retinal reattachment.