Schwartz-Matsuo Syndrome

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Article initiated by:

Nhi Nguyen, MD

All contributors:

Mark L.Helm, MD, Leo A. Kim, MD, PhD, Nhi Nguyen, MD, Christina Y. Weng, MD, MBA, Jennifer I Lim MD, Ines Lains, MD, PhD, Isaac Bleicher, MD

Assigned editor:

Ines Lains, MD, PhD

Review:

Assigned status Up to Date

 by Ines Lains, MD, PhD on February 19, 2025.

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Contributing Editors:	add

Schwartz-Matsuo syndrome

Disease Entity

Disease

Schwartz-Matsuo syndrome is a form of open angle glaucoma associated with rhegmatogenous Retinal Detachment, which is secondary to photoreceptor outer segments and/or retinal pigment epithelium (RPE) pigment occluding the trabecular meshwork.

Pathophysiology and historic context

Schwartz-Matsuo syndrome was first described by Schwartz in 1973. Typically, a retinal detachment is associated with low intraocular pressure (IOP), as a result of increased outflow by active pumping of fluid through the exposed RPE . In patients with Schwartz-Matsuo syndrome, elevated IOP is observed. 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 RPE could migrate anteriorly within the aqueous humor and obstruct the trabecular meshwork . 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.

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 and suggested the photoreceptor outer segments as the underlying etiology. In Schwartz-Matsuo syndrome, the IOP typically returns to normal following the surgical repair of a retinal detachment.

Risk factors

• Shallow retinal detachment involving the vitreous base
  • a shallow detachment allows for constant sloughing of dying photoreceptor outer segments
  • involvement of the vitreous base allows for the photoreceptor outer segments to gain access to the anterior chamber (otherwise the hyaloid would not allow them to reach the anterior segment)
• Retinal dialysis or retinal tears at the ora serrata
• Ocular trauma
• Myopia

Diagnosis

Symptoms

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

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
• Gonioscopy will show an open angle and can rule out other etiologies of increased IOP: angle recession, angle closure, peripheral anterior synechiae, neovascularization, etc.
• Slit lamp examination with assessment of anterior chamber cell. Of note, it is thought that there is no true iridocyclitis on Schwartz-Matsuo syndrome and that the observed findings in the anterior chamber are photoreceptor outer segments and not anterior chamber cell.
• Dilated fundus examination (to look for retinal detachment)
  • The retinal detachment is often shallow and involves a wide area, including the macula

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
• High IOP with marked fluctuation
• Rhegmatogenous retinal detachment

Diagnostic procedures

The diagnosis of Schwartz-Matsuo syndrome is clinical. B-scan ultrasonography can assist in identification of tears or retinal detachment if the view is suboptimal. Optical coherence tomography can also help identify the presence of subretinal fluid. Gonioscopy is critical in evaluation the status of the angle.

Laboratory test

None

Differential diagnosis

1. Iritis. As mentioned, the anterior chamber findings in Schwartz-Matsuo syndrome are not inflammatory. The presence of anterior synechiae and keratic precipitates indicates an inflammatory condition which would rule out this condition. It should also be noted that the aqueous cells in Schwartz-Matsuo syndrome are unresponsive to corticosteroid treatment.
2. Open-angle or other secondary glaucomas 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

The definite treatment for this syndrome is retinal detachment repair.

Medical therapy

IOP-lowering topical therapies or oral carbonic anhydrase inhibitors (i.e., acetazolamide 500 mg p.o. bid) can be used to reduce the IOP while awaiting surgery. Although off-label, pilocarpine may help to open the trabecular meshwork pores and aid in lowering IOP. Corticosteroids do not improve the anterior chamber "cell" nor improve the intraocular pressure as the cell is not inflammatory.

Surgery

The definite treatment is retinal reattachment by standard methods, including scleral buckle or pars plana vitrectomy. The surgical repair typically normalizes the intraocular pressure. Incisional or minimally-invasive glaucoma surgery can be considered if the intraocular pressure remains uncontrolled after retinal reattachment.

Prognosis

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