Schwartz Matsuo Syndrome

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Original article contributed by: Nhi Nguyen, MD
All contributors: Mark L.Helm, MD and Nhi Nguyen, MD
Assigned editor:
Review: Not reviewed



Schwartz-Matsuo syndrome

Disease Entity

Disease

Schwartz-Matsuo syndrome is an elevated IOP and aqueous cell condition associated with rhegmatogenous retinal detachment, and was described by Schwartz in 1973.


Risk Factors

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

General Pathology

Pathophysiology

Typically, retinal detachment is associated with low IOP, as a result of increase outflow by active pumping of fluid through the exposed retinal pigment epithelium [1]. In Schwartz Matsuo syndrome, the IOP is elevated. 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. A suggestion made by Davidorf for a possible mechanism of this syndrome is that pigment granules released from the retinal pigment epithelium could migrate anteriorly within the aqueous humor to obstruct the trabecular meshwork [2][3]. 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 facts did not support Davidorf's suggestion that pigment in the subretinal space and vitreous flowed into the aqueous humor [2]. Recently, Matsuo and colleagues isolated photoreceptor outer segments and inflamatory cells in aqueuos 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, producing outflow obstruction. The presence of photoreceptor outer segments in the aqueous humor could explain clearly why the aqueous cells (namely photoreceptor outer segments) disappear, then the IOP returns to normal after surgical retinal detachment [2][3][4].


Diagnosis

History

  • Blunt ocular trauma and subsequent retinal detachment.
  • Postoperative
  • Atopic dermatitis [5][6][4].


Physical examination

  • Systemic examination (Marfan's syndrome or atopic dermatitis, may suggest 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.

Signs

Three major signs are:

  • Aqueous cells.
  • Elevated IOP with fluctuation.
  • Rhegmatogenous retinal detachment with tears around the ora serrata.[2][5][1][6]
  • The angle is often open. Angle recession may indicate prior blunt ocular trauma.

Symptoms

  • Symptoms of high IOP may include eye pain, blurred vision, headache, nausa, or vomiting.
  • Symptoms of retinal detachment: visual field loss, photopsia, floaters, decreased vistual acuity.

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 usually flat 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.


The high IOP tends to normalize and the aqueous cells disappear after the retinal detachment is repaired. [2][1]

Diagnostic procedures

Laboratory test

Electron microscopy may identify photoreceptor outer segments [2][5].

Differential diagnosis

  1. Iritis. The presence of anterior synechiae and keratic precipitates indicates an inflammatory condition and this is not consistent with Schwartz-Matsuo syndrome. On the other hand, the aqueous cells in Schwartz Matsuo syndrome are unresponsive to corticosteroid treatment.[2]
  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

An oral carbonic anhydrase inhibitor (acetazolamide 500 mg sequel p.o. bid) can be used to reduce the IOP while waiting for surgery.

Although unpproved, pilocarpine may help to open the trabecular meshwork pores. [7]


Surgery

Reattach the retina by standard methods including scleral buckle or vitrectomy. [2][7]


Prognosis

The high IOP tends to normalize and the aqueous cells disappear after a successful retinal reattachment. [2][1]


References

  1. 1.0 1.1 1.2 1.3 Cioffi G A, Durcan F J, Gupta N, Salmuelson T W, Tanna A P, Barton K, O'Connell S S (2014): Open-angle Glaucoma. In Glaucoma, Basic and clinical science course, Section 10, p 102 - 103.
  2. 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 2.8 Matsuo T (1994): Photoreceptor outer segments in aqueous humor: key to understanding a new syndrome. Surv of ophthalmol 39: 211 - 233.
  3. 3.0 3.1 Sofinski S J, Burke J F (1994): Glaucoma associated with disorders of the retina, vitreous, and choroid. In Principle and practice of ophthalmology, Vol 3, p 1561.
  4. 4.0 4.1 Rechardgreen W (2009): Photoreceptor outer segment glaucoma in rhegmatogenous retinal detachment. Arch ophthalmol 127 (8): 1053-1054.
  5. 5.0 5.1 5.2 Matsuo T, Muraoka N, Shigara F, Matsuo N (1998): Schwartz - Matsuo syndrome in retinal detachment with tears of the nonpigmented epithelium of the ciliary body. Acta Ophthalmol. Scand 76: 481 - 485.
  6. 6.0 6.1 Callender D, Jay J L (1997): Schwartz-Matsuo syndrome: atypical presentation as acute open angle glaucoma. Br J Ophthalmol 81: 609-610.
  7. 7.0 7.1 Heatley G, Michael Pro, Harasymowicz P (2006): Schwartz - Matsuo syndrome. J Glaucoma 15: 562 - 564.