Ophthalmic Surgery, Lasers and Imaging Retina

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Case Report 

Severe Hypotony Associated With Parry–Romberg Syndrome

Susan Hung, BS; Tina Rutar, MD; Shan Lin, MD; Ira G. Wong, MD

Abstract

Parry–Romberg syndrome is a rare inflammatory disorder characterized by progressive hemifacial atrophy and ocular involvement. Two patients with Parry–Romberg syndrome who had mild heterochromic uveitis but developed profound ocular hypotony were evaluated. A 17-year-old girl and a 32-year-old woman with Parry–Romberg syndrome developed chronic uveitis with gradual worsening of intraocular pressure to 0 mm Hg. For the first time, ultrasound biomicrosopy found evidence of inflammation of the ipsilateral ciliary muscle in patients with hemifacial atrophy. The profound hypotony concomitant with ciliary body edema in two patients with Parry–Romberg syndrome provides a clue linking the systemic disease to the ocular findings.

Abstract

Parry–Romberg syndrome is a rare inflammatory disorder characterized by progressive hemifacial atrophy and ocular involvement. Two patients with Parry–Romberg syndrome who had mild heterochromic uveitis but developed profound ocular hypotony were evaluated. A 17-year-old girl and a 32-year-old woman with Parry–Romberg syndrome developed chronic uveitis with gradual worsening of intraocular pressure to 0 mm Hg. For the first time, ultrasound biomicrosopy found evidence of inflammation of the ipsilateral ciliary muscle in patients with hemifacial atrophy. The profound hypotony concomitant with ciliary body edema in two patients with Parry–Romberg syndrome provides a clue linking the systemic disease to the ocular findings.

Severe Hypotony Associated With Parry–Romberg Syndrome

From the University of California–San Francisco School of Medicine (SH); the Department of Ophthalmology (TR, SL), University of California–San Francisco; and the Francis I. Proctor Foundation for Research in Ophthalmology (IGW), University of California–San Francisco, San Francisco, California.

Supported by an unrestricted grant from Research to Prevent Blindness, New York, New York; That Man May See, Inc., San Francisco, California; and an institutional P30 core grant from the National Institutes of Health, NEI EY002162-31.

The authors have no financial or proprietary interest in the materials presented herein.

Address correspondence to Tina Rutar, MD, Department of Ophthalmology, Pediatric Ophthalmology and Strabismus, University of California–San Francisco, 10 Koret Way, K301, San Francisco, CA 94143.

Accepted: October 16, 2008
Posted Online: March 09, 2010

Introduction

Parry–Romberg syndrome is a rare inflammatory disorder of the skin, soft tissues, and muscles of half of the face that leads to hemifacial atrophy and enophthalmos.1,2 Uveitis is occasionally present.3 The pathogenic relationship between the uveitis and the facial atrophy is unknown.4 Although uveitis and hypotony often coexist, mild uveitis is rarely associated with severe and vision-threatening hypotony.5 We evaluated two patients with Parry–Romberg syndrome who had mild heterochromic uveitis but developed profound ocular hypotony. We found for the first time evidence on ultrasound biomicrosopy of inflammation of the ipsilateral ciliary muscle in patients with hemifacial atrophy.

Case Reports

Case 1

A 17-year-old girl was diagnosed as having Parry–Romberg syndrome based on atrophy involving the right side of her face, forehead, nose, and orbit (Fig. 1A) at the age of 3 years. At 6 years of age, she developed mild chronic unilateral non-granulomatous anterior heterochromic uveitis. At onset, visual acuity was 20/20 in each eye and her intraocular pressure was 9 mm Hg in the affected right eye and 18 mm Hg in the left eye.

(A) A 17-Year-Old Girl (case 1) and (B) a 32-Year-Old Woman (case 2) with Parry–Romberg Syndrome Involving the Right Side of the Face.

Figure 1. (A) A 17-Year-Old Girl (case 1) and (B) a 32-Year-Old Woman (case 2) with Parry–Romberg Syndrome Involving the Right Side of the Face.

Over the following 11 years, her visual acuity in the right eye gradually worsened to 20/100 as the intraocular pressure declined to 0 mm Hg and macular folds developed. Her uveitis was characterized by trace cell and flare, a few small keratic precipitates, and few peripheral anterior and posterior synechiae. Ultrasound biomicrosopy of the right eye detected ciliary body edema from the 6-o’clock to 10-o’clock positions (Fig. 2A).

(A) Ultrasound Biomicroscopy of Case 1 Showing Ciliary Body Edema at the 10-o’clock Position (arrow) and (B) Case 2 Showing Ciliary Body Edema at the 9-o’clock Position (arrow).

Figure 2. (A) Ultrasound Biomicroscopy of Case 1 Showing Ciliary Body Edema at the 10-o’clock Position (arrow) and (B) Case 2 Showing Ciliary Body Edema at the 9-o’clock Position (arrow).

Her chronic uveitis was managed with topical prednisolone acetate 1% and oral prednisone and methotrexate, which reduced the aqueous cells but did not reverse the hypotony. Topical ibopamine 2% therapy also failed to raise the intraocular pressure.

Case 2

A 32-year-old woman with a 20-year history of right hemifacial atrophy (Fig. 1B) developed unilateral anterior non-granulomatous uveitis at age 25 years. At onset, her visual acuity was 20/25 in each eye and her intraocular pressure was 9 mm Hg in the right eye and 17 mm Hg in the left eye. Over the next 7 years, she had bouts of recurrent mild anterior uveitis treated with topical prednisolone acetate 1% and cycloplegics. Her visual acuity in the right eye gradually worsened to 20/200 from macular folds and disc edema as the intraocular pressure declined to 0 mm Hg.

Unlike the patient in case 1, she responded to prednisolone acetate 1% and cycloplegia with resolution of the anterior uveitis and an increase in the intraocular pressure to 17 mm Hg. Her visual acuity improved to 20/70 as the macular folds and disc edema diminished. Post-treatment ultrasound biomicrosopy showed ciliary body edema at the 9-o’clock position in the right eye (Fig. 2B).

Discussion

Biopsies of areas of hemifacial atrophy in Parry–Romberg syndrome show chronic inflammation of the skin, subcutaneous tissue, muscles, and vessels.6 The swelling of the muscular portion of the ciliary body identified with ultrasound biomicrosopy may represent the same pathogenic process occurring in the facial muscles. Inflammation of the ciliary muscle may involve the overlying pars plicata, leading to uveitis and decreased aqueous production. Ultrasound biomicrosopy did not show any evidence of ciliary body detachment, membranes, or atrophy to account for the hypotony. Hypotony occurring in the setting of mild uveitis is unusual. The profound hypotony concomitant with ciliary body edema in two patients with Parry–Romberg syndrome provides a clue linking the systemic disease to the ocular findings.

References

  1. Cory RC, Clayman DA, Faillance WJ, McKee SW, Gama CH. Clinical and radiologic findings in progressive facial hemiatrophy. Am J Neuroradiol. 1997;18:751–757.
  2. Muchnick RS, Aston JS, Rees TD. Ocular manifestations and treatment of hemifacial atrophy. Am J Ophthalmol. 1979;88:889–897.
  3. Dawczynski J, Thorwarth M, Koenigsdoerffer E, Schultze-Mosgau S. Interdisciplinary treatment and ophthalmological findings in Parry-Romberg syndrome. J Craniofac Surg. 2006;17:1175–1176. doi:10.1097/01.scs.0000236440.20592.be [CrossRef]
  4. Miller MT, Spencer MA. Progressive hemifacial atrophy: a natural history study. Trans Am Ophthalmol Soc. 1995;93:203–215.
  5. Tran VT, Mermoud A, Herbort CP. Appraisal and management of ocular hypotony and glaucoma associated with uveitis. Int Ophthalmol Clin. 2000;40:175–203. doi:10.1097/00004397-200004000-00014 [CrossRef]
  6. Loewenfeld IE, Thompson HS. Fuchs’s heterochromic cyclitis: a critical review of the literature: I. Clinical characteristics of the syndrome. Surv Ophthalmol. 1973;17:394–457.
Authors

From the University of California–San Francisco School of Medicine (SH); the Department of Ophthalmology (TR, SL), University of California–San Francisco; and the Francis I. Proctor Foundation for Research in Ophthalmology (IGW), University of California–San Francisco, San Francisco, California.

Supported by an unrestricted grant from Research to Prevent Blindness, New York, New York; That Man May See, Inc., San Francisco, California; and an institutional P30 core grant from the National Institutes of Health, NEI EY002162-31.

The authors have no financial or proprietary interest in the materials presented herein.

Address correspondence to Tina Rutar, MD, Department of Ophthalmology, Pediatric Ophthalmology and Strabismus, University of California–San Francisco, 10 Koret Way, K301, San Francisco, CA 94143.

10.3928/15428877-20100216-06

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