GLAUCOMA IN THE MARFAN SYNDROME*

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Abraham Lincoln is the prototype of Marfan's whenever we think ofthis syndrome. He had the unusual height, the elongated fingers, toes, and limbs, but also had ...
GLAUCOMA IN THE MARFAN SYNDROME* BY Natalio J Izquierdo, MD (BY INVITATION),

Elias I. Traboulsi, MD (BY INVITATION), Cheryl Enger, MS (BY INVITATION) AND Irene H. Maumenee, MD INTRODUCTION

PREVIOUS STUDIES

OF PATIENTS WITH THE

MARFAN SYNDROME SUG-

gested that glaucoma may be a common ocular finding. Glaucoma has been reported in patients with ectopic lenses as part of the syndrome. 1,2 Maumenee3 reported five patients with the Marfan syndrome out of 160 who lost significant visual acuity due to glaucoma. Cross and Jensen4 diagnosed glaucoma in 7.7% of eyes with ectopia lentis and in 14.8% of aphakic eyes in patients with the Marfan syndrome. Wakita and coworkers5 found glaucoma in 22 eyes of 104 patients with the Marfan syndrome. We report on the prevalence of glaucoma in 573 patients with the Marfan syndrome. MATERIALS AND METHODS

Over the last 20 years, 1069 patients were evaluated at the Johns Hopkins Center for Hereditary Eye Diseases. These patients had an ocular examination as part of their systemic evaluation for the Marfan syndrome. A definite diagnosis was established in 573 patients on the basis of their skeletal, cardiovascular, and ocular findings. Twenty-nine patients were diagnosed with glaucoma because of increased intraocular pressure (IOP) (> 22 mm Hg), cupping of the optic nerve head, and visual field defects. Fourteen patients were suspected of having glaucoma following initial evaluation.

*From the Johns Hopkins Center for Hereditary Eye Diseases, The Johns Hopkins Medical Institutions, Baltimore. Supported in part by the Medical Sciences Campus of the University of Puerto Rico and the Laura Kriebel and the Walter Edel Funds of the Johns Hopkins Center for Hereditary Eye Diseases. TR. AM. OPHTH. Soc. vol. LXXXX, 1992

Maumenee et al

112

RESULTS

PAIENT CHARACTERISTICS

Both sexes were equally affected. Thirty percent of patients were white, and 2% were black. Race was not recorded in 68% of the patients. Age distribution is depicted in Fig 1. The mean age was 29.6 years (range, 1 to 79 years). MECHANISMS LEADING TO GLAUCOMA

Open-angle glaucoma (OAG) was diagnosed in 13 patients, angle-closure glaucoma and phacolytic glaucoma in 2, and neovascular glaucoma in 1 (Table I). Glaucoma following ocular surgery was found in a total of 11 patients (in 6 patients following lens surgery and in 5 following scleral buckling procedure). TABLE I: ETIOLOGY OF GLAUCOMA IN 29 PATIENTS WITH MARFAN'S SYNDROME

Primary open-angle Acute angle-closure

Phacolytic glaucoma Neovascular glaucoma Post-lens extraction Post-scleral buckling procedure

13 2 2 1 6 5

PREVALENCE OF GIAUCOMA

A total of 422 patients were younger than 40 years (group 1) and 147 patients were 40 years of age or older (group 2) (Fig 2); age was not recorded in four patients (< 1%). Glaucoma (all types included) was present in 10 patients in group 1, with a prevalence of 23.7/1000. OAG was present in seven patients in group 1, with a prevalence of 16.6/1000. Glaucoma (all types included) was present in 19 patients in group 2, with a prevalence of 129.3/1000. OAG was present in six patients in group 2, with a prevalence of 40.8/1000. DISCUSSION

Glaucoma in the Marfan syndrome can be due to a variety of mechanisms. Pupillary block can be produced by an ectopic lens, the vitreous, or both. In the present series two patients had pupillary block with complete

Glaucoma in Marfan's 30

113

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25 20 c

0 h-

15 -

0D

a.

10 50- _

0-9

10-19 20-29 30-39 40-49 50-59 60-69 70-79

Age Group FIGURE 1

Age distribution of patients with Marfan syndrome.

200 180o 0 °

160-

_~ Any Type of Glaucoma Open Angle Glaucoma

140-

*D a. 1200 c

*

10080-

>

60-

IL

40-

20 0-

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