Posters

Marfan syndrome pupillary blockage approach

Poster Details

First Author: F. March de Ribot SPAIN

Co Author(s):    P. López Lizárraga   A. March de Ribot                 

Abstract Details

Purpose:

Ectopia lentis (EL) is a major criteria for the diagnosis of Marfan syndrome. It may vary from an asymptomatic mild displacement of the lens to a significant subluxation that places its equator in the pupillary axis or cause a pupillary block glaucoma or even a lens luxation to the anterior chamber or posterior pole. The purpose of this work is to present a patient with Marfan syndrome with lens luxation to the anterior chamber and the specific approach in this case.

Setting:

Universitary Hospital, Ophthalmology Service

Methods:

A 43-year-old male arrived to the emergency department presenting severe pain and loss of visual acuity in the right eye. He had no known previous medical history. The visual acuity was of light perception in the right eye and the intraocular pressure was of 53 mmHg. The right eye presented red eye with ciliary injection, corneal edema, and pupillary block. The lens was luxated to the anterior chamber in contact with the inferior part of the cornea. The approach of the case was a Marfan syndrome with an acute glaucoma secondary to a lens luxation into the anterior chamber.

Results:

The patient was external and had special requirements for the medical attention. In this case was considered to dilate the pupil with cyclopelgics and put the patient in supine position to displace the lens form the anterior chamber to the posterior pole. Half an hour after this maneuver , the pain disappeared and the intraocular pressure dropped to normal values. It was prescribed further ophthalmological attention and systemic evaluation including a cardiovascular study.

Conclusions:

Patients with Marfan syndrome have several ocular manifestations and management is complex. Anterior segment surgery to extract the luxated lens is advisable. Nevertheless in this case it was considered a crystalline lens luxation to the posterior pole in order to control a glaucomatous crisis and prepare a posterior segment surgery in a second time. Surgical risks have to be considered individually. In this case, this option was considered for the symptomatology, time of presentation and difficulty to get a surgical room for the patient among others.

Financial Disclosure:

NONE