[Sensory and motor clinical presentation of congenital retraction syndromes: Stilling-Duane and Brown syndrome]

Abstract

Introduction: Congenital Brown syndrome and Stilling-Duane syndrome, two rare causes of strabismus are caused by fibrosis of one or more extraocular muscles. This series aims to report the clinical sensory and motor features of patients with Brown or Stilling-Duane syndrome.

Methods: Seventeen patients' records were retrospectively assessed for: the ocular deviation in primary position and in the 9 positions of gaze, head tilt, visual acuity and binocular vision.

Results: Eleven patients with Stilling-Duane syndrome had a mean age of 12 years, and monocular involvement, most frequently of type I. The ocular deviation was variable; 16 patients had an abnormal head position, while 50 % presented with amblyopia, and only 37 % achieved fusion in the compensatory head posture. Six patients presented with congenital Brown syndrome at mean age of 6 years. Three had a moderate form, 3 had a severe form with vertical deviation in primary position, compensatory head position, amblyopia and binocular vision impairment.

Discussion: The motility impairments depend highly on the identified syndrome, its classification and its severity. Therefore, these two retraction syndromes present some common features. Strabismus in primary position may lead to amblyopia and binocular vision impairment.

Conclusion: Clinical motility and sensory assessment is essential, though difficult, to establish the diagnosis and the management of patients with retraction syndromes.

Keywords: Brown syndrome; Clinical presentation; Présentation clinique; Stilling-Duane syndrome; Syndrome de Brown; Syndrome de Stilling-Duane.