Elsevier

Ophthalmology

Volume 111, Issue 3, March 2004, Pages 596-599
Ophthalmology

Case report
Head tilt–dependent esotropia associated with trisomy 21

Presented at: American Academy of Ophthalmology Annual Meeting, October, 2002; Orlando.
https://doi.org/10.1016/j.ophtha.2003.07.007Get rights and content

Abstract

Objective

To describe a series of patients who used a head tilt to control esotropia.

Design

Retrospective noncomparative case series.

Participants

Seven children with esotropia that decreased with their compensatory abnormal head tilt. Six of the patients had trisomy 21. The patients had no other identifiable etiology for their head tilt, including no oblique muscle dysfunction, nystagmus that changed with head tilt, or uncorrected refractive error.

Intervention

Six patients underwent horizontal extraocular muscle surgery. Preoperative evaluation in 4 patients included assessment of the change in head position with either monocular occlusion or prisms.

Main outcome measures

Ocular alignment in primary position and improvement in abnormal head tilt after surgery.

Results

In the 4 patients who underwent preoperative testing, the abnormal head tilt resolved with either monocular occlusion or prisms. The head tilt and esotropia were eliminated or improved in all patients who underwent strabismus surgery.

Conclusions

Abnormal head tilt may be used as a compensatory maneuver to improve purely horizontal strabismus. This finding appears to be associated with trisomy 21. Horizontal extraocular muscle surgery may improve the head tilt in such patients.

Section snippets

Materials and methods

Patients were identified from the clinical practices of the authors as having isolated cases that the authors had noted since 1985. The data were recovered from review of the patients' medical records. Patients were included if they demonstrated a head tilt with an esotropia that decreased in the AHP, and increased when the head was tilted in the opposite direction. Patients were excluded if they had any other identifiable cause of the head tilt, including vertical strabismus, oblique muscle

Patient 1

This male with trisomy 21 was initially evaluated at 15 months of age, at which time no strabismus was present. He returned at 28 months of age and was found to have a 40° right head tilt. He had a 12–prism diopter (PD) esotropia in the primary position and a 25-PD esotropia in a left head tilt, and was orthophoric in a right head tilt. He had a 15-PD A-pattern. He had neither nystagmus nor overaction of the oblique muscles.

At age 30 months he underwent bilateral medial rectus muscle recessions

Discussion

This report describes 7 children with head tilts that seemed to result solely from horizontal strabismus. All of the patients demonstrated an esotropia that decreased in the preferred head tilt, and that increased in the primary position and with the head tilted in the opposite direction. No other etiology for the AHP could be identified, including the absence of vertical strabismus, overaction of the oblique muscles, and nystagmus. All of the patients who underwent surgery had an improvement

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