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Case Reports
. 2020 Jan;64(1):86-92.
doi: 10.1007/s10384-019-00707-1. Epub 2019 Dec 17.

Surgical management of pediatric patients with congenital fibrosis of the extraocular muscles

Yoichi Okita  1 , Akiko Kimura  2 , Mana Okamoto  2 , Osamu Mimura  2 , Fumi Gomi  2
Affiliations
Case Reports

Surgical management of pediatric patients with congenital fibrosis of the extraocular muscles

Yoichi Okita et al. Jpn J Ophthalmol. 2020 Jan.

Abstract

Purpose: Congenital fibrosis of the extraocular muscles (CFEOM) is a rare nonprogressive disorder characterized by bilateral ptosis, with severely limited ocular motility. We report the treatment outcomes and problems in 3 cases of pediatric CFEOM in which extraocular muscle surgery was performed.

Cases: All the cases showed bilateral ptosis and a chin-up abnormal head posture (AHP). Case 1 A 6-year-old girl. Both eyes were fixed downward with esotropia and could not elevate above the horizontal midline. She underwent simultaneous bilateral inferior rectus (IR) and medial rectus (MR) recession. Postoperatively, 8-prism-diopter (PD) exotropia was observed, and the AHP were improved, but MR advancement in the right eye was necessary because A-pattern exotropia became prominent starting about 10 months postoperatively. Case 2 A 7-year-old girl. Both eyes were fixed downward and did not elevate over the midline. She underwent bilateral IR recession. Postoperatively, 8-PD exotropia was observed; however, A-pattern exotropia became prominent gradually at about 1 year and 7 months postoperatively, and bilateral lateral rectus (LR) recession was added. Case 3 A 6-year-old girl. Both eyes were fixed downward but could be elevated above the horizontal midline by upward effort. She underwent bilateral IR recession, which resulted in improvement of the AHP and ptosis. About 8 months postoperatively, exotropia was evident only in the downward gaze.

Conclusions: Bilateral IR recession in pediatric patients with CFEOM was effective in improving AHP, but postoperative exotropia appeared to be inevitable owing to the diminished adducted function caused by IR recession. Thus, horizontal strabismus surgery should be planned after the results of IR recession become evident.

Keywords: Abnormal head posture; Congenital fibrosis of the extraocular muscles in childhood; Limitation of ocular motility; Ptosis; Strabismus surgery.

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Supplementary concepts