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Ocular Findings in Children with Orbital Hypertelorism

Hwang, Jeong-Min M.D.; Baek, Rong-Min M.D.; Lee, Sang Woo M.D.

Plastic and Reconstructive Surgery: October 2012 - Volume 130 - Issue 4 - p 624e–627e
doi: 10.1097/PRS.0b013e318262f460

Department of Ophthalmology (Hwang)

Department of Surgery, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, Korea (Baek, Lee)

Correspondence to Dr. Baek, Department of Plastic Surgery, Seoul National University College of Medicine, Seoul National University Bundang Hospital, 166 Gumiro, Bundang-gu, Seongnam, Gyeonggi-do 463-707, Korea,

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Orbital hypertelorism is a congenital anomaly of the skull characterized by an increased distance between the medial orbital walls.1,2 Abnormal ocular findings include strabismus, optic atrophy, blepharophimosis, microphthalmos, and amblyopia.3 However, detailed description of strabismus or stereopsis in these patients has not been documented. This study was performed to analyze in detail the ocular findings in Asian patients with orbital hypertelorism.

Medical records of five consecutive Asian patients with orbital hypertelorism with an interocular distance greater than 30 mm measured with orbital computed tomography were reviewed retrospectively. All patients were boys with an average age of 5.8 years (range, 3 to 9 years) at the initial ophthalmologic examination (Table 1). The average interorbital distance was 37.8 mm (range, 32 to 48 mm). All patients had exotropia of 40 to 80 prism diopters. Patients 2 through 5 could not recognize any of the figures, animals, or dots of the Randot stereotest; patient 1 could not perform the Randot stereotest because he was too young to understand how to perform it.

Table 1

Table 1

All of the patients showed epiblepharon with multiple punctate corneal epithelial erosions. Four had “with-the-rule” astigmatism greater than 0.50 D, and three patients had astigmatism greater than or equal to 2.00 D. Patients 2 and 3 showed anisometropia greater than or equal to ±1.00 D and amblyopia (Table 1). None of them had brain abnormalities, developmental delay, or deafness.

Patient 1 was a 3-year-old boy who was born prematurely at week 37 of gestation with a birth weight of 2.52 kg. On ophthalmologic examination, he fixed and followed a 5-inch object at near with each eye. He had right exotropia 80 PD in the primary position at distance and near on the alternate prism cover test. Ductions and versions were full, with bilateral superior oblique overaction (Fig. 1). He did not recognize any of the figures, animals, or dots in the Randot stereotest. Both lower eyelids showed epiblepharon in the medial half of the lid margin (Fig. 2) accompanied by multiple punctate corneal erosions. Computed tomography of the orbit showed an increased distance of 35 mm between both medial orbital walls.

Fig. 1

Fig. 1

Fig. 2

Fig. 2

In the present study, all of the patients with orbital hypertelorism showed epiblepharon accompanied by multiple punctate corneal epithelial erosions; these observations have not been reported thus far. In addition, all five patients showed large-angle exotropia along with poor stereoacuity, and three patients showed overaction of the inferior oblique muscles without V pattern. Two patients showed amblyopia associated with strabismus and anisometropic astigmatism. These findings might be similar to the Asian patients with Down syndrome who showed high incidences of epiblepharon and exotropia that had not been reported.4,5

In patients with orbital hypertelorism, the lesser wings of the sphenoid bone forming the optic canal might be enlarged, leading to narrowing and malformation of the optic canals, which may further cause optic neuropathy. However, none of our patients showed dyschromatopsia, visual field defect, or optic disc pallor.

In conclusion, all patients with orbital hypertelorism showed unreported high incidences of epiblepharon accompanied by astigmatism and large-angle exotropia accompanied by poor stereopsis. These abnormal ocular findings should be identified and treated suitably by both plastic surgeons and ophthalmologists.

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This work was supported by the Seoul R&BD Program (ST090841) and by a grant from the Korea Health 21 R&D Project, Ministry of Health, Welfare, and Family Affairs, Republic of Korea (A080299).

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The authors have no financial interest to declare in relation to the content of this communication. No outside funding was received.

Jeong-Min Hwang, M.D.

Department of Ophthalmology

Rong-Min Baek, M.D.

Sang Woo Lee, M.D.

Department of Surgery

Seoul National University College of Medicine

Seoul National University Bundang Hospital

Seongnam, Korea

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1. Tessier P. Experiences in the treatment of orbital hypertelorism. Plast Reconstr Surg.. 1974;53:1–18
2. Ortiz-Monasterio F, Molina F. Orbital hypertelorism. Clin Plast Surg.. 1994;21:599–612
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5. Kim U, Hwang JM. Refractive errors and strabismus in Asian patients with Down syndrome. Eye (Lond.). 2009;23:1560–1564
©2012American Society of Plastic Surgeons