LETTER TO THE EDITOR
Contractile peripapillary staphyloma mimicking
morning-glory disc anomaly
Dear Editor,
Although peripapillary staphyloma and morning glory disc anomaly are rarely encountered, accurate differential diagnosis based on disc appearance is essential because of their similar presentations [1]. However, few cases of contractile peripapillary staphyloma and contractile morning glory disc anomaly have been reported. This study describes a rare case of contractile peripapillary staph-yloma that phasically mimicked a morning glory disc anomaly.
A 2-year-old Taiwanese boy presented with a crossed right eye since birth. His medical history was unremarkable. His ocular anterior segment was normal. The patient showed esotropia (ET) of 30D and a relative afferent pupillary defect in the right eye. Ocular motility was normal. Cycloplegic refraction was þ0.75 þ 0.75 30 in the right eye (RE) andþ1.50 in the left eye (LE).
Ophthalmoscopic examination revealed a RE contractile peripapillary staphyloma (Fig. 1). Examination during con-tracted stage showed retinal vessels originating from the periphery of an excavated disc and coursing radially toward
Figure 1. (A) Ophthalmoscopic images of contracted stage showing disc appearance resembling that of morning glory disc anomaly; (B, C, and D) image series of disc in expanded stage showing funnel-shaped peripapillary staphyloma.
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the peripheral retina. A ring of hyperpigmentation sur-rounded the optic disc. A deep, funnel-shaped peripapillary staphyloma was noted in the expanded stage. The disc was at the bottom of the staphyloma, and the central trunk of the retinal vessels was visible. Brain magnetic resonance imaging (MRI) results were normal.
The patient was unavailable for examination for 5 years. At age 8 years, general examinations of the patient revealed normal development. Vision was 0.02 RE and 1.2 LE. During the 5-year interval, the patient had not consis-tently complied with the recommendation to occlude the left eye by wearing an eye patch. Cycloplegic refraction was -4.25þ 2.50 90 RE andþ1.00 LE. The ET was 20D,
and no stereopsis was detected. Ophthalmoscopic exami-nation revealed a mild dysplastic disc with posterior staphyloma but no pattern of contractility.
The embryogenesis of morning glory disc anomaly and peripapillary staphyloma is unknown. A case series study of patients with frontonasal dysplasia and basal encephaocele reported by Hodgkins et al revealed that all patients had either peripapillary staphyloma or morning glory optic disc abnormalities [2]. The authors suggested that these disc abnormalities have similar embryological origins. We speculate the morning glory disc anomaly observed in the current case resulted from a peripapillary staphyloma that did not adequately expand during embryogenesis.
Contractile cycles are reportedly related to respiration [3], light stimulation in the opposite eye[4], and transient visual loss [5]. Since morning glory disc anomaly is also associated with endocrine and central nervous system (CNS) anomalies, CNS imaging is essential[1]. The normal MRI results and normal development observed in the current patient suggested that the disease did not substantially affect other systems. Disc contractility also decreased as the patient grew older.
In congenital optic nerve diseases, visual acuity can range from 1.0 to complete lack of light perception. Although the current patient was treated with occlusion therapy in accordance with the literature, his final visual acuity was only 0.02. Together, the severely impaired vision, esotropia, and abnormal disc appearance suggested an underlying microscopic anomaly of retina/optic disc.
Acknowledgment
The authors thank Dr. William F. Hoyt for his valuable suggestions regarding data presentation. This work was
supported by grant No: DOH100-TD-C-111-002 from the Department of Health, Executive Yuan, Taiwan.
References
[1] Pollock S. The morning glory disc anomaly: contractile move-ment, classification, and embryogenesis. Doc Ophthalmol 1987; 65:439e60.
[2] Hodgkins P, Lees M, Lawson J, Reardon W, Leitch J, Thorogood P, et al. Optic disc anomalies and frontonasal dysplasia. Br J Ophthalmol 1998;82:290e3.
[3] Sugar HS, Beckman H. Peripapillary staphyloma with respira-tory pulsation. Am J Ophthalmol 1969;68:895e7.
[4] Farah ME, Uno F, Bonomo PP, Nobrega M, Hofling-Lima AL. Contractile peripapillary staphyloma with light stimulus to the contralateral eye. Arch Ophthalmol 2001;119:1216e7. [5] Brodsky MC. Contractile morning glory disc causing transient
monocular blindness in a child. Arch Ophthalmol 2006;124: 1199e201.
Yu-Hung Lai Department of Ophthalmology, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan Department of Ophthalmology, School of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan Graduate Institute of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan Go¨lge Acaroglu Department of Neuro-Ophthalmology and Orbital disease, Ulucanlar Eye Hospital, Ankara, Turkey Hwei-Zu Wang Department of Ophthalmology, School of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan Hsin-Tien Hsu* School of Nursing, College of Nursing, Kaohsiung Medical University, Kaohsiung, Taiwan *Corresponding author. 100 Zih-You 1stRoad, Kaohsiung 807, Taiwan E-mail address:[email protected]
