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Clinicopathologic Reports, Case Reports, and Small Case Series
August 2001

Contractile Peripapillary Staphyloma With Light Stimulus to the Contralateral Eye

Arch Ophthalmol. 2001;119(8):1216-1217. doi:

A peripapillary staphyloma is a sporadic, unilateral, congenital defect characterized by an excavation surrounding a usually normal optic disc and often accompanied by decreased vision1,2 or by enlargement of the blind spot.3 A less common occurrence is when the wall of the peripapillary staphyloma is contractile, which is caused by 1 of 2 possible mechanisms, "pressure balance" or "muscular contraction."2 This article provides the first documentation of a contractile peripapillary staphyloma through sequential pictures and discusses the main differential diagnosis and the possible pathophysiological mechanism.

Report of a Case

A 23-year-old white woman was referred for evaluation after undergoing a vitrectomy on the left eye for removal of a peripapillary cisticercus, which disappeared after retrobulbar anesthesia. The best-corrected visual acuity was 20/25 OD and 20/30 OS. The pupillary reflexes were normal. The left eye had a peripapillary staphyloma showing circular-type contractile movements characterized by a short time delay, following the light stimulus to the contralateral eye. The correlations were negative with the Valsalva maneuver, neck venous compression, forced lid closure, increase of ocular pressure with contact lens, respiratory movements, accommodation, and illumination of the affected eye.

The visual field showed enlargement of the blind spot. Fluorescein angiography revealed a window defect in the peripapillary region. Optical coherence tomography, B-scan ultrasonography, computed tomography, and nuclear magnetic resonance in combination revealed a parietal ectasia. The orbital Doppler was normal. No retrobulbar tumors, inflammation, abnormal vessels, or other congenital anomalies were identified. Changes in size and shape affecting the disc and the peripapillary zone were documented by serial photographs and video documentation obtained from biomicroscopy and scanning laser ophthalmoscopy (Figure 1 and Figure 2).

Figure 1.
Serial fundus photographs of the
peripapillary staphyloma, showing its contractile movements and shape and
size modifications in response to light stimulation to the contralateral eye.
A, Normal appearance of the ectasia with an indefinite nasal margin of the
disc. B, Initial contraction of the anomaly, allowing a partial identification
of the nasal margin of the disc. C, Progressive contraction revealing a normal
shape of the disc. D, Final appearance of the region after a circular contraction
pattern.

Serial fundus photographs of the peripapillary staphyloma, showing its contractile movements and shape and size modifications in response to light stimulation to the contralateral eye. A, Normal appearance of the ectasia with an indefinite nasal margin of the disc. B, Initial contraction of the anomaly, allowing a partial identification of the nasal margin of the disc. C, Progressive contraction revealing a normal shape of the disc. D, Final appearance of the region after a circular contraction pattern.

Figure 2.
Serial ultrasonographic pictures,
revealing changes of ectasia deepness at the nasal aspect of the peripapillary
staphyloma. A, Initial appearance after the light stimulus in the contralateral
eye. B-D, Progressive contraction following the provocative test.

Serial ultrasonographic pictures, revealing changes of ectasia deepness at the nasal aspect of the peripapillary staphyloma. A, Initial appearance after the light stimulus in the contralateral eye. B-D, Progressive contraction following the provocative test.

Comment

The condition described herein must be distinguished from coloboma of the optic disc, in which the defect is within the nerve head, or myopic conus, in which the defect is usually secondary to an abnormal disc.2 In this case report, an error during embryological development seems likely. An area that should have become sclera may have become a circular muscle4 instead, using concentrically oriented smooth strands and forming an incomplete ring around the nerve.2,5

We favor a neuromuscular contraction mechanism as the basis for the observed phenomenon.4 A circular, heterotopic smooth muscle situated at the posterior pole of the eye, associated with an autonomic cholinergic reflex, and innervated by a ciliary nerve is, in our estimation, the most likely cause for these intraocular motions.2 The contraction was noticeably changed by retrobulbar anesthesia. The relevant mechanism may be rudimentary and may explain the nonsynchronous response with a latency period after the pupillary reflex and the negative correlation to direct illumination.

We thank Tercio Guia for the serial pictures and video documentation.

Corresponding author: Michel E. Farah, MD, Avenida Ibijaú, 331, 4° andar, CEP 04524-020, São Paulo–SP, Brazil (e-mail: michelfarah@uol.com.br).

References
1.
Caldwell  JSears  MGilman  M Bilateral peripapillary staphyloma with normal vision. Am J Ophthalmol. 1971;71423- 425
2.
Kral  KSvarc  D Contractile peripapillary staphyloma. Am J Ophthalmol. 1971;711090- 1092
3.
Konstas  PKatikos  GVatakas  L Contractile peripapillary staphyloma. Ophthalmologica. 1976;172379- 381Article
4.
Willis  RZimmerman  LO'Grady  R  et al.  Heterotopic adipose tissue and smooth muscle in the optic disc: associations with isolated colobomas. Arch Ophthalmol. 1972;88139- 146Article
5.
Wise  JMaclean  AGass  D Contractile peripapillary staphyloma. Arch Ophthalmol. 1966;75626- 630Article
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