Customize your JAMA Network experience by selecting one or more topics from the list below.
Copyright 2001 American Medical Association. All Rights Reserved.
Applicable FARS/DFARS Restrictions Apply to Government Use.2001
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.
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
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).
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
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.
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
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: email@example.com).
Farah ME, Uno F, Bonomo PP, Nóbrega M, Höfling-Lima AL. Contractile Peripapillary Staphyloma With Light Stimulus to the Contralateral Eye. Arch Ophthalmol. 2001;119(8):1216–1217. doi: