Epiretinal Membrane Formation Is a Late
Manifestation of Shaken Baby Syndrome
Anna L. Ells, MD, FRCS(C), Amine Kherani, MD, FRCS(C), and David Lee, Ad Dip(MSc)
Shaken baby syndrome is a constellation of injuries
resulting from the intentional shaking type movement
of a child who is usually younger than 3 years old. This
rapid acceleration– deceleration movement of the
head is responsible for lesions attributed to shearing
forces placed on the vitreoretinal structures and men-
ingeal vessels across the dura.
1
The ophthalmic find-
ings include intraocular hemorrhages, perimacular
retinal folds, and peripheral retinoschisis in the pres-
ence of intracranial injuries such as subdural hemato-
mas without obvious external signs of head trauma.
2
We describe a case of late development of an epiretinal
membrane in a child with a history of shaken baby
syndrome and propose a differential diagnosis list for
epiretinal membrane formation in the pediatric age
group.
CASE REPORT
A 6-week-old infant, previously healthy, presented to the
emergency room with seizures and decreased level of con-
sciousness. Her injuries included multiple subdural hema-
tomas, bilateral occipital lobe contusions, and scattered
intraretinal hemorrhages in the posterior pole of the right
eye only. No perimacular retinal folds or retinoschisis
were identified. Forensic investigation confirmed child
abuse, and the perpertrators were prosecuted by the crown
prosecutor.
At 2 years of age, the patient was seen in the eye
clinic. She had severe developmental delay, cerebral
palsy, and central visual impairment. Flash visual evoked
potentials were absent to stimulation of both eyes. On
examination, she had an inconsistent fix-and-following
reflex with roving eye movements. There was no signif-
icant refractive error. The retina demonstrated slight
vitreous condensation over the area of the macula in the
right eye and bilateral optic nerve atrophy. The left
macula was unremarkable.
She was seen again at age 3 years, and her ophthalmo-
logical assessment was unchanged compared with the pre-
vious year’s assessment. At age 4 years, her visual status
was unchanged; however, retinal examination of her right
eye showed a large epiretinal membrane extending over
the entire macula (Figure 1). The epiretinal membrane
caused significant traction and dragging, thus disrupting
the macular architecture. Bilateral optic nerve atrophy was
unchanged (Figures 1 and 2).
A membrane peeling procedure would have improved
the retinal architecture, however, after careful review of
the patient’s clinical status, it was decided that vitrec-
tomy surgery would not improve her cortical visual
impairment, optic atrophy, and current level of func-
tioning. Therefore, intraocular surgery was not recom-
mended.
DISCUSSION
Shaken baby syndrome represents one specific form of
child abuse. In the United States, 1800 children are
diagnosed with shaken baby syndrome per year, a third
of whom die of their injuries.
3
The occult nature of
shaken baby syndrome means that patients with milder
involvement may escape early clinical detection and
present with a late manifestation of the syndrome, such as
an epiretinal membrane. Epiretinal membrane formation
as a late manifestation of shaken baby syndrome has been
reported in only 1 previous article.
4
Other ocular sequalae
From the Division of Opthalmology, Alberta Children’s Hospital, University of Calgary,
Calgary, Alberta, Canada.
Submitted December 16, 2002.
Revision accepted December 16, 2002.
Reprint requests: Anna Ells, MD, FRCS(C), Pediatric Ophthalmology, Alberta Children’s
Hospital, 1820 Richmond Rd, SW, Calgary, Alberta T2T 5C7, Canada.
Copyright © 2003 by the American Association for Pediatric Ophthalmology and
Strabismus.
1091-8531/2003/$35.00 + 0
doi:10.1016/S1091-8531(03)00005-3
FIG 1. Wide-angle image of the right eye with an epiretinal mem-
brane over the macula. Note retinal vascular tortuosity from the
puckering epiretinal membrane as well as optic nerve atrophy.
Journal of AAPOS June 2003 223