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Ophthalmologic Signs of Shunt Failure in Children with Hydrocephalus
Journal of AAPOS Volume 10 Number 1 February 2006 Ophthalmologic Signs of Shunt Failure in Children with Hydrocephalus Paul H. Phillips MD, Barbara Ku MD, Nazer Qureshi MD, Bahid Adada MD; University of Arkansas for Medical Sciences, Little Rock, AR Purpose: To determine the prevalence of ophthalmologic findings in hydrocephalic children with shunt malfunction. Methods: Retrospective analysis of all children diagnosed with shunt malfunction at Arkansas Children’s Hospital between March 2005 and September 2005. Each patient had a complete ophthalmologic examination and shunt malfunction confirmed by neurosurgery. Children were excluded from the analysis if they had a central nervous system tumor or an unrelated eye disease. Results: Eighteen patients were diagnosed with shunt failure. The underlying etiology of the hydrocephalus included aqueductal stenosis (six patients), intraventricular hemorrhage (four patients), spina bifida (four patients), Dandy– Walker syndrome (three patients), and intracranial hemorrhage from trauma (one patient). Shunt failure was attributed to mechanical blockage in 14 patients and infection in 4 patients. Two patients presented with ophthalmologic signs as the only indication of shunt failure including one patient with papilledema and one patient with dorsal midbrain syndrome. These signs resolved after surgical shunt repair. Two other patients had optic nerve atrophy of unknown duration. The remaining 14 patients had a normal ophthalmologic examination. Conclusions: In some children, ophthalmologic signs are the only indication of shunt failure and facilitate the prompt diagnosis of shunt malfunction. However, most children with shunt malfunction have a normal ophthalmologic examination. Therefore, a normal eye examination does not preclude the presence of shunt failure. Ophthalmic Manifestations of Danon Disease F. Ryan Prall MD, Arlene Drack MD, Matthew Taylor MD, Lisa Ku MA, Jeff Olson MD, Naresh Mandava MD; University of Colorado, Denver, CO Purpose: To describe the ophthalmic findings in patients with Danon disease, an X-linked cardiomyopathy. Methods: Retrospective study from a large pedigree with genetically proven Danon disease. Complete eye examination, ERG, color vision, visual fields, and fluorescein angiography were performed. Results: Five females and two males were examined. The four affected females demonstrated peripheral pigmentary retinopathy and myopia. Other tests were unremarkable. The male patients demonstrated a near-complete loss of pigment in the retinal pigment epithelium. Conclusion: We report the first description of a characteristic retinopathy in patients with Danon disease and the first extracardiac manifestations in females. Retinopathy potentially could be used to identify asymptomatic carriers.
Abstracts
91
Doctor, Why Are You Operating on my Good Eye? Alan B. Richards MD; LSU Medical Center, Shreveport, LA Introduction: This paper describes a series of five patients with reduced vision in one eye preoperatively who had bilateral strabismus surgery and developed a visually threatening or actually visually destructive complication in the better seeing eye related to the strabismus surgery. Two complications were from retinal detachments, one from optic atrophy from a retro bulbar hemorrhage, and one from crystalline keratopathy from postoperative use of Ciloxan in the better seeing eye. Methods: A questionnaire was sent by mail to all members of AAPOS and the literature was surveyed. Results: Five patients were identified who had visually threatening or actual vision loss to the better seeing eye following bilateral strabismus surgery. Three of the complications did not involve the use of the scleral bite to reinsert the muscle, illustrating the diverse types of complications that can occur with strabismus surgery. The worst complication in a patient with retrobulbar hemorrhage (resulting in NLP vision) occurred with a superior oblique tenectomy with no suture in the tendon at all. Discussion: The decision of whether to perform strabismus surgery on the better seeing eye occurs frequently in a wide variety of problems, including torticollis, pattern strabismus, and restrictive strabismus. Conclusions: Textbooks and the ophthalmic literature as well as resident education do not directly address the question of when to perform bilateral strabismus surgery in patients with reduced vision in one eye. More concise recommendations from AAPOS would be helpful in guiding pediatric ophthalmologists and in defense of litigation. Duration of Binocular Decorrelation Predicts the Intensity of Fusion Maldevelopment (Latent) Nystagmus in Strabismic Macaque Monkeys Michael Richards, Agnes Wong MD, Paul Foeller MSc, Dolores Bradley PhD, Lawrence Tychsen MD; Washington University School of Medicine, St. Louis, MO Purpose: Infantile esotropia is linked strongly to latent nystagmus (LN) in human infants, but many features of this comorbidity are unknown. The purpose of this study was to determine how the duration of early-onset strabismus affects the prevalence and magnitude of LN. Methods: Optical strabismus was created in nine infant macaques by fitting them with prism goggles on DOL 1. The goggles were removed after 3 weeks (n ⫽ 2), 3 months (n ⫽ 2), or 6 months (n ⫽ 3), emulating surgical repair of strabismus in humans at 3, 12, and 24 months of age, respectively. Two control monkeys wore plano lenses. Several months after the goggles were removed, eye movements during fixation tasks were recorded using search coils. Results: Each animal in the 3- and 6-month-duration groups showed LN of ⬃2 to 6 deg/s during monocular fixation. In the 3-week-duration group, one monkey exhibited subtle LN (⬃0.5 deg/s) in only one eye, while the other monkey exhibited no LN (indistinguishable from controls). The longer the duration of decorrelation, the greater the LN: mean slow-phase eye velocity (SPEV) in the 6-month-duration group was 3⫻ greater than that in the 3-month group, and 20⫻ greater than in the 3-week group (ANOVA, P ⫽ 0.006). Mean LN intensity (LN amplitude ⫻ frequency) followed the same pattern: the intensity in the 6-month group was 3⫻ greater than that in the 3-month group, and 20⫻ greater than that in the 3-week group (ANOVA, P ⫽ 0.01). Conclusions: Early correction of strabismus (ie, reduction of binocular decorrelation) reduces fusion maldevelopment/latent fixation nystagmus in primates. These findings indicate that early correction of infantile strabismus is beneficial for brain development.
Abstracts
91
Doctor, Why Are You Operating on my Good Eye? Alan B. Richards MD; LSU Medical Center, Shreveport, LA Introduction: This paper describes a series of five patients with reduced vision in one eye preoperatively who had bilateral strabismus surgery and developed a visually threatening or actually visually destructive complication in the better seeing eye related to the strabismus surgery. Two complications were from retinal detachments, one from optic atrophy from a retro bulbar hemorrhage, and one from crystalline keratopathy from postoperative use of Ciloxan in the better seeing eye. Methods: A questionnaire was sent by mail to all members of AAPOS and the literature was surveyed. Results: Five patients were identified who had visually threatening or actual vision loss to the better seeing eye following bilateral strabismus surgery. Three of the complications did not involve the use of the scleral bite to reinsert the muscle, illustrating the diverse types of complications that can occur with strabismus surgery. The worst complication in a patient with retrobulbar hemorrhage (resulting in NLP vision) occurred with a superior oblique tenectomy with no suture in the tendon at all. Discussion: The decision of whether to perform strabismus surgery on the better seeing eye occurs frequently in a wide variety of problems, including torticollis, pattern strabismus, and restrictive strabismus. Conclusions: Textbooks and the ophthalmic literature as well as resident education do not directly address the question of when to perform bilateral strabismus surgery in patients with reduced vision in one eye. More concise recommendations from AAPOS would be helpful in guiding pediatric ophthalmologists and in defense of litigation. Duration of Binocular Decorrelation Predicts the Intensity of Fusion Maldevelopment (Latent) Nystagmus in Strabismic Macaque Monkeys Michael Richards, Agnes Wong MD, Paul Foeller MSc, Dolores Bradley PhD, Lawrence Tychsen MD; Washington University School of Medicine, St. Louis, MO Purpose: Infantile esotropia is linked strongly to latent nystagmus (LN) in human infants, but many features of this comorbidity are unknown. The purpose of this study was to determine how the duration of early-onset strabismus affects the prevalence and magnitude of LN. Methods: Optical strabismus was created in nine infant macaques by fitting them with prism goggles on DOL 1. The goggles were removed after 3 weeks (n ⫽ 2), 3 months (n ⫽ 2), or 6 months (n ⫽ 3), emulating surgical repair of strabismus in humans at 3, 12, and 24 months of age, respectively. Two control monkeys wore plano lenses. Several months after the goggles were removed, eye movements during fixation tasks were recorded using search coils. Results: Each animal in the 3- and 6-month-duration groups showed LN of ⬃2 to 6 deg/s during monocular fixation. In the 3-week-duration group, one monkey exhibited subtle LN (⬃0.5 deg/s) in only one eye, while the other monkey exhibited no LN (indistinguishable from controls). The longer the duration of decorrelation, the greater the LN: mean slow-phase eye velocity (SPEV) in the 6-month-duration group was 3⫻ greater than that in the 3-month group, and 20⫻ greater than in the 3-week group (ANOVA, P ⫽ 0.006). Mean LN intensity (LN amplitude ⫻ frequency) followed the same pattern: the intensity in the 6-month group was 3⫻ greater than that in the 3-month group, and 20⫻ greater than that in the 3-week group (ANOVA, P ⫽ 0.01). Conclusions: Early correction of strabismus (ie, reduction of binocular decorrelation) reduces fusion maldevelopment/latent fixation nystagmus in primates. These findings indicate that early correction of infantile strabismus is beneficial for brain development.