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At least 451 records · Page 25Linked to original sources
PubMed2003

Screening for retinoblastoma: presenting signs as prognosticators of patient and ocular survival.

OBJECTIVE: To correlate 3 common presenting signs of retinoblastoma with patient and ocular survival and to assess the efficacy of current pediatric screening practices for retinoblastoma. METHODS: A retrospective study was conducted of 1831 retinoblastoma patients from our center (1914-June 2000). Patient survival (excluding deaths from other primary neoplasms) and ocular survival (presenting eyes) rates were calculated and analyzed using the Kaplan-Meier method. RESULTS: Leukocoria correlated with excellent patient survival (>86%, 5 years) but poor ocular survival in unilateral (4%, 5 years) and bilateral patients (29%, 5 years). A total of 308 (19%) of 1654 patients presented with strabismus: patient survival was excellent (90%, 5 years), and ocular survival was poor (17%, 5 years) yet better than leukocoria. Patients who had a family history of retinoblastoma and were clinically screened for retinal tumors from birth were diagnosed younger (8 months of age) and earlier (Reese Ellsworth group 1 = 26 [58%] of 45) and had better ocular survival than nonscreened patients with a family history. More patients were initially detected by family/friends (1315 [80%] of 1632) than pediatricians (123 [8%] of 1632) or ophthalmologists (156 [10%] of 1632). CONCLUSION: Most US children whose retinoblastoma is diagnosed initially present with leukocoria detected by a parent, despite routine pediatric screening for leukocoria via the red reflex test. Initial disease detection at the point of leukocoria or strabismus correlated with high patient survival rates and poor ocular survival rates for the presenting eye. Saving eyes and vision requires disease recognition before leukocoria, as demonstrated by the better ocular salvage rate among patients who had a positive family history and received clinical surveillance via early, routine dilated funduscopic examinations by an ophthalmologist.

Humans↗
PubMed1993

Pupillary abnormalities in type I diabetes occurring during adolescence. Comparisons with cardiovascular reflexes.

OBJECTIVE: To evaluate computerized infrared pupillometry for the assessment of autonomic neuropathy in adolescents with type I diabetes. RESEARCH DESIGN AND METHODS: We measured resting pupil diameters and pupillary light reflexes in 142 adolescents with type I diabetes (72 boys and 70 girls, 10.4-19.8 yr of age, duration of diabetes 0.7-18.3 yr) and in 75 nondiabetic control subjects (29 boys, 46 girls, 11.3-19.8 yr of age). All study participants were assessed using four standard cardiovascular tests: maximum-minimum heart rate during deep breathing (mean of three cycles); heart-rate change during a Valsalva maneuver (Valsalva ratio, mean of three maneuvers); lying-to-standing heart-rate change (30:15 ratio); and lying-to-standing BP change. RESULTS: Mean resting pupil diameters were significantly smaller in the diabetic group: 6.28 +/- 0.06 vs. 6.77 +/- 0.11 mm, P < 0.0001); and significantly smaller with greater duration of diabetes (r = -0.29, P = 0.0006) and higher levels of GHb (r = -0.24, P = 0.004). Patients with retinopathy grade 30 or more (Wisconsin 191 grading) had significantly smaller resting pupil diameters: 5.9 +/- 0.16 vs. 6.4 +/- 0.12 mm, P = 0.008). The phasic light reflex as determined by reflex amplitude and maximum constriction velocity was significantly reduced in the diabetic group: 2.27 +/- 0.03 vs. 2.44 +/- 0.04 mm, P = 0.0009; and 6.68 +/- 0.12 vs. 7.24 +/- 0.16 mm/s, P = 0.007). Reduced reflex amplitude was related to a longer postpubertal duration of diabetes (r = -0.18, P = 0.04). We found no association between pupillary and cardiovascular tests. CONCLUSIONS: Infrared computerized pupillometry demonstrates subclinical diabetic autonomic neuropathy as early as adolescence. Its presence seems to be related to longer duration of diabetes and unfavorable metabolic control.

Adolescent↗
PubMed2002

Pupil signs of sympathetic autonomic neuropathy in patients with type 1 diabetes.

OBJECTIVE: Pupillary autonomic neuropathy is considered an early sign of the development of systemic autonomic neuropathy. Sympathetic denervation is related to the duration of diabetes and the development of systemic autonomic dysfunction. We investigated pupil responsiveness to directly and indirectly acting sympathomimetics in type 1 diabetic patients with and without long-term complications, defined as cardiac autonomic neuropathy (CAN), peripheral sensomotor neuropathy, retinopathy, and nephropathy, and in healthy subjects. RESEARCH DESIGN AND METHODS: A total of 47 randomly chosen type 1 diabetic patients and 20 healthy subjects were selected for this study. Patients were divided into groups determined by whether they had long-term diabetic complications. Pharmacological tests were performed with cocaine 4%, epinephrine 1%, and pholedrine 5% eye drops. Horizontal pupil diameter (HPD) was measured at the beginning of the pharmacological tests and at defined time points after instillation of the eye drops. RESULTS: Statistical analysis showed a significantly smaller HPD in the patients before instillating eye drops (P = 0.011). In particular, the HPD was significantly smaller in the patient group without CAN when compared with healthy subjects (P = 0.004). Maximal cocaine reaction was diminished in the complication group (P < 0.001). Epinephrine test, visual acuity, ocular pressure, and HbA(1c) did not differ in patients with or without long-term complications. The noncomplication group showed no significant differences in pupillary responses as compared with healthy subjects. The complication group showed a smaller HPD (P = 0.022), reduced pupillary responses in the cocaine (P = 0.037) and pholedrine tests (P < 0.001), and anisocor pupil sizes after instillation of the eye drops (P = 0.034). CONCLUSIONS: Our results clearly show that sympathetic denervation does exist in the pupil of diabetic patients and that it can be rapidly assessed using the cocaine test. These data and the results of the epinephrine test suggest a mixed pre- and postganglionic dysfunction of the sympathetic plexus. The significant smaller HPD in patients without CAN compared with that of healthy subjects could be a sign for early involvement of the pupil function before cardiac manifestation of systemic autonomic diabetic neuropathy.

Adult↗
PubMed1986

Hypnotherapy: a possible alternative for treating pupils affected with attention deficit disorder.

The purpose of this study was to ascertain the effectiveness of hypnotherapy in treating attention-deficit-hyperactivity by certified psychologist and physicians. The 7 boys and 4 girls were enrolled in a Special Day classroom for hyperkinesis and behavior problems and were being given methylphenidate (Ritalin). Three attempts were made to hypnotize these subjects; however, the group had to be reduced from 11 to 3 and finally, from 3 to a single child to be successful. A significant improvement from pre- and post-hypnotic sessions was noted.

Attention Deficit Disorder with Hyperactivity↗
PubMed1990

Relative afferent pupillary defect in the "better" eye.

A relative afferent pupillary defect usually occurs in an eye with unilateral or asymmetric optic nerve or extensive retinal disease. In general, the eye with poorer visual acuity has the afferent pupillary defect. Twenty-five patients are reported, however, in whom an afferent pupillary defect occurred in the eye with better visual acuity. These eyes had optic nerve or retinal dysfunction. The eyes with worse visual acuity but no afferent pupillary defect had an abnormality of the ocular media (corneal opacity, hyphema, anterior segment membrane, cataract, or vitreous opacity), amblyopia, refractive error, age-related macular degeneration, or cystoid macular edema. An afferent pupillary defect does not necessarily occur in the eye with poorer visual acuity.

Adolescent↗
PubMed1991

Fourth nerve paresis and ipsilateral relative afferent pupillary defect without visual sensory disturbance. A sign of contralateral dorsal midbrain disease.

We describe a patient with a left trochlear nerve paresis and a left relative afferent pupillary defect despite normal visual acuity, color vision, visual fields, and fundus examination. Magnetic resonance imaging revealed a lesion in the right dorsal midbrain extending from the brachium of the superior colliculus to the inferior colliculus. The anatomy and physiology of the pupillary light reflex are reviewed, as are possible mechanisms for the laterality of afferent pupillary defects with midbrain lesions. The presence of a trochlear nerve paresis with an ipsilateral relative afferent pupillary defect and an otherwise normal ophthalmic exam indicates a lesion in the contralateral dorsocaudal midbrain.

Adult↗
PubMed1994

A diagnostic quartet in leptomeningeal infiltration of the optic nerve sheath.

A rare case of optic leptomeningeal carcinomatosis secondary to a rectal adenocarcinoma is recorded. The presentation of rectal disease with blindness is unique. A diagnostic quartet of symptoms and signs of leptomeningeal infiltration of the optic nerve sheath is proposed and its value emphasized by the inability of further investigation to confirm the clinical diagnosis. The investigation and treatment of leptomeningeal carcinomatosis is reviewed.

Adenocarcinoma↗
PubMed1992

Autosomal dominant congenital miosis with megalocornea.

A family with AD congenital miosis is presented. The ocular symptoms were: megalocornea, iris translucency, microcoria with poor pupillary dilatation and goniodysgenesis with anterior insertion of the iris. This observation confirms that in congenital miosis abnormal development of the whole anterior eye segment may occur. The patients have an increased risk to develop glaucoma. If retinoscopy is impossible due to pin-point pupils, ultrasonic biometry to determine the axial length is recommended. An optical iridectomy could improve visual performance at low illumination; the complaints of photophobia, which are related to the iris translucency, persist.

Adolescent↗
PubMed1990

The abnormal pupil in Cheyne-Stokes respiration. Case report.

Cheyne-Stokes respiration commonly induces a rhythmic pupillary dilatation during hyperpnea and constriction during apnea. Failure of a pupil to dilate during hyperventilation indicates underlying sympathetic nerve paralysis. This report deals with an instance in which one pupil failed to constrict during apnea due to oculomotor nerve compression. The periodic respirations and anisocoria disappeared following surgical evacuation of a large ipsilateral subdural hematoma.

Anisocoria↗
PubMed1995

One-year outcome following craniotomy for traumatic hematoma in patients with fixed dilated pupils.

Forty consecutive patients who underwent craniotomy for traumatic hematoma after developing bilateral fixed dilated pupils were studied to determine the factors influencing quality of survival and to seek criteria for management. Clinical and computerized tomography (CT) data were correlated with outcome 1 year after craniotomy. The functional recovery (good outcome or moderate disability) rate was 25%, with a mortality rate of 43%. Patients with subdural hematoma had a higher mortality rate (64%) compared to patients with extradural hematoma (18%) (chi-square test, p > 0.05). Other factors associated with markedly increased morbidity and mortality were increasing age (> 20 years), a prolonged interval (> 3 hours) between loss of pupillary reactivity and craniotomy, compression of basal cisterns, and presence of subarachnoid hemorrhage on CT. There were no survivors among patients exhibiting any of the following features: surgery 6 hours or more after bilateral loss of pupillary reactivity; age greater than 65 years; or absent motor response. Apart from the latter group, the nature of motor response (before pharmacological paralysis and intubation) was not a reliable predictor of mortality. The results suggest that the presence of an acute subdural hematoma is the single most important predictor of negative outcome in patients with bilateral unresponsive pupils.

Adolescent↗
PubMed2004

Successful use of the new high-dose mannitol treatment in patients with Glasgow Coma Scale scores of 3 and bilateral abnormal pupillary widening: a randomized trial.

OBJECT: The authors evaluated long-term clinical outcomes in selected acutely comatose patients with severe diffuse brain swelling and recent clinical signs of impending brain death who received a novel high-dose mannitol treatment compared with those who received conventional-dose mannitol in the emergency room. METHODS: Forty-four adult patients with traumatic, nonmissile-inflicted, acute, severe diffuse brain swelling were prospectively and randomly evaluated. All patients were selected based on the presence of recent clinical signs of impending brain death on the first emergency room evaluation. These signs included bilateral abnormal pupillary widening and lack of motor responses to painful stimulation (Glasgow Coma Scale score of 3). The study group (23 patients) received ultra-early and fast intravenous high-dose mannitol treatment (approximately 1.4 g/kg), whereas the control group (21 patients) received half that dose (approximately 0.7 g/kg). Ultra-early improvement of bilateral abnormal pupillary widening was significantly more frequent in the high-dose mannitol group than in the conventional-dose group (p < 0.02). High-dose mannitol treatment in the emergency room was also associated with significantly better 6-month clinical outcomes (p < 0.02); the best rate of favorable outcomes was 43.5%, compared with only 9.5% in the conventional-dose mannitol group. The two groups of patients were well matched with respect to all emergency room and head computerized tomography findings, as well as the timing of initial mannitol treatment (approximately 80-90 minutes after the first evaluation at the scene of the injury). Comparative evaluation of bilateral pupillary widening between the scene of the injury and the emergency room showed no significant differences between groups, whereas mannitol dose dependence was statistically significant (p < 0.05), insofar as early pupillary improvement in the emergency room was concerned. CONCLUSIONS: Ultra-early high-dose mannitol administration in the emergency room is the first known treatment strategy significantly to reverse recent clinical signs of impending brain death, and also to contribute directly to improved long-term clinical outcomes for these patients who have previously been considered unsalvageable.

Acute Disease↗
PubMed1994

Quantitative analysis of changes of automated perimetric thresholds after pupillary dilation and induced myopia in normal subjects.

We quantitatively analyzed the changes of the (retinal) threshold sensitivities between normal status and dilated emmetropic status, and between dilated emmetropic status and -1.0D induced myopic status. Three consecutive visual field examinations by Humphrey Field Analyzer C-30-2 threshold test and STATPAC program were performed in 36 eyes of 21 normal subjects who ranged in age from 23 to 40 years. The results were as follows: 1) There was a statistically significant decrease (0.87 +/- 0.91dB)in the mean threshold sensitivity within the central 30 degrees at the dilated emmetropic status (27.90dB) as compared with the normal status (28.77dB)(p = 0.0001). 2) There was a statistically significant decrease(0.99 +/- 0.61dB) at -1.0D induced myopic status(26.91dB) as compared with the dilated emmetropic status(27.90dB)(p = 0.0001). 3) After dilation, the mean threshold sensitivity between 20 and 30 degrees of field showed statistically significant decrease as compared with those within the central 20 degrees of visual field(p < 0.05). In case of -1.0D induced myopia, there were uniform decreases in the mean threshold sensitivities within the central 30 degrees of visual field (p > 0.05). Therefore, we emphasize the importance of consistent pupillary size and correct refractive state in performing the serial automated visual field tests.

Adult↗
PubMedDate not supplied

Congenital central hypoventilation syndrome: ocular findings in 37 children.

BACKGROUND: Congenital central hypoventilation syndrome (CCHS) is a rare cause of central sleep apnea. Although ophthalmic abnormalities have been reported, the ocular findings have not been discussed in detail. METHODS: We examined or obtained the records of 37 children with CCHS. RESULTS: Twenty-seven patients were found to have abnormal pupils, most of which were miotic and reacted poorly to light. In 18 cases, the anterior surface of the iris was unusually smooth. Ten of the children with abnormal pupils also demonstrated light-near dissociation. Twenty had strabismus of various types, and 18 showed evidence of convergence insufficiency. CONCLUSIONS: The high incidence of strabismus, pupillary abnormalities, and convergence insufficiency may be a result of neurologic defects in the midbrain.

Adolescent↗
PubMedDate not supplied

Clinical manifestations and treatment outcome of optic neuropathy in thyroid-related orbitopathy.

BACKGROUND AND OBJECTIVE: To investigate the incidence and outcome of optic neuropathy associated with thyroid-related orbitopathy. PATIENTS AND METHODS: All patients diagnosed as having optic neuropathy associated with thyroid-related orbitopathy who underwent treatment for optic neuropathy between January 1, 1999, and March 1, 2003, were reviewed. Demographic and clinical data were extracted from the oculoplastic registry (electronic medical record). RESULTS: Optic neuropathy occurred in 20 of 595 patients with thyroid-related orbitopathy (3.4%). All patients received systemic steroids, 7 patients received orbital steroid injections, and 2 patients were treated with orbital radiotherapy. Orbital decompression was performed in all 26 orbits. Visual acuity improved from a mean of 20/150 to 20/30 (P < .001). Color vision improved from a mean of 5.2/14 to 11/14 (P = .001). The afferent pupillary defect disappeared shortly after treatment for all but 1 case, and improved in all cases. Exophthalmos decreased from 26.4 +/- 2.5 to 21.5 +/- 2.1 mm (P< .001; 95% confidence interval, 3.8 to 5.7). CONCLUSIONS: Optic neuropathy manifests rarely in patients with thyroid-related orbitopathy. Monitoring visual acuity or afferent pupillary response may be a reliable way of assessing and monitoring optic neuropathy. In this study, patients had improved visual acuity and optic nerve function after a combination of medical and surgical treatment.

Adult↗