Anomalous extraocular muscles with strabismus.

SUMMARY: An 8-month-old boy with Gorlin syndrome presented with a large right-face turn and constant exotropia of the left eye. Eight-millimeter recession of the left lateral rectus muscle was performed at 23 months of age without complete postoperative improvement. Orbital imaging revealed bilateral anomalous extraocular muscles inferolateral to the optic nerves. Surgical resection of the tissue confirmed the accessory musculature with postoperative correction of the strabismus. To our knowledge, this appears to be the first reported case in the radiologic literature.

Infantile infection and diabetes insipidus in children with optic nerve hypoplasia.

BACKGROUND: Bilateral optic nerve hypoplasia (BONH) is often associated with other central nervous system midline abnormalities (septo-optic dysplasia). Hormonal dysfunction, caused by anterior (cortisol) and posterior (ADH) pituitary involvement, can be sudden, severe, and life threatening. METHODS: Case series. Three cases of septo-optic dysplasia (SOD) presenting as infantile infection with associated diabetes insipidus are reported. The diagnosis of SOD was suspected only after ophthalmological evaluation; further evaluation led to the diagnosis of panhypopituitarism. CONCLUSIONS: A high index of suspicion is required to diagnose SOD in children when the disorder presents with infantile infection and hypernatraemia. Early warning signs of neonatal jaundice and hypoglycaemia should prompt ophthalmological evaluation.

Age-based refinement of referral criteria for photoscreening.

OBJECTIVE: To evaluate examination results from preschool children referred from photoscreening, and to adjust referral criteria for suspected astigmatism. DESIGN: Cross-sectional study and noncomparative case series. PARTICIPANTS: Thirty-one thousand fifty-three preschool children. METHODS: Analysis of (1) referral rate and unreadable photograph rate for all children screened, (2) examination results and treatment plan for all children referred for suspected astigmatism, and (3) examination results and treatment for all referred children aged less than 1 year. MAIN OUTCOME MEASURES: Referral rate, unreadable photograph rate, predictive value positive, treatment plan. RESULTS: The referral rate dropped from 7.8% for children 6 to 11 months to 5.3% for all other ages. The unreadable photograph rate declined exponentially from 12.1% for children aged 6 to 11 months to 1.1% for children aged 4 years. The predictive value positive of a photoscreen referral for all children in the 6- to 11-month age group was 30%, and only 12 of the 94 referred children were treated. The predictive value positive for children less than 1 year of age referred with suspected astigmatism was even lower (25%), and only one child in this age group was treated. The predictive value positive increased with age, and a higher percentage of older children were treated. For children at least 3 years old referred for suspected astigmatism, the predictive value positive was 67% when the examination was performed by a pediatric ophthalmologist. Strabismus, anisometropia, and high hypermetropia were diagnosed in such patients age 2 and older but never in younger children. CONCLUSIONS: Children less than 1 year of age have a much lower pass rate from photoscreening than do older children because of a higher referral rate and higher unreadable rate in this age group. When these children are examined, significant pathosis is usually absent, and intervention is rarely initiated. Most children age 2 and older who are referred for suspected astigmatism have a high likelihood of significant pathosis. It is probably unnecessary to examine children less than age 2 when their photoscreening suggests only astigmatism; conversely, referrals should still be provided for these children when their screening suggests other potentially amblyogenic factors.

Skew deviation and inferior oblique palsy.

PURPOSE: To describe ocular motility and neuro-ophthalmologic findings in six patients with an ocular tilt reaction (OTR) that mimicked an inferior oblique palsy (IOP). DESIGN: Observational Case Series. METHODS: Series of six patients presenting to tertiary care pediatric or neuro-ophthalmologist. RESULTS: Five patients had ocular motility and three-step test results suggesting an IOP; one patient had a suspected bilateral IOP. All six patients had excyclotorsion of the hypotropic eye, and four had incyclotorsion of the hypertropic eye. This is contrary to that expected with an IOP (incyclotorsion of the hypotropic eye). In addition, all six patients had other neurologic findings in the history or examination that were associated with neurologic insult rather than an isolated IOP. Two patients had surgery consisting of a superior rectus recession; this was successful in eliminating diplopia in both patients and in eliminating the vertical deviation and head posturing in one patient. CONCLUSION: While many vertical deviations that appear to be due to an inferior oblique palsy based on the results of the three-step test may be caused by inferior oblique weakness, skew deviation should also be considered in any patient with a history of head trauma, or other neurologic findings. The cyclotorsion observed in IOP is opposite that seen with OTR, and differentiates the two entities clinically. We postulate that these deviations are caused by damage to the otolithic projections that correspond to those from the ipsilateral posterior semicircular canal (on the side of the hypotropic eye).

Primary oblique muscle overaction: the brain throws a wild pitch.

BACKGROUND: Sensorimotor and orbital anatomical mechanisms have been invoked to explain primary oblique muscle overaction. METHODS: Review of primitive visuo-vestibular reflexes and neuroanatomical pathways corresponding to vestibulo-ocular reflexes, and correlation with known clinical abnormalities in patients with primary oblique muscle overaction. RESULTS: Bilateral superior oblique muscle overaction, which corresponds to a backward pitch in lateral-eyed animals, can occur when structural lesions involving the brainstem or cerebellum increase central otolithic input to the extraocular muscle subnuclei that modulate downward extraocular muscle tonus. Bilateral inferior oblique overaction, which corresponds to a forward pitch in lateral-eyed animals, may result from visual disinhibition of central vestibular pathways to the extraocular muscle subnuclei that modulate upward extraocular muscle tonus. CONCLUSIONS: Primary oblique muscle overaction recapitulates the torsional eye movements that occur in lateral-eyed animals during body movements or directional luminance shifts in the pitch plane. These primitive ocular motor reflexes become manifest in humans when early-onset strabismus or structural lesions within the posterior fossa alter central vestibular tone in the pitch plane.

SITA visual field testing in children.

PURPOSE: The Swedish Interactive Thresholding Algorithm (SITA) is a new testing strategy for the Humphrey perimeter. The standard SITA algorithm shortens test time in adults without increasing variability, but its usefulness for detecting field defects in children has not been investigated. METHODS: We evaluated 92 standard SITA 24-2 visual fields of children, most of whom had various types of optic neuropathies (pediatric idiopathic intracranial hypertension, homonymous defects, bitemporal defects, papilledema from brain tumors), and compared them with 49 full threshold 24-2 fields obtained in similar patients. We evaluated outcome measures of foveal threshold, mean defect, pattern standard deviation, false-negative and false-positive rates, and test time. Five children (9 eyes) had both SITA and full threshold testing (FTT). RESULTS: The SITA decreased test time by over 50% compared with FTT (12.6 +/- 3.0 minutes vs 6.6 +/- 1.6 minutes [P <.00001]). When patients with field defects were eliminated, the pattern standard deviation was lower with SITA than FTT (P <.002), indicating lower intratest variability of SITA in subjects with normal fields. No detectable difference was observed in the other outcome measures. Subjective analysis of gray-scale fields in patients who underwent testing with the use of both strategies showed marked similarities. CONCLUSIONS: SITA shortens test time significantly compared with FTT and does so without jeopardizing interpretability. SITA has less intratest variability than FTT and therefore should be better for detecting and following defects. Caution is advised when following a visual field defect unless the same strategy is used for each evaluation. Switching strategies in the absence of a stable field defect is not recommended.

Recurrence of idiopathic intracranial hypertension after weight loss: the carrot craver.

PURPOSE: To describe a patient with stable idiopathic intracranial hypertension whose papilledema worsened. METHOD: Case report. RESULTS: A patient with documented idiopathic intracranial hypertension had resolution of disc edema with weight loss. Recurrence of papilledema led to the discovery that she consumed large quantities of raw carrots to help maintain her weight. Her increased vitamin A levels normalized, and the disc edema resolved when she stopped eating carrots. CONCLUSION: Patients with idiopathic intracranial hypertension should be counseled regarding carrot intake.

Screening for amblyogenic factors using a volunteer lay network and the MTI photoscreener. Initial results from 15,000 preschool children in a statewide effort.

PURPOSE: To describe the results from a statewide preschool vision screening program using the MTI PhotoScreener (Medical Technology and Innovations, Inc., Cedar Falls, IA). DESIGN: Cross-sectional study. PARTICIPANTS: A total of 15,059 children aged 6 to 47 months enrolled in childcare and preschool settings throughout the state of Tennessee. METHODS: Volunteers from local Lions Clubs took photoscreening photographs of children in a statewide effort. Photographs were interpreted at the Vanderbilt Ophthalmic Photography Reading Center using predetermined criteria. Children who failed the screening were referred to community ophthalmologists or optometrists who performed a comprehensive evaluation and forwarded the results to the authors. MAIN OUTCOME MEASURES: Referral rate, unreadable rate, and predictive value positive (PVP). RESULTS: During the 2 years of the screening program, 15,059 children were screened in 850 screenings. The screening referred 1013 children (6.7%), and 704 photographs (4.7%) were unreadable. Children who failed the screening had a significant abnormality (strabismus, anisometropia, high hypermetropia, high astigmatism, or high myopia) in 320 of the 531 cases where adequate follow-up results were reported. The PVP ranged from 84% when a diagnosis of strabismus was suggested by the photoscreen reading to 41% for astigmatism. Despite intense attention to follow-up, many children who failed the screening never received a formal eye examination. CONCLUSIONS: The MTI PhotoScreener can be used by volunteers to screen preschool children and can have a high PVP in organized settings, provided that meticulous attention is paid to photograph interpretation and quality control. The PVP of the MTI PhotoScreener depends on the diagnosis suggested when the photograph is read. Significant obstacles exist in obtaining care for those who fail screening.

Prognostic indicators for vision and mortality in shaken baby syndrome.

OBJECTIVE: To study ocular and nonocular signs of patients diagnosed as having "shaken baby syndrome" and determine prognostic indicators for vision and mortality. METHODS: Medical records of child abuse cases involving bilateral retinal hemorrhages were reviewed. Particular attention was paid to visual function and pupillary light reaction at the time of admission as well as the location of retinal hemorrhages, neuroimaging findings, ventilatory requirement, and associated skeletal injuries. These findings were correlated with visual prognosis and mortality. RESULTS: Thirty consecutive cases met the criteria for review. At the initial visit, mean age of the children was 9.3 months (range, 1-39 months) and 12 children (40%) had at least fix-and-follow vision. Preretinal and intraretinal hemorrhages (93% [n = 28] and 100% [n = 30]) were more common than vitreous hemorrhage (10% [n = 3]). Subdural hematomas were detected in 21 patients (70%). Twenty children (67%) had seizures and 16 (53%) required ventilatory support; bruises and long bone fractures were seen in 14 (47%) and 4 (13%) children, respectively. Eight patients died. All patients with nonreactive pupils on presentation died, while all patients with a pupillary light reaction lived (P<.001). Six (86%) of 7 patients with midline shift died, whereas 21 (91%) of 23 with no midline shift lived (P<.001). At follow-up, retinal hemorrhages had resolved in nearly all children by 4 months, and 16 children (73%) had at least fix-and-follow vision. Ventilatory requirement was associated with poorer vision (P<.01). CONCLUSIONS: Nonreactive pupils and midline shift of the brain structures correlate highly with mortality. Ventilatory requirement, but not visual acuity on presentation, predicts visual outcome.

Unilateral retinal hemorrhages and ipsilateral intracranial bleeds in nonaccidental trauma.

The classic ophthalmologic finding in nonaccidental traumatic injury is bilateral widespread retinal hemorrhage with or without intracranial hemorrhage. We present 3 cases of unilateral retinal hemorrhage associated with ipsilateral intracranial bleeds to extend the many different presentations of nonaccidental trauma.

Global visual field involvement in acute unilateral optic neuritis.

PURPOSE: To quantify automated visual field defects seen at entry in the Optic Neuritis Treatment Trial (ONTT) to determine whether particular areas of the field are preferentially affected and to determine the extent of visual field involvement in patients having "localized" field defects. METHODS: Review of Humphrey 30-2 Visual Field (Allergan-Humphrey, Inc, San Leandro, CA) data from the involved and fellow eyes of 440 patients who were enrolled in the ONTT. Field defects were evaluated by comparing the involved eye to the fellow eye. RESULTS: Patients with diffuse visual field defects had a relatively equal diminution of visual threshold throughout the tested 30-2 field. Patients with localized central and cecocentral scotomas had their greatest depression of threshold centrally; however, even those patients with mild defects (mean defect, <6 dB) had diminution of visual threshold throughout the entire tested 30-degree field. Patients with moderate (mean defect, 6 to 20 dB) and severe (mean defect, >20 dB) central and cecocentral defects had even greater peripheral depression. Patients with altitudinal or quadrant defects had involvement of the "unaffected" field that also varied with the mean defect. The overall average depression of visual threshold for all patients averaged 36%+/-4% and was relatively uniform throughout the tested field. CONCLUSIONS: Optic neuritis affects the entire central 30-2 field, even in patients who appear to have localized depression of visual threshold. Optic neuritis does not appear to have a predilection for any particular area of the visual field.

Recovery of visual field function in the optic neuritis treatment trial.

PURPOSE: To assess the pattern of recovery of the visual field of patients with optic neuritis and to determine whether all affected portions of the visual field recover similarly or certain portions of the visual field have greater recovery. METHODS: We reviewed the Humphrey Visual Field (Allergan-Humphrey Inc, San Leandro, California) data from the initial and 6-month examination for the involved and fellow eyes of patients enrolled in the Optic Neuritis Treatment Trial (ONTT). The average threshold for each patient was calculated for the entire tested field and for locations within concentric rings having a radius 3, 9, 15, 21, and 27 degrees from fixation. The absolute amount of improvement and percentage improvement in average threshold between entry and the 6-month follow-up examination were determined for each patient. These measurements were compared within the concentric rings to assess patterns of recovery. RESULTS: Patients with localized defects recovered 86%+/-20% of their initial defect in average threshold, whereas those having diffuse defects recovered an average of 85%+/-23%. The area about fixation had the greatest relative recovery of threshold (87%+/-21% at 3 degrees); the relative recovery decreased with increasing eccentricity from fixation (P<.01). CONCLUSIONS: Patients with optic neuritis have a marked return of visual field function that does not appear to differ between patients with diffuse or localized field defects. The reduced redundancy of axons in the periphery of the field compared with near fixation may be responsible for the greater relative recovery of threshold near fixation.

Visual field defects after temporal lobe resection: a prospective quantitative analysis.

OBJECTIVE: To evaluate and quantify prospectively visual field changes in patients undergoing temporal lobe resections for intractable epilepsy. BACKGROUND: Visual field abnormalities occur after temporal lobe resections for epilepsy; however, we have not encountered published reports using automated static visual field analysis. METHODS: Humphrey visual fields (program 30-2) were obtained before and after partial temporal lobe resection in 32 consecutive patients with intractable epilepsy. A quantitative point-by-point analysis was made in the affected superior quadrant, and the defects were averaged for the whole patient group. RESULTS: Thirty-one patients developed a visual field defect, but none was aware of the defect. The points nearest fixation were relatively spared. The defects were greatest in the sector closest to the vertical meridian in the eye ipsilateral to the resection. The ipsilateral and contralateral mean field defects also differed in both topography and depth. A significant correlation was found between the extent of lateral temporal lobe resection and the degree of the defect in the contralateral eye. CONCLUSIONS: There are differences in the shape and depth of the ipsilateral and the contralateral field defects not previously reported. These findings demonstrate that certain fibers from the ipsilateral eye travel more anteriorly and laterally in Meyer's loop, and support the hypothesis that visual field defects due to anterior retrogeniculate lesions are relatively incongruous because of anatomic differences in the afferent pathways. Automated perimetry is a sensitive method of evaluating and quantifying visual field defects.