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Background: Kawasaki disease (KD) is an acute and sometimes fatal febrile vasculitis of childhood, the presenting signs of which include conjunctival vessel dilatation and iridocyclitis. Consultation with a pediatric ophthalmologist is helpful for early recognition of the disease, especially In identifying "incomplete" cases, ie, those which lack all of the classical, systemic signs. Methods: After encountering a case of incomplete KD in which diagnosis was delayed, we reviewed the hospital records of 37 children with KD to establish how often the disease manifested "incompletely," and how often pediatricians consulted pediatric ophthalmologists to help in its early diagnosis. Results: Forty-five percent of the children eventually diagnosed with KD lacked the complete diagnostic criteria of KD when admitted to the hospital, and diagnosis and treatment therefore were delayed. Coronary artery aneurysms, a complication that might have less serious consequences if treated earlier, developed in 24% of these patients. Although 67% presented to their pediatrician with bilateral conjunctival injection and/or iridocyclitis, pediatricians requested ophthalmologic consultation for only 5% of them. Conclusions: Our review of these cases indicates that the eye findings in KD could play a role in earlier diagnosis and treatment. Pediatric ophthalmologists and pediatricians should be more aware of their combined responsibilities for expethent recognition of KD.

Caudal regression syndrome (CRS) is a rare embryopathy characterized by maldevelopment of the vertebrae, visceral organs, and lower extremities. Patients with CRS have deformities of the sacrococcygeal vertebrae and develop neurogenic bladder from dysfunction of the sacral nerve root. 1-3 Additional anomalies include congenital heart defects, intestinal malrotation, agenesis of the genitourinary system, imperforate anus, and malformation of the lower limbs. 1-3 Ocular findings described in the nonophthalmic literature have been confined to hypertelorism, epibulbar dermoid, and anophthalmia. 3,4 We report a patient with CRS who presented with infantile glaucoma and punctal atresia. A computerized search of the medical literature showed no previous reports of CRS in ophthalmic journals and no previous reports of an association with glaucoma or nasolacrimal anomalies. CASE REPORT A 10-week-old infant presented with a 1-week history of bilateral epiphora and photophobia. The patient had neonatal documentation of lumbosacral vertebral anomalies, atrioseptal defect, bifid clitoris, imperforate anus, dislocated hip, and syndactyly of both feet (Fig 1). At 9 weeks, she underwent repair of a malrotated intestine and vesicostomy for neurogenic bladder. The mother’s health and prenatal care were excellent, and the pregnancy and delivery were unremarkable. Family history was negative for parental consanguinity or hereditary ocular or systemic disease. The infant was noted to have hypertelorism, and lower lid punctal agenesis. Both corneas exhibited diffuse stromal thickening, which was more severe in the left eye. Horizontal corneal diameters measured 11.5 mm in the right eye and 12.5 mm in the left eye. Intraocular pressures (IOP) measured serially using a hand-held applanation tonometer were 30 mm Hg in each eye. Portable slit-lamp examination showed no evidence of anterior segment dysgenesis. Fundoscopic examination showed cup-to-disc ratios of 0.5 (right eye) and 0.6 (left eye). The next day, an examination was performed under anesthesia to confirm the diagnosis of glaucoma, after which 90° goniotomies were performed first in the right eye and 1 week later in the left eye. The corneal edema progressively resolved during the next month, and the cup-to-disc ratios decreased to 0.3 in both eyes. At 14 months, bilateral punctoplasty and nasolacrimal duct reconstruction were carried out to repair the punctal atresia. At 3 years, repeat bilateral goniotomies were performed

Purpose To assess the effect of a pediatric eyelid speculum on the measurement of intraocular pressure (IOP) in children. Design Observational case series. Methods Intraocular pressure measurements were performed during examination under anesthesia immediately after induction. The measurements were made with and without a pediatric eyelid speculum in place. Results Intraocular pressure measurements were made in 77 eyes of 45 consecutive children. The mean IOP without the speculum was 16.33 mm Hg in the right eye and 16.55 mm Hg in the left eye. With the lid speculum in place the mean IOP was 20.26 mm Hg in the right eye and 20.05 mm Hg in the left eye ( P Conclusions The presence of an eyelid speculum raised IOP an average of 4 mm Hg. This effect should be considered when interpreting IOP measurements in children.

Several skeletal muscles are composed of multiple neuromuscular compartments that can be controlled individually by corresponding motor neuron pools.1–3 The transversus abdominis is composed of distinct regions that contract differentially.3 Similarly, the human cricothyroid muscle has three bellies with distinct functions innervated by separate motor nerve branches.3 The triceps brachii is composed of multiple fascicles that may be considered as distinct muscles with completely independent motoneuron subnucleus innervation.4 There exist more extraocular muscle (EOM) fibers and motor neurons than apparently required by conventionally recognized mechanisms of ocular motility.5,6 Could this be because individual EOMs are compartmentalized to implement multiple functions? One such example is the active pulley hypothesis that proposes that orbital layers of EOMs insert in connective tissue rings, called pulleys, through which pass the global layer fibers that in turn insert on the sclera to rotate the eye. Consequently, during EOM contraction, shifts in pulley positions influence EOM pulling directions.7–10 This laminar aspect of compartmentalization is evident in all oculorotary EOMs. Peng et al.11 traced the intramuscular arborization of the abducens nerve (CN6) within the lateral rectus (LR) muscles of two macaque monkeys and two humans, demonstrating that CN6 bifurcates externally to the EOM into two major trunks whose arborizations remain segregated into superior versus inferior zones throughout the EOM's length. Based on this finding, Peng et al.11 proposed that selective neural control of the two LR zones could execute significant torsional and vertical actions that are not classically recognized. This neuroanatomical finding of Peng et al.11 is consistent with the LR's dual-headed origin in the deep orbit.12,13 Some aspects of the LR are unique among EOMs, however, so that compartmentalization of LR innervation might be a developmental artifact rather than neural control strategy. Older studies of EOM embryology claimed that the LR originates from two different myotomes.14,15 More recent studies in birds demonstrate that the LR arises from somitomeres 4 and 5,16,17 and that CN6 arises from both rhombomeres 5 and 6.16 Separation of CN6 into two parallel nerve trunks is not rare. Indeed, 8–15% of CN6 in humans are split in this way.18–21 The LR can also exhibit longitudinal splitting, most prominently in congenital disorders of cranial nerve development such as processes involving the EOM or cranial nerve development such as congenital fibrosis of the extraocular muscles type 1,22 congenital oculomotor palsy,23 congenital trochlear palsy,23 and Duane syndrome.24–26 The possibility of selective activation of compartments requires topographical projection of motor nerves within segregated EOM regions. If selective control of rectus EOM compartments is a general neural strategy, evidence of compartmentalization should be evident in the intramuscular innervation of other rectus EOMs besides the LR. This study aimed at confirming, in additional specimens, Peng et al.'s report of compartmentalized LR innervation11 and extending study by tridimensional reconstruction of nerve arborizations, to the inferior (IR), medial (MR), and superior rectus (SR) muscles of humans and monkeys.

Intraocular pressure (IOP) measurements in children are frequently performed under halothane-nitrous oxide anesthesia; however, anesthesia face masks may limit access to the eyes, and tracheal intubation is associated with transient increases in IOP. Use of the laryngeal mask airway (LMA) permits the maintenance of a patent airway without the need for laryngoscopy and tracheal intubation. In a randomized study of 41 children, we compared the IOP, hemoglobin oxygen saturation, and hemodynamic responses to the insertion of an LMA or tracheal tube during a standardized steady-state anesthetic technique consisting of 1 MAC halothane and 66% nitrous oxide. Baseline measurements of IOP, hemoglobin oxygen saturation, heart rate, and arterial blood pressure were recorded and repeated within 15-30 s after insertion of the airway device and at 1-min intervals for 5 min. Insertion of the LMA required significantly less time (26 +/- 16 vs 39 +/- 17 s [mean +/- SD]) and was associated with higher hemoglobin oxygen saturation values compared with the tracheal intubation. The LMA did not increase IOP, heart rate, or arterial blood pressure above baseline values. In contrast, tracheal intubation was associated with significant increases of IOP, heart rate, and arterial blood pressure. We concluded that the laryngeal mask offers advantages over tracheal intubation and the face mask for airway management in patients undergoing IOP measurements.

These are the hard cases and the children with greatest need. Their spherical-equivalent myopia or hyperopia exceeds approximately 4 to 5 diopters (D). The uncorrected myopia translates to a distant acuity of 20/200 or worse (legal blindness). The uncorrected acuity for the child with hyperopia can be better during epochs when the child accommodates partially, but accommodation is often impaired in children with neurobehavioral disorders. The prevalence of isoametropic amblyopia in children with uncorrected high hyperopia approaches 50%. Add to this the substantial risk (about 80%) of refractive esophoria or esotropia. A variety of neurobehavioral disorders impede spectacle wear. The most common are moderate to severe cerebral palsy (ie, Gross Motor Function Classification Scale levels 3-5), autism, Angelman syndrome, more severe Down syndrome, and suboptimally controlled seizure disorders. Other causes are idiopathic developmental delay/ mental retardation and progressive childhood encephalopathies. The uncorrected ametropia exacerbates the neurobehavioral disorder. The child lives in a cocoon of blur, promoting fearfulness, reduced interest in the outside world, and blunted social interactions (a constellation labeled “visual autism”). The extreme refractive error is seldom the only visual deficit. Common comorbidities include strabismus, amblyopia, optic neuropathies, foveopathies, and nystagmus. Comorbidities do not mean that the refractive error is unimportant or that correcting the error would not improve visual function. Too many ophthalmologists, after examining a child with an intimidating array of neurobehavioral and visuomotor deficits, succumb to the bigotry of low expectations. The uncorrected vision is dismissed as “good enough for a child with multiple disabilities.” Why is prescribing glasses ineffective in a substantial fraction of these children? Many are aversive to wearing any head or facial appliance. They dislodge glasses repeatedly, despite heroic efforts to enforce compliance on the part of parents, teachers, therapists, and caregivers. If the battle is won to keep the glasses in place, the benefit may be negated by poor motor control of the head, neck, and trunk or gaze palsies and apraxias. The motorcontrol disorders cause chronic viewing over, around, or at the edges of spectacle lenses. Contact lenses are seldom a suitable option. Insertion and removal may require bodily restraint by multiple family members and vigorous eyelid manipulation to overcome intense blepharospasm. Repeated rounds of this exhaust— physically and emotionally—even the most committed parents.

PURPOSE. Cerebral palsy (CP) is a permanent, nonprogressive disorder of movement and posture due to a lesion of the fetal or infant brain. The goal was to determine whether children with different severities of CP, as defined using the Gross Motor Function Classification System (GMFCS), had different degrees or types of visual dysfunction. METHODS. An observational, cross-sectional‐design study was conducted by using neurologic and masked ophthalmic measurements on a representative cohort of 50 children with CP. Mean age was 5.6 years (range, 2‐19.5 years); mean gestational age was 31 weeks. RESULTS. The likelihood of debilitating visual deficits was greater in children with higher GMFCS scores, independent of gestational age. Children with level 5 disease (most severe) were at greatest risk for high myopia, absence of binocular fusion, dyskinetic strabismus, severe gaze dysfunction, and optic neuropathy or cerebral visual impairment (CVI). These deficits were rare or absent in children with the mildest disease, level 1. When categorized by anatomic or physiologic CP subtype, diplegic and spastic children were more often hyperopic and esotropic, but had the highest prevalence of fusion and stereopsis. In contrast, children with quadriplegic and mixed CP (dyskinetic, athetoid, hypotonic, and ataxic) more often had high myopia, CVI, dyskinetic strabismus, and gaze dysfunction. CONCLUSIONS. Visual deficits differ in children who have mild versus severe CP. Children with GMFCS level 1 to 2 have sensorimotor deficits resembling those of neurologically normal children with strabismus and amblyopia; children at level 3 to 5 have more severe deficits, not observed in neurologically normal children. (Invest Ophthalmol Vis Sci. 2008;49: 572‐580) DOI:10.1167/iovs.07-0525

sotemporal Pursuit Asymmetries in Strabismic Macaque Monkeys Aasim Hasany, Agnes Wong MD, Paul Foeller MSc, Dolores Bradley PhD, Lawrence Tychsen MD; Washington University School of Medicine, St. Louis, MO Purpose: Infantile esotropia is associated with nasotemporal asymmetries of pursuit during monocular viewing. The purpose of this study was to determine how the duration of binocular decorrelation, which can be strictly controlled in primates, influences directional pursuit asymmetries. Methods: Optical strabismus was created in seven infant macaques by fitting them with prism goggles on DOL 1. The goggles were removed after 3 weeks (n 2), 3 months (n 1), or 6 months (n 2), emulating surgical repair of strabismus in humans at 3, 12, and 24 months of age, respectively. Two control monkeys wore plano lenses. After the goggles were removed, horizontal pursuit was recorded using search coils, evoked by “stepramp” target motion. A nasal bias index (NBI) was calculated as (Sn St)/(Sn St), where Sn is nasally directed and St is temporally directed mean eye speed. Results: Each animal in the 3and 6-month-duration groups showed asymmetries of pursuit at both target speeds. The longer the duration, the more severe the asymmetry: for 15 deg/s targets, the NBI in the 6-month group was 5 greater than that in the 3-month group, and 30 X greater than that in the 3-week group (ANOVA, P 0.03). For 30 deg/s target motion, the NBI in the 6-month-duration group was 4 greater than that in the 3-month group, and 12 greater than that in the 3-week group (ANOVA, P 0.05). Controls had symmetric pursuit. Conclusions: Early correction of strabismus (ie, elimination of binocular decorrelation) prevents maldevelopment of the pursuit system. These findings indicate that early correction of infantile strabismus is beneficial for brain development.

Idiopathic trochleitis is a cause of superimposed ocular pain in patients with migraine. Trochleitis usually presents as an orbital pain without obvious ocular signs. Like greater occipital neuralgia, trochleitis may sustain or trigger the pain of chronic migraine. Diagnosis is confirmed by peritrochlear steroid injection, which produces a quick relief of periocular symptoms and may improve headache control.

Thirteen patients complained of recent fluctuating aching of one orbit, punctuated by stabbing pains. All had exquisite point tenderness over the trochlea and in half of the patients the pain was aggravated by eye movement. Standardized A-scan echography demonstrated swelling of the peritrochlear tissue and thickening of the superior oblique muscle with low internal acoustic reflectivity, typical of myositis. CT scan showed a soft tissue density in the region of the trochlea. Biopsy, performed on two patients, revealed peri-trochlear inflammation. In all patients the symptoms resolved within a period of weeks or months: indomethacin or naproxen were not effective, but oral or locally injected corticosteroids shortened the course compared to no treatment. None of the patients had ptosis, proptosis, Brown's syndrome, or a click, nor did they have echographic or radiographic signs of sinusitis or inflammation away from the trochlea. This probably represents a highly localized subtype of idiopathic orbital inflammation ("pseudotumor").

• To determine if infantile (congenital) strabismus is associated with a defect of ocular pursuit or vestibulo-ocular reflexes, eye movements were recorded in 14 subjects. Subjects with infantile strabismus had an impairment of temporally directed pursuit when monocularly viewing a moving target. They were unable to normally enhance or cancel the vestibulo-ocular reflex using visual tracking. Vestibulo-ocular reflexes were normal during rotation at 0.3 Hz in the dark. The pursuit defect was not found in subjects with noninfantile strabismus. We suggest that patients with infantile strabismus have a defect of cerebral pursuit control.

To determine the effect of catecholamine depletion on ocular motor pathways in humans, we studied the eye movements of 3 normal subjects who received the drug metyrosine (alpha-methylparatyrosine). This drug temporarily depleted dopamine and norepinephrine, as measured by a reduction in the metabolite, 3-methoxy-4-hydroxy-phenylethyleneglycol (MHPG). Saccadic, pursuit, and vestibulo-ocular eye movements were recorded using infrared oculography with subjects both on placebo and on metyrosine. The most consistent effect observed with metyrosine was an increase in the amplitude and frequency of saccadic intrusions during fixation and pursuit. Two of the 3 subjects also had shortened time constants for the vestibulo-ocular reflex, attributable in part to the sedative effect of catecholamine depletion. The increase in saccadic intrusions implies that catecholamines modulate the activity of a subpopulation of suppressor motor neurons in the human brainstem.

• The evidence for cherry-red maculas in Gaucher's disease is questionable, but in occasional patients, scattered white spots in and on the retina appear to be characteristic. These white spots consist of clusters of swollen histiocytes (Gaucher cells).

Pigeons worked individually in a chamber containing a response key and a mirror. Responding on the key was controlled by a multiple schedule in which a brief period of continuous food reinforcement alternated with a 5 min period of extinction. Under baseline conditions, aggressive behavior (responding on the mirror) occurred at the onset of each extinction period. After 10 saline control sessions, 5 mg/kg of chlordiazepoxide was injected IM 30 min presession for 60 daily sessions. The drug initially produced a marked decrease in aggressive behavior but had little or no effect on key pecking. The aggressive behavior generally remained suppressed during the chronic drug regimen and returned to control levels when the drug was withdrawn. It was concluded that the technique of extinction-induced mirror responding in pigeons provides a stable, sensitive and recoverable baseline for objectively assessing selective drug effects on aggression.

• A 65-year-old man was unable to generate normal horizontal saccadic eye movements. Smooth pursuit of sinusoidal target motion was normal. The saccadic palsy resolved rapidly, twice, after treatment with intravenous corticosteroids. Computed tomography showed a lesion in the pons, and seven months later he was found to have metastatic adenocarcinoma.

• Neurologic evaluation of a child with a severe headache revealed a bilateral inability to move the eyes above the horizontal plane. Computed tomographic and magnetic resonance scans of the brain obtained for a suspected midbrain lesion were normal. Photographs from early childhood confirmed that the deficit was congenital.

To the Editor. —In his interesting article, Lessell 1 found that a disproportionate number of esotropes had anomalous handedness compared with a control population. Anomalous handedness presumably reflects anomalous cerebral development, which led Lessell to comment that esotropic strabismus might be the result of maldevelopment of the visual cortex. Recent work from the University of California, San Francisco, and the Smith-Kettlewell Eye Research Foundation, San Francisco, provides physiologic support for this notion. Humans who develop esotropia in infancy have been found to have striking inversions and topographic distortions of motion processing in the visual cortex. 2 The maldevelopment appears to involve both the primary visual area and the human homologue of monkey visual area MT in extrastriate cortex. 3 Several lines of evidence argue that the cortical maldevelopment causes the ocular motor signs. 2

• Magnetic resonance images were obtained from two patients with isolated congenital hemianopia. Computed tomographic investigations failed to show occipital abnormalities commensurate with the field defects. Magnetic resonance imaging identified dysplasia imaging of the striate cortex and underlying white matter.