Cranial nerve palsies in childhood.

We review ocular motor cranial nerve palsies in childhood and highlight many of the features that differentiate these from their occurrence in adulthood. The clinical characteristics of cranial nerve palsies in childhood are affected by the child's impressive ability to repair and regenerate after injury. Thus, aberrant regeneration is very common after congenital III palsy; Duane syndrome, the result of early repair after congenital VI palsy, is invariably associated with retraction of the globe in adduction related to the innervation of the lateral rectus by the III nerve causing co-contraction in adduction. Clinical features that may be of concern in adulthood may not be relevant in childhood; whereas the presence of mydriasis in III palsy suggests a compressive aetiology in adults, this is not the case in children. However, the frequency of associated CNS abnormalities in III palsy and the risk of tumour in VI palsy can be indications for early neuroimaging depending on presenting features elicited through a careful history and clinical examination. The latter should include the neighbouring cranial nerves. We discuss the impact of our evolving knowledge of congenital cranial dysinnervation syndromes on this field.

Diplopia following cataract surgery: a review of 150 patients.

AIM: To study the motility pattern, underlying mechanism, and management of patients who complained of double vision after cataract surgery. METHODS: A retrospective case note analysis of 150 patients presenting with diplopia after cataract surgery to an orthoptic clinic over a 70-month period. Information was retrieved from orthoptic, ophthalmological, and operating room records. RESULTS: A total of 3% of patients presenting to the orthoptic clinic had diplopia after cataract surgery. We grouped these according to the underlying mechanisms which were: (1) decompensating pre-existing strabismus (34%), (2) extraocular muscle restriction/paresis (25%), (3) refractive (8.5%), (4) concurrent onset of systemic disease (5%), (5) central fusion disruption (5%), and (6) monocular diplopia (2.5%). Twenty per cent of the patients could not be categorised with certainty. After infiltrational anaesthesia, extraocular muscle restriction/paresis was the commonest presentation, while decompensation of preexisting strabismus was commonest with topical anaesthesia.For the 150 patients seen, prisms were the commonest form of treatment prescribed (64%) either in isolation or in combination with other treatment, including surgery (19%). Convergence and divergence insufficiency/paresis patterns were also common. A changing motility pattern was noted in some patients who had early documentation, with increasing comitance over time (spread of comitance). Partial resolution made it difficult to clearly identify the underlying mechanism in patients with late documentation. CONCLUSION: Double vision is a troublesome complication of otherwise successful cataract surgery. The use of topical anaesthesia does not abolish this surgical risk.

Light, dark, and melatonin: emerging evidence for the importance of melatonin in ocular physiology.

Melatonin is a hormone, which is mainly produced by the pineal gland, a vestigial eye. Rather than the rods and cones, it is a newly discovered subgroup of photosensitive retinal ganglion cells, which is responsible for mediating the light-dark cycles, thus regulating melatonin's secretion. One of the correlates of the circadian rhythm of melatonin release is the habitual sleep pattern. Patients with circadian rhythm sleep disorders, including some blind patients with no light-induced suppression of melatonin, benefit from melatonin treatment. Melatonin is synthesized in the retina, lens, ciliary body as well as other parts of the body. In this review, we discuss the physiological roles of melatonin in the eye, as well as the potential therapeutic avenues currently under study.

Argon laser photocoagulation for retinopathy of prematurity: long-term outcome.

PURPOSE: To assess the visual, refractive, ocular structural, and neurodevelopmental outcome in a group of children who were screened, treated with laser photocoagulation for threshold retinopathy of prematurity (ROP), and followed up at our institution. METHODS: The 21 survivors of a cohort of 23 children were recalled at the age of 5 years or more. They underwent a full ophthalmologic examination. A paediatric neurologist and/or developmental paediatrician performed a neurodevelopmental assessment and a neuropsychologist performed psychological testing. RESULTS: A total of 30 eyes (71.4%) had a best-corrected visual acuity (BCVA) of 20/40 or better. In all, 26 (62%) eyes were myopic; the overall mean spherical equivalent was -4.95 D. No eye had Stage 4 findings and only one eye (2.4%) progressed to stage 5 despite photocoagulation. In total, 14 patients (66.6%) showed evidence of stereopsis. Strabismus was seen in six patients (28.5%) and nystagmus in three (14.3%). Periventricular leukomalacia (PVL) was identified in five patients (23.8%), cerebral palsy (CP) in seven (33.3%), and intraventricular haemorrhage (IVH) in 13 (62%). Cerebral palsy and IVH associated strongly with visual acuity of less than 20/40 (P=0.009 and 0.047). There was also a strong association between visual acuity of 20/40 or above and a better cognitive outcome (P=0.013). CONCLUSIONS: Retinal scarring and detachment are relatively rare causes of visual morbidity 5 years or more after laser treatment for threshold ROP, but the neurological sequelae of extreme prematurity and amblyopia remain important causes of impaired visual function.

Leopard spot retinal pigmentation in infancy indicating a peroxisomal disorder.

BACKGROUND: Neonatal adrenoleucodystrophy (NALD) is a rare disorder resulting from abnormal peroxisomal biogenesis. Affected patients present in infancy with developmental delay, hypotonia, and seizures. Blindness and nystagmus are prominent features. The authors suggest a characteristic leopard spot pigmentary pattern in the peripheral retina to be diagnostic. METHODS: Three patients are reported with this presentation; the characteristic retinal appearance resulted in early diagnosis for one of these. CONCLUSION: Leopard spot retinopathy in an infant with hypotonia, seizures, developmental delay, with or without dysmorphic features and hearing impairment, is a clue to the diagnosis of NALD.

Visual impairment due to a dyskinetic eye movement disorder in children with dyskinetic cerebral palsy.

Neurological lesions that cause dyskinetic cerebral palsy (CP) commonly involve ocular movements. This report describes a group of 14 children (nine males, five females) whose CP is associated with severe dyskinetic eye movements. Ages ranged from 4 months to 13 years (mean 6.9 years). Clinical features of this eye movement disorder are discussed and defined. The visual function of these children is slow, variable, and highly inefficient. They are often misdiagnosed as blind, due to cortical visual impairment. Early recognition of dyskinetic eye moment disorder and appropriate developmental and educational management are important.

Cortical visual impairment in children with infantile spasms.

BACKGROUND: Infantile spasms are often associated with cortical visual impairment. METHOD: We report a series of 10 patients with infantile spasms, hypsarrhythmic electroencephalograms, and developmental delay, (ie, West syndrome), who presented with severe visual inattention despite a normal ocular examination. RESULTS: At follow-up (14 months to 6 years), 5 patients (50%) had no improvement in their visual behaviour. Although some degree of improvement was observed in the others, their visual function remained abnormal. All patients had moderate or severe mental retardation. CONCLUSIONS: The clinical features of West syndrome are reviewed, and the cause of the associated cortical visual impairment is discussed. Cortical visual impairment with infantile spasms is an important association, which pediatric ophthalmologists should recognize.

Neurologic abnormalities associated with persistent hyperplastic primary vitreous.

BACKGROUND: Persistent hyperplastic primary vitreous (PHPV) is usually a unilateral ocular abnormality not associated with other systemic findings. We describe 16 patients, 7 with unilateral and 9 with bilateral PHPV, in whom significant neurologic abnormalities were identified. METHODS: The clinical records of 16 children referred to the Visually Impaired Program at a university-affiliated children's hospital in Vancouver were studied from 1970 to 1996. Further investigations, including electroencephalography, computed tomography and pediatric neurologic evaluation, were performed to ascertain neurologic deficits. RESULTS: Neurologic abnormalities were identified in three of the children with unilateral PHPV (hemiparesis, impaired coordination and homonymous hemianopia) and seven of the children with bilateral PHPV (ataxia, impaired coordination, hypotonia, spastic quadriplegia, microcephaly and deafness). INTERPRETATION: The possibility of underlying neurologic abnormality should be considered in patients with PHPV, particularly those with bilateral disease. Referral to a pediatric neurologist may be appropriate.

Eyelid pruritus with intravenous morphine.

The cause of eyelid pruritus (itching) may be difficult to determine, particularly in preverbal children. Tearing, photophobia, or eye rubbing may be the first manifestation of leukemic infiltration or graft-versus-host disease, necessitating early appropriate therapy. We report a child in whom persistent eye rubbing developed after bone marrow transplantation for leukemia. Morphine administration was found to be the cause of this symptom.

Acute acquired comitant esotropia: a prospective study.

PURPOSE: To define the clinical characteristics of patients presenting with acute onset esotropia and features suggestive of possible underlying central nervous system pathology. To assess the prognosis for the return of binocular function and to consider the most appropriate management. METHODS: A prospective clinical study was carried out of all patients presenting to the department of paediatric ophthalmology at a university teaching hospital over the period January 1994 to April 1997. Each patient underwent a full ophthalmological examination (including assessment of sensory status). All patients were referred to a paediatric neurologist for examination and CT and/or MRI scan. RESULTS: Ten patients presented during the study period. Uncorrected hypermetropia and/or decompensated monofixation syndrome were the commonest aetiological factors. One patient was found to have a cerebellar tumour. In 5 patients prescription of the full hypermetropic correction alone was sufficient to restore binocularity. Five patients required bilateral medial rectus recession. Binocular function was restored in all cases--in 5 cases with bifoveal fusion. CONCLUSION: Decompensation of a pre-existing phoria or monofixation syndrome appears the commonest aetiology. Prescription of the full hypermetropic correction found at cycloplegic refraction forms an essential part of initial management. No single clinical sign can reliably indicate the rare patient harbouring a tumour. A high index of suspicion should be maintained and neuro-imaging considered in the absence of expected findings such as hypermetropia or fusion potential or in the presence of atypical features or neurological signs.

Congenital endophthalmitis following maternal shellfish ingestion.

PURPOSE: To highlight an unusual organism causing a unilateral endophthalmitis by transplacental spread. METHOD: We report a case of Plesiomonas shigelloides endophthalmitis, presenting in a newborn, with co-existing septicaemia and meningitis. There was a significant maternal history of diarrhoea associated with the ingestion of oysters 2 weeks prior to delivery. RESULT: The endophthalmitis was treated with parenteral antibiotics and topical mydriatics with complete resolution, although subsequent assessment of the affected eye suggests a poor visual outcome. CONCLUSION: Endophthalmitis in the newborn is an unusual clinical finding and usually presents with other manifestations of bacteraemia. Plesiomonas shigelloides is fortunately an infrequent cause of neonatal infection, but is associated with a high degree of morbidity and mortality. We postulate that this neonate acquired P. shigelloides via the transplacental route, and suggest that this organism be included in the list of 'other' causes of transplacental infection that has been abbreviated to 'O' in the acronym 'TORCH'.

Anterior segment dysgenesis and congenital glaucoma associated with partial trisomy of chromosome 1 (1q32-qter).

A child born with partial trisomy of chromosome 1 (1q32-qter) survived and was seen for anterior segment dysgenesis and congenital glaucoma. Pure trisomy 1q is rarely seen in live-born infants and has not previously been described in association with congenital glaucoma. The genetic basis for glaucoma is complicated and multifactorial and probably determined by a number of genes on a variety of chromosomes. The current case provides some evidence that part of chromosome 1 may be involved with the etiology of a glaucomatous process.

Simple choristoma of the anterior segment containing brain tissue.

We report an unusual case of a simple choristoma of the anterior segment that contained only brain tissue. The clinical characteristics and findings of pathological examination of this unusual ocular malformation were reviewed. A newborn girl was seen with a fleshy, highly vascular cystic mass arising from the inferior limbus and extending across the cornea. On a computed tomographic scan, gross disruption of the anterior segment was present, with subluxation of the lens into the cyst. Excision of the abnormal tissue was followed by evisceration; polyglactin (Vicryl) ball implantation; patch graft of the globe; and, later, prosthetic fitting. Pathologic findings showed a choristomatous malformation, containing only mature brain tissue. To our knowledge, a choristoma in which the sole constituent is brain tissue has not previously been reported.