Lyme neuroborreliosis: a treatable cause of acute ocular motor disturbances in children.

BACKGROUND: Lyme neuroborreliosis (LNB) designates central nervous system involvement caused by the tick-borne spirochaete Borrelia burgdorferi (Bb). The present study describes a spectrum of acquired ocular motor disorders in children with LNB. METHODS: Six paediatric patients (age 3-15 years) with ocular motor symptoms as first manifestations of LNB evaluated by a paediatrician and ophthalmologist are presented. Diagnosis was based on new onset ocular motor disturbances and detection of cerebrospinal fluid (CSF) pleocytosis and intrathecal synthesis of Bb IgM and/or IgG antibodies by lumbar puncture. The children were evaluated before and after antibiotic treatment with a follow-up time of 1-7 months. Videos were obtained both pre and post treatment in four patients. RESULTS: Two children presented with acquired nystagmus, one with combined nystagmus and partial sixth nerve palsy, one with partial sixth nerve palsy, one with ptosis and one with Adie's pupil. Five of the patients presented with severe fatigue, malaise, nausea, headache and fever. Four had recognised a tick bite recently, and two developed erythema migrans. Intrathecal synthesis of IgM and/or IgG antibodies specific for Bb was positive in all children, and five showed CSF pleocytosis. Cerebral MRI or CT of the brain were normal. Treatment with intravenous or oral antibiotics produced rapid clinical improvement in five of the six children. CONCLUSIONS: LNB can present as acute ocular motor disorders in conjunction with fatigue and other clinical manifestations. In endemic areas, children with unexplained, acquired ocular motor abnormalities should be evaluated for LNB, a treatable medical condition.

When is isolated inferior oblique muscle surgery an appropriate treatment for superior oblique palsy?

PURPOSE: To evaluate the efficacy of isolated inferior oblique muscle weakening in the treatment of superior oblique palsy. METHODS: Forty-seven patients with superior oblique palsy underwent either single-muscle surgery (anteriorization or recession of the inferior oblique muscle) or two-muscle surgery (anteriorization of the inferior oblique muscle combined with recession of the contralateral inferior rectus muscle according to the amount of vertical deviation). In a retrospective noncomparative study the objective surgical effect was calculated as the difference between the deviation at the day before surgery and the deviations 6 weeks and at least 1 year after surgery. Pre- and postoperative sensorimotor status and subjective outcome were evaluated. RESULTS: In patients who underwent isolated inferior oblique muscle surgery the mean preoperative vertical deviation decreased from 15+/-9 (distance)/16+/-10 (near) prism diopters (PD) (anteriorization) and 7+/-5 (distance)/9+/-8 (near) PD (recession) to 4+/-4 (distance)/4+/-6 (near) PD (anteriorization) and 2+/-2 (distance)/2+/-3 (near) PD (recession) at the 1-year follow-up. In patients who underwent two-muscle surgery the mean vertical deviation decreased from 20+/-11 (distance)/21+/-10 (near) PD preoperatively and 6+/-7 (distance)/6+/-6 (near) PD at 1-year follow-up. Subjective assessment showed excellent scores among the patients treated with single-muscle surgery and slightly lower but also favorable scores among the patients treated with combined techniques. A direct comparison of the different outcome scores was not possible because of the more difficult initial situation in patients who underwent combined surgery. CONCLUSIONS: Isolated inferior oblique muscle weakening is an effective treatment option for superior oblique palsy up to 15 PD of vertical deviation in primary position. Two-muscle surgery should be reserved for patients with larger vertical deviations.

Intermittent exotropia increasing with near fixation: a "soft" sign of neurological disease.

AIM: To examine the association of distance-near disparity with neurological disease in children with intermittent exotropia. METHODS: A retrospective analysis was performed of the medical records of all children with intermittent exotropia examined at the Arkansas Children's Hospital between 1989 and 2002. The study group consisted of children with intermittent exotropia who had a near deviation that exceeded the deviation at distance by at least 10 prism dioptres. The control group consisted of children with intermittent exotropia who had a distance deviation greater than or equal to the deviation at near. The main outcome measures were the prevalence of neurological abnormalities in the study and control groups. RESULTS: Among the 29 patients in the study group, 19 (66%) had a history of concurrent neurological abnormalities. Associated neurological conditions included developmental delay (10 patients), attention deficit disorder (four patients), cerebral palsy (four patients), history of intracranial haemorrhage (four patients), periventricular leucomalacia (three patients), seizures (two patients), cortical visual impairment (two patients), hydrocephalus (one patient), history of anoxic brain damage (one patient), history of encephalitis (one patient), and autism (one patient). Among the 37 patients in the control group, seven (19%) had a history of concurrent neurological abnormalities. The difference in the prevalence of neurological disease between the study group and the control group was significant (p = 0.0002). CONCLUSION: Intermittent exotropia increasing with near fixation is associated with neurological disease in children.

Intermittent exotropia increasing with near fixation: a "soft" sign of neurological disease.

AIM: To examine the association of distance-near disparity with neurological disease in children with intermittent exotropia. METHODS: A retrospective analysis was performed of the medical records of all children with intermittent exotropia examined at the Arkansas Children's Hospital between 1989 and 2002. The study group consisted of children with intermittent exotropia who had a near deviation that exceeded the deviation at distance by at least 10 prism dioptres. The control group consisted of children with intermittent exotropia who had a distance deviation greater than or equal to the deviation at near. The main outcome measure was the prevalence of neurological abnormalities in the study and control groups. RESULTS: Among the 29 patients in the study group, 19 (66%) had a history of concurrent neurological abnormalities. Associated neurological conditions included developmental delay (10 patients), attention deficit disorder (four patients), cerebral palsy (four patients), history of intracranial haemorrhage (four patients), periventricular leucomalacia (three patients), seizures (two patients), cortical visual impairment (two patients), hydrocephalus (one patient), history of anoxic brain damage (one patient), history of encephalitis (one patient), and autism (one patient). Among the 37 patients in the control group, seven (19%) had a history of concurrent neurological abnormalities. The difference in the prevalence of neurological disease between the study group and the control group was significant (p=0.0002). CONCLUSION: Intermittent exotropia increasing with near fixation is associated with neurological disease in children.

Unexplained head tilt following surgical treatment of congenital esotropia: a postural manifestation of dissociated vertical divergence.

BACKGROUND: Strabismus surgery for congenital esotropia can be complicated by the development of a postoperative head tilt. PURPOSE: To determine the pathophysiology of acquired head tilting following horizontal realignment of the eyes in children with congenital esotropia. MATERIALS AND METHODS: Retrospective analysis of nine children with congenital esotropia who developed unexplained head tilts following horizontal realignment of the eyes. RESULTS: Shortly after strabismus surgery, each child developed a head tilt in association with asymmetrical dissociated vertical divergence (DVD). Five children maintained a head tilt toward the side of the fixing eye (group 1), which did not serve to control the DVD. Four children maintained a head tilt toward the side of the hyperdeviating eye, which served to control the DVD (group 2). Children in group 2 had earlier horizontal muscle surgery and developed better stereopsis than those in group 1, suggesting that the higher degree of single binocular vision and stereopsis in these children may have led to a compensatory torticollis to control an asymmetrical DVD. CONCLUSIONS: The onset of an unexpected head tilt after congenital esotropia surgery is usually a postural manifestation of asymmetrical DVD. In this setting, a head tilt toward the side of the fixing eye corresponds with a postural manifestation of the underlying central vestibular imbalance that produces DVD, while a head tilt toward the side of the hyperdeviating eye serves to counteract the hyperdeviation and stabilise binocular vision.

Retinal neovascular markers in retinopathy of prematurity: aetiological implications.

AIM: (1) To determine if expression of the blood-tissue barrier associated glucose transporter GLUT1 is preserved by the neovasculature of retinopathy of prematurity (ROP), in contrast with the reported loss of GLUT1 expression in preretinal vessels of proliferative diabetic retinopathy. (2) To compare the vascular immunophenotype of ROP to juvenile haemangioma, another perinatal neovascular disorder that has recently been shown to express placental type vascular antigens, including GLUT1 and Lewis Y antigen. METHODS: A retrospective case report was carried out. Immunoreactivities for GLUT1 and Lewis Y antigen were assessed in a human eye with stage 3 ROP and compared with those in a control (paediatric) eye. The presence or absence of endothelial GLUT1 and Lewis Y immunoreactivity was determined in preretinal and intraretinal vessels. RESULTS: Immunoreactivity was positive for GLUT1 and negative for Lewis Y in the intraretinal and preretinal neovasculature of the ROP affected eye and in the normal retinal vessels of the control eye. CONCLUSIONS: Retention of immunoreactivity for GLUT1 distinguishes ROP from proliferative diabetic retinopathy. Furthermore, absence of Lewis Y antigen co-expression distinguishes ROP from juvenile haemangioma, a perinatal form of GLUT1 positive neovascularisation that has recently been linked to placental vasculature.

Magnetic resonance diagnosis of congenital hypopituitarism in children with optic nerve hypoplasia.

PURPOSE: To determine whether structural abnormalities of the neurohypophysis, as detected by magnetic resonance imaging (MRI), can be used to diagnose hypopituitarism in children with optic nerve hypoplasia. METHODS: Retrospective analysis of 67 children with optic nerve hypoplasia who had MRI and pediatric endocrinologic evaluation at Arkansas Children's Hospital from 1989 to 1999. RESULTS: A total of 26 children with optic nerve hypoplasia had pituitary hormone deficiency. MRI disclosed posterior pituitary ectopia in 16 of these cases, absence of the pituitary infundibulum and posterior pituitary bright spot in 7 of these cases, and a normal neurohypophysis in 3 of these cases. All 41 children with optic nerve hypoplasia and normal endocrinologic function had a normal neurohypophysis. CONCLUSION: MRI of the neurohypophysis can predict when congenital hypopituitarism will be associated with optic nerve hypoplasia.

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.

Accommodative esotropia: an unrecognized cause of hemifacial spasm in children.

Pediatric hemifacial spasm can be a sign of an intracranial tumor. We examined two females, ages 2 and 6 years, who presented with hemifacial spasm as an early sign of accommodative esotropia. Initial ophthalmologic examination disclosed high hyperopia without detectable esotropia. Both children developed an intermittent esotropia over the following month. In the first child, the hemifacial spasm resolved concurrent with the onset of amblyopia. In the second child, spectacle correction produced immediate cessation of the spasms. In both children, squinting to avoid diplopia produced an overflow facial movement that manifested as hemifacial spasm. Accommodative esotropia should be considered in the differential diagnosis of pediatric hemifacial spasm.

Severe penetrating ocular injury from ninja stars in two children.

The authors describe two cases of penetrating ocular trauma in children resulting from ninja stars. In the first case, despite a scleral laceration, loss of iris tissue, and a vitreous hemorrhage, the child had a good result with a final best corrected visual acuity of 20/20. Unfortunately, the child in the second case did not fare as well. In this case, the child suffered a large corneal laceration and traumatic cataract. He ultimately required a penetrating keratoplasty, and he is currently being treated for amblyopia, strabismus, and elevated intraocular pressures. His best corrected visual acuity is 20/70.