Paul H. Phillips

Fourth cranial nerve palsy in pediatric patients with pseudotumor cerebri

PURPOSE To describe three children with acute fourth cranial nerve palsy secondary to pseudotumor cerebri. METHODS We reviewed the medical records of children younger than 18 years who were diagnosed with pseudotumor cerebri between 1977 and 1997. Pseudotumor cerebri was defined by normal neuro-imaging, elevated intracranial pressure measured by lumbar puncture, and normal cerebrospinal fluid composition. RESULTS Three children with pseudotumor cerebri presented with vertical diplopia and clinical signs of fourth cranial nerve palsy including a hypertropia of the affected eye, which increased with adduction and ipsilateral head tilt. The fourth cranial nerve palsy resolved after reduction of the intracranial pressure in all three children. CONCLUSIONS Fourth cranial nerve palsy may occur in children with pseudotumor cerebri and may be a nonspecific sign of elevated intracranial pressure.

Sensitivity of papilledema as a sign of shunt failure in children

PURPOSE Papilledema is considered one of the cardinal ophthalmologic signs of shunt failure. However, the prevalence of papilledema in children with shunt malfunction has not been systematically investigated. The purpose of this study is to determine the sensitivity of papilledema as a sign of shunt failure in children. METHODS A prospective evaluation was undertaken of all children (n = 29; <19 years of age) with surgically confirmed shunt malfunction who were treated at Arkansas Childrens Hospital between July 2007 and April 2008. Each child had a dilated fundus examination and/or RetCam photograph while under anesthesia immediately before shunt repair. The optic disk was evaluated for the presence of papilledema and pallor. Intracranial pressure was measured during surgical shunt repair at the discretion of the neurosurgeon. RESULTS Twenty-nine patients had surgically confirmed shunt failure. Patients age ranged from 36 days to 18 years, 8 months. Four patients (14%) had papilledema, and 1 patient had severe optic disk pallor. The remaining 24 patients had flat optic disks. Five patients had flat optic disks despite an intracranial pressure >/=300 mm H(2)O. CONCLUSIONS Papilledema is not a sensitive sign of shunt failure. Even children with severe elevations in intracranial pressure from shunt malfunction may have flat optic disks. Therefore, physicians that evaluate children with shunts should be aware that a normal optic disk does not preclude shunt malfunction.

Painless orbital apex syndrome from mucormycosis

A 66-year-old woman with a history of non-insulin-dependent diabetes mellitus, hypertension, and hypothy-roidism presented with a painless orbital apex syndrome without any sign of orbital cellulitis or acute systemic disease. Her blood glucose was mildly elevated, but there was no diabetic ketoacidosis. Neuroimaging revealed only mild sinus disease. Transnasal sphenoidal mucosal biopsy showed an inflammatory mass with cellular atypia on frozen sections, suggesting squamous cell carcinoma. However, review of the permanent sections showed broad, nonseptate hyphae consistent with mucormycosis. The patient was treated with a 3-month course of intravenous amphotericin B and no further surgery. Examination 3 months after presentation revealed complete resolution of her ocular motility deficits and partial resolution of her optic neuropathy. Mucormycosis should be suspected in any case of orbital apex syndrome, especially in the diabetic patient.

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.

Congenital ocular motor apraxia with autosomal dominant inheritance

PURPOSE To document congenital ocular motor apraxia in five first-degree relatives. METHODS Case series. Five family members with a history of horizontal head thrusting had neuro-ophthalmologic evaluation. Magnetic resonance imaging of the brain was obtained in the proband. RESULTS Four siblings (one boy and three girls) had congenital ocular motor apraxia. The father had head thrusting as a child and displayed residual signs of the disorder. Magnetic resonance imaging disclosed no central nervous system abnormalities in the father. CONCLUSION Congenital ocular motor apraxia can be inherited as an autosomal dominant trait.

Retinal hemorrhages in a patient with dyskeratosis congenita

Dyskeratosis congenita, also referred to as Zinsser-Cole-Engman syndrome, is characterized by reticular skin pigmentation, dystrophic nail changes, and mucosal leukoplakia. Other findings include carious teeth, premature graying of hair, and osteoporosis. Approximately 50% of patients develop pancytopenia. Ophthalmologic manifestations include blepharitis, conjunctivitis, obliteration of the lacrimal puncta, nasolacrimal duct obstruction, ectropion, and loss of eyelashes. We report bilateral retinal hemorrhages from pancytopenia in a patient with dyskeratosis congenita.

Cavernous sinus syndrome from an internal carotid artery aneurysm in an infant with tuberous sclerosis.

Aneurysms are associated with tuberous sclerosis complex. We describe the first case of cavernous sinus syndrome from an intracavernous internal carotid artery aneurysm in a 9-month-old boy with tuberous sclerosis. The presence of an intracranial aneurysm should be considered in the differential diagnosis of children with tuberous sclerosis who develop cranial nerve deficits.

Neuro-ophthalmologic manifestations of adenoid cystic carcinoma

Intracranial adenoid cystic carcinoma is uncommon. We report two unusual cases of intracranial adenoid cystic carcinoma. The first patient presented with a steroid-responsive optic neuropathy from an orbital mass that simulated orbital pseudotumor, and subsequently developed intracranial involvement, presumably by contiguous perineural spread. The second patient presented with proptosis of the left eye, right facial weakness and numbness, and an intracranial mass, presumably from hematogenous metastatic spread.

Visual recovery after direct traumatic optic neuropathy.

Direct traumatic optic neuropathy is a rare complication of endoscopic sinus surgery and can result in irreversible damage to the optic nerve. We report a case of direct traumatic optic neuropathy after transnasal endoscopic orbital decompression for Gravess disease in a patient who experienced near-complete recovery of vision. We discuss possible mechanisms.

Strabismus surgery in the treatment of paralytic strabismus.

Strabismus surgery may restore limited binocular function and improve cosmesis in patients with paralytic strabismus. Evaluation of the amount of residual function of the affected extraocular muscles is essential to determine which surgical procedure will yield the best results. Standard muscle resection techniques are effective for patients who have paretic extraocular muscles with residual function. Muscle transposition procedures are indicated for patients with total paralysis of an extraocular muscle. Autogenous and alloplastic materials may be required to fix the eye in primary position in patients with total paralysis of multiple extraocular muscles. This article discusses the recent literature regarding the evaluation and management of patients with paralytic strabismus. Current concepts regarding extraocular muscle transposition and the use of autogenous materials are emphasized.