Clinical Characteristics of Idiopathic Intracranial Hypertension in Older Adults.

BACKGROUND: Idiopathic intracranial hypertension (IIH) is a condition that classically affects obese women of child-bearing age. However, it is sometimes encountered in older patients. The purpose of this study was to help clinicians better understand how this disease can present differently in these age groups. METHODS: This is a retrospective chart review from a single academic center of baseline characteristics of adult patients diagnosed with IIH based on the modified Dandy criteria. The patients were divided into 2 groups: (1) those 18-44 years old and (2) those older than 45 years at diagnosis. RESULTS: One hundred sixty-seven patients were identified; 135 in the younger group and 32 in the older group. The younger group had a higher rate of headaches (90% vs 63%, P = 0.0004), higher body mass index (38.9 vs 36.1, P = 0.046), higher opening pressure (38 vs 31 cm H2O, P = 0.005), and thicker peripapillary retinal nerve fiber layer average thickness (right eye 178 vs 131 µm, P = 0.02; left eye 184 vs 136 µm, P = 0.045). The older group had higher rates of empty sella (90% vs 62%, P = 0.0039). In addition in the younger group, there was a trend toward higher rates of pulsatile tinnitus (63% vs 45%, P = 0.08), transient visual obscurations (50% vs 32%, P = 0.07), and lower rates of spontaneous cerebrospinal fluid leak (4% vs 13%, P = 0.08). Sex, rates of obesity, other MRI findings typical of elevated intracranial pressure, frequency and Frisen grading of papilledema, and visual field loss were not statistically different between the groups. CONCLUSIONS: The older age group had milder signs and symptoms of IIH and a higher prevalence of empty sella than the younger group, but otherwise had typical characteristics. These findings suggest that IIH in the older age group may represent milder chronic disease that was previously undiagnosed.

A misleading frozen section in a lacrimal gland pleomorphic adenoma of a nine-year-old.

INTRODUCTION: Benign pleomorphic adenomas of the lacrimal gland usually present as a painless, slow growing mass in healthy adults and rarely present in childhood. This report describes a pediatric patient found to have a lacrimal gland pleomorphic adenoma that mimicked rhabdomyosarcoma on frozen section. REPORT: A nine-year-old African American male presented with two months of unilateral proptosis and was found to have a left orbital mass on computed topography. Through a lateral orbitotomy, the mass was excised, but noted to appear distinct from the lacrimal gland. An intraoperative frozen section demonstrated a myxoid matrix with scattered cells mimicking rhabdomyoblasts. Because this sample lacked additional elements, the diagnosis of rhabdomyosarcoma could not be excluded, especially considering the patient's age, short time course of symptoms, and the tumor's intraoperative appearance. Permanent sections of the whole lesion provided definitive diagnosis. CONCLUSIONS: In the rare pediatric presentations, pleomorphic adenomas of the lacrimal gland can be difficult to quickly and definitively differentiate from a rhabdomyosarcoma. The unusual age of presentation and the misleading frozen section appearance in this case demonstrate the intricacies of managing pediatric orbital tumors and highlight the importance of permanent sections for definitive diagnosis.

Wolfram syndrome with vitelliform retinal deposits.

BACKGROUND: : Wolfram Syndrome is a rare genetic disorder usually inherited in an autosomal recessive manner. The acronym DIDMOAD characterizes the classic constellation of findings: diabetes insipidus, diabetes mellitus, optic atrophy, and deafness. However, other ocular and systemic manifestations may also be present. The aim of this report is to present a patient with Wolfram Syndrome presenting with vitelliform changes in the retina - an association that has not been previously reported. MATERIALS AND METHODS: : Case Report. RESULTS: : Ophthalmologic examination and imaging studies showed bilateral optic neuropathy and scattered bilateral subretinal vitelliform deposits. Genetic testing was positive for Wolfram Syndrome. CONCLUSION: : This patient showed optic atrophy with associated vitelliform retinal changes. The previously unreported association of these findings present possible associations in the molecular pathophysiology underlying both Wolfram syndrome and the spectrum of retinal disorders associated with vitelliform changes.

Pigmented Epithelioid Melanocytoma of the Cheek, Orbit, and Intracranial Cavity: A Case Report.

Pigmented epithelioid melanocytoma (PEM) of the skin has been rarely reported in ophthalmology. The purpose of this case report is to present a young male born with a progressive, hyperpigmented lesion involving the orbit and intracranial cavity diagnosed as PEM. The case is unique given the young age and the size, multifocality, and growth of this tumor. Identification of this lesion is paramount due to its low-grade malignant potential.