Idiopathic intracranial hypertension in a child with Bardet-Biedl syndrome.

BACKGROUND: Bardet-Biedl syndrome (BBS) is known to be associated with hydrocephalus, but not with idiopathic intracranial hypertension (IIH). Case presentation: We describe such a case and propose the pathogenesis. We also discuss the challenges of diagnosis, treatment, and monitoring outcomes in this population that is already at high risk of vision loss from retinal dystrophy. CONCLUSION: IIH can result from a combination of risk factors in conjunction with the underlying dysfunctional cilia in BBS patients. Monitoring disease progression is difficult, and as such IIH may be underdiagnosed or missed. Management must be adjusted to account for BBS patients' impaired metabolic and renal physiology. It is important that clinicians be aware of these challenges in this vulnerable population, and regular monitoring should be done to avoid preventable vision loss.

Magnetic Resonance Imaging Features and Clinical Findings in Pediatric Idiopathic Intracranial Hypertension: A Case-Control Study.

The purpose of this study is to identify salient magnetic resonance imaging (MRI) findings of pediatric IIH, to determine the relevance of these findings with regard to disease pathogenesis, and to relate these findings to the clinical presentation towards identification of risk factors of disease. A retrospective, a case-control study of 38 pediatric patients with and 24 pediatric patients without IIH from the ophthalmology department at a tertiary care center was performed. Clinical data, including ophthalmic findings and lumbar puncture results, were recorded. Neuroimaging, including both MRI and magnetic resonance venography (MRV), was evaluated for perioptic subarachnoid space diameter enlargement, posterior globe flattening, optic nerve head protrusion, empty or partially empty sella turcica, dural venous sinus abnormalities, skull base crowding, and prominent arachnoid granulations. Compared with controls, IIH patients had larger perioptic subarachnoid space diameters, higher incidences of posterior globe flattening, protrusion of the optic nerve heads, an empty sella turcica, and dural venous sinus abnormalities. A perioptic subarachnoid space diameter of ≥5.2 mm was identified as an independent predictor of IIH (p < 0.001) with sensitivity of 87% and specificity of 67%. Several significant MRI findings in pediatric IIH were identified. Using a model that uniquely incorporated clinical and MRI findings at presentation, we provide a framework for risk stratification for the diagnosis of pediatric IIH which may be utilized to facilitate diagnosis. Future prospective work is needed to further validate the model developed in this study.

A review of pediatric idiopathic intracranial hypertension.

It is now well recognized that idiopathic intracranial hypertension (IIH) can occur in children. Relative to the adult population, the demographic features and clinical presentation of IIH as well as the diagnosis and treatment guidelines for children are quite different. Although these differences are well established, specific diagnostic and treatment guidelines for pediatric patients have largely been based on the adult literature. Exciting new evidence is now available to assist the clinician in managing pediatric patients with IIH.

A case of pediatric idiopathic intracranial hypertension presenting with divergence insufficiency.

An 11-year-old female presenting diplopia only at distance was found to have comitant esotropia of 20 prism diopters (PD) at distance and normal alignment at nearer proximity. Other ocular movement, including abduction, was normal and a thorough neurologic examination was also normal. The deviation angle of esotropia was increased to 35 PD in 6 months, and a brain magnetic resonance imaging with venogram at that time demonstrated no intracranial lesion. A lumbar puncture showed increased opening pressure but the cerebrospinal fluid composition was normal. The patient was diagnosed as having idiopathic intracranial hypertension and treated with oral acetazolamide. Three months after treatment, the deviation angle decreased to 10 PD. This is a case report of divergence insufficiency in pediatric idiopathic intracranial hypertension, with an increasing deviation angle of esotropia. Although sixth cranial nerve palsy is a common neurologic manifestation in intracranial hypertension, clinicians should be aware of the possibility of divergence insufficiency. Also, ophthalmoparesis may not be apparent and typical at first presentation, as seen in this case, and therefore ophthalmologists should be aware of this fact, while conducting careful and proper evaluation, follow-up, and intervention.

A Case of Pediatric Idiopathic Intracranial Hypertension Presenting with Divergence Insufficiency

An 11-year-old female presenting diplopia only at distance was found to have comitant esotropia of 20 prism diopters (PD) at distance and normal alignment at nearer proximity. Other ocular movement, including abduction, was normal and a thorough neurologic examination was also normal. The deviation angle of esotropia was increased to 35 PD in 6 months, and a brain magnetic resonance imaging with venogram at that time demonstrated no intracranial lesion. A lumbar puncture showed increased opening pressure but the cerebrospinal fluid composition was normal. The patient was diagnosed as having idiopathic intracranial hypertension and treated with oral acetazolamide. Three months after treatment, the deviation angle decreased to 10 PD. This is a case report of divergence insufficiency in pediatric idiopathic intracranial hypertension, with an increasing deviation angle of esotropia. Although sixth cranial nerve palsy is a common neurologic manifestation in intracranial hypertension, clinicians should be aware of the possibility of divergence insufficiency. Also, ophthalmoparesis may not be apparent and typical at first presentation, as seen in this case, and therefore ophthalmologists should be aware of this fact, while conducting careful and proper evaluation, follow-up, and intervention.

A Case of Bilateral Papilledema and Visual Field Defect in Pediatric Idiopathic Intracranial Hypertension

PURPOSE: To report a case of bilateral papilledema and visual field defect in pediatric idiopathic intracranial hypertension. CASE SUMMARY: The 5-year-old female patient was admitted to the hospital, complaining of headache and vomiting of 3 weeks duration. After admission, she complained of diplopia. The uncorrected visual acuity was 0.3 in the right eye and 0.8 in the left. An alternative prism cover test showed approximately 35 PD esotropia, with a -2 abduction limitation of both eyes. Fundus examination showed bilateral papilledema and peripapillary retinal hemorrhages. No abnormality was found in the MRI and CT, symptoms of headache, vomiting, bilateral papilledema, and esotropia with normal neurologic examination. Therefore, she was diagnosed with pediatric idiopathic intracranial hypertension. In Humphrey visual field test, MD was -14.15 dB in right and -16.58 dB in the left eye. Also, the general sensitivity of visual field decreased. Acetazolamide (Diamox(R)) was given orally for 30 days. Forty-four days after the initial visit, peripapillary retinal hemorrhages and vessel tortuosity decreased. Furthermore, visual acuity improved to 1.0 in the right eye and 0.9 in the left. The esotropia reduced to 5 PD, and MD improved to -4.83 dB in the right eye and -5.24 dB in the left.