Secondary Intracranial Hypertension in Pediatric Lyme Meningitis.

Lyme disease is the most common vector-borne disease in the United States and has been associated with secondary intracranial hypertension. We reviewed 11 pediatric patients with Lyme-associated secondary intracranial hypertension. All patients presented with headache, ten had papilledema, 7 with a rash, and 5 with a cranial nerve palsy. All patients were treated with acetazolamide, and 3 received combination therapy with furosemide. Three patients were considered to have fulminant intracranial hypertension because of the severity in their presenting courses. Two of the fulminant intracranial hypertension patients were treated with a temporary lumbar drain in addition to medications, whereas 1 fulminant intracranial hypertension patient was treated exclusively with medical therapy alone. The addition of a lumbar drain decreased the time to resolution of papilledema compared to medical management alone. Final visual acuity was 20/20 in each eye of all patients, suggesting that a titrated approach to therapy depending on the severity of presentation can result in good visual outcomes in these cases. Additionally, symptoms can recur after medication wean, so patients should be monitored closely with any discontinuation of intracranial pressure lowering medications.

Current Indications for Management Options in Pseudotumor Cerebri.

There are surgical options available for those patients with idiopathic intracranial hypertension (IIH) who have significant visual threat or visual deterioration despite best medical management or whose visual deterioration is rapid enough to warrant urgent intervention. Optic nerve sheath fenestrations, venous sinus stenting, and cerebrospinal fluid diversion via ventriculoperitoneal and lumboperitoneal shunting are useful adjuncts in the management of this condition. Significant resources are used in the care of patients with IIH. Further understanding of the pathophysiology of IIH will likely direct future treatment options to more targeted therapeutics including surgery for IIH in the future.

Intracranial Hypertension Recurrence Risk After Wean of Intracranial Pressure-Lowering Medication.

BACKGROUND: The proportion of children with recurrent signs and symptoms of intracranial hypertension after medication wean has been reported to be between 18% and 50%. Few studies have reported intracranial hypertension recurrence risk in children while adjusting for each individual's observed follow-up time after medication wean. In addition, the role of intracranial hypertension etiology on the risk of disease recurrence has not been widely studied. METHODS: The medical charts of patients with intracranial hypertension treated with intracranial pressure-lowering medication were analyzed retrospectively for disease recurrence. Baseline characteristics from diagnosis were recorded in addition to information regarding duration of therapy, medication wean, and recurrence. Survival analyses as well as Poisson regression models with time under observation as an offset were performed. RESULTS: One hundred and thirty-three patients were included in the study. The cumulative risk of intracranial hypertension recurrence increased rapidly within the first six months after medication wean and was 1.5% at one month, 9.5% at three months, and 20% at six months. This risk leveled off near 12 to 18 months. CONCLUSIONS: While the cumulative risk of intracranial hypertension recurrence increases most dramatically within the first six months after medication wean, it does not appear to taper until 12 to 18 months. Given the possibility of delayed or asymptomatic recurrences, long-term follow-up is ideal, although patients can likely be seen less frequently after the first 12 to 18 months after medication wean.

Equivalence of Oral Fluorescein Angiography to Intravenous Fluorescein Angiography in Evaluating Pediatric Optic Nerve Pathology.

Differentiating true optic nerve edema from pseudo-optic nerve edema is a diagnostic dilemma faced by pediatric ophthalmologists. This case series suggests that oral fluorescein angiography is equivalent to intravenous fluorescein angiography in making this distinction. [J Pediatr Ophthalmol Strabismus. 2019;56:e68-e72.].

Surgical intervention in pediatric intracranial hypertension: incidence, risk factors, and visual outcomes.

PURPOSE: To evaluate the incidence of surgical intervention in pediatric intracranial hypertension (IH), evaluate the visual outcomes of surgically managed patients, and identify potential predictors for surgical intervention. METHODS: The medical records of patients with primary and secondary IH at Nationwide Children's Hospital from 2010 to 2017 were reviewed retrospectively. Presenting characteristics of medically and surgically managed patients were compared, and the clinical courses of surgically managed patients were reviewed. RESULTS: A total of 129 medically and 14 surgically managed patients were included. The surgical incidence was 9.8%. Final visual acuity in 27 of 28 surgically managed eyes was 20/25 or better. In combined primary and secondary IH patients, elevations in body mass index (BMI; OR = 1.06; 95% CI, 1.01-1.11; P = 0.022) and lumbar puncture opening pressures ≥52 cm H2O (OR = 6.17; 95% CI, 1.93-19.67; P = 0.002) were significantly associated with the likelihood of surgical intervention when assessed by univariate logistic regression; grade of papilledema >2 was of marginal significance. After controlling for BMI, a lumbar puncture opening pressure of ≥52 cm H2O was more likely to result in surgery (adjusted OR = 4.69; 95% CI = 1.39-15.98; P = 0.013). CONCLUSIONS: Most pediatric IH can be treated medically. Patients with lumbar puncture opening pressures ≥52 cm H2O at the time of diagnosis are at a higher risk of surgical intervention and should be monitored closely. Elevations in presenting BMI and grade of papilledema may also increase the odds of surgery.

Diagnostic criteria in pediatric intracranial hypertension.

BACKGROUND: The modified Dandy criteria and the newer diagnostic criteria for pseudotumor cerebri syndrome (PTCS) are both used to diagnose intracranial hypertension (IH). In comparison to the modified Dandy criteria, the PTCS criteria stratify the IH diagnosis into definite, probable, and suggested categories, exclude clinical symptoms, and use radiologic evidence for diagnosis. There is a lack of consensus on which criteria should be used in the pediatric population. The purpose of this study was to compare the diagnostic criteria for PTCS to the modified Dandy criteria and to identify limitations within both sets of criteria. METHODS: The PTCS criteria were retrospectively applied to 50 patients originally diagnosed with IH under the modified Dandy criteria. RESULTS: Of the 50 patients, 31 (62%) met diagnostic criteria for definite PTCS, 10 (20%) met criteria for probable PTCS, and 9 patients (18%) failed to meet sufficient PTCS criteria for diagnosis. CONCLUSIONS: Although the PTCS criteria use objective data to make the IH diagnosis, we found subjective symptoms to be useful indicators of disease in this group of patients. Additionally, distinguishing probable from definite IH may not have clinical relevance, because both groups were treated similarly. The absence of radiographic evidence of IH should not preclude a diagnosis of the condition, as it was present in a minority of patients included in this study. Further research is needed to clarify the disease process in patients who present with signs and symptoms of elevated intracranial pressure but lack ocular pathology.