Idiopathic intracranial hypertension in pediatric and adolescent patients

Abstract

Objectives Pediatric idiopathic intracranial hypertension is a rare condition, but inappropriate diagnosis and management may lead to devastating outcome with loss of vision and lifelong handicap. Dandy criteria are used for diagnosis of idiopathic intracranial hypertension (IIH) in adult, but these criteria cannot be applied in all pediatric patients. The aim of this study is to evaluate the diagnostic criteria and outcome of management of IIH in pediatric patients. Methods Nineteen patients with IIH and age less than 16 years old have been evaluated for the diagnostic criteria and outcome of management. The patients were classified according to the secondary sexual criteria into adolescent IIH and pediatric IIH. Full neurological and ophthalmological evaluation was completed in all patients. The patients were managed and followed up for a mean period of 12 +/- 8.6 months at Zagazig University hospitals or Kingdom Hospital in the time period from 2009 to 2018. Results Nineteen patients (16 females and 3 males) had been diagnosed and treated with idiopathic intracranial hypertension, their age is between 4 and 15 years, and the patients have been divided into two groups. Group I (pediatric IIH) involved 9 patients (6 females and 3 males), and their age is ranging between 4 and 9 years with mean age 5.56 +/- 1.9 years while group II (adolescent IIH) involved 10 patients, all of them are females and their age ranges between 12 and 15 years with mean age 13.5 +/- 1.3. Diagnostic criteria of the patients are papilledema, symptoms and signs of intracranial hypertension, and elevated CSF opening pressure with normal MRI. Headache, delayed school performance, and sixth nerve palsy are the most common clinical finding in the patients of group I while headache is the most common presenting symptom in group II. Anemia and otitis media are the most common associated risk factors in group I while obesity and female gender were the most common associated risk factors in group II. The minimum value for opening pressure in group I is 180 mmH(2)O while the minimum value in group II is 250 mm. 16 patients improved after the first lumbar puncture and drainage of CSF; two patients improved after repeated lumbar puncture while lumbo-peritoneal shunt was inserted for two patients, both of them were in group II. Recurrence was diagnosed in four patients (21%), one of them (11.1%) was in group I while three of them (30%) were in group II. Conclusion Pediatric idiopathic intracranial hypertension can be classified into two subtypes: pediatric type and adolescent type according to the secondary sexual criteria. Modified Dandy criteria can be applied for the diagnosis of adolescent type, but pediatric type is not associated with obesity, has no female predominance, and usually responds to the initial lumbar puncture with a low rate of recurrence.