Infantile idiopathic intracranial hypertension: case report and review of the literature

Abstract

Background Idiopathic intracranial hypertension is an infrequent condition of childhood, and is extremely rare in infants, with only 26 cases described. The etiology is still unknown. Typical clinical manifestations change with age, and symptoms are atypical in infants, thus the diagnosis could be late. This is based on increased opening pressure at lumbar puncture, papilloedema and normal cerebral MRI. The measurement of cerebrospinal fluid opening pressure in infants is an issue because many factors may affect it, and data about normal values are scanty. The mainstay of treatment is acetazolamide, which allows to relieve symptoms and to avoid permanent visual loss if promptly administered. Case presentation We report the case of an 8-month-old infant admitted because of vomit, loss of appetite and irritability; later, also bulging anterior fontanel was observed. Cerebral MRI and cerebrospinal fluid analysis resulted negative and after two lumbar punctures he experienced initial symptom relief. Once the diagnosis of idiopathic intracranial hypertension was made, he received oral acetazolamide, and corticosteroids, with progressive symptom resolution. Conclusions Infantile idiopathic intracranial hypertension is extremely rare, and not well described yet. Bulging anterior fontanel in otherwise healthy infants with normal neuroimaging should be always considered suggestive, but can be a late sign, while irritability and anorexia, especially if associated with vomiting, may represent an early sign. In such cases, lumbar puncture should be always done, hopefully with cerebrospinal fluid opening pressure measurement, which is among coded diagnostic criteria, but whose threshold is controversial in infants. Early diagnosis, timely treatment and strict follow-up help to prevent vision loss or death of affected infants.

Optic nerve sheath fenestration for salvaging acutely threatened vision in Idiopathic Intracranial Hypertension - A two-year completed follow up

“…Idiopathic Intracranial Hypertension was previously known as Benign Intracranial Hypertension 13 but the name was discarded from use because of the significant visual morbidity associated with it. 14 While most symptoms 5 can be controlled with weight loss and medical management with acetazolamide 6 , acutely threatened vision and progressive visual loss despite maximum medical therapy needs surgical intervention. 8 Ventriculoperitoneal shunting or lumbo-peritoneal shunting procedures were associated with 0.5% mortality.…”

“…4 For all practical purposes, the criteria laid down by Dandy; as signs and symptoms of raised intracranial pressure in an otherwise awake and alert patient, in the absence of any neurological signs (bilateral sixth nerve paresis being an exception), in the absence of any anatomical or physiological abnormalities of the CSF formation, circulation, drainage, and ventricular system, with normal magnetic resonance imaging and venography except for signs of raised ICP (empty Sella, stenosis of the transverse sinus, dilated optic nerve sheath and flattening of the posterior pole of the globe) and opening pressure of more than 250 mm Hg on Lumbar Puncture; is still used for diagnosing IIH. 5 Symptoms of IIH range from positional headaches and tinnitus to transient visual obscurations associated with postural change. 6 Papilledema is invariably discovered on examination.…”

Advances for pediatricians in 2022: allergy, anesthesiology, cardiology, dermatology, endocrinology, gastroenterology, genetics, global health, infectious diseases, metabolism, neonatology, neurology, oncology, pulmonology

“…Idiopathic intracranial hypertension is very rare in the pediatric age, and etiology is still largely unknown. Del Monte et al [176] presented a case report of an 8-month old male affected by idiopathic intracranial hypertension, and provide discussion on what literature suggests about optimal management also including the therapeutic strategies in idiopathic intracranial hypertension. The presented case was admitted with vomit, anorexia, irritability and bulging anterior fontanel.…”

Infantile Idiopathic Intracranial Hypertension: Case Report of an 8-Month-Old Child

“…Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a clinical disorder characterized by increased intracranial pressure of unknown reasons. 1,2 It is mostly the disease of young obese women, also seen in pediatric population; however, it is extremely rare in infants. 1,2 A limited number of cases of infantile IIH have been reported in the literature, resulting in a scarcity of diagnostic and treatment guidelines.…”

“…1,2 It is mostly the disease of young obese women, also seen in pediatric population; however, it is extremely rare in infants. 1,2 A limited number of cases of infantile IIH have been reported in the literature, resulting in a scarcity of diagnostic and treatment guidelines. 3,4 In this report, we present the case of an 8-month-old female child admitted to the pediatric emergency department (PED) with IIH, highlighting our approach.…”