Management of idiopathic intracranial hypertension in an infant with bilateral congenital cataract and associated comitant sensory esotropia

Matalia, Jyoti; Shirke, Sheetal; Anaspure, Hemant; Ghalla, Pooja; Kekatpure, Minal

doi:10.4103/ijo.ijo_345_17

Cited by 3 publications

(1 citation statement)

References 8 publications

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“…3 Till date, 25 infants with idiopathic intracranial hypertension have been reported; however, only 5 of those infants met idiopathic intracranial hypertension diagnostic criteria. 9,10…”

Section: Discussionmentioning

confidence: 99%

Infantile Idiopathic Intracranial Hypertension: A Case Study and Review of the Literature

et al. 2019

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Idiopathic intracranial hypertension, or pseudotumor cerebri, is an increase in cerebrospinal fluid pressure of unknown etiology. It is mostly seen in adults, less frequently in adolescents, rarely in younger children. Only 5 infants meeting idiopathic intracranial hypertension criteria have been mentioned in the literature. We report a case of a previously healthy 9-month-old boy who presented with irritability, decreased appetite, and a bulging fontanelle. Computed tomography (CT) head imaging and cerebrospinal fluid studies revealed normal results. The patient’s symptoms transiently resolved after the initial lumbar puncture, but 11 days later, his fontanelle bulged again. A second lumbar puncture revealed an elevated opening pressure of 35 cmH2O and led to a diagnosis of idiopathic intracranial hypertension in accordance with the modified Dandy Criteria. Treatment with acetazolamide at a dose of 25 mg/kg/d was initiated and the patient remained symptom-free for 6 weeks, followed by another relapse. His acetazolamide dose was increased to 37 mg/kg/d, with no further relapses to date. A diagnosis of idiopathic intracranial hypertension is challenging in infants, because the patients cannot yet verbalize typical idiopathic intracranial hypertension–related symptoms such as positional headaches, diplopia, or pulsatile tinnitus. Furthermore, it is more difficult to assess papilledema in that age group. If undetected and untreated, idiopathic intracranial hypertension may result in permanent visual deficits. Little is known about idiopathic intracranial hypertension in infants, and age-specific treatment guidelines are lacking. We discuss this rare case of infantile idiopathic intracranial hypertension and provide a review of the literature, including an overview of disease characteristics and outcomes of idiopathic intracranial hypertension in this very young age group.

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“…3 Till date, 25 infants with idiopathic intracranial hypertension have been reported; however, only 5 of those infants met idiopathic intracranial hypertension diagnostic criteria. 9,10…”

Section: Discussionmentioning

confidence: 99%

Infantile Idiopathic Intracranial Hypertension: A Case Study and Review of the Literature

et al. 2019

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Inaccuracy of idiopathic intracranial hypertension diagnosis in case reports

Eshtiaghi

Margolin

Micieli

2023

Eye

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

et al. 2022

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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.

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Management of idiopathic intracranial hypertension in an infant with bilateral congenital cataract and associated comitant sensory esotropia

Cited by 3 publications

References 8 publications

Infantile Idiopathic Intracranial Hypertension: A Case Study and Review of the Literature

Infantile Idiopathic Intracranial Hypertension: A Case Study and Review of the Literature

Inaccuracy of idiopathic intracranial hypertension diagnosis in case reports

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

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