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Predictors of disease course and long-term outcomes of idiopathic intracranial hypertension in children and adolescents

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Abstract

This study aimed to identify predictors for unfavorable disease course and clinical and visual outcomes in pediatric patients with idiopathic intracranial hypertension (IIH). Employing a multi-tiered approach, we retrospectively analyzed clinical, ophthalmic, and neuroimaging data from patients diagnosed with IIH between 2003 and 2021. Of the 97 patients included, 56 (58%) were females. The median age was 12 years [Interquartile range (IQR) 9, 14], and the median follow-up time was 39.0 months (IQR 14.8, 90.9). Forty-two (43%) patients had an unfavorable disease course, 28 (29%) had persistence of headache at last follow-up, and 16 (18%) had a poor visual outcome, most of them with mild visual disturbances. Poor visual outcome was more common in females compared to males [16/47 (34%) vs. 0/39, p < 0.001)]. On multivariate regression analysis, female sex and disease recurrence were significantly associated with poor visual outcomes (OR: 18.5, CI:1.3–270, P = 0.03, and OR: 5.1, CI: 1.2–22.5, P = 0.03, respectively). Patients with persistent headaches exhibited lower incidence of papilledema, lower opening pressure, and fewer neuroimaging markers indicating elevated intracranial pressure.

Conclusions: This study provides insights into predictive factors for an unfavorable disease course, persistent headaches, and poor visual outcomes in patients with childhood IIH. Patients with persistent headaches may have a variant of a chronic pain syndrome warranting a different therapeutic approach.

What is Known:

• Childhood-onset Idiopathic Intracranial hypertension (IIH) is a heterogenous disease. The knowledge on disease trajectory and long-term outcomes and its predictors is limited.

What is New:

• A higher opening pressure and factors suggestive of the metabolic syndrome predict an unfavorable disease course whereas female sex and disease recurrence are significantly associated with poor visual outcomes

• A third of the patients diagnosed with IIH experience ongoing headaches despite achieving favorable visual outcomes. This subset, characterized by lower disease-severity indicators at onset may represent a distinct subgroup warranting a different therapeutic approach

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Abbreviations

BMI:

Body mass index

CSF:

Cerebrospinal fluid

ICP:

Intracranial hypertension

IIH:

Idiopathic intracranial hypertension

LP:

Lumbar puncture

OA:

Optic atrophy

OCT:

Optical coherence tomography

ON:

Optic neuropathy

OP:

Opening pressure

RNFL:

Retinal nerve fiber layer

VF:

Visual field

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Authors and Affiliations

  1. Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel

    Noam Senderowich, Anat Bachar-Zipori, Alexis Mitelpunkt, Itay Tokatly Latzer, Ainat Klein, Daphna Mezad-Koursh, Aviva Fattal-Valevski & Moran Hausman-Kedem

  2. Ophthalmology Division, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel

    Anat Bachar-Zipori, Ainat Klein & Daphna Mezad-Koursh

  3. Pediatric Neurology Institute, Dana-Dwek Children’s Hospital, Tel Aviv Sourasky Medical Center, 6 Weizmann Street, Tel Aviv, 6423906, Israel

    Alexis Mitelpunkt, Itay Tokatly Latzer, Aviva Fattal-Valevski & Moran Hausman-Kedem

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  1. Noam Senderowich
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Contributions

All authors contributed to the study conception and design. Material preparation, data collection and analysis were performed by NS, AB, AM, ITL, and MHK. The first draft of the manuscript was written by NS and MHK and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Moran Hausman-Kedem.

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Ethical approval

The study was approved by the Internal Review Board of the Tel Aviv Sourasky Medical Center. As the case recruitment was conducted retrospectively, obtaining informed consent was deemed unnecessary.

Conflict of interest

The authors have no potential, perceived, or real conflict of interest to disclose. This work was previously presented as an abstract or poster.

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Communicated by Peter de Winter

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Senderowich, N., Bachar-Zipori, A., Mitelpunkt, A. et al. Predictors of disease course and long-term outcomes of idiopathic intracranial hypertension in children and adolescents. Eur J Pediatr 182, 5137–5147 (2023). https://doi.org/10.1007/s00431-023-05173-z

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