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Paediatric giant cavernomas: report of three cases with a review of the literature

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Abstract

Introduction

Cavernous angiomas of the brain (CCM) are being increasingly diagnosed, especially in the paediatric age group. Though classic presentations with haemorrhage or seizures are well recognised, presentation as a large lesion with mass effect is rare and creates difficulty in diagnosis as well as management.

Methods

Our cases of paediatric giant CCMs that presented as a ‘mass lesion’ are reported here, and the PubMed database for giant CCMs in the paediatric population is reviewed. All articles where the size of the lesion was reported to be > 4 cm were selected for analysis to study the varying modes of presentation, treatment, and outcome; to gain a proper perspective on this distinct entity of ‘giant CCMs’.

Results

Analysis of a total of 53 cases (inclusive of our 3 cases) reported so far showed slight male preponderance (58.49%). The largest reported lesion was 14 cm in largest diameter. Most of the lesions (83.02%) occurred in the supratentorial region. In the infratentorial region, paediatric giant CCMs were more commonly seen in the cerebellum than in the brainstem. Seizures were observed in 47.17% at presentation. Features of mass effect were the mode of presentation in all our cases, and literature analysis has shown raised intracranial pressure in 37.74% (20 patients) and focal neurological deficit in 33.96% (18 patients) at presentation. Macrocephaly was seen in younger children up to the age of 7 years (16.98% or 9 patients). Gross total resection was carried out (with a good outcome) in all our cases and in 36 of the other 49 analysed patients who were operated on.

Discussion

About one-fourth of CCMs occur in paediatric patients. Giant CCMs are rare but can present in children even in the immediate post-natal period. Features of a mass lesion such as raised intracranial pressure, macrocephaly, and focal neurological deficit are much more common than their smaller counterparts. Their appearance on imaging also often causes diagnostic dilemmas with other intracranial mass lesions. Timely surgery with standard microsurgical principles leads to a favourable outcome in the majority.

Conclusion

Giant CCMs, though rare, often present as a diagnostic challenge. Presentation with mass effect is common, and complete microsurgical excision remains the mainstay of treatment. Though transient neurological deficits may be encountered with this strategy, the long-term outcome remains favourable.

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Data availability

All data generated or analysed during this study are included in this manuscript.

Abbreviations

CCM:

Cerebral cavernous malformation

MRI:

Magnetic resonance imaging

CT:

Computed tomography

ISSVA:

International Society for the Study of Vascular Anomalies

SWI:

Susceptibility weighted imaging

AVM:

Arteriovenous malformation

GTR:

Gross total resection

STR:

Subtotal resection

NR:

Not reported

CPA:

Cerebellopontine angle

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Authors and Affiliations

  1. Department of Neurosurgery, Bombay Hospital Institute of Medical Sciences, New Marine Lines, Mumbai, Maharashtra, India, 400020

    Krishna Shroff, Chandrashekhar Deopujari, Vikram Karmarkar & Chandan Mohanty

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  1. Krishna Shroff
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  2. Chandrashekhar Deopujari
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  3. Vikram Karmarkar
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  4. Chandan Mohanty
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Correspondence to Chandrashekhar Deopujari.

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As this is a retrospective study and there was no deviation from standard of care that was provided to the patients, ethics approval was not required from the Institutional Ethics Committee.

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Parents of the patients included in the study were informed that their patient’s clinical data and imaging photographs may be used for educational purposes such as presentation in conferences/journals, and consent was obtained. No personal identifying information has been submitted in this manuscript or in Figs. 1, 2, and 3.

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Shroff, K., Deopujari, C., Karmarkar, V. et al. Paediatric giant cavernomas: report of three cases with a review of the literature. Childs Nerv Syst 37, 3835–3845 (2021). https://doi.org/10.1007/s00381-021-05286-6

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