Abstract

Introduction: Intracranial dermoid cysts are rare, congenital and, benign lesions. The etiology of these lesions is related to an embryonic defect during neurulation. Case presentation: The present study describes a case of a 3-year-old girl with a giant cerebellar dermoid cyst, which initially manifested as hydrocephalus. Discussion: We discuss its epidemiological characteristics as well as diagnostic and therapeutic management. The combination of high clinical suspicion, anamnesis, thorough physical examination, and adequate interpretation of neuroimaging data is crucial for the early diagnosis and timely therapeutic intervention for such cysts. Conclusion: Surgical approach involving complete lesion resection considerably improves prognosis.

Highlights

  • Intracranial dermoid cysts are rare, congenital and, benign lesions

  • This study describes a rare case of a giant dermoid cysts (DCs) of the posterior fossa in a pediatric patient and provides a review of the related literature

  • The patient underwent brain magnetic resonance imaging (MRI), which revealed a large, expansive cerebellar lesion associated with supratentorial hydrocephalus (Figure 2)

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Summary

Introduction

Intracranial dermoid cysts (DCs) are rare congenital lesions, accounting for less than 0.5% of primary intracranial tumors (1), and they are 4 to 9 times less common than epidermoid cysts (1). DCs are formed by the separation of the neuroectoderm during neurulation between the third and fifth weeks of embryonic development, leading to the sequestration of ectodermal remains (2,3). They usually occur at the midline of the sellar, parasellar, and frontonasal regions (1). Physical examination revealed rotatory nystagmus, right sixth nerve palsy, and occipital dermal sinus at the midline (Figure 1). The patient underwent brain magnetic resonance imaging (MRI), which revealed a large, expansive cerebellar lesion associated with supratentorial hydrocephalus (Figure 2). The patient was discharged with right sixth nerve palsy and a clinical picture compatible with akinetic mutism, which improved completely in 30 days

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