Pediatric meningoencephaloceles and nasal obstruction: A case for endoscopic repair

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Summary

Objectives

Congenital anterior skull base defects with meningoencephaloceles causing nasal obstruction are rare clinical entities. Nasal obstruction in children may also be a symptom of multiple benign nasal and allergic disorders, making the initial diagnosis of meningoencephalocele difficult. Traditionally, skull base defects have been repaired via a bifrontal craniotomy approach. With the advent of pediatric endoscopic instrumentation, more of these lesions are accessible via an intranasal endoscopic approach, even in the infant population. However, owing to the rarity of these lesions, there is a paucity of data demonstrating successful adaptation of endoscopic skull base techniques to the pediatric population.

Methods

Retrospective review of two cases of endoscopic repair of anterior skull base defects with meningoencephaloceles at a tertiary care medical center.

Results

Two children, ages 15 months and 6 years, underwent successful endoscopic closure of their anterior skull base defects and resection of their intranasal meningoencephalocele with resolution of their nasal obstruction and cerebrospinal fluid rhinorrhea.

Conclusions

Pediatric nasal meningoencephaloceles with anterior skull base defects can be successfully repaired via a transnasal endoscopic technique, thus minimizing the complications associated with craniotomy and frontal lobe retraction. Triplanar computed tomographic and magnetic resonance imaging is paramount to evaluate the caliber of the skull base defect, consistency of the herniated intracranial contents, as well as the presence of cerebral vasculature.

Introduction

Congenital anterior skull base defects are a rare clinical entity. Traditionally, herniation of the intracranial contents into the nasal cavity has been repaired via a bicoronal approach with frontal craniotomy and pericranial flap reconstruction of the skull base. Though very successful, this approach necessitates an extended hospitalization and risks scarring, anosmia, cerebral edema, intracranial hemorrhage, and other complications associated with frontal lobe retraction [1]. However, with the recent advances in pediatric endoscopic sinus instrumentation and the availability of thin-cut triplanar computed tomography scans, more authors have successfully demonstrated endoscopic closure of skull base defects [2], [3], [4], [5], [6].

Dermoids, encephaloceles, and gliomas are included in the differential diagnosis of a pediatric midline nasal mass. When the mass extends into the nasal cavity it can often be mistaken for a benign nasal polyp. Less commonly in the pediatric population, a neoplastic lesion may be considered. Though not restricted to the midline, a hemangioma is also included in the differential diagnosis of a pediatric nasal mass. The difficulty in treating these lesions, when they are discovered in the nasal cavity, lies in the ability to adequately address the potential for intracranial extension.

In this article we present our experience with the diagnosis and management of two skull base defects, associated with congenital nasal meningoencephaloceles, which were closed via an endoscopic approach. To our knowledge, this also represents the youngest patient (15 months) with a meningoencephalocele closed via this approach that has been reported in the literature to date. This underscores the important role of the otorhinolaryngologist in the management of these patients at a young age.

Section snippets

Case 1

A 15-month-old female, former 27-week premature infant, presented to the neurosurgery service with history of a chronic, right-sided, clear rhinorrhea and pneumococcal meningitis at age of 13 months, in addition to sleep disordered breathing and chronic mouth breathing. Her past medical history was significant for a grade IV intraventricular hemorrhage, extending into the right frontal lobe. A right frontoethmoid encephalocele and cribriform plate defect was diagnosed by CT scan of the

Discussion

Most examples of endoscopic repair of skull base defects in the literature are focused on the adult population [7], [8], [9], [10]. There is a paucity of data demonstrating successful outcomes in pediatric patients [2], [3], [4], [5], [6]. Given the inherent difficulty in the diagnosis of these lesions coupled with their very rare occurrence, endoscopic management represents a unique challenge to the pediatric rhinologist.

The incidence of congenital nasal masses is estimated at one in every

Conclusion

Congenital meningoencephaloceles and skull base defects are rare clinical entities. While these lesions have traditionally been repaired via a bifrontal craniotomy approach, more recent advances in endoscopic equipment and tri-planar imaging have enhanced endoscopic approaches to the skull base. However, a paucity of data exists in the literature applying these techniques to a pediatric population.

Our cases illustrate the importance of early diagnosis and endoscopic management with layered

References (17)

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