International Journal of Pediatric Otorhinolaryngology
Pediatric meningoencephaloceles and nasal obstruction: A case for endoscopic repair
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)
- et al.
Endoscopic repair of a congenital intranasal encephalocele in a 23 months old infant
Int. J. Pediatr. Otorhinolaryngol.
(2005) - et al.
Three-dimensional computed tomography of congenital nasal anomalies
Int. J. Pediatr. Otorhinolaryngol.
(2002) - et al.
Endoscopic cerebrospinal fluid rhinorrhea repair: is a lumbar drain necessary?
Otolaryngol. Head Neck Surg.
(1999) - et al.
Long-term outcome of facial growth after functional endoscopic sinus surgery
Otolaryngol. Head Neck Surg.
(2002) - et al.
Extracranial repair of cerebrospinal fluid fistulas: technique and results in 37 patients
Neurosurgery
(1990) - et al.
Evolutions in the management of congenital intranasal skull base defects
Arch. Otolaryngol. Head Neck Surg.
(2004) - et al.
Nasal glioma and encephalocele: diagnosis and management
Laryngoscope
(2003) - et al.
Transnasal endoscopic repair of congenital defects of the skull base in children
Arch. Otolaryngol. Head Neck Surg.
(1999)
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Endonasal endoscopic surgery for pediatric anterior cranial fossa encephaloceles: A systematic review
2020, International Journal of Pediatric OtorhinolaryngologyCitation Excerpt :Twenty studies reported on lesion etiology [21–32,34–41], with 84.6% [73.1–93.3%] of cases representing congenital lesions and 16.6% [7.7–28.7%] cases acquired by iatrogenic or traumatic causes. The maximum diameter of the skull base defect ranged from 3 mm to 24 mm across seven studies [22,25,26,29,31,37,40]. Table 1 details the overall study characteristics.
Current management of congenital anterior cranial base encephaloceles
2020, International Journal of Pediatric OtorhinolaryngologyCitation Excerpt :While the paradigm continues to shift towards endoscopic only approaches for pediatric lesions of the anterior skull base, a major concern is the compact anatomy that may preclude endoscopic repair, especially in very young children. However, several studies suggest that endoscopic resection and repair is feasible and has a more favorable complication profile than open approaches, even in children as young as 8 months old [25–31]. Despite data indicating safety and feasibility, many contemporary authors continue to utilize primarily open or combined approaches in pediatric skull base surgery [8,14,15,32].
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2018, Clinics in PerinatologyCitation Excerpt :The goal of the surgery is to remove the entire lesion and close off any potential intracranial communication to prevent complications such as a cerebrospinal leak or meningitis. If the lesion is small and intranasal, an endoscopic technique to remove the lesion and its attachments is possible and is the most reasonable approach.25 Larger lesions that have an intracranial component require a combined approach, with a neurosurgeon performing a craniotomy to resect any intracranial component of the lesion, herniated dura, and neural tissue and close the cerebrospinal fluid leak and an otolaryngologist removing the nasal component of the mass.
Endoscopic endonasal surgery for sinonasal and skull base lesions in the pediatric population
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