Diagnosis and Management of Pediatric Nasal CSF Leaks and Encephaloceles
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Pediatric Dermatology and Allergy (J Lee, Section Editor)
Diagnosis and Management of Pediatric Nasal CSF Leaks and Encephaloceles Cynthia S. Wang, MD Clarice Brown, MD Ron B. Mitchell, MD Gopi Shah, MD, MPH* Address * Department of Otolaryngology Head and Neck Surgery, Division of Pediatric Otolaryngology, University of Texas Southwestern Medical Center and Children’s Health, 2350 N. Stemmons Freeway, F6.211, Dallas, TX, 75207, USA Email: [email protected]
* Springer Nature Switzerland AG 2020
This article is part of the Topical Collection on Pediatric Dermatology and Allergy Keywords Pediatric CSF leak I Encephalocele I Meningoencephalocele I Endoscopic encephalocele repair
Abstract Purpose of review To describe the clinical presentation, diagnosis, and management of pediatric nasal encephaloceles. Recent findings Encephaloceles and meningoencephaloceles are on the differential diagnosis for congenital midline nasal masses. These lesions are treated surgically to prevent craniofacial deformities and meningitis and to address symptoms such as a nasal obstruction, feeding difficulties, and cerebrospinal fluid (CSF) rhinorrhea. With the advent of endoscopic skull base approaches and instruments, surgical treatment of nasal encephaloceles have transitioned from large bicoronal external approaches to more minimally invasive endonasal endoscopic approaches. Endoscopic surgical approaches are safe in pediatric patients. Summary Pediatric intranasal encephaloceles are more frequently managed endoscopically. However, due to the rarity of this condition, further multi-institutional research is needed to examine long-term outcomes for this surgical approach.
Background An encephalocele is a protrusion of brain and meninges through a cranial opening, while a meningocele is a protrusion of the meninges alone [1, 2]. Prevalence of
congenital encephaloceles ranges from 1 in 3000 to 1 in 30,000 live births in North America and Europe. They are more common in Southeast Asian countries, with a
Pediatric Dermatology and Allergy (J Lee, Section Editor) reported incidence of 1 in 6000 live births [3]. They can present as a midline nasal mass in an infant or child as part of a group of congenital midline nasal masses including nasal dermoids and gliomas (nasal cerebral heterotopia). These defects can arise due to an anterior neuropore anomaly during development [4]. Dermoids and epidermoids represent up to 60% of these lesions, and up to 20% of dermoids can contain intracranial tissue [4, 5]. Approximately 30% of gliomas present as intranasal masses [4], and the majority no longer have intracranial connections except for a fibrous stalk in 15%. The differential diagnosis can be broad and includes nasal polyp, papilloma, hemangioma, hairy or teratoid polyp, fibroma, lipoma, lipoblastoma, fibrosarcoma, rhabdomyosarcoma, granulocytic sarcoma, extranodal non-T or T cell lymphoma, schwannoma, mucosal melanoma, squamous cell carcinoma, adenoid cystic carcinoma, and adenocarcinoma [4]. Magnetic resonance imaging (MRI) is t
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