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Transmastoid encephaloceles. A case report
1Los Angeles County-USC Medical Center, Childrens Hospital of Los Angeles 90027.
Insights
Congenital encephalocele is a rare condition where brain tissue protrudes through skull defects. This case highlights a congenital subcutaneous transmastoid encephalocele presenting as a postauricular mass, challenging initial diagnosis.
Area of Science:
- Pediatric Neurosurgery
- Otolaryngology
- Congenital Malformations
Background:
- Encephaloceles are uncommon pediatric abnormalities, typically found in the nasopharynx or nose.
- Mastoid encephaloceles are rare, often linked to surgery, trauma, or infection.
- Congenital encephalocele presenting in the mastoid with a subcutaneous postauricular mass is exceptionally rare.
Observation:
- A case of congenital subcutaneous transmastoid encephalocele in a child is presented.
- The right temporal lobe herniated through congenital defects in the tegmen and mastoid cortex.
- The encephalocele presented as a postauricular subcutaneous mass, initially misdiagnosed as a subperiosteal abscess.
Findings:
- Congenital encephalocele can manifest unusually, mimicking other conditions.
- Preoperative CT scans may not reveal congenital transmastoid encephaloceles.
- Surgical exploration is crucial for accurate diagnosis of such rare conditions.
Implications:
- Highlights the importance of considering rare congenital anomalies in pediatric differential diagnoses.
- Emphasizes the limitations of imaging in diagnosing complex congenital skull base defects.
- Informs otolaryngologists and neurosurgeons on managing challenging encephalocele cases.
Abstract:
Encephaloceles are uncommon abnormalities occurring in the pediatric age group. For the otolaryngologist they are usually encountered in the nasopharynx or nose, but may also be found in other sites adjacent to the brain. Encephaloceles usually develop as a result of prior mastoid surgery, trauma, or infections; but it is extremely rare for large encephaloceles to occur congenitally in the mastoid. It is even more uncommon for there to be a second mastoid defect which would allow the brain to present as a subcutaneous postauricular mass. The case history of a child with congenital subcutaneous transmastoid encephalocele is presented and the management discussed. In this child, the right temporal lobe of the brain herniated through a congenital defect in the tegmen and then through the mastoid cavity to exit through a lateral wall defect in the mastoid cortex. It then presented as a postauricular subcutaneous mass. The initial diagnosis was a subperiosteal abscess. The correct diagnosis was not made until surgery despite a normal preoperative CT evaluation. The world literature is reviewed and similar diagnostic and therapeutic problems are discussed.