Junior Specialist UC Irvine Department of Neurosurgery Santa Clarita, California, United States
Introduction: Cholesteatomas are growths of squamous epithelium that can form inside the middle ear and mastoid cavity. While most are benign, they can expand and damage nearby structures, contributing to conductive hearing loss, otomastoiditis, facial nerve palsy, abscess, and death. Cholesteatomas can occur in pediatric patients at the petrous apex, located on the medial aspect of the temporal bone. The primary goal of petrous apex cholesteatoma (PAC) resection is gross total removal with tympanoplasty and canal-wall up or canal-wall down tympanomastoidectomy. Although there remains no definitive approach supported by greater than Level 4 evidence in the literature to date, surgery is performed to reduce the risk of recurrence and improve symptoms, while minimizing operative morbidity.To better characterize the clinical and pathological characteristics, diagnostic and treatment approaches, and functional outcomes associated with pediatric PACs.
Methods: A systematic review was conducted utilizing PubMed, Embase, and Scopus databases. Articles were screened and selected to be reviewed in full-text. The articles that met inclusion criteria were reviewed for relevant data. Data analysis, means and standard deviations were calculated using Microsoft Excel.
Results: After screening, 5 articles were included in the systematic review. There were a total of 8 pediatric patients included with 9 total cholesteatomas removed. Conductive hearing loss was the most common (77%) presenting symptom. Perforations were noted in seven ears (86%). Recurrence was noted in 50% of patients with an average recurrence rate of 3.5 years (SD = 1.73). Canal wall-up was the most commonly utilized technique (60%) and there were no surgical complications across the entire cohort.
Conclusion : Due to its rarity, diagnostic evaluation and treatment of pediatric PAC can vary, but it is critical that each patient be evaluated individually to achieve optimal outcomes and prevent recurrence. Further multi-institutional investigation is necessary to develop population-level management protocols for pediatric patients affected by PACs.
