Surgical Management of Petrous Apex Cholesteatomas in the Pediatric Population

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.

Objective: To better characterize the clinical and pathological characteristics, diagnostic and treatment approaches, and functional outcomes associated with pediatric PACs.

Method: 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.

Result: A review of the literature yielded 5 articles that met inclusion criteria, four of which were case reports and one a retrospective cohort study. There were 8 pediatric patients (63% males) with cholesteatomas of the petrous apex that were surgically removed in 9 total ears within this systematic review. Conductive hearing loss was a presenting symptom in seven (88%) of the children with one patient having a mixed conductive and sensorineural hearing loss. All patients underwent a CT or MRI scan to image the cholesteatomas, while 25% had a non-echo-planar DWI. One case report specifically mentioned wanting to get a confirmatory DWI scan, but could not due to dental hardware. Only one patient had bilateral cholesteatomas, while the rest had unilateral ones with 86% being on the left side. There was involvement of the internal carotid artery and facial nerve in only two of the children (25%). Perforations were noted in seven ears (78%) with the most common being in the attic and posterosuperior portions of the tympanic membrane. Mean age of diagnosis and surgical intervention was 10.25 years (SD = 4.63). Recurrence was reported in 50% of the patients. The average rate of recurrence was 3.5 years (SD = 1.73). Average length of follow-up was 32.6 months (SD = 21.7) with one article not reporting this data. The most utilized surgical techniques were the canal wall-up (CWU) and tympanostomy (60%). All four patients in the case reports had a complete resection. Extent of resection was not specified in the retrospective cohort review. No technical complications were reported in any of the studies included. Adjuvant therapy was not required for any patients in the follow-up period aside from surgical resection following recurrence. Following surgical intervention, five out of the seven (71%) patients that experienced perforations had improvement in their hearing loss ([Figs. 1] and [2]).

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.

Publication History

Article published online:
01 February 2023