Pediatric cholesteatoma occurs in one of two forms: congenital cholesteatoma, developing from embryonic epidermal cell rests or acquired cholesteatoma, associated with a focal defect in the tympanic membrane. This disease has been traditionally managed with the operating microscope, often requiring mastoidectomy for adequate visualization of and access to the middle ear and mastoid cavities. Recently, advances in endoscopic equipment have enabled otologists to manage most cases of pediatric cholesteatoma via a minimally-invasive, transcanal endoscopic approach. This review discusses the current literature relating to the etiopathogenesis, assessment and endoscopic management of pediatric cholesteatoma. Early outcomes of endoscopic treatment, emerging trends and technologies are also reviewed.
Congenital and acquired pediatric cholesteatoma has been successfully managed with the microscope for many decades. Minimally invasive approaches that seek to minimize trauma to normal tissue yet achieve similar surgical goals would be logical to apply to the pediatric population. In the last 5 years, with the introduction of high quality narrow diameter endoscopes, cold light sources, ultra-high definition cameras and screens, endoscopic ear surgery (EES) has evolved into a method to successfully treat pediatric cholesteatoma in a minimally invasive manner. This review seeks to outline the current methods and results of EES in the pediatric cholesteatoma population.
The true incidence of pediatric cholesteatoma is not known, and epidemiological studies are sparse. A retrospective review of 122 children operated on for cholesteatoma in Denmark between 1965 and 1978 inferred an annual incidence of pediatric cholesteatoma of 2.9 per 100 000 children (Tos, 1983). This reported incidence remains a good estimate despite its limitation to a single centre in a well-developed country with excellent access to health care. In children, there appears to be a slight male predilection (relative risk 1.4) (Olszewska et al., 2004). The mean ± SD age of children at diagnosis is 5.6 ± 2.8 years in congenital cholesteatoma and 9.7 ± 3.3 years in acquired cholesteatoma (Nelson et al., 2002). A large historical cohort study (Djurhuus et al., 2015) demonstrated a 20-fold increase in the incidence of surgically-treated middle ear cholesteatoma in individuals with cleft palate and a 14-fold increase in the incidence among individuals with cleft lip and palate. However, no difference in incidence was observed in individuals with cleft lip alone. Girls with Turner syndrome are at a higher risk of developing middle ear disease, including cholesteatoma (Hall et al., 2009) with a reported lifetime incidence in this population of approximately 4% (Lim et al., 2014).