Abstract
Objective
The clinical features and patterns of growth, relative to age, were examined in patients with congenital cholesteatoma to investigate disease progression and site of origin.
Patients and methods
We retrospectively reviewed 72 children younger than 15 years with intraoperatively confirmed congenital cholesteatoma diagnosed using the inclusion criteria of Levenson et al. Patient demographics, history, and otoscopic and operative findings were evaluated. Correlation between mass volumetric and operation age was analyzed in patients with closed-type masses. Parameters of disease extent of middle ear quadrant, ossicular erosion, attic involvement, invasion of the mastoid cavity, and type of mass were assessed relative to age at operation.
Results
Mean patient age was 63.1 months (range, 20–179 months), with 51 patients (70.8%) being asymptomatic and diagnosed incidentally. Nineteen patients (26.4%) had closed-type cystic masses, and 53 (73.6%) had open-type lesions. The volume of closed-type masses was linearly correlated with age at operation. Logistic regression showed that increased age at operation was associated with a lower proportion of anterosuperior quadrant lesions, resulting in uncertainty about the site of origin. The possibility of open-type masses also increased according to age at operation.
Conclusion
Congenital cholesteatoma shows growth and extension over time. Early detection and intervention are necessary to avoid advanced disease.
1
Introduction
Congenital cholesteatoma , defined as a whitish mass lesion in the middle ear cleft behind an intact tympanic membrane early in life, is a relatively rare disease. The term congenital has been used rather conventionally because the pathogenesis of congenital cholesteatoma remains unclear and suggested hypotheses range from formation during fetal period to an acquired condition in infant . These lesions might have been associated with a history of acute otitis media, otitis media with effusion, brief otorrhea, or myringotomy . Early diagnosis and intervention have been emphasized because late detection may be associated with extensive disease . More advanced disease has been observed in older children; and recurrence has been associated with advanced disease, suggesting that lesion growth and disease progression are associated with age . Passage of time influences not only patient outcome but understanding of the nature of the disease. Wider lesion extent and infiltration in patients with advanced disease can result in uncertainly about the site of origin .
Although age at operation has been suggested as an important factor influencing treatment outcome and the idea that the congenital cholesteatoma grows over time looks quite clear, the effect of aging on congenital cholesteatoma growth has not been sufficiently demonstrated so far. Thus, the aim of this study was to present how congenital cholesteatoma changes in size, location, and type according to the time passage. We therefore assessed the patterns of mass growth relative to patient age to support the understanding of disease progression and site of origin. The clinical features of patients with congenital cholesteatoma were also investigated in this study.
2
Subjects and methods
We retrospectively reviewed the medical records of 72 consecutive children younger than 15 years with surgically confirmed congenital cholesteatoma, who underwent operations by a single surgeon from January 1999 to September 2010. Our study was approved by the Institutional Review Boards of Asan Medical Center. Patients were diagnosed using the inclusion criteria of Levenson et al , which included the presence of a whitish middle ear mass behind an intact tympanic membrane, absence of tympanic membrane perforation, and no previous otologic procedures, such as ventilation tube insertion. Patients with a history of acute otitis media, otitis media with effusion, brief otorrhea, or myringotomy were not excluded. Demographic characteristics examined included age at operation, sex, and chief complaint at the time of initial diagnosis. Tympanic membranes were examined with ototelescopes before surgery; and intraoperative findings included the location of each mass in the middle ear, the extent of disease, and the erosion of ossicles. Masses were classified as either open (flat keratinizing epithelium without formation of an epithelial cyst or pearl) or closed (epithelial cyst without exposure of keratin) . Volumetric estimation of each closed-type cholesteatoma was performed on 0.6 mm-thickness high-resolution computed tomography images rather than by direct measurement in the operation fields because the mass was often ruptured or its shape could change during the procedures. Under 6-fold magnification, mass volume was calculated on the axial image by volumetric conversion after measuring the area on each section. The relationship between mass volume and age at operation was analyzed in closed-type cholesteatomas by nonparametric correlation analysis. Logistic regression analysis was used to assess mass type, location, and extent of disease relative to age at operation for both types of cholesteatoma.
2
Subjects and methods
We retrospectively reviewed the medical records of 72 consecutive children younger than 15 years with surgically confirmed congenital cholesteatoma, who underwent operations by a single surgeon from January 1999 to September 2010. Our study was approved by the Institutional Review Boards of Asan Medical Center. Patients were diagnosed using the inclusion criteria of Levenson et al , which included the presence of a whitish middle ear mass behind an intact tympanic membrane, absence of tympanic membrane perforation, and no previous otologic procedures, such as ventilation tube insertion. Patients with a history of acute otitis media, otitis media with effusion, brief otorrhea, or myringotomy were not excluded. Demographic characteristics examined included age at operation, sex, and chief complaint at the time of initial diagnosis. Tympanic membranes were examined with ototelescopes before surgery; and intraoperative findings included the location of each mass in the middle ear, the extent of disease, and the erosion of ossicles. Masses were classified as either open (flat keratinizing epithelium without formation of an epithelial cyst or pearl) or closed (epithelial cyst without exposure of keratin) . Volumetric estimation of each closed-type cholesteatoma was performed on 0.6 mm-thickness high-resolution computed tomography images rather than by direct measurement in the operation fields because the mass was often ruptured or its shape could change during the procedures. Under 6-fold magnification, mass volume was calculated on the axial image by volumetric conversion after measuring the area on each section. The relationship between mass volume and age at operation was analyzed in closed-type cholesteatomas by nonparametric correlation analysis. Logistic regression analysis was used to assess mass type, location, and extent of disease relative to age at operation for both types of cholesteatoma.
3
Results
3.1
Demographic features and otoscopic findings
The characteristics of the 72 patients are summarized in Table 1 . There were 54 boys (75.0%) and 18 girls (25.0%), of mean age 63.1 months (range, 20–179 months), with the largest number being 4 to 5 years of age ( Fig. 1 ). Fifty-one (70.8%) patients were asymptomatic at the time of diagnosis. The diagnosis was made incidentally in primary care clinics during routine tympanic membrane checkup for other diseases, mostly common cold. Ten patients (13.9%) visited for hearing disturbance, and 9 (12.5%) were referred for prolonged middle ear effusion. In terms of otoscopic findings, a whitish mass beyond an intact tympanic membrane was found in 68 (94.6%) patients. Thirteen patients (18.1%) also had middle ear effusion. Four (5.6%) patients with conductive hearing loss had normal tympanic membranes on otoscopic examination. They were included because we found definite epithelial pearls during explorative surgery for conductive hearing loss.
| Demographics | No. of patient (%) |
|---|---|
| Sex | |
| Male | 54 (75.0%) |
| Female | 18 (25.0%) |
| Chief complaint | |
| Incidental | 51 (70.8%) |
| Hearing disturbance | 10 (13.9%) |
| Prolonged middle ear effusion | 9 (12.5%) |
| Otalgia | 1 (1.4%) |
| Facial paralysis | 1 (1.4%) |
| Otoscopic findings | |
| Whitish mass | 55 (76.4%) |
| Whitish mass with effusion | 13 (18.1%) |
| Normal | 4 (5.6%) |
