Bilateral congenital cholesteatoma: Surgical treatment and considerations☆
Introduction
Congenital cholesteatoma (CC) is typically present under an intact tympanic membrane without any history suggestive of squamous epithelium implantation into the middle ear [1], [2]. It is an uncommon condition comprising only a minority of all cholesteatomas with reported prevalence between 2 and 15% [3], [4]. The mechanism of origin is thought to be persistent growth of squamous cells from the epibranchial placode or Michael's body [5], [6] which leads to the formation of a cystic lesion in proximity to the tensor tympani tendon. With either a congenital or an acquired mechanism of origin, it tends to affect only one ear. Bilateral cholesteatoma is rare; it is estimated to occur in approximately 3% of patients affected by congenital cholesteatoma [7]. Published literature on congenital bilateral cases is comprised of few small series and case reports during previous decades [8], [9], [10], [11].
Bilateral congenital cholesteatoma poses unique challenges in clinical and surgical management. Unlike acquired cholesteatoma, carers of the child will not be alerted to the presence of the cholesteatoma by odorous otorrhoea and it is most often identified as an incidental finding in routine otoscopy. Hearing loss may be caused by effusion secondary to blockage of the protympanum or ossicular erosion [12]. Preservation of normal hearing thresholds is not just challenged by the difficulty of early identification, but by its location. Most commonly arising in the antero-superior mesotympanum, it may grow into the medial epitympanum making surgical removal difficult without ossicular disruption. Aimi suggested that this origin near the tympanic isthmus of the middle ear is related to the fact that the tympanic isthmus is the junction of the first and second branchial arches [13]. Less commonly, CC is found in the postero-superior mesotympanum where erosion of the stapes superstructure and long process of incus occurs more readily. Otalgia and otorrhoea are rare although some series report up to 50% of previous episodes of otitis media [7].
In BCC cases, hearing preservation is more crucial than in unilateral cases. As it often originates around the tympanic isthmus, it is difficult to eradicate the disease while maintaining an intact ossicular chain. In addition to the priority of optimizing hearing outcomes in bilateral disease, it is necessary to minimize other surgical morbidity. The prospect of four surgeries from two staged bilateral endoscopic or canal wall up tympanomastoidectomies has to be compared with the risk of creating two large and onerous mastoid cavities from a relatively well-pneumatised mastoid with canal wall down surgery.
Advances in otologic care over the last decade provide new options to optimize the care of children with bilateral congenital cholesteatoma. The requirement for second stage surgery can be reduced by endoscope-guided surgery and non-echoplanar diffusion weighted MRI follow up [15], [16]. Totally endoscopic surgery reduces post-operative morbidity thus minimizing the impact of surgery on the child and carers [17].
We present our multicenter experience of bilateral congenital cholesteatoma to review the impact of current management options in the treatment of this rare condition.
Section snippets
Materials and methods
A retrospective chart and surgical video review was completed for patients with BCC who underwent surgery at Otolaryngology Department of Modena and Verona University Hospitals and the Hospital for Sick Children, Toronto. Approval for this review was granted by the Research Ethics Boards in the respective institutions.
Children affected by BCC over the last 14 years were identified using clinical databases. Information regarding demographics, features of cholesteatoma at presentation, surgical
Results
The final study group was composed of six patients (12 ears) (Table 2). The median age was 4 years (range 2–7 years). Four subjects were male and two were female.
Discussion
The surgical management of BCC should be carefully planned in order to minimize the effects of treatment on hearing function and quality of life. Experience developed over the last decade has provided important lessons regarding the most appropriate care of children with this rare condition.
Over the 14 year time period of this study, surgical strategies have evolved. Since the introduction of totally endoscopic ear surgery, it is now possible to remove most of the congenital cholesteatomas by a
Conclusion
The TEA can have a valuable role in BCC; given that both ears are involved, a minimally invasive technique that allows good surgical control in hidden areas while preserving mastoid cells and ossicular chain whenever possible is of utmost importance.
Conflict of interest
None.
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All of the authors have participated in the planning writing or revising the manuscript. None of them has any financial relationship to disclose.