Abstract
Cholesteatoma is a mass of keratin debris in the middle ear cavity or mastoid. Congenital cholesteatomas may remain asymptomatic for many years and present during adulthood or may even be an incidental finding. We hereby describe a case of 41-year male with an isolated mastoid congenital cholesteatoma with sigmoid plate erosion and posterior cranial dura exposure extending into digastric muscle. Tympanomastoid exploration with sigmoid sinus resurfacing with hydroxyapatite granules was done. However, the case got complicated by extrusion of granules which was then successfully managed. This case report throws light on diagnosis and surgical management of isolated mastoid cholesteatoma. From this misadventure, we learnt that a successful mastoid cavity obliteration (using hydroxyapatite granules) in a canal wall up mastoidectomy can be achieved by providing hermetic seal.
Keywords: Isolated, Congenital, Cholesteatoma, Hydroxyapatite
Introduction
Cholesteatoma is a mass formed due to progressive accumulation of keratin debris in the middle ear cavity or mastoid. Congenital cholesteatomas (CC) are assumed to be present before birth and generally present during infancy or childhood [1]. They may even remain asymptomatic for many years and present during adulthood or may even be an incidental finding. If left untreated may act as a gateway to intracranial complications.
Case Report
41year male, soldier by profession was transferred to our tertiary care centre from a peripheral hospital.
At peripheral hospital, he had presented with mucopurulent discharge from right ear of 8 months duration with a small perforation in tympanic membrane. He was diagnosed as a case of right mucosal chronic otitis media (COM) and was planned for cortical mastoidectomy with tympanoplasty by an otolaryngologist. There, the surgery was aborted in view of intra operative findings which revealed erosion of bony sigmoid plate. The patient was hence transferred to our centre.
The patient was evaluated clinico radiologically. High Resolution Computed tomography of temporal bone (HRCT-TB) revealed soft tissue density in entire mastoid air cells with bony erosion of posterior cranial fossa (PCF) and bony plate of sigmoid sinus (SS) (Fig. 1a). Magnetic Resonance Imaging (MRI) showed hyper intense lesion in T2 images with restriction of diffusion in diffusion weighted (DW)images (Fig. 1b). Intra operatively, a large pearly cholesteatoma was seen occupying the mastoid in its entirety with erosion of the surrounding bone and exposing the posterior fossa dura, the sigmoid sinus and infiltrating the digastric muscle. The posterior wall of external auditory canal was found intact with subtle erosion (Fig. 1c). There was no disease seen in aditus ad antrum, epitympanum or the mesotympanum. These findings were indicative of an isolated mastoid cholesteatoma. The surgery was aimed at complete clearance of the disease, reconstruction of sigmoid and posterior cranial fossa defects to provide a safe ear. A scrupulous removal of the cholesteatoma was done. Sigmoid sinus resurfacing with mastoid cavity obliteration was done in layers using conchal cartilage, hydroxyapatite (HA) bone crystals and bone dust (Fig. 1d). In addition, type I tympanoplasty was done.
Fig. 1.
(a) HRCT-TB showing erosion of bone over sigmoid sinus and posterior cranial fossa (arrows); (b) DW image of MRI showing soft tissue in entire mastoid which shows diffusion restriction ; (c) Intra operative findings (EAC- external auditory canal, LSCC- lateral semicircular canal); (d) Mastoid cavity filled with HA granules (BIPP-Bismuth Iodoform Paraffin Paste)
On seventh post operative day, there was extrusion of the HA granules from the inferior end of wound (Fig. 2a). The HRCT-TB confirmed the HA granules in operated cavity and collection in subcutaneous plane with a tract connecting it with skin (Fig. 2b). Exploration of the surgical site was planned; the sinus tract was excised along with extra granules. A part of HA granules was well accepted at the mastoid tip which was covered with granulation tissue (Fig. 2c). To achieve a hermetic seal for the mastoid cavity, the surgical site was strengthened with fascia lata, fat, temporalis muscle rotation flap and reinforced by tissue glue in each layer (Fig. 2d). This gave a good result when followed up at 6 months post operatively. Also, neotympanum was intact with normal hearing.
Fig. 2.
(a) Extrusion of HA granules from post auricular surgical site; (b) HRCT-TB showing HA granules extrusion tract from mastoid till subcutaneous tissue (yellow circle) ; (c) Intraoperative view of mastoid cavity on exploration; (d) Intraoperative picture showing temporalis muscle rotation flap being placed over fat and fascia lata strengthened with fibrin glue
Discussion
Congenital Cholesteatoma (CC) is a locally destructive mass of keratin debris. It is an epidermoid cyst derived from congenital remnant of keratinizing squamous epithelium of temporal bone. Various sites of CC are petrous apex, cerebello-pontine angle, middle ear cavity, external auditory canal and mastoid process.
Isolated mastoid cholesteatoma is extremely rare [1] with only 24 cases reported in literature till now [2]. An extensive literature search was done to look for cases of isolated mastoid cholesteatoma in PubMed and Google Scholar. According to literature review conducted by Annalisa P et al., isolated mastoid congenital cholesteatoma with no involvement of aditus ad antrum and middle ear, has an elderly preponderance in presentation with mean age of 51(range 13–87) [2].
There are various theories explaining the origin of CC which is well elaborated by Hong et al. [3] namely the metaplasia theory, invagination, embryonal epithelial rest, and implantation theory.
On scrutinising the presentation and the findings of our case, the most plausible cause in our case is the implantation theory in which squamous epithelium is trapped within the tympanomastoid suture during closure of mastoid fontanelle. As explained by Northrop et al. [4] during intra uterine life, the amniotic fluid when enters the middle ear cavity through the Eustachian tube with debris in the mastoid, this may act as nidus to the formation of cholesteatoma which explains our case. In such cases the patient remains asymptomatic for extended period and may present when there is erosion of the adjacent structures.
Hydroxyapatite is a member of calcium apatite with a composition similar to inorganic form of natural bone and teeth. It occurs both naturally and can be manufactured synthetically. Owing to the properties of excellent bioavailability, bioactivity and biodegradability, it has wide applications as an implant material for bone and dental implants. For the above advantages many studies recommend it as a synthetic agent of choice for mastoid obliteration as well. [5–7]
Therapeutic misadventure is defined as an injury or an adverse event caused by medical or surgical management rather than by an underlying disease. [8, 9] Despite the endeavour of sticking to the rule “first do no harm” sometimes in reality it is difficult.
After having being faced the complication in our case, on further analysing the procedure we could infer the reason for extrusion of granules. Due to lack of hermitic seal which acts as a strong barrier and provides wound strength, there are chances of granules extrusion. [10] Thus it is essential to have a hermetic seal made up of layers of flap, fat and fascia to strengthen the wound and aid the successful uptake of the HA granules.
Conclusion
An isolated mastoid cholesteatoma can be called a clandestine keratin ball as it silently erodes the surrounding bone exposing the critical structures. Timely diagnosis and appropriate surgical treatment is the key for prevention of alarming intracranial complications.
Owing to the rarity of its presentation, our case report would be a contribution to the existing literature describing the presentation and diagnosis. Also based on this experience, we have inculcated a successful use of the hydroxyapatite in otological cases.
Acknowledgements
The authors would like to thank Dr Samrat Ghosh, Assistant Professor, Dept of Radiodiagnosis, Command Hospital (Eastern Command), Kolkata, India.
Funding
No funding was received to assist with the preparation of this manuscript.
Declarations
Research Involving Human Participation and/or Animals
Nil.
Informed Consent
Informed consent was taken from patient for the publication.
Competing Interests
The authors have no relevant financial or non-financial interests to disclose.
Footnotes
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