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Congenital cholesteatoma of occipital bone or intradiploic epidermoid cyst? One and the same disease

  • M P A Clark (a1), P M Pretorius (a2), D Beaumont (a3) and C A Milford (a1)

We report an extremely rare case of congenital cholesteatoma affecting the occipital bone.


We present a case report, plus a review of the world literature on similar lesions.


This case report describes the presentation and treatment of a congenital cholesteatoma arising in an apparently unique location within the occipital bone, with no effect on middle-ear structure or function. The different imaging characteristics of this lesion are described and illustrated. The discussion centres on the differentiation of this lesion from intradiploic epidermoid cysts, more commonly described in the neurosurgical literature. The possible methods of pathogenesis are discussed, along with treatment suggestions.


Congenital cholesteatomas and intradiploic epidermoid cysts are indistinguishable both histologically and radiologically, and would appear to be the same disease.

Corresponding author
Address for correspondence: Mr Matthew P A Clark, 239 Woodstock Rd, Oxford OX2 7AD, UK. E-mail:
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1 Lee, JH, Hong, SJ, Park, CH, Jung, SH. Congenital cholesteatoma of mastoid origin. J Laryngol Otol 2007;121:e20
2 Constans, JP, Mede, JF, De Divitiis, E, Donzelli, R, Maiuri, F. Giant intradiploic epidermoid cysts of the skull. J Neurosurg 1985;62:445–8
3 Canalis, RF, Shapiro, N, Lufkin, R, Becker, DP. Congenital implantation cholesteatomas of the occipitoparietotemporal junction. Ann Otol Rhinol Laryngol 2002;111:778–82
4 Ciappetti, P, Artico, M, Raco, A, Gagliardi, FM. Intradiploic epidermoid cysts of the skull: report of 10 cases and review of the literature. Acta Neurochir 1990;102:33–7
5 King, JEJ. Extradural diploic and intradural epidermoid tumors (cholesteatoma). Ann Surg 1939;109:649–88
6 Warren, FM, Bennet, ML, Wiggins, RH, Saltzman, KL, Blevins, KS, Shelton, C et al. Congenital cholesteatoma of the mastoid temporal bone. Laryngoscope 2007;117:1389–94
7 Sand, M, Bechara, FG, Altmeyer, P, Hoffmann, K, Scholz, M, Sand, D. A case of regressive occipital cholesteatoma mimicking an ordinary pilar cyst. Ann Plast Surg 2006;56:701–2
8 Persaud, R, Hajioff, D, Trinidade, A, Khemani, S, Bhattacharyya, MN, Papadimitrious, N et al. Evidence-based review of aetiopathogenic theories of congenital and acquired cholesteatoma. J Laryngol Otol 2007;121:1013–19
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The Journal of Laryngology & Otology
  • ISSN: 0022-2151
  • EISSN: 1748-5460
  • URL: /core/journals/journal-of-laryngology-and-otology
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