Cholesteatoma

COM, ear discharge

1. cholesteatoma Dr Safika Zaman Post-Graduate Trainee Dept of ENT & Head Neck surgery VIMS,RKMSP

2. Introduction Abramson et al defined cholesteatoma as – “A three dimensional epidermal and connective tissue structure, usually in the form of a sac, and frequently conforming to the architecture of various spaces of the middle ear, attic and mastoid. This structure has the capacity for progressive and independent growth at the expense of underlying bone and has the tendency to recur after removal. . Gray’s definition : cholesteatoma is “skin in the wrong place

3. History of cholesteatoma The French anatomist Du Verney first reported a case of cholesteatoma-like symptoms in 1683 . In 1829, Cruveilhier described the pathologic features of what he referred to as pearly tumor . In 1838 , Johannes Muller, a German anatomopathologist,coined the term to describe a tumor that appeared “greasy in nature”. Nonetheless, this is something of a misnomer .

4. Epidemiology Annual incidence of acquired cholesteatoma ranges from approximately 9 to 12.6 cases per 100,000 adults and from 3 to 15 cases per 100,000 children. Male predominance of - 1.4 : 1 In the paediatric population, cholesteatomas account for10% of chronic otitis media cases. There is a high prevalence in Caucasian populations. Middle ear cholesteatoma peaks in the second and third decade of life.

5. Classification of COM Based on the disease pathogenesis: 1.Congenital 2.Acquired

6. Congenital cholesteatoma Derlacki and Levenson’s critria Approximately 2% of the entire cholesteatoma cases . Mean age of presentation is 4.5 yrs

7. Theories of Congenital Cholesteatoma Epidermal rest theory : This theory is based on a finding of cell rests of non-keratinizing squamous epithelial cells, localized in the lateral wall of the Eustachian tube, close to the tympanic ring. Inclusion Theory : Other authors favor even a way of migration from cells coming initially from the external ear through non evident injuries of the tympanic membrane

8. Acquired cholesteatoma Most common type of cholesteatoma. Divided into – 1.Primary acquired. 2.Secondary acquired

9. Primary acquired . 1.pars flaccida pocket, 2 pars tensa pocket, 3. combined forms

10. Secondary cholesteatoma Secondary to infection, trauma or perforation

11. Prominent theories of etiopathogenesis of acquired cholesteatoma 1 .Invagination theory (retraction pocket theory) 2.Epithelial invasion or migration (immigration theory) 3.Squamous metaplasia theory. 4. basal cell hyperplasia theory (papillary ingrowth theory) Etiopathogenesis of Acquired Cholesteatoma: Prominent Theories and Recent Advances in Biomolecular Research Chin-Lung Kuo , MD

12. Invagination Theory (Retraction Pocket Theory) of Toss & Wittmaack Two things are present in cholesteatoma and not present in safe retraction pocket: epithelial hyperproliferation and abnormal Skin migration

13. Theory of Epithelial Migration (Immigration Theory) of Habermann & Bezold Perforation of the eardrum, traumatic or iatrogenic, gives access to the squamous epithelium of the eardrum or of the outer ear canal skin, to invade or migrate into the middle ear leading to the formation of a Secondary acquired cholesteatoma excessive production of keratin lead to a cholesteatoma formation.

14. The squamous metaplasia theory of Wendt The metaplasia theory stipulated that the epithelium of the middle ear changes into squamous epithelium under the effect of a persistent chronic inflammation.

15. Basal Cell Hyperplasia Theory (Papillary Ingrowth Theory) of Lange Basal cell hyperplasia theory postulates that keratin-filled microcysts , buds, or pseudopods formed in the basal layer of the pars flaccida epithelium, invade the sub-epithelial tissue, fuse together, resulting in the formation of cholesteatoma of Prussak’s space.

16. Arise from postero superior quadrent Nonaerated spaces growth Stable atelectasis and cholesteatoma Normal Eustachian tube and tube Tympanostomy does not prevent Associated with mastoid hypo- pneumatization

17. Cont …

18. CHOLE CLASSIFICATION

19. Paediatric vs Adult cholesteatoma Paediatric cholesteatomas  present a more exacerbated inflammatory degree . In children recurrence rates were higher than that in adults. 80% retraction pockets of the pars tensa whereas in adults, Postoperative hearing levels were better in children,

20. Histopathology of cholesteatoma

21. Electron microscopy of cholesteatoma matrix has the same histological and cellular structure as the epidermis of EAC. Inflammatory cells, Langerhans’ cells , and Merkel cells are identified in the stratum spinosum layer of the cholesteatoma matrix in a higher amount compared to the normal epidermis

22. Genomic alteration the cholesteatoma epithelium exhibits a significantly higher percentage of proliferation marker-labelled cells. Aneusomy of chromosomes 7 and 17 has also been suggested to play a crucial role in cholesteatoma growth and bone destruction. Microarray analysis by. revealed that the expression of GJB2 gene is higher in cholesteatoma tissue than in the skin of the external auditory canal(codes connecxin 26). James et al. found that only 14% of children with cholesteatoma present variants of the gene GJB2.

23. Moleculer pathogenesis Langerhans’ cells require the cooperation of activated T-lymphocytes to become functional. These activated T-lymphocytes represent the “vital union” . Langerhans cells have tropism towards the keratinized squamous epithelium .

24. Cont … The fundamental difference between the healing process in normal skin and in cholesteatoma, is that in cholesteatoma there is a loss of the growth inhibition by “cell to cell contact”. Two factors are involved in this loss of growth control in cholesteatoma: 1.The cholesteatoma develops beyond its normal anatomical site for a “skin”. The middle ear environment is not adequate to induce the habitual cell contact inhibition. 2. The inflammatory process produces a self-maintained immunological cycle

25. Moleculer pathogenesis

26. Role of angiogenesis Release a variety of angiogenic factorsvascular ( VEGF, EGF, TGF-a, PDGF, IL8), Angiogenesis within the perimatrix Migration of keratinocytes into the middle ear cavity Progression of disease

27. Bone absorption the labyrinth- most rigid bone of the body. Factors are- inflammation local pressure, specific enzymes –MMPs, collagenase prostaglandin E Acidic pH , of keratin debris is a critical factor in bone destruction

28. Role of infection Pseudomonas lipopolysaccharide has been shown to activate keratinocyte hyperproliferation Bacteria also prevent the cholesteatoma epithelium from activating terminal differentiation and returning to a quiescent state.

29. Biofilm formation The keratin layer of cholesteatoma is an ideal environment for biofilm development. The presence of antibiotic-resistant bacterial biofilms in cholesteatomas may also explain their aggressiveness Evidence for microbial biofilms in cholesteatomas. Richard A. Chole , Brian T. Faddis Published 2002 Biology, Medicine Archives of otolaryngology--head & neck surgery

30. Pathogenesis

31. Pathway Growth pattern of the acquired cholesteatoma is oriented by two main factors: 1. site of origin of the cholesteatoma. 2.the anatomical compartments in the middle ear cleft. The ligaments, mucosal folds, ossicles , and walls of the middle ear separating do not play the role of barriers but guide the growth of cholesteatoma into distinct pathways throughout the middle ear cleft. Identification of pathway is not possible in advanced stage.

32. Routes of spread

33. Clinical presentation Foul smelling discharge Hearing loss Persistent otitis externa Vertigo, tinnitus Balance disorder Facial paralysis. Mastoid abscess Headache, otalgia, vomiting, seizure

34. Diagnosis Otoscopic examination:

35. Audiometric evaluation Pure tone audiometry- CHL with good word recognition score. A conductive deficit more than 40 dB indicates ossicular discontinuity.

36. CT Scan Not to diagnose To learn disease extent. HRCT of the temporal bone is indispensable to otologists for surgical planning Reading a cd rather than a plate is more informative.

37. What to look for in a CT-Scan extent of disease possible osseous destruction middle ear hypoplasia, jugular bulb variations bony dehiscence of the facial nerve sclerotic or diploic mastoids anterior sigmoid sinuses other complications like fistula or dehiscence.

38. Limitation of a CT Scan & Cone beam CT similarities in the density of CT scans for cholesteatoma, granulation tissue, fibrous tissue,mucosal edema , and effusion greatly limit the ability of HRCT to distinguish among these disease entities. Exposure to radiation Morphologic examination of the temporal bone by cone beam computed tomography: Comparison with multislice helical computed tomography Author links open overlay panel M .Dahmani-Causse a M.Marx a O.Deguine a B.Fraysse a B.Lepage b B.Escudé c https://doi.org/10.1016/j.anorl.2011.02.016 There was no significant difference in morphologic assessment of the temporal bones on the two techniques of CBCT and MSCT. CBCT delivered 22 times less radiation than MSCT .

39. DW-MRI Diffusion-weighted (DW) sequences are highly promising in differentiating recurrent cholesteatoma from granulation tissue DW MRI depends on the difference in diffusion of water molecules in different biological tissues. Water molecules in cholesteatoma are less mobile giving rise to a hyperintense signal. In granulation tissue, water molecules are more mobile and thus appear less intense on DW sequence. Currently, single-shot echo-planar DWI is the most used DWI technique.

40. DW-MRI

41. Treatment Treatment is essentialy surgical. Choice of surgery will depend upon- Extent of disease Available facilities Surgeon’s expertise Patients willingness towards long term follow up

42. Patient counselling

43. Cont …

44. Objective of surgery 1.Total eradication of cholesteatoma to obtain a safe and dry ear. 2.Maintain the best condition for a successful wound healing process in the ear. 3. Restore or maintain the best functional status of hearing.

45. Surgical Procedures 1 ) A CWD mastoidectomy 2) CWU mastoidectomy 3) Other procedures: • Reconstruction of the ear canal defect.. • Atticoantral mastoid obliteration can be done after CWU or CWD. • Ossicular reconstruction must be decided.

46. Transcanal anterior Atticotomy indicated for a cholesteatoma with limited involvement of the middle ear, with intact ossicular chain and/or a healthy epitympanum . Reconstruction of the resulting cavity is done with cartilage and perichondrium .

47. CWD Mastoidectomy Indications • Cholesteatoma of an only hearing ear, • A major erosion of the posterior bony canal wall, • A history of vertigo due to a labyrinthine fistula, • A poor Eustachian tube function, • A sclerotic mastoid with limited access to the epitympanum . • Patient non-compliant for follow-up.

48. CWD Advantages • Relatively short duration of the surgery • Easy detection of the postoperative residual disease. reduced rate of recurrences; the facial recess is well exteriorized as well as the attic. • Any postoperative cholesteatoma regrowth can readily dealt . Disadvantages • Hearing reconstruction is less successful. • Open cavity: the mastoid bowl maintenance can be a lifelong problem. Unpleasant appearance of the meatoplasty Wet cavity Difficulty in fitting hearing aid

49. CWU Mastoidectomy In modern otosurgery , CWU must be the first choice for most cholesteatoma cases. Disadvantages • Long duration of the surgical procedure in extended pathologies. • Unsatisfactory exposure and high rate of residual disease. • Staging and multiple surgical looks

50. Endoscopic and Microscopic endoscope assisted-microsurgery allows the use of the most efficient tools to face difficult sites of localization of the cholesteatoma, and allow its complete removal.

51. Follow-up 1. A surgical second-look procedure should be proposed always for patients when a complete removal of the disease during the primary surgery was uncertain. 2. MRI evaluation may be appropriate to avoid a surgical second look for patients when the otologist was sure that all the disease has been completely excised by his first surgery and when an unequivocal normal microscopic examination is observed during the first 6 months of the postoperative period .

52. Complication of cholesteatoma Otogenic intracranial complication: meningitis, Otitic hydrocephalus, lateral sinus thrombosis, cavernous sinus thrombosis. intracranial sepsis-extradural, epidural, subdural, peri -sigmoid sinus and cerebral abscess formation.

53. Complication of cholesteatoma Labyrinthine fistula Labyrinthitis Facial nerve paralysis

54. Atypical cholesteatoma Congenital Cholesteatoma Localized to the Tip of the Mastoid Bone: A Case Report and Possible Etiology Seok Min Hong ,  Jun Ho Lee ,   Chan Hum Park , and  Hyung -Jong Kim

55. conclusion Recent advances in biomolecular research have enhanced our understanding of the etiopathogenesis of acquired cholesteatoma. Complex and hybrid procedure. Treatment is surgical. Choice of surgery is variable Follow up is essential

56. Thank you