Otorhinolaryngologia - Head and Neck Surgery Issue 45, July - August - September 2011, pages 5-14

REVIEW

Pediatric cholesteatoma: personal experience and review of the literature.

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VICTOR VITAL Professor of Otorhinolaryngology, Aristotelian University of Thessaloniki, Greece Chairman of 1st Academic ENT Department, AHEPA Hospital
Author of correspondence: Victor Vital, 1st Academic ENT Department, Aristotle University of Thessaloniki, AHEPA Hospital. 1, Stilponos Kyriakidi St., GR 546 36 Thessaloniki, Greece, Tel.: +30 2310 994762, Fax: +30 2310 994 916, e-mail: dr.victorvital@gmail.com

Abstract The cholesteatoma is an accumulation of exfoliated keratin epithelium in a cystic structure, which is recovered with stratified squamous epithelium (matrix) and surrounded by a fibrous tissue stroma (perimatrix), usually involving the middle ear and mastoid. The pediatric cholesteatoma can be classified as congenital, primary acquired and secondary acquired after iatrogenic perforation or infection. The possible origin of congenital cholesteatoma is embryonic cell rests which remain in the middle ear after birth; for the etiopathogenesis of the acquired cholesteatoma four theories are implicated, the metaplasia, immigration, basal hyperplasia and invagination theory or retraction pocket. Diagnosis of cholesteatoma is based on patients symptoms and otoscopy examination. The CT-scan may show the extension of the disease, but does not establish the diagnosis. The pediatric cholesteatoma is an aggressive disease and presents with higher rates of residual and recurrent disease compared with adults. Therefore, treatment of choice is surgical removal of cholesteatoma with two alternative approaches: canal wall up mastoidectomy and canal wall down mastoidectomy. The debate between these two approaches is continued until now. Keywords: cholesteatoma, children, mastoidectomy, canal wall up (CWU), canal wall down (CWD). , ( ) () (), . , , . . 4 : , , . . . . : ( ) ( ). . : , , , , .

Introduction The word cholesteatoma was used for the first time by the German anatomist Johannes Muller in 1838.1 The use of the term cholesteatoma is inappropriate as this benign tumorous lesion (-oma) contains neither cholesterine (cho-) nor fat (-stea-). In 1683, Verney probably gave the first description of a cholesteatoma-like mass, which he called steatoma.2 Other suggested denominations were tumeur perle (pearl tumor) by Cruveilhier in 1829, margaritoma by Graigie in 1891, epidermal cholesteatoma by Cushing in 1922, epidermoid by Critchley and Ferguson in 1928, and keratoma by Schuknecht in 1974. Definition The cholesteatoma is defined as an accumulation of exfoliated keratin squames in a cystic structure, which is recovered with stratified squamous epithelium (matrix) and surrounded by a fibrous tissue stroma (perimatrix).3 Schuknecht4 in 1974 defined cholesteatoma as skin on the wrong side, with accumulation of exfoliated keratin inside the middle ear and originated from the keratinized squamous epithelium. Epidemiology The annual incidence of cholesteatoma was found to be about 3 per 100,000 in children and 12.6 per 100,000 in Caucasians.5 According to Nelson et al,6 the incidence is about 1.4 times higher in males than in females; in the same study it was shown that the mean +/- SD age of children with congenital cholesteatoma was 5.6+/-2.8 years, while the of children with acquired cholesteatoma was 9.7+/-3.3 years. Potsic et al7 showed a high prevalence of cholesteatoma in Caucasian populations, followed by people of African descent, while it is scarcely seen in (non-Indian) Asians. Kempainen et al8 and Chinski9 have stated that the cholesteatoma has a similar incidence among social groups; these authors showed that grommets placed in the ear reduce the prevalence of this disease. Ratnesar10 has suggested that the prevalence of cholesteatoma is lower in Eskimos, because the larger nasopharynx in this ethic group facilitates middle ear aeration and thereby avoids the complications of chronic otitis media. Classification The pediatric cholesteatoma can be classified into three forms: 1) congenital, when it presents as a white mass behind an intact tympanic membrane, and is often called primary or genuine, 2) primary acquired, which appears as a limited diverticulum of the pars flaccida and occurs without evidence of preexisting perforation or infection, 3) secondary acquired, which is the result of traumatic or iatrogenic perforation or infection, and it is found in the pars tensa

region of the tympanic membrane. Tos11 proposed another classification based on the site of origin of cholesteatoma, which considers it as an important factor for the surgical procedure and the prognosis. Thus, according to Tos11 there are three categories of cholesteatoma: 1) attic cholesteatoma, defined as a retraction of the pars flaccida or Shrapnells membrane, extending into the attic or aditus and eventually into the antrum, mastoid or tympanic cavity, 2) sinus cholesteatoma, as a posterosuperior retraction or perforation of the pars tensa extending to the tympanic sinus and posterior portion of the tympanic membrane, and 3) pars tensa cholesteatoma, as a retraction and adhesion of the entire pars tensa involving the tympanic orifice of the Eustachian tube. Saleh and Mills12 presented a classification of cholesteatoma in relation to the ossicle chain condition through the following score: 0- if the ossicle chain is intact 1- if incus is eroded and without chain discontinuity 2- if incus and stapes superstructures are eroded 3-  if the malleus head and incus are absent and stapes superstructure is eroded. Moreover, Saleh and Mills12 also proposed another classification considering preoperative complications; it is usually related to the stage of disease and is important during management. Five complications are observed: fistula of the lateral semicircular canal, facial palsy, total sensorineural hearing loss, sinus thrombosis and intracranial invasion. Three stages are distinguished: C0 for no complications, C1 for occurrence of one complication and C2 for two or more complications. Etiopathogenesis Congenital cholesteatoma. Congenital cholesteatoma represent only the 2-4% of pediatric cholesteatoma.13 The origin of congenital cholesteatoma is not completely understood yet. Among several theories, there are two main theories: embryonic cell rests and the epithelial migration theory.14 Teed14 in 1936 and Michaels15 in 1986 identified epidermoid remnants in the anterosuperior quadrant of the middle ear of temporal bones of human fetuses, which Michaels15 called the epidermoid formation. The epidermoid formation could not be found after the 33rd week of gestation, and it was postulated that its persistence results in the development of congenital cholesteatoma. These epithelial remnants of the embryonic period may occur throughout the body, with the higher prevalence in the meninges, the brain, the skull base and the temporal bone.

The histology is similar to that of the acquired forms: a squamous epithelial-lined cavity filled with concentric layers of keratin, and a pseudoconnective stroma encapsulating it.16 According to the epithelial migration theory,17 the ectoderm from the embryonic external auditory canal surmounts the hypothetical restrictive mechanism of the tympanic ring and migrates into the middle ear. Embryologically, the tympanic ring plays an important role in limiting the medial extent of the external ear canal to the level of the tympanic annulus; however, by failure of this inhibitory function of the tympanic ring, ectodermal tissue derived form the external ear canal enters into the middle ear beyond the annulus, leading to formation of congenital cholesteatoma. In any case, the congenital cholesteatoma is more commonly originates in the anterior superior quadrant,18 and then progressively grows towards the posterior superior quadrant and attic and then into the mastoid;18 its contact with the ossicular chain may result in ossicular discontinuity and in conductive hearing loss. Acquired cholesteatoma. There a number of studies about the pathogenesis of the acquired cholesteatoma, but there is still a lot to be clarified.19 Four mechanisms may be responsible for the development of this disease, but an overlapping of these two or more of them is not excluded: 1) squamous metaplasia of middle ear epithelium due to infection, 2) epithelial immigration through a perforation of the tympanic membrane, 3) invasive hyperplasia of the basal cell layers, and 4) retraction pockets and invaginations of the tympanic membrane due to Eustachian tube dysfunction. The metaplasia theory was proposed by Sad et al20 and is based on their observation that squamous metaplasia, some with keratinization, can occur in the middle ear in response to otitis media, and this could result in cholesteatoma. The metaplastic epithelium grows because of the accumulation of keratin, and recurrent infection and inflammation leads to lysis and perforation of the tympanic membrane resulting in the typical appearance of attic cholesteatoma.21 The immigration theory was first proposed by Habermann22 in 1888 and Bezold23 in 1890 suggesting that squamous epithelium migrates from the margin of a tympanic membrane perforation into the middle ear spaces. In 1901, Politzer observed that the epidermis of the external ear canal can grow over a perforation of the tympanic membrane perforation into the middle ear cavity. The basal hyperplasia theory was first supported by Lange24 in 1925 reporting that epithelial cells from the keratinizing epithelium of Shrapnells membrane could proliferate into the subepithelial tissue of Prussaks space and form an attic cholesteatoma without perforation or invagination. Experimental studies in animals confirmed this theory show-

ing that microcholesteatoma may arise within or behind an intact tympanic membrane; pseudopodia from the basal cell layer seem to break the basal lamina, allowing epidermal keratinocytes to invade the lamina propria and forming cholesteatoma.25,26 The invagination theory or retraction pocket is generally accepted as the most common pathogenic mechanism. Patients with acquired cholesteatoma have a functional obstruction of the Eustachian tube and are thus predisposed to high negative middle ear pressures. The areas of the tympanic membrane most susceptible to these forces are the pars flaccida, posterosuperior tympanic membrane and the old tympanostomy-tubes sites. The retention of accumulating keratin within the deep retraction pocket forms a cholesteatoma. An altered epithelial migration pattern in these deep retraction pockets was observed by Sad.27

Fig. 1a

Fig. 1b

Diagnosis Children with cholesteatoma may present with foul-smelling discharge and often bleeding, slight otalgia or headache, and occasionally dizziness. In the otomicroscopy, the congenital cholesteatoma can be seen as a white spherical mass with well defined margins behind an intact tympanic membrane (figure 1a,b). The acquired cholesteatoma can be present with squames or keratine debris and granulation tissue/polypes in the epitympanum (figure 2). The attic and/or the posterior wall of

contours of the external ear canal and mastoid.33 Healing of intact canal mastoidectomy is usually rapid, postoperative ear cleaning are not necessary, the rate of external ear canal infections is low, avoids restrictions on water exposure, does not require meatoplasty and provides greater choice on hearing aids.9,34 However, it is a procedure which demands high surgical skills, as in cases which the disease is extended into the facial recess or the sinus tympani. Therefore, most of surgeons prefer to perform a second look exploration of the middle ear and mastoid if necessary in children.9,35,36 Relative contraindications to a CWU mastoidectomy procedure include a sclerotic mastoid, labyrinthine fistula, only hearing ear, poor Eustachian tube function.37 In addition, one of the disadvantages of CWU mastoidectomy is the development of a deep retraction pocket formation and the potential for recurrent cholesteatoma.38 Moreover, there is an increased risk for residual cholesteatoma if it is located in the attic or mastoid cavity.38 Canal wall down mastoidectomy (CWD) The CWD mastoidectomy involves taking down the posterior wall of the external ear canal to the vertical facial ridge. A CWD operation is performed when cholesteatoma is large, extending beyond the aditus.39 It provides a better visualization, compared to CWU, of the lateral epitympanum, sinus tympani and posterior crus of the stapes footplate.40 In particular anatomic conditions, such as low-lying middle cranial fossa tegmen and an anteriorly positioned sigmoid dura CWD mastoidectomy is the preferable surgical method. Moreover, CWD operation is advisable in the presence of matrix over the lateral canal fistula or in case of significant erosion of the posterior wall of the external ear canal. It is also provides better visual control in postoperative follow-ups for recurrent or residual disease and is highly recommended in case of recurrent cholesteatoma after CWU procedure. In several studies, the CWD mastoidectomy is followed by canal wall reconstruction using auto-bone plate,41 or sculpted cortical bone chips42 or by mastoid obliteration in order to reduce the size of mastoid cavity.43 Hearing results are variable44 although the use of cartilage-wirefascia as total ossicular replacement prosthesis (TORP) has reported to have good hearing results, with air-bone gap 25dB in 88% of cases.45 However, CWD mastoidectomy requires regular cavity cleaning which often is not well tolerated by younger patients in the office; in addition, CWD operation is associated with water intolerance, recurrent infection, caloric-induced vertigo, and the diminished ability to wear a hearing aid, which hopefully would be rarely necessary in children.42

Fig. 2

the external ear canal can be eroded by the disease. In a discharge ear, a partial stenosis of the external ear canal due to oedema may be found. The tonal audiogram may reveal a conductive or mixed hearing loss in the diseased ear. A computerizing tomography of the temporal bone may be useful to identify possible erosion of the tegmen, facial nerve dehiscence, and labyrinth fistula. Treatment The pediatric population poses a greater challenge than adults regarding treatment of cholesteatoma, because of the higher rates of residual and recurrent disease compared with adults, possibly due to anatomic and physiologic differences.28,29 Eustachian tube anatomy and dysfunction predispose children to more frequent infections and retraction pockets, and well pneumatized mastoids in children allow for more extensive disease compared with more sclerotic mastoid bones.30,31 Another explanation could be the significant higher proliferative activity in cholesteatoma keratinocytes in children compared to adults.32 The pediatric cholesteatoma is a surgical disease, but antibiotic-steroid drops should be applied to decrease the inflammation and granulation tissue prior to surgery. The goals of surgery for cholesteatoma in children are: 1) to completely eradicate the disease, 2) prevent recurrent disease and 3) to optimizing hearing. To achieve these goals, two main surgical techniques exist: the canal wall up mastoidectomy (CWU) and canal wall down mastoidectomy (CWD). Acquired cholesteatoma Canal wall up mastoidectomy (CWU) This procedure aims to preserve the posterior wall of the external ear canal with conservation of normal anatomical

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Comparison of CWU CWD mastoidectomies The CWU mastoidectomy has a higher rate of residual and recurrent cholesteatoma than CWD operation in children.42,46,47,48 According to Table 1, the residual/recurrent cholesteatoma rates following CWU mastoidectomy may reach a level almost more than twice as high as those after CWD mastoidectomy. The CWU procedure may have better hearing results than the CWD mastoidectomy in children,36,49 although in some studies this difference was not significant34,43. Soldati and Mudry,46 in a study of 59 children, reported that an air-bone gap of less than 20dB was achieved in 51% of the ears, and 80% had a gap of less than 40dB; moreover, no difference in hearing outcome was found between type IV reconstruction with TOPR performed with either CWU or CWD procedures. It is worthy to note Hildman and Sudhoffs statement: For treatment of cholesteatoma nowadays the closed technique (CWU) is an exception. 53 Following the disease Because both CWU and CWD techniques provide advantages and disadvantages, perhaps an intermediate method is preferable before CWD is performed. Sad50 introduced a less invasive approach to the pediatric cholesteatoma with a creation of a smaller mastoid cavity. Briefly, atticotomy was performed using an endaural incision, and after tympanomeatal flap was elevated the underlined scutum was drilled; the idea was to follow the disease until the end of cholesteatoma sac was found, necessitating sometimes a larger mastoidectomy. The posterior wall of the external ear canal was preserved, except in case of extensive mastoid or middle ear involvement when it should be drilled out down to facial ridge. A tympanoplasty type III was preferred depending on the ossicles involved. In a long-term followup study, 53 children undergone atticotomy for cholesteatoma,50 and the total rate of recurrent and residual cholesteatomas was found to be limited (21%). Congenital cholesteatoma Surgical approach depends on the location and extension of the cholesteatoma, patient age and ossicular integrity.13 Over 30% of congenital cholesteatoma can be removed through a transcanal approach in early stage.6 In case of posterior extension of the disease into the middle ear with involvement of the ossicles, a CWU mastoidectomy should be performed, whereas CWD procedure is rarely required.13 Predictors of residual-recurrent cholesteatoma in children In a study with 114 children undergone first-time surgery for acquired cholesteatoma (CWU and CWD) and with a follow-up ranging from 1 to 16 years, Stangerup et al51 had the following interesting results for cholesteatoma recurrence: the extension of cholesteatoma was the most impor-

tant predictor of recurrence; in decreasing order, negative preoperative middle ear ventilation (negative Valsalva test) and ossicular chain resorption (the malleus was eroded in 7%, the incus body in 15%, the long process of incus in 61%, the stapedial arch in 15% of cases). In the same study,51 recurrence of cholesteatoma may develop up to 8 years after surgery; significant higher recurrence rates was found in younger than 8 years old children (47% vs 24% in children 8 years); this finding was consistent with the aggressive nature of childhood cholesteatoma. In another series of 231 children treated surgically for cholesteatoma,52 the most residual cholesteatomas arose from the oval window area where the removal of the disease was difficult for obvious reasons and from the sinus tympani where the disease may grow medial to the vertical portion of the facial nerve, an area difficult to view; ossicular erosion and relative lack of experience of the surgeon were also found to be risk factors for residual cholesteatoma. The cholesteatoma extension, childs age, previous ear surgery, exact nature of the disease (congenital, acquired, iatrogenic) or type of surgical procedure (CWU, CWD) did not related to the cholesteatoma residual rate.52 In the same study,52 it was underlined that when the above factor/s were present, the possibility of residual cholesteatoma is very high in the first 2 years after surgery, and so a second look was recommended in the first 9-18 months after surgery.

Conclusions The diagnosis of the pediatric cholesteatoma is based on clinical and otomicroscopical examination. An ear with fetid otorrhea which does not stop after local treatment with antibiotic-steroids drops for one week is Fig. 3 suspicious for cholesteatoma. The CT-scan may show the extension of the disease, but does not establish the diagnosis. There are two main surgical alternatives for the treatment of cholesteatoma: the canal wall up and the canal wall down mastoidectomy. Because of the high rate of residual/recurrent pediatric cholesteatoma, in case of canal wall up operation, the parents must be informed for a second look procedure performed one year later. My personal experience45 consists of following the disease approach. After the epitympanotomy, the cholesteatoma is followed until its total removal. An open cavity is then

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created posteriorly, which gives the possibility postoperatively to detect any residual/recurrent cholesteatoma and to clean it without operating the ear. The local use of peroxide of hydrogen and antibiotic-steroid drops succeeds at variant times to dry the ear. If all the ossicles are eroded, the cartilage-wire-fascia prosthesis (figure 3) may result in good hearing with a small tympanic cavity. The postoperative surgical results of the cholesteatoma depend on the experience of the surgeon and the approach he is used to perform. The parents should be informed for a long-term follow-up of their children.
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