International Journal of Pediatric Otorhinolaryngology 78 (2014) 918–922
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International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Acquired middle ear cholesteatoma in children with cleft palate: Experience from 18 surgical cases Vincenzo Vincenti *, Francesca Marra, Barbara Bertoldi, Daniela Tonni, Maria Silvia Saccardi, Salvatore Bacciu, Enrico Pasanisi Department of Clinical and Experimental Medicine, Unit of Audiology and Pediatric Otorhinolaryngology, University of Parma, Italy
A R T I C L E I N F O
A B S T R A C T
Article history: Received 2 November 2013 Received in revised form 5 March 2014 Accepted 6 March 2014 Available online 15 March 2014
Objectives: To review an institutional experience with the surgical management of middle ear cholesteatoma in children with cleft palate. Materials and methods: We analyzed retrospectively 18 children diagnosed with cleft palate who underwent surgery for acquired middle ear cholesteatoma between 2000 and 2007. The following data were recorded: age, sex, history of ventilation tube insertion, status of the contralateral ear, cholesteatoma location and extension, and surgical technique involved. Cholesteatoma recidivism, stable mastoid cavity and hearing levels were the main outcomes measured. Results: Follow-up ranged from 5 to 12 years (mean 8 years). Twelve children underwent planned staged canal wall up mastoidectomy: a residual cholesteatoma was found and removed during the second-look procedure in 2 ears (16.6%); two children (16.6%) showed a recurrent cholesteatoma and required conversion to canal wall down mastoidectomy. A modified Bondy technique was chosen in two children with an epitympanic cholesteatoma with an intact tympano-ossicular system, while in the remaining four subjects a canal wall down mastoidectomy was performed because of an irreparable erosion of the postero-superior canal wall: no cases of recurrent cholesteatoma were observed in these 6 children; revision mastoidectomy was needed in one patient for cavity granulation. A postoperative air-bone gap result of 0–20 dB was achieved in 11 children (61.1%); in 5 cases (27.7%) postoperative air-bone gap was between 21 and 30 dB, while in 2 (11.1%) was >30 dB. Bone conduction thresholds remained unaffected in all cases. Conclusions: Our results indicate that most cleft palate children with cholesteatoma can be managed with a canal wall up mastoidectomy with low complication rates. In extensive disease with large erosion of the canal wall as well in presence of a retraction pocket in the contralateral ear, a canal wall down mastoidectomy should be considered. In epitympanic cholesteatomas with an intact tympano-ossicular system and mesotympanum free of disease, the modified Bondy procedure is an effective surgical option. As in the general pediatric population, improvement or preservation of hearing can be obtained in most patients. ß 2014 Elsevier Ireland Ltd. All rights reserved.
Keywords: Cholesteatoma Cleft palate Middle ear surgery Tympanoplasty Mastoidectomy Hearing results
1. Introduction The incidence of cholesteatoma in childhood has been reported at 3–6 per 100,000 [1], while in children with cleft palate (CP) is much higher, ranging from 1.8% [2] to 9.2% [3].
* Corresponding author at: Department of Clinical and Experimental Medicine, Unit of Audiology and Pediatric Otorhinolaryngology, University of Parma, Via Gramsci 14, 43126 Parma, Italy. Tel.: +39 0521 703204; fax: +39 0521 703788. E-mail address: [email protected] (V. Vincenti). http://dx.doi.org/10.1016/j.ijporl.2014.03.007 0165-5876/ß 2014 Elsevier Ireland Ltd. All rights reserved.
A poor Eustachian tube (ET) function leading to reduced middle ear pressure and tympanic membrane retraction contributes to the elevated incidence of acquired cholesteatoma in these children. Beyond the traditional explanation of ET dysfunction, potential genetic contribution to the pathogenesis of cholesteatoma has also been suggested [4]. In addition, since children with CP are more likely to undergo one or more ventilation tube insertions for persistent otitis media with effusion (OME), it has been discussed over the role of grommet insertion as an iatrogenic cause of secondary cholesteatoma. Vlastarakos et al. [5] in a study on complications associated with ventilation tube insertion reported an incidence of secondary cholesteatoma around 1%.
V. Vincenti et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 918–922
If untreated, middle ear cholesteatoma can lead to facial palsy, labyrinthine fistula with sensorineural hearing loss as well as endocranial complications; until novel medical management emerges, surgical treatment is our only tool to avoid such complications. Historically, a controversy exists in the literature about the optimal management of pediatric cholesteatomas: some surgeons have advocated the canal wall up (CWU) technique [6,7], others have preferred the canal wall down (CWD) procedure [8,9], and a number of surgeons have suggested hybrid and reconstructive techniques [10,11]. Many surgeons believe that most cholesteatomas can be managed with a CWU technique and consider relative contraindications to this procedure a poor ET function, only hearing ear, labyrinthine fistula, and contracted mastoid [12]. Since ET dysfunction is an almost universal finding of CP, children with this facial malformation do not seem ideal candidates to a CWU procedure. On the other hand, it has been demonstrated that ET function improves with increasing age, facial skeletal growth and after CP repair, reaching normal levels by the age of 10 years [13]. Furthermore, there is some evidence that results of tympanoplasty in patients with CP are similar to those in patients without CP [14,15]. To our knowledge, no study has addressed specifically the results of surgical treatment of cholesteatoma in children with CP. In this study, we retrospectively reviewed our experience with acquired middle ear cholesteatoma in children with a history of CP repair and described the long-term anatomical and functional results. 2. Materials and methods Our otological database was used to search out all children (0– 16 years) diagnosed with cleft palate who underwent surgery for acquired middle ear cholesteatoma at the Department of Otolaryngology of the University of Parma between January 2000 and September 2007. The study protocol was approved by the institutional review board and an informed written consent was obtained from all patients. Cholesteatoma was diagnosed otoscopically and confirmed by high resolution computed tomography in all children. Medical reports were analyzed by age, sex, history of ventilation tube insertion, cholesteatoma location and extension, status of the contralateral ear, surgical procedure adopted, preoperative and postoperative audiograms. Cholesteatoma recidivism, ears with CWU mastoidectomy later requiring conversion to CWD mastoidectomy, postoperative complications, and hearing levels were the main outcomes measured. Hearing results were evaluated according to guidelines set forth by the Committee on Hearing and Equilibrium of the American Academy of Otolaryngology and Neck Surgery for the evaluation of results of treatment of conductive hearing loss [16].
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Pure-tone average (PTA) was calculated as the mean of 500, 1000, 2000, and 3000 Hz thresholds. The air-bone gap was reported as the four-tone PTA for air-conduction and bone-conduction values determined at the same time. Hearing results were determined at the last follow-up. In our department the surgical procedure of choice for the treatment of childhood cholesteatoma is CWU mastoidectomy; in most cases a second-look procedure is performed 12–18 months after the first surgery as it is considered an integral part of CWU mastoidectomy for the detection and removal of residual cholesteatoma. In all patients of this series, when a CWU mastoidectomy had been chosen, the first-stage operation consisted in the exposure of the bony external canal and mastoid by retroauricular approach, mastoidectomy, atticotomy and posterior tympanotomy. The removal of the cholesteatoma was performed using a combined approach (transcanal–transmastoid) with the support of chemically assisted dissection, as previously described [17,18]. At the end of the extirpation of the disease, a silastic sheet with a projection into the ET was placed through the epitympanum and posterior tympanotomy to completely cover the medial wall of the middle ear and the mastoid. Defects of the postero-superior canal wall were repaired using bone pate` and myringoplasty was performed using the temporalis fascia. The second-stage procedure was performed at least 12 months after the first-stage operation using the same facial recess approach created during the primary surgery. Following the silastic sheet removal, the exploration of all the cavities of the middle ear and mastoid was performed using the same combined technique as in the first surgical step. In all cases small micromirrors or 308 or 708 angled endoscopes were used to search for residual disease. Residual cholesteatoma was defined as a collection of squamous epithelium found in the middle ear considered to have developed from epidermal debris left inadvertently in place during the first stage of surgery. 3. Results A total of 18 children were identified and included in this study: 11 boys and 7 girls. Ten of these 18 patients belonged to the group of 293 children treated and followed up at our Department; thus, the incidence of cholesteatoma in our group of cleft patients was 3.3%. The remaining 8 children were referred to our department from other hospitals. Twelve (60%) out of the 18 patients developed a cholesteatoma despite one or more ventilation tube insertion (Table 1), while the remaining 6 (all of these subjects belonged to the group of patients referred to us from other hospitals) had a cholesteatoma with a negative history for ventilation tube insertion
Table 1 Relevant patient demographic data, cholesteatoma extension, type of surgery and postoperative complications in patients with a positive history for ventilation tube insertion. Patient
Age/sex
Cholesteatoma extension
Number of VTI
Surgical technique
Postoperative complications
1 2 3 4 5 7 9 11 13 14 15 18
6/M 11/F 13/M 9/M 8/M 16/F 10/M 10/M 11//F 12/F 9/M 7/M
R E E,A,M E,R,A,M E,A,M R E,R,A,M E,A,M E,A,M E,R,A,M E,R,A,M E
1 1 2 1 1 2 1 2 2 1 1 1
Staged CWUM Staged CWUM Staged CWUM Staged CWUM Staged CWUM Staged CWUM Staged CWUM Staged CWUM CWDM CWDM CWDM MBT
None None RC requiring CWDM None None RC requiring CWDM None None None Otorrhea requiring revision None None
R: retrotympanum; E: epitympanum; A: antrum; M: mastoid; CWUM: canal wall up mastoidectomy; CWDM: canal wall down mastoidectomy; MBT: modified Bondy technique; VTI: ventilation tube insertion; RC: recurrent cholesteatoma.
V. Vincenti et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 918–922
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Table 2 Relevant patient demographic data, cholesteatoma extension, type of surgery and postoperative complications in patients with a negative history for ventilation tube insertion. Patient
Age/sex
Cholesteatoma extension
Surgical technique
Postoperative complications
6 8 10 12 16 17
12/F 11/M 8/F 14/M 8/F 10/M
E,A.M E,A,M E,R,A,M E,R,A,M E,A,M E
Staged CWUM Staged CWUM Staged CWUM Staged CWUM CWDM MBT
None None None None None None
R: retrotympanum; E: epitympanum; A: antrum; M: mastoid; CWUM: canal wall up mastoidectomy; CWDM: canal wall down mastoidectomy; MBT: modified Bondy technique. Table 3 Relevant patient demographic data, type of surgery and postoperative complications in patients with a normal contralateral ear. Patient
Age/sex
Surgical technique
Postoperative complications
1 2 4 5 6 9 11 12 13 15 16 17
6/M 11/F 9/M 8/M 12/F 10/M 10/M 14/M 11//F 9/M 8/F 10/M
Staged CWUM Staged CWUM Staged CWUM Staged CWUM Staged CWUM Staged CWUM Staged CWUM Staged CWUM CWDM CWDM CWDM MBT
None None None None None None None None None None None None
was found and removed during the second-look procedure in 2 ears (16.6%). The only postoperative complication observed in children treated with CWD mastoidectomy was granulation tissue in the mastoid cavity causing recurrent episodes of otorrhea resistant to the medical therapy; for this reason, a revision mastoidectomy was performed. No complications were observed in patients who underwent modified Bondy technique. Results comparing preoperative and postoperative hearing are shown in Tables 5 and 6. The mean preoperative to postoperative four-tone air-bone change was as follows: with CWU mastoidectomy, an improvement from 32.9 to 15.4 dB; with CWD mastoidectomy an improvement from 33.7 to 30 dB; and with Bondy technique air-bone gap remained unchanged. No cases of sensorineural hearing loss were observed.
CWUM: canal wall up mastoidectomy; CWDM: canal wall down mastoidectomy; MBT: modified Bondy technique.
4. Discussion
(Table 2). The median age at the time of the first surgery was 10.7 years (range, 6–16 years). Ten children had a CP and 8 had a combination of cleft lip and palate. All patients had fresh primary acquired middle ear cholesteatomas. The average follow-up time was 8 years (range 5 to 12 years). No patient was lost and adherence to the long-term follow-up was facilitated by the presence in our Department of a reference center with a multidisciplinary team treating the various rehabilitative aspects of children with cleft lip and palate. The location of the cholesteatoma was as follows: only in the epitympanum in 3 patients, in epitympanum, antrum and mastoid in 7 children, only in the retrotympanum in 2 cases, retrotympanum, epitympanum and mastoid in 6 children. The ossicular chain was intact in 2 ears with small epitympanic cholesteatomas and interrupted in all remaining 16 ears; among these 16 cases, 10 had intact stapes. Contralateral ear was normal in 12 children; four subjects presented a retraction pocket and 2 a tympanic membrane perforation (Tables 3 and 4). In 2 children with epitympanic cholesteatoma and intact tympano-ossicular system, a modified Bondy technique was performed. Twelve patients underwent staged CWU mastoidectomy and 4 had a CWD procedure because of extensive disease with large erosion of the posterosuperior canal wall. Among the 12 children who underwent staged CWU mastoidectomy, 2 developed a recurrent cholesteatoma, respectively one and three years after surgery, and required conversion to CWD mastoidectomy. A residual cholesteatoma
Several clinical and anatomic studies have showed the impact of CP on the incidence of OME as a consequence of a nearly universal ET dysfunction [19–21]. There is wide international variation in the protocols used for OME management in children with CP. Some authors have suggested that all cleft patients should have routine ventilation tubes insertion with the rationale of preventing otologic complications [22]. In agreement with others authors [23], we prefer a more conservative approach in which ventilation tubes are reserved only for patients with recurrent middle ear infections or significant hearing loss. Despite surgical correction of CP and early treatment of OME, structural changes of the tympanic membrane progress until to the development of retraction pockets and cholesteatomas in a percentage of patients up to 9.2% [3]. There are many studies in the literature focusing on the outcomes of treatment of OME in children with CP, but little data have been published regarding the surgical treatment of middle ear cholesteatoma in such patients. Vartiainen [14] and Gardner and Dornhoffer [15] reported on results of surgery for chronic otitis media in patients with CP; both studies included patients operated on for a variety of diagnoses (tympanic perforation, atelectatic ear, acquired and congenital cholesteatoma, and tympanosclerosis) but specific results with regard to cholesteatoma management were not reported. Based on their results, the authors [14,15] stated that ET dysfunction in CP patients improves at some point after repair of the palate and
Table 4 Relevant patient demographic data, type of surgery and postoperative complications in patients with a diseased contralateral ear. Pt
Age/sex
Contralateral ear
Surgical technique
Postoperative complications
3 7 8 10 14 18
13/M 16/F 11/M 8/F 12/F 7/M
RP RP TM perforation RP RP TM perforation
Staged CWUM Staged CWUM Staged CWUM Staged CWUM CWDM MBT
RC requiring CWDM RC requiring CWDM None None Otorrhea requiring revision None
RP: retraction pocket; TM: tympanic membrane; CWUM: canal wall up mastoidectomy; CWDM: canal wall down mastoidectomy; MBT: modified Bondy technique; RC: recurrent cholesteatoma.
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Table 5 Pure tone averages and air bone gap preoperatively, and at last follow-up, in canal wall up mastoidectomy. Patient
Preoperative BC PTA (dB)
Postoperative BC PTA (dB)
Preoperative AC PTA (dB)
Postoperative AC PTA (dB)
Preoperative ABG (dB)
Postoperative ABG (dB)
ABG gain (dB)
1 2 3 4 5 6 7 8 9 10 11 12
10 20 20 20 25 20 10 15 10 20 10 10
10 20 20 20 25 20 10 15 10 20 10 10
40 55 60 50 60 65 50 40 30 50 45 40
20 40 50 45 40 35 50 15 20 30 10 20
30 35 40 30 35 45 40 25 20 30 35 30
10 20 30 25 15 15 40 0 10 10 0 10
20 15 10 5 20 30 0 25 10 20 35 20
BC: bone conduction; AC: air conduction; PTA: pure tone average; ABG: air bone gap.
Table 6 Pure tone averages and air bone gap preoperatively, and at last follow-up, in open techniques. Patient
Surgical technique
Preoperative BC PTA (dB)
Postoperative BC PTA (dB)
Preoperative AC PTA (dB)
Postoperative AC PTA (dB)
Preoperative ABG (dB)
Postoperative ABG (dB)
ABG gain (dB)
13 14 15 16 17 18
CWDM CWDM CWDM CWDM MBT MBT
10 15 10 20 10 10
10 15 10 20 10 10
45 55 40 50 20 25
35 55 35 50 20 25
35 40 30 30 10 15
25 40 25 30 10 15
10 0 5 0 0 0
BC: bone conduction; AC: air conduction; PTA: pure tone average; ABG: air bone gap; CWDM: canal wall down mastoidectomy; MBT: modified Bondy technique.
concluded that middle ear surgery should be offered to the patients with a CP with the same indications as in other patients with chronic otitis media. An interesting study on chronic ear surgery in patients with various syndromes and congenital malformations (Down syndrome, Turner syndrome, conotruncal cardiac defects, Pierre Robin syndrome, etc.) all characterized by ET dysfunction, has been published by O’Malley et al. [24] in 2007. Of the 43 surgeries involving mastoidectomy, intact canal wall surgery was performed in 22 cases (51%); of these, only 2 (9%) required a subsequent procedure that involved removal of the canal wall. The authors [24] stated that the use of canal down techniques should not be regarded as mandatory and suggested that canal wall preservation techniques should be applied in appropriately selected patients with congenital malformations associated to ET dysfunction. Our criteria for choosing the surgical technique in the management of acquired middle ear cholesteatoma in children with CP were the same used in the general pediatric population and remained constant over the study period. CWU mastoidectomy represents our preferred approach to pediatric cholesteatoma; a second-look surgery is considered an integral part of the technique allowing the detection and the removal of residual disease as well as the reconstruction of the ossicular chain in a stabilized ear. In presence of irreparable erosions of the posterosuperior canal wall, dead ear, labyrinthine fistula, contracted mastoid or, if the child is judged either unable or unwilling to collaborate with adequate follow-up, we prefer a one-stage CWD mastoidectomy. In presence of epitympanic cholesteatoma with an intact tympano-ossicular system, an air bone gap < 25 dB, and mesotympanum free of disease we perform a modified Bondy technique, as described by Sanna et al. [25]. Consistent with the notion that ET function improves with age and after CP repair to such an extent as to allow to take the canal wall up, 10 patients (83.3%) out of a total 12 operated using staged CWU mastoidectomy were found to have a safe and dry ear in a follow-up period of at least 5 years. Reconstruction of even minimal loss of substance of the postero-superior canal wall using autogenous bone pate` graft may have contributed to the
prevention of attic retraction and recurrence of cholesteatoma [26]. Interestingly, both patients who experienced a recurrent cholesteatoma after CWU mastoidectomy, had a retraction pocket in their contralateral ear, despite a history of repeated ventilation tube insertions. On the contrary, among the ten children without recurrent disease after surgery, only one had a retraction pocket in the contralateral ear. Although contradictory results have been reported about the significance of the contralateral ear status in predicting tympanoplasty success, according to other authors [27,28], we believe that evidence of contralateral ET dysfunction (i.e., persistent OME despite repeated ventilation tube insertions, atelectasis and retraction pocket) may represent a negative prognostic factor and has to be considered in the choice of surgical technique either demolitive or conservative. Both open procedures, the classic CWD mastoidectomy and the Bondy technique, allowed to obtain a safe and dry ear with a single surgery in all but one case; yet, restriction on water exposure and the necessity of periodic cleaning of open mastoid cavities were not well accepted by the children, especially owing to their difficulty to cope with lifelong aural toilets. Similarly to other studies addressing hearing results of chronic ear surgery in CP patients [14,15] we found that CWU mastoidectomy was characterized by favorable audiological outcomes in the vast majority of patients; the mean air-bone gap improved from a preoperative value of 32.9 dB to a postoperative value of 15.4 dB and a socially serviceable hearing (PTA 30 dB) was observed in 83% of cases. Satisfying hearing results were achieved also with the CWD procedures. Although the number of patients of the present study is too small to establish some guidelines, our results showed that middle ear cholesteatoma in children with CP can be approached using the same criteria used in the general pediatric population. The majority of patients can be treated by using a CWU procedure with curative and functional results similar to those of children without CP; the high rate of success of closed technique indirectly supports the notion that ET function improves with age and after tympanoplasty. CWD mastoidectomy should be used in presence
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of extensive disease with unreconstructable erosion of the posterior canal wall and if a recurrent cholesteatoma occurs. The modified Bondy is an appropriate, one-stage surgical technique when attempting to preserve preoperative hearing in epitympanic cholesteatomas with intact pars tensa and ossicular chain and mesotympanum free of disease. References [1] L.A. Harker, Cholesteatoma: an incidence study, in: Cholesteatoma: First International Conference, Aesculapius Publishing Company, Birmingham, AL, 1977, pp. 308–309. [2] J.L. Goldman, S.A. Martinez, T.M. Ganzel, Eustachian tube dysfunction and its sequelae in patients with cleft palate, South. Med. J. 86 (1993) 1236–1237. [3] S. Dominguez, L.A. Harker, Incidence of cholesteatoma with cleft palate, Ann. Otol. Rhinol. Laryngol. 97 (1988) 659–660. [4] A.L. James, N.K. Chadha, B.C. Papsin, T.I. Stockley, Pediatric cholesteatoma and variants in the gene encoding connexin 26, Laryngoscope 120 (2010) 183–187. [5] P.V. Vlastarakos, T.P. Nikolopoulos, S. Korres, E. Tavoulari, A. Tzagaroulakis, E. Ferekidis, Grommets in otitis media with effusion: the most frequent operation in children. But it is associated with significant complications, Eur. J. Pediatr. 166 (2007) 385–391. [6] E.E. Dodson, G.T. Hashisaki, T.C. Hobgood, P.R. Lambert, Intact canal wall mastoidectomy with tympanoplasty for cholesteatoma in children, Laryngoscope 108 (1998) 977–983. [7] S.E. Kinney, Five years experience using the intact canal wall tympanoplasty with mastoidectomy for cholesteatoma: preliminary report, Laryngoscope 92 (1982) 1395–1400. [8] K. Palva, J. Pekka, Karja, Cholesteatoma in children, Arch. Otolaryngol. 103 (1977) 74–77. [9] G.D. Smyth, Cholesteatoma surgery: the influence of the canal wall, Laryngoscope 95 (1985) 92–96. [10] M. Hatano, M. Ito, T. Yoshizaki, Retrograde mastoidectomy on demand with softwall reconstruction in pediatric cholesteatoma, Acta Otolaryngol. 130 (2010) 1113–1118. [11] R.A. Godinho, S.H. Kamil, J.N. Lubianca, I.J. Keogh, R.D. Eavey, Pediatric cholesteatoma: canal wall window alternative to canal wall down mastoidectomy, Otol. Neurotol. 26 (2005) 466–471. [12] M.A. Shirazi, K. Muzaffar, J.P. Leonetti, S. Marzo, Surgical treatment of pediatric cholesteatoma, Laryngoscope 116 (2006) 1603–1607.
[13] K. Timmermans, V. Vander Poorten, C. Desloovere, F. Debruyne, The middle ear of cleft palate patients in their early teens: a literature study and preliminary file study, B-ENT 2 (Suppl. 4) (2006) 95–101. [14] E. Vartiainen, Results of surgery of surgery for chronic otitis media in patients with a cleft palate, Clin. Otolaryngol 17 (1992) 284–286. [15] E. Gardner, J.L. Dornhoffer, Tympanoplasty results in patients with cleft palate: an age- and procedure-matched comparison of preliminary results with patients without cleft palate, Otolaryngol. Head Neck Surg. 126 (2002) 518–523. [16] Committee on hearing and equilibrium guidelines for the evaluation of results of treatment of conductive hearing loss, Otolaryngol. Head Neck Surg. 113 (1995) 186–187. [17] V. Vincenti, J. Magnan, C. Zini, Cochlear effects of intraoperative use of Mesna in cholesteatoma surgery, Acta Biomed. 85 (2014) 11–15. [18] V. Vincenti, M. Mondain, E. Pasanisi, F. Piazza, J.L. Puel, S. Bacciu, et al., Cochlear effects of mesna application into the middle ear, Ann. N. Y. Acad. Sci. 884 (1999) 425–432. [19] D. Sadler_Kimes, M.I. Siegel, J.S. Todhunter, Age-related morphologic differences in the components of the Eustachian tube/middle-ear system, Ann. Otol. Rhinol. Laryngol. 98 (1989) 854–858. [20] W.J. Doyle, E.I. Cantekin, C.D. Bluestone, Eustachian tube function in cleft palate children, Ann. Otol. Rhinol. Laryngol. Suppl. 89 (1980) 34–40. [21] Y. Tasaka, M. Kawano, I. Honjo, Eustachian tube function in OME patients with cleft palate. Special reference to the prognosis of otitis media with effusion, Acta Otolaryngol. Suppl. 471 (1990) 5–8. [22] T. Klockars, J. Rautio, Early placement of ventilation tubes in cleft lip and palate patients: does palatal closure affect tube occlusion and short-term outcome? Int. J. Pediatr. Otorhinolaryngol. 76 (2012) 1481–1484. [23] Y.S. Phua, L.J. Salkeld, T.M.B. de Chalain, Middle ear disease in children with cleft palate: protocols for management, Int. J. Pediatr. Otorhinolaryngol. 73 (2009) 307–313. [24] M.R. O’Malley, D.M. Kaylie, D.J. Van Himbergen, M.L. Bennett, C.G. Jackson, Chronic ear surgery in patients with syndromes and multiple congenital malformations, Laryngoscope 117 (2007) 1993–1998. [25] M. Sanna, M. Agarwal, T. Khrais, G. Di Trapani, Modified Bondy’s technique for epitympanic cholesteatoma, Laryngoscope 113 (2003) 2218–2221. [26] A. Bacciu, E. Pasanisi, V. Vincenti, F. Di Lella, S. Bacciu, Reconstruction of outer attic wall defects using bone pate`: long-term clinical and histological evaluation, Eur. Arch. Otorhinolaryngol. 263 (2006) 983–987. [27] A. Deguine, The controlateral ear in cholesteatoma, Rev. Laryngol. Otol. Rhinol. 111 (1990) 463–468. [28] W.O. Collins, F.F. Telischi, T.J. Balkany, C.A. Buchman, Pediatric tympanoplasty. Effect of contralateral ear status on outcomes, Arch. Otolaryngol. Head Neck Surg. 129 (2003) 646–651.
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Acquired middle ear cholesteatoma in children with cleft palate: Experience from 18 surgical cases Vincenzo Vincenti *, Francesca Marra, Barbara Bertoldi, Daniela Tonni, Maria Silvia Saccardi, Salvatore Bacciu, Enrico Pasanisi Department of Clinical and Experimental Medicine, Unit of Audiology and Pediatric Otorhinolaryngology, University of Parma, Italy
A R T I C L E I N F O
A B S T R A C T
Article history: Received 2 November 2013 Received in revised form 5 March 2014 Accepted 6 March 2014 Available online 15 March 2014
Objectives: To review an institutional experience with the surgical management of middle ear cholesteatoma in children with cleft palate. Materials and methods: We analyzed retrospectively 18 children diagnosed with cleft palate who underwent surgery for acquired middle ear cholesteatoma between 2000 and 2007. The following data were recorded: age, sex, history of ventilation tube insertion, status of the contralateral ear, cholesteatoma location and extension, and surgical technique involved. Cholesteatoma recidivism, stable mastoid cavity and hearing levels were the main outcomes measured. Results: Follow-up ranged from 5 to 12 years (mean 8 years). Twelve children underwent planned staged canal wall up mastoidectomy: a residual cholesteatoma was found and removed during the second-look procedure in 2 ears (16.6%); two children (16.6%) showed a recurrent cholesteatoma and required conversion to canal wall down mastoidectomy. A modified Bondy technique was chosen in two children with an epitympanic cholesteatoma with an intact tympano-ossicular system, while in the remaining four subjects a canal wall down mastoidectomy was performed because of an irreparable erosion of the postero-superior canal wall: no cases of recurrent cholesteatoma were observed in these 6 children; revision mastoidectomy was needed in one patient for cavity granulation. A postoperative air-bone gap result of 0–20 dB was achieved in 11 children (61.1%); in 5 cases (27.7%) postoperative air-bone gap was between 21 and 30 dB, while in 2 (11.1%) was >30 dB. Bone conduction thresholds remained unaffected in all cases. Conclusions: Our results indicate that most cleft palate children with cholesteatoma can be managed with a canal wall up mastoidectomy with low complication rates. In extensive disease with large erosion of the canal wall as well in presence of a retraction pocket in the contralateral ear, a canal wall down mastoidectomy should be considered. In epitympanic cholesteatomas with an intact tympano-ossicular system and mesotympanum free of disease, the modified Bondy procedure is an effective surgical option. As in the general pediatric population, improvement or preservation of hearing can be obtained in most patients. ß 2014 Elsevier Ireland Ltd. All rights reserved.
Keywords: Cholesteatoma Cleft palate Middle ear surgery Tympanoplasty Mastoidectomy Hearing results
1. Introduction The incidence of cholesteatoma in childhood has been reported at 3–6 per 100,000 [1], while in children with cleft palate (CP) is much higher, ranging from 1.8% [2] to 9.2% [3].
* Corresponding author at: Department of Clinical and Experimental Medicine, Unit of Audiology and Pediatric Otorhinolaryngology, University of Parma, Via Gramsci 14, 43126 Parma, Italy. Tel.: +39 0521 703204; fax: +39 0521 703788. E-mail address: [email protected] (V. Vincenti). http://dx.doi.org/10.1016/j.ijporl.2014.03.007 0165-5876/ß 2014 Elsevier Ireland Ltd. All rights reserved.
A poor Eustachian tube (ET) function leading to reduced middle ear pressure and tympanic membrane retraction contributes to the elevated incidence of acquired cholesteatoma in these children. Beyond the traditional explanation of ET dysfunction, potential genetic contribution to the pathogenesis of cholesteatoma has also been suggested [4]. In addition, since children with CP are more likely to undergo one or more ventilation tube insertions for persistent otitis media with effusion (OME), it has been discussed over the role of grommet insertion as an iatrogenic cause of secondary cholesteatoma. Vlastarakos et al. [5] in a study on complications associated with ventilation tube insertion reported an incidence of secondary cholesteatoma around 1%.
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If untreated, middle ear cholesteatoma can lead to facial palsy, labyrinthine fistula with sensorineural hearing loss as well as endocranial complications; until novel medical management emerges, surgical treatment is our only tool to avoid such complications. Historically, a controversy exists in the literature about the optimal management of pediatric cholesteatomas: some surgeons have advocated the canal wall up (CWU) technique [6,7], others have preferred the canal wall down (CWD) procedure [8,9], and a number of surgeons have suggested hybrid and reconstructive techniques [10,11]. Many surgeons believe that most cholesteatomas can be managed with a CWU technique and consider relative contraindications to this procedure a poor ET function, only hearing ear, labyrinthine fistula, and contracted mastoid [12]. Since ET dysfunction is an almost universal finding of CP, children with this facial malformation do not seem ideal candidates to a CWU procedure. On the other hand, it has been demonstrated that ET function improves with increasing age, facial skeletal growth and after CP repair, reaching normal levels by the age of 10 years [13]. Furthermore, there is some evidence that results of tympanoplasty in patients with CP are similar to those in patients without CP [14,15]. To our knowledge, no study has addressed specifically the results of surgical treatment of cholesteatoma in children with CP. In this study, we retrospectively reviewed our experience with acquired middle ear cholesteatoma in children with a history of CP repair and described the long-term anatomical and functional results. 2. Materials and methods Our otological database was used to search out all children (0– 16 years) diagnosed with cleft palate who underwent surgery for acquired middle ear cholesteatoma at the Department of Otolaryngology of the University of Parma between January 2000 and September 2007. The study protocol was approved by the institutional review board and an informed written consent was obtained from all patients. Cholesteatoma was diagnosed otoscopically and confirmed by high resolution computed tomography in all children. Medical reports were analyzed by age, sex, history of ventilation tube insertion, cholesteatoma location and extension, status of the contralateral ear, surgical procedure adopted, preoperative and postoperative audiograms. Cholesteatoma recidivism, ears with CWU mastoidectomy later requiring conversion to CWD mastoidectomy, postoperative complications, and hearing levels were the main outcomes measured. Hearing results were evaluated according to guidelines set forth by the Committee on Hearing and Equilibrium of the American Academy of Otolaryngology and Neck Surgery for the evaluation of results of treatment of conductive hearing loss [16].
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Pure-tone average (PTA) was calculated as the mean of 500, 1000, 2000, and 3000 Hz thresholds. The air-bone gap was reported as the four-tone PTA for air-conduction and bone-conduction values determined at the same time. Hearing results were determined at the last follow-up. In our department the surgical procedure of choice for the treatment of childhood cholesteatoma is CWU mastoidectomy; in most cases a second-look procedure is performed 12–18 months after the first surgery as it is considered an integral part of CWU mastoidectomy for the detection and removal of residual cholesteatoma. In all patients of this series, when a CWU mastoidectomy had been chosen, the first-stage operation consisted in the exposure of the bony external canal and mastoid by retroauricular approach, mastoidectomy, atticotomy and posterior tympanotomy. The removal of the cholesteatoma was performed using a combined approach (transcanal–transmastoid) with the support of chemically assisted dissection, as previously described [17,18]. At the end of the extirpation of the disease, a silastic sheet with a projection into the ET was placed through the epitympanum and posterior tympanotomy to completely cover the medial wall of the middle ear and the mastoid. Defects of the postero-superior canal wall were repaired using bone pate` and myringoplasty was performed using the temporalis fascia. The second-stage procedure was performed at least 12 months after the first-stage operation using the same facial recess approach created during the primary surgery. Following the silastic sheet removal, the exploration of all the cavities of the middle ear and mastoid was performed using the same combined technique as in the first surgical step. In all cases small micromirrors or 308 or 708 angled endoscopes were used to search for residual disease. Residual cholesteatoma was defined as a collection of squamous epithelium found in the middle ear considered to have developed from epidermal debris left inadvertently in place during the first stage of surgery. 3. Results A total of 18 children were identified and included in this study: 11 boys and 7 girls. Ten of these 18 patients belonged to the group of 293 children treated and followed up at our Department; thus, the incidence of cholesteatoma in our group of cleft patients was 3.3%. The remaining 8 children were referred to our department from other hospitals. Twelve (60%) out of the 18 patients developed a cholesteatoma despite one or more ventilation tube insertion (Table 1), while the remaining 6 (all of these subjects belonged to the group of patients referred to us from other hospitals) had a cholesteatoma with a negative history for ventilation tube insertion
Table 1 Relevant patient demographic data, cholesteatoma extension, type of surgery and postoperative complications in patients with a positive history for ventilation tube insertion. Patient
Age/sex
Cholesteatoma extension
Number of VTI
Surgical technique
Postoperative complications
1 2 3 4 5 7 9 11 13 14 15 18
6/M 11/F 13/M 9/M 8/M 16/F 10/M 10/M 11//F 12/F 9/M 7/M
R E E,A,M E,R,A,M E,A,M R E,R,A,M E,A,M E,A,M E,R,A,M E,R,A,M E
1 1 2 1 1 2 1 2 2 1 1 1
Staged CWUM Staged CWUM Staged CWUM Staged CWUM Staged CWUM Staged CWUM Staged CWUM Staged CWUM CWDM CWDM CWDM MBT
None None RC requiring CWDM None None RC requiring CWDM None None None Otorrhea requiring revision None None
R: retrotympanum; E: epitympanum; A: antrum; M: mastoid; CWUM: canal wall up mastoidectomy; CWDM: canal wall down mastoidectomy; MBT: modified Bondy technique; VTI: ventilation tube insertion; RC: recurrent cholesteatoma.
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Table 2 Relevant patient demographic data, cholesteatoma extension, type of surgery and postoperative complications in patients with a negative history for ventilation tube insertion. Patient
Age/sex
Cholesteatoma extension
Surgical technique
Postoperative complications
6 8 10 12 16 17
12/F 11/M 8/F 14/M 8/F 10/M
E,A.M E,A,M E,R,A,M E,R,A,M E,A,M E
Staged CWUM Staged CWUM Staged CWUM Staged CWUM CWDM MBT
None None None None None None
R: retrotympanum; E: epitympanum; A: antrum; M: mastoid; CWUM: canal wall up mastoidectomy; CWDM: canal wall down mastoidectomy; MBT: modified Bondy technique. Table 3 Relevant patient demographic data, type of surgery and postoperative complications in patients with a normal contralateral ear. Patient
Age/sex
Surgical technique
Postoperative complications
1 2 4 5 6 9 11 12 13 15 16 17
6/M 11/F 9/M 8/M 12/F 10/M 10/M 14/M 11//F 9/M 8/F 10/M
Staged CWUM Staged CWUM Staged CWUM Staged CWUM Staged CWUM Staged CWUM Staged CWUM Staged CWUM CWDM CWDM CWDM MBT
None None None None None None None None None None None None
was found and removed during the second-look procedure in 2 ears (16.6%). The only postoperative complication observed in children treated with CWD mastoidectomy was granulation tissue in the mastoid cavity causing recurrent episodes of otorrhea resistant to the medical therapy; for this reason, a revision mastoidectomy was performed. No complications were observed in patients who underwent modified Bondy technique. Results comparing preoperative and postoperative hearing are shown in Tables 5 and 6. The mean preoperative to postoperative four-tone air-bone change was as follows: with CWU mastoidectomy, an improvement from 32.9 to 15.4 dB; with CWD mastoidectomy an improvement from 33.7 to 30 dB; and with Bondy technique air-bone gap remained unchanged. No cases of sensorineural hearing loss were observed.
CWUM: canal wall up mastoidectomy; CWDM: canal wall down mastoidectomy; MBT: modified Bondy technique.
4. Discussion
(Table 2). The median age at the time of the first surgery was 10.7 years (range, 6–16 years). Ten children had a CP and 8 had a combination of cleft lip and palate. All patients had fresh primary acquired middle ear cholesteatomas. The average follow-up time was 8 years (range 5 to 12 years). No patient was lost and adherence to the long-term follow-up was facilitated by the presence in our Department of a reference center with a multidisciplinary team treating the various rehabilitative aspects of children with cleft lip and palate. The location of the cholesteatoma was as follows: only in the epitympanum in 3 patients, in epitympanum, antrum and mastoid in 7 children, only in the retrotympanum in 2 cases, retrotympanum, epitympanum and mastoid in 6 children. The ossicular chain was intact in 2 ears with small epitympanic cholesteatomas and interrupted in all remaining 16 ears; among these 16 cases, 10 had intact stapes. Contralateral ear was normal in 12 children; four subjects presented a retraction pocket and 2 a tympanic membrane perforation (Tables 3 and 4). In 2 children with epitympanic cholesteatoma and intact tympano-ossicular system, a modified Bondy technique was performed. Twelve patients underwent staged CWU mastoidectomy and 4 had a CWD procedure because of extensive disease with large erosion of the posterosuperior canal wall. Among the 12 children who underwent staged CWU mastoidectomy, 2 developed a recurrent cholesteatoma, respectively one and three years after surgery, and required conversion to CWD mastoidectomy. A residual cholesteatoma
Several clinical and anatomic studies have showed the impact of CP on the incidence of OME as a consequence of a nearly universal ET dysfunction [19–21]. There is wide international variation in the protocols used for OME management in children with CP. Some authors have suggested that all cleft patients should have routine ventilation tubes insertion with the rationale of preventing otologic complications [22]. In agreement with others authors [23], we prefer a more conservative approach in which ventilation tubes are reserved only for patients with recurrent middle ear infections or significant hearing loss. Despite surgical correction of CP and early treatment of OME, structural changes of the tympanic membrane progress until to the development of retraction pockets and cholesteatomas in a percentage of patients up to 9.2% [3]. There are many studies in the literature focusing on the outcomes of treatment of OME in children with CP, but little data have been published regarding the surgical treatment of middle ear cholesteatoma in such patients. Vartiainen [14] and Gardner and Dornhoffer [15] reported on results of surgery for chronic otitis media in patients with CP; both studies included patients operated on for a variety of diagnoses (tympanic perforation, atelectatic ear, acquired and congenital cholesteatoma, and tympanosclerosis) but specific results with regard to cholesteatoma management were not reported. Based on their results, the authors [14,15] stated that ET dysfunction in CP patients improves at some point after repair of the palate and
Table 4 Relevant patient demographic data, type of surgery and postoperative complications in patients with a diseased contralateral ear. Pt
Age/sex
Contralateral ear
Surgical technique
Postoperative complications
3 7 8 10 14 18
13/M 16/F 11/M 8/F 12/F 7/M
RP RP TM perforation RP RP TM perforation
Staged CWUM Staged CWUM Staged CWUM Staged CWUM CWDM MBT
RC requiring CWDM RC requiring CWDM None None Otorrhea requiring revision None
RP: retraction pocket; TM: tympanic membrane; CWUM: canal wall up mastoidectomy; CWDM: canal wall down mastoidectomy; MBT: modified Bondy technique; RC: recurrent cholesteatoma.
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Table 5 Pure tone averages and air bone gap preoperatively, and at last follow-up, in canal wall up mastoidectomy. Patient
Preoperative BC PTA (dB)
Postoperative BC PTA (dB)
Preoperative AC PTA (dB)
Postoperative AC PTA (dB)
Preoperative ABG (dB)
Postoperative ABG (dB)
ABG gain (dB)
1 2 3 4 5 6 7 8 9 10 11 12
10 20 20 20 25 20 10 15 10 20 10 10
10 20 20 20 25 20 10 15 10 20 10 10
40 55 60 50 60 65 50 40 30 50 45 40
20 40 50 45 40 35 50 15 20 30 10 20
30 35 40 30 35 45 40 25 20 30 35 30
10 20 30 25 15 15 40 0 10 10 0 10
20 15 10 5 20 30 0 25 10 20 35 20
BC: bone conduction; AC: air conduction; PTA: pure tone average; ABG: air bone gap.
Table 6 Pure tone averages and air bone gap preoperatively, and at last follow-up, in open techniques. Patient
Surgical technique
Preoperative BC PTA (dB)
Postoperative BC PTA (dB)
Preoperative AC PTA (dB)
Postoperative AC PTA (dB)
Preoperative ABG (dB)
Postoperative ABG (dB)
ABG gain (dB)
13 14 15 16 17 18
CWDM CWDM CWDM CWDM MBT MBT
10 15 10 20 10 10
10 15 10 20 10 10
45 55 40 50 20 25
35 55 35 50 20 25
35 40 30 30 10 15
25 40 25 30 10 15
10 0 5 0 0 0
BC: bone conduction; AC: air conduction; PTA: pure tone average; ABG: air bone gap; CWDM: canal wall down mastoidectomy; MBT: modified Bondy technique.
concluded that middle ear surgery should be offered to the patients with a CP with the same indications as in other patients with chronic otitis media. An interesting study on chronic ear surgery in patients with various syndromes and congenital malformations (Down syndrome, Turner syndrome, conotruncal cardiac defects, Pierre Robin syndrome, etc.) all characterized by ET dysfunction, has been published by O’Malley et al. [24] in 2007. Of the 43 surgeries involving mastoidectomy, intact canal wall surgery was performed in 22 cases (51%); of these, only 2 (9%) required a subsequent procedure that involved removal of the canal wall. The authors [24] stated that the use of canal down techniques should not be regarded as mandatory and suggested that canal wall preservation techniques should be applied in appropriately selected patients with congenital malformations associated to ET dysfunction. Our criteria for choosing the surgical technique in the management of acquired middle ear cholesteatoma in children with CP were the same used in the general pediatric population and remained constant over the study period. CWU mastoidectomy represents our preferred approach to pediatric cholesteatoma; a second-look surgery is considered an integral part of the technique allowing the detection and the removal of residual disease as well as the reconstruction of the ossicular chain in a stabilized ear. In presence of irreparable erosions of the posterosuperior canal wall, dead ear, labyrinthine fistula, contracted mastoid or, if the child is judged either unable or unwilling to collaborate with adequate follow-up, we prefer a one-stage CWD mastoidectomy. In presence of epitympanic cholesteatoma with an intact tympano-ossicular system, an air bone gap < 25 dB, and mesotympanum free of disease we perform a modified Bondy technique, as described by Sanna et al. [25]. Consistent with the notion that ET function improves with age and after CP repair to such an extent as to allow to take the canal wall up, 10 patients (83.3%) out of a total 12 operated using staged CWU mastoidectomy were found to have a safe and dry ear in a follow-up period of at least 5 years. Reconstruction of even minimal loss of substance of the postero-superior canal wall using autogenous bone pate` graft may have contributed to the
prevention of attic retraction and recurrence of cholesteatoma [26]. Interestingly, both patients who experienced a recurrent cholesteatoma after CWU mastoidectomy, had a retraction pocket in their contralateral ear, despite a history of repeated ventilation tube insertions. On the contrary, among the ten children without recurrent disease after surgery, only one had a retraction pocket in the contralateral ear. Although contradictory results have been reported about the significance of the contralateral ear status in predicting tympanoplasty success, according to other authors [27,28], we believe that evidence of contralateral ET dysfunction (i.e., persistent OME despite repeated ventilation tube insertions, atelectasis and retraction pocket) may represent a negative prognostic factor and has to be considered in the choice of surgical technique either demolitive or conservative. Both open procedures, the classic CWD mastoidectomy and the Bondy technique, allowed to obtain a safe and dry ear with a single surgery in all but one case; yet, restriction on water exposure and the necessity of periodic cleaning of open mastoid cavities were not well accepted by the children, especially owing to their difficulty to cope with lifelong aural toilets. Similarly to other studies addressing hearing results of chronic ear surgery in CP patients [14,15] we found that CWU mastoidectomy was characterized by favorable audiological outcomes in the vast majority of patients; the mean air-bone gap improved from a preoperative value of 32.9 dB to a postoperative value of 15.4 dB and a socially serviceable hearing (PTA 30 dB) was observed in 83% of cases. Satisfying hearing results were achieved also with the CWD procedures. Although the number of patients of the present study is too small to establish some guidelines, our results showed that middle ear cholesteatoma in children with CP can be approached using the same criteria used in the general pediatric population. The majority of patients can be treated by using a CWU procedure with curative and functional results similar to those of children without CP; the high rate of success of closed technique indirectly supports the notion that ET function improves with age and after tympanoplasty. CWD mastoidectomy should be used in presence
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of extensive disease with unreconstructable erosion of the posterior canal wall and if a recurrent cholesteatoma occurs. The modified Bondy is an appropriate, one-stage surgical technique when attempting to preserve preoperative hearing in epitympanic cholesteatomas with intact pars tensa and ossicular chain and mesotympanum free of disease. References [1] L.A. Harker, Cholesteatoma: an incidence study, in: Cholesteatoma: First International Conference, Aesculapius Publishing Company, Birmingham, AL, 1977, pp. 308–309. [2] J.L. Goldman, S.A. Martinez, T.M. Ganzel, Eustachian tube dysfunction and its sequelae in patients with cleft palate, South. Med. J. 86 (1993) 1236–1237. [3] S. Dominguez, L.A. Harker, Incidence of cholesteatoma with cleft palate, Ann. Otol. Rhinol. Laryngol. 97 (1988) 659–660. [4] A.L. James, N.K. Chadha, B.C. Papsin, T.I. Stockley, Pediatric cholesteatoma and variants in the gene encoding connexin 26, Laryngoscope 120 (2010) 183–187. [5] P.V. Vlastarakos, T.P. Nikolopoulos, S. Korres, E. Tavoulari, A. Tzagaroulakis, E. Ferekidis, Grommets in otitis media with effusion: the most frequent operation in children. But it is associated with significant complications, Eur. J. Pediatr. 166 (2007) 385–391. [6] E.E. Dodson, G.T. Hashisaki, T.C. Hobgood, P.R. Lambert, Intact canal wall mastoidectomy with tympanoplasty for cholesteatoma in children, Laryngoscope 108 (1998) 977–983. [7] S.E. Kinney, Five years experience using the intact canal wall tympanoplasty with mastoidectomy for cholesteatoma: preliminary report, Laryngoscope 92 (1982) 1395–1400. [8] K. Palva, J. Pekka, Karja, Cholesteatoma in children, Arch. Otolaryngol. 103 (1977) 74–77. [9] G.D. Smyth, Cholesteatoma surgery: the influence of the canal wall, Laryngoscope 95 (1985) 92–96. [10] M. Hatano, M. Ito, T. Yoshizaki, Retrograde mastoidectomy on demand with softwall reconstruction in pediatric cholesteatoma, Acta Otolaryngol. 130 (2010) 1113–1118. [11] R.A. Godinho, S.H. Kamil, J.N. Lubianca, I.J. Keogh, R.D. Eavey, Pediatric cholesteatoma: canal wall window alternative to canal wall down mastoidectomy, Otol. Neurotol. 26 (2005) 466–471. [12] M.A. Shirazi, K. Muzaffar, J.P. Leonetti, S. Marzo, Surgical treatment of pediatric cholesteatoma, Laryngoscope 116 (2006) 1603–1607.
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