Problems in diagnosis and treatment of cholesteatoma in children
Mirza Dzinic1991, International Journal of Pediatric Otorhinolaryngology
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Abstract
Our clinical study includes 56 cases of cholesteatoma in children aged 3-14 years, treated in a 6-year period. Cholesteatoma was localized in attic (6 cases), in middle ear (6 cases), in attic plus middle ear (16 cases) and in attic + middle ear + mastoid (28 cases or 50% of the total number). In the surgical treatment combined approach tympanoplasty (intact canal wall technique) was used in 76.8%, radical tympanomastoidectomy in 16.1%, and other techniques in 7.1%. Intact ossicular chain was found in 25%. Reconstruction of the ossicular chain (including autograft, homograft and allograft material) was done in 59%, and the remaining 16% were treated by classic radical surgery. Hearing results: unchanged, 48%; improved, 45%; slightly worsened, 7%; and no dead ear. Recurrency in 31% is considerably higher as compared to 15% in adults found in another comparable study by us.
Key takeaways
- Cholesteatoma is, by its nature, a potentially dangerous disease, as in its growth it shows tendency to destroy anatomical structures of the ear.
- We analysed the group according to the distribution of cholesteatoma in the ear, its extension and aggressiveness.
- Out of these 28 cases with total cholesteatoma involvement of the middle ear and mastoid, radical tympanomastoidectomy was done in 9 cases, while in the remaining 19 a second-stage revision procedure was planned.
- We believe that the primary choice of operative technique for cholesteatoma, like for other inflammatory diseases of the middle ear, is combined approach tympanoplasty.
- If a large residual cholesteatoma is found at the second-stage operation, the third stage should be planned or the intact canal wall technique should be converted into an obliterative technique.
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Aural acquired cholesteatoma in children: Surgical findings, recurrence and functional results
International Journal of Pediatric Otorhinolaryngology, 2006
Trans-canal endoscopic ear surgery and canal wall-up tympano-mastoidectomy for pediatric middle ear cholesteatoma
European Archives of Oto-rhino-laryngology, 2019
Purpose To evaluate clinical parameters, outcomes and complications of transcanal endoscopic ear surgeries (EES) and canal wall-up tympano-mastoidectomy (CWU) for middle ear cholesteatoma in children and to compare between the two surgical approaches. Methods A retrospective chart review of all children (< 16 years) who underwent surgery for cholesteatoma involving the middle ear only with a minimal follow-up period of 12 months. Demographic features, site and extent of disease, outcome and complications were reviewed and compared between the groups. Results Thirty EES and 19 CWU were included. The overall disease relapse rates in the EES and CWU groups were 20% (n = 6, residual rate = 10%, recurrence rate = 10%) and 47% (n = 9, residual rate = 11%, recurrence rate = 37%), respectively (p = 0.04), with mean duration of follow-up of 32.6 and 37.2 months, respectively. In the EES and CWU groups, the most common site of residual disease was the mastoid cavity/antrum (n = 2, 66% and n = 2, 100%, respectively). Most recurrences involved the epitympanum and extended into the tympanic cavity (n = 2, 66%) in the EES group and into the tympanic cavity, posterior mesotympanum and mastoid cavity/antrum (n = 3, 43%, each) in the CWU group. The overall complication rates in the EES and CWU groups were 10% (n = 3) and 11% (n = 2), respectively (p = 0.61). Conclusions Endoscopic ear surgeries in children were found to be an acceptable and safe technique for the treatment of cholesteatoma limited to the middle ear cavity. A better overall success rate and a similar complication rate were found in the EES group when compared to CWU.
Acquired cholesteatoma in children: Strategies and medium-term results
European Annals of Otorhinolaryngology, Head and Neck Diseases, 2012
Objectives: To assess paediatric cholesteatoma surgical management strategies, residual disease and recurrence rates and especially the medium-term auditory impact. Material and methods: Retrospective study of 22 cases of acquired middle ear cholesteatoma selected from a series of 77 children under the age of 16 operated for cholesteatoma between 1st January 2000 and 31st December 2003 on the basis of the following criteria: first-line surgical management with postoperative follow-up greater than four years. Surgical strategies, preoperative and postoperative (at 1 year and at the final visit) audiograms and residual disease and recurrence rates were analysed. Results: A canal wall up tympanoplasty was performed in 82% of cases as first-line procedure and a canal wall down tympanoplasty was performed in 32% of cases. Residual cholesteatoma was observed in 9% of cases and recurrent disease was observed in 18% of cases. The mean preoperative hearing loss was 26 dB with an air-bone gap of 23 dB with values of 26 dB and 20 dB respectively at the end of follow-up. The majority of children were operated by two-stage canal wall up tympanoplasty. Long-term hearing results remained stable and close to preoperative values. The recurrence rate (residual disease and relapse) was low (27%), as reported in the literature.
Surgical strategy for cholesteatoma in children
Auris Nasus Larynx, 2001
Objecti6e: We reviewed our experience with childhood cholesteatoma in children under 15 years old. Based on cumulative postoperative data, we propose a modified canal-wall-up technique in conjunction with a planned, staged operation. Methods: From 1982 to 1997, 56 children with cholesteatoma (58 ears, total) underwent surgery in our department. In the early period (1982)(1983)(1984)(1985)(1986)(1987)(1988)(1989)(1990), canal wall-down mastoidectomy was performed in 52% (21 of 40 ears), and canal wall-up mastoidectomy in 48% (the remaining 19 ears). In the late period (1991)(1992)(1993)(1994)(1995)(1996)(1997), 18 ears with cholesteatoma underwent surgery. The canal-wall up mastoidectomy was performed in 89% (16 ears), and canal-wall-down mastoidectomy in the remaining 11% (two ears). Results: In the early period (1982)(1983)(1984)(1985)(1986)(1987)(1988)(1989)(1990), cholesteatoma recurred more frequently in the canal-wall-up mastoidectomy group than in the canal-wall down mastoidectomy group (53 vs. 14%). Other postoperative complications, such as erosion of the mastoid cavity, otorrhea, and perforation of the eardrum, occurred more frequently in the canal-wall-down mastoidectomy group than in the canal-wall-up mastoidectomy group. In the late period (1991)(1992)(1993)(1994)(1995)(1996)(1997), in the canal-wall-up mastoidectomy group, ten ears underwent one-stage surgery. Planned staged tympanoplasty was completed in six ears. After one-stage surgery, four of ten ears experienced residual cholesteatoma. Two of the recurrent ears had undergone planned staged tympanoplasty. As revealed by postoperative computed tomography (CT) images, 12 of 15 ears had aeration in the attic and antrum as well as in the tympanic cavity. In these cases, no attic retraction pocket formation was observed. Conclusion: Our strategy for pediatric cholesteatoma in the future is to use canal-wall-up mastoidectomy when possible. If aeration in the attic and antrum is observed by preoperative CT-scan image and no erosion in the malleus and incus exists, the one-stage surgery will be chosen. If no aeration is observed by CT-scan and/or erosion exists in the surgical findings, planned staged tympanoplasty will be necessary. This strategy allows a high incidence of aeration of the attic and antrum, and prevents the formation of the attic retraction pocket while enabling the early detection of residual cholesteatoma by means of CT.
Dealing with paediatric cholesteatoma: how we changed our management
Acta Otorhinolaryngologica Italica, 2014
SUMMARY We reviewed our series of surgeries for paediatric cholesteatoma to assess outcomes and functional results considering the extension of disease and surgical techniques. Between January 2003 and December 2009, 36 patients (range 6-14 years) were operated on for cholesteatoma. We considered the sites involved by the cholesteatoma (mastoid, antrum, attic, middle ear, Eustachian tube), surgical techniques used (intact canal wall, canal wall down) and how our habits changed over the years; moreover, we evaluated ossicular chain conditions and how we managed the ossiculoplasty. As outcomes, we considered the percentage of residual and recurrent cholesteatoma for each technique and hearing function (air bone gap closure, high frequencies bone conduction hearing loss) at follow-up. Intact canal wall was performed in 20 patients and canal wall down in 13 patients, in 9 as first surgery. In both groups, we observed improvement of the air bone gap; in the intact canal wall group, a res...
Acquired cholesteatoma in children following congenital cholesteatoma surgery
International Journal of Pediatric Otorhinolaryngology, 2011
Congenital cholesteatoma of the middle ear - uncommon clinical presentation
Vojnosanitetski pregled, 2014
Introduction. Congenital cholesteatoma of the middle ear is un uncommon and yet not well-defined disease. Only few cases of cholesteatoma in the fossa ovalis with unusual clinical presentation have been reported in medical literature. Case report. We reported a 16-year-old girl with congenital cholesteatoma in the fossa ovalis with minimal clinical presentation. A small mass was found occluding the fossa ovalis and mimicking otosclerotic process within tympanic cavity. The operation started as stapedotomy, and when the process was confirmed it converted to mastoidectomy via the retroauricular approach. Conclusion. The diagnosis of congenital cholesteatoma in children should always be considered, even if the clinical symptoms imitate other ear disorders, in our case otosclerosis.
Pediatric cholesteatoma: A retrospective review
Otolaryngology - Head and Neck Surgery, 2004
Problem: This study addressed the surgical management of pediatric cholesteatoma.Methods: The study is a 10-year retrospective review of children undergoing surgical intervention for cholesteatoma at a tertiary care pediatric hospital between July 1, 1993 and December 30, 2002.Results: During the study period 278 children underwent surgical resection of their cholesteatoma. Of these children, 221 were managed via an intact canal wall approach while the remaining 57 underwent an open cavity procedure. The overall recurrence rate in this series was 16%. Ossicular reconstruction, if necessary, was performed in a staged fashion in 89% of the intact canal wall group. Hearing results along with postoperative complications will be presented.Conclusion: Management of pediatric cholesteatoma requires a highly individualized approach that takes into account anatomic, clinical, and social factors to determine the most successful surgical treatment paradigm.Significance: The optimal treatment for pediatric cholesteatoma remains a controversial issue. Management decisions including intact canal wall versus open cavity techniques, second-look procedures, and staging ossicular reconstruction continue to be debated.Support: None reported.
Unusual cases of congenital cholesteatoma of the ear
The Journal of Laryngology & Otology, 1999
Congenital cholesteatoma may originate at various sites in the temporal bone. For example, in the petrous apex, the cerebellopontine angle, the middle ear cavity, the mastoid process or the external auditory canal. The least common site being the mastoid process. We present two cases of congenital cholesteatoma of the mastoid process, each presenting with different symptoms and at different ages. Both patients underwent surgical treatment, which confirmed the diagnosis and radiological findings.
The Bony Obliteration Tympanoplasty in Pediatric Cholesteatoma: Long-term Safety and Hygienic Results
Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology, 2015
To present the safety and hygienic results of a 5-year longitudinal study in a pediatric population undergoing surgery for extensive cholesteatoma using a canal wall up approach with bony obliteration of the mastoid and epitympanic space. Retrospective consecutive study. Thirty-three children (≤18 yr) undergoing surgery for cholesteatoma (34 ears) between 1997 and 2009. Therapeutic. Tertiary referral center. 1) Residual and recurrent cholesteatoma rates at 5-year postsurgery, 2) postoperative waterproofing and hygienic status of the ear, and 3) required operation rate to achieve the safety and hygienic goals. At 5 years no patients were lost in follow-up. This consecutive series design is rare in chronical otitis media treatment reporting. The standard residual rate at 5 years was 5.8%, representing two residual cholesteatomas in the middle ear. The standard recurrence rate at 5 years was 2.9%, representing one recurrent cholesteatoma. At 5-year follow-up all ears were free of otorr...
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Cholesteatoma in the pediatric patient
International journal of pediatric otorhinolaryngology, 1987
A review of patients with cholesteatoma at the Children's Hospital of Philadelphia from 1981 to 1986 yielded 161 children. Analysis of data from both outpatient and hospital charts revealed a higher incidence of males to females, and the peak incidence of cholesteatoma appeared to be in the 6-10 year age group. Otorrhea was the most common symptom; and on physical examination, the posterior-superior quadrant was most often affected. Both intact canal wall and open cavity procedures were employed depending upon the site and extent of disease. Seventy-six percent of patients underwent an intact canal wall or middle ear procedure initially, with the remainder requiring an open cavity procedure. Recurrent or residual cholesteatoma was found in 32% of 148 patients at the second procedure, and in 7% of 148 patients if a third procedure was necessary. Pre- and post-operative audiometric findings for 91 patients revealed 21% with decreased hearing post-surgically, 50% with no change in ...
Potential Factors for Recurrence of Cholesteatoma in Children
Acta Scientific Orthopaedics, 2021
Objective: Cholesteatoma of middle ear in children has a more aggressive development process and faster growth than that among adults. The aim of this study was to establish the dominant causes of cholesteatoma recurrence in children and to determine the optimal surgical treatment. Methods: This study describes 92 cases of cholesteatomas found on 89 children. The first group consists of 44 cases of cholesteatomas that were operated with the wall up surgical technique, and the second group consists of 48 cases operated with the wall down surgical technique. Results: Recurrence cholesteatoma was diagnosed in 18 (19.5%) patients. In cases where the wall up, surgical technique was applied cholesteatoma recurrence was diagnosed in 14 (32%) cases. By contrast, in the wall down surgical technique recurrence was found in only 4 (8%). Conclusion: Factors that can influence the appearance of recurrence are the: patient's age, type of applied surgical technique, size of mastoid process, degree of damage on the hearing chain and pathological changes in the second ear.
Cholestéatome acquis de l’enfant : stratégies et résultats à moyen terme
Annales françaises d'Oto-rhino-laryngologie et de Pathologie Cervico-faciale, 2012
Objectives: To assess paediatric cholesteatoma surgical management strategies, residual disease and recurrence rates and especially the medium-term auditory impact. Material and methods: Retrospective study of 22 cases of acquired middle ear cholesteatoma selected from a series of 77 children under the age of 16 operated for cholesteatoma between 1st January 2000 and 31st December 2003 on the basis of the following criteria: first-line surgical management with postoperative follow-up greater than four years. Surgical strategies, preoperative and postoperative (at 1 year and at the final visit) audiograms and residual disease and recurrence rates were analysed. Results: A canal wall up tympanoplasty was performed in 82% of cases as first-line procedure and a canal wall down tympanoplasty was performed in 32% of cases. Residual cholesteatoma was observed in 9% of cases and recurrent disease was observed in 18% of cases. The mean preoperative hearing loss was 26 dB with an air-bone gap of 23 dB with values of 26 dB and 20 dB respectively at the end of follow-up. The majority of children were operated by two-stage canal wall up tympanoplasty. Long-term hearing results remained stable and close to preoperative values. The recurrence rate (residual disease and relapse) was low (27%), as reported in the literature.
Evolving considerations in the surgical management of cholesteatoma in the only hearing ear
2014
To describe a contemporary, pragmatic approach to managing cholesteatoma in the only hearing ear. Study Design: Retrospective case series. Setting: Single tertiary referral center. Patients: All patients that underwent cholesteatoma surgery, having profound hearing loss in the contralateral ear. Intervention(s): Cholesteatoma surgery. Main Outcome Measure(s): Surgical strategy, preoperative and postoperative audiometric outcomes, short-and long-term complications, recidivism. Results: Twenty-eight patients met criteria, representing 0.25% of all chronic ear surgeries performed between 1970 and 2012. Patients undergoing surgery in the latter half of the study underwent intact canal wall procedures and ossicular chain reconstruction more frequently despite having similar severities of disease. All patients with inner ear fistula underwent an opencavity operation. In the early postoperative period, 86% of ears had stable or improved hearing levels, and all patients maintained preoperative bone conduction thresholds. At a mean follow-up of 48 months, 79% of patients maintained stable or improved pure tone thresholds, whereas 2 subjects experienced delayed sensorineural hearing loss and 2 experienced isolated declining speech discrimination. Notably, 3 of the latter 4 patients were diagnosed with labyrinthine fistula and had undergone radical mastoidectomy. None of the patients who received an intact canal wall tympanomastoidectomy experienced worsening bone conduction thresholds, whereas 1 subject demonstrated a delayed decline in speech discrimination and another recurred. Conclusion: It is commonly held that the radical or classic modified radical mastoidectomy is the procedure of choice when managing cholesteatoma in the only hearing ear while intact canal wall techniques are contraindicated. Over the last 20 years, we have adopted a less-rigid, functional approach favoring intact canal wall procedures in the absence of inner ear fistula rather than unequivocally committing to an open cavity. This strategy has been influenced by advancements in preoperative evaluation, increasing familiarity and refinement of closed-cavity techniques, postoperative imaging surveillance options, and the potential for cochlear implant ''salvage'' in the rare case of profound hearing loss. Based on the current series, this approach appears safe when performed by an experienced surgeon, and reliable long-term patient follow-up is maintained.
Results of cholesteatoma surgery: the influence of age
International Congress Series, 2003
Hypothesis: There is a difference in clinical characteristics, type of surgery and outcome between pediatric and adult cholesteatoma. Background: Pediatric cholesteatoma is a chronic disease with many challenges concerning diagnosis and treatment. There are no universally accepted opinions among otologists about the course, consequences and outcome of surgery for cholesteatoma in pediatric patients. There is also a difference in opinion concerning the choice of surgical technique. Methods: The patients were divided in three groups: children younger than 9 years (17), adolescents-10 to 16 years (25), and adults (265 cases). For all the children, closed tympanoplasty was performed, and reoperation or conversion to open tympanoplasty was done later if needed. Results: Post-operative audiometry had good audiological results, comparable to cholesteatoma surgery in adults. An air -bone gap up to 20 dB was achieved in 60% of children under 9 years, in 65% of children aged between 10 and 16, and in 65% of adults. During a post-operative course, retraction of neomembrane was found in 30% of younger children, 29% of adolescents, and in 9% of adults. Also, residual and recurrent cholesteatoma were more than twice as frequent in children. Reoperation was performed in 40% of children younger than 9, in 28% of children aged 10 to 16, and in 13% of adults. In half the cases of pediatric cholesteatoma reoperations, conversion to open tympanoplasty was done because of extensive disease. Conclusion: Adequate preoperative medicamentous and local treatment of cases with pediatric cholesteatoma are needed because of different sensitivity of bacteria and potential ototoxicity of antibiotics. Post-operatively functional and audiological results are comparable to adults, but during further follow-up, retraction pockets (30%) and recurrent cholesteatoma (20%) are found. Reoperation was performed in 32% children and conversion to open tympanoplasty in 25% of pediatric cholesteatoma cases. The age of the patient is a very important factor that determines the type of surgical procedure and the results of surgery for middle ear cholesteatoma. D
Atypical presentation of congenital cholesteatoma in an adult case with good hearing result
Annals of Medicine and Surgery, 2015
Introduction: Congenital cholesteatoma is thought to be caused by inadequate folding of the epidermoid formation inside the middle ear cleft. During development of the middle ear mucosa, stratified squamous epithelium accumulates in the embryonic life. Its typical appearance is a "pearl" beneath the anterosuperior quadrant of the tympanic membrane. Presentation of case: We report 28 years-old case with congenital cholesteatoma in the posterosuperior quadrant of middle ear cavity. The main complaint was the hearing loss which had developed slowly over several years. Discussion: The case was surgically treated. Postoperative hearing result was satisfactory. Conclusion: Congenital cholesteatoma may occur in atypical locations and ages. Many authors prefer canal wall down tympanomastoidectomy. But it can also be treated successfully by intact canal wall tympanomastoidectomy with good hearing results.
Acquired Cholesteatoma of the Ear; Comparative Analysis of Histopathological Findings in Adults, Children and Recurrent Cases
2012
Objective: To compare the histopathological characteristics of acquired cholesteatoma in adults, children and recurrent cases. Methods: A retrospective analysis of 60 histopathological specimens for 60 patients aged 9 to 63 years who underwent otologic surgery for chronic otitis media with cholesteatoma was carried out at King Hussein Medical Centre between January 2006 till July 2010. Patients were divided into three groups as follows; group A patients aged > 16 years with no history of previous ear surgery, group B patients aged > 16 years and had history of previous otologic surgery for cholesteatoma and group C patients aged ≤ 16 years. Histopathological analysis was performed for specimens. Results for group A were compared with results of groups B & C separately. Results: After histopathological analysis; atrophy was present in 26(84%) specimens in group A, 10 (71%)specimens in group B and 11(73%) specimens in group C. Twenty- seven (87%) specimens had acanthosis in grou...
A Unified Classification of Middle Ear and Petrous Bone Congenital Cholesteatomas
The Journal of International Advanced Otology
BACKGROUND: The aim of this study was to classify congenital cholesteatoma along an entire spectrum of involvement ranging from the middle ear to petrous apex. METHODS: A total of 131 patients (85 adults and 46 children) underwent operations for congenital cholesteatoma over the duration of 27 years. RESULTS: For most cases, middle ear mucosa was normal, the first ossicle eroded by the mass was the stapes, and the mastoid air cell system was well-pneumatized on intraoperative and radiographic views. Totally 34% of patients presented with facial nerve weakness and 45% of these cholesteatomas arose from the supralabyrinthine area (32.8%) and from the petrous apex (12.2%). CONCLUSION: In this unified classification system, the otologist sees congenital cholesteatoma as a continuum, with facial nerve involvement and anacusis as part of the picture. This system of congenital cholesteatoma accommodates the supralabyrinthine and petrous bone locations of the disease.
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