Eur Arch Otorhinolaryngol DOI 10.1007/s00405-015-3679-5
OTOLOGY
Pediatric middle ear cholesteatoma: the comparative study of congenital cholesteatoma and acquired cholesteatoma Yuka Morita1 • Yutaka Yamamoto1 • Shinsuke Oshima1 • Kuniyuki Takahashi1 Sugata Takahashi1
•
Received: 27 March 2015 / Accepted: 29 May 2015 Ó Springer-Verlag Berlin Heidelberg 2015
Abstract This study examined the differences between congenital cholesteatoma (CC) and acquired cholesteatomas (AC) in children by comparing clinical features and treatment courses. This was a retrospective study which retrospectively evaluated 127 children with middle ear cholesteatomas using medical records from January 1999 to December 2012 in the Department of Otolaryngology, Niigata University Hospital. The study comprised 69 and 58 cases of CC and AC, respectively. The main outcome measures include patient backgrounds, the opportunities for consultations, mastoid cell development, intraoperative finding of stapes, surgical procedure and number of surgeries. The average age at operation was 6.4 and 9.8 years in CC and AC, respectively. AC was more prevalent in boys. Mastoid development was better in CC than in AC. We adopted a two-stage operation in 17 cases (25 %) of CC and in 22 cases (38 %) of AC. The repeat surgery rate was 11.6 % in CC and 27.6 % in AC. Three times as many operations were required for three cases (4.3 %) of CC and 10 cases (17.2 %) of AC. The lesions in AC were more difficult to control. In the treatment of pediatric middle ear cholesteatoma, we had to keep the outcome in mind. Keywords Mastoid development Stapes Hearing results Children
& Yuka Morita [email protected] 1
Department of Otolaryngology Head and Neck Surgery, Niigata University Faculty of Medicine, Asahimachi-dori 1, Chuo-ku, Niigata 951-8510, Japan
Introduction In general, pediatric middle ear cholesteatoma is difficult to manage and treat. The recurrence rate is higher in children than in adult [1, 2]. In adults, acquired cholesteatomas (AC) constitute the majority of cases; however, congenital cholesteatomas (CC) are not rare in children. Pathogenesis of CC is unclear; however, there is a hypothesis that CC is a straying remnant of embryonic epithelial tissue. AC is due to middle ear ventilation deficiency, and the clinical condition is quite different. There are reports regarding pediatric cholesteatomas; however, there are few studies which differentiate between CC and AC in children. Therefore, it is very important to understand the differences between the pathogenesis to provide adequate treatment. The aim of this study was to investigate the differences in etiology and pathology of pediatric cholesteatomas by comparing the clinical courses of CC and AC.
Materials and methods A retrospective review was carried out to access the clinical presentation, surgical management and post outcomes of middle ear cholesteatomas at our institute from January 1999 to December 2012. Incursion criteria were pediatric (\15 years) congenital cholesteatoma and pediatric acquired cholesteatoma which were initially treated at our hospital. To make a diagnosis of congenital cholesteatoma, we used the criteria of Levine et al. [3]. We confirmed that there was no epithelial continuity between the tympanic membrane and cholesteatoma. We analyzed the following features: age at time of operation, gender, consultation opportunities, mastoid cell development, status of stapes, surgical methods, and results of surgery.
123
Eur Arch Otorhinolaryngol
In our institute, we routinely use canal wall-down tympanoplasty with mastoid obliteration in adults with acquired cholesteatoma. In contrast, canal wall-up tympanoplasty and transcanal atticotomy are selected for pediatric patients if the procedures are suitable. If epithelial invasion is severe, we select canal wall-down and canal wall reconstruction or mastoid obliteration for older pediatric patients. A planned two-stage surgery, including a second-look surgery, is chosen for some severe cases. Mastoid development was assessed with a preoperative computed tomography (CT) scan and classified into one of the following four stages: MC0, MC1, MC2, or MC3. Similarly, the status of the stapes was intraoperatively evaluated and classified into one of the following three stages: S0, S1, or S2 according to the Classification and Staging of Cholesteatoma that was proposed by the Japan Otological Society in 2010 [4, 5]. Preoperative and postoperative hearing thresholds were analyzed according to the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) 4-frequency average (0.5, 1, 2, and 3) in cases in which a hearing examination was possible. The hearing threshold for 3 kHz was calculated as the average of data for 2 and 4 kHz. A postoperative audiogram was conducted at least 12 months after surgery. The mean follow-up periods were 56.3 (8–125) months in the CC group and 71.0 (4–177) months in the AC group. Statistical analysis of the data was performed using SPSS version 21.0 computer software for windows. Statistical tests were performed using Mann–Whitney U tests and v2 tests, and statistical significance was set at p \ 0.05.
and 47:11 in the AC group. The proportion of boys was higher in both the CC and AC groups. Opportunity for consultation (Fig. 2) Opportunities for consultations differed between the CC and AC groups. In the CC group, detection of a white mass through the tympanic membrane was the most popular reason that they were referred to our hospital. Among the 69 patients, 36 (52.2 %) were found to have a lesion during routine examinations of their ears, and the next 29 (42.1 %) were informed of hearing loss during their medical examinations. Most of the CC cases were asymptomatic. In contrast, most of patients in the AC group had some clinical symptoms, such as otorrhea, hearing loss, and otalgia. Mastoid cell development (Fig. 3) According to the CT classification, two cases were classified as MC0, 18 as MC1, 24 as MC2, and 25 as MC3 in the CC group. In the AC group, 20, 28, eight, and two cases were classified as MC0, MC1, MC2, and MC3, respectively. There was a significant trend toward better mastoid development in the CC group (Mann–Whitney U tests, p \ 0.0001). In the CC group, eight cases were classified as MC1, 15 as MC2, and 23 as MC3 with aerated mastoids. In the AC group, 13 cases classified as MC1, four as MC2, and one as MC3 had an aerated mastoid. Pneumatization was good in the CC group, and the mastoid had developed better when it had better aeration. Intraoperative finding of stapes (Fig. 4)
Results Disease type, age at the operation and gender (Fig. 1) In this study, 124 pediatric patients (\15 years) were included. This corresponded to 24.6 % of the initial surgery of whole middle ear cholesteatomas during the same period and comprised 69 CCs and 58 ACs. The CC group included 67 cases of tympanic cavities, one case of mastoid, and one case of a petrous type. The AC group had 46 pars flaccid-type cases and 58 cases of the pars tensa type. The average age of patients in the CC group and AC group was 6.4 and 9.8 years, respectively. CC was found often in children aged 3–6 years. In contrast, the age distribution of the AC group was wide. Both the average and median ages of the AC group were higher in children (3–4 years) than those of the CC group, and there was a significant difference. The male to female ratio was 55:14 in the CC group
123
According to the stapes classification, 28 cases were classified as S0, nine as S1, and 32 as S2; the superstructure of the stapes was lost in 46.4 % of the CC group. In the AC group, 34, 10, and 14 cases were classified as S0, S1, and S2, respectively. The disappearance rate of the stapes superstructure was 24.1 % in the AC group. The deformity of the superstructure was significantly advanced in the CC group (Mann–Whitney U tests, p = 0.026). Surgical procedure and number of surgeries (Fig. 5) Transcanal atticotomies were performed in 37 cases of the 69 CC patients, canal wall-up tympanoplasties was done in 30 cases, and canal wall-down tympanoplasties was done in two cases. In the AC group, canal wall-down tympanoplasties were chosen for 36 cases, canal wall-up tympanoplasties were done in 16 cases, and transcanal atticotomies were done in six cases. Thus, 52 cases (90 %) from the AC group required mastoidectomies.
Eur Arch Otorhinolaryngol Fig. 1 Age at the operation. CC was found often in children aged 3–6 years. In contrast, the age distribution of the AC group was wide
years
congenital
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15
aquired average 6.4 p
OTOLOGY
Pediatric middle ear cholesteatoma: the comparative study of congenital cholesteatoma and acquired cholesteatoma Yuka Morita1 • Yutaka Yamamoto1 • Shinsuke Oshima1 • Kuniyuki Takahashi1 Sugata Takahashi1
•
Received: 27 March 2015 / Accepted: 29 May 2015 Ó Springer-Verlag Berlin Heidelberg 2015
Abstract This study examined the differences between congenital cholesteatoma (CC) and acquired cholesteatomas (AC) in children by comparing clinical features and treatment courses. This was a retrospective study which retrospectively evaluated 127 children with middle ear cholesteatomas using medical records from January 1999 to December 2012 in the Department of Otolaryngology, Niigata University Hospital. The study comprised 69 and 58 cases of CC and AC, respectively. The main outcome measures include patient backgrounds, the opportunities for consultations, mastoid cell development, intraoperative finding of stapes, surgical procedure and number of surgeries. The average age at operation was 6.4 and 9.8 years in CC and AC, respectively. AC was more prevalent in boys. Mastoid development was better in CC than in AC. We adopted a two-stage operation in 17 cases (25 %) of CC and in 22 cases (38 %) of AC. The repeat surgery rate was 11.6 % in CC and 27.6 % in AC. Three times as many operations were required for three cases (4.3 %) of CC and 10 cases (17.2 %) of AC. The lesions in AC were more difficult to control. In the treatment of pediatric middle ear cholesteatoma, we had to keep the outcome in mind. Keywords Mastoid development Stapes Hearing results Children
& Yuka Morita [email protected] 1
Department of Otolaryngology Head and Neck Surgery, Niigata University Faculty of Medicine, Asahimachi-dori 1, Chuo-ku, Niigata 951-8510, Japan
Introduction In general, pediatric middle ear cholesteatoma is difficult to manage and treat. The recurrence rate is higher in children than in adult [1, 2]. In adults, acquired cholesteatomas (AC) constitute the majority of cases; however, congenital cholesteatomas (CC) are not rare in children. Pathogenesis of CC is unclear; however, there is a hypothesis that CC is a straying remnant of embryonic epithelial tissue. AC is due to middle ear ventilation deficiency, and the clinical condition is quite different. There are reports regarding pediatric cholesteatomas; however, there are few studies which differentiate between CC and AC in children. Therefore, it is very important to understand the differences between the pathogenesis to provide adequate treatment. The aim of this study was to investigate the differences in etiology and pathology of pediatric cholesteatomas by comparing the clinical courses of CC and AC.
Materials and methods A retrospective review was carried out to access the clinical presentation, surgical management and post outcomes of middle ear cholesteatomas at our institute from January 1999 to December 2012. Incursion criteria were pediatric (\15 years) congenital cholesteatoma and pediatric acquired cholesteatoma which were initially treated at our hospital. To make a diagnosis of congenital cholesteatoma, we used the criteria of Levine et al. [3]. We confirmed that there was no epithelial continuity between the tympanic membrane and cholesteatoma. We analyzed the following features: age at time of operation, gender, consultation opportunities, mastoid cell development, status of stapes, surgical methods, and results of surgery.
123
Eur Arch Otorhinolaryngol
In our institute, we routinely use canal wall-down tympanoplasty with mastoid obliteration in adults with acquired cholesteatoma. In contrast, canal wall-up tympanoplasty and transcanal atticotomy are selected for pediatric patients if the procedures are suitable. If epithelial invasion is severe, we select canal wall-down and canal wall reconstruction or mastoid obliteration for older pediatric patients. A planned two-stage surgery, including a second-look surgery, is chosen for some severe cases. Mastoid development was assessed with a preoperative computed tomography (CT) scan and classified into one of the following four stages: MC0, MC1, MC2, or MC3. Similarly, the status of the stapes was intraoperatively evaluated and classified into one of the following three stages: S0, S1, or S2 according to the Classification and Staging of Cholesteatoma that was proposed by the Japan Otological Society in 2010 [4, 5]. Preoperative and postoperative hearing thresholds were analyzed according to the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) 4-frequency average (0.5, 1, 2, and 3) in cases in which a hearing examination was possible. The hearing threshold for 3 kHz was calculated as the average of data for 2 and 4 kHz. A postoperative audiogram was conducted at least 12 months after surgery. The mean follow-up periods were 56.3 (8–125) months in the CC group and 71.0 (4–177) months in the AC group. Statistical analysis of the data was performed using SPSS version 21.0 computer software for windows. Statistical tests were performed using Mann–Whitney U tests and v2 tests, and statistical significance was set at p \ 0.05.
and 47:11 in the AC group. The proportion of boys was higher in both the CC and AC groups. Opportunity for consultation (Fig. 2) Opportunities for consultations differed between the CC and AC groups. In the CC group, detection of a white mass through the tympanic membrane was the most popular reason that they were referred to our hospital. Among the 69 patients, 36 (52.2 %) were found to have a lesion during routine examinations of their ears, and the next 29 (42.1 %) were informed of hearing loss during their medical examinations. Most of the CC cases were asymptomatic. In contrast, most of patients in the AC group had some clinical symptoms, such as otorrhea, hearing loss, and otalgia. Mastoid cell development (Fig. 3) According to the CT classification, two cases were classified as MC0, 18 as MC1, 24 as MC2, and 25 as MC3 in the CC group. In the AC group, 20, 28, eight, and two cases were classified as MC0, MC1, MC2, and MC3, respectively. There was a significant trend toward better mastoid development in the CC group (Mann–Whitney U tests, p \ 0.0001). In the CC group, eight cases were classified as MC1, 15 as MC2, and 23 as MC3 with aerated mastoids. In the AC group, 13 cases classified as MC1, four as MC2, and one as MC3 had an aerated mastoid. Pneumatization was good in the CC group, and the mastoid had developed better when it had better aeration. Intraoperative finding of stapes (Fig. 4)
Results Disease type, age at the operation and gender (Fig. 1) In this study, 124 pediatric patients (\15 years) were included. This corresponded to 24.6 % of the initial surgery of whole middle ear cholesteatomas during the same period and comprised 69 CCs and 58 ACs. The CC group included 67 cases of tympanic cavities, one case of mastoid, and one case of a petrous type. The AC group had 46 pars flaccid-type cases and 58 cases of the pars tensa type. The average age of patients in the CC group and AC group was 6.4 and 9.8 years, respectively. CC was found often in children aged 3–6 years. In contrast, the age distribution of the AC group was wide. Both the average and median ages of the AC group were higher in children (3–4 years) than those of the CC group, and there was a significant difference. The male to female ratio was 55:14 in the CC group
123
According to the stapes classification, 28 cases were classified as S0, nine as S1, and 32 as S2; the superstructure of the stapes was lost in 46.4 % of the CC group. In the AC group, 34, 10, and 14 cases were classified as S0, S1, and S2, respectively. The disappearance rate of the stapes superstructure was 24.1 % in the AC group. The deformity of the superstructure was significantly advanced in the CC group (Mann–Whitney U tests, p = 0.026). Surgical procedure and number of surgeries (Fig. 5) Transcanal atticotomies were performed in 37 cases of the 69 CC patients, canal wall-up tympanoplasties was done in 30 cases, and canal wall-down tympanoplasties was done in two cases. In the AC group, canal wall-down tympanoplasties were chosen for 36 cases, canal wall-up tympanoplasties were done in 16 cases, and transcanal atticotomies were done in six cases. Thus, 52 cases (90 %) from the AC group required mastoidectomies.
Eur Arch Otorhinolaryngol Fig. 1 Age at the operation. CC was found often in children aged 3–6 years. In contrast, the age distribution of the AC group was wide
years
congenital
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15
aquired average 6.4 p
