Eur Arch Otorhinolaryngol DOI 10.1007/s00405-013-2820-6
OTOLOGY
Clinical characteristics of spontaneous cholesteatoma of the external auditory canal in children comparing with cholesteatoma in adults Chang Woo Kim • So-Hye Baek • Sang-Hyo Lee Go-Woon Kim • Bum-Ki Cho
•
Received: 23 September 2013 / Accepted: 7 November 2013 Ó Springer-Verlag Berlin Heidelberg 2013
Abstract The purpose of this study was to investigate the characteristics of external auditory canal cholesteatoma (EACC) in children through evaluation of the clinical and radiologic features as well as treatment outcomes. The clinical records were retrospectively reviewed for children under 15 years of age diagnosed with spontaneous EACC between March 2004 and December 2011. The clinical data of adults diagnosed with spontaneous EACC during the same period were evaluated to compare with EACC in children. Eight patients (3 males and 5 females) with pediatric EACC and 18 patients (7 males and 11 females, 20 ears) with adult EACC were included within the boundary of the study. The mean ages were 12.4 years (age range 9–15) for pediatric EACC and 49.8 years (age range 29–79) for adult EACC patients. Follow-up periods ranged from 8 to 86 months (mean 32.5 ± 8.62) in pediatric EACC and from 6 to 72 months (mean 22.2 ± 5.36) in adult EACC. Pediatric EACC, showed involvement most commonly in the posterior wall, while the inferior wall was most commonly involved in adult EACC. Pediatric EACC tended to show a more focal involvement and was not as extensive as adult EACC. Extension into the adjacent structures was similar in both groups, but bony destruction was more common in the adult group. Two children and eight adult patients were treated with surgery, but four adult cases needed more extensive surgical treatment because their disease was widely spread to included areas such as the mastoid segment of facial nerve and the
C. W. Kim (&) S.-H. Baek S.-H. Lee G.-W. Kim B.-K. Cho Department of Otorhinolaryngology-Head and Neck Surgery, College of Medicine, Hallym University, 445 Gil-Dong, Gangdong-gu, Seoul 134-701, Korea e-mail: [email protected]
temporomandibular joint. Six pediatric cases treated with conservative management showed no progression of disease on physical examination at the last visit, but two cases of adults progressed and required canaloplasty. Pediatric EACC shows less aggressive behavior compared to adult EACC. Adequate management may work better in pediatric than in adult EACC, even though the treatment modality is conservative management. Keywords Children
Cholesteatoma External auditory canal
Introduction External auditory canal cholesteatoma (EACC) is a rare disease, with an estimated incidence of 1:1,000 of all new otologic patients [1], and 0.3 cases per year per 1,00,000 inhabitants; this is 60 times less than the incidence of middle ear cholesteatoma [2]. EACC may arise spontaneously or secondary to another condition or surgical procedure [3]. Spontaneous EACC is very rare and has no pathognomonic symptoms or signs. Therefore, it may be sometimes indistinguishable from keratosis obturans in clinical characteristics and otoscopic examination. The bony erosion pattern is important for differentiating these disorders; keratosis obturans is associated with a greatly widened ear canal due to circumferential erosion of bone, whereas EACC reveals a localized erosion and shows a bony necrosis or sequestration of the underlying bone [4]. Differentiation of EACC from necrotizing external otitis or malignancy, such as squamous cell carcinoma of the external auditory canal (EAC), is also important. Cholesteatoma arising from the middle ear cavity has been frequently observed to present a more aggressive
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growth pattern and higher recurrence rate in children than in adults [5]. Revision operation is common in children because of a significant rate of recidivism, and proper treatment requires an individualized approach and sufficient experience of the surgeon [6]. However, whether spontaneous EACC is more aggressive in children than in adults is not clear. Spontaneous EACC usually occurs in elderly patients, while the pediatric form is rarely presented, appearing as case reports with extensive cholesteatoma or complicated disease, or as small-sized case series [7–10]. Therefore, information is limited regarding the characteristics of pediatric EACC. The aim of this study was to analyze the characteristics of spontaneous EACC in children through evaluation of the clinical and radiologic features, as well as to assess treatment outcomes and compare the characteristics to those of adult EACC. Our hope is that this study will provide background knowledge of pediatric EACC and help in its proper treatment.
Materials and methods The clinical records were retrospectively reviewed for children under 15 years of age diagnosed with spontaneous EACC at a tertiary care referral center (Kangdong Sacred Heart Hospital of Seoul, Korea) between March 2004 and December 2011. The EACC was diagnosed clinically by endoscopic and microscopic examinations and by temporal bone CT images showing a focal disruption of the skin with underlying bony erosion, and was further supported by histopathologic examination. All patients had an intact tympanic membrane, and patients with prior trauma, otologic surgery (including tympanostomy tube insertion), ear canal stenosis, or disease associated with middle ear or mastoid were excluded because of the possibility of secondary cholesteatoma. We analyzed the main symptoms and clinical findings of cholesteatoma such as location, localization and stage by endoscopy, microscopy and temporal bone CT findings, and treatment outcomes for evaluation of characteristics of the spontaneous EACC. The clinical data of adults diagnosed with spontaneous EACC during the same period were evaluated to compare with EACC in children. Location was defined as the wall involved with cholesteatoma, according to the endoscopy and microscopy findings, with the umbo as a pivot and the manubrium of the malleus as a baseline of the angle: anterior with between 45° and 135°, inferior with between 135° and 225°, posterior with between 225° and 315°, and superior with between 315° and 45°. Localization was classified as medial, lateral, and widespread according to the distribution of the cholesteatoma. Stage of spontaneous EACC was
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Table 1 Stage of spontaneous EAC cholesteatoma with modification of the staging system of Naim et al. [11] Stage
Characteristics
I
Hyperplasia and hyperemia of the auditory meatal epithelium
II
Localized inflammation of the hyperproliferated epithelium and adjacent bony erosion
III IV
Destruction of the bony ear canal with sequestrated bone Spontaneous destruction of the adjacent anatomical structures such as mastoid, skull base, temporomandibular joint and facial nerve
defined by endoscopy, microscopy and temporal bone CT findings and classified into four based on the staging system of Naim (Table 1) [11]: stage I with hyperplasia and hyperemia of the auditory canal epithelium; stage II with localized inflammation of the hyperproliferated epithelium and adjacent bony erosion; stage III with destruction of the bony ear canal with sequestrated bone; and stage IV with bony destruction and invasion into the adjacent anatomical structures such as mastoid, skull base, temporomandibular joint, and facial nerve (Fig. 1). A pure tone audiogram was taken and the mean values for frequencies 0.5, 1, 2, and 3 kHz were calculated for a hearing test. This study was performed in keeping with the Declaration of Helsinki’s mandate.
Results During the study period, eight patients (3 males and 5 females) with pediatric EACC and 18 patients (7 males and 11 females) with adult EACC were included in the study (Table 2). The mean age of patients was 12.4 years (age range 9–15) in the pediatric EACC cases and 49.8 years (age range 29–79) in the adult EACC cases. Every child had a unilateral cholesteatoma, but two adults had bilateral cholesteatomas. Otalgia was the most common symptom, followed by otorrhea in both groups (Table 3). Two children and five adults had otorrhea and bacterial culture study revealed a case of MRSA and the other case with no organism in the pediatric EACC group, and four cases of MRSA and a case of pseudomonas in the adult EACC group. No complaints of hearing loss were noted in the pediatric EACC group, and every child but one (case no. 8) had normal hearing. The pure tone audiogram for case no. 8 revealed a 40 dB conductive hearing loss with an airbone gap of 30 dB. On the other hand, twelve cases of the adult EACC (60 %) had mild sensorineural hearing loss, perhaps due to the aging process, and one patient complained of hearing loss with 50 dB conductive hearing loss. Pediatric and adult EACC seem to invade similar neighboring structures, however, adult patients presented
Eur Arch Otorhinolaryngol
Fig. 1 Axial and coronal temporal bone CT of EAC cholesteatoma (arrows). a Pictures of patient 2. Bony erosion appears medially on the anterior and inferior wall of the right ear (stage II). b Pictures of patient 5. A soft tissue lesion is located laterally, with destruction of the posterior and inferior bony wall of the left ear (stage III). c Pictures of patient 7. A soft tissue lesion of the left ear canal is
located laterally, with destruction of the posterior bony wall and invasion into the mastoid (stage IV). d Pictures of patient 8. A soft tissue lesion of the right ear canal has widespread location, with destruction of the posterior bony wall and invasion into the mastoid (stage IV)
Table 2 Characteristics of EAC cholesteatoma of children Patient no
Age (years)
Sex
Side
Symptom
Location
Localization
Stage
Treatment
f/u (month)
1
10
F
R
Earfullness
P
L
II
Conservative
8
2
13
M
R
Otalgia
AI
M
II
Conservative
26
3
12
F
R
Otalgia
P
L
II
Conservative
22
4
14
F
R
Earfullness
I
M
II
Conservative
41
5
15
M
L
Otalgia
PI
L
III
Conservative
25
6
15
F
L
Otalgia
PI
M
III
Conservative
36
7
9
F
L
Otorrhea
P
L
IV
IBM
14
8
11
M
R
Otorrhea
P
W
IV
IBM
86
M male, F female, R right, L left, P posterior, I inferior, A anterior, L lateral, M medial, W widespread, IBM intact bridge mastoidectomy
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Eur Arch Otorhinolaryngol Table 3 Patients’ data of pediatric and adult EAC cholesteatomas Pediatric EACC (n = 8)
Adult EACC (n = 18)
Male
3 (37.5 %)
7 (38.9 %)
Female
5 (62.5 %)
11 (61.1 %)
12.4 ± 2.56
49.8 ± 16.17
8 (100 %)
16 (88.9 %)
Sex
Age Laterality Unilateral Bilateral Symptoma
2 (11.1 %)
Otalgia
4
10
Otorrhea
2
5
Earfullness
2
1
Itching sense
2
Hearing loss
1
Incidental
1
Follow-up (month)
32.5 ± 8.62
22.2 ± 5.36
Operation
2 (IBM 2)
8 (IBM 2, CWD 4 CP 2)
Conservative
6
10b
Treatment
IBM intact bridge mastoidectomy, CWD canal wall down mastoidectomy, CP canaloplasty a
Two patients with bilateral disease in adult group
b
Included two patients with refusal of operation
Table 4 Comparison of pediatric and adult EAC cholesteatomas Pediatric EACC (n = 8)
Adult EACC (n = 20)
II
4
3
III
2
11
IV
2 (M2)
6 (FN3, TMJ2, M1)
Stage I
Discussion
Location Posterior
6 (75 %)
8 (40 %)
Inferior
4 (50 %)
16 (80 %)
Anterior
1 (12.5 %)
9 (45 %)
Superior Localization Medial
2 (10 %) 3
5
Lateral
4
6
Widespread
1
9
EACC external auditory canal cholesteatoma, M mastoid, FN facial nerve, TMJ temporomandibular joint
with much more advanced stage of bone erosion (stage III and IV according to Naim) (Table 4). Two pediatric patients had extension of disease into the mastoid cavity, and six patients with adult EACC had extension into mastoid cavity, facial nerve, and temporomandibular joint.
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Five cases in the pediatric EACC group (62.5 %) had involvement of single wall, whereas half of the adult EACC had involvement in more than one wall. Pediatric EACC cases had involvement most commonly in the posterior wall (75 %), followed by the inferior and anterior walls. However, the adult EACC cases had involvement most commonly in the inferior wall (80 %), followed by the anterior, posterior, and superior walls. Similar involvement of cholesteatoma was found in the medial or lateral parts of the EAC in both groups, but was more widespread in the adult EACC group. Six cases of pediatric EACC were treated conservatively with serial office-based debridement and application of otic solution (Tarivid, 0.3 % ofloxacin, Daiichi Sankyo), with a follow-up schedule of biweekly for a month and then every 3 months. Five drops of otic solution were applied to the affected ear two times daily for 2 weeks. Frequent cleansing and removal of keratin debris was performed relatively easily without local anesthesia. Two cases of class IV underwent intact bridge mastoidectomy (canal wall down mastoidectomy with preserving the most medial portion (bridge) of the posterosuperior meatal wall) [12] because of invasion into the mastoid cavity with intact tympanic membrane and middle ear cavity. Ten patients in the adult group required surgical treatment, but two patients refused to have an operation, so eight patients underwent surgical treatment. The remaining patients were treated with serial debridement, and no progression of disease was observed except in two cases of adult EACC. Interestingly, the bony destruction in a case of pediatric EACC (case no. 6) had improved 2 years after conservative treatment (Fig. 2). Follow-up ranged from 8 to 86 months (mean 32.5 ± 8.62) in pediatric EACC and from 6 to 72 months (mean 22.2 ± 5.36) in adult EACC.
Middle ear cholesteatoma in children has been recognized to show a biologically different behavior from that found in adults. Many authors have assumed that the difference may be due to anatomic and physiologic states of children [6]. The Eustachian tube anatomy and resulting poor function in children predispose them to more frequent infections and retraction pockets. Moreover, the well-pneumatized mastoids in children compared with more sclerotic mastoid bone in adults allow for more extensive disease. Several studies have shown histopathologic differences between adult and pediatric cholesteatoma. Bujia et al. [13] reported that the proliferative rate of pediatric keratinocytes was significantly greater than that of adults, so pediatric cholesteatoma had a more aggressive clinical behavior. Dornelles et al. [14] reported that the pediatric
Eur Arch Otorhinolaryngol
Fig. 2 Axial and coronal temporal bone CT of patient 6. a Bony destruction (arrows) is noted in posterior wall with a soft tissue lesion (stage III). b Bony lesion (arrows) is improved 2 years after conservative management
cholesteatomas presented more aggressive characteristics because of a thicker perimatrix, a more exacerbated inflammatory process and a higher degree of angiogenesis were found in cholesteatomas of adult patients. Based on the morphological studies mentioned above, middle ear cholesteatoma is generally believed to be faster growing, with more widespread disease and shows a greater propensity to relapse in children than in adults. In EACC, only few histological studies and biological studies have been done in the adult population but data for pediatric patients are lacking [15–17]. Spontaneous EACC in children also seems quite aggressive, according to several reports [8–10]. Spontaneous EACC is an entity that occurs mostly in the elderly, and pediatric EACC is very rare; only 13 cases have been reported in the literature [7–10, 18]. Pediatric spontaneous EACC has been presumed to have a more aggressive growth pattern and higher recurrence rate than the adult form due to its low occurrence, which may be related to underreporting. Cheng et al. [8] reported two pediatric cases of spontaneous EACC with extensive invasion into the mastoid cavity and they reviewed previous reports describing adult cases of EACC with extensive invasion into the mastoid cavity. They found 13 reported cases of EACC with mastoid fistula, but spontaneous EACC in children accounted for only 2 cases. Quantin et al. [9] and Choi et al. [10] each reported a case of congenital EACC with cystic mass; the patients’ ages were 7 months and 2 years, respectively. Lin [18] reported a 12-year-old patient with extensive spontaneous EACC into the temporomandibular joint among the 45 EACC patients investigated. Yoon et al. [7] reported the largest case series of EACC in the pediatric population, and described two cases of EACC with mastoid invasion among the eight cases of spontaneous EACC. They reported that the inferior wall of EAC was the most commonly involved and that repeated
minor traumas such as Q-tip use to the EAC may play an important role in the development of pediatric EACC. In our study, the finding of cholesteatoma with extension into the adjacent structures was similar in both pediatric (25 %) and adult (30 %) EACCs. Most of the invasive pediatric EACC were limited with their extension to the mastoid cavity. On the contrary, four (20 %) of the adult EACC showed invasion of the mastoid segment of the facial nerve (n = 3) and even the temporomandibular joint (n = 2). Stage III EACC, which is regarded as a pre-stage of invasion into neighboring structures, was more commonly found in the adult EACC (55 %) than in pediatric EACC (25 %) group. Pediatric EACC also showed a tendency for a more focal involvement when compared with adult EACC. Similar involvement of the medial or lateral part of the EAC was seen in both groups, but cholesteatoma involvement was more widespread in adult EACC: one case (13 %) of pediatric EACC and nine cases (45 %) of adult EACC had widespread involvement. Three of eight (38 %) children had single wall involvement, whereas half of the adults had multiple involvements, with four cases (20 %) having involvement of more than three walls. The posterior canal wall was most commonly involved in pediatric EACC, whereas the inferior wall was most commonly involved in adult EACC. This finding is different from that reported in Yoon’s study [7] and other previous reports. The posterior wall of the EACC may easily invade into the mastoid air cell, so pediatric EACC may present extensive disease. Makino and Amatsu [19] reported a reduced epithelial migratory rate of the inferior canal wall epithelium in patients with EACC compared with controls. Many authors have referred to Makino’s study to explain the pathogenesis of primary EACC where abnormal migration of the inferior wall of the EAC caused erratic keratin deposition and poor blood supply in the inferior canal wall, leading to
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EACC formation [2, 11, 20]. However, a control study recently showed no significant differential migration velocity of the canal epithelium between normal and EACC ears [21]. The inferior wall of the EAC is recognized as the most commonly involved site of spontaneous EACC in adults, although the pathogenesis is not clear. Some factors such as recurrent microtraumas and smoking with potential microangiopathy may cause local inflammation and changes in epithelial and periosteal homeostasis, and result in EACC. Treatment of spontaneous EACC depends on the extent of the disease. Small lesions can be usually controlled with regular microscopic debridement combining topical antibiotics and acidifying agents, while large and destructive lesions require surgery [20, 22]. We treated conservatively with frequent cleansing and removal of keratin debris in stage II and III cases. Stage IV cases and some cases of stage III whose disease was not completely visible underwent surgical treatment. Two cases of children were treated with intact bridge mastoidectomy, whereas four cases of adults needed more extensive surgical treatment because their disease was widely spread, including such areas as the mastoid segment of the facial nerve and the temporomandibular joint. In six pediatric cases treated with conservative management, no progression of disease was observed on physical examination at the last visit, but two cases of adults had progressed and required canaloplasty. One pediatric case (case no. 6) had healed bony destruction after conservative management. We think this is a noteworthy feature of pediatric EACC. The bony volume is balanced by remodeling activity, where the bony resorption site is replaced by new bone tissue through osteoblast activity [23]. This process is indeed elevated in young children, and decreases after the age of puberty. The patient’s bony wall seemed to be regenerated by remodeling activity. Successful local debridement and topical therapy has been described in the adult population and our data suggest that in some cases conservative treatment may work even better in the pediatric population due to the bone remodeling capabilities of the infant temporal bone.
Conclusion Our opinion is that pediatric EACC is less aggressive than adult EACC. Our study revealed three characteristics of pediatric EACC with respect to involvement pattern and response to treatment. First, pediatric EACC developed most commonly in the posterior canal wall, so it is possible that pediatric EACC may easily invade into the mastoid air cell if not detected early and properly managed. Second, pediatric EACC tended to show a more focal involvement and was not as extensive as the adult form. Finally, the majority of our
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pediatric patients were treated conservatively and showed good local control. In one case, we could document even a reversion of bone erosion by new bone regeneration. Conservative treatment was better feasible and successful in the pediatric compared to the adult population.
References 1. Anthony PF, Anthony WP (1982) Surgical treatment of external auditory canal cholesteatoma. Laryngoscope 92:70–75 2. Owen HH, Rosborg J, Gaihede M (2006) Cholesteatoma of the external ear canal: etiological factors, symptoms and clinical findings in a series of 48 cases. BMC Ear Nose Throat Disord 6:16 3. Holt JJ (1992) Ear canal cholesteatoma. Laryngoscope 102:608–613 4. Pipergerdes JC, Kramer BM (1980) Keratosis obturans and external auditory canal cholesteatoma. Laryngoscope 90:383–391 5. Palva A, Karma P, Ka¨rja¨ J (1977) Cholesteatoma in children. Arch Otolaryngol 103:74–77 6. Shohet JA, De Jong AL (2002) The management of pediatric cholesteatoma. Otolaryngol Clin N Am 35:841–851 7. Yoon YH, Park CH, Kim EH, Park YH (2008) Clinical characteristics of external auditory canal cholesteatoma in children. Otolaryngol Head Neck Surg 139:661–664 8. Cheng YF, Chiao AS, Lien CF (2005) Pediatric external canal cholesteatoma with extensive invasion into the mastoid cavity. Int J Pediatr Otorhinolaryngol 69:561–566 9. Quantin L, Fernandez SC, Moretti J (2002) Congenital cholesteatoma of external auditory canal. Int J Pediatr Otorhinolaryngol 62:175–179 10. Choi JH, Woo HY, Yoo YS, Cho KR (2011) Congenital primary cholesteatoma of external auditory canal. Am J Otolaryngol 32:247–249 11. Naim R, Linthicum R, Shen T, Bran G, Horman K (2005) Classification of the external auditory canal cholesteatoma. Laryngoscope 115:455–460 12. Paparella MM, Jung TT (1984) Intact-bridge tympanomastoidectomy. Otolaryngol Head Neck Surg 92(3):334–338 13. Bujia J, Holly A, Antoli-Candela F, Tapia MG, Kastenbauer E (1996) Immunobiological peculiarities of cholesteatoma in children: quantification of epithelial proliferation by MIB1. Laryngoscope 106:865–868 14. Dornelles Cde C, da Costa SS, Meurer L, Rosito LP, da Silva AR, Alves SL (2009) Comparison of acquired cholesteatoma between pediatric and adult patients. Eur Arch Otorhinolaryngol 266:1553–1561 15. Dubach P, Mantokoudis G, Banz Y, Caversaccio M, Senn P (2013) Histopathologic case of the month—idiopathic ear canal cholesteatoma. Otol Neurotol 34(5):e40–e41 16. Naim R, Shen T, Riedel F, Bran G, Sadick H (2005) Regulation of apoptosis in external auditory canal cholesteatoma by hepatocyte growth factor/scatter factor. ORL 67:45–50 17. Naim R, Sadick H, Schafer C, Hormann K (2004) External auditory canal cholesteatoma: analysis of the integrity of the tissue structure. Int J Mol Med 14:601–604 18. Lin YS (2009) Surgical results of external canal cholesteatoma. Acta Otolaryngol 129:615–623 19. Makino K, Amatsu M (1986) Epithelial migration on the tympanic membrane and external canal. Arch Otorhinolaryngol 243:39–42 20. Dubach P, Mantokoudis G, Caversaccio M (2010) Ear canal cholesteatoma: meta-analysis of clinical characteristics with
Eur Arch Otorhinolaryngol update on classification, staging and treatment. Curr Opin Otolaryngol Head Neck Surg 18:369–376 21. Bonding P, Raven T (2008) Primary cholesteatoma of the external auditory canal: is the epithelial migration defective? Otol Neurotol 29:334–338 22. Terayama Y, Sakata A, Murata Y, Harada K, Ohashi M (2010) Burow’s solution treatment for external auditory canal and
mastoid cavity cholesteatoma. Nihon Jibiinkoka Gakkai Kaiho 113(6):549–555 23. Rauch F (2006) Watching bone cells at work: what we can see from bone biopsy. Pediatr Nephrol 21:457–462
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OTOLOGY
Clinical characteristics of spontaneous cholesteatoma of the external auditory canal in children comparing with cholesteatoma in adults Chang Woo Kim • So-Hye Baek • Sang-Hyo Lee Go-Woon Kim • Bum-Ki Cho
•
Received: 23 September 2013 / Accepted: 7 November 2013 Ó Springer-Verlag Berlin Heidelberg 2013
Abstract The purpose of this study was to investigate the characteristics of external auditory canal cholesteatoma (EACC) in children through evaluation of the clinical and radiologic features as well as treatment outcomes. The clinical records were retrospectively reviewed for children under 15 years of age diagnosed with spontaneous EACC between March 2004 and December 2011. The clinical data of adults diagnosed with spontaneous EACC during the same period were evaluated to compare with EACC in children. Eight patients (3 males and 5 females) with pediatric EACC and 18 patients (7 males and 11 females, 20 ears) with adult EACC were included within the boundary of the study. The mean ages were 12.4 years (age range 9–15) for pediatric EACC and 49.8 years (age range 29–79) for adult EACC patients. Follow-up periods ranged from 8 to 86 months (mean 32.5 ± 8.62) in pediatric EACC and from 6 to 72 months (mean 22.2 ± 5.36) in adult EACC. Pediatric EACC, showed involvement most commonly in the posterior wall, while the inferior wall was most commonly involved in adult EACC. Pediatric EACC tended to show a more focal involvement and was not as extensive as adult EACC. Extension into the adjacent structures was similar in both groups, but bony destruction was more common in the adult group. Two children and eight adult patients were treated with surgery, but four adult cases needed more extensive surgical treatment because their disease was widely spread to included areas such as the mastoid segment of facial nerve and the
C. W. Kim (&) S.-H. Baek S.-H. Lee G.-W. Kim B.-K. Cho Department of Otorhinolaryngology-Head and Neck Surgery, College of Medicine, Hallym University, 445 Gil-Dong, Gangdong-gu, Seoul 134-701, Korea e-mail: [email protected]
temporomandibular joint. Six pediatric cases treated with conservative management showed no progression of disease on physical examination at the last visit, but two cases of adults progressed and required canaloplasty. Pediatric EACC shows less aggressive behavior compared to adult EACC. Adequate management may work better in pediatric than in adult EACC, even though the treatment modality is conservative management. Keywords Children
Cholesteatoma External auditory canal
Introduction External auditory canal cholesteatoma (EACC) is a rare disease, with an estimated incidence of 1:1,000 of all new otologic patients [1], and 0.3 cases per year per 1,00,000 inhabitants; this is 60 times less than the incidence of middle ear cholesteatoma [2]. EACC may arise spontaneously or secondary to another condition or surgical procedure [3]. Spontaneous EACC is very rare and has no pathognomonic symptoms or signs. Therefore, it may be sometimes indistinguishable from keratosis obturans in clinical characteristics and otoscopic examination. The bony erosion pattern is important for differentiating these disorders; keratosis obturans is associated with a greatly widened ear canal due to circumferential erosion of bone, whereas EACC reveals a localized erosion and shows a bony necrosis or sequestration of the underlying bone [4]. Differentiation of EACC from necrotizing external otitis or malignancy, such as squamous cell carcinoma of the external auditory canal (EAC), is also important. Cholesteatoma arising from the middle ear cavity has been frequently observed to present a more aggressive
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growth pattern and higher recurrence rate in children than in adults [5]. Revision operation is common in children because of a significant rate of recidivism, and proper treatment requires an individualized approach and sufficient experience of the surgeon [6]. However, whether spontaneous EACC is more aggressive in children than in adults is not clear. Spontaneous EACC usually occurs in elderly patients, while the pediatric form is rarely presented, appearing as case reports with extensive cholesteatoma or complicated disease, or as small-sized case series [7–10]. Therefore, information is limited regarding the characteristics of pediatric EACC. The aim of this study was to analyze the characteristics of spontaneous EACC in children through evaluation of the clinical and radiologic features, as well as to assess treatment outcomes and compare the characteristics to those of adult EACC. Our hope is that this study will provide background knowledge of pediatric EACC and help in its proper treatment.
Materials and methods The clinical records were retrospectively reviewed for children under 15 years of age diagnosed with spontaneous EACC at a tertiary care referral center (Kangdong Sacred Heart Hospital of Seoul, Korea) between March 2004 and December 2011. The EACC was diagnosed clinically by endoscopic and microscopic examinations and by temporal bone CT images showing a focal disruption of the skin with underlying bony erosion, and was further supported by histopathologic examination. All patients had an intact tympanic membrane, and patients with prior trauma, otologic surgery (including tympanostomy tube insertion), ear canal stenosis, or disease associated with middle ear or mastoid were excluded because of the possibility of secondary cholesteatoma. We analyzed the main symptoms and clinical findings of cholesteatoma such as location, localization and stage by endoscopy, microscopy and temporal bone CT findings, and treatment outcomes for evaluation of characteristics of the spontaneous EACC. The clinical data of adults diagnosed with spontaneous EACC during the same period were evaluated to compare with EACC in children. Location was defined as the wall involved with cholesteatoma, according to the endoscopy and microscopy findings, with the umbo as a pivot and the manubrium of the malleus as a baseline of the angle: anterior with between 45° and 135°, inferior with between 135° and 225°, posterior with between 225° and 315°, and superior with between 315° and 45°. Localization was classified as medial, lateral, and widespread according to the distribution of the cholesteatoma. Stage of spontaneous EACC was
123
Table 1 Stage of spontaneous EAC cholesteatoma with modification of the staging system of Naim et al. [11] Stage
Characteristics
I
Hyperplasia and hyperemia of the auditory meatal epithelium
II
Localized inflammation of the hyperproliferated epithelium and adjacent bony erosion
III IV
Destruction of the bony ear canal with sequestrated bone Spontaneous destruction of the adjacent anatomical structures such as mastoid, skull base, temporomandibular joint and facial nerve
defined by endoscopy, microscopy and temporal bone CT findings and classified into four based on the staging system of Naim (Table 1) [11]: stage I with hyperplasia and hyperemia of the auditory canal epithelium; stage II with localized inflammation of the hyperproliferated epithelium and adjacent bony erosion; stage III with destruction of the bony ear canal with sequestrated bone; and stage IV with bony destruction and invasion into the adjacent anatomical structures such as mastoid, skull base, temporomandibular joint, and facial nerve (Fig. 1). A pure tone audiogram was taken and the mean values for frequencies 0.5, 1, 2, and 3 kHz were calculated for a hearing test. This study was performed in keeping with the Declaration of Helsinki’s mandate.
Results During the study period, eight patients (3 males and 5 females) with pediatric EACC and 18 patients (7 males and 11 females) with adult EACC were included in the study (Table 2). The mean age of patients was 12.4 years (age range 9–15) in the pediatric EACC cases and 49.8 years (age range 29–79) in the adult EACC cases. Every child had a unilateral cholesteatoma, but two adults had bilateral cholesteatomas. Otalgia was the most common symptom, followed by otorrhea in both groups (Table 3). Two children and five adults had otorrhea and bacterial culture study revealed a case of MRSA and the other case with no organism in the pediatric EACC group, and four cases of MRSA and a case of pseudomonas in the adult EACC group. No complaints of hearing loss were noted in the pediatric EACC group, and every child but one (case no. 8) had normal hearing. The pure tone audiogram for case no. 8 revealed a 40 dB conductive hearing loss with an airbone gap of 30 dB. On the other hand, twelve cases of the adult EACC (60 %) had mild sensorineural hearing loss, perhaps due to the aging process, and one patient complained of hearing loss with 50 dB conductive hearing loss. Pediatric and adult EACC seem to invade similar neighboring structures, however, adult patients presented
Eur Arch Otorhinolaryngol
Fig. 1 Axial and coronal temporal bone CT of EAC cholesteatoma (arrows). a Pictures of patient 2. Bony erosion appears medially on the anterior and inferior wall of the right ear (stage II). b Pictures of patient 5. A soft tissue lesion is located laterally, with destruction of the posterior and inferior bony wall of the left ear (stage III). c Pictures of patient 7. A soft tissue lesion of the left ear canal is
located laterally, with destruction of the posterior bony wall and invasion into the mastoid (stage IV). d Pictures of patient 8. A soft tissue lesion of the right ear canal has widespread location, with destruction of the posterior bony wall and invasion into the mastoid (stage IV)
Table 2 Characteristics of EAC cholesteatoma of children Patient no
Age (years)
Sex
Side
Symptom
Location
Localization
Stage
Treatment
f/u (month)
1
10
F
R
Earfullness
P
L
II
Conservative
8
2
13
M
R
Otalgia
AI
M
II
Conservative
26
3
12
F
R
Otalgia
P
L
II
Conservative
22
4
14
F
R
Earfullness
I
M
II
Conservative
41
5
15
M
L
Otalgia
PI
L
III
Conservative
25
6
15
F
L
Otalgia
PI
M
III
Conservative
36
7
9
F
L
Otorrhea
P
L
IV
IBM
14
8
11
M
R
Otorrhea
P
W
IV
IBM
86
M male, F female, R right, L left, P posterior, I inferior, A anterior, L lateral, M medial, W widespread, IBM intact bridge mastoidectomy
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Eur Arch Otorhinolaryngol Table 3 Patients’ data of pediatric and adult EAC cholesteatomas Pediatric EACC (n = 8)
Adult EACC (n = 18)
Male
3 (37.5 %)
7 (38.9 %)
Female
5 (62.5 %)
11 (61.1 %)
12.4 ± 2.56
49.8 ± 16.17
8 (100 %)
16 (88.9 %)
Sex
Age Laterality Unilateral Bilateral Symptoma
2 (11.1 %)
Otalgia
4
10
Otorrhea
2
5
Earfullness
2
1
Itching sense
2
Hearing loss
1
Incidental
1
Follow-up (month)
32.5 ± 8.62
22.2 ± 5.36
Operation
2 (IBM 2)
8 (IBM 2, CWD 4 CP 2)
Conservative
6
10b
Treatment
IBM intact bridge mastoidectomy, CWD canal wall down mastoidectomy, CP canaloplasty a
Two patients with bilateral disease in adult group
b
Included two patients with refusal of operation
Table 4 Comparison of pediatric and adult EAC cholesteatomas Pediatric EACC (n = 8)
Adult EACC (n = 20)
II
4
3
III
2
11
IV
2 (M2)
6 (FN3, TMJ2, M1)
Stage I
Discussion
Location Posterior
6 (75 %)
8 (40 %)
Inferior
4 (50 %)
16 (80 %)
Anterior
1 (12.5 %)
9 (45 %)
Superior Localization Medial
2 (10 %) 3
5
Lateral
4
6
Widespread
1
9
EACC external auditory canal cholesteatoma, M mastoid, FN facial nerve, TMJ temporomandibular joint
with much more advanced stage of bone erosion (stage III and IV according to Naim) (Table 4). Two pediatric patients had extension of disease into the mastoid cavity, and six patients with adult EACC had extension into mastoid cavity, facial nerve, and temporomandibular joint.
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Five cases in the pediatric EACC group (62.5 %) had involvement of single wall, whereas half of the adult EACC had involvement in more than one wall. Pediatric EACC cases had involvement most commonly in the posterior wall (75 %), followed by the inferior and anterior walls. However, the adult EACC cases had involvement most commonly in the inferior wall (80 %), followed by the anterior, posterior, and superior walls. Similar involvement of cholesteatoma was found in the medial or lateral parts of the EAC in both groups, but was more widespread in the adult EACC group. Six cases of pediatric EACC were treated conservatively with serial office-based debridement and application of otic solution (Tarivid, 0.3 % ofloxacin, Daiichi Sankyo), with a follow-up schedule of biweekly for a month and then every 3 months. Five drops of otic solution were applied to the affected ear two times daily for 2 weeks. Frequent cleansing and removal of keratin debris was performed relatively easily without local anesthesia. Two cases of class IV underwent intact bridge mastoidectomy (canal wall down mastoidectomy with preserving the most medial portion (bridge) of the posterosuperior meatal wall) [12] because of invasion into the mastoid cavity with intact tympanic membrane and middle ear cavity. Ten patients in the adult group required surgical treatment, but two patients refused to have an operation, so eight patients underwent surgical treatment. The remaining patients were treated with serial debridement, and no progression of disease was observed except in two cases of adult EACC. Interestingly, the bony destruction in a case of pediatric EACC (case no. 6) had improved 2 years after conservative treatment (Fig. 2). Follow-up ranged from 8 to 86 months (mean 32.5 ± 8.62) in pediatric EACC and from 6 to 72 months (mean 22.2 ± 5.36) in adult EACC.
Middle ear cholesteatoma in children has been recognized to show a biologically different behavior from that found in adults. Many authors have assumed that the difference may be due to anatomic and physiologic states of children [6]. The Eustachian tube anatomy and resulting poor function in children predispose them to more frequent infections and retraction pockets. Moreover, the well-pneumatized mastoids in children compared with more sclerotic mastoid bone in adults allow for more extensive disease. Several studies have shown histopathologic differences between adult and pediatric cholesteatoma. Bujia et al. [13] reported that the proliferative rate of pediatric keratinocytes was significantly greater than that of adults, so pediatric cholesteatoma had a more aggressive clinical behavior. Dornelles et al. [14] reported that the pediatric
Eur Arch Otorhinolaryngol
Fig. 2 Axial and coronal temporal bone CT of patient 6. a Bony destruction (arrows) is noted in posterior wall with a soft tissue lesion (stage III). b Bony lesion (arrows) is improved 2 years after conservative management
cholesteatomas presented more aggressive characteristics because of a thicker perimatrix, a more exacerbated inflammatory process and a higher degree of angiogenesis were found in cholesteatomas of adult patients. Based on the morphological studies mentioned above, middle ear cholesteatoma is generally believed to be faster growing, with more widespread disease and shows a greater propensity to relapse in children than in adults. In EACC, only few histological studies and biological studies have been done in the adult population but data for pediatric patients are lacking [15–17]. Spontaneous EACC in children also seems quite aggressive, according to several reports [8–10]. Spontaneous EACC is an entity that occurs mostly in the elderly, and pediatric EACC is very rare; only 13 cases have been reported in the literature [7–10, 18]. Pediatric spontaneous EACC has been presumed to have a more aggressive growth pattern and higher recurrence rate than the adult form due to its low occurrence, which may be related to underreporting. Cheng et al. [8] reported two pediatric cases of spontaneous EACC with extensive invasion into the mastoid cavity and they reviewed previous reports describing adult cases of EACC with extensive invasion into the mastoid cavity. They found 13 reported cases of EACC with mastoid fistula, but spontaneous EACC in children accounted for only 2 cases. Quantin et al. [9] and Choi et al. [10] each reported a case of congenital EACC with cystic mass; the patients’ ages were 7 months and 2 years, respectively. Lin [18] reported a 12-year-old patient with extensive spontaneous EACC into the temporomandibular joint among the 45 EACC patients investigated. Yoon et al. [7] reported the largest case series of EACC in the pediatric population, and described two cases of EACC with mastoid invasion among the eight cases of spontaneous EACC. They reported that the inferior wall of EAC was the most commonly involved and that repeated
minor traumas such as Q-tip use to the EAC may play an important role in the development of pediatric EACC. In our study, the finding of cholesteatoma with extension into the adjacent structures was similar in both pediatric (25 %) and adult (30 %) EACCs. Most of the invasive pediatric EACC were limited with their extension to the mastoid cavity. On the contrary, four (20 %) of the adult EACC showed invasion of the mastoid segment of the facial nerve (n = 3) and even the temporomandibular joint (n = 2). Stage III EACC, which is regarded as a pre-stage of invasion into neighboring structures, was more commonly found in the adult EACC (55 %) than in pediatric EACC (25 %) group. Pediatric EACC also showed a tendency for a more focal involvement when compared with adult EACC. Similar involvement of the medial or lateral part of the EAC was seen in both groups, but cholesteatoma involvement was more widespread in adult EACC: one case (13 %) of pediatric EACC and nine cases (45 %) of adult EACC had widespread involvement. Three of eight (38 %) children had single wall involvement, whereas half of the adults had multiple involvements, with four cases (20 %) having involvement of more than three walls. The posterior canal wall was most commonly involved in pediatric EACC, whereas the inferior wall was most commonly involved in adult EACC. This finding is different from that reported in Yoon’s study [7] and other previous reports. The posterior wall of the EACC may easily invade into the mastoid air cell, so pediatric EACC may present extensive disease. Makino and Amatsu [19] reported a reduced epithelial migratory rate of the inferior canal wall epithelium in patients with EACC compared with controls. Many authors have referred to Makino’s study to explain the pathogenesis of primary EACC where abnormal migration of the inferior wall of the EAC caused erratic keratin deposition and poor blood supply in the inferior canal wall, leading to
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EACC formation [2, 11, 20]. However, a control study recently showed no significant differential migration velocity of the canal epithelium between normal and EACC ears [21]. The inferior wall of the EAC is recognized as the most commonly involved site of spontaneous EACC in adults, although the pathogenesis is not clear. Some factors such as recurrent microtraumas and smoking with potential microangiopathy may cause local inflammation and changes in epithelial and periosteal homeostasis, and result in EACC. Treatment of spontaneous EACC depends on the extent of the disease. Small lesions can be usually controlled with regular microscopic debridement combining topical antibiotics and acidifying agents, while large and destructive lesions require surgery [20, 22]. We treated conservatively with frequent cleansing and removal of keratin debris in stage II and III cases. Stage IV cases and some cases of stage III whose disease was not completely visible underwent surgical treatment. Two cases of children were treated with intact bridge mastoidectomy, whereas four cases of adults needed more extensive surgical treatment because their disease was widely spread, including such areas as the mastoid segment of the facial nerve and the temporomandibular joint. In six pediatric cases treated with conservative management, no progression of disease was observed on physical examination at the last visit, but two cases of adults had progressed and required canaloplasty. One pediatric case (case no. 6) had healed bony destruction after conservative management. We think this is a noteworthy feature of pediatric EACC. The bony volume is balanced by remodeling activity, where the bony resorption site is replaced by new bone tissue through osteoblast activity [23]. This process is indeed elevated in young children, and decreases after the age of puberty. The patient’s bony wall seemed to be regenerated by remodeling activity. Successful local debridement and topical therapy has been described in the adult population and our data suggest that in some cases conservative treatment may work even better in the pediatric population due to the bone remodeling capabilities of the infant temporal bone.
Conclusion Our opinion is that pediatric EACC is less aggressive than adult EACC. Our study revealed three characteristics of pediatric EACC with respect to involvement pattern and response to treatment. First, pediatric EACC developed most commonly in the posterior canal wall, so it is possible that pediatric EACC may easily invade into the mastoid air cell if not detected early and properly managed. Second, pediatric EACC tended to show a more focal involvement and was not as extensive as the adult form. Finally, the majority of our
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pediatric patients were treated conservatively and showed good local control. In one case, we could document even a reversion of bone erosion by new bone regeneration. Conservative treatment was better feasible and successful in the pediatric compared to the adult population.
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