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Two cases of spontaneous regression of congenital cholesteatomas
International Journal of Pediatric Otorhinolaryngology 76 (2012) 142–144
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Case report
Two cases of spontaneous regression of congenital cholesteatomas Kozue Kodama *, Mariko Hara, Masayo Hasegawa, Shingo Matsuzawa, Masahiro Shinnabe, Hiromi Kanazawa, Naohiro Yoshida, Yukiko Iino Jichi Medical University, Saitama Medical Center, 1-847 Amanuma, Saitama 330-8503, Japan
A R T I C L E I N F O
A B S T R A C T
Article history: Received 1 April 2011 Received in revised form 1 July 2011 Accepted 2 July 2011 Available online 5 October 2011
Congenital cholesteatoma arises behind a normal, intact tympanic membrane and is most plausibly explained by the persistence of fetal epidermoid formation. It is generally accepted that cholesteatoma in children has greater aggressive growth activity than that in adults. The congenital type of cholesteatoma starts as a small pearl in the middle ear and eventually grows to involve the ossicles and then the attic and mastoid, causing varying degrees of destruction and functional impairment. However, here we report the cases of two children with congenital cholesteatoma which disappeared spontaneously during the follow-up period. To the best of our knowledge, this is the first report on the spontaneous regression of congenital cholesteatoma. On the basis of the two cases, we could take a ‘‘wait and watch’’ attitude for congenital cholesteatoma in the absence of severe infection, hearing loss, or bone destruction in the middle ear. ß 2011 Elsevier Ireland Ltd. All rights reserved.
Keywords: Congenital cholesteatoma Cholesteatoma Spontaneous regression
1. Introduction
2. Case report
The precise otological examination using an otomicroscope and an otoendoscope in a general ENT clinic well as the use of highresolution computed tomography (CT) have resulted in the more frequent diagnosis of congenital cholesteatoma in Japan. The management of pediatric cholesteatoma remains a challenge [1]. Middle ear surgery is technically difficult in children with chronically and severely inflamed mucosa and granulation formation; it can lead to incomplete resection and residual disease. A high incidence of persistent eustachian tube dysfunction in the pediatric age group can also lead to reconstructive failure, residual conductive hearing loss from middle ear effusion, and recurrence of cholesteatoma after otherwise adequate surgery [2,3], not only in cases of acquired cholesteatoma but also in those of congenital cholesteatoma. Furthermore, it is generally accepted that cholesteatoma in children has greater aggressive growth activity than that in adults. The congenital type of cholesteatoma starts as a small pearl in the middle ear and eventually grows to involve the ossicles and then the attic and mastoid, causing varying degrees of destruction and functional impairment. However, here we report the cases of two children with congenital cholesteatoma which disappeared spontaneously during the follow-up period. To the best of our knowledge, this is the first report on the spontaneous regression of congenital cholesteatoma.
Case 1: A 4-year-old boy was examined by his local otolaryngologist for plugged ear wax. During the examination the doctor found a small whitish mass behind the left tympanic membrane. He was referred to our hospital for further investigation. Otomicroscopic examination revealed a normal tympanic membrane and canal in the right ear; however, a small whitish mass was observed behind the intact tympanic membrane of the left ear (Fig. 1). The patient had no history of otitis media, otorrhea, ear trauma, otologic surgery, or other congenital abnormalities. A pure-tone audiometry revealed normal hearing in both ears. A high-resolution CT scan of the temporal bones showed a soft tissue mass around the malleus and the incudostapedial joint in the aerated mesotympanum of the left ear (Fig. 2). Although the patient was scheduled to have a tympanoplasty, the operation was cancelled because he had common cold. One and a half year later, the mass was not evident by the otomicoroscopy or on CT images (Fig. 3). An exploratory tympanotomy was performed to confirm the absence of cholesteatoma in the middle ear. There was no choleteatoma matrix or debris in the middle ear, although the long process of the incus was slightly eroded (Fig. 4). Histological examination of the tissue taken from the area around the long process of the incus showed scar tissue containing large macrophages. Case 2: A 3-year-old boy was examined by his local otolaryngologist for an upper respiratory tract infection and was found to have a small whitish mass behind the left tympanic membrane. Hence, he was referred to our hospital for further investigation. Otomicroscopic examination revealed a normal right
* Corresponding author. Fax: +81 48 648 5188. E-mail address: [email protected] (K. Kodama). 0165-5876/$ – see front matter ß 2011 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijporl.2011.07.008
K. Kodama et al. / International Journal of Pediatric Otorhinolaryngology 76 (2012) 142–144
143
tympanic membrane and canal. However, a small whitish mass was observed behind the intact tympanic membrane of the left ear. The patient had no history of otitis media, otorrhea, ear trauma, otologic surgery, or other congenital abnormalities. The auditory brainstem response and conditioned orientation response audiometry was normal. A CT scan of the temporal bones showed a small soft tissue mass anterior to the manubrium of the malleus (Fig. 5). During the follow-up period, the whitish mass behind the eardrum gradually decreased in size. One year and 8 months after the first examination, there was no mass observed by otomicroscopy or temporal bone CT images of the middle ear (Fig. 6).
3. Discussion
Fig. 1. Otoscopic finding of the left ear in case 1: the presence of small whitish mass behind the intact eardrum was noted.
The criteria for diagnosis of congenital cholesteatoma are as follows: (1) pearly white mass medial to an intact tympanic membrane, normal pars tensa and flaccida, and (2) no history of tympanic membrane perforation or history of otologic procedures [4,5]. Although many congenital cholesteatomas manifest as a round mass, we sometimes encounter the cholesteatoma matrix like a sheet on the middle ear mucosa with or without debris. Cholesteatoma in the cystic form is referred to as the closed type, and cholesteatoma in the sheet form is referred to as the open type.
Fig. 2. Temporal bone CT of case 1 at the initial examination: the soft tissue mass (indicated by arrow) around the malleus and the incudostapedial joint in the aerated mesotympanum of the left ear is seen.
Fig. 3. Temporal bone CT of case 1 one and a half year later: the mass was not identified on the CT images (indicated by arrow).
Fig. 4. Operation view of case 1 at the time of exploratory tympanotomy: no cholesteatoma was found although the long process of the incus was slightly eroded.
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K. Kodama et al. / International Journal of Pediatric Otorhinolaryngology 76 (2012) 142–144
Fig. 5. Temporal bone CT of case 2 at the initial examination: a small irregular mass was present around the manubrium of the malleus.
Fig. 6. Temporal bone CT of case 2 one year and 8 months later: no shadow or abnormality is detected in the middle ear.
Most congenital cholesteatomas are of the closed type and appear as a small whitish mass behind the intact eardrum. Otomicroscopic examination of open type cholesteatoma indicates a normal tympanic membrane and canal. The debris associated with the open type does not accumulate so much as a result of selfcleansing within the tympanic cavity unless inflammation occurs. So, it may exist for a long period of time without any symptoms. In the case of the two patients described here, a small whitish mass was found behind the intact tympanic membrane by chance, and temporal bone CT showed a small soft tissue mass in the middle ear without hearing loss. Two mechanisms responsible for the formation of congenital cholesteatoma have been proposed, one is the migration theory and the other is residual ectodermal tissue theory. Aimi suggested that the source of ectodermal tissue in congenital cholesteatoma might be the migration of squamous epithelium from the ear canal in the posterosuperior part of the tympanic ring prior to the development of the connective tissue annulus [6]. Michaels described the features of epidermoid formation occuring at a precise location in the middle ear in all cases, i.e., the upper anterior quadrant of the tympanic membrane and the region just anterior to it. He suggested that epidermal cells might be the origin of cholesteatoma [7]. In any case, ectodermal tissue in the middle ear disappeares via apoptosis during the normal development of the ear [8]. In this paper, we have presented some unique features of congenital cholesteatoma, which regressed and disappeared spontaneously without treatment approximately 2 years after initial detection. Although the mechanism responsible for the spontaneous regression has not yet been elucidated, possible explanations have been proposed to explain this phenomenon. One possible explanation is related to apoptosis. Normally, ectodermal tissue in the middle ear disappears via apoptosis during development of the ear. If the onset of apoptosis occurs late after birth, the congenital cholesteatoma might diappear [8]. The second possible explanation is related to the self-cleansing mechanism of the ear. In adults, residual cholesteatoma, which cannot be completely removed from around the tympanic sinus or the footplate of the stapes during primary surgery, sometimes disappears with
additional surgeries. Most congenital cholesteatoma cases are not associated with otitis media or eustachian tube dysfunction. This self-cleansing mechanism in the eustachian tube and the reverse metaplasia from epidermal cells to columnar cells could result in the regression of cholesteatoma. Although we were not able to find out a columnar cells in the histological examination in case 1, the middle ear mucosa showed the presence of large macrophages, which might play a role in phagocytosis of the cholesteatoma. Another possibility is that cell death occurs in the epithelium of congenital cholesteatoma because of their short telomeres [9]. Cholesteatoma usually grows rapidly in children; therefore, there should be more emphasis on the surgical procedures used to manage cholesteatoma in children than those used in adults. On the basis of the two cases of spontaneous regression of congenital cholesteatoma in this paper, we could take a ‘‘wait and watch’’ attitude for some limited pearl-shaped congenital cholesteatoma in the absence of severe infection, hearing loss, or bone destruction in the middle ear.
References [1] M.A. Arriaga, Cholesteatoma in children, Otolaryngol. Clin. North Am. 27 (1994) 573–591. [2] J.A. Shohet, A.L. Jong, The management of pediatric cholesteatoma, Otolaryngol. Clin. North Am. 35 (2002) 841–851. [3] J. Silvola, T. Palva, One-stage revision surgery for pediatric cholesteatoma: longterm results and comparison with primary surgery, Int. J. Pediatr. Otorhinolaryngol. 56 (2000) 135–139. [4] E.L. Derlacki, G.D. Clemis, Congenital cholesteatoma of the middle ear and mastoid, Ann. Otol. Rhinol. Laryngol. 74 (1965) 706–727. [5] M.J. Levenson, S.C. Parisier, P. Chute, S. Wenig, C. Juarbe, A review of twenty congenital cholesteatomas of the middle ear in children, Otolaryngol. Head Neck Surg. 94 (1986) 560–567. [6] K. Aimi, Role of the tympanic ring in the pathogenesis of congenital choesteatoma, Laryngoscope 9 (1983) 1140–1146. [7] L. Michaels, An epidermoid formation in the developing middle ear: possible source of cholesteatoma, J. Otolaryngol. 15 (1986) 169–174. [8] J. Friedberg, Congental cholesteatoma, Laryngoscope 104 (1986) 1–24. [9] K. Kojima, H. Miyazaki, M. Shiwa, H. Tanaka, H. Moriyama, Molecular biological diagnosis of congenital and acquired cholesteatoma on the basis of differences in telomere length, Laryngoscope 111 (2001) 867–873.
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Case report
Two cases of spontaneous regression of congenital cholesteatomas Kozue Kodama *, Mariko Hara, Masayo Hasegawa, Shingo Matsuzawa, Masahiro Shinnabe, Hiromi Kanazawa, Naohiro Yoshida, Yukiko Iino Jichi Medical University, Saitama Medical Center, 1-847 Amanuma, Saitama 330-8503, Japan
A R T I C L E I N F O
A B S T R A C T
Article history: Received 1 April 2011 Received in revised form 1 July 2011 Accepted 2 July 2011 Available online 5 October 2011
Congenital cholesteatoma arises behind a normal, intact tympanic membrane and is most plausibly explained by the persistence of fetal epidermoid formation. It is generally accepted that cholesteatoma in children has greater aggressive growth activity than that in adults. The congenital type of cholesteatoma starts as a small pearl in the middle ear and eventually grows to involve the ossicles and then the attic and mastoid, causing varying degrees of destruction and functional impairment. However, here we report the cases of two children with congenital cholesteatoma which disappeared spontaneously during the follow-up period. To the best of our knowledge, this is the first report on the spontaneous regression of congenital cholesteatoma. On the basis of the two cases, we could take a ‘‘wait and watch’’ attitude for congenital cholesteatoma in the absence of severe infection, hearing loss, or bone destruction in the middle ear. ß 2011 Elsevier Ireland Ltd. All rights reserved.
Keywords: Congenital cholesteatoma Cholesteatoma Spontaneous regression
1. Introduction
2. Case report
The precise otological examination using an otomicroscope and an otoendoscope in a general ENT clinic well as the use of highresolution computed tomography (CT) have resulted in the more frequent diagnosis of congenital cholesteatoma in Japan. The management of pediatric cholesteatoma remains a challenge [1]. Middle ear surgery is technically difficult in children with chronically and severely inflamed mucosa and granulation formation; it can lead to incomplete resection and residual disease. A high incidence of persistent eustachian tube dysfunction in the pediatric age group can also lead to reconstructive failure, residual conductive hearing loss from middle ear effusion, and recurrence of cholesteatoma after otherwise adequate surgery [2,3], not only in cases of acquired cholesteatoma but also in those of congenital cholesteatoma. Furthermore, it is generally accepted that cholesteatoma in children has greater aggressive growth activity than that in adults. The congenital type of cholesteatoma starts as a small pearl in the middle ear and eventually grows to involve the ossicles and then the attic and mastoid, causing varying degrees of destruction and functional impairment. However, here we report the cases of two children with congenital cholesteatoma which disappeared spontaneously during the follow-up period. To the best of our knowledge, this is the first report on the spontaneous regression of congenital cholesteatoma.
Case 1: A 4-year-old boy was examined by his local otolaryngologist for plugged ear wax. During the examination the doctor found a small whitish mass behind the left tympanic membrane. He was referred to our hospital for further investigation. Otomicroscopic examination revealed a normal tympanic membrane and canal in the right ear; however, a small whitish mass was observed behind the intact tympanic membrane of the left ear (Fig. 1). The patient had no history of otitis media, otorrhea, ear trauma, otologic surgery, or other congenital abnormalities. A pure-tone audiometry revealed normal hearing in both ears. A high-resolution CT scan of the temporal bones showed a soft tissue mass around the malleus and the incudostapedial joint in the aerated mesotympanum of the left ear (Fig. 2). Although the patient was scheduled to have a tympanoplasty, the operation was cancelled because he had common cold. One and a half year later, the mass was not evident by the otomicoroscopy or on CT images (Fig. 3). An exploratory tympanotomy was performed to confirm the absence of cholesteatoma in the middle ear. There was no choleteatoma matrix or debris in the middle ear, although the long process of the incus was slightly eroded (Fig. 4). Histological examination of the tissue taken from the area around the long process of the incus showed scar tissue containing large macrophages. Case 2: A 3-year-old boy was examined by his local otolaryngologist for an upper respiratory tract infection and was found to have a small whitish mass behind the left tympanic membrane. Hence, he was referred to our hospital for further investigation. Otomicroscopic examination revealed a normal right
* Corresponding author. Fax: +81 48 648 5188. E-mail address: [email protected] (K. Kodama). 0165-5876/$ – see front matter ß 2011 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijporl.2011.07.008
K. Kodama et al. / International Journal of Pediatric Otorhinolaryngology 76 (2012) 142–144
143
tympanic membrane and canal. However, a small whitish mass was observed behind the intact tympanic membrane of the left ear. The patient had no history of otitis media, otorrhea, ear trauma, otologic surgery, or other congenital abnormalities. The auditory brainstem response and conditioned orientation response audiometry was normal. A CT scan of the temporal bones showed a small soft tissue mass anterior to the manubrium of the malleus (Fig. 5). During the follow-up period, the whitish mass behind the eardrum gradually decreased in size. One year and 8 months after the first examination, there was no mass observed by otomicroscopy or temporal bone CT images of the middle ear (Fig. 6).
3. Discussion
Fig. 1. Otoscopic finding of the left ear in case 1: the presence of small whitish mass behind the intact eardrum was noted.
The criteria for diagnosis of congenital cholesteatoma are as follows: (1) pearly white mass medial to an intact tympanic membrane, normal pars tensa and flaccida, and (2) no history of tympanic membrane perforation or history of otologic procedures [4,5]. Although many congenital cholesteatomas manifest as a round mass, we sometimes encounter the cholesteatoma matrix like a sheet on the middle ear mucosa with or without debris. Cholesteatoma in the cystic form is referred to as the closed type, and cholesteatoma in the sheet form is referred to as the open type.
Fig. 2. Temporal bone CT of case 1 at the initial examination: the soft tissue mass (indicated by arrow) around the malleus and the incudostapedial joint in the aerated mesotympanum of the left ear is seen.
Fig. 3. Temporal bone CT of case 1 one and a half year later: the mass was not identified on the CT images (indicated by arrow).
Fig. 4. Operation view of case 1 at the time of exploratory tympanotomy: no cholesteatoma was found although the long process of the incus was slightly eroded.
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K. Kodama et al. / International Journal of Pediatric Otorhinolaryngology 76 (2012) 142–144
Fig. 5. Temporal bone CT of case 2 at the initial examination: a small irregular mass was present around the manubrium of the malleus.
Fig. 6. Temporal bone CT of case 2 one year and 8 months later: no shadow or abnormality is detected in the middle ear.
Most congenital cholesteatomas are of the closed type and appear as a small whitish mass behind the intact eardrum. Otomicroscopic examination of open type cholesteatoma indicates a normal tympanic membrane and canal. The debris associated with the open type does not accumulate so much as a result of selfcleansing within the tympanic cavity unless inflammation occurs. So, it may exist for a long period of time without any symptoms. In the case of the two patients described here, a small whitish mass was found behind the intact tympanic membrane by chance, and temporal bone CT showed a small soft tissue mass in the middle ear without hearing loss. Two mechanisms responsible for the formation of congenital cholesteatoma have been proposed, one is the migration theory and the other is residual ectodermal tissue theory. Aimi suggested that the source of ectodermal tissue in congenital cholesteatoma might be the migration of squamous epithelium from the ear canal in the posterosuperior part of the tympanic ring prior to the development of the connective tissue annulus [6]. Michaels described the features of epidermoid formation occuring at a precise location in the middle ear in all cases, i.e., the upper anterior quadrant of the tympanic membrane and the region just anterior to it. He suggested that epidermal cells might be the origin of cholesteatoma [7]. In any case, ectodermal tissue in the middle ear disappeares via apoptosis during the normal development of the ear [8]. In this paper, we have presented some unique features of congenital cholesteatoma, which regressed and disappeared spontaneously without treatment approximately 2 years after initial detection. Although the mechanism responsible for the spontaneous regression has not yet been elucidated, possible explanations have been proposed to explain this phenomenon. One possible explanation is related to apoptosis. Normally, ectodermal tissue in the middle ear disappears via apoptosis during development of the ear. If the onset of apoptosis occurs late after birth, the congenital cholesteatoma might diappear [8]. The second possible explanation is related to the self-cleansing mechanism of the ear. In adults, residual cholesteatoma, which cannot be completely removed from around the tympanic sinus or the footplate of the stapes during primary surgery, sometimes disappears with
additional surgeries. Most congenital cholesteatoma cases are not associated with otitis media or eustachian tube dysfunction. This self-cleansing mechanism in the eustachian tube and the reverse metaplasia from epidermal cells to columnar cells could result in the regression of cholesteatoma. Although we were not able to find out a columnar cells in the histological examination in case 1, the middle ear mucosa showed the presence of large macrophages, which might play a role in phagocytosis of the cholesteatoma. Another possibility is that cell death occurs in the epithelium of congenital cholesteatoma because of their short telomeres [9]. Cholesteatoma usually grows rapidly in children; therefore, there should be more emphasis on the surgical procedures used to manage cholesteatoma in children than those used in adults. On the basis of the two cases of spontaneous regression of congenital cholesteatoma in this paper, we could take a ‘‘wait and watch’’ attitude for some limited pearl-shaped congenital cholesteatoma in the absence of severe infection, hearing loss, or bone destruction in the middle ear.
References [1] M.A. Arriaga, Cholesteatoma in children, Otolaryngol. Clin. North Am. 27 (1994) 573–591. [2] J.A. Shohet, A.L. Jong, The management of pediatric cholesteatoma, Otolaryngol. Clin. North Am. 35 (2002) 841–851. [3] J. Silvola, T. Palva, One-stage revision surgery for pediatric cholesteatoma: longterm results and comparison with primary surgery, Int. J. Pediatr. Otorhinolaryngol. 56 (2000) 135–139. [4] E.L. Derlacki, G.D. Clemis, Congenital cholesteatoma of the middle ear and mastoid, Ann. Otol. Rhinol. Laryngol. 74 (1965) 706–727. [5] M.J. Levenson, S.C. Parisier, P. Chute, S. Wenig, C. Juarbe, A review of twenty congenital cholesteatomas of the middle ear in children, Otolaryngol. Head Neck Surg. 94 (1986) 560–567. [6] K. Aimi, Role of the tympanic ring in the pathogenesis of congenital choesteatoma, Laryngoscope 9 (1983) 1140–1146. [7] L. Michaels, An epidermoid formation in the developing middle ear: possible source of cholesteatoma, J. Otolaryngol. 15 (1986) 169–174. [8] J. Friedberg, Congental cholesteatoma, Laryngoscope 104 (1986) 1–24. [9] K. Kojima, H. Miyazaki, M. Shiwa, H. Tanaka, H. Moriyama, Molecular biological diagnosis of congenital and acquired cholesteatoma on the basis of differences in telomere length, Laryngoscope 111 (2001) 867–873.