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Fibrous dysplasia of the temporal bone and maxillofacial region associated with cholesteatoma of the middle ear
Auris Nasus Larynx 26 (1999) 79 – 81
Case report
Fibrous dysplasia of the temporal bone and maxillofacial region associated with cholesteatoma of the middle ear Dragoslava Djeric´ *, Predrag Stefanovic´ Institute of Otorhinolaryngology and Maxillofacial Surgery, Clinical Center of Serbia, Pastero6a 2, 11000 Belgrade, Yugosla6ia Received 10 April 1998; received in revised form 16 June 1998; accepted 17 July 1998
Abstract Fibrous dysplasia of the temporal bone is a rare disease which may lead to progressive stenosis of the external auditory canal and the development of cholesteatoma. We present a case in which minimal symptoms were present despite a massive temporal bone fibrous dysplasia. Cholesteatoma resulted most probably secondary to external auditory canal stenosis. Retroauricular fistula developed as a result of destructive effect of cholesteatoma, that influenced previous diagnosis and treatment of this clinically silent disease. © 1999 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Fibrous dysplasia; Temporal bone; Cholesteatoma
1. Introduction Fibrous dysplasia is a disease with unelucidated etiology, characterized by skeletal abnormality which may be associated with endocrinopathies, abnormal pigmentation of the skin and mucosal tissues, as well as by some other pathological disorders [1]. It is a relatively uncommon disease, which may occur in several clinical forms: (i) * Corresponding author. Tel.: + 381 11 643694; fax: + 381 11 643034.
monosotic fibrous dysplasia (MFD)—involvement of a single bone (70%); and (ii) polyostotic fibrous dysplasia—involvement of multiple bones (30%) and McCune–Albright syndrome—bony involvement associated with skin lesions and endcrinopathies which is very rare [2]. Although MFD occurs on craniofacial structures only in 10% of cases, this is the most frequent type of fibrous dysplasia (FD) in the head and neck region. The maxilla and mandible are most commonly affected cranio-maxillo-facial bones [3]. The temporal bone is affected only in 18% of
0385-8146/99/$ - see front matter © 1999 Elsevier Science Ireland Ltd. All rights reserved. PII S0385-8146(98)00061-3
80
D. Djeric´, P. Stefano6ic´ / Auris Nasus Larynx 26 (1999) 79–81
cases and the lesions are more frequently monostotic than polyostotic forms [4]. The aim of this report is present a patient with cholesteatoma developing in a case of fibrous dysplasia of the temporal bone and maxillofacial region leading to a retroauricular fistula.
2. Case report A 22-year-old man was referred to the Institute of Otorhinolaryngology and Maxillofacial Surgery, University Medical Center in Belgrade with otorrhea and hearing loss. His recurrent left-sided otorrhea began at the age of seven. He had frequent episodes for 6 years. However, in puberty, a progressive left-sided hearing loss developed associated with deformity at the left side of the face, most prominent in the zygomatic part. In the following 5 years the left mastoid region enlarged gradually and foul otorrhea recurred. Owing to deformity in the left mastoid region, the patient was subjected to surgery at a local ENT clinic twice, at the ages of 18 and 20. After the operations, the progressive hearing loss persisted and otorrhea was somewhat milder. However, leakage from the left retroauricular region occurred. A month prior to admission to our Institute the patient noticed edema in the left mastoid region, occasional ipsilateral pain and suppuration from an opening on the skin localized in the upper retroauricular region. On physical examination facial asymmetry was evidenced with a protrusion in the left zygomatic area was noted. Otoscopic examination revealed a stenosis of the left external auditory canal. Medially to the stenosis a massive cholesteatoma was seen, filling the middle ear spaces. In the left retroauricular region, a fistula was present with foul discharge. The Weber and Rinne tuning fork evaluation suggested a left-sided conductive hearing loss. The remaining part of the head and neck appeared normal. Audiologic examination revealed a severe mixed hearing loss on the left ear and normal findings on the right. Serum chemistry was all normal except for alkaline phorphatgase value which was four-fold. Calcium and phosphorus levels were also normal.
A CT scan of the temporal bone and paranasal sinuses revealed a massive fibrous dysplasia of the left temporal bone with involvement of otic capsule structures. Besides, the process extended on the adjoining bones, affecting the maxillar and ethmoidal bones. Obliteration of the left maxillar and ethmoidal sinuses was also evident. The area of fibrous dysplasia of the temporal bone was presented as a soft-tissue shadow in the region of the middle ear and mastoid (Fig. 1). Tympanomastoidectomy of the left ear was performed eradicating the pathological process. Intraoperatively a large cholesteatoma was found filling the middle ear spaces and mastoid air cells. Auditory ossicles were completely destroyed, except for the footplate of stapes. Signs of osseous destruction were present on the walls of the cavum tympani and mastoid. Fibroproliferative process invaded the middle ear region spreading on the surrounding bony structures, i.e. the left maxillofacial region. Cholesteatoma and fibrous dysplasia were confirmed histologically. Postoperatively, the patient was well and without any sings of recurrence of cholesteatoma about 2 years.
Fig. 1. A CT scan showed a massive fibrous dysplasia of the left temporal bone involving the middle ear, mastoid and inner ear. Fibroproliferative process extended to the maxillar and ethmoidal bones.
D. Djeric´, P. Stefano6ic´ / Auris Nasus Larynx 26 (1999) 79–81
3. Discussion MFD is a slow-growing benign process clinically manifested only after puberty. Progressive conductive hearing loss developing due to obstruction of the external auditory canal, Eustachian tube or restricted mobility of auditory ossicles is the most common otologic symptom of MFD. Development of pathological fibroproliferative process leads to obstruction of the external auditory canal and keratin deposit, which may gradually lead to cholesteatoma. Insidious development of cholesteatoma may last longer with minor clinical signs, in spite of extensive involvement of the temporal bone. In some cases the disease is diagnosed only after the development of complications. Yagoda and Selesnick [5] reported a case of temporal bone MFD with secondary cholesteatoma leading to parapharyngeal abscess with minimal otologic symptoms. Our case may be of interest because of insidious development of cholesteatoma which was silent until a retroauricular fistule developed. It was a decisive factor for diagnosis and indication for
.
81
surgical treatment. A CT scan was important in the preoperative evaluation of the disease, although soft tissue structures such as ‘cholesteatoma-fibroproliferative process’ were difficult to distinguish. At surgery of the temporal bone, it was found that the area of fibrous dysplasia of the middle ear was filled with a massive cholesteatoma. Surgical eradication of the pathological process was very important to prevent the development of the other complications.
References [1] Nager G, Kennedy D, Kopstein E. Fibrous dysplasia: a review of the disease and its manifestations in the temporal bone. Ann Otol Rhinol Laryngol 1982;91(Suppl. 92):1 – 52. [2] Lichtenstein L. Polyostotic fibrous dysplasia. Arch Surg 1938;36:874 – 98. [3] Shapeero L, Vanel D, Ackerman L, Terrier-Lacombe M. Aggressive fibrous dysplasia of the maxillary sinus. Skeletal-Radiol 1993;22(8):563– 8. [4] Younis M, Haleem A. Monostotic fibrous dysplasia of the temporal bone. J Laryngol Otol 1987;101:1070 – 4. [5] Yagoda M, Selesnick H. Temporal bone fibrous dysplasia and cholesteatoma leading to the development of a parapharyngeal abscess. J Laryngol Otol 1994;108:51 – 3.
Case report
Fibrous dysplasia of the temporal bone and maxillofacial region associated with cholesteatoma of the middle ear Dragoslava Djeric´ *, Predrag Stefanovic´ Institute of Otorhinolaryngology and Maxillofacial Surgery, Clinical Center of Serbia, Pastero6a 2, 11000 Belgrade, Yugosla6ia Received 10 April 1998; received in revised form 16 June 1998; accepted 17 July 1998
Abstract Fibrous dysplasia of the temporal bone is a rare disease which may lead to progressive stenosis of the external auditory canal and the development of cholesteatoma. We present a case in which minimal symptoms were present despite a massive temporal bone fibrous dysplasia. Cholesteatoma resulted most probably secondary to external auditory canal stenosis. Retroauricular fistula developed as a result of destructive effect of cholesteatoma, that influenced previous diagnosis and treatment of this clinically silent disease. © 1999 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Fibrous dysplasia; Temporal bone; Cholesteatoma
1. Introduction Fibrous dysplasia is a disease with unelucidated etiology, characterized by skeletal abnormality which may be associated with endocrinopathies, abnormal pigmentation of the skin and mucosal tissues, as well as by some other pathological disorders [1]. It is a relatively uncommon disease, which may occur in several clinical forms: (i) * Corresponding author. Tel.: + 381 11 643694; fax: + 381 11 643034.
monosotic fibrous dysplasia (MFD)—involvement of a single bone (70%); and (ii) polyostotic fibrous dysplasia—involvement of multiple bones (30%) and McCune–Albright syndrome—bony involvement associated with skin lesions and endcrinopathies which is very rare [2]. Although MFD occurs on craniofacial structures only in 10% of cases, this is the most frequent type of fibrous dysplasia (FD) in the head and neck region. The maxilla and mandible are most commonly affected cranio-maxillo-facial bones [3]. The temporal bone is affected only in 18% of
0385-8146/99/$ - see front matter © 1999 Elsevier Science Ireland Ltd. All rights reserved. PII S0385-8146(98)00061-3
80
D. Djeric´, P. Stefano6ic´ / Auris Nasus Larynx 26 (1999) 79–81
cases and the lesions are more frequently monostotic than polyostotic forms [4]. The aim of this report is present a patient with cholesteatoma developing in a case of fibrous dysplasia of the temporal bone and maxillofacial region leading to a retroauricular fistula.
2. Case report A 22-year-old man was referred to the Institute of Otorhinolaryngology and Maxillofacial Surgery, University Medical Center in Belgrade with otorrhea and hearing loss. His recurrent left-sided otorrhea began at the age of seven. He had frequent episodes for 6 years. However, in puberty, a progressive left-sided hearing loss developed associated with deformity at the left side of the face, most prominent in the zygomatic part. In the following 5 years the left mastoid region enlarged gradually and foul otorrhea recurred. Owing to deformity in the left mastoid region, the patient was subjected to surgery at a local ENT clinic twice, at the ages of 18 and 20. After the operations, the progressive hearing loss persisted and otorrhea was somewhat milder. However, leakage from the left retroauricular region occurred. A month prior to admission to our Institute the patient noticed edema in the left mastoid region, occasional ipsilateral pain and suppuration from an opening on the skin localized in the upper retroauricular region. On physical examination facial asymmetry was evidenced with a protrusion in the left zygomatic area was noted. Otoscopic examination revealed a stenosis of the left external auditory canal. Medially to the stenosis a massive cholesteatoma was seen, filling the middle ear spaces. In the left retroauricular region, a fistula was present with foul discharge. The Weber and Rinne tuning fork evaluation suggested a left-sided conductive hearing loss. The remaining part of the head and neck appeared normal. Audiologic examination revealed a severe mixed hearing loss on the left ear and normal findings on the right. Serum chemistry was all normal except for alkaline phorphatgase value which was four-fold. Calcium and phosphorus levels were also normal.
A CT scan of the temporal bone and paranasal sinuses revealed a massive fibrous dysplasia of the left temporal bone with involvement of otic capsule structures. Besides, the process extended on the adjoining bones, affecting the maxillar and ethmoidal bones. Obliteration of the left maxillar and ethmoidal sinuses was also evident. The area of fibrous dysplasia of the temporal bone was presented as a soft-tissue shadow in the region of the middle ear and mastoid (Fig. 1). Tympanomastoidectomy of the left ear was performed eradicating the pathological process. Intraoperatively a large cholesteatoma was found filling the middle ear spaces and mastoid air cells. Auditory ossicles were completely destroyed, except for the footplate of stapes. Signs of osseous destruction were present on the walls of the cavum tympani and mastoid. Fibroproliferative process invaded the middle ear region spreading on the surrounding bony structures, i.e. the left maxillofacial region. Cholesteatoma and fibrous dysplasia were confirmed histologically. Postoperatively, the patient was well and without any sings of recurrence of cholesteatoma about 2 years.
Fig. 1. A CT scan showed a massive fibrous dysplasia of the left temporal bone involving the middle ear, mastoid and inner ear. Fibroproliferative process extended to the maxillar and ethmoidal bones.
D. Djeric´, P. Stefano6ic´ / Auris Nasus Larynx 26 (1999) 79–81
3. Discussion MFD is a slow-growing benign process clinically manifested only after puberty. Progressive conductive hearing loss developing due to obstruction of the external auditory canal, Eustachian tube or restricted mobility of auditory ossicles is the most common otologic symptom of MFD. Development of pathological fibroproliferative process leads to obstruction of the external auditory canal and keratin deposit, which may gradually lead to cholesteatoma. Insidious development of cholesteatoma may last longer with minor clinical signs, in spite of extensive involvement of the temporal bone. In some cases the disease is diagnosed only after the development of complications. Yagoda and Selesnick [5] reported a case of temporal bone MFD with secondary cholesteatoma leading to parapharyngeal abscess with minimal otologic symptoms. Our case may be of interest because of insidious development of cholesteatoma which was silent until a retroauricular fistule developed. It was a decisive factor for diagnosis and indication for
.
81
surgical treatment. A CT scan was important in the preoperative evaluation of the disease, although soft tissue structures such as ‘cholesteatoma-fibroproliferative process’ were difficult to distinguish. At surgery of the temporal bone, it was found that the area of fibrous dysplasia of the middle ear was filled with a massive cholesteatoma. Surgical eradication of the pathological process was very important to prevent the development of the other complications.
References [1] Nager G, Kennedy D, Kopstein E. Fibrous dysplasia: a review of the disease and its manifestations in the temporal bone. Ann Otol Rhinol Laryngol 1982;91(Suppl. 92):1 – 52. [2] Lichtenstein L. Polyostotic fibrous dysplasia. Arch Surg 1938;36:874 – 98. [3] Shapeero L, Vanel D, Ackerman L, Terrier-Lacombe M. Aggressive fibrous dysplasia of the maxillary sinus. Skeletal-Radiol 1993;22(8):563– 8. [4] Younis M, Haleem A. Monostotic fibrous dysplasia of the temporal bone. J Laryngol Otol 1987;101:1070 – 4. [5] Yagoda M, Selesnick H. Temporal bone fibrous dysplasia and cholesteatoma leading to the development of a parapharyngeal abscess. J Laryngol Otol 1994;108:51 – 3.