Massive acquired cholesteatoma of the temporal bone J. L. W. WRIGHT*, B. H. COLMAN and A. F. CONNOR (Oxford) Introduction
FEW otologists would disagree with the term congenital cholesteatoma (Cawthorne and Griffith, 1961; Cawthorne, 1963), when applied to epidermoid rests which occur deep in the petrous bone. Patients are generally young and present with neurological signs, the commonest being facial and trigeminal nerve deficits (Titus and Pennybacker, 1956). However similar gross destruction of the petrous bone with severe morbidity can occur in patients with acquired cholesteatoma who have a history of chronic ear disease. We would like to report and discuss the management of nine patients with massive temporal bone cholesteatoma seen and treated by the two senior authors. Eight patients were seen at the Radcliffe Infirmary, Oxford, and one patient at St. Mary's Hospital, London, W2. Clinical features
There were five male and four female patients with ages ranging from 17 to 59 years. Seven patients had had chronic suppurative ear disease for more than one year while two patients with short histories presented with acute infections. Chronic otorrhoea and profound hearing loss in the affected ear were the commonest symptoms but six patients had additional symptoms such as otalgia, vertigo, external swelling and facial nerve palsay. Four patients had previously undergone a form of mastoid surgery (Table I). All patients had a profound mixed conductive and sensorineural hearing loss preoperatively, and a total hearing loss postoperatively on the affected side, as the inner ear was removed to facilitate access to the petrous apex. Radiological findings
Plain radiographs of the skull were helpful in assessing the degree of pneumatization, and in one case showed an extradural collection of gas in the petrous bone and middle cranial fossa (Fig. 1). However for accurate preoperative delineation of the extent of the lesion polytomography was * Formerly First Assistant, Radcliffe Infirmary, Oxford. Now Consultant Surgeon, St. Mary's Hospital, London.
257
J. L. W. Wright, B. H. Colman and A. F. Connor found to be most useful. Tomographs taken in the coronal plane were used to evaluate base of skull (Fig. 2), and tegmen plate defects (Fig. 3). TABLE 1. CLINICAL FEATURES IN NINE PATIENTS WITH MASSIVE ACQUIRED CHOLESTEATOMA OF THE TEMPORAL BONE
Patient
Age Sex
Length of History
Presentation
Previous surgery Cortical Mastoidectomy
1
17
M
9 years
Otorrhoea Otalgia
2
32
M
1 year
Otorrhoea Meningitis
Nil
3
38
M
3 weeks
Facial palsy
Nil
4
43
F
30 years
Facial palsy. Meningitis
Mastoidectomy (Unknown)
5
47
M
8 years
Vertigo
Atticotomy
6
49
F
18 years
Otorrhoea
Atticotomy
7
52
F
2 years
Bezold's abscess
Nil
8
52
M
4 months
Facial palsy
Nil
2 years
Vertigo
Nil
59
Surgical procedure
At operation cholesteatoma was found to be extending to the petrous apex with variable destruction of the semicircular canals, vestibule and cochlea. In two cases the bony labyrinth was freely mobile in the petrous bone due to complete bone erosion around it. Cholesteatoma matrix was found in the internal auditory meatus, on the internal carotid artery and the jugular bulb. It invariably surrounded the facial nerve. The aims of surgery were total removal of cholesteatoma and preservation of the facial nerve. Any slight residual hearing present was sacrificed in order to achieve good access to the deeper portion of the petrous bone. The facial nerve was decompressed from the internal auditory meatus to the stylomastoid foramen and retracted anteriorly out of the surgical field. The inner ear was then ablated and extensions of cholesteatoma were removed from the internal meatus, from the jugular bulb, and from around the internal carotid artery. Following clearance of cholesteatoma the posterior portion of the cavity was filled with a superiorly based temporalis muscle pedicle. This provided a soft bed on which to replace the facial nerve, and support for the large area of middle fossa dura exposed, thus lessening the possibility of a subsequent cerebral hernia. The anterior portion of the cavity was left open for inspection and cleaning. A large Koerner flap meatoplasty was 258
Massive acquired cholesteatoma of the temporal bone
FIG. I.
Plain skull radiograph showing extradural gas in the petrous bone and middle cranial fossa.
FIG. 2. Base of skull polytomograph in the antero-posterior plane showing severe bone erosion in the region of the jugular bulb.
259
J. L. W. Wright, B. H. Colman and A. F. Connor
FIG. 3. Antero-posterior polytomograph showing a large defect in the tegmen plate.
then performed to provide good access to the cavity via the external meatus. Morbidity
There was cerebrospinal fluid leakage from the internal meatus during surgery in four patients, and this was sealed with a small free muscle graft. The muscle pedicle then provided a good second layer closure and there were no cases of persistent leakage, or postoperative meningitis. Three patients had a preoperative total facial nerve palsy and nerve conductivity tests showed evidence of degeneration. Recovery was incomplete at a one year follow-up. Six patients with normal facial nerve function preoperatively had a partial palsy in the immediate postoperative period. This was a neuropraxia due to handling of the nerve during operation and full recovery was seen within four weeks. Postoperative vertigo with a paralytic nystagmus was seen in seven patients but symptoms were not severe and early ambulation was possible. One patient with residual ataxia had bilateral disease and both labyrinths had been removed (Table II). Discussion
There has been much previous discussion on the aetiology of massive cholesteatoma with extensive bone erosion. Cawthorne (1963) and Derlacki and Clemis (1965) felt that many cases may be congenital in origin. They 260
Massive acquired cholesteatoma of the temporal bone TABLE II. RESIDUAL DISABILITY IN NINE PATIENTS WITH MASSIVE ACQUIRED CHOLESTEATOMA. ALL PATIENTS HAD A TOTAL HEARING LOSS IN THE AFFECTED EAR
Patient
Age
I 2
17 32
3 4 5 6 7 8 9
38 43 47 49 52 52
59
Sex Disability M M M F M F F M F
Nil
Headaches Facial palsy Facial palsy Nil
Ataxia, total bilateral hearing loss Nil
Facial palsy Nil
postulated that expansion of the lesion was followed by exteriorization via the attic and subsequent infection with foul discharge. In support of this view Smith (1968) described his operative findings in a case of attic cholesteatoma where there was no apparent connection with the tympanic membrane. The lesion was lined by non-keratinizing basal cells. On the other hand Buckingham (1968) reported a photographic study of cholesteatoma development behind an intact tympanic membrane in a child with recurring serous otitis media, and Ruedi (1963) found nonkeratinizing basal cells in histological specimens of secondary acquired cholesteatoma. Recent light and electron microscopic studies by Lim and Saunders (1972) and by Sade and Halevy (1974) have shown that osteoclastic activity during infection in cholesteatomatous ears leads to rapid bone erosion, and that pressure plays very little part in the process. We do not therefore advocate a conservative atticotomy or suction clearance approach to attic disease (Cronin et al., 1974), based on exteriorization or uncapping the lesion, but rather a radical and thorough operative removal of cholesteatoma matrix before massive erosion can occur. Summary Nine patients with massive acquired cholesteatoma of the temporal bone were treated by radical mastoidectomy, facial nerve decompression, and excision of the inner ear. The commonest presenting symptoms were otorrhoea and deafness, but otalgia, vertigo, external swelling and facial nerve palsies were also seen. Polytomography was helpful in assessing the degree of bone erosion preoperatively. The large cavity present following cholesteatoma excision was partially obliterated by a superiorly based temporalis muscle pedicle which helped to seal the internal auditory meatus and prevented the development of a cerebral hernia. Those patients with preoperative facial nerve palsies showed evidence of nerve degeneration and had incomplete recovery at a one year follow-up. 261
J. L. W. Wright, B. H. Colman and A. F. Connor REFERENCES BUCKINGHAM, R. A. (1968) Annals of Otology, Rhinology and Laryngology, 77, 1054. CAWTHORNE, T. (1963) Archives of Otolaryngology, 78, 248. CAWTHORNE, T., and GRIFFITH, A. (1961) Archives of Otolaryngology, 73, 252. CRONIN, J., DOGRA, T. S., and KHAN, I. A. (1974) Journal of Laryngology and Otology,
88, 113. DERLACKI, E. L., and CLEMIS, J. D. (1965) Annals of Otology, Rhinology and Laryngology, 74, 706. LIM, D. J., and SAUNDERS, W. H. (1972) Annals of Otology, Rhinology and Laryngology, 81, 2. RUEDI, L. (1963) Archives of Otolaryngology, 78, 252. SADE, J., and HALEVY, A. (1974) Journal of Laryngology and Otology, 88, 139. SMITH, A. BROWNLIE (1968) Journal of Laryngology and Otology, 82, 1049. TITUS, J. S., and PENNYBACKER, J. (1956) Journal of Neurology, Neurosurgery and Psychiatry, 19, 241. E.N.T. Department, St. Mary's Hospital, London W2.
262
j
FEW otologists would disagree with the term congenital cholesteatoma (Cawthorne and Griffith, 1961; Cawthorne, 1963), when applied to epidermoid rests which occur deep in the petrous bone. Patients are generally young and present with neurological signs, the commonest being facial and trigeminal nerve deficits (Titus and Pennybacker, 1956). However similar gross destruction of the petrous bone with severe morbidity can occur in patients with acquired cholesteatoma who have a history of chronic ear disease. We would like to report and discuss the management of nine patients with massive temporal bone cholesteatoma seen and treated by the two senior authors. Eight patients were seen at the Radcliffe Infirmary, Oxford, and one patient at St. Mary's Hospital, London, W2. Clinical features
There were five male and four female patients with ages ranging from 17 to 59 years. Seven patients had had chronic suppurative ear disease for more than one year while two patients with short histories presented with acute infections. Chronic otorrhoea and profound hearing loss in the affected ear were the commonest symptoms but six patients had additional symptoms such as otalgia, vertigo, external swelling and facial nerve palsay. Four patients had previously undergone a form of mastoid surgery (Table I). All patients had a profound mixed conductive and sensorineural hearing loss preoperatively, and a total hearing loss postoperatively on the affected side, as the inner ear was removed to facilitate access to the petrous apex. Radiological findings
Plain radiographs of the skull were helpful in assessing the degree of pneumatization, and in one case showed an extradural collection of gas in the petrous bone and middle cranial fossa (Fig. 1). However for accurate preoperative delineation of the extent of the lesion polytomography was * Formerly First Assistant, Radcliffe Infirmary, Oxford. Now Consultant Surgeon, St. Mary's Hospital, London.
257
J. L. W. Wright, B. H. Colman and A. F. Connor found to be most useful. Tomographs taken in the coronal plane were used to evaluate base of skull (Fig. 2), and tegmen plate defects (Fig. 3). TABLE 1. CLINICAL FEATURES IN NINE PATIENTS WITH MASSIVE ACQUIRED CHOLESTEATOMA OF THE TEMPORAL BONE
Patient
Age Sex
Length of History
Presentation
Previous surgery Cortical Mastoidectomy
1
17
M
9 years
Otorrhoea Otalgia
2
32
M
1 year
Otorrhoea Meningitis
Nil
3
38
M
3 weeks
Facial palsy
Nil
4
43
F
30 years
Facial palsy. Meningitis
Mastoidectomy (Unknown)
5
47
M
8 years
Vertigo
Atticotomy
6
49
F
18 years
Otorrhoea
Atticotomy
7
52
F
2 years
Bezold's abscess
Nil
8
52
M
4 months
Facial palsy
Nil
2 years
Vertigo
Nil
59
Surgical procedure
At operation cholesteatoma was found to be extending to the petrous apex with variable destruction of the semicircular canals, vestibule and cochlea. In two cases the bony labyrinth was freely mobile in the petrous bone due to complete bone erosion around it. Cholesteatoma matrix was found in the internal auditory meatus, on the internal carotid artery and the jugular bulb. It invariably surrounded the facial nerve. The aims of surgery were total removal of cholesteatoma and preservation of the facial nerve. Any slight residual hearing present was sacrificed in order to achieve good access to the deeper portion of the petrous bone. The facial nerve was decompressed from the internal auditory meatus to the stylomastoid foramen and retracted anteriorly out of the surgical field. The inner ear was then ablated and extensions of cholesteatoma were removed from the internal meatus, from the jugular bulb, and from around the internal carotid artery. Following clearance of cholesteatoma the posterior portion of the cavity was filled with a superiorly based temporalis muscle pedicle. This provided a soft bed on which to replace the facial nerve, and support for the large area of middle fossa dura exposed, thus lessening the possibility of a subsequent cerebral hernia. The anterior portion of the cavity was left open for inspection and cleaning. A large Koerner flap meatoplasty was 258
Massive acquired cholesteatoma of the temporal bone
FIG. I.
Plain skull radiograph showing extradural gas in the petrous bone and middle cranial fossa.
FIG. 2. Base of skull polytomograph in the antero-posterior plane showing severe bone erosion in the region of the jugular bulb.
259
J. L. W. Wright, B. H. Colman and A. F. Connor
FIG. 3. Antero-posterior polytomograph showing a large defect in the tegmen plate.
then performed to provide good access to the cavity via the external meatus. Morbidity
There was cerebrospinal fluid leakage from the internal meatus during surgery in four patients, and this was sealed with a small free muscle graft. The muscle pedicle then provided a good second layer closure and there were no cases of persistent leakage, or postoperative meningitis. Three patients had a preoperative total facial nerve palsy and nerve conductivity tests showed evidence of degeneration. Recovery was incomplete at a one year follow-up. Six patients with normal facial nerve function preoperatively had a partial palsy in the immediate postoperative period. This was a neuropraxia due to handling of the nerve during operation and full recovery was seen within four weeks. Postoperative vertigo with a paralytic nystagmus was seen in seven patients but symptoms were not severe and early ambulation was possible. One patient with residual ataxia had bilateral disease and both labyrinths had been removed (Table II). Discussion
There has been much previous discussion on the aetiology of massive cholesteatoma with extensive bone erosion. Cawthorne (1963) and Derlacki and Clemis (1965) felt that many cases may be congenital in origin. They 260
Massive acquired cholesteatoma of the temporal bone TABLE II. RESIDUAL DISABILITY IN NINE PATIENTS WITH MASSIVE ACQUIRED CHOLESTEATOMA. ALL PATIENTS HAD A TOTAL HEARING LOSS IN THE AFFECTED EAR
Patient
Age
I 2
17 32
3 4 5 6 7 8 9
38 43 47 49 52 52
59
Sex Disability M M M F M F F M F
Nil
Headaches Facial palsy Facial palsy Nil
Ataxia, total bilateral hearing loss Nil
Facial palsy Nil
postulated that expansion of the lesion was followed by exteriorization via the attic and subsequent infection with foul discharge. In support of this view Smith (1968) described his operative findings in a case of attic cholesteatoma where there was no apparent connection with the tympanic membrane. The lesion was lined by non-keratinizing basal cells. On the other hand Buckingham (1968) reported a photographic study of cholesteatoma development behind an intact tympanic membrane in a child with recurring serous otitis media, and Ruedi (1963) found nonkeratinizing basal cells in histological specimens of secondary acquired cholesteatoma. Recent light and electron microscopic studies by Lim and Saunders (1972) and by Sade and Halevy (1974) have shown that osteoclastic activity during infection in cholesteatomatous ears leads to rapid bone erosion, and that pressure plays very little part in the process. We do not therefore advocate a conservative atticotomy or suction clearance approach to attic disease (Cronin et al., 1974), based on exteriorization or uncapping the lesion, but rather a radical and thorough operative removal of cholesteatoma matrix before massive erosion can occur. Summary Nine patients with massive acquired cholesteatoma of the temporal bone were treated by radical mastoidectomy, facial nerve decompression, and excision of the inner ear. The commonest presenting symptoms were otorrhoea and deafness, but otalgia, vertigo, external swelling and facial nerve palsies were also seen. Polytomography was helpful in assessing the degree of bone erosion preoperatively. The large cavity present following cholesteatoma excision was partially obliterated by a superiorly based temporalis muscle pedicle which helped to seal the internal auditory meatus and prevented the development of a cerebral hernia. Those patients with preoperative facial nerve palsies showed evidence of nerve degeneration and had incomplete recovery at a one year follow-up. 261
J. L. W. Wright, B. H. Colman and A. F. Connor REFERENCES BUCKINGHAM, R. A. (1968) Annals of Otology, Rhinology and Laryngology, 77, 1054. CAWTHORNE, T. (1963) Archives of Otolaryngology, 78, 248. CAWTHORNE, T., and GRIFFITH, A. (1961) Archives of Otolaryngology, 73, 252. CRONIN, J., DOGRA, T. S., and KHAN, I. A. (1974) Journal of Laryngology and Otology,
88, 113. DERLACKI, E. L., and CLEMIS, J. D. (1965) Annals of Otology, Rhinology and Laryngology, 74, 706. LIM, D. J., and SAUNDERS, W. H. (1972) Annals of Otology, Rhinology and Laryngology, 81, 2. RUEDI, L. (1963) Archives of Otolaryngology, 78, 252. SADE, J., and HALEVY, A. (1974) Journal of Laryngology and Otology, 88, 139. SMITH, A. BROWNLIE (1968) Journal of Laryngology and Otology, 82, 1049. TITUS, J. S., and PENNYBACKER, J. (1956) Journal of Neurology, Neurosurgery and Psychiatry, 19, 241. E.N.T. Department, St. Mary's Hospital, London W2.
262
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