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Facial Paralysis Due to
Recurrent Cholesteatoma Seventeen Years After
First Surgery
Hilal A.A. and Al Shaikhly A.J.
ENT Section, Department of Surgery, Hamad Medical
Corporation Doha, Qatar
Abstract:
We present a case of facial nerve palsy induced
by recurrent cholesteatoma in a quiescent ear,
17 years after initial mastoidectomy. The pathogenesis,
clinical and radiological findings and the management
strategies are discussed with an emphasis on regular
long-term follow up.
Key words: Cholesteatoma, Recurrent, Facial palsy,
Mastoidectomy
  Introduction:
Recurrent disease still remains a problem to
be solved in the management of middle-ear cholesteatoma
to ensure a long-lasting remission. Complete removal
of the cholesteatoma matrix is the aim that is
not always feasible at the initial surgery even
with the advent of modern microsurgical techniques.
The spectrum of complications induced by cholesteatoma
is very wide ranging from mild quiescent to severe
life-threatening ones. We report a case of a recurrent
cholesteatoma presenting as facial nerve palsy
17 years after the initial surgery.
 Case
Report:
A 27 year-old female presented to the A &
E Department of Hamad General Hospital (Doha)
with a history of rapidly progressing left facial
weakness of ten days duration. She had no ear
pain, discharge, swelling or recent hearing loss
but there was a past history of ear surgery 17
years back in the same hospital when, aged ten,
she had an attic cholesteatoma that was managed
by a modified-radical mastoidectomy, canal-wall-down
procedure. There had been no facial weakness before
or after the surgery and she had disappeared from
follow-up after the second post-operative visit..
Clinical examination revealed a grade 4/6 House-Brackman
left-sided facial weakness, an old post-auricular
scar and severe external auditory canal stenosis.
Retained wax was difficult to remove and made
it impossible to inspect the tympano-mastoid cavity
but the ear was totally dry.
The patient was investigated by audiometry (50
db conductive hearing loss) and by temporal bone
CT scan (Figure 1) which demonstrated the presence
of an expansile soft tissue mass within the external
canal, middle ear and mastoid cavities, eroding
their walls and incorporating the facial canal.
The inner ear structures and the tegmen seemed
to be spared. That picture raised the possibility
of a malignant neoplasm and so an MRI scan was
requested (Figure 2) which confirmed the presence
of a mixed signal intensity mass (in T1 and T2-weighted
images) in the middle ear cavity suggesting either
a cholesteatoma or a poorly vascularized tumor.
Figure
1: Axial temporal bone CT scan showing
an extensive lytic soft tissue lesion within
the left petrous bone
A revision mastoid exploration using a post-auricular
approach confirmed the presence of an extensive
keratin-filled sac (about 3 cm in diameter) within
the pre-existing tympano-mastoid cavity (Figure
3) plus a retained piece of cotton wool and a
narrowed external auditory meatus. The cholesteatoma
matrix was peeled easily from the intact bony
walls of the cavity. The bony facial nerve canal
was partially eroded in its tympanic segment but
with preserved nerve continuity in its tympanic
and mastoid portions. There were no identifiable
middle ear structures apart from the foot-plate
of the stapes.
After complete removal of the disease, the bony
contours were re-fashioned and a wide meatoplasty
was done. The cav-
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Figure 2: (a)
T1-weighted and (b) T2-weighted MR images
showing heterogenous signal intensity mass in the
left middle ear and mastoid cavities.
Figure 3: The keratin sac (cholesteatoma)
after complete removal from the
middle ear.
ity was packed gently with Gelfoam
and a BIPP pack. The patient had no post-operative
problems; she continued the ten-day course of
systemic corticosteroids started at the time of
presentation three days prior to surgery and a
course of systemic antibiotics until the pack
was removed three weeks later. The facial nerve
function started to recover starting from the
second post-operative week aided by vigorous facial
physiotherapy (Figure 4) and the cavity healed
without complications.
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(a) |
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(b) |
Figure 4:
The left facial nerve function: (a) before
and (b) 3 months after the intervention.
Discussion:
Cholesteatoma in children has been the subject
of numerous publications in the past two decades
but significant controversy remains concerning
the natural history and optimal management of
this challenging disorder. Specific issues relate
to the differences between cholesteatoma in children
and adults, canal wall up (CWU) versus canal wall
down (CWD) mastoidectomy, the timing and the need
for a second-look procedures, the length of post-operative
follow up and the potentially unique behavior
of congenital cholesteatoma(1).
To avoid residual cholesteatoma and hence recurrent
ear disease or complications, complete removal
of the cholesteatoma matrix is necessary at the
initial procedure otherwise a second-look operation
is indicated. Many authors revised the possible
factors predicting residual/ recurrent cholesteatoma:
Ogawa et al. divided the expectation of residual
cholesteatoma into four groups (‘none’, ‘low-rate’,
high-rate’, and ‘certain’) based on the maintenance
of continuity of the cholesteatoma matrix during
the initial surgery(2).
While Iino et al. determined the risk factors
for recidivism by male gender, pars flaccida type
of cholesteatoma and the association of otitis
media with effusion(3). The rate of residual/
recurrent cholesteatoma after an average of 10
years from the initial surgery (CWD) varied in
the reviewed literature from 6%(4),
17%(5), 29%(6),
to 38%(1). However, most authors agree that the
rate of residual/ recurrent cholesteatoma is directly
related to the duration of follow up; the longer
the follow up, the higher the chance of developing
recidivism and the necessity of a lifelong observation
especially in the first six years of surgery(1).
In our case the patient was lost to follow-up
a few months after her first operation until she
presented with a major complication 17 years later;
a condition that proved that the cholesteatoma
was growing slowly over the years and was possibly
aggravated by poor cavity ventilation due to the
missed piece of cotton-wool. However, recurrence
usually manifests more rapidly in the form of
otorrhea, aggravated hearing loss, or inner ear
disease.
It is important not to miss cholesteatoma as a
primary cause of facial nerve palsy, although
neoplastic factors and tumour-like conditions
such as cholesteatoma constitute only 5 –13% of
the aetiology of facial nerve palsy(7
,8). The
most likely pathogenesis for facial palsy in cholesteatoma
is a direct compression or enzymatic resorption
of the nerve and its bony canal, although there
has been a single reported case of nerve transection
by a middle ear cholesteatoma(9). The most vulnerable
portion is the tympanic segment, in a case of
acquired cholesteatoma (as was our case), or the
area of the geniculate ganglion, in a case of
congenital type.
CT and MRI scans were very helpful in establishing
the pre-operative diagnosis which showed an expansile
lytic lesion in the tymanomastoid cavity (on CT
scan), and typical features of cholesteatoma namely
hypointensity on T1- and hyperintensity on T2-weighted
images. If we consider the CT scan findings alone,
less common pathologies should also be considered
in the differential diagnosis (e.g. facial neuroma,
glomus tumors, meningioma, adenoma, adenocarcinoma,
osteosarcoma, rhabdomyosarcoma, and metastatic
tumors)(10 ,11).
Whatever was the case, a mastoid exploration was
necessary to decompress the facial nerve and to
identify the pathology by a tissue diagnosis.
The principle of surgical management is to exteriorize
and, if possible, to completely excise the cholesteatoma
sac without opening the perineural sheath of the
facial nerve in case of chronic infection as in
our case. Most of the cavity was found to be nicely
lined with squamous epithelium, where only a small
piece of temporalis fascia was necessary to cover
the denuded part of the facial nerve followed
by a wide meatoplasty, to allow an easily accessible
post-operative cavity for inspection and toilet.
The facial weakness recovered within a few weeks
of removal of the compressing mass, as is the
experience of most authors. However, there is
a role for a primary facial nerve repair (by nerve
graft) in patients with complete facial palsy
of significant duration pre-operatively and secondary
grafting should be considered when facial nerve
function does not recover at all within 6-9 months
or when only minimal function is present at 12-15
months after operation(12).
Conclusion:
The recurrence rate of childhood cholesteatoma
can approach up to 70%; therefore a second planned
surgery is highly advisable in 6-12 months regardless
of the type of primary procedure (CWD or CWU)
which is decided on an individual case-by-case
basis(1,4). Intense follow-up is of prime importance
especially in the first three years for early
detection of residual/ recurrent disease which
rarely manifests itself as facial palsy. Radiological
diagnostic tools are very helpful to establish
the pre-operative diagnosis. The prognosis of
facial nerve insult depends on the exact neuropathology
(compression, resorption or transection), the
delay in ear exploration, and complete eradication
of the disease(12).
References:
1. Rosenfeld R, Moura R, Bluestone
C. Predictors of residual- recurrent cholesteatoma
in children. Arch Otolaryngol Head Neck Surg 1992;
118: 384-92.
2. Ogawa H, Ohtani I, Akaike T. Examination for
prediction of residual cholesteatoma. Nippon Jibiinkoka
Gakkai Kaiho 1998; 101: 1-8.
3. Iino Y, Imamura Y, Kojima C, Takegoshi S, Suzuki
JI. Risk factors for recurrent and residual cholesteatoma
in children determined by second stage operation.
Int J Pediatr Otorhinolaryngol 1998; 46: 57-65.
4. Castrillon R, Kos I, Montandon P, Guyot JP.
Long-term results of canal-wall-down mastoidectomy.
Schweiz Med Wochenschr 2000; Suppl: 12558s-61s.
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paralysis. Otolaryngol Clin North Am 1997; 30:
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nerve transection. J Laryngol Otol 2001; 115:
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10. Cureoglu S, Osma U, Oktay F, Nazaroglu H,
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11. Oghalai J, Favrot S, Coker N. Imaging quiz
case 2. Arch Otolaryngol Head Neck Surg 1996;
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12. Bartels LJ. Facial nerve and medially invasive
petrous bone cholesteatomas. Ann Otorhinol Laryngol
1991; 100: 308-17.
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