Another clinical dilemma

Submitted by pmelo on Tue, 10/16/2018 - 15:16

Dear Colleagues,

I bring the following case to discussion and would appreciate your thoughts on how to manage this difficult case:

This is a 63-year-old female patient from Mozambique who has had recurrent episodes of bilateral otorrhea for about ten years. In 2013 she underwent bilateral otologic surgery in another hospital. According to previous medical report, CT scan of the temporal bones was performed before surgery and revealed: "bilateral middle ear filling with destruction of the ossicular chain. Erosion of the otic capsule on the right side at the level of the cochlear basal turn and vestibule". According to the same clinical record, she was submitted to simultaneous bilateral mastoidectomy. The middle ear was filled with "soft tissue" and a sample was sent to histopathological analysis whose result was inconclusive. Microbiological analysis (bacteriological and mycological) was also negative.

She was sent to our hospital in 2018 and by that time she was already bilaterally severely deafened.

Otoscopy revealed bilateral stenosis of the membranous portion of the external auditory canal, making it impossible to visualize the tympanic membrane. There were no inflammatory exudates. The retro-auricular regions had no inflammatory signs and operative scars were present bilaterally.

She performed pure-tone audiometry that showed bilateral profound sensorineural hearing loss. A recent CT scan of the temporal bones revealed bilateral complete filling of both mastoid and middle ear spaces with nonspecific soft tissue mass and extensive demineralization / erosion of middle ear structures, labyrinth and otic capsules (images in attached files).

She has a pacemaker which makes it difficult to perform magnetic resonance imaging.

Blood analysis showed normal hemogram, renal and liver functions. Analysis targeting autoimmune diseases revealed a positive ANA (antinuclear antibodies) with centromere pattern and a small monoclonal IgG kappa peak. Serum IgG subclasses were within the normal range. Rheumatoid factor, ANCA (anti-neutrophil cytoplasmic antibodies), ESR (erythrocyte sedimentation rate) and ACE (angiotensin-converting enzyme) levels were normal. Serological testing for HIV, Hepatitis B and C and syphilis were negative. Urinalysis was normal.

This patient was recently sent to a rheumatology consultation and is still being studied (autoimmune disease? IgG4 related disease?).

For now we are considering surgery to perform only tissue biopsy that could help targeting diagnosis and treatment.

In the long term, what is your advice to this situation (considering its aggressive and destructive behavior)? Any thoughts on differential diagnosis?

Thank you for your help

Best regards

Patrícia Melo

Lisbon, Portugal

LinderThomas

Wed, 10/17/2018 - 17:57

Dear Particia Melo

As differential diagnosis you may think of tuberculosis (I guess you had already inverstigated this option), histiocytosis or another granulating disease.  You did not provide the Hearing test results, but the Cochlea on the left side is eroded and I assume the Hearing loss is severe. I did not see the right side on your CT Scans, or may have missed it.

I suggest to test for tuberculosis, once this is excluded I suggest to perform a Revisions-Sutotal petrosectomy with complete removal of the disease on the left side and eventually plan a cochlear implant (either during the same surgery, or staged; staged may be difficult if the Cochlea is already opened during the removal of the disease. Final histology will provide the diagnosis.  I do not suggest to make a surgery for a small biopsy. It may turn out as "Granulation tissue" only.

These are my thoughts

Best regards

 

Thomas Linder, Luzern

Dear Doctor Linder,

Thank you so much for your comments.
With regard to your observations, I unintentionally didn’t mention but we did test for tuberculosis (including latent infection) and IGRA test (IFN-ɣ release assay) was negative. Also, I did not attach the file, but the patient has a profound hearing loss with no useful hearing (file with hearing test results attached).
Concerning CT Scan imaging, right side images are in file “CT scan 1” (axial right: pages 9-15) and file “CT scan 2” (coronal right: pages 12-14).

We will consider your advice and keep you informed on results.

Best regards,
Patrícia Melo

ZaniniOtavio

Tue, 10/23/2018 - 14:55

Dear colleagues,

I agree with the opinion of Prof. Linder regarding possible diagnostics and treatment. I would also consider taking a bone sample for analysis. It seems kind of dysplastic for me.

Regarding the side for first intervention, this is a difficult discussion.

On the right, it seems that the disease is less agressive and the inner ear is less compromised. 

On the left, we see a large exposure of the posterior fossa dura (probably in contact with disease/granulation tissue) and apparent superior canal ampulla erosion. 

As we don't have the MRI to analyse patency of the cochlea, I suppose that the right side may have a better hearing prognosis for a CI.

My suggestion would be starting with the right side, doing a Subtotal Petrosectomy and possibly a Cochlear Implantation in one stage.

Facial nerve may be in contact with this lesion/granulation tissue, specially in the right side. The best and safest way to find the facial nerve is at the stylomastoid foramen level. It seems to be covered by bone in this location.

Thanks for sharing this interesting case.

Best regards,

Otavio Zanini

Dear Dr. Patricia Melo,

Your case is indeed a special diagnostic challenge. I agree with the evaluations of Prof. Linder and Dr. Zanini, however, I would follow your  suggestion to obtain first tissue biopsy that could help targeting diagnosis and treatment.  I expect that with the biopsy (performed on the left ear through a retro auricular skin incision) you obtain sufficient material to confirm or exclude inflammatory, infectious or neoplastic disease of the temporal bone. The sharp lytic lesion shown in your HRCT could be due to some type of osteomyelitis, a pseudotumor or to some exotic unknown disease. The further therapeutic steps depend - as you correctly say - from the diagnosis. If the biopsy shows that surgery is needed you can consider the proposals of Thomas Linder and Otavio Zanini.
Let us know how you solve the true clinical dilemma. Thank you for the interesting case and best regards.

Ugo Fisch
 

Dear colleagues,
Prof. Linder
Dr. Zanini
Prof. Fisch

Thank you for your comments and suggestions. I truly appreciate your time and help in trying to solve this difficult case. 
I will keep you informed on the progress and outcomes.

Kind regards,
Patrícia Melo