الفهرس 
TYMPANIC PARAGANGLIOMA
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Abstract
Temporal bone paragangliomas are the most frequently encountered temporal bone neoplasm after the acoustic neuroma. They are divided into two broad categories on the basis of their site of origin: tympanic and jugular. Tympanic paraganglioma is the commonest ME neoplasm.
The most frequent manifestations of tympanic paragangliomas are pulsatile tinnitus and conductive hearing loss. The tumor may also be an incidental finding on routine physical examination. Erosion of the TM with the appearance of a pulsating polyp in the EAC is another finding in some patients.
Diagnosis is suspected clinically based on the findings on physical examination. Otoscopic examination of a tympanic paragangliomas frequently demonstrates a reddish-blue pulsatile mass behind the TM, with blanching of the mass on positive pressure during pneumatic otoscopy (Brown sign). Ipsilateral carotid compression can diminish the pulsatile nature of the tumor (positive Aquino sign). Auscultation over the mastoid or infra-auricular area may reveal an audible bruit. Hemorrhagic polyp can occur if the tumor extends through the TM. Lower cranial nerve palsies are commonly present with tumors involving the jugular foramen than those of the middle cleft.
Characteristic radiological features may aid in confirming the diagnosis and define the extension of the lesion. Diagnosis of tympanic paragangliomas is based mainly on CT finding of the presence of ME mass with intact bony plate over the jugular bulb. MRI could be useful on the occasion of the presence of mastoid opacification to define the posterior extension of the tumor and exclude mastoid involvement. The classic MRI finding of “salt-and pepper appearance” is not frequently seen in tympanic paraganglioma. Angiography is not required in tympanic paraganglioma.
Different classifications based on the extension of the tumor both clinically and radiologically have been described. The most popularly used classification systems are the Fisch and Mattox and Glasscock-Jackson classifications, however the modified Fisch classification provide detailed description of the tumor class based on different compartment involvement.
Treatment modalities include surgical excision, radiotherapy (FRT or SRT), wait and scan policy and angioembolization without removal in selected cases. Surgical excision remains the primary treatment modality. Small tumors isolated to the promontory can be successfully removed via the transcanal or hypotympanotomy approach. More advanced lesions of the middle ear and mastoid can be exposed with an extended facial recess approach through the mastoid. Different tools like laser or piezosurgery can assist tumor excision.
Endoscopic ear surgery is an evolving technique for managing ear conditions with many proved advantages over the microscope, including wider angle of view, better visualization of structures that are parallel to the axis of the microscope, visualization of deep recesses and hidden structures (i.e., around corners), and the ability to visualize beyond the shaft of the surgical instrument. On the other hand, the disadvantages of endoscopes include loss of depth of perception and binocular vision, the inevitable one-handed surgical technique necessitated, the need for a strictly bloodless field, fogging and smearing of the tip of the endoscope, the mandatory requirement for reliable physician training, and the cost of the equipment.
Our study aimed to assess the possibility of performing transcanal endoscopic excision of tympanic paraganglioma avoiding the need for external incisions and unnecessary bony work.
Eleven patients suffering from tympanic paragangliomas operated at the otorhinolaryngology department of Alexandria university hospitals between January 2013 and December 2018 were included in our study. Patients were subjected to preoperative clinical, audiological and radiological assessment.
All the cases in our series were females with a mean age at presentation of 52.27 years old. The lesions were left sided in 63.64% and right sided in 36.36%. The commonest presenting symptom was pulsatile tinnitus (90.91%) while in 9.09% aural fullness was the presenting symptom. Duration of symptoms ranged from 6 to 74 months. Symptoms in our series were; tinnitus (100%), hearing loss (81.82%), aural fullness (27.27%), otalgia (27.27%) and otorrhea (9.09%). No family history nor systemic symptoms were seen in any of our patients. Pulsatile mass behind the TM was seen in 90.91% while a pulsating aural polyp was seen in 9.09%. CES could be assessed in 10 patients and was CES 0 (90%) and CES 1p (10%).
In 90.91% the tumor was class B1 according to the modified Fisch classification while it was B3 in 9.09%. Class 3 EES according to Massachusetts Eye and Ear Infirmary Endoscopic Ear Surgery Classification System was performed in all patients with an operative duration ranging between 100 and 246 minutes. The involved ME compartment were mesotympanum (100%), hypotympanum (90.91%), retrotympanum (90.91%), protympanum (36.36%), epitympanum (27.27%) and EAC (9.09%). In 54.55% 3 compartments were involved, 4 compartments in 18.18%, two compartments in 9.09% and all the ME with EAC in 9.09%. Bony work was performed in 54.55% and not required in 45.45%. The ossicular chain could be preserved in all except one case (9.09%). Primary grafting of the TM was performed in 63.64% materials used were tragal cartilage/perichondrium composite graft (71.43%), temporalis fascia (14.29%) and tragal perichondrium in one case (14.29%).
Regarding the complications in our series postoperative temporary facial nerve paresis was seen in 2 cases where none of the cases showed persistent palsy, TM perforation in 3 cases and recurrence in 1 case.
The procedure showed to us obvious view of the tumor and middle ear around, comparable overall tumor control and less postoperative time. The relatively long time will sure be less with more training and getting support from surgical tools like Laser and Piezosurgery.