Jugular foramen lesions and their management.pptx

A detail about anatomy and different lesions of jugular foramen

1. Jugular foramen lesions and their management Moderator- Dr.shubha verma Presentor - Dr.Falaq faiyaz .

2. Pathological conditions involving jugular foramen can be divided into 3 categories- neoplasia , infectious conditions and miscellaneous. Most commonly neoplastic conditions are seen. Classification system proposed by bertalanffy and ulrich sure is applicable to any type of lesion at level of jugular foramen. Introduction

3. Type 1 – small lesions confined to jugular foramen. Type II- intrinsic lesions of lowerbrainstem located in vicinity of jugular foramen. Type III – lesions of jugular foramen are predominantly intradural extensions located above level of foramen magnum. Type IV- lesions of jugular foramen are intradural extensions beyond level of foramen magnum into spinal canal.

4. Type V – lesions of jugular foramen are with intra and extradural extension into petrous bone. Type VI- lesions of jugular foramen are with predominant extradural extension . Lesions of jugular foramen- glomus tumor,schwannoma,meningioma,osteoclastoma,myxoma,neurofibroma,hemangiopericytoma,chondromyxoid fibroma,angioma,ceruminoma,giant cell tumor , plasmycytoma,cholestrol cyst, amyloidoma,adenoid cystic carcinoma.

5. Mc lesions in jugular foramen,schwanommas and meningiomas are 2 nd and 3 rd most frequent lesions. Glomus tumors originate from paraganglionic cells which are cells of sympathetic system responsible for secretions of catecholamines such as epinephrine,norepinephrine and dopamine. Hence there is catecholamine and sympathetic excess in glomus tumors. Glomus jugulare

6. Glomus tumors originate from jugular foramen and involve cranial nerves IX,X, or XI. Schwannomas usually develop from cranial nerves IX,X orXI . Meningiomas presumably originate intracranially and eventually invade jugular foramen. Contd …

7. Large majority of glomus tumors that originate in temoral bone invade jugular foramen. Depending on localization they can be divided into one of 3 categories. Glomus jugulare - originates from jugular,it is glomus jugulare . Glomus tumours

8. Glomus jugulotympanicum and glomus tympanicum – middle ear has promontory with plexus.tumor originating from tympanic plexus is called glomus tympanicum . Tumor developing from jugular is k/a glomus jugulare , both together are called jugulotympanicum . Glomus temporate - temporal bone has squamous,mastoid,tympanic and petrous parts.glomus temporate is general name for glomus tumors in temporal bone.

9. These glomus tumors originate from chemoreceptor jugular vein,chemoreceptor are present along major blood vessels for autoregulation and are responsible for homeostasis. Glomus jugulare tumor arises in adventitia of dome of jugular bulb. Tumour is part of neuroendocrine system and is highly vascularized , hence present with bleeding as primary manifestation.

10. Muscular layer is absent in blood vessel that feeds tumor,biopsy,FNAC and probe are contraindicated in such tumors as risk of bleeding is high. Glomulus tumors represent 0.6% of neoplasm of head and neck. Glomus jugulare tumors are typcally located just under skull base,at bulb of IJV,tumor may spread sup-jugular vein and into jugular foramen ,causing IX,X and XI deficits.

11. Primary blood supply to jugulare tumors is via ascending pharyngeal artery, occipital and post. Auricular arteries. Biopsies & FNAC are contraindicated due to high vascularity of glomus tumors. Intraoperative ligation of external carotid artery can significantly reduce haemorrhage .

12. Females are 5 times higher incidence than males. Appearance of lesion is lobular,pulsatile and red-purple in colour . Present with pulsatile tinnitus. May present with CHL ,aural bleeding and aural discharge. Clinical features

13. There may be snhl ,vertigo ,aural pain and cranial neuropathy due to tumour growth leading to involvement of cochlea 7 th ,9 th and 10 th nerves. Functioning tumors- rare,increase risk of mortality,active tumors secrete catecholamines which lead to clinical manifestations of HTN , headaches,palpitation and tachycardia.

14. Red reflex-near floor of middle ear –rising sun appearance. On inserting siegel’s pneumatic speculum into ME and increasing pressure.red tumor undergoes vigorous vibration,blanches & regains its colour on pressure reduction.- browns’s sign. Compression of carotid artery on I/L side,tumor becomes pale- Aquino’s sign.

15. Otoscopic examination reveals charateristic,pulsatile,reddish -blue tumor behind TM . PTA- mixed hearing loss , where sensorineural component tends to be more significant with larger tumors. Imaging- axial & coronal CT with thin sections demosntrates extent of bone destruction

16. CT scan shows bone destruction of post. Part of petrous apex near jugular foramen from glomus jugulare . Erosion of carotidojugular spine on CT scan is called as phelps sign. MRI with gadolinium- diethylenetriamine pentaacetic acid (DTPA) contrast is best for delineating tumor limits

17. Glomus tumors T1 and T2 weighted MRI have characteristic softtissue mixed intensity with intermixed high intensity signals and signal voids- salt and pepper appearance representing fast-flowing blood. Combination of CT scanning and contrast MRI is IOC for glomus jugulare Non-invasive investigations are preferred over carotid arteriography except for large tumors involving internal carotid preoperative carotid arteriography with cross-compression or trial balloon occlusion is recommended.

18. Preoperative balloon occlusion test is crucial when operating on glomus tumor in close proximity to artery- insight into contralateral circulation in event of damage to I/L circuit during surgery. Balloon occlusion test Balloon occlusion test involving angiography Carotid artery temporarily blocked during test EEG measurements are taken to assess C/L circulation

19. Fisch classification of temporal bone glomus tumor

20. Known as nerve sheath tumor,neuroma,neurilemoma and neurinoma . Lesion develops from schwann cells in perineurium . Pars-nervosa of jugular foramen is site of origin and cranial nerves IX,X or XI are affected. Schwannoma is usually localized in medial part of jugular foramen. Schwannoma

21. Following path of least resistance,these tumors may expand sup. Into post. Fossa cistern or inf. Into parapharyngeal space. Present cranial nerve deficits depending on nerve involved. Second common presentation is a cerebellopontine angle mass with ipsilateral sensorineural loss,tinnitus and vertigo.

22. Intracrnial meningiomas represent about 15-20 % of all brain tumors. Basal meningiomas penetrate through skull base through any of natural foramina,eg - foramen magnum , optic canal and jugular foramen. Locally aggressive meningiomas sometimes invade bone and form new openings eg - from ant. Fossa through cribriform plate into nasal cavity ,middle fossa into temporal bone meningiomas

23. Abt 1 in 5 intracranial mningiomas have such extracranial extensions. Histopathology and vascularity of these tumors varies widely. Primary jugular foramne meningiomas arise from ectopic granulation tissue or pacchionian bodies associated with lower cranial nerves

24. First invade hypotympanum intralabyrinthine air cell tracts and middle ear.simultaneous intracranial and extracranial extension into cervical spaces is common. Usually present with weak voices and dysphagia due to cranial nerve involvement.

25. Middle ear has epitympanum,mesotympanum and hypotympanum , A high jugular bulb is defined as reaching beyond level of hypotympanum beyond level of annulus. In pts with high jugular bulb,myringotomy and tympanoplasty may lead to severe haemorrhage if not recognised . Jugular diverticulum is another vascular disorder.diverticulum develops from jugular bulb and extends towards post. Scc,IAM or post. Fossa . Miscellaneous

26. Vestibular symptoms,sensorineural hearing loss or even facial nerve palsy may occur. Diagnosis of jugular foramen lesions – pts should undergo standard triad of –clinical examinations , INVESTIGATIONS- PTA,vestibular testing and electrophysiological examinations of cranial nerves. Imaging – CT AND MRI

27. May occur due to damage from contents of jugular formen IX,X,XI or venous structures of sigmoid jugular complex. Dysfunctions of cochleovestibular system or facial nerve. Clinical presentation of jugular foramen lesions is dependent on size,extent and pathology of tumor. Anterior extension encasing cavernous sinus and internal carotid artery produced horner’s syndrome and 3 rd ,4 th ,5 th and 6 th nerve palsy . SYMPTOMS

28. Intracranial extension can produce post. Fossa symptoms such as nystagmus,ataxia,hemiparesis and increased intracranial tension. Extracranial extension along internal jugular vein can produce visible mass in oropharynx or palpable mass in neck . Intraluminal growth can block venous drainage and occlude sigmoid sinus and if present b/l ,increased intracranial tension can occur.

29. Intracranial extension superiorly can produce cp angle syndrome – deafness ,tinnitus and VII nerve palsy and those extending lateraly produce bloody otorrhea Visible mass through tympanic membrane and bruit over mastoid. Levels of vanillyl mandelic acid(VMA) and metanephrine in 24 hrs urine sample indicate neurosecretory activity.

30. High resolution computed tomography and magnetic resonance imaging are methods of choice and should be acquired in all patients. T1 amd T2 weighted MRI sequences before and after administration of gadolinium can detect the most common lesions in the jugular foramen, such as shwannomas and meningiomas . However, in case of glomus tumors, besides visualization of the size and extent of tumor, adequate mapping of vascular structures is a prerequisite to accurate diagnosis.

31. This can be achieved by either MRA (MR Angiography) or conventional carotid angiography. Scientific techniques may be useful in diagnosing lesions aroundthe jugular foramen. The endocrine activity in glomus tumors can be demonstrated with Iodine-123 metaiodobenzyl guanidine. Octreotide scintigraphy may contribute to detection of glomus tumors at multiple locations.

32. 18F DOPA PET and 18 FDG glucose PET have been used to effect in detecting paragangliomas . The imaging modality is particularly useful in detecting multiple sites in those patients with either the SDHD or SDHB gene mutations.

33. Preoperative embolization should be considered in patients with lesions surrounding the jugular foramen. Glomus tumors are the most common lesions found in the jugular foramen. If surgical removal is indicated, some tumors should be previously embolized . Selective arteriography is performed 24-48 hours prior to surgery. Preoperative embolization

34. During the procedure, the tumor is embolized via branches of external carotid artery. Care is taken to embolize only those branches of external carotid artery that are feeders to the tumors, to avoid neurological problems caused by the reflex of embolic material into the intracranial circulation. There is general agreement that preoperative embolization significantly reduces blood loss during surgery and this facilitates the complete removal of tumors.

35. Intraoperative haemorrhage is also main cause of problems during surgical removal of meningiomas , excessive bleeding can lead to incomplete tumor resection . Glomus tumors and meningiomas should be investigated conventional angiography and embolization should be undertaken where indicated.

36. Transtemporal surgical approach – wide retro auricular incision made to gain access to lateral aspect of temporal bone . Incision extended downwards into neck to expose structures in infratemporal region. Wide cortical attico-mastoidectomy with skeletonization of sigmoid sinus. Mastoid segment of fallopian canal is exposed and skeletonized from second genu towards stylomastoid foramen. Surgical approaches

37. Bone ant. To this part of facial nerve is removed and dome of jugular bulb is exposed. Diamond burr is used to remove as much bone as possible that covers sigmoid sinus and jugular bulb so fallopian bridge is only structure that overlies jugular bulb. Facial nerve may impair adequate exposure of lesions involving jugular foramen , but degree of access required also depends on pathology

38. Vertical segment of facial nerve has to be transposed ,particularly if lesions are extensive and bulky. Performing a subtotal petrosectomy to gain wide access to facial nerve.

39. Anatomical relationship between mastoid segment of facial nerve and jugular bulb varies . In pts with small tumor and sufficient space b/w vertical segment of nerve and jugular bulb it is possible to leave facial nerve undisturbed. Facial nerve transposition in ear canal wall up –complete removal of bony covering of mastoid segment of facial nerve from second genu to stylomastoid foramen Conservative facial nerve management.

40. Outside stylomastoid foramne ,nerve is followed and mobilized over its intraparotid course – 1 st main trunk an dthen temporal zygomatic and cervicofacial branches. Subdivisions of these branches are also mobilized and overlying parotid tissue is removed. There is some mobility of nerve such that it can move ant. To jugular bulb

41. Atticomastoidectomy is extended to subtotal petrosectomy which involves removal of skin of EAC,along with TM,malleus and incus drilling away post. Canal wall and extensive exenteration of pneumatic cells. Facial nerve can be uncovered not only in its mastoid segment but also in its tympanic part. Nerve can be rerouted ant. After mobilization of intraparotid facial nerve Facial nerve tranposition in ear canal wall down

42. Hinge point is located at 2 nd genu , but wider exposure is achieved when nerve is displaced ant. With hinge point at 1 st genu . Management of sigmoid-jugular complex- damage to sigmoid sinus or jugular bulb is serious risk during jugular foramen surgery. If vessel wall is puncturedinitial bleeding is fairly simple to control but retrograde haemorrhage from IJV may follow

43. Venous blood flow must be blocked at 2 levels cranially and caudally to lesion Blocking blood flow can be done by ligature and packaging with surgicel . Upper neck is explored by extending retro auricular incision downwards.

44. IJV can be found in later stage of after SCM has been detached from mastoid process and tip. Identify surrounding structures ,such as internal carotid artery & IX,X,XI and XII,IJVshould be ligated high up to avoid bleeding in upper neck . Inf. And sup. Petrosal sinus and mastoid and condylar emissary veins drain into jugular complex

45. Main blood supply of entire cranial circulation If tumor is attached to ICA or in close contact with it ,hence visualisation b/w tumor and petrous carotid artery is extremely important. Glomus tumors invade vertical part and eventually along horizontal part of foramen lacerum . Management of ICA

46. It may also receive blood supply from petrous carotid artery eg - via carotid caroticotympanic or dural branches. Very large tumors,permanent balloon occlusion of ICA if there is sufficient collateral circulation . Collateral circulation can be tested pre-operatively during angiographic procedure by electroencephalographic monitoring

47. Pts may not have sufficient collateral circulation to allow permanent balloon occlusion- extracranial or intracranial bypass procedure is considered for which cross clamping of major intracranial arteries is done. An extracranial -intracranial bypass is considerably safer bacause it precludes ligation of intracranial vessel 1 st venous graft or transplant is connected to bifurcation of carotid artery in neck connected to post. Cerebral artery.

48. Glomus tumors usually arise from lateral part of jugular foramen and schwanomma tend to be located medially Dumb-bell shaped tumors are fairly common because they extend intracranially as well into infratemporal fossa .

49. Cardiovascular stablity must be monitored and safeguarded. Facial nerve palsy ,hearing loss , vertigo , dysphagia and hoarseness are mc symptoms. Functional deficits of cranial nerve XI & XII are less burdening Post-operative management and rehabilitation

50. Advanced age /poor physical condition are c/I for surgery. Inoperable malignant tumors can be treated with chemotherapy,radiotherapy or both. Stereotactic radiosurgery might be considered for benign lesions that are inoperable due to larger size,age or physical condition of pt. Non surgical management

51. Thank you