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Clival Pathology.ppt
- 1. BY Dr. Mahmoud Saad ClivalPathology
- 2. Congenital: 1-Proatlas segmentation failure 2-Neuroentericcysts Acquired: 1-Basilar Invagination 2-Basilar Impression 3-Rickets Paget's disease 4-Osteogenesis imperfecta 5- Clival fractures 1ry Neoplastic: 1-Eosinophilic granuloma 2-Fibrous dysplasia 3-Chordoma 4-Chondroma 5-Chondrosarcoma 6-Plasmocytoma 2ry Neoplastic: 1-Metastatic 2-Nasopharyngeal malignancy 3-Ectopic pituitary adenoma 4-Craniopharyngioma Clival Pathology
- 3. Extra-Intra Dural: 1-Meningioma 2-Neurofibroma 3-Chordoma 4-Glomus tumour 5-Rhabdomyosarcoma 2ryNeoplastic: 1-Metastatic 2-Nasopharyngeal malignancy 3-Ectopic pituitary adenoma 4-Craniopharyngioma
- 4. • Malformations ofthe most caudal of the occipital sclerotomes • Surround the foramen magnum involve the posterior arches of C1. • A hindbrain herniation is associated in 33%. • The Proatlas component of the dens fail to separate from that portion that forms the basiocciput of the clivus the anterior arch of the atlas comes to rest above the axis body. • The proatlas abnormality is united with the clivus, distorting cervicomedullary junction ventrally. Pro Atlas Segmentation Failures
- 5. Treatment: • Relief ofneurovascular compression. • Prevention of recurrence by stabilization.
- 6. • (AKA enterogenouscysts, endodermal cysts, archenteric cysts, gastrocystomas, intestinomas, cystic teratomas, foregut cysts). • 0.7% of tumors and 16% of cysts in the CNS. • During normal development, the neuroenteric canal closes and the notochord separates from the primitive gut. • Transient adhesion occurs between the neural ectoderm and endoderm. • When it persists because of the incomplete separation at this adherence or remnant canal, the cyst forms. • Intracranial neurenteric cysts are typically intradural, extra-axial posterior fossa masses. Neuroenteric cyst
- 7. Enteric cyst (H&E):Cyst lined by simple columnar and cuboidal epithelium.
- 8. D.D: • A Rathkecleft cyst is usually sellar or suprasellar • Colloid cyst the anterior wall of the third ventricle adjacent to foramen of Monro. Treatment: • Surgical excision. • Simple aspiration. • Cyst wall marsupialization. • Cysto-subarchnoid shunt may be employed when total removal is not possible. • No role for radiotherapy or chemotherapy.
- 9. Basilar Invagination: • Rarecondition. Etiology: • Anomalies of occiput, atlas, axis. • Softened bone: rickets / osteomalacia, Paget's • Fibrous dysplasia • Dysostoses: OI, achondroplasia, cleidocranial dysostosis Occurs when the top of the second vertebrae moves upward. It can cause the foramen magnum to close. It also may press on the lower brainstem.
- 10. Symptoms: • If thereis pressure on the lower brainstem, its first symptom may be death. – Periods of confusion – Difficulty swallowing or (dysarthria) – Dizziness – Loss of sensation – Pain in the back of the head – Loss of the ability to know how joints are positioned
- 11. Complications: • Hydrocephalus orsyringomyelia because it blocks the flow of fluid around the brain and spinal cord. Diagnosis: • X-ray • MRI provides the most information. • CT Scan. Treatment: Conservative approach: • Drugs: aspirin, without steroids to relieve inflammation (tenderness and swelling) • Neck traction (neck collar) thus relieving pressure on the spinal cord. If there is pressure on the spinal cord, surgery is recommended.
- 12. • A raremechanical deformity of the CVJ • Migration of the upper cervical spine and the odontoid peg into the base of the skull. • Brain stem and cerebellum compression. • Disturbance of cerebrospinal fluid circulation. • Mechanical stretching of cranial nerves. • Progressive lead to rapid neurological deterioration respiratory arrest or even sudden death. • Basilar impression is sometimes referred to as basilar invagination, and these terms are used interchangeably Basilar impression
- 13. • Genetic disorder:mutation on a gene that affects the body’s production of the collagen found in bones, and other tissues. • Fragile bones that break easily “brittle bone disease. • OI: muscle weakness, hearing loss, fatigue, joint laxity, curved bones, scoliosis, blue sclerae, dentinogenesis imperfecta (brittle teeth), and short stature. Osteogenesis Imperfecta (OI)
- 14. Treatment: • Physical therapy •Casts, splints • Orthopedic surgery implanting rods • Medications to strengthen bones • Mobility aids walkers, or wheelchairs • Medications • Bisphosphonates: ©Fosamax ©Reclast ©Forteo for adults only • Growth Hormone • Increased vitamin D intake • Physical activity • Potential for gene therapy • At this time, there is no cure.
- 15. • 3 categories •Longitudinal injuries injuries to vertebrobasilar vessels. • Dissection or occlusion: brain stem infarction. • Traumatic aneurysms. • Transverse injuries to anterior circulation. • Oblique. • 1-CN deficits: III, VI. • 2- CSF leak. • 3- Diabetes Inspidus. Clival Fractures Clival Fractures are Lethal
- 16. • 3 clinicalvariants of Langerhans cell histiocytosis 1- Letterer-Siwe disease 2-Hand-Schüller-Christian disease 3- EG (formerly termed histiocytosis X). • EG single or multiple skeletal lesions • Solitary lesions are more common than multiple lesions. • Sites: skull, mandible, spine, ribs, and long bones. • Symptoms: localized pain, tenderness, swelling, fever, and leukocytosis. Eosinophilic Granuloma
- 17. Pathology: • Proliferation offoamy and vacuolated histiocytes is associated with a variable admixture of neutrophils, eosinophils, lymphocytes, and plasma cells. • Spontaneous resolution by fibrosis occurring within 1-2 years. • Curettage, excision, or local irradiation leads to cure
- 18. • Chronic disorderin which bone expands due to abnormal development of fibrous tissue • Prognosis of fibrous dysplasia is generally good • Malignant degeneration and aggressive behaviour. • The incidence of malignant transformation is highest for monostotic craniofacial lesion There are two types: • Monostotic FD • Polyostotic FD • McCune-Albright syndrome: Triad 1-Polyostotic FD 2-Skin hyperpigmentation 3-Endocrine dysfunction (hyperthyroid, precocious puberty, Cushing syndrome, acromegaly etc). Fibrous Dysplasia
- 19. Clinical: • Asymptomatic • Headachein severe cases. • Cranial nerve involvement. Diagnosis: • X-ray • 3 patterns of FD: Cyst-like, Sclerotic and Mixed “Pagetoid”. Treatment: 1-Mainly conservative 2-Surgical treatment: – Cranial nerve compromise – Severe headache – Development of a malignancy .
- 20. • Solitary massof neoplastic monoclonal plasma cells in either bone or soft tissue (extramedullary). Types: • Soft-tissue or nonosseous extramedullary plasmacytoma (EMP) • Solitary bone plasmacytoma (SBP) • Multifocal form of multiple myeloma. • Multiple myeloma • Plasmablastic sarcoma Solitary plasmacytomas: • Plasmacytoma of the skeletal system (SBP) • Extramedullary plasmacytoma (EMP) Plasmacytoma
- 21. • 0.2% ofall intracranial tumors. • 1ry malignant tumour of spine 55% and clivus 35% • Benign tumors; critical location, local invasion, recurrence, occasional metastatic spread. • Characteristic physaliphorous cells (intracellular mucin). • Slowly growing & radioresitant • Treatment of choice: wide en bloc resection, proton beam radiation • Tumors originating from embryonic remnants of the primitive notochord (differentiate to the nucleus pulposus of IVD). • Chordomas lie in bone: extradural and induce bone destruction. Chordomas in adults and rarely in younger than 30 years. Chordoma of the Clivus
- 22. Presentation: • Compression andinvasion of important neighboring structures. • Headache and cranial nerve deficits (CN VI abducens). • Dysphagia, facial pain, facial paresis, visual loss, hearing loss, and ataxia. Examination: • MRI • CT scanning (assess the degree of bone involvement or destruction)
- 24. Craniopharyngioma • 1-3% ofintracranial tumors and 13% of suprasellar tumors. • In children, 5-10% of all tumors and 56% of sellar and suprasellar tumors. • Slow-growing, extra-axial, epithelial-squamous, calcified cystic tumour • Proteinaceous material brownish-yellow color (high content of floating cholesterol crystals) machinery oil fluid. • Arising from remnants of the craniopharyngeal duct and/or Rathke cleft • Occupying the (supra) sellar region • It arises in the pituitary stalk and projects into the hypothalamus. • Rare locations: nasopharyngeal. Pure posterior fossa extending down the cervical spine.
- 25. 1- Embryogenetic theory •Development of the adenohypophysis and transformation of the remnant ectoblastic cells of the craniopharyngeal duct and the involuted Rathke pouch. 2-Metaplastic theory • Metaplasia residual squamous epithelium (derived from stomodeum) 3-Dual theory • Adamantinous type (childhood) to embryonic remnants • Adult type (squamous papillary) to metaplastic foci derived from mature cells of the anterior hypophysis 3 Theory
- 26. Clinical: • Headache (55-86%). •Endocrine dysfunction (66-90%). • Visual disturbances (37-68%). • Three clinical syndromes. • Prechiasmal: optic atrophy. • Retrochiasmal: hydrocephalus with signs of ICT. • Intrasellar: headache and endocrinopathy.
- 27. • Uncommon benignneoplasms • Modified smooth muscle cells called glomus cells. • Two variants exist: – Solitary glomus tumors – Multiple glomus tumors, known as glomangiomas or glomulovenous malformations. Glomus Tumors
- 28. Pathophysiology Glomus tumors arisefrom: • Arterial portion of the glomus body. • Sucquet-Hoyer canal, AV shunt in the dermis that contributes to temperature regulation. Causes: • Proliferation of glomus cells (portion of the glomus body). • Multiple glomus tumors, autosomal dominant pattern.
- 29. • Benign tumorsof peripheral nerves. • Arise from Schwann cells, fibroblasts, and perineural cells. • Most commonly is the vestibulocochlear nerve. • Neurofibromas can be single or multiple. • When multiple, associated with NF type I a genetic disorder AKA Von Recklinghausen disease. Symptoms: • Painless, slow-growing mass. • Tinel sign: an electric-like shock when light pressure is applied to the tumor. • Affection of motor or sensory nerve function. Neurofibromas
- 30. Diagnosis: • Characteristic appearanceon MRI. • A biopsy of the tumor enables positive diagnosis. Treatment: • Surgery is the treatment of choice • Neurofibromas of the skull base expanded endonasal approach (EEA).
- 31. • Posterior fossameningioma 10% of total intracranial meningioma • 50% involve the petroclival region. • Skull base meningioma has a predilection for female sex. • Petroclival lesions usually present in 5th or 6th decade. • Meningioma the commonest lesion in the petroclival region followed by chordoma. • Chondrosarcoma and neurinoams are rare lesions of the petroclival region. Clival Meningioma
- 32. Castellano and Ruggieroclassify posterior fossa meningiomas (site of dural implant) 1- Cerebellar convexity. 2- Tentorium. 3- Posterior surface of the petrous bone. 4- Clivus. 5- Foramen magnum.
- 33. Desgeorges et al.divided the posterior surface of petrous bone meningiomas according to the exact site of implant in relation to the internal auditory canal (IAC) and labyrinth into: 1-Anterior group 2- Median group (centered on the IAC), 3- Posterior group.
- 35. Sekhar et al.had classified clival meningiomas based on volume and tumor size into medium, large and giant subtypes. Giant tumors were those having maximum diameter more than 4.5 cm.
- 37. Yasargil et al.classify basal posterior fossa meningiomas into five subgroups: 1. Clival. 2. Petroclival. 3. Sphenopetroclival. 4. Foramen magnum. 5. CPA meningiomas.
- 38. • One surgicalgroup, which we call "petroclival Meningiomas 1-The clival (medial origin). 2-Petroclival (origin at the petrous tip medial to the V nerve). 3-Sphenopetroclival (origin at Meckel's Cave) meningiomas.
- 40. Investigations: • Computerized tomography(CT) scans • Magnetic resonance imaging (MRI) with contrast enhancement. • Cerebral angiography visualization of carotid and vertebral circulation. • MR angiography and venography.
- 42. • The angiographicrelationship of the basilar artery: 1-The basilar artery is usually pushed posteriorly and to one side. 2-The posterior and superior cerebellar arteries usually are frequently elevated on the same side of the tumor. • The venous phase of the angiogram demonstrating: 1-Presence and contribution of two transverse sigmoid sinuses. 2-Delineating the course and dominance of the vein of Labbé.
- 44. • Most commonsoft tissue sarcoma in children. • Greek words rhabdo = rod shape, myo = muscle. • Tumor arise from a primitive muscle cells rhabdomyoblast. • Histologic groups. • Embryonal rhabdomyosarcoma (ERMS). • Alveolar rhabdomyosarcoma (ARMS). • Undifferentiated sarcoma (UDS). Rhabdomyosarcoma
- 45. Clinical: • Expanding mass. •Pain. • Metastatic disease presentation.
- 46.