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29 Cerebrospinal Fluid Rhinorrhea
335
12. Eftekhar B, Ghodsi M, Nejat F, Ketabchi E, Esmaeeli B. Prophylactic administration of ceftriaxone for the prevention of meningitis after traumatic pneu­mocephalus: results of a clinical trial. J Neurosurg. 2004;101:757–61.
13. Eljamel MS, Foy PM. Acute traumatic CSF stu­lae: he risk of intracranial infection. Br J Neurosurg. 1990;4:381–5.
14. Ohnishi T. Bony defects and dehiscences of the roof of the ethmoid cells. Rhinology. 1981;19(4): 195–202.
15. Badia L, Loughran S, Lund V. Primary spontane­ous cerebrospinal uid rhinorrhea and obesity. Am J Rhinol. 2001;15:117–9.
16. El-Sayed IH, Roediger FC, Goldberg AN, et al. Endoscopic reconstruction of skull base defects with the nasal septal ap. Skull Base. 2008;18(6):385–94.
17. Normansell DE, Stacy EK, Booker CF, etal. Detection of beta-2 transferrin in otorrhea and rhinorrhea in a routine clinical laboratory setting. Clin Diagn Lab Immunol. 1994;1:68–70.
18. Yilmazlar S, Arslan E, Kocaeli H, etal. Cerebrospinal uid leakage complicating skull base fractures: analy­sis of 81 cases. Neurosurg Rev. 2006;29:64–71.
19. Bell RB, Dierks EJ, Homer L, et al. Management of cerebrospinal uid leak associated with cra­niomaxillofacial trauma. J Oral Maxillofac Surg. 2004;62(6):676–84.
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21. Dandy WE. Pneumocephalus (intracranial pneumo­cele or aerocele). Arch Surg. 1926;12:949–82.
Anterior andMidline Central Skull
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Base Tumors
SaraAshkanani andAbhishekMenon
30
Key Points
• Mainstay of treatment for sinonasal squamous cell CA is surgical resection. Radiotherapy may be used as adjuvant therapy, except in nonresectable tumors, where they are used as mainstay.
• Perineural invasion is more common in adenocarcinoma.
• Clinical presentation and examination of olfactory neuroblastoma can mimic inammatory polyps.
• Pituitary adenomas are usually seen in the third decade of life. MRI with gado­linium enhancement is the single best radiological test for midline central skul base tumor diagnosis. Treatment is usu­ally surgical excision.
• Chordomas and chondrosarcomas are derived from embryonic remnants of the notochord.
S. Ashkanani (*) Otolaryngology-Head and Neck Surgery Division, Department of Surgery, Hamad Medical Corporation, Doha, Qatar
Department of Otolaryngology-Head and Neck Surgery Division, Weill Cornell Medicine-Qatar, Doha, Qatar e-mail: sashkanani@hamad.qa
30.1 Introduction
The skull base refers to the most inferior part of the neurocranium, on which the brain rests, and it is the major bony and soft tissue interface for the intracranial and extracranial compartments. Its anatomy is complex, containing multiple tra­versing foramina that act as conduits for various neurovascular structures. They are divided into anterior, central, and posterior parts (Fig.30.1). In this chapter, we will be dealing with the ante­rior skull base and midline aspect of the central skull base and its pathologies. The various fora­men and the structures passing through them are mentioned in Tables 30.1, 30.2, and 30.3.
30.2 Anatomy
The anterior skull base proper is formed medially by the cribriform plate, making up the roof of the nasal cavity, laterally by the orbital plates of the frontal bone which form the roof of the orbits and ethtmoid air cells and, posteriorly, by the planum sphenoidale and lesser wings of the sphenoid.
A. Menon Otolaryngology-Head and Neck Surgery Division, Department of Surgery, Hamad Medical Corporation, Doha, Qatar e-mail: AMenon1@hamad.qa
© Springer Nature Switzerland AG 2021 A. Al-Qahtani et al. (eds.), Textbook of Clinical Otolaryngology,
https://doi.org/10.1007/978-3-030-54088-3_30
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S. Ashkanani and A. Menon
Fig. 30.1 Skull base showing the different parts and ssures within them
Table 30.1 The foramina of the anterior skull base and
their traversing neurovascular structures
Foramen Nerve Vessel Foramen
caecum Cribriform plate Optic canal Optic nerve Ophthalmic artery
Olfactory nerve
Emissary vein to superior sagittal sinus
Table 30.2 The foramina of the central skull base and
their traversing neurovascular structures
Foramen Nerve Vessel Superior orbital
ssure
Foramen rotundum Foramen ovale Mandibular nerve
The central skull base comprises the body and greater wing of sphenoid bone and squamous temporal bones, and is demarcated from the pos­terior skull base by the petrous temporal bone. They are divided into:
Midline Central Skull base Compartment:
Foramen spinosum
Vidian (pterygoid) canal Meckel’s canal Trigeminal nerve
Includes the pituitary (sella), sphenoid body, and
clivus, and abuts the extracranial nasopharynx.
Paramidline Central Skull Base Compartment:
Includes a cluster of neurovascular foramina,
the cavernous sinuses, and Meckel’s caves.
lopontine angle, internal auditory meatus, petro­mastoid, jugular foramen, and hypoglossal canal.
The dura is particularly closely attached to the
anterior skull base and so is prone to injury fol-
This posterior skull base compartment includes the anatomical structures of the cerebel-
lowing a skull base fracture, potentially with pneumocephalus or cerebrospinal uid (CSF)
CN III, IV, V
Maxillary nerve (CN V2)
(CN V3) Meningeal branch of mandibular nerve Vidian nerve Vidian artery/vein
ganglion
, VI Superior and
1
inferior ophthalmic vein
Accessory meningeal artery Middle meningeal artery
30 Anterior andMidline Central Skull Base Tumors
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Table 30.3 The foramina of the posterior skull base and
their traversing neurovascular structures
Foramen Nerve Vessel Facial nerve
canal Internal auditory meatus Petrous carotid canal
Foramen lacerum
Jugular foramen
Foramen magnum
Hypoglossal canal
Facial nerve (CN VII) Facial (CN VII) and vestibulocochlear (CN VIII) nerves
Vidian nerve leaves its anterior aspect
Glossopharyngeal (CN IX) Vagus (CN X) Spinal accessory nerve (CN XI) Medulla oblongata Vertebral artery
Hypoglossal nerve
Labyrinthine artery
Petrous segment of the internal carotid artery Artery of pterygoid canal Emissary veins (carotid artery is immediately superior) Inferior petrosal sinus Jugular bulb
Anterior and posterior spinal arteries
rhinorrhoea. The anterior skull base also has a rich plexus of valveless venous perforations (e.g., the vein of Breschet) and so paranasal sinus infection can spread either into the frontal bones as osteomyelitis or traverse the anterior skull base giving intracranial sepsis.
MRI can clearly delineate the olfactory bulbs, which may have reduced volume in some neuro­degenerative processes. The foramen caecum is generally blind ended or transmits an emissary vein in adulthood, but is also the site of a dural diverticulum in early life. If this diverticulum persists in the context of midline nasal develop­mental anomalies such as dermoid cysts, then it may predispose to ascending meningitis.
30.3 Tumors oftheAnterior
SkullBase
Pathologic processes affecting the anterior skull base may arise from the bones of the skull base proper, intracranially from the meningeal cover-
ings, or exocranially from the sinonasal mucosa investing the nasal cavity, ethmoid and frontal sinuses, from the olfactory mucosa covering the roof of the nasal cavity, from the orbit, from cra­nial nerves and vessels, from the chondro-
vomerian
synchondrosis, or from embryonic remnants.
30.3.1 Sinonasal Neoplasms
Most sinonasal neoplasms are epithelial in origin and include, in decreasing order of frequency, squamous cell carcinoma, intestinal type adeno­carcinoma, and minor salivary gland neoplasms. The remainder comprise neoplasms arising from the olfactory mucosa, (esthesioneuroblastomas), nasal lymphoma, melanoma, and a variety of mes­enchymal tumors including rhabdomyossarcoma, the most common in the pediatric age group.
30.3.1.1 Squamous Cell Carcinoma
• Most common sinonasal tumor.
• Constitute 70% of all malignancies.
• Presents with nasal congestion, blockage, fre­quent epistaxis, headache.
• Imaging features are non-specic and include intermediate signal intensity on T2W images reecting high cellularity and aggressive bone destruction occasionally with small bony frag­ments engulfed within the lesion [1, 2].
• Mainstay of treatment is surgical resection.
• Radiotherapy may be used as adjuvant ther­apy, except in nonresectable tumors, where they are used as mainstay.
30.3.1.2 Adenocarcinoma
• Comprises 10% of all sinonasal neoplasms.
• Perineural invasion more common.
• Three types:
– Minor salivary gland tumors – Intestinal type adenocarcinoma – Neuroendocrine neoplasms
• Distinctive imaging features include T2W prolongation [35] and, in low-grade tumors, a less aggressive pattern of bone involvement, usually with remodelling and thinning rather than permeative destruction of surrounding bone (Fig.30.2).
• Treatment is surgical resection.
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S. Ashkanani and A. Menon
a
b
c
Fig. 30.2 (a) Coronal CT sinus showing adenocarci-
noma extending from the roof of the left ethmoid sinus distally and medially through the olfactory recess eroding the nasal septum crossing into the contralateral side. (b) Coronal T1W MRI sinus showing intermediate signal intensity lesion mildly expanding predominantly left nasal roof and olfactory recess mass lesion inseparable from medial lamella of left middle turbinate, and inltrat­ing the nasal septum crossing to and expanding contralat-
eral olfactory recess, with remodelling of cribriform plate with no denite intracranial extension. (c) Coronal T2W MRI sinus showing inhomogeneous mixed signal inten­sity lesion mildly expanding predominantly left nasal roof and olfactory recess mass lesion inseparable from medial lamella of left middle turbinate, and inltrating the nasal septum crossing to and expanding contralateral olfactory recess, with remodelling of cribriform plate with no de­nite intracranial extension
ab
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30.3.1.3 Olfactory Neuroblastoma
• Also known as esthesioneuroblastoma.
• It is a rare tumor of neural crest origin which arises from the olfactory mucosa lining the nasal vault and cribiform plate and therefore is a site specic neoplasm.
• Clinical presentation and examination mimics inammatory polyps.
• Intracranial extension through cribriform plate and orbital invasion through lamina pap­yracea are common.
• Meningeal spread and parenchymal invasion of the olfactory bulbs and inferior frontal lobes are often seen in advanced tumors.
• Typical imaging presentation is that of a soli­tary, polypoid, expansile soft tissue mass cen­tered in the nasal vault and cribiform plate.
• This radiographic grading scale is as follows:
– A—limited to nasal cavity. – B—nasal and paranasal cavity. – C—beyond nasal and paranasal cavity
(invades cribriform plate, anterior cranial base or orbit, intracranial, and maxillary sinus).
• They tend to be slightly hyperdense on CT and of intermediate signal intensity on both T1W and T2W MR images (Fig.30.3) [6, 7].
• Treatment is surgical enbloc resection.
30.3.1.4 Others
• Less common tumors include: – Malignant melanoma – B Cell lymphoma – T Cell lymphoma
30.3.2 Orbital Tumors
• Although less commonly, a variety of neo-
plasms arising from the orbital contents may affect the anterior skull base from below. These include lacrimal gland neoplasms, neu­rogenic tumors, lymphoma, rhadomyosar­coma, chloroma, and metastasis to name the most common.
• Benign lacrimal gland tumors manifest as
mass lesions originating in the superior and outer aspect of the orbit, the normal anatomic location of the lacrimal gland. When large enough, they displace the globe and extra­ocular muscles inferiorly and medially and remodel and erode the orbital roof.
Fig. 30.3 (a) T1W MRI showing low T1 lesion with small bright patches in a post-op patient. (b) T2W MRI showing
mixed signal, heterogenously enhancong lesion in a post-op patient
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S. Ashkanani and A. Menon
• Malignant neoplasms arising in the orbital compartment show aggressive features and may extend posteriorly through the orbital apex and orbital ssures into the medial cra­nial fossa.
30.4 Tumors Arising fromAbove
theAnterior Skull Base
Besides meningiomas and their malignant coun­terparts, the skull base is rarely affected by other tumors arising from the intracranial compart­ment. Only rarely do primary CNS tumors grow outside the brain and when this occurs it is often along the path of a surgical defect.
30.4.1 Olfactory Groove Meningioma
• Most common intracranial lesion affecting the anterior skull base from above.
• Does not cause symptoms until tumor has grown to large size.
• Symptoms include anosmia, blurred vision, loss of memory, headache, nausea, vomiting, and personality changes.
• They are best characterized with magnetic resonance imaging.
• They are usually isointense to brain on both T1W and T2W images, well-circumscribed and show intense and homogeneous gadolin­ium enhancement.
• Treatment is usually surgical resection.
30.5 Pseudotumors
Many infectious inammatory conditions such as mucoceles, sinonasal polyposis, abscesses, fun­gal infection, osteomyelitis and several granulo­matous diseases including Wegener’s can affect the skull base and even grow intracranially mim­icking tumoral lesions. Correlation with clinical history and laboratory ndings is mandatory to reach the diagnosis. Imaging features that might suggest inammatory pseudotumor include the presence of MR signal changes, particularly on long TR images, in excess to the amount of soft tissue mass seen on CT and the presence of vessel occlusion. Whereas neoplasms invading the cav­ernous sinus tend to narrow or compress the internal carotid artery, rarely leading to a com­plete occlusion, infection and inammation reaching the skull base may rapidly extend to the petrous or cavernous segments of the carotid artery, leading to inammation of the vessel wall (endarteritis) and occlusion.
30.6 Tumors oftheMidline Central Skull Base
Midline central skull base tumors may arise from the sphenoid sinus and clivus, sella or the extra­cranial tissues (nasopharynx). Most (>90%) sel­lar lesions are pituitary in origin, and the remaining can be meningiomas and craniopharyngiomas.
30.4.2 Subfrontal Schwannomas
• Rare neoplasms of unclear origin, thought to arise from the olfactory nerve or from a men­ingeal branch of cranial nerve V.
• Benign slow-growing lesions which tend to remodel adjacent bone and may be associated to bone sclerosis and intratumoral calcications.
• Often misdiagnosed for olfactory groove men­ingomas or olfactory neuroblastomas due to their particular location imaging features.
• Treatment is surgical excision.
30.6.1 Pituitary Adenomas
• They are the most common causes of midline central skull base tumors [8].
• Pituitary adenomas are benign tumors of the adenohypophysis.
• Usually seen in the third decade of life.
• MEN1, Gs-alpha and AIP mutations have been shown to play a role in the development of the tumor.
• Types based on size:
Microadenoma: less than 1cm – Macroadenoma: more than 1cm
30 Anterior andMidline Central Skull Base Tumors
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• Types based on cell type: – Gonadotroph adenomas: Clinically
nonfuctioning
Thyrotroph adenomas: Clinically nonfunc-
tioning, but can cause hyperthyroidism
Corticotroph adenomas: Can cause
Cushing’s disease
Lactotroph adenomas: Causes
hyperprolactinemia
Somatotroph adenomas: Causes increased
GH
• Symptoms include visual defects, diplopia,
headches, and symptoms assoicated with hor­monal imbalance.
• MRI with gadolinium enhancement is the
single best radiological test for midline cen­tral skul base tumor diagnosis. Other test include CT.
• Hormanal studies are also recommended.
• Treatment is surgical excision or debulking
(transnasal or open cranial approach), except in the case of pituitary incidentaloma where the patient has an incidental nding of pitu­itary tumor >1 cm on radiography, but no symptoms (Fig.30.4).
30.6.2 Chordomas
andChondrosarcomas
• Chondromas are rare, locally aggressive,
slow-growing neoplasms.
• Derived from embryonic remnants of the
notochord.
• Can involve parts of the body other than skull
base such as sacrococcygeal region, and verte­bral column.
• Three types of chordomas: – Conventional: Absent cartilaginous or
mesenchymal components.
Chondroid: Contains both chordomatous
and cartilaginous features. Usually seen in sphenooccipital region.
Dedifferentialted or sarcomatous type.
Grossly they appear as gelatinous pink or
gray masses with solid and cystic areas.
• Whereas chondrosarcomas are rare malignant
cartilaginous tumors.
• Chondrosarcomas grossly appear as smooth, lobulated, hard tumors.
• Microscopically chondrosarcomas are three types:
Well differentiatedModerately differntiatedPoorly differntiated
• Surgical excision is preferred for both chordo­mas and chondrosarcomas (transnasal or transfacial approach).
• Radiotherapy/adjuavant radiotherapy is also used in treatment of chordomas and chondrosarcomas.
30.6.3 Craniopharyngomas
• Craniopharyngiomas are benign epithelial tumors that usually arise in the pituitary stalk in the suprasellar region, adjacent to the optic chiasm. A small percentage arise within the sella, and a few tumors have been described within the optic system or the third ventricle.
• Slow growing.
• Symptoms include headache, visual symp­toms, hormonal deciencies, growth failure, nausea, and vomiting.
• MRI and CT are used for radiography.
• Characteristic niding is a cystic, calcied suprasellar mass.
• Pretreatment requisites include adrenal, thy­roid function and neuroopthalmic evaluation.
• Treatment needs multidisciplinary team with surgical resection and radiotherapy followed by post-op hormonal therapy if required.
30.6.4 Others
• Meningioma: Benign tumor arising from the meninges.
• Pituicytoma: Uncommon, low-grade, indolent glioma arising from the pituicytes of the pos­terior pituitary. It presents as a sellar mass, which is usually mistaken for a pituitary ade­noma, and has no known hormonal secretory function.
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S. Ashkanani and A. Menon
c
Fig. 30.4 (a) Ct head sagittal view showing sellar mass. (b) T1W MRI showing enhancing suprasellar lesion encroach-
ing the cavernous sinus. (c) T2W MRI showing intensely enhancing suprasellar lesion encroaching the cavernous sinus
30.7 Tumors ofPosterior SkullBase
Take Home Messages
• The skull base is a highly complex region that includes multiple bones. Eleven
• Cerebellopontine tumors – Vestibular schwannomas – Meningioma – Epidermoid schwannomas – Non-vestibular schwannoma – Arachnoid cyst (Table30.4)
pairs of cranial nerves and the olfactory nerves (CN I) pass through the inner table of the skull via seven pairs of bony foramina and the cribriform plate (CN I).
• Lesions and tumors can pose a chal­lenge due to its location. Hence, apt knowledge of anatomy is necessary.
30 Anterior andMidline Central Skull Base Tumors
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Table 30.4 CT vs. MRI for skull base imaging
CT MRI
Advantages Good for spatial
Bone imaging
Used specically for
Cuts Submillimetric
resolution Cortical bone well imaged
Fractures, sinonasal disease, middle ear and mastoid disease
(mm) with multiplanar reformats
Good for soft tissue resolution Medullary bone is well imaged (central skull base) Extracranial, central skull base or intracranial extension of disease, cranial nerve pathology 3mm sections in at least two planes (including coronal) or increasingly volumetric. STIR (short tau inversion recovery), T1-weighted precontrast and T1-weighted fat-saturated post gadolinium
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8. Flint PW, Haughey BH, Lund VJ, Niparko JK, Robbins KT, Thomas JR, Lesperance MM. Cummings otolaryngology: head and neck surgery. 6th ed.
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