This is a clinical presentation on the diseases of Base of the Skull

1. DR. ATM HASIBUL HASAN
MD (Neurology) thesis part student,
DMCH
DISEASES OF THE SKULL
AND SKULL BONES

2. SPECIES RULING THE WORLD

4. ???

5. HUMAN AS THE RULER

6. SKULL IN SPECIES

7. EVOLUTION

8. Imaging of Skull
Disorders related to Skull
 Disease of Calavaria
 Disease of Base of Skull
TOPICS FOR DISCUSSION

9. NORMAL VARIATION OF SKULL

11. BRACHYCEPHALIC

12. MESOCEPHALIC DOLICOCEPHALIC
SKULL VARIATION

13. X ray
CT Scan
MRI
IMAGING MODALITIES

14. X-RAY SKULL

18. CT SCAN

24. 3D CT

25. 3D VRT

26. 3D VRT

28. 3D VRT SKULL BASE

29. 3D PRINT

30. MRI

31. MRI

34. Structural
Traumatic
Vascular
Inflammatory
Neoplastic
Systemic Disease
DISEASES OF SKULL

35. DISEASES OF CALAVARIA

36.  Failure of Development:
 Anencephaly
 Microcephaly
 Abnormal Development:
 Craniosynostosis
 Craniofacial Dysmorphic Syndrome
DEVELOPMENTAL DISORDERS

37. Features: (NINDS)
 Blind
 Deaf
 Unaware of surroundings
 Unable to feel pain
Cause:
 Diet: Lack of folic acid
 Drugs & Toxins
Diagnosis: (Screening)
 USG
 AFP
Prognosis:
 Death within hours to days if not still born
ANENCEPHALY

38.  Definition:
o HC >2SD below the mean
 Cause:
 Prenatal
 Congenital: Isolated, Syndromic
 Acquired: Injury, Infection, Drug
 Postnatal
 Syndromic:
 Acquired: Injury, Infection, Toxin,
Malnutrition
 Presentation:
 Developmental delay
 Intellectual disability
 Varying degree of motor deficit
 Seizure
MICROCEPHALY

39. CRANIOSYNOSTOSIS

40. SCAPHOCEPHALY

42. TRIGONCEPHALY

43. PLAGIOCEPHALY

44. BRACHYCEPHALY

45. 3D CT

46. TURRYCEPHALY

47. CLOVERLEAF DEFORMITY

48. Syndrome Craniofacial feature Association
CROUZON (Craniofacial
dystosis)
CSO of Coronal and Basal
Skull sutures
Maxillary Hypoplasia
Shallow Orbit
Proptosis
Rarely Hydrocephalus
APERT(Acrocephalosyndactyly) Same Syndactyly of digit 2, 3, 4
Shortened Upper Limb
Hydrocephalus
KLEEBLATTSHADEL CSO with Trilobular skull May be associated with
APERT
CRANIOFACIAL DYSMORPHIC
SYNDROME

49. GENETIC BASIS

50. Facts:
 AD Branchial arch syndrome (1st
Arch)
 Early fusion of skull and facial bones
 Low set ears, craniosynostosis,
exopthalmos, hypertelorism,
hypoplastic maxilla with relative
prognathism. PDA, Coarctation
aorta.
CROUZON SYNDROME

51. Facts:(Acrocephalosyndactyly)
 Affects 1st Branchial arch.
 Acro Cephalo= Peaked Head
 Syndactyly= Webbing of fingers and toes
 Different forms of Craniosynostosis may
be present
APERT SYNDROME

52. DISEASES OF SKULL BASE

53. Congenital
 Dermoid and epidermoid
 Meningoencephalocoele
 Nasal Glioma
Developmental
 Chiari malformation
 Basilar impression
 Occipitalization of Atlas
Infec/Inflammatory
 Tuberculosis, Sarcoidosis
 Wegners granulomatosis
 Mucocoele
 Invesive fungal lesion
Tumor
 Sinonasal: Juv AF, Sq Ca, Adeno Ca,
Melanoma, Lymphoma
 Intracranial: Meningioma,
Schwannoma, Pituitary Adenoma,
Craniopharyngioma
Trauma
DISORDERS OF SKULL BASE

54. Der moid Epider moid
CONGENITAL DISORDERS

55. A 53-year-old female presented with two years history
of frequent and intense headaches with left nasal
obstruction, recurrent post nasal drip and a gradually
developing anosmia and left intermittent watery
rhinorrhea that worsens when she cough, sneezes and
defecate.
CLINICAL SCENARIO

56. MENINGOENCEPHALOCELE

57.  Chiari malformation
 Occipitalization of atlas
 Basilar Impression
DEVELOPMENTAL DISORDERS

58. TRAIT CHIARI I CHIAR-II
Usual Age Young Adult and Older Children Infant
Usual Presentation Cervical pain,
Suboccipital Headache
Myelopathy
Lower Brainstem Symptoms
Central Cord Symptoms
Features of Brainstem
Dysfunction (Difficulty in
swallowing, stridor, apnoea)
Progressive hydrocephalus
Weakness of extremity
Caudal Descend Tonsil Inferior vermis, Medulla, 4th
Ventricle
Caudal Descend of Medulla Unusual Yes
Spina Bifida Unusual Rarely Absent
Hydrocephalus <10% Very common
Medullary Kink Absent Present
Associated Anomaly Craniocervical Hypermobility
Kippel-Feil , NF-II
Agenesis of Corpus Callosum
Gyral abnormality

59. CHIARI-I CHIARI-II

60. CHIARI III CHIARI IV
Dysmorphic cerebellar and brainstem tissue Hypoplasia or aplasia of the cerebellum
DIFFERENCES

61. BASILAR IMPRESSION

62. OCCIPITALIZATION OF ATLAS

63. VASCULAR LESION

64.  A 15 year-old girl was bitten by an unknown
insect to her right forehead.
 Two days later she developed a sudden right
complete ptosis.
 Examination revealed poor ocular motility
along with sensory deficit in V1 distribution.
CLINICAL SCENARIO

65. Cavernous
Sinus
Thrombosis

66.  A 32 years old man presented with pain full
swelling of left eye along with double vision
following a RTA.
 Examination revealed pulsatile exophthalmos
along with bruit on the left eye.
CLINICAL SCENARIO

67. CAROTID CAVERNOUS FISTULA

68. A 43 years old man with
worsening left sided
headache.
CLINICAL SCENARIO
Cavernous Angioma

69.  A 35 years old man
presented with
headache, double
vision, difficulty in
deglutition and
heaviness in tongue.
 Examination revealed
bilateral deficit of CN
III, VI, VII, X and XII.
INFECTION
SKULL BASE OSTEOMYELITIS

70. A 17 years old girl presented with 2 weeks history of
sharp peri orbital pain with blurring of vision, double
vision and drooping of left eyelid.
She had a similar episode of attack more than a year
back.
On clinical examination she had left sided complete
opthalmoplegia along with ptosis. But there was no
facial sensory loss.
CLINICAL SCENARIO

71. THS
After 4 months of treatment

72. IHS DIAGNOSTIC CRITERIA -THS

73. THS VS CST
TRAIT THS CST
Site Usually unilateral Initially unilateral, but
often bilateral
Involvement of
5th nerve
Unusual Often involved
Response to
steroid
Good None

74. HEAD INJURY
NEUROTRAUMA

75. CLASSIFICATION

76. EVALUATION

77. INDICATION OF IMAGING

78. MILD HEAD INJURY

79. Type Presentation Mechanism Treatment
EDH Lucid interval between time of impact and
presentation.
Headache, Vomiting, Focal seizure, Deficit
Arterial Bleed Burr Hole
Contusion Headache, confusion, LOC, seizure
Difficulties with memory, speech, vision
Small vessel bleed in
parenchyma
Supportive
SDH Gradually increasing headache, confusion,
dizziness, amnesia
Bridging venous
bleed
Surgical
evacuation
C SDH Altered mentation, recurrent fall, headache,
seizure, transient neurologic deficit
Surgical
evacuation
SAH Unconscious, neck stiffness Supportive
DAI Coma and 90% never regain consciousness, no
focal sign.
Traumatic shearing
force resulting in
diffuse white matter
injury
Supportive
Polyethyline
glycol

80. INTRACRANIAL INJURY

81. FRACTURE OF SKULL BONES
Skull Fracture
Vault Fracture
Linear
Depressed
Comminuted
Compound
Open
Base Fracture
Anterior Fossa
Middle Fossa
Posterior Fossa

82. DEPRESSED FRACTURE

83. Linear Comminuted

84. COMPOUND FRACTURE

85. Symptoms and signs:
o Battle’s sign
o Raccoon eyes
o CSF Rhinorrhoea
o Cranial nerve palsy
o Bleeding from nose, ear
o Vomiting
SKULL BASE FRACTURE

86. TEMPORAL BONE FRACTURE

87. SKULL BASE TUMORS

88. COMMON TUMORS
Anterior Cranial Fossa Middle Cranial Fossa Posterior Cranial Fossa
• Juvenile Angiofibroma
• Inverted Papilloma
• Lymphoma
• Nasopharyngeal carcinoma
 Benign:
• Pituitary adenoma
• Craniopharyngioma
• Cavernous sinus
meningioma
 Malignant:
• Pituitary chondrosarcoma
and osteogenic sarcoma
• Secondaries
• Meningioma of Foramen
magnum, Cleivus and CP
angel
• Epidermoid
• Dermoid
• Chondroma
• Chordoma

89.  Benign vascular neoplasm
 Affects children and
adolescents
 Involve sphenopalatine
foramen early.
JUVENILE NP ANGIOFIBROMA

90. A 32 years old woman presented with headache,
blurring of vision along with protrusion of right
eye and menstrual irregularities. Examination
revealed marked optic atrophy on right and early
atrophic changes on the left eye.
CASE SCENARIO

91. PITUITARY ADENOMA

92. Snowman Sign

93. CRANIOPHARYNGIOMA

94. SUPRA SELLAR MENINGIOMA

95. RADIOLOGY OF SELLA

96.  Low grade malignancy
 Arise from primitive
notochord
 Age: 20-40 years
 Involves clivus
 Presentation: Headache,
neck pain, cranial nerve
palsy
CHORDOMA

97. Multiple myeloma
Thalassemia
Acromegaly
Peget’s Disease
SYSTEMIC DISEASE

98. THALASSEAMIA MULTIPLE MYELOMA

99. ACROMEGALY PEGETS DISEASE