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IMAGING OF
   NEUROCUTANEOUS
      SYNDROME
      Moderators: Prof. (HOD) R .K. GOGOI




PRESENTATION BY-
CHARUSMITA CHAUDHARY
INTRODUCTION
INTRODUCTION
INTRODUCTION
Phakomatoses (or "neurocutaneous syndromes")
 are multisystem disorders

inherited or spontaneous mutation
Common ectodermal origin


Advances in molecular biology have been able to
 localize the genetic abnormality
NEUROFIBROMATOSIS TYPE 1

NF1 is the classic von Recklinghausen or "peripheral"
 disease.
AD disorder/Spontaneous mutation
Most common, 1 in 2000 to 3000 live births
Inherited predisposition for the development of
 benign peripheral nerve sheath tumors
 (neurofibromas) true CNS neoplasms
Diagnostic criteria
Two or more of the following:
    Six or more cafe´ au lait spots 0.5 cm or larger in prepubertal
     individuals 1.5 cm or larger in postpubertal individuals

one plexiform neurofibromas or 2/more neurofibromas of any
 type
Two or more Lisch nodules (benign hamartomas)
Freckling in the axilla or groin
Optic gliomas
A distinctive bony lesion Dysplasia of the sphenoid bone
 ,Dysplasia or thinning of long bone cortex

First degree relative with NF1
Neuroimaging Findings

CNS lesions in 15-20%
      brain, spinal cord, dural, orbital, vascular
Hamartomatous and neoplastic lesions
Multifocal T2 hyperintense signal changes in 80% of
 patients
Optic nerve(5-15%) and non optic glioma(low grade
 astrocytoma)
Plexiform Neurofibromas in the head and neck(1/3rd
 of patients)-diagnostic
Optic Nerve Gliomas
An important and often diagnostic feature of NF1
Surveillance is important, because up to 80% of
 patients with ONGs are asymptomatic
B/L ONGs is considered specific for NF1
Primary findings of ONG include
abnormal optic nerve thickening
beading and elongation
abnormal enhancement
Plexiform neurofibroma

cutaneous or subcutaneous neurofibromas
intraorbital and facial br of CN III - VI,
MC affects CN V
Diffuse plexiform neurofibroma of the face and
 eyelids,
Sphenoid dysplasia is one of the "distinctive bone
 lesions”
SKELETAL MANIFESTATIONS :
      MOST COMMONLY INVOLVED AREAS-SPINE & SKULL.
   1.SPINE:
KYPHOSCOLIOSIS OF LOWER THORACIC SPINE(MOST
  COMMON),CERVICAL SPINE.
 POSTERIOR VERTEBRAL BODY SCALLOPING;
              POSTEROCENTRAL DUE TO DURAL ECTASIA.
              ECCENTRIC UNILATERAL DUE TO DUMBBELL
  NEUROFIBROMA.
      ENLARGED INTERVERTEBRAL FORAMEN
      PARASPINAL SOFT TISSUE.
             DUMBBELL NEUROFIBROMA
            INTRATHORACIC meningocele
 2.SKULL:
 -AGENESIS OR HYPOPLASIS OF POSTERIOR WALL OF ORBIT,
  WINGS OF SPHENOID & ORBITAL PLATE OF FRONTAL.
 -OPTIC FORAMEN ENLARGEMENT
 -GEOGRAPHIC BONE LESION AROUND THE LAMDOID SUTURE
  ALONG WITH MASTOID HYPOPLASIA.
 MACROCRANIUM.
 3. RIB:
 - TWISTED RIBBON RIB– THIN IRREGULAR, SCALLOPED
  ATTENUATED APPEARANCE OF RIBS.
 4. LONG BONES:
 - PSEUDOARTHOSIS.
 -FOCAL GIGANTISM
 -SUBPERIOSTEAL OR CORTICAL LUCENCIES DUE TO
  INTRAOSSEOUS NEUROFIBROMA.
 -CORTICAL PRESSURE RESORPTION DUE TO ADJACENT SOFT
  TISSUE
ABSENCE OF SPHENOID WINGS
KYPHOSIS & ENLARGEMENT OF NEURAL
FORAMINA
HYPERTROPHIED & TWISTED RIBBON
RIBS
CASE 1: Cranio-orbital-temporal Neurofibromatosis
(Plexiform neurofibroma of ophthalmic division of
trigeminal nerve)

                         History :-
                         # 15 year old female patient presented with
                         a large painless swelling over right eyelid
                         and face causing cosmetic deformity.

                         Clinical examination :-
                         # A large boggy swelling over right upper lid
                         and temporal regions with a feeling of bag
                         of worms on palpation. No vascular
                         pulsation noted.
Fig 2b T2WI       Fig 2c T2WI           Fig 2d T1WI-GD
Fig 2a T1WI


                            MRI (T1WI) & 3D Volume rendered CT
                            images reveal –
                            # Enlargement of the right middle
                            cranial fossa with herniation of the right
                            temporal lobe into the posterior aspect
                            of the right orbit with a preceding
                            anterior sleeve of CSF (fig 2a)
                            # Hypoplasia of the greater wing of the
                            sphenoid bone and superiorly displaced
  Fig 2e        Fig 2f
                            lesser wing, together giving the typical
                            ‘Bare orbit sign’ (fig 2e)
CASE2: Cutaneous Neurofibroma with Extracranial Arterio-
venous malformation and Atlanto-axial dislocation




 # 25 year old male patient presented with vertigo, tinnitus in left ear and a gradually
 progressing quadriparesis for 25 days.
 # Clinical examination revealed:
 1. Multiple subcutaneous nodules with plexiform neurofibroma
 2. Decreased power of all the limbs with intact sensations and bilateral extensor plantar jerks
 3. Otoscopic examination, audiometry and Laboratory parameters were unremarkable.
Fig 3a T2FS sagittal     Fig 3b T2FS sagittal               Fig 3c T2 Coronal

                       Sagittal and coronal MR images shows :-
                       # Atlanto-axial dislocation with retropulsion of odontoid tip
                       leading to secondary foramen magnum stenosis (fig 3a).
                       # Dilated, Tortuous V3 segment of left vertebral artery with
                       formation of multiple blood filled channels. (fig 3c,d).
                       # Distended venous sac in anterior epidural space displacing
                       the spinal cord posteriorly and to the right, causing
                       compressive cord myelopathic changes (Fig 3b,c )


Fig 3d T2 coronal
Fig 3d




      Fig 3a                  Fig 3b                 Fig 3c


MR TOF Angiography images reveal:-
                                                                   Fig 3e
# Arterio-venous malformation in posterolateral aspect of left
  side of neck .
Feeders --- V3 segment of vertebral artery (fig 3a,b) &
   an anomalous artery arising from 1st part of left subclavian
  artery (fig 3c).
Draining --- left sigmoid sinus (fig 3e) with a hugely distended
  venous sac in cervical canal.


                                                                     Fig 3f
Case 3 : Neurofibromas of the vagus nerve of the neck

 32-years old female patient .
 Gradual onset of swelling , increasing on size on the right
   side of the neck for about few months.




 Clinical examination :An ill-defined, palpable lobulated
   lesion was observed on the left side of the neck. Café au lit
   spots were not detected on the skin.
Carotid an-giography done at Apollo hospital Delhi
                                     Biopsy.
excluded a tumor of carotid wall


              Histological examination showed that the typical
              features of neu-rofibromatic tumour -spiral cells
              and highly collagenised stroma.


        Neurofibromas of the vagus nerve
       on the neck are extremely rare .
NF-1 : MR Signal Abnormalities
    - Globus pallidus
  T2W bright foci w/o mass, don’t enhance
  - Cerebellar peduncles, pons, midbrain
  - Globus pallidus , thalamus , optic radiations
  What in the heck are they??
   - intracellular proteinous fluid?
  -Dysmyelination ??
  T1W bright foci
Neurofibromatosis 2



 NF2 also has an AD pattern,1 in 50000
 Multiple cranial nerve schwannomas are the hallmark
 MC in vestibulocochlear nerve
DIAGNOSTIC CRITERIA FOR
NEUROFIBROMATOSIS 2 (NF2)


Bilateral CP angle masses (histologic proof not
 required)

A first-degree relative with NF2 and either
-A unilateral CPA mass or
-Any two of the following:
schwannoma, meningioma, glioma, neurofibroma, or
 juvenile posterior subcapsular cataract
CNS lesions -100%
Brain - CN VII schwannomas, multiple schwannomas
 of other cranial nerves,
Meningiomas,
Spinal cord/roots - Cord ependymomas, multilevel
 bulky schwannoma, Meningioma
Spine-Secondary changes
Cutaneous manifestations rare
M.I.S.M.E.
M MULTIPLE
I INHERITED
S SCHWANNOMA
M MENINGIOMA
E EPENDYMOMA
ENCEPHALOTRIGEMINAL ANGIOMATOSIS
DIAGNOSTIC CRITERIA FOR STURGE-WEBER
SYNDROME

Seizures
Mental handicap
Port-wine stain (neveus flammeus)
Leptomeningeal capillary/venous malformation
 (ipsilateral to no. 1)
Cerebral hemiatrophy (ipsilateral to no. 1)
Facial hemihypertrophy (ipsilateral to no. 1)
Somatic hemiatrophy (contralateral to no. 1)
CT and MR can reveal the secondary
changes,
 cerebral cortical atrophy,
 gyriform cerebral calcification (tram-track),
 compensatory ventricular enlargement,
 "angiomatous“ enlargement of the ipsilateral choroid
  plexus, and
 calvarial hemihypertrophy
 MR- direct visualization of the persistent embryologic
  plexus in subarachnoid space
 Ocular lesions - Buphthalmos, Scleral/choroidal
  angiomata
STURGE- WEBER SYNDROME :
Port wine stain ( PWS)
Facial neveus flammeus
Blanches with pressure
Trigeminal dermatome
V1-opthlmic
V2- maxilary
V3-mandibular
PIAL ANGIOMATOSIS,MEDULLARY COLL
           & CEREBRAL ATROPHY
Case II
5 yr female
Complaints of focal seizure
 involving right side of body
 ,impaired milestone , right sided
 weakness
Clinically- portwine strain + ,
 Buphthalmus left
MR imaging…..
Gyriform (linear, convoluted, or serpentine)
calcifications



  infarction, glioma, purulent meningitis,
  leukemia (following intrathecal administration
   of methotrexateand skull irradiation), ossifying
   meningoencephalopathy,
  and subarachoid fat
Dyke-Davidoff-Masson syndrome
 Dyke-Davidoff-Masson syndrome (DDMS) was initially described as 
changes in the skull seen on skull X-ray in patients with cerebral 
hemiatrophy, but is now applied more broadly to cross-sectional imaging 
also. It is characterised by :
•thickening of the skull vault (compensatory)
•enlargement of the frontal sinus (also ethmoidal and mastoid air-cells)
•elevation of the petrous ridge
•ipsilateral falcine displacement
In some sources it is equated to hemispheric infarction, whereas in other 
sources any cause ofcerebral hemiatrophy are included.   
Etymology
Initially described by C.G Dyke , L.M Davidoff and C.B Masson in 
1933 5
Differential diagnosis
General considerations include
hemimegalencephaly :
Sturge-Weber syndrome : can also be an association
Rasmussen encephalitis : tends not to have calvarial changes
Tuberous sclerosis
AD; 50% from new spontaneous mutations
1 in 20,000 to 1 in 50,000
nearly 40% of patients die by the age of
35 years
prominent cutaneous, visceral, and CNS
 manifestations
Most lesions are hamartomas
DIAGNOSTIC CRITERIA FOR TSC

Definite TSC
Two major features, or
one major plus two minor features.


Probable TSC
One major plus one minor feature.


Possible TSC
one major feature, or
two or more minor features.
DIAGNOSTIC CRITERIA FOR TSC

MAJOR FEATURES
Hypomelanotic macules (three or more), Shagreen
 patch
Facial angiofibromas (adenoma sebaceum) or ungual
 or periungual fibromas
Multiple retinal nodular hamartomas,
Cortical tubers, Subependymal nodule,
 Subependymal giant cell astrocytoma
Cardiac rhabdomyoma - single or multiple
Lymphangiomyomatosis.
Renal angiomyolipoma
DIAGNOSTIC CRITERIA FOR TSC
   MINOR FEATURES
   Multiple pits in dental enamel
   Gingival fibromas
   Hamartomatous rectal polyps
   Bone cysts
   Cerebral white matter radial migration lines
   Retinal achromic patch
   Multiple renal cysts
   Nonrenal hamartoma
   "Confetti" skin lesions
Pringle’s disease
Subungal fibroma
Cortical tubers:
considered to be closely related to neurologic
 manifestations of TS - epilepsy, cognitive disability,
 and neurolobehavioral abnormalities
50% seen in frontal lobe
Hypointense on T1-WI
Hyperintense on T2-WI, FLAIR
Only 10 % enhance
Subependymal Nodules

represent hamartomatous change.
seen in 98%


calcification detected in CT (88%)
hyperintense on T1WI
Iso- to hyp0intense on T2WI
SGCAs


proliferative astrocytes and giant cells.
1.7%–26% prevalence
Typically in foramen of Monro
Differ from other cerebral astrocytomas in having a
 benign biologic and pathologic features (slow growth,
 minimal or no attendant brain edema, and minimal
 invasiveness)
tend to be larger tumors (>1 cm) with incomplete
 calcifn & more intense enhancement
MRS shows high Cho/Cr and low NAA/Cr r
White Matter Abnormalities


Superficial white matter abnormalities associated
 with cortical tubers,

 Radial white matter bands (15%–27%)


Cystlike white matter lesions(15%–44%)
PULMONARY AND THORACIC
INVOLVEMENT

lymphangioleiomyomatosis (LAM)
multifocal micronodular pneumocyte hyperplasia
 (MMPH).
approx 1%–2.3% of TS patients.
  complications of LAM Pneumothorax and chylous
   pleural effusion
  ascites.
round, thin-walled cysts of variable size and contour At thin-section CT, multiple tiny nodules (1–8 mm 
                                                       in diameter) are diffusely scattered throughout the 
                                                       lung in a random distribution 
RENAL AND RETROPERITONEAL
INVOLVEMENT

Renal angiomyolipoma (AML),
renal cysts, and
RCC
Renal AML in 55%–75% patients with TS
Retroperitoneal LAM in up to 20% of patients with
 pulmonary LAM.
AMLs
MC benign tumors of the kidney.
characterized by variable amounts of abnormal
 vessels, immature smooth-muscle and fat cells
Compared with sporadic lesions, AMLs seen in
 patients with TS tend to manifest at a younger age
multiple, larger, and bilateral and
Tend to grow.


noncalcified cortical tumors containing fat of less
 than −20 HU
Retroperitoneal LAM


thick- or thin-walled cystic lesions.
may reflect dilatation of lymph vessels due to
 obstruction
SKELETAL INVOLVEMENT


Cyst like lesions,
hyperostosis of the inner
 table of the calvaria ,
osteoblastic changes,
periosteal new bone
 formation, and
scoliosis.
VON HIPPEL-LINDAU DISEASE


AD disorder linked to defect on the short arm of
 chromosome 3p.
Prevalence is approximately 1 in 40,000 to 1 in 50,000
 people
Causes of death - cerebellar hemangioblastoma and
 RCC
Screening is important because the lesions in VHL
 disease are treatable
Manifestations of VHL Disease according to
Prevalence
 Cerebellar hemangioblastoma 44–72%
 Medullary hemangioblastoma 5%
 Spinal cord hemangioblastoma 13–59%
 Retinal hemangioblastoma 45–59%
 Renal cell carcinoma 24–45%
 Pheochromocytoma 0–60%
 Neuroendocrine tumor of the pancreas 5–17%
 Serous cystadenoma of the pancreas 12%
 Pancreatic cysts 50–91%
 Renal cysts 59–63%
 Papillary cystadenoma of epididymis 10–60 %.
NATIONAL INSTITUTES OF HEALTH CLASSIFICATION
 Type I
 VHL without pheochromocytoma
 most common type
 Renal and pancreatic cysts, RCC


 Type II
 VHL with pheochromocytoma
 IIa - Islet cell tumors (no cysts)
 Iib - Renal/pancreatic disease (least common)
DIAGNOSTIC CRITERIA

More than one CNS hemangioblastoma,


One CNS hemangioblastoma + visceral
 manifestations of VHL disease,

Any manifestation and a known family history of VHL
 disease.
Hemangioblastoma
Hallmark of VHL
Seen in 2/3 of patients
20-50 yrs of age
Typically multiple
MC in cerebellum
Other::::medulla > pons, spinal cord, and
 supratentorially in optic N and cerebrum
Retinal angiomas




are actually hemangioblastomas (40-50%)
asymptomatic or cause a blind spot.
may hemorrhage and can cause retinal detachment
Higher signal intensity than normal vitreous on non-
  enhanced T1WI
Renal lesions



Renal cysts in 59%–63%
RCC in 24%–45% either multicentric and bilateral
 solid hypervascular masses or complex cystic masses
Complex or solid lesions enhance on postcontrast T1-
 WI
A hypointense pseudocapsule on T2-WI
Pancreatic involvement
simple pancreatic cysts
 (50%–91%)
serous microcystic
 adenomas
Pancreatic
 neuroendocrine tumors
 (5%–17%)
Pancreatic lesions may
 be the only abdominal
 manifestation and may
 precede any other
 manifestation by
 several years
Annual screening examinations of the abdomen, by
 ultrasound or CT, have been recommended for some
 patients with VHL
Ataxia-telangiectasia

AR disorder
1 in 20,000-100,000
Telangiectasias in skin (face) and eyes,
cerebellar ataxia
immunodeficiency syndromes, and recurrent
 infections and susceptibility to certain neoplastic
 processes
MR FINDINGS



Telangiectasia of pia mater and white matter
Hypointense WM foci on T1- and T2-WI
Diffuse symmetric increased T2 white matter signal
Severe cerebellar atrophy
Hypointense WM foci on T1- and T2-WI
THE PHACE SYNDROME
Posterior fossa malformations
Hemangiomas
Arterial anomalies
Coarctation of the aorta , cardiac defects
Eye abnormalities
 Sometimes, an S is added making it PHACES, with
 the S standing for Sternal defects and/or
 Supraumbilical raphe.
Large facial hemangiomas may be associated with a Dandy-
 Walker malformation, vascular anomalies (coarctation of
 aorta, aplasia or hypoplastic carotid arteries, aneurysmal
 carotid dilation, aberrant left subclavian artery), glaucoma,
 cataracts, microphthalmia, optic nerve hypoplasia, and ventral
 defects (sternal clefts)
Facial hemangioma is typically ipsilateral to the aortic arch
Female predominance


Patients with large facial cutaneous (S1-S4) hemangiomas
  were especially at risk of CNS structural and cerebrovascular
  anomalies; S1 with ocular anomalies; and S3 with airway,
  ventral, and cardiac anomalies.
Gorlin syndrome
 Diagnostic criteria
 A clinical diagnosis can be made using major and minor criteria.
  To make the diagnosis, either two major or, one major and two minor 
  criteria must be met.
 Major criteria( Classical triad )
 basal cell cancers : > 2 or 1 under the age 20
 odontogenic keratocysts (see case 1)
 palmar pits : 3 or more
 bilamellar calcification of the falx cerebri
 rib anomalies : bifid rib fused, splayed
 first degree relative with Gorlin syndrome
 Minor criteria
 macrocephaly
 frontal bossing, cleft lip or hypertelorism
 Sprengel deformity,  pectus excavatum or pectus carinatum, syndactyly
 bridging of the sella turcica, hemivertebrae, flame shaped radiolucencies
 ovarian fibroma, medulloblastoma
Gorlin syndrome
Conclusion


Phakomatoses are a diverse group of disorders
Most common phakomatoses (excluding SWS) are
 AD; therefore, a correct diagnosis has genetic
 implications
A screening evaluation of all first-degree relatives to
 see if they are also affected is mandatory
A routine follow-up surveillance program should be
 established. This typically includes annual CNS
 imaging studies and, where appropriate, abdominal
 ultrasound, CT, or MR.
THANK YOU

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Imaging of neurocutaneous syndrome overview

  • 1. IMAGING OF NEUROCUTANEOUS SYNDROME Moderators: Prof. (HOD) R .K. GOGOI PRESENTATION BY- CHARUSMITA CHAUDHARY
  • 4. INTRODUCTION Phakomatoses (or "neurocutaneous syndromes") are multisystem disorders inherited or spontaneous mutation Common ectodermal origin Advances in molecular biology have been able to localize the genetic abnormality
  • 5.
  • 6.
  • 7. NEUROFIBROMATOSIS TYPE 1 NF1 is the classic von Recklinghausen or "peripheral" disease. AD disorder/Spontaneous mutation Most common, 1 in 2000 to 3000 live births Inherited predisposition for the development of benign peripheral nerve sheath tumors (neurofibromas) true CNS neoplasms
  • 8.
  • 9. Diagnostic criteria Two or more of the following:  Six or more cafe´ au lait spots 0.5 cm or larger in prepubertal individuals 1.5 cm or larger in postpubertal individuals one plexiform neurofibromas or 2/more neurofibromas of any type Two or more Lisch nodules (benign hamartomas) Freckling in the axilla or groin Optic gliomas A distinctive bony lesion Dysplasia of the sphenoid bone ,Dysplasia or thinning of long bone cortex First degree relative with NF1
  • 10.
  • 11. Neuroimaging Findings CNS lesions in 15-20% brain, spinal cord, dural, orbital, vascular Hamartomatous and neoplastic lesions Multifocal T2 hyperintense signal changes in 80% of patients Optic nerve(5-15%) and non optic glioma(low grade astrocytoma) Plexiform Neurofibromas in the head and neck(1/3rd of patients)-diagnostic
  • 12. Optic Nerve Gliomas An important and often diagnostic feature of NF1 Surveillance is important, because up to 80% of patients with ONGs are asymptomatic B/L ONGs is considered specific for NF1 Primary findings of ONG include abnormal optic nerve thickening beading and elongation abnormal enhancement
  • 13.
  • 14. Plexiform neurofibroma cutaneous or subcutaneous neurofibromas intraorbital and facial br of CN III - VI, MC affects CN V Diffuse plexiform neurofibroma of the face and eyelids, Sphenoid dysplasia is one of the "distinctive bone lesions”
  • 15. SKELETAL MANIFESTATIONS : MOST COMMONLY INVOLVED AREAS-SPINE & SKULL. 1.SPINE: KYPHOSCOLIOSIS OF LOWER THORACIC SPINE(MOST COMMON),CERVICAL SPINE.  POSTERIOR VERTEBRAL BODY SCALLOPING;  POSTEROCENTRAL DUE TO DURAL ECTASIA.  ECCENTRIC UNILATERAL DUE TO DUMBBELL NEUROFIBROMA.  ENLARGED INTERVERTEBRAL FORAMEN  PARASPINAL SOFT TISSUE. DUMBBELL NEUROFIBROMA INTRATHORACIC meningocele
  • 16.  2.SKULL:  -AGENESIS OR HYPOPLASIS OF POSTERIOR WALL OF ORBIT, WINGS OF SPHENOID & ORBITAL PLATE OF FRONTAL.  -OPTIC FORAMEN ENLARGEMENT  -GEOGRAPHIC BONE LESION AROUND THE LAMDOID SUTURE ALONG WITH MASTOID HYPOPLASIA.  MACROCRANIUM.  3. RIB:  - TWISTED RIBBON RIB– THIN IRREGULAR, SCALLOPED ATTENUATED APPEARANCE OF RIBS.  4. LONG BONES:  - PSEUDOARTHOSIS.  -FOCAL GIGANTISM  -SUBPERIOSTEAL OR CORTICAL LUCENCIES DUE TO INTRAOSSEOUS NEUROFIBROMA.  -CORTICAL PRESSURE RESORPTION DUE TO ADJACENT SOFT TISSUE
  • 17.
  • 19.
  • 20.
  • 21.
  • 22.
  • 23. KYPHOSIS & ENLARGEMENT OF NEURAL FORAMINA
  • 24.
  • 25.
  • 26.
  • 27. HYPERTROPHIED & TWISTED RIBBON RIBS
  • 28.
  • 29.
  • 30.
  • 31.
  • 32.
  • 33. CASE 1: Cranio-orbital-temporal Neurofibromatosis (Plexiform neurofibroma of ophthalmic division of trigeminal nerve) History :- # 15 year old female patient presented with a large painless swelling over right eyelid and face causing cosmetic deformity. Clinical examination :- # A large boggy swelling over right upper lid and temporal regions with a feeling of bag of worms on palpation. No vascular pulsation noted.
  • 34. Fig 2b T2WI Fig 2c T2WI Fig 2d T1WI-GD Fig 2a T1WI MRI (T1WI) & 3D Volume rendered CT images reveal – # Enlargement of the right middle cranial fossa with herniation of the right temporal lobe into the posterior aspect of the right orbit with a preceding anterior sleeve of CSF (fig 2a) # Hypoplasia of the greater wing of the sphenoid bone and superiorly displaced Fig 2e Fig 2f lesser wing, together giving the typical ‘Bare orbit sign’ (fig 2e)
  • 35. CASE2: Cutaneous Neurofibroma with Extracranial Arterio- venous malformation and Atlanto-axial dislocation # 25 year old male patient presented with vertigo, tinnitus in left ear and a gradually progressing quadriparesis for 25 days. # Clinical examination revealed: 1. Multiple subcutaneous nodules with plexiform neurofibroma 2. Decreased power of all the limbs with intact sensations and bilateral extensor plantar jerks 3. Otoscopic examination, audiometry and Laboratory parameters were unremarkable.
  • 36. Fig 3a T2FS sagittal Fig 3b T2FS sagittal Fig 3c T2 Coronal Sagittal and coronal MR images shows :- # Atlanto-axial dislocation with retropulsion of odontoid tip leading to secondary foramen magnum stenosis (fig 3a). # Dilated, Tortuous V3 segment of left vertebral artery with formation of multiple blood filled channels. (fig 3c,d). # Distended venous sac in anterior epidural space displacing the spinal cord posteriorly and to the right, causing compressive cord myelopathic changes (Fig 3b,c ) Fig 3d T2 coronal
  • 37. Fig 3d Fig 3a Fig 3b Fig 3c MR TOF Angiography images reveal:- Fig 3e # Arterio-venous malformation in posterolateral aspect of left side of neck . Feeders --- V3 segment of vertebral artery (fig 3a,b) & an anomalous artery arising from 1st part of left subclavian artery (fig 3c). Draining --- left sigmoid sinus (fig 3e) with a hugely distended venous sac in cervical canal. Fig 3f
  • 38. Case 3 : Neurofibromas of the vagus nerve of the neck 32-years old female patient . Gradual onset of swelling , increasing on size on the right side of the neck for about few months. Clinical examination :An ill-defined, palpable lobulated lesion was observed on the left side of the neck. Café au lit spots were not detected on the skin.
  • 39. Carotid an-giography done at Apollo hospital Delhi Biopsy. excluded a tumor of carotid wall Histological examination showed that the typical features of neu-rofibromatic tumour -spiral cells and highly collagenised stroma. Neurofibromas of the vagus nerve on the neck are extremely rare .
  • 40. NF-1 : MR Signal Abnormalities - Globus pallidus T2W bright foci w/o mass, don’t enhance - Cerebellar peduncles, pons, midbrain - Globus pallidus , thalamus , optic radiations What in the heck are they?? - intracellular proteinous fluid? -Dysmyelination ?? T1W bright foci
  • 41.
  • 42. Neurofibromatosis 2 NF2 also has an AD pattern,1 in 50000 Multiple cranial nerve schwannomas are the hallmark MC in vestibulocochlear nerve
  • 43. DIAGNOSTIC CRITERIA FOR NEUROFIBROMATOSIS 2 (NF2) Bilateral CP angle masses (histologic proof not required) A first-degree relative with NF2 and either -A unilateral CPA mass or -Any two of the following: schwannoma, meningioma, glioma, neurofibroma, or juvenile posterior subcapsular cataract
  • 44. CNS lesions -100% Brain - CN VII schwannomas, multiple schwannomas of other cranial nerves, Meningiomas, Spinal cord/roots - Cord ependymomas, multilevel bulky schwannoma, Meningioma Spine-Secondary changes Cutaneous manifestations rare
  • 45.
  • 46.
  • 47. M.I.S.M.E. M MULTIPLE I INHERITED S SCHWANNOMA M MENINGIOMA E EPENDYMOMA
  • 48.
  • 49.
  • 50.
  • 52.
  • 53. DIAGNOSTIC CRITERIA FOR STURGE-WEBER SYNDROME Seizures Mental handicap Port-wine stain (neveus flammeus) Leptomeningeal capillary/venous malformation (ipsilateral to no. 1) Cerebral hemiatrophy (ipsilateral to no. 1) Facial hemihypertrophy (ipsilateral to no. 1) Somatic hemiatrophy (contralateral to no. 1)
  • 54. CT and MR can reveal the secondary changes, cerebral cortical atrophy, gyriform cerebral calcification (tram-track), compensatory ventricular enlargement, "angiomatous“ enlargement of the ipsilateral choroid plexus, and calvarial hemihypertrophy MR- direct visualization of the persistent embryologic plexus in subarachnoid space Ocular lesions - Buphthalmos, Scleral/choroidal angiomata
  • 55. STURGE- WEBER SYNDROME : Port wine stain ( PWS) Facial neveus flammeus Blanches with pressure Trigeminal dermatome V1-opthlmic V2- maxilary V3-mandibular
  • 56. PIAL ANGIOMATOSIS,MEDULLARY COLL & CEREBRAL ATROPHY
  • 57.
  • 58.
  • 59. Case II 5 yr female Complaints of focal seizure involving right side of body ,impaired milestone , right sided weakness Clinically- portwine strain + , Buphthalmus left MR imaging…..
  • 60.
  • 61.
  • 62. Gyriform (linear, convoluted, or serpentine) calcifications infarction, glioma, purulent meningitis, leukemia (following intrathecal administration of methotrexateand skull irradiation), ossifying meningoencephalopathy, and subarachoid fat
  • 63.
  • 65.  Dyke-Davidoff-Masson syndrome (DDMS) was initially described as  changes in the skull seen on skull X-ray in patients with cerebral  hemiatrophy, but is now applied more broadly to cross-sectional imaging  also. It is characterised by : •thickening of the skull vault (compensatory) •enlargement of the frontal sinus (also ethmoidal and mastoid air-cells) •elevation of the petrous ridge •ipsilateral falcine displacement In some sources it is equated to hemispheric infarction, whereas in other  sources any cause ofcerebral hemiatrophy are included.    Etymology Initially described by C.G Dyke , L.M Davidoff and C.B Masson in  1933 5 Differential diagnosis General considerations include hemimegalencephaly : Sturge-Weber syndrome : can also be an association Rasmussen encephalitis : tends not to have calvarial changes
  • 66.
  • 67.
  • 68. Tuberous sclerosis AD; 50% from new spontaneous mutations 1 in 20,000 to 1 in 50,000 nearly 40% of patients die by the age of 35 years prominent cutaneous, visceral, and CNS manifestations Most lesions are hamartomas
  • 69. DIAGNOSTIC CRITERIA FOR TSC Definite TSC Two major features, or one major plus two minor features. Probable TSC One major plus one minor feature. Possible TSC one major feature, or two or more minor features.
  • 70. DIAGNOSTIC CRITERIA FOR TSC MAJOR FEATURES Hypomelanotic macules (three or more), Shagreen patch Facial angiofibromas (adenoma sebaceum) or ungual or periungual fibromas Multiple retinal nodular hamartomas, Cortical tubers, Subependymal nodule, Subependymal giant cell astrocytoma Cardiac rhabdomyoma - single or multiple Lymphangiomyomatosis. Renal angiomyolipoma
  • 71. DIAGNOSTIC CRITERIA FOR TSC MINOR FEATURES Multiple pits in dental enamel Gingival fibromas Hamartomatous rectal polyps Bone cysts Cerebral white matter radial migration lines Retinal achromic patch Multiple renal cysts Nonrenal hamartoma "Confetti" skin lesions
  • 72.
  • 75.
  • 76.
  • 77.
  • 78.
  • 79.
  • 80. Cortical tubers: considered to be closely related to neurologic manifestations of TS - epilepsy, cognitive disability, and neurolobehavioral abnormalities 50% seen in frontal lobe Hypointense on T1-WI Hyperintense on T2-WI, FLAIR Only 10 % enhance
  • 81.
  • 82.
  • 83.
  • 84. Subependymal Nodules represent hamartomatous change. seen in 98% calcification detected in CT (88%) hyperintense on T1WI Iso- to hyp0intense on T2WI
  • 85.
  • 86. SGCAs proliferative astrocytes and giant cells. 1.7%–26% prevalence Typically in foramen of Monro Differ from other cerebral astrocytomas in having a benign biologic and pathologic features (slow growth, minimal or no attendant brain edema, and minimal invasiveness) tend to be larger tumors (>1 cm) with incomplete calcifn & more intense enhancement MRS shows high Cho/Cr and low NAA/Cr r
  • 87.
  • 88. White Matter Abnormalities Superficial white matter abnormalities associated with cortical tubers,  Radial white matter bands (15%–27%) Cystlike white matter lesions(15%–44%)
  • 89.
  • 90. PULMONARY AND THORACIC INVOLVEMENT lymphangioleiomyomatosis (LAM) multifocal micronodular pneumocyte hyperplasia (MMPH). approx 1%–2.3% of TS patients. complications of LAM Pneumothorax and chylous pleural effusion ascites.
  • 91. round, thin-walled cysts of variable size and contour At thin-section CT, multiple tiny nodules (1–8 mm  in diameter) are diffusely scattered throughout the  lung in a random distribution 
  • 92. RENAL AND RETROPERITONEAL INVOLVEMENT Renal angiomyolipoma (AML), renal cysts, and RCC Renal AML in 55%–75% patients with TS Retroperitoneal LAM in up to 20% of patients with pulmonary LAM.
  • 93. AMLs MC benign tumors of the kidney. characterized by variable amounts of abnormal vessels, immature smooth-muscle and fat cells Compared with sporadic lesions, AMLs seen in patients with TS tend to manifest at a younger age multiple, larger, and bilateral and Tend to grow. noncalcified cortical tumors containing fat of less than −20 HU
  • 94.
  • 95.
  • 96. Retroperitoneal LAM thick- or thin-walled cystic lesions. may reflect dilatation of lymph vessels due to obstruction
  • 97. SKELETAL INVOLVEMENT Cyst like lesions, hyperostosis of the inner table of the calvaria , osteoblastic changes, periosteal new bone formation, and scoliosis.
  • 98. VON HIPPEL-LINDAU DISEASE AD disorder linked to defect on the short arm of chromosome 3p. Prevalence is approximately 1 in 40,000 to 1 in 50,000 people Causes of death - cerebellar hemangioblastoma and RCC Screening is important because the lesions in VHL disease are treatable
  • 99. Manifestations of VHL Disease according to Prevalence Cerebellar hemangioblastoma 44–72% Medullary hemangioblastoma 5% Spinal cord hemangioblastoma 13–59% Retinal hemangioblastoma 45–59% Renal cell carcinoma 24–45% Pheochromocytoma 0–60% Neuroendocrine tumor of the pancreas 5–17% Serous cystadenoma of the pancreas 12% Pancreatic cysts 50–91% Renal cysts 59–63% Papillary cystadenoma of epididymis 10–60 %.
  • 100. NATIONAL INSTITUTES OF HEALTH CLASSIFICATION Type I VHL without pheochromocytoma most common type Renal and pancreatic cysts, RCC Type II VHL with pheochromocytoma IIa - Islet cell tumors (no cysts) Iib - Renal/pancreatic disease (least common)
  • 101. DIAGNOSTIC CRITERIA More than one CNS hemangioblastoma, One CNS hemangioblastoma + visceral manifestations of VHL disease, Any manifestation and a known family history of VHL disease.
  • 102. Hemangioblastoma Hallmark of VHL Seen in 2/3 of patients 20-50 yrs of age Typically multiple MC in cerebellum Other::::medulla > pons, spinal cord, and supratentorially in optic N and cerebrum
  • 103.
  • 104.
  • 105. Retinal angiomas are actually hemangioblastomas (40-50%) asymptomatic or cause a blind spot. may hemorrhage and can cause retinal detachment Higher signal intensity than normal vitreous on non- enhanced T1WI
  • 106. Renal lesions Renal cysts in 59%–63% RCC in 24%–45% either multicentric and bilateral solid hypervascular masses or complex cystic masses Complex or solid lesions enhance on postcontrast T1- WI A hypointense pseudocapsule on T2-WI
  • 107.
  • 108. Pancreatic involvement simple pancreatic cysts (50%–91%) serous microcystic adenomas Pancreatic neuroendocrine tumors (5%–17%) Pancreatic lesions may be the only abdominal manifestation and may precede any other manifestation by several years
  • 109. Annual screening examinations of the abdomen, by ultrasound or CT, have been recommended for some patients with VHL
  • 110. Ataxia-telangiectasia AR disorder 1 in 20,000-100,000 Telangiectasias in skin (face) and eyes, cerebellar ataxia immunodeficiency syndromes, and recurrent infections and susceptibility to certain neoplastic processes
  • 111. MR FINDINGS Telangiectasia of pia mater and white matter Hypointense WM foci on T1- and T2-WI Diffuse symmetric increased T2 white matter signal Severe cerebellar atrophy
  • 112. Hypointense WM foci on T1- and T2-WI
  • 113.
  • 114. THE PHACE SYNDROME Posterior fossa malformations Hemangiomas Arterial anomalies Coarctation of the aorta , cardiac defects Eye abnormalities  Sometimes, an S is added making it PHACES, with the S standing for Sternal defects and/or Supraumbilical raphe.
  • 115.
  • 116. Large facial hemangiomas may be associated with a Dandy- Walker malformation, vascular anomalies (coarctation of aorta, aplasia or hypoplastic carotid arteries, aneurysmal carotid dilation, aberrant left subclavian artery), glaucoma, cataracts, microphthalmia, optic nerve hypoplasia, and ventral defects (sternal clefts) Facial hemangioma is typically ipsilateral to the aortic arch Female predominance Patients with large facial cutaneous (S1-S4) hemangiomas were especially at risk of CNS structural and cerebrovascular anomalies; S1 with ocular anomalies; and S3 with airway, ventral, and cardiac anomalies.
  • 117.
  • 118. Gorlin syndrome  Diagnostic criteria  A clinical diagnosis can be made using major and minor criteria.   To make the diagnosis, either two major or, one major and two minor  criteria must be met.  Major criteria( Classical triad )  basal cell cancers : > 2 or 1 under the age 20  odontogenic keratocysts (see case 1)  palmar pits : 3 or more  bilamellar calcification of the falx cerebri  rib anomalies : bifid rib fused, splayed  first degree relative with Gorlin syndrome  Minor criteria  macrocephaly  frontal bossing, cleft lip or hypertelorism  Sprengel deformity,  pectus excavatum or pectus carinatum, syndactyly  bridging of the sella turcica, hemivertebrae, flame shaped radiolucencies  ovarian fibroma, medulloblastoma
  • 120. Conclusion Phakomatoses are a diverse group of disorders Most common phakomatoses (excluding SWS) are AD; therefore, a correct diagnosis has genetic implications A screening evaluation of all first-degree relatives to see if they are also affected is mandatory A routine follow-up surveillance program should be established. This typically includes annual CNS imaging studies and, where appropriate, abdominal ultrasound, CT, or MR.