Soft tissue lesions involving cranio-vertebral junction

1. Soft tissue lesions involving
cranio-vertebral junction
Dr Amit Agrawal
Professor, Department of Neurosurgery
Narayana Medical College Hospital
Chinthareddypalem, Nellore-524003
Andhra Pradesh (India)

2.  The craniovertebral junction (CVJ) is a transition zone between
cranium and spinal column and collectively includes the
occiput (posterior skull base), atlas, axis, and supporting
ligaments
 Surrounding soft tissue structures can give rise to spectrum of
complex pathologies ranging from infection to tumors
Introduction

3. Infections
• Tuberculosis
• Pyogenic Osteomyelitis
• Grisel Syndrome
Arthropathies
• Rheumatoid Arthritis
Congenital (uncommonly acquired)
• Chiari Malformation
Tumors
• Chondroma
• Chondrosarcoma
• Chordoma
• Eosinophilic granuloma
• Extension of local malignancy
• Fibrous dysplasia
• Glomus tumor
• Meningioma
• Metastases
• Nasopharyngeal malignancy
• Neurofibroma
• Osteoblastoma
• Giant cell tumor
• Osteoid osteoma
• Plasmacytoma
• Rhabdomyosarcoma
Soft tissue pathologies involving the
CVJ
{Colli, 2001 #3;George, 1997 #4;Menezes, 2008 #58;Das, 2017 #10} {Shenoy, 2004
#7}{Vhora, 2014 #8} {Singh, 2010 #14} {Rousselin, 1990 #12}

4.  Irrespective of the underlying pathology involvement
of CV junction is characterized by
 Neck pain
 Symptoms due to involvement of neural and vascular
structures
 Spinal instability
 Alteration of CSF flow dynamics
Clinical features
{Menezes, 2004 #55;Rhoton, 2000 #61;Meyer, 1984 #22}

5.  Neck pain (with headaches)
 Dermatomal distribution (especially when C2 nerve root is involved)
 Headache can be a symptom of associated hydrocephalus
 Involvement of the neural structures (the pons, medulla, rostral
cervical region, cranial nerves, cerebellar symptoms or spinal cord
deficits)
 Sensory or motor disturbances
 Gait disturbances
 Impaired proprioception, coordination
 Respiratory compromise
 Involvement of the vascular structures
 Vertebrobasilar ischemia (intermittent syncope, drop attacks, vertigo, confusion or
altered consciousness, waxing-waning weakness and visual disturbance)
 Instability
 Can lead to neck pain and repeated injuries to neural as well as vascular structures
 Syringomyelia (with hydrocephalus)
 Due to alterations of cerebrospinal fluid (CSF) circulation
Clinical features

6.  Traditionally radiographs (Antero-posterior view, lateral view, open
mouth view for dens, stress X-Rays: neutral, flexion, extension)
 Any bone destruction or paravertebral shadow
 CT scan and MRI (with or without contrast)
 CT scan will delineate the bony details and help in planning for spinal
instrumentation
 Magnetic resonance imaging (MRI)
 Investigation of choice
 Shall visualize the neural as well as soft tissue structures
 Details of spinal cord compression
 CT scan (with 3D reconstruction) will further help to assess the
extent of bony involvement
 Selected cases may need cerebral angiography (CTA, MRA or DSA)
Investigations

7.  In the background of clinical presentation, a careful evaluation
and interpretation of imaging findings shall help to make a
diagnosis
Important!

8.  Restoration of normal functioning
Management: Objective

9.  A multidisciplinary and multi-modal approach
 Need experience and thorough understating of
anatomy and pathologies of CV junction*
 The standard approach to the CVJ junction is midline
suboccipital craniotomy with high cervical
laminectomy
 Other approaches **
 Anterior transoral
 Endonasal transclival
 Lateral transcervical
 Posterolateral suboccipital approaches
Management
*{Visocchi, 2017 #75;Morales-Valero, 2015 #76}
**{Menezes, 1994 #1;Menezes, 2004 #55;Menezes, 1996 #20}

10.  Chiari malformation is a structural defect which involves
cerebellum and characterized downward displacement of
cerebellar tonsils
 Usually congenital rarely acquired
 Based on the structures involved it can be of following types:
Type I to Type IV
 “Chiari type 0- and Chiari type 1.5*
Arnold-Chiari Malformation
{Sarnat, 2007 #162;Woodward, 2016 #160}{Milhorat, 2010 #159;Galarza, 2010
#157;Mutchnick, 2010 #158}
* {Tubbs, 2007 #154}

11.  Clinical features of the Chiari malformation vary according to
the severity, age at presentation and presence of
hydrocephalus/syringomyelia
Arnold-Chiari Malformation: Clinical
features

12.  Asymptomatic patients with non-progressive symptoms can
be managed conservatively and can be followed up clinically or
with imaging (MRI) at regular intervals
 The objective of surgery is to improve the symptoms and halt
the progression of the disease
 The core principal is to establish CSF circulation pathway and
to relieve the pressure on brain (hydrocephalus) and spinal
cord (syringomyelia)
 Posterior fossa decompression (Foramen magnum
decompression) preferably with duroplasty and C1 arch
excision (if necessary)
Arnold-Chiari Malformation:
Management

13.  Characterized by cyst (syrinx) formation which is filled with
cerebrospinal fluid and lined by gliotic-tissue
 Exact pathogenesis of syrinx formation is not known
 Involves the lower cervical and upper thoracic spinal cord
 May be holocord, can extend into the medulla (Syringobulbia)
 Several theories
 Gardner's hydrodynamic theory
 William's theory
 Oldfield's theory
 Intramedullary pulse pressure theory
Syringomyelia
{Milhorat, 2000 #147} {Brodbelt, 2003 #109} {Gardner, 1976 #136;Gardner, 1965 #137}
{Williams, 1970 #150;Williams, 1973 #151} {Levine, 2004 #153}

14.  Congenital
 Chiari malformation
 Neural tube defects (myelomeningocele and tethered cord syndrome)
 Klippel—Feil syndrome
 Acquired
 Spinal cord injury
 Arachnoiditis
 Inflammatory
 Infectious conditions (e.g. Tuberculosis)
 Neoplasms (Intramedullary as well as Extramedullary)
 Hemangioblastomas
 Ependymomas
 Astrocytomas
 Hydrocephalus leading to acquired Chiari-I malformation with resultant syringomyelia
 Vascular malformations
 Familial cases (Rare)
 Idiopathic (Usually benign and asymptomatic)
Syringomyelia: Etiology
{Busis, 1985 #145;Guille, 1995 #143;Srivatanakul, 2009 #102;Finsterer, 2000 #149;Roy,
2011 #129}

15.  Younger age (between 15 and 35 years)
 The symptom onset is gradual but progressive course
 Neurological manifestation depend on the extent of the syrinx
formation (localized, holocord or extending into the lower
brainstem)
Syringomyelia: Clinical Presentation

16.  Detailed clinical evaluation including a good clinical history,
physical and neurological examination
 Radiographs may help to identify the bony abnormalities
 Magnetic resonance imaging (MRI) of the CV junction (if
necessary spine and brain)
 Three-dimensional (3D) or constructive interference in steady
state (CISS) image sequences
 Electrocardiographically gated flow- sensitive techniques
 CT myelography where the MRI is not available or
contraindicated (i.e. metal implants, pacemaker etc.).
Syringomyelia: Investigations
{Steinbok, 2004 #108} {Bunck, 2011 #112;Chavhan, 2008 #110;Roser, 2008 #111}

17.  Small syrinx which is asymptomatic or present with non-
progressive relatively mild symptoms and patient is willing for
regular monitoring with MRI and for close follow up
 The principal of surgery are to address the underlying cause of
the syringomyelia and restoration of the normal CSF flow*
 Chiari I Malformation with syringomyelia: Posterior fossa
decompression with or without duroplasty and with or
without tonsillar resection/direct cyst drainage**
Syringomyelia: Management
*{Menezes, 2012 #122;Akhtar, 2008 #123;Fernández, 2009 #127;Roy, 2011 #129;Sharma,
2006 #124}
** {Attenello, 2008 #120;Aghakhani, 2009 #121}

18.  Degenerative
 Rheumatoid arthritis (up to 50% patients of RA)
 Amyloid arthopathy
 Juvenile rheumatoid arthritis
 Ankylosing spondylitis
Common Arthropathies
{Cone, 1981 #63;Suarez-Almazor, 1988 #2}

19.  An autoimmune disorder of unknown etiology that can involve
cervical spine as well*
 Pannus formation
 Chronic synovitis
 Destruction of ligaments, tendons, cartilage and bone
Atlantoaxial and occipitoatlantal hypermobility, instability and
subluxation**
Rheumatoid arthritis
* {Shen, 2004 #70;Wollowick, 2007 #69;Eduardo Carelli Teixeira da Silva, 2017 #73}
** {Shen, 2004 #70;Wollowick, 2007 #69;Eduardo Carelli Teixeira da Silva, 2017 #73;da
Côrte, 2014 #34;Morizono, 1987 #35}

20.  Chordomas*
 Chordomas arise from notochordal remnants
 Mainstay of treatment is surgical resection while restoring
the spinal stability
 CV junction (usually foramen magnum)**
 A surgical challenge
 2-3%
Tumors
*{McLoughlin, 2010 #64;Kalsi, 2012 #13} {Singh, 2010 #14;Menezes, 2014 #15}
** {Al-Mefty, 1997 #27;Bassiouni, 2006 #29;Menezes, 2008 #58;Meyer, 1984
#22;Passacantilli, 2005 #24}

21.  Metastasis
 Rare (0.5%)
 Breast (35%)
 Non-small cell lung cancer (13%)
 Prostate cancer (10%)
 In most of the cases at best only palliative treatment
Tumors
{Moulding, 2010 #62;Sherk, 1975 #16} {Tuchman, 2014 #71}

22.  CV junction is secondarily involved due to disease elsewhere in
the body and accounts <1% spinal tuberculosis
Tuberculosis

23.  Deep location of the lesions and proximity to vital
neural and vascular structures, the CV junction lesions
poses a unique management challenge for
neurosurgeons
 Approach to these lesions requires
 A comprehensive knowledge of CVJ anatomy
 A good understanding of clinical neurology
 Judicious use of imaging techniques and technical expertise
Conclusion

24.  Whether there is any spinal instability?
 Whether there is spinal compression?
 What is the mechanism and direction of the compression?
 Whether there is neurological deterioration?
 What may be the etiology (Infection, inflammatory pathology versus
benign lesions versus malignant lesions)?
 Whether there is any associated vascular or intramedullary
involvement (e.g., syringomyelia)
 Is there a need for pain control?
 Whether there shall be need to control systemic disease
(Tuberculosis, rheumatoid arthritis or malignancy)?
 What is the age of the patient and how is his general condition?
Checklist

25.  CV junction lesions do not present with a single symptom or
neurological finding that can be called as pathognomonic for a
particular lesion
 It is always a challenge for the clinician is to correctly
differentiate clinically significant pathologies from each other
 It is worth to repeat that “misdiagnosis or inadequate
approach can result is serious complications and may be life-
threatening as well”
Take Home Message!

26.  Available online sources and standard text books
References