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Scoliosis and Devices Instrumentation
KEY FACTS
TERMINOLOGY
• Spinal fusion surgery recommended when curve magnitude > 40-45° for adolescent idiopathic scoliosis
• Adult scoliosis presents with lumbar back ± leg pain, L3-L4 rotatory subluxation, L4-L5 tilt, and L5-S1 disc degeneration on radiographs
IMAGING
• Radiographs
Devices and Instrumentation
○ Main thoracic, thoracolumbar, and lumbar curves should
be assessed for structural characteristics
○ 36" standing anteroposterior & lateral radiographs &
supine side-bending radiographs
○ In adult scoliosis, assess for degenerated changes and
rotatory ± lateral listhesis
• CT ○ Assess integrity of hardware ○ Look for osseous bridging at levels of interbody fusion
and lucency along screw tracks
(Left) Anteroposterior radiograph shows sigmoid scoliosis of the thoracic and lumbar spines st. Fusion is extended to L5 if there is fixed tilt or subluxation at L4-L5, or to the sacrum if L5-S1 central or foraminal decompression is needed. (Right) Anteroposterior radiograph depicts posterior fusion from the thoracolumbar junction st to the sacrum ﬉. Extension of the fusion to the sacrum increases the incidence of pseudarthrosis and reoperation.
• MR ○ Preoperative planning to evaluate for central &/or
foraminal stenosis and disc degeneration
CLINICAL ISSUES
• Adult bones tend to be weaker or osteoporotic, making instrumentation and fusion more difficult
• Degenerative disc changes, spinal stenosis, and facet arthropathy can be exacerbated and in turn exacerbate scoliosis, leading to more rigid spines
• Goals: Prevent progression, restore acceptability of clinical deformity, reduce curvature, prevent neurologic deficit ○ Resolve pain ± make it more controllable with
medications
○ Fuse spine in as normal anatomical position as possible
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(Left) AP and lateral radiographs show fusion from the thoracic spine st to the L5 level ſt. Fusion is not terminated next to a severely degenerated segment. Proximal extension of the fusion should not stop distal to a proximal thoracic curve. Cross-links ﬊ improve torsional stiffness. (Right) Lateral radiograph shows posterior fusion st and L3 pedicle subtraction osteotomy ſt to restore lumbar lordosis. Lumbar scoliosis results in loss of lumbar lordosis with positive sagittal balance.
Scoliosis and Devices Instrumentation

TERMINOLOGY

Abbreviations
• Scoliosis instrumentation (SI)
Synonyms
• Scoliosis surgery, long spinal fusion surgery (> 4 vertebral segments)
Definitions
• Spinal fusion surgery recommended when curve magnitude > 40-45° for adolescent idiopathic scoliosis (AIS)
• Adult scoliosis patient presents with lumbar back ± leg pain, L3-L4 rotatory subluxation, L4-L5 tilt, and L5-S1 disc degeneration on radiographs ○ Goals: Prevent progression, restore acceptability of
clinical deformity, reduce curvature, correct positive sagittal malalignment, prevent neurologic deficit and pain reduction

IMAGING

Radiographic Findings
• Main thoracic, thoracolumbar, and lumbar curves should be assessed for structural characteristics ○ 36" standing AP & lateral radiographs and supine side-
bending radiographs
○ ± 36" supine lateral or hyperextension lateral radiograph
over bolster – Supine evaluation is helpful to determine curve
flexibility
is defined by Cobb angle 25° on side-bending radiographs
○ Overall flexibility is important to note → indicates
expected curve correction
○ Coronal & sagittal balance, including center sacral vertical
line and C7 plumbline
○ Shoulder height, apical vertebral translation of thoracic
and lumbar curves, and relative curve magnitudes
○ In adult scoliosis, assess for degenerated changes and
rotatory &/or lateral listhesis
CT Findings
• Look for osseous bridging at levels of interbody fusion and lucency along screw tracks
• Assess integrity of instrumentation
• May be helpful in delineating congenital osseous abnormalities (such as hemivertebrae or fused vertebrae)
• 3D reformations may be helpful for preoperative planning and postoperative evaluation
MR Findings
• Preoperative planning to evaluate for central &/or foraminal stenosis and disc degeneration

DIFFERENTIAL DIAGNOSIS

Plates and Screws
• Instrumentation in fusion surgery used to stabilize bony elements
Devices and Instrumentation
Flat Back Syndrome
• Loss of lumbar lordosis after lumbar or scoliosis distraction instrumentation, vertebral fracture, ankylosing spondylitis, degenerative disease

CLINICAL ISSUES

Natural History & Prognosis
Complications ○ Surgical: Severe blood loss, UTI, pancreatitis, obstructive
bowel dysfunction due to bowel immobilization, neurological damage – Retroperitoneal approach: Abdominal visceral, great
vessel, and superior hypogastric nerve plexus injury
– Transthoracic approach: Injury to great vessels,
pulmonary complications, chylothorax, and post-
thoracotomy pain syndrome ○ Migration of graft, implant breakage ○ Penetration of implants into canal or dorsally into
cutaneous tissues
○ Compression of nerve roots by implant components
• Pseudoarthrosis ○ Can occur years after surgery ○ Most likely to occur at thoracolumbar junction ○ Reported in 15-27% of cases
• Irreversible loss of normal active range of movement of spinal column, including nonfused segments
• Strain on unfused skeletal framework ○ Postsurgical degenerative changes within 2 years ○ Higher degree of correction results in higher rate of
degenerative osteoarthritis
• Curvature progression: Crank shaft phenomenon described in children: Spinal growth causes rotation around fusion
• Pain at iliac graft site, rib resection site
• Infections reported in 5-10% of patients at 11-45 months after surgery
• Venous thromboembolism (pulmonary embolism and deep vein thrombosis)
Treatment
• Goal of surgery in adult scoliosis ○ Resolve pain ± make it more controllable with
medications ○ Fuse spine in as normal anatomical position as possible ○ Biggest operative decision is to determine proximal and
distal extent of instrumentation and fusion
– Operative levels include fusion of Cobb angle, typically
L3 or L4, and extending to L5 when there is lateral or rotatory listhesis present
– Restoration of sagittal balance reported to be primary
parameter associated with outcome
• SI recommended when magnitude of curvature exceeds 40-45° in AIS ○ Approaches: Posterior instrumentation & fusion alone,
anterior instrumentation & fusion alone, & anterior release (to restore spinal flexibility) combined with posterior fusion, posterior only
○ Fusion often extended to sacrum if
– L5-S1 spondylolisthesis, or prior laminectomy – L5-S1 stenosis – Severe L5-S1 disc degeneration
81
Pumps and Catheters
KEY FACTS
TERMINOLOGY
• Intrathecal baclofen (ITB)
• Intraspinal drug delivery (IDD)
• Malfunction or malposition of pump or catheter delivery system
PATHOLOGY
• ITB used for treatment of spasticity ○ Typically used in children with severe quadriplegic
Devices and Instrumentation
pattern cerebral palsy
• IDD system therapy widely utilized in patients with intractable, nonmalignant, and malignant pain ○ Epidural analgesia trial may be conducted to document
efficacy prior to implantation of permanent intrathecal drug delivery pump
CLINICAL ISSUES
ITB rate of complications varies widely, range: 8-30%
(Left) This is a syringopleural
shunt failure due to formation of a pseudocyst at the catheter site in the chest. Initial T2W MR study after placement of the shunt shows postoperative change ſt with a small epidural fluid collection in the midthoracic spine and syrinx in the upper thoracic cord ﬇. (Right) Sagittal T1WI MR in the same patient 1 year later now shows marked expansion of the syrinx throughout the thoracic cord ſt.
Baclofen complications relate to withdrawal and
overdose ○ Infection rates: 8-10%Hardware-related complications from 5-20% ○ Majority of complications requiring reoperation involve
the catheter
IDD systems ○ Granuloma formation at catheter tip relatively common ○ Effects include loss of analgesia or new/progressive
neurologic symptoms
DIAGNOSTIC CHECKLIST
• Malfunction must be evaluated from pump level to distal aspect of catheter ○ CT abdomen following baclofen side port contrast
injection extending cephalad beyond intradural catheter tip
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(Left) AP chest film in the same patient shows a loculated pleural collection at the site of the coiled shunt catheter ﬇. (Right) This is a syringopleural shunt failure with expansion of the extensive cord syrinx due to formation of a pseudocyst at the catheter site in the chest. Axial T1 MR in the same patient shows the large thoracic cord syrinx ſt and a large fluid collection in the left chest at the site of catheter placement ﬇.
Pumps and Catheters

TERMINOLOGY

Abbreviations
• Intrathecal baclofen (ITB)
• Intraspinal drug delivery (IDD)
Definitions
• Malfunction or malposition of pump or catheter delivery system
Devices and Instrumentation
– Multiple agents, including morphine, may cause
formation □ Dose escalation and increased dosage increase risk
of formation
– Effects include loss of analgesia or new/progressive
neurologic symptoms
• Other signs/symptoms ○ Pump malfunction may present with spinal/abdominal
fluid collection/effusion

IMAGING

General Features
• Best diagnostic clue ○ Abnormal contrast accumulation after pump side port
injection
• Location ○ Anywhere along course of pump and catheter

DIFFERENTIAL DIAGNOSIS

Intrathecal or Epidural Catheter Granuloma
• May mimic epidural neoplasm or abscess

PATHOLOGY

General Features
• Etiology ○ ITB used for treatment of spasticity
– Decrease level of spasticity to improve range of
motion, facilitate movement, reduce contractures, and improve quality of life
– Typically used in children with severe quadriplegic
pattern cerebral palsy
○ IDD system therapy widely utilized in patients with
intractable, nonmalignant, and malignant pain – Epidural or intrathecal – Epidural analgesia trial may be conducted to
document efficacy prior to implantation of permanent intrathecal drug delivery pump

CLINICAL ISSUES

Presentation
• Most common signs/symptoms ○ ITB rate of complications varies widely, range: 8-30%Related to (a) baclofen, (b) infection, (c) hardwareBaclofen complications relate to withdrawal and
overdose
Infection rates: 8-10%
– Common reason for infection is skin breakdown or
dehiscence over pump
– Subfascial implantation provides greater soft tissue
coverage over pump
Hardware-related complications from 5-20%
– Majority of complications requiring reoperation
involve catheter – CSF leak from site of catheter penetration of dura – Catheter fracture or disconnection – Low rate of pump failure
IDD systems
– Granuloma formation at catheter tip relatively
common

DIAGNOSTIC CHECKLIST

Consider
• Malfunction must be evaluated from pump level to distal aspect of catheter ○ CT abdomen following baclofen side port contrast
injection extending cephalad beyond intradural catheter tip

SELECTED REFERENCES

1. Haranhalli N et al: Intrathecal baclofen therapy: complication avoidance and
management. Childs Nerv Syst. 27(3):421-7, 2011
2. Lawson EF et al: Current developments in intraspinal agents for cancer and
noncancer pain. Curr Pain Headache Rep. 14(1):8-16, 2010
3. Maugans TA: Intracranial migration of a fractured intrathecal catheter from a
baclofen pump system: case report and analysis of possible causes. Neurosurgery. 66(2):319-22, 2010
4. Myers J et al: Intraspinal techniques for pain management in cancer patients:
a systematic review. Support Care Cancer. 18(2):137-49, 2010
5. Stetkarova I et al: Procedure- and device-related complications of intrathecal
baclofen administration for management of adult muscle hypertonia: a review. Neurorehabil Neural Repair. 24(7):609-19, 2010
6. Fukuhara T et al: Tangled catheter as a rare cause of baclofen pump
malfunction. Surg Neurol. 72(1):80-2; discussion 82, 2009
7. van Rijn MA et al: Intrathecal baclofen for dystonia of complex regional pain
syndrome. Pain. 143(1-2):41-7, 2009
8. Borowski A et al: Baclofen pump implantation and spinal fusion in children:
techniques and complications. Spine (Phila Pa 1976). 33(18):1995-2000, 2008
9. Caird MS et al: Outcomes of posterior spinal fusion and instrumentation in
patients with continuous intrathecal baclofen infusion pumps. Spine (Phila Pa 1976). 33(4):E94-9, 2008
10. Ruan X et al: Edema caused by continuous epidural hydromorphone
infusion: a case report and review of the literature. J Opioid Manag. 4(4):255­9, 2008
11. Kallweit U et al: Successful treatment of methicillin-resistant Staphylococcus
aureus meningitis using linezolid without removal of intrathecal infusion pump. Case report. J Neurosurg. 107(3):651-3, 2007
12. Markman JD et al: Interventional approaches to pain management. Med Clin
North Am. 91(2):271-86, 2007
13. Motta F et al: The use of intrathecal baclofen pump implants in children and
adolescents: safety and complications in 200 consecutive cases. J Neurosurg. 107(1 Suppl):32-5, 2007
14. Narouze SN et al: Erosion of the inferior epigastric artery: a rare complication
of intrathecal drug delivery systems. Pain Med. 8(5):468-70, 2007
15. Vender JR et al: Identification and management of intrathecal baclofen
pump complications: a comparison of pediatric and adult patients. J Neurosurg. 104(1 Suppl):9-15, 2006
16. Amar AP et al: Percutaneous spinal interventions. Neurosurg Clin N Am.
16(3):561-8, vii, 2005
17. Hassenbusch SJ et al: Polyanalgesic Consensus Conference 2003: an update
on the management of pain by intraspinal drug delivery-- report of an expert panel. J Pain Symptom Manage. 27(6):540-63, 2004
18. Gooch JL et al: Complications of intrathecal baclofen pumps in children.
Pediatr Neurosurg. 39(1):1-6, 2003
19. McMillan MR et al: Catheter-associated masses in patients receiving
intrathecal analgesic therapy. Anesth Analg. 96(1):186-90, table of contents, 2003
83
SECTION 3
Congenital and Genetic Disorders

Bony Variations

C2-C3 Fusion 86 C1 Assimilation Ponticulus Posticus Ossiculum Terminale Paracondylar Process Condylus Tertius 91 Posterior Arch Rachischisis Split Atlas
87 88 89 90
92 93
Diagnoses
Odontoid Hypoplasia/Aplasia 94 C1 Dysmorphism/Hypoplastic Arch Chiari 1 Complex Chiari Chiari 2 Chiari 3 99 Myelomeningocele Lipomyelomeningocele Lipoma Dorsal Dermal Sinus Simple Coccygeal Dimple 104 Dermoid Cysts Epidermoid Cysts Tethered Spinal Cord Segmental Spinal Dysgenesis Caudal Regression Syndrome 109 Terminal Myelocystocele Anterior Sacral Meningocele Occult Intrasacral Meningocele Sacrococcygeal Teratoma Klippel-Feil Spectrum 114 Failure of Vertebral Formation
95 96 97 98
100 101 102 103
105 106 107 108
110 111 112 113
115
Vertebral Segmentation Failure 116 Diastematomyelia Neurenteric Cyst Os Odontoideum Lateral Meningocele
117 118 119 120
Genetic
Neurobromatosis Type 1 122 Neurobromatosis Type 2
Down Syndrome Mucopolysaccharidoses Achondroplasia Osteogenesis Imperfecta 133 Spondyloepiphyseal Dysplasia
126 130 131 132
134
C2-C3 Fusion
KEY FACTS
TERMINOLOGY
• Congenital C2-C3 vertebral segmentation failure
IMAGING
• Rudimentary C2-C3 intervertebral disc space with narrow "waist" ○ C2-C3 disc space smaller than normal disc spaces in
remainder of cervical spine
○ ± facet fusion
• Chiari 1 malformation may be present
• Spinal cord compression, syringomyelia unusual
Congenital and Genetic Disorders
TOP DIFFERENTIAL DIAGNOSES
• Juvenile inflammatory arthritis
• Surgical fusion
• Chronic sequelae of discitis
PATHOLOGY
• Congenital cervical fusion
(Left) Sagittal T1WI MR of the craniovertebral junction demonstrates the characteristic rudimentary C2­C3 disc space ſt with normal vertebral body marrow and intervertebral disc signal. The fused C2 and C3 spinous processes ﬇ are relatively inapparent on MR. (Right) Sagittal bone CT of the craniovertebral junction depicts incomplete vertebral segmentation at C2-C3 ſt, with characteristic rudimentary intervertebral disc space (narrow "waist") and fusion of the spinous processes ﬇.
○ Secondary to failure of normal cervical somite
segmentation (3rd → 8th weeks)
• C2-C3 fusion (type II Klippel-Feil spectrum) → autosomal dominant with variable penetrance
CLINICAL ISSUES
• Usually asymptomatic ○ Identified incidentally during spinal imaging for other
reasons
• Predisposition for accelerated degenerative changes below fused level
DIAGNOSTIC CHECKLIST
• C2-C3 segmentation failure is usually incidental finding discovered while imaging for other reasons
• Less often it is associated with multiple vertebral segmentation anomalies (Klippel-Feil spectrum)
86
(Left) Coronal bone CT shows congenital fusion of C2-C3 with a small and rudimentary disc margin ſt. Note the normal-sized discs at the lower levels. There is also assimilation of C1 into the occiput ﬇. (Right) Coronal bone CT of the craniovertebral junction in a child shows asymmetric, incomplete C2-C3 segmentation ſt that results in mild upper cervical curvature convex to the right. The atlantooccipital and atlantoaxial articulations are normal.
C1 Assimilation
KEY FACTS
Congenital and Genetic Disorders
TERMINOLOGY
• Synonyms: Atlantooccipital assimilation, atlantal assimilation, "occipitalization"
• Nonresegmentation of proatlas sclerotome → failure of C1 to correctly segment from occipital bone
IMAGING
• Partial or complete atlas assimilation into occipital bone ○ ± C1 ring hypoplasia → canal stenosis ○ ± posterior C2 arch hypertrophy → canal stenosis
• ± posterior fossa, spinal cord anomalies
TOP DIFFERENTIAL DIAGNOSES
• Juvenile idiopathic arthritis
• Craniovertebral junction surgical fusion
PATHOLOGY
• Inappropriate PAX1 repression (humans) probably contributory, not sole mechanism
• Associated anomalies include ○ Atlantoaxial instability (up to 60%) ○ C2/3 segmentation failure ○ Klippel-Feil syndrome ○ Basilar invagination ○ Chiari 1 malformation
CLINICAL ISSUES
• May remain asymptomatic throughout life
• Symptomatic patients report neck pain, stiffness, myelopathy ○ Neurological deficits usually begin in 3rd-4th decade,
worsen with age
DIAGNOSTIC CHECKLIST
• Clinical symptoms related to presence of atlantoaxial instability, cervical stenosis, and severity of associated anomalies
(Left) Coronal bone CT confirms segmentation failure of the occipital condyles ſt and C1 lateral masses ﬇ (zone 2 assimilation). Formation of the proatlas is asymmetric, producing a mild head tilt to the right. (Right) Sagittal bone CT obtained in the midline shows normal alignment of clivus and odontoid tip. The anterior C1 ring ﬇ is in normal position, but the posterior C1 ring st is fused to the foramen magnum opisthion (zone 3 assimilation).
(Left) Sagittal midline bone CT shows no posterior C1 arch due to C1 assimilation to occiput. The odontoid is upwardly placed (basilar invagination) ſt, and the atlantodental interval is widened ﬇. There is a rudimentary disc at C2-C3 st. (Right) Sagittal T1WI MR shows basilar invagination with mass effect on the cervicomedullary junction ſt. There is a Chiari 1 malformation ﬇ and cervical cord syrinx st as well as widening of the atlantodental interval .
87
Ponticulus Posticus
KEY FACTS
TERMINOLOGY
• Latin: "Little posterior bridge"
• Synonyms: Arcuate foramen, sagittale foramen, canalis vertebralis, retroarticular canal, retroarticular/retrocondylar vertebral artery ring, upper retroarticular foramen, and atlantal posterior foramen
IMAGING
• Osseous roof along superior C1 arch covers C1 vertebral artery foramen ○ Vertebral artery passes through osseous tunnel
• May be partial or complete
Congenital and Genetic Disorders
• Unilateral or bilateral
TOP DIFFERENTIAL DIAGNOSES
• Broad C1 posterior arch ○ Mistaking ponticulus posticus for broad posterior C1 arch
→ vertebral artery injury during C1 lateral mass screw placement
(Left) Lateral radiograph of the upper cervical spine demonstrates a partial osseous roof ſt over the C1 vertebral artery foramen, characteristic of ponticulus posticus (incomplete variant). (Right) Lateral radiograph of the upper cervical spine reveals a complete osseous roof ﬈ over the C1 vertebral artery foramen, typical of classic ponticulus posticus (complete variant).
PATHOLOGY
• Postulated to arise from ossification of lateral segment of posterior atlantooccipital ligament or joint capsule
• Morphologically normal bone
CLINICAL ISSUES
• Most common: Asymptomatic
• Other symptoms ○ Vertebrobasilar ischemia or infarction ○ Vertigo ○ Headache ○ Neck pain
DIAGNOSTIC CHECKLIST
• Vertebral arteries predisposed to surgical injury during lateral mass screw placement
• Multiplanar bone CT with 3D reformats best demonstrate ponticulus posticus
88
(Left) Sagittal CTA depicts a complete C1 arch with a robust osseous covering (complete ponticulus posticus) ﬈ over the vertebral artery ﬊ along the superior aspect of the C1 arch. (Right) Anteroposterior view of a CTA 3D reformat shows a dominant left vertebral artery ﬇ and smaller (nondominant) right vertebral artery ﬊. Both are covered by a complete osseous bridge along the superior aspect of the C1 arch.
Ossiculum Terminale
KEY FACTS
Congenital and Genetic Disorders
TERMINOLOGY
• Synonym: Ossiculum terminale persistens
• Persistence of unfused odontoid tip ossification center into adulthood
IMAGING
• Separate ossicle with intact cortical margin positioned above normal-sized odontoid process in adolescent or adult ○ May be dystopic or orthotopic
• Soft tissue pannus suggests atlantoaxial instability
• Myelomalacia, brainstem compression in rare cases
TOP DIFFERENTIAL DIAGNOSES
• Os odontoideum
• Type I or II odontoid fracture
• Normal unfused odontoid tip synchondrosis
• Degenerative remodeling of odontoid process
PATHOLOGY
• Terminal ossicle normally appears by age 3, fuses with odontoid body by age 12
• Terminal ossicle located above transverse ligament ○ Atlantoaxial instability less common than with os
odontoideum
○ Unstable ossiculum terminale usually dystopic
CLINICAL ISSUES
• Usually asymptomatic
• Uncommonly, neck pain or myelopathy related to CVJ instability
DIAGNOSTIC CHECKLIST
• Consider ossiculum terminale in patients > 12 years with persistent terminal ossicle at odontoid tip
• Evaluate for atlantoaxial instability if dystopic ossicle, excessive soft tissue pannus, or trisomy 21 patient
(Left) Sagittal bone CT demonstrates a small ossiculum terminale ſt located adjacent to the odontoid tip. The ossicle is in orthotopic position, and CVJ alignment is normal. (Right) Coronal bone CT confirms orthotopic placement of a small orthotopic ossiculum terminale ſt. Note that there is minimal flattening of the odontoid tip adjacent to the ossicle, supporting classification as a persistent terminal odontoid ossicle.
(Left) Sagittal bone CT reveals a small dystopic ossiculum terminale ſt positioned between the odontoid tip and clivus. The odontoid tip has remodeled. (Right) Sagittal bone CT shows subluxation of the anterior C1 ring ﬇ relative to the nearly normal­sized odontoid process st. There is a large dystopic ossiculum terminale ſt. Although debatable, the nearly normal size of the odontoid process favors classification as an ossiculum terminale rather than os odontoideum.
89