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Congenital anomalies and Normal skeletal variants- Cervical spine

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Congenital anomalies and Normal skeletal variants- Cervical spine

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Congenital anomalies and Normal skeletal variants- Cervical spine

  1. 1. Congenital anomalies and Normal skeletal variants- Cervical spine Dr . M Sanal kumar Radiology resident
  2. 2. Platybasia (Flat skull base, Martin’s anomaly) •Flattening of the angle between the clivus and body of the sphenoid. •Platybasia may occur as an isolated congenital anomaly or • In conjunction with skeletal dysplasia - achondroplasia, osteogenesis imperfecta.. or
  3. 3. •Majority are asymptomatic. •Rarely, upward displacement secondary to degenerative changes of the upper cervical joints and ligaments may precipitate symptoms. • Neurological complications of the brainstem- infarction and syrinx formation; •Cerebellar dysfunction from infarction and gliosis; Chiari malformation
  4. 4. Radiologic Features •The skull base angle (Martin’s basilar angle) can be determined by measuring the angle subtended between lines from the nasion to sella turcica to the anterior foramen magnum. •The normal range is 123–152°. •Platybasia is designated when the angle is > 152°.
  5. 5. Martin’s basilar angle The three points are the nasion (frontal–nasal junction), the center of the sella turcica (mid-point between the clinoid processes), and the basion (anterior margin of the foramen magnum).
  6. 6. Basilar Invagination/ Basilar Impressio Basilar impression describes the condition of a relative cephalad position of the upper cervical vertebra to the base of the skull. No gender predilection. Majority are asymptomatic . Two types of basilar impression are described: primary and secondary. Primary Basilar Impression- congenital ,often
  7. 7. Secondary Basilar Impression- acquired condition , resulting from disease softening of the occipital bone in which the weight of the cranium deforms the base.(Paget’s disease, osteomalacia, fibrous dysplasia, and osteogenesis imperfecta.) Rheumatoid arthritis - most common of the arthritides to cause secondary basilar impression-bone and transverse ligament destruction. The onset of symptoms typically begins in the 3rd–
  8. 8. Chamberlain's Line Chamberlain's line is a line drawn on lateral skull radiograph between posterior end of hard palate to the posterior lip of the foramen magnum. If the tip of the dens is greater than 3mm above Chamberlain's line then basilar invagination is present.
  9. 9. McGregor's Line McGregor's line extends from the upper surface of the posterior edge of the hard palate to the most caudal point of the occipital curve. If the tip of the odontoid process is more than 4.5mm above McGregor's line then basilar invagination is likely.
  10. 10. McRae's line McRae's line is defined by the opening of the foramen magnum. If the tip of the dens migrates above this line then impaction is present.
  11. 11. Boogard’s Line and Angle Boogard’s line -A line is drawn connecting the nasion to the opisthion. Boogard’s angle -(a) A line is drawn between the basion and opisthion (Macrae’s line) (b) A second line is drawn from the dorsum sellae to basion along the plane of clivus. (c) The angle between these two lines is measured. In basilar impression—the basion will be above Boogard’s line, and the angle will be > 135°.
  12. 12. Anomalies of the atlas
  13. 13. Occipitalization of the Atlas (Atlas assimilation, atlanto-occipital fusion, block atlas) •Failure of segmentation and separation of the most caudal occipital sclerotome during the first few weeks of fetal life. • It is the most cephalic block vertebra of the spine and the most common anomaly of the craniovertebral junction. •M: F- 5:1 ; Population incidence - 0.75–3%.
  14. 14. Clinical features •A short neck, low hair line, and restricted neck motion is found in more than two thirds of cases. •Nuchal and facet joint pain. •Premature and severe degenerative joint disease at C1–C2 and C2–C3 are common. •Risk for significant neurological dysfunction increases with age, usually beginning in the 3rd–
  15. 15. Radiological features •A spectrum of non-segmentation ranging from complete to incomplete forms. •Complete fusion -fusion of the anterior and posterior arches and bilateral atlanto-occipital joint fusion. •In virtually all cases the anterior arch will be fused to the anterior margin (basion) of the foramen magnum, and the transverse process of the atlas will be either absent or fused to the occiput.
  16. 16. •In 90% of cases, details of the fused atlas posterior arch can be discerned at the occiput on a plain film. •Up to 70% of occipitalizations will have an accompanying block vertebra of C2–C3. • Anomalies of the dens like agenesis, hypoplasia, os odontoideum, ossiculum terminale, and abnormal tilt are common- predispose the patient to instability. •Basilar impression is a common tandem finding and should be considered in the assessment
  17. 17. Occipital vertebrae The occipital bone is formed from the union of four to five somites, which normally fuse together to encircle the foramen magnum.  The last occipital somite, or pro- atlas somite, may fail to fully incorporate into the occiput, resulting in occipital or pro-atlas vertebrae.  Manifestations of occipital vertebrae include the third condyle, paramastoid process, epitransverse process, and various occipital ossicles.
  18. 18. •Third condyle is the most common form of an occipital vertebra. • An anterior midline bony process located between the two occipital condyles and continuous with the anterior foramen magnum extends a variable distance caudally. •It occasionally forms an articulation with the apex of the odontoid process or anterior arch of the atlas. Radiologic Features. Large third condyles may be seen on true lateral radiographs as oval or round bone densities equal in size or smaller than the anterior arch of the atlas.
  19. 19. Paracondylar, Paramastoid and Epitransverse Processes •These are variations of congenital bone bars that extend between the occiput and transverse processes of the atlas. • They may be unilateral or bilateral •The paramastoid process is a bony protuberance that originates from the jugular process of the occiput and projects inferiorly toward the atlas transverse process. •A paracondylar process arises slightly more
  20. 20. •An epitransverse process is attached to the atlas transverse process and directed superiorly toward the adjacent occiput. •Clinical Features •Symptoms due to the paracondylar process are unusual but may be found in conjunction with muscle contracture and pain. •A paramastoid process occurs in at least 20% of occipitalizations. Large processes may be palpable in asthenic individuals and may inhibit atlanto-occipital movements.
  21. 21. Radiologic Features •Frequently overlooked on both AP and lateral radiographs because of the superimposed anatomy. •A slightly rotated open-mouth view will shift the molars on the side opposite the direction of head rotation away from the area of interest. •The process is typically cone-shaped, broader at its occipital base, and narrower distally; it often curves medially. •An accessory joint may be present between the
  22. 22. Vertebralization of the Atlas (Regressive occipital vertebra, double atlas) •Vertebralization is an embryologic tendency for a part of the pro-atlas to not incorporate into the occiput; it is rare. • This results in duplication of all or part of the atlas vertebra at the C0–C1 interspace. •A complete double atlas is very rare . There may be an extra posterior arch, anterior arch, transverse processes, or lateral masses.
  23. 23. Radiologic Features •Combined fusion of the anterior atlas arch with the basion (occipitalization) and the posterior arch with the axis (vertebralization) has been recorded. • The condition may precipitate atlantoaxial instability with insufficiency of the transverse ligament. •The odontoid process is often elongated and may be malformed. Various degrees of bony fusion of the upper joint surfaces with the occiput is seen.
  24. 24. Agenesis of the Atlas Posterior Arch•Lack of ossification of the posterior arch of the atlas may be complete and bilateral, may be purely unilateral, or may manifest as small clefts (i.e., spina bifida). • Dense fibrous connective tissue remains at the site devoid of ossification. •Pain or neurological complications are rare. •Atlantoaxial instability has been described. • There is occasional association with C2–C4 block vertebrae and Klippel-Feil syndrome.
  25. 25. • It needs to be differentiated from occipitalization, osteolytic metastases, aneurysmal bone cyst, and osteoblastoma Radiologic Features •The lateral view is the best projection for identifying the various forms of aplasia. Bilateral Posterior Arch Agenesis-The characteristic triad of findings is absence of the atlas posterior arch, union of the posterior tubercle to the axis spinous process (axis megaspinous sign), and compensatory enlargement and sclerosis of the
  26. 26. •Unilateral Posterior Arch Agenesis (Hemi-Atlas) -Uncommon. The condition is best determined on the AP open mouth view and CT. •Isolated Clefts of the Posterior Arch-Isolated clefts of the posterior arch are most common in the midline posteriorly, accounting for 97% of arch clefts. These clefts range from 1 to 5 mm and are best seen on oblique and slightly off-lateral projections.
  27. 27. •Thin posterior arch- The width of the posterior arch is thin and attenuated maximally at the vertebral artery sulcus. An association with Turner’s syndrome and gonadal dysgenesis has been suggested. • Short posterior arch- The atlas posterior arch is thick and bulky, and the diameter of the spinal canal is diminished. A described tandem finding is a thick, bulky dens that may contribute to symptomatic •Hypoplasia of the Posterior Arch- Two forms of hypoplasia of the posterior arch are described: thin and short.
  28. 28. Accessory Atlantoaxial Joint (Cervical Baastrup’s Disease) •Enlargement of the posterior arch occasionally forms an accessory joint space with the adjacent axis spinous process. •The radiologic hallmarks are enlargement of the posterior tubercle, flat corticated opposing surfaces, and a smooth joint space. •Occasionally, signs of degeneration—including osteophytes, sclerosis, and a narrowed joint space— are seen.
  29. 29. Posterior Spina Bifida Occulta of the Atlas•Spina bifida occulta (SBO) is an embryologic failure in midline ossification of the vertebral neural arch. The gap is filled with fibrous tissue. •Seen in 1–5% of the population, and this site is the second most common region to be affected, after the lumbosacral region. •Can occur simultaneously with occipitalization and anterior arch spina bifida (bipartite atlas). •Axial compression trauma may result in fracture through the site of non-union.
  30. 30. Myelomeningoceles of the craniocervical junction have an associated atlas SBO in 70% of cases. Radiologic Features •On the lateral view there is absence of the spinolaminar junction line and the posterior arch is thin and attenuated, with club-shaped, tapered, or beaked ends. •Differentiation from fracture is based on location and the clubbed smoothly corticated opposing ends. •The anterior tubercle is often sclerotic and enlarged and is a useful sign for a congenital absence of posterior continuity.
  31. 31. Ponticles of the Atlas •Calcification or ossification along the margins of normally occurring foramina in the atlanto-occipital ligament is referred to as ponticles. •There are two types of atlas ponticles: posterior and lateral.
  32. 32. Posterior Ponticle •A posterior ponticle of the atlas is ossification or calcification of the oblique portion of the atlanto- occipital membrane that bridges the posterior lateral mass and the posterior arch. •It forms the peripheral border of the arcuate foramen, which transmits the vertebral artery and veins, the first cervical nerve, and the perivascular sympathetic nerves. •It is usually bilateral and asymmetrical and exhibits no gender dominance
  33. 33. Three clinical associations have been made: vertebrobasilar insufficiency, Barre-Lieou syndrome, and chronic upper cervical syndrome. •Vertebrobasilar Insufficiency- the adventitia of the vertebral artery is contiguous with the periosteum of the ponticle, and atlantoaxial movements may create traction or compression of the artery. Barre-Lieou Syndrome. Headaches; retrorbital pain; facial vasomotor disturbance; and visual, phonation, and swallowing difficulties. Chronic Upper Cervical Syndrome- The symptom
  34. 34. Radiologic Features. A posterior ponticle is best seen on a lateral radiograph of the cervical spine; it forms a partial or complete foramen at the ventral and superior aspect of the vertebral arch.
  35. 35. Lateral Ponticle (Ponticulus lateralis, pons lateralis) •A lateral ponticle is ossification in the oblique occipital membrane as it passes laterally from the superolateral aspect of the atlas lateral mass to the transverse process. •The anomaly appears to have no clinical relevance and is incidentally seen in 3% of cervical radiographs. Radiologic Features-The lateral ponticle is seen only on open mouth view and is manifest as a curvilinear ossification between the transverse process and the lateral mass of the atlas, often forming a distinct
  36. 36. Agenesis of the Atlas Anterior Arch •Isolated congenital absence of the anterior arch of the atlas is rare. •Acquired destruction of the anterior arch may occur secondary to inflammatory arthropathy, especially rheumatoid arthritis, tumor, and infection. •Lateral radiographs demonstrate absence of the D- shaped, corticated anterior arch of the first cervical vertebra.
  37. 37. Non-Union of the Atlas Anterior Arc •Non-union of the atlas anterior arch may occur as an isolated anomaly or in tandem with a cleft of the posterior arch (bipartite atlas). •The cleft through the anterior arch is vertically orientated and usually in the midline. On AP open mouth views the defect may be visible superimposed over the dens and may resemble a bipartite dens
  38. 38. Anomalies of the axis
  39. 39. •The most common congenital anomalies of the axis vertebra involve the odontoid process. •Generally discovered when radiographs are taken of the cervical spine for other clinical reasons, most commonly trauma. •The most common anomalies of C2 are ossiculum terminale, os odontoideum, agenesis, and hypoplasia. •Other anomalies of the axis include block vertebra
  40. 40. Ossiculum terminale persistens (Of bergmann) Terminal ossicle, ununited summit epiphysis,Bergmann ossicle.•The secondary apical ossification center at the tip of the dens (summit epiphysis) normally appears at 2 years of age and unites to the dens by age 12–13 years. •Failure of union to the dens produces an isolated ossicle at the tip.
  41. 41. Clinical Features •Rarely, it has been associated with brainstem symptoms when the transverse ligament dislocates into the cleft to allow atlantoaxial instability. •There is an increased incidence of a terminal ossicle in Down’s syndrome, which may also be a factor in atlantoaxial instability.
  42. 42. •On conventional radiographs the ossicle appears as a 3- to 5-mm, discrete, ovoid, or diamond-shaped ossicle visible at the most cephalic portion (odontoid summit) of the dens. •The tapered inferior margin may be mildly invaginated into a V-shaped cleft in the subadjacent dens. •This is a normal finding of the odontoid seen in 25% of patients < 12 yrs of age; but it is usually not seen afterthis age, at which time it constitutes non-union Radiologic Features
  43. 43. Os odontoideum •Os odontoideum is defined as non-union of the dens with the axis body. • A transverse, radiolucent cleft separates an ossicle of variable size from the axis body. • The transverse ligament is usually intact. •Normal development of the dens includes a synchondrosis separating the base of the dens from the C2 vertebral body. •It normally ossifies to connect the odontoid to the
  44. 44. •The majority of patients are asymptomatic. •Postural changes of the cervical spine may be present. •Palpable anterior gliding of the atlas may be detected during cervical flexion. •Compression syndromes of the upper cervical cord and brainstem are encountered. •Os odontoideum is better tolerated than other causes of atlantoaxial instability because mobility of the dens prevents cord compression between the dens and the atlas posterior arch (guillotine mechanism).
  45. 45. Radiologic Features •The ossicle is readily overlooked or obscured unless carefully looked for. •The open mouth view is particularly important for depicting the upper cervical Complex. •On this view the ossicle is round to oval in shape and is usually about one half the size of the normal odontoid. • There is typically a distinct circumferential cortex. • The separating cleft lies above the level of the superior articular facets of the axis, and a residual
  46. 46. •The ossicle may be aligned with the stump (orthoptic) or subluxed laterally (dystopic). •On the lateral film - altered appearance of the anterior arch of the atlas may be the only marker which includes enlargement, sclerosis, and an angular posterior surface where it invaginates into the cleft. •Hypertrophy of the atlas anterior arch is often present as an indication of chronic biomechanical stress and is useful in ruling out an acute dens fracture. •The transverse ligament is usually intact, and the
  47. 47. Hypoplastic and agenetic Odontoid process•A failure of the dens to form and ossify is an uncommon anomaly. •Total absence of the dens is referred to as agenesis, whereas partial formation is hypoplasia. •Hypoplasia of the dens is present when it measures < 12 mm in vertical height. •The majority of cases are discovered incidentally on
  48. 48. •Torticollis, suboccipital pain, and neuralgia; upper cervical crepitus; and vertebrobasilar symptoms may be present. • Occasionally with atlantoaxial instability, neurological symptoms from dysfunction of the cerebellum, brainstem, and cervical cord may occur. •With absence of the odontoid, atlantoaxial instability of up to 10 mm may remain asymptomatic because the cord is not readily compressed. •In true agenesis there is no evidence of a dens on any projection.
  49. 49. •On the lateral film no dens will be visible and there are no dental landmarks to assess the atlantodental interval. • The anterior arch of the atlas may take a number of altered forms, including enlargement, sclerosis, and a rounded posterior surface. •The posterior arch of the atlas may be hypoplastic, is occasionally sclerotic, and may exhibit a claw-like spur from the posterior tubercle curving superiorly.
  50. 50. Block vertebrae (Congenital synostosis, blocked vertebrae, congenital vertebral fusion, failure of vertebral segmentation, intercorporeal fusion) •Embryological failure of normal spinal segmentation resulting in fusion of one or more contiguous vertebral segments is described as congenital block vertebrae. •This most likely is the result of locally decreased blood supply during the 3rd– 8th week of fetal development.• Incidence range from 0.4% to 0.7% , with no sex predilection •The cervical spine is most commonly involved, followed by the lumbar and thoracic areas.
  51. 51. •The most common individual motion segments involved are reported as C5–C6, C2–C3, T12–L1, and L4–L5. •Most vertebral fusions are rarely symptomatic and are usually found incidentally on radiographs. •Physical examination is typically unremarkable. Head tilt, neck deformity, or loss of intersegmental mobility may be identified, particularly when multiple motion segments are involved. •Neurological changes may be identified from nerve
  52. 52. Radiologic Features •Radiographs in the frontal, lateral, and oblique planes should be supplemented with flexion– extension views. •The lateral view shows the characteristic triad of vertebral body hypoplasia, small disc, and variable posterior arch fusion. •The involved vertebral bodies are maximally hypoplastic in their sagittal dimension near the intervening vestigial disc space, such that the
  53. 53. •The overall vertical dimension of a congenital block vertebra is roughly equal to the height of two normal vertebrae plus an intervertebral disc (law of blocks). •The involved disc space is thin and variably ossified. The nucleus pulposus is often visible as a dense central calcification. •There is variable fusion of the posterior arches in up to 50% of cases at the level of body union, reflected as a single large spinous, laminae, or articular pillar.
  54. 54. •Immediately adjacent to the fusion site the first mobile vertebral body is often flattened and widened. Immediately adjoining intervertebral disc spaces may develop premature degenerative joint disease as early as the 2nd or 3rd decade of life.
  55. 55. Surgically fused vertebrae.
  56. 56. Klippel-feil syndrome (Congenital brevicollis, cervical assimilation, cervicalthoracic cage, cervico-oculo-acoustic syndrome)
  57. 57. Klippel-Feil syndrome (KFS) is currently a loosely applied eponym used to describe congenital segmentation defects of the cervical spine. The term Klippel-Feil syndrome should be reserved for congenital fusions involving more than one motion segment (i.e., more than two cervical vertebrae) Klippel and Feil’s original classification KFS I- extensive cervical segmental fusion anomalies
  58. 58. Clinical Features •The classic Klippel-Feil triad of short webbed neck, low posterior hairline, and reduced cervical motion is present in approximately 50% percent of cases. •There may be the illusion of no neck, with the head seeming to be continuous with the shoulders. • The most consistent finding is limitation of neck motion •The inability of the infant to rotate the head without
  59. 59. Radiologic Features •Multiple block vertebrae produce vertebrae that are hypoplastic with anterior concavity (wasp-waist sign). •The intervertebral disc spaces are small and may have calcification of the nucleus pulposus. Hemivertebrae may be visible on the frontal study. •Careful attention must be given to assessing the integrity of the dens and stability of the upper cervical complex. •Flexion– extension studies should be obtained to
  60. 60. •KFS patients at highest risk for neurological complications are those with atlantoaxial anomalies, more than one block vertebra, and degenerative changes at the adjacent mobile levels. •The vertebral artery is prone to anomalous development, and vertebrobasilar ischemia and infarction have been recorded. •Identification of spinal cord, nerve, and vertebral artery abnormalities may require MRI and MRA examinations.
  61. 61. Sprengel’s shoulder
  62. 62. •At the 3rd fetal week the scapula develops in the neck, at the C4–C5 level. • Under ordinary conditions the scapula migrates to its normal position by the 15th day of gestation. •Failure of migration results in sprengel’s shoulder.
  63. 63. Sprengel’s deformity shows elevation of the scapula and limited humeral abduction. Torticollis, with or without muscle spasm, may be present.
  64. 64. Radiologic Features. •The scapula is hypoplastic, shortened vertically and broad •Scapula is rotated so that the glenoid process is directed inferiorly. •The inferior angle rests above the normal T7 level. Omovertebral Bone- Present in 30–40% of Sprengel’s deformity cases. It is not always bone; it may also be composed of cartilage or fibrous
  65. 65. The Rigault’s radiographic classification Grade I - superomedial angle lower than T2 but above T4 transverse process. Grade II - superomedial angle located between C5 and T2 transverse process. Grade III - superomedial angle above C5 transverse process
  66. 66. Rigault’s radiological classification
  67. 67. Associations • Two thirds of patients presenting with sprengel’s shoulder demonstrate associated scoliosis, hemivertebrae, block vertebrae, spina bifida occulta, or cervical ribs. • It also occurs in 20–25% of Klippel-Feil syndrome cases.
  68. 68. Cervical rib(Dorsalization of the cervical spine)
  69. 69. •A costal bony process that originates from the cervical vertebra and forms true articulations with the transverse process and vertebral body constitutes a cervical rib. •At least 95% of cervical ribs occur at C7, but they have been recorded as high as C4. •They vary greatly in size, shape, and anatomic course and exhibit variation in their termination.
  70. 70. Clinical features •M: F- 2:1; Incidence- 0.5% of population, 66% are bilateral •Most cervical ribs are asymptomatic and are discovered incidentally. •Symptoms, when present are commonly related to compression of neural or vascular structures in the thoracic outlet (thoracic outlet syndrome).
  71. 71. Radiologic Features •An osseous rib-like structure closely adjoining but not fused to the distal end of the C7 transverse process is typically seen projecting anteriorly and inferiorly over a course of variable distance. •Articulation with the C7 vertebral body is seen in the classic presentation • To avoid mistaking a cervical rib for an atypical first thoracic rib- remember that C7 transverse
  72. 72. •Cervical ribs are one of many causes for neurovascular compression syndromes of the upper limb. The incidence of symptomatic cervical ribs is probably < 5%. •If these ribs cause symptoms, it is usually after middle age, when the shoulders begin to droop. The dominant arm is involved more commonly. •The brachial plexus may be divided by a cervical rib.
  73. 73. •Pathologic abnormalities associated with cervical ribs include poststenotic subclavian artery aneurysm and kinking or thrombosis of subclavian artery or vein. •Swelling of the upper limb may indicate obstruction of the subclavian vein, either from mechanical deformity or venous thrombosis. •Arterial compression may result in a Raynaud-like
  74. 74. Elongation of the C7 Transverse Process ( False cervical rib, droopy transverse process syndrome, apophysomegaly,and mega-apophysis. ) •Elongation of the C7 transverse process is a congenital anomaly. • The transverse processes curve and taper distally, lack costotransverse and costovertebral joints and are usually < 2 cm, and do not fuse with the first rib.
  75. 75. Elongation of the C5 and C6 Transverse P •Anterior tubercle of the transverse process is the costal element homologue. •It may be elongated, especially at C5 and C6, and is occasionally encountered on cervical spine radiographs. •Rarely associated with brachial plexus pain syndromes.
  76. 76. •Hypoplasia of the involved vertebral segments and intervening intervertebral disc (IVD) space may also be seen. •Complete or incomplete bony union may occur. • Flexion– extension radiographs show reduced mobility at C5–C6 and increased mobility at C4–C5. •Mimics of the condition include osteophytes, fracture, and expansile neoplasm.
  77. 77. Droopy Shoulder Syndrome •Women are more commonly affected • Radiographically, the thoracic spine may be visualized down to the T2 vertebra on a routine lateral cervical projection •A long gracile neck with low-set shoulders may be associated with thoracic outlet syndrome and fatigue syndromes of the shoulder girdle. • Exacerbation of the symptoms may occur if loads are carried on the shoulders.
  78. 78. Common pitfalls
  79. 79. Mach band effect
  80. 80. Ossification of stylohyoid ligament
  81. 81. Calcification of thyroid cartilage
  82. 82. Lymph node calcification
  83. 83. Ossification of nuchal ligament
  84. 84. Calcification of tracheal cartilage
  85. 85. Hair artifact
  86. 86. Paraodontoid notches.
  87. 87. Laryngeal constriction
  88. 88. Central incisor gap
  89. 89. Non-union of the spinous Process.
  90. 90. Thank you..

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