Platyspondyly

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► [Abnormal flattening of the vertebral bodies]

Vertebral flattening can be congenital or acquired. When it is acquired, a single vertebra, two adjacent vertebrae, or several noncontiguous vertebrae may be collapsed. Extreme flattening of a single vertebra is referred to as vertebra plana. Acquired conditions causing collapse of either single or multiple vertebral bodies include trauma, osteoporosis, osteomalacia, steroid or radiation therapy, and infections, as well as focal (hemangioma, eosinophilic granuloma, giant cell tumor, chordoma, etc.) and diffuse (plasma cell

Eosinophilic Granuloma The Skin
Fig. 3.9 a, b. Ehlers-Danlos syndrome in an 11-year-old boy. a Note flattening of the lumbar vertebral bodies, most prominent from L-3 to L-5. b There is lumbar scoliosis, which is convex to the right. (From Kozlowski et al. 1991)

myeloma, leukemia, lymphoma, metastases - especially from breast, lung, and prostate carcinomas and from neuroblastoma, Paget disease, etc.) vertebral lesions.

Congenital flattening of the vertebral bodies can be generalized or confined to a given spinal level. Occasionally, a more irregular distribution is observed, with involvement of several noncontiguous vertebrae alternating with unaffected vertebrae (Kozlowski et al. 1995).

In campomelic dysplasia (OMIM 114290) flattening is confined to the cervical spine and is associated with scarcity of cervical ossification. Similarly, cervical platyspondyly is seen in spondylocamptodactyly (OMIM 600000), a disorder inherited as an autosomal dominant mutation in which scoliosis and camp-todactyly are features (Lizcano-Gil et al. 1995). In children with homocystinuria (OMIM 236200) the vertebral bodies, especially in the thoracic spine, are flattened and anteroposteriorly elongated, and may be biconcave in shape. Scoliosis and generalized osteoporosis with compression fractures can be seen (Smith 1967). In Ehlers-Danlos syndrome, platyspondyly affecting only the lumbar spine is characteristic (Kozlowski et al. 1991) (Fig. 3.9 a,b). The most likely cause of the flattening is muscular imbalance with increased stress on the lumbar vertebrae. Additional spinal changes consistently observed in this condition include thoracolumbar kyphoscoliosis, which is often present at birth, anterior wedging of the vertebral bodies (in later years), spondylolysis with spondylolisthesis, and posterior scalloping of the vertebral bodies (Beighton and Thomas 1969). SPONASTRIME dysplasia (OMIM 271510), an association of spondylar and nasal alterations, and vertical striations in the metaphyses of the long bones, shows a peculiar pattern of lumbar spine involvement, characterized by a predictable evolution over time (Langer et al. 1997). In infancy, the vertebral bodies are very flat, with straight endplates posteriorly, biconvex end-plates anteriorly, and anterior tonguing. With advancing age platyspondyly tends to resolve, while a moderate deformity of the vertebral bodies, consisting in smooth endplate concavity posterior to the midpoint of the body, persists into adulthood (Fig. 3.10 a-d).

Generalized platyspondyly is a valuable radiographic sign of many skeletal dysplasias, metabolic disorders, and malformation syndromes. Universal platyspondyly, often of severe degree, develops with increasing age in patients with mucopolysaccharido-sis IV (Morquio syndrome, OMIM 253000), a condition that has long been confused with Dyggve-Mel-chior-Clausen dysplasia owing to the association between short-trunk dwarfism and platyspondyly. The vertebral end-plates are irregular, but usually not notched, and tongue-like protrusions arise from the anterior wall of the vertebral body, especially at the apex of the thoracolumbar gibbus deformity (Levin et al. 1997) (Fig. 3.11). In Dyggve-Melchior-Clausen dysplasia (OMIM 223800, 304950) there is generalized platyspondyly, with anterior vertebral projections and undulating defects in the end-plates ('camel hump' appearance) (Spranger et al. 1975) (Fig. 3.12). Marked vertebral flattening is a feature in spondylometaphyseal dysplasia, Kozlowski type (OMIM 184252) (Fig. 3.13). Elongated tongue-like projections of the vertebral body can be seen, the anterior beaking being further exacerbated in the thoracic tract by the spinal kyphosis. Other well-differentiated spondylometaphyseal dysplasias show mild to moderate platyspondyly (Kozlowski and Bellemore 1989; Kozlowski et al. 1988). In spondyloepiphyseal dysplasia congenita (OMIM 183900) the skeletal changes, including those involving the spine, vary with age. In infancy flattening of the vertebral bodies with dorsal wedging (pear-shaped appearance) occurs in the thoracic and upper lumbar spine (Fig. 3.14). In childhood the vertebral bodies are flat and anteriorly pointed, especially at the thoracolum-

Fig. 3.10 a-d. SPONASTRIME dysplasia in children at different ages. a In this newborn note severe platyspondyly of the lumbar spine. The shape of the vertebral bodies is characteristic: a posterior part with straight end-plates, an anterior part with convex endplates, and a centrally located anterior bony protrusion. The pattern of ossification is irregular, with lucent areas present. b In this 13-month-old child the vertebral bodies are relatively taller than in the newborn. The anterior part is taller than the posterior part and has convex end-plates. The posterior part has straight or slightly convex end-plates. c In this 36-month-old child the posterior part of the body has slightly convex end-plates that tilt, so that the junction between anterior and posterior parts is the narrowest in the body. d In this 15-year-old patient the vertebral bodies are of normal height, and uniform in shape and size. The end-plate concavity is smooth, and its center is posterior to the midpoint of the body. (From Langer et al. 1997)

Vertebral Artery

bar junction. Odontoid hypoplasia also becomes apparent at this age. In adulthood the dorsal vertebrae are flat and irregular, with narrowing of the disc spaces, and wedged, with resultant kyphoscoliosis (Spranger and Langer 1970). Spondyloepiphyseal dysplasia tarda, X-linked (OMIM 313400) shows a typical vertebral configuration, which develops at the lumbar level in late childhood or early adolescence: bulging in the central and posterior portions of the superior and inferior vertebral end-plates, producing characteristic 'humps.' The intervertebral disc spaces are narrow posteriorly and wide anteriorly (Fig. 3.15). Platyspondyly with no end-plate humps and no tongue-like projections of the vertebral bodies is observed in spondyloepiphyseal dysplasia, Maroteaux type (OMIM 184095). Abnormalities in this condition are confined to the skeleton and include shortening of the hands and feet and genu valgum deformity (Doman et al. 1990). Multiple epiphyseal dysplasia (OMIM 132400) displays more irregular vertebral

Morquio Syndrome Platyspondyly
Fig. 3.11. Mucopolysaccharidosis IV (Morquio syndrome). There is severe platyspondyly, marked irregularity of vertebral end-plates, and tongue-like projections from the anterior wall of the vertebral bodies (arrows). Note also thoracolumbar kyphosis and posterior scalloping of vertebral bodies

end-plates and a less severe degree of platyspondyly than spondyloepiphyseal dysplasia tarda. Additional distinguishing features in multiple epiphyseal dysplasia include absence of the vertebral humps and a more severe degree of epiphyseal dysplasia in the distal extremities (Murphy et al. 1973). 'Brachyolmia' is the designation for a heterogeneous group of conditions characterized clinically by short stature, and radiographically by generalized platyspondyly with minimal involvement of the limbs. Four types have been recognized. (1) Hobaek type (OMIM 271530) is associated with mild flattening of the vertebral bodies, irregular end-plates, and lateral projection of the vertebral profile beyond the pedicles. (2) In Toledo type (OMIM 271630) platyspondyly occurs in association with corneal opacities and precocious ossification of the costal cartilages. (3) Maroteaux type (OMIM 271530) is characterized by a round aspect of the anterior and posterior vertebral borders, with mild lateral elongation. All these three disorders are caused by autosomal recessive mutations. (4) The existence of an autosomal dominant type characterized by the most severe spinal changes (severe platy-spondyly, decreased interpediculate distance with vertebral bodies projecting laterally beyond the pedicles) has been suggested (Horton et al. 1983; Shohat et al. 1989; Darcan et al. 2000). Generalized, severe

Fig. 3.12. Dyggve-Melchior-Clausen syndrome in a 17-year-old girl. Note platyspon-dyly and the unique vertebral body configuration, with round anterior vertebral projections and notch-like defects of superior and inferior endplates, leading to a camel-hump appearance. (From Hall-Crag-gs and Chapman 1987)

platyspondyly with a distinctive shape of the vertebral bodies occurs in metatropic dysplasia and parastremmatic dwarfism. Owing to defective ossification, the vertebral bodies in metatropic dysplasia (OMIM 250600) are underdeveloped (diamond-shaped), flattened, and anteriorly wedged, especially in the dorsal spine (Fig. 3.16 a,b). The precocious appearance of kyphoscoliosis, the severity of epiphy-seal changes, and the characteristic pelvic configuration (flaring of the iliac wings with marked hypo-plasia of the basilar portions) allow differentiation from spondylometaphyseal dysplasia, Kozlowski type (Kozlowski et al. 1976). Spinal changes in parastremmatic dwarfism (OMIM 168400) include flat and broad vertebral bodies, irregular ossification of the vertebral end-plates giving the bone a 'flocky' appearance, and severe kyphoscoliosis (Fig. 3.17 a,b). The irregular pattern of ossification ('flocky bone')

Girls With Spondyloperipheral Dysplasia

Fig. 3.13. Spondylometaphyseal dysplasia, Kozlowski type. There is severe platyspondyly with anteroposterior elongation of the vertebral bodies. The end-plates are irregular

Spondylometaphyseal Dysplasia

Fig. 3.14. Spondyloepiphyseal dysplasia congenita. The vertebral bodies are flattened and pear shaped. The intervertebral disc spaces are wider than normal

Fig. 3.13. Spondylometaphyseal dysplasia, Kozlowski type. There is severe platyspondyly with anteroposterior elongation of the vertebral bodies. The end-plates are irregular

Fig. 3.14. Spondyloepiphyseal dysplasia congenita. The vertebral bodies are flattened and pear shaped. The intervertebral disc spaces are wider than normal affects the whole skeleton, notably the iliac wings, the scapulae, and the epiphyseal-metaphyseal junction of the tubular bones and tends to disappear in later adolescence and adulthood (Langer et al. 1970). Severe platyspondyly is also a feature in several lethal bone dysplasias, including thanatophoric dysplasia, fibrochondrogenesis, homozygous achondroplasia, and lethal short-limbed dysplasia with platyspondyly (San Diego, Torrance, Luton, Calgary, Yamagata, Perth, and Shiraz types) (Kozlowski et al. 1995). Pseu-dodiastrophic dysplasia (OMIM 264180), an early lethal bone dysplasia resembling diastrophic dyspla-sia in many respects, including the rhizomelic type of limb shortening and the severe clubfoot deformity (Burgio et al. 1974), show more pronounced platy-spondyly, interphalangeal and metacarpophalangeal joint dislocations, and absence of the 'hitchhiker thumb' (abduction of the hypermobile and proximal-ly inserted thumb) typical of diastrophic dysplasia. Platyspondyly with hypoplastic, 'wafer-thin' vertebrae has been described in association with general-

Platyspondyly
Fig. 3.15. Spondyloepiphyseal dysplasia tarda, X-linked. Platyspondyly is associated with a typical configuration of lumbar vertebrae: bulging of the superior and inferior endplates in the shape of characteristic humps
Hitchhiker Thumb ArthritisHitchhiker Thumb Arthritis

of the thorax, shortening of long bones with marked metaphyseal widening,'battle-axe' appearance of proximal femurs, epiphyseal ossification delay, short ilia with round margins and flat acetabula. (From Westvik and Lachman 1998)

Bone Histology And Metatropic Dysplasia
Fig. 3.16 a, b. Metatropic dysplasia. Postmortem radiograph of a male infant who died 30 min after birth. a There is severe platyspondyly and midline nonunion of the posterior elements. b Additional features include narrowing and elongation

of the thorax, shortening of long bones with marked metaphyseal widening,'battle-axe' appearance of proximal femurs, epiphyseal ossification delay, short ilia with round margins and flat acetabula. (From Westvik and Lachman 1998)

ized osteosclerosis in a unique form of sclerosing lethal skeletal dysplasia (Brodie et al. 1998).

Progressive pseudorheumatoid arthropathy (OMIM 208230) is a bone dysplasia whose clinical symptoms develop in children between 3 and 8 years of age and which is characterized by progressive arthropathy resembling rheumatoid arthritis, and platyspondyly. Arthropathy manifests with painful swelling and joint stiffness involving the hips, the cervical spine, and the finger joints in the hands. Laboratory findings (negative rheumatoid factor, normal sedimentation rate) and histology (normal synovium, nesting of chondrocytes in resting and proliferative cartilage, loss of normal cell columnar organization in growth zones) exclude rheumatoid arthritis.Vertebral changes mimic those in Scheuermann disease, including flattening with anterior ossification defects. Platyspondyly resulting in short stature, sometimes associated with kyphoscoliosis, is typical (Spranger et al. 1983). Abnormal epiphyses, with irregularity and flattening, are seen at multiple sites (Kaibara et al. 1983). In pseudoachondroplasia (OMIM 177170) there is moderate flattening of the vertebral bodies in childhood, but they typically tend to recover during adolescence. The vertebrae show superior and inferior 'humps,' resulting in a biconvex appearance, end-plate irregularities, and anterior tongue-like projections of variable degree (Finidori et al. 1980) (Fig. 3.18).

Based on the observation of a father and son, both born to consanguineous parents, of which the boy had severe platyspondyly and metaphyseal manifestations of enchondromatosis (Ollier disease, OMIM 166000) and his father had moderate platyspondyly only, it was suggested that platyspondyly could represent (1) a manifestation of the carrier state for an autosomal recessive trait, (2) a minor expression of the same autosomal recessive disease, or (3) less likely, the variable expression of an autosomal dominant trait (Halal and Azouz 1991). The condition, referred to as spondylo-enchondro-dysplasia (OMIM 271550), is characterized by an association of enchondro-matosis similar to that of Ollier disease,platyspondy-ly with end-plate irregularities (Fig. 3.19 a,b), multiple radiolucent defects (due to enchondromas) in the metaphyses of long and flat bones, and kyphoscolio-

Fig.3.17a,b. Parastremmatic dwarfism in a 9-year-old child. a Note flattening and marked end-plate irregularities of the vertebral bodies. The abnormal pattern of ossification gives rise to a unique 'flocky bone' appearance of vertebral end-plates. b Note also thoracic scoliosis

End Plate Ossification

Fig.3.17a,b. Parastremmatic dwarfism in a 9-year-old child. a Note flattening and marked end-plate irregularities of the vertebral bodies. The abnormal pattern of ossification gives rise to a unique 'flocky bone' appearance of vertebral end-plates. b Note also thoracic scoliosis

Biconvex Vertebrae
Fig. 3.18. Pseudoachondroplastic dysplasia in a 2-year-old child. Note superoinferior convexity (humps) of the lumbar vertebral bodies, with anterior tongue-like beaking. (From Unger et al.2001 )

sis (Schorr et al. 1976; Frydman et al. 1986). Platy-spondyly associated with short stature, severe hip changes, distal shortening of ulna, and signs of peripheral dysostosis, with short hands and feet, occurs in spondyloperipheral dysplasia with short ulna (OMIM 271700) (Kelly et al. 1977). A boxer-like face, sensorineural deafness and mental retardation are further features (Sorge et al. 1995). A previously unreported form of skeletal dysplasia characterized by platyspondyly with amelogenesis imperfecta (OMIM 601216) has been described by Verloes et al. (1996) in two sibs born to consanguineous parents. In this autosomal recessive disorder the vertebrae are flat and rectangular, with posterior scalloping, short pedicles, and narrow interpediculate distances. Absence of the enamel cap in the permanent teeth is a cardinal feature. Additional manifestations include short stature, short hands, and broad femoral necks. A unique generalized bone dysplasia with severe platyspondyly and distinctive peripheral anomalies has been reported in two patients by Kozlowski et al. (1999) (Fig. 3.20). The first patient showed short stature, short trunk, valgus deformity of the knees, short hands and feet, meta-epiphyseal changes of

Fig. 3.19 a,b. Spondylo-en-chondrodysplasia in a 14-year-old patient. Note a platy-spondyly of the thoracic vertebrae and b irregular endplates with defects of the frontal parts of the thoracic and upper lumbar vertebrae. (From Uhlmann et al. 1998)

Fig. 3.19 a,b. Spondylo-en-chondrodysplasia in a 14-year-old patient. Note a platy-spondyly of the thoracic vertebrae and b irregular endplates with defects of the frontal parts of the thoracic and upper lumbar vertebrae. (From Uhlmann et al. 1998)

Giant Cell Chondrodysplasia

variable degree, and rectangular vertebrae. The second patient had some of the features seen in brachy-olmia, the most important phenotypic sign being shortening of the trunk. The vertebral bodies were flat, with thick and irregularly calcified ring apophyses. However, involvement of the long tubular bones and round bones, as well as pelvic and scapular involvement, delineates a previously unreported bone dysplasia.

A special type of collapsed vertebra, the 'H-shaped' vertebra, is typically seen in sickle cell anemia. This configuration is characterized by squared-off depression of the central portion of the endplates, which is seen on lateral radiograms and is probably related to growth disturbances resulting from ischemia of the vertebral growth plate (Reynolds 1966). H-Shaped vertebrae are occasionally seen in other types of anemia, Gaucher's disease (Hansen and Gold 1977), and osteoporosis (Fig. 3.21), although a smooth biconcave contour rather than a step-like depression of the vertebral end-plate is more typical in the latter conditions. An H-shaped vertebral configuration on frontal radiograms is observed in thanatophoric dysplasia.

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  • sabine
    What is morquio syndrome pictures?
    8 years ago
  • adonay
    Is L3 hemangioma disappear?
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