The Pediatric Vertebral Column: Anomalies of Vertebral Body Shape and Size
Decreased Height
Generalized of Multiple Flattened Vertebrae (Platyspondyly)
In all cases of platyspondyly, it should be ascertained whether the dens is hypoplastic. A short dens predisposes to the dangerous atlantoaxial subluxation, particularly during sudden flexion or extension of the cervical spine (see, for example, Fig. 4.200 ).
Diagnosis | Findings | Comments |
Achondroplasia | Short rectangular vertebral bodies with vertically widened disk spaces, lumbar lordosis, and acute lumbar sacral angle anteroinferiorly. Narrowing of the distal lumbar spinal canal on the AP projection. | Short stature with short extremities and typical changes of the bony pelvis. Ice cream scoop en face proximal femurs in infancy. Occasional symptoms from narrow cervical spinal canal. |
Thanatophoric dysplasia | Universal striking platyspondyly, much more severe than achondroplasia. Vertebrae appear like an “H” or a “U” on the frontal projection. European landline telephone receiver–shaped femora. | A member of the achondroplasia family, with much more severe enchondral slowing. This dysplasia, as implied by its name, is lethal. Survival can occur in the Torrance variant of thanatophoric dysplasia (straight femora). |
Hypothyroidism | Vertebral body flattening, beaklike projection from the anteroinferior proximal lumbar bodies in infancy. Relatively dense bone normal in neonates. | Delayed skeletal age. Neonatal screening via thyroid stimulating hormone levels now detects early. |
Osteogenesis imperfecta | One neonatal presentation as a result of intrauterine osteoporosis is collapse of many vertebral bodies. | Osteoporosis. Multiple fractures and healing or healed fractures elsewhere in the skeleton. |
Spondyloepiphyseal dysplasia congenita | Pear shaped vertebral bodies with significant decrease in height, kyphoscoliosis, and irregular epiphyses. | Short trunk. Delayed ossification of pubic bones. A lethal form exists with thoracic dysplasia and pulmonary hypoplasia. |
Hypophosphatasia | A neonatal lethal form has severe zones of rachitic-like changes and platyspondyly. | Phosphoethanolamine is excreted in the urine. |
Congenital Cushing disease | Marked osteoporosis, fractures frequent. | Excessive steroids. |
Achondrogenesis and homozygous achondroplasia | Variably deficient ossification of the axial skeleton. | Both are lethal dysplasias. Relatively large head. |
Metatropic dysplasia | Universal platyspondyly. Wide clefts between the vertebral body and neural arches; older: kyphoscoliosis. | Rhizomelic shortening. Markedly widened metaphyses. Tubular bones have a dumbbell or diabolo shape. |
Kyphomelic dysplasia | Relatively mild platyspondyly. Short long bones, some bowed, and ribs (often only 11 pairs). | Severe rhizomelic shortening, may have camptodactyly. |
Hallermann-Streiff syndrome | Narrow long bones and thin calvarium; multiple wormian bones. | Oculomandibulofacial syndrome. |
Osteoglophonic dysplasia | Multiple fibrous metaphyseal defects; platyspondyly with anterior projection; acromelic shortening. | Craniofacial dysostosis with fibrous metaphyseal defects. |
Diagnosis | Findings | Comments |
Osteoporosis | Generalized demineralization of bone with preservation of the cartilaginous zones of provisional calcification, including vertebral end plates (so that they are more conspicuous than normal). Multiple pathologic compression fractures. Thoracic vertebrae become flattened or anteriorly wedged; codfish (biconcave) vertebral deformity predominantly in the lumber region. | Many causes including steroid or heparin therapy, Cushing syndrome, idiopathic, sickle cell and other chronic anemias, leukemia, neuromuscular disease such as Duchenne muscular dystrophy, and homo-cystinuria. |
Secondary (or primary) hyperparathyroidism | If rickets is also present, the vertebral end plates (zones of provisional calcification) become difficult to see, which is the opposite of the situation in osteoporosis. | May also be a manifestation of renal osteodystrophy. |
Osteogenesis imperfecta | Multiple (sometimes universal), flat, or biconcave (codfish) vertebrae. Demineralization (osteoporosis), compression fractures. Multiple wormian bones. | Blue sclerae. Clinical course quite variable depending on severity. Sometimes mistaken for child abuse. |
Hypothyroidism Fig. 4.195, p. 415 | Vertebral body flattening, beaklike projection from anterior inferior proximal lumbar vertebral bodies. | Delayed skeletal age. Multiple wormian bones in congenital hypothyroidism. Neonatal screening usually identifies congenital involvement. |
NF1 | May have platyspondyly, idiopathic or localized acute scoliosis, plexiform neuromas, and other spinal area tumors. | Café au lait spots. |
Morquio syndrome Fig. 4.199, p. 418 Fig. 4.200a, b, p. 418 | Anterior midbody tonguelike projections off the lumbar vertebrae, hypoplastic dens. | MPS IV. Pectus carinatum. Increased urinary keratan sulfate. Spinal cord may become severely damaged from atlantoaxial subluxation related to the short dens. Normal or high intelligence. |
Other MPS and heteroglycanoses | In most types, such as Hurler and Hunter syndromes, there is an anteroinferior beaklike projection from lower thoracic or upper lumber vertebrae. | The vertebral change resembles hypothyroidism. Skeletal changes are more pronounced in MPS I–H, i.e., Hurler syndrome. Diagnostic findings in urine help s eparate the types. Also includes most mucolipidoses. |
Spondyloepiphyseal dysplasia tarda | Diminished height of the anterior portion of vertebral bodies. Bodies are middle and posteriorly biconvex. Occasional disk calcification. | Dysplastic femoral epiphyses, bilaterally symmetric, and also other epiphyses. Early onset of arthritis. |
Progressive pseudorheumatoid dysplasia | Large limb joints look like JIA; vertebral bodies more like pseudoachondroplastic dysplasia. | A dysplasia and not an inflammatory condition. Rheumatoid factor normal, for example. |
Treated (by transfusions) thalassemia major | May even be changes of scurvy; coarse trabeculae and wide diploic space of thalassemia may remain. | Anemia often quite pronounced. |
Pyle disease | Biconcave platyspondyly. Abnormally wide diametaphyseal regions of long bones that lack normal concavity. | Familial metaphyseal dysplasia. |
Spondylometaphyseal dysplasia, Kozlowski type Fig. 4.203, p. 420 | Marked platyspondyly. | AD. Limitation of joint motion. |
Kniest disease Fig. 4.204, p. 420 | Multiple flattened vertebral bodies with irregular end plates. Coronal clefts in the midposterior vertebral bodies (common), spinal stenosis, kyphoscoliosis. | Spondyloepimetaphyseal dysplasia with widened metaphyses. Stiff, thickened joints. Myopia, deafness. |
Dyggve-Melchior-Clausen dysplasia Fig. 4.205, p. 420 | Vertebral bodies with anterior and posterior humps above and below (“Bactrian camel back”). Lacy iliac crests. | Short extremities, mental retardation. Similar changes with normal mental development indicates Smith-McCort syndrome. |
Pseudoachondroplasia Fig. 4.206, p. 420 | Vertebrae are biconvex in neonates; later diminished height with anterior wedging. End plates are irregular, as are epiphyses. Kyphoscoliosis. | A spondyloepiphyseal dysplasia. Short extremities including hands and feet. |
Stickler syndrome (arthro-ophthalmopathy) | Significantly low vertebral body height. | Marfan-like body habitus, progressive myopia, and retinal detachment. Epiphyseal dysplasia. Early onset of arthritis. |
Dysosteosclerosis | Platyspondyly and osteosclerosis (punctate margins of vertebral bodies). Resorption of phalangeal tufts. | Multiple fractures. |
Loss of Height in Single or Multiple Vertebral Bodies
Diagnosis | Findings | Comments |
Spondylitis-diskitis (osteomyelitis) Fig. 4.278, p. 477 | The most common cause of decreased vertebral height with destruction in childhood. Early radiographic changes: decreased disk space and destruction of the adjacent end plate, with possible posterior displacement of vertebral body. Earlier positive findings on MRI or NM. Later, occasionally wedging or fusion in the healing phase. Rarely, central osteomyelitis in the vertebral body without disk space involvement. | Pyogenic bacteria, tuberculosis (starts anteri-orly), brucellosis (also starts anteriorly). Also often no organism found. Differentiation from tuberculosis in early phase may be difficult; there is later calcification and gibbus formation in tuberculosis. |
Fracture Fig. 4.208, p. 422 | Many involve more than one vertebral body. Anterior wedging with decreased height (exception: a Chance fracture is transverse through body and arch, which may lead to “taller” vertebra). May be a nose-shaped projection from an anterior corner. Dense callus seen within the bone after 10 d. Disk spaces are maintained. | Caution: Lateral compression on frontal projection may be subtle. CT and MRI are indicated in comminuted fractures for position of fragments and spinal cord involvement, respectively. |
Child abuse | Often fractures are multiple; spinous processes alone may be fractured. Many other typical fractures elsewhere in skeleton. | On chest images, look for paraspinal hematoma or rib fractures. |
LCH (eosinophilic granuloma) Fig. 4.209, p. 422 | Initial slight flattening, finally becoming vertebra plana. May affect single or multiple vertebral bodies. Arch elements less often affected; disk spaces intact. Regains height after the process is no longer active (the end plate zone of provisional calcification continues its enchondral bone growth). | Additional foci may be sought by bone scintigraphy, or, indeed, by PET scan. Typically painless in early stage. |
Leukemia Osteoporosis, pathologic compression fractures. Disk spaces maintained. | Osteoporosis and vertebral compression can be the presenting findings of leukemia (may even simulate child abuse). | |
Sickle cell anemia Fig. 4.228, p. 434 | Associated with the “Lincoln Log” notched indentation of middle, upper, and lower vertebral body margins. | Anemia. Look also for calcified gallstones. |
Fibrous dysplasia | Flattening of a single vertebra can occur; may show scoliosis. | Café au lait spots, advanced bone age. |
Following irradiation | Irregular growth disturbance. | Especially after radiation for renal or adrenal tumors. Dose-dependent (possible after 30 Gy; usual after 40 Gy). |
Tumors, metastases Fig. 4.211, p. 424 | Destruction and loss of height of one or more vertebral bodies. Disk spaces preserved. Pedicle destruction may occur. | Metastases: especially neuroblastoma (may be painless at first), rarely rhabdomyosarcoma, Wilms tumor, or Ewing sarcoma. Primary: Ewing sarcoma, may be sclerotic; vertebral body aneurysmal bone cyst, lucent and may collapse. |
Chronic recurrent multifocal osteomyelitis | Associated sternal, clavicle, or long bone lesions. | May have acne or plantopalmar pustulosis (SAPHO [s ynovitis, a cne, p almoplantar pustulosis, h yperostosis, and o steitis] association). Many plasma cells but no organism on biopsy. |
Fungal infection | Vertebral body destruction without disk space decrease. | One cause is disseminated coccidioidomycosis. |
Tuberculosis and brucellosis Fig. 4.212a, b, p. 424 | When vertebral body is affected, the anterior body typically loses height sooner than the posterior. | Tubercuosis: skin testing; brucellosis: contact with affected livestock. |
Thalassemia major; Gaucher disease | Single vertebra can be affected. |
True or Apparent Enlargement of the Vertebral Bodies
A compensatory increase in height occurs in vertebrae adjacent to a hemivertebra, acquired fused vertebrae after spondylitis, and in surgical posterior fusion in several types of scoliosis.
Diagnosis | Findings | Comments |
Nonambulatory in early childhood: CNS impairment and neuromuscular disease | Tall or barrel-shaped vertebrae on the lateral image, predominantly in lower thoracic and the lumbar vertebrae. | May be already evident in neonates; changes increase with age and indicate that a child is not walking. |
Polyostotic FD, Albright syndrome | Barrel-shaped vertebrae. Advanced bone age. | Café-au-lait spots. |
Hox gene dependent increased height adjacent to hemivertebra | Although tilted, the disk height tends to be normal. Bodies adjacent to butterfly vertebrae similar have medial lengthening in compensation. | May be a part of VATER association. |
Caudal regression syndrome | Sacral aplasia; may have vertical overgrowth of remaining lumber bodies. | May be associated with maternal diabetes. |
Rachischisis | Vertebral bodies may be enlarged on frontal view; disk spaces decreased. | Spina bifida aperta, perhaps with associated Chiari type II malformation. |
Scheuermann disease | At the kyphosis, bodies may have increased sagittal and coronal width, along with reduced height especially anteriorly. | Often familial. |
Compression fracture | Enlargement can occur in width sagittally or coronally; callus after 10 d. | MRI for associated cord damage. |
Mass in the spinal canal | Apparent increase in height on lateral image is caused by narrowing in the sagittal dimension. | MRI to show the mass. |
Tumors and metastases; aneurysmal bone cyst | Vertebral elements may be expanded by tumor growth or else diminished by pathologic fracture or collapse. | Hemangioma, lymphangioma, giant cell tumor, osteoblastoma, and renal cell carcinoma are possibilities. |
Diagnosis | Findings | Comments |
Pycnodysostosis | Relatively tall vertebral bodies. Bones abnormally dense and somewhat fragile. | Multiple wormian bones; obtuse angle of the mandible. |
Kenny-Caffey syndrome | Tall vertebrae with hourglass shape. Gracile long bones. | Also known as tubular stenosis dysplasia. |
Melnick-Needles syndrome | Relatively tall vertebral bodies with small lumbar disk spaces. “Twisted” ribs and long bones. | Also known as osteodysplasty. |
Cartilage hair hypoplasia | Vertebral bodies look tall because of decreased transverse dimensions. | Also known as metaphyseal chondrodysplasia, McKusick type. Sparse, thin, light-colored hair. |
Fuhrmann syndrome | Narrow tall vertebral bodies. | Bowed femurs; absent fibulas. |
Freeman-Sheldon syndrome | Narrow tall vertebral bodies, including cervical. Some bodies may be flattened instead. | “Whistling face.” Ulnar deviation of hands. |
Hajdu-Cheney syndrome | Tall lumbar vertebral bodies with narrow disk spaces. Collapse may also occur. | Also known as arthrodentosteodysplasia. Short stature. Multiple wormian bones. |
Dolichospondylic dysplasia | High vertebral body dwarfism. | Gracile long bones, short stature. |
3-M syndrome | Sagittally shorter lumbar vertebral bodies look tall. Slender ribs and tubular bones. | May be allelic with dolichospondylic dysplasia, which it resembles. The three “M”s represent the lead authors of the original description. |