REVIEWARTICLE

Paediatric acquired pathological vertebral collapse

Hassan Hirji & Asif Saifuddin

Received: 24 July 2013 /Revised: 28 October 2013 /Accepted: 26 November 2013 /Published online: 9 January 2014# ISS 2014

Abstract Vertebral collapse is a significant event in the pae-diatric patient with a real potential for associated deformityand morbidity. While in adults the causes tend towards themalignant, particularly metastatic and metabolic disease, thepaediatric population demonstrates a different range ofdiagnoses. This article reviews the typical imaging find-ings of the more common underlying acquired patho-logical causes of vertebral collapse in children, includ-ing Langerhans cell histiocytosis, chronic recurrent mul-tifocal osteomyelitis, osteogenesis imperfecta. Othercauses include pyogenic osteomyelitis and tuberculosis andneoplastic lesions, either primary, metastatic or of haemato-logical origin.

Keywords Spinal diseases . Spinal neoplasms . Paediatricacquired pathological vertebral collapse

Introduction

Vertebral collapse is an extremely rare, but significant event inthe paediatric patient, with a real potential for associated pain,deformity and morbidity. While in adults the causes tendtowards the malignant, particularly metastatic and metabolicdisease, in the paediatric population a different range of dif-ferential diagnoses is encountered, reflecting the nature of the

developing spine with a higher preponderance of primarylesions. Furthermore, the natural on-going growth of the pae-diatric spine may permit good recovery even with conserva-tive management. This article seeks to provide a review of thetypical imaging findings of the more common underlyingacquired pathological causes of vertebral collapse in children.Collapse secondary to trauma has not been included, nor havediseases of a congenital origin.

Anatomy and normal imaging appearances

As in the adult, the spine consists of bony vertebrae separatedby compressible intervertebral discs. The vertebrae have largebodies that consist of a dense outer bony cortex and containthe highly vascular marrow. Unlike the adult spine, the softtissues are more flexible and the age-related changes ofosteopenia and disc degeneration are less frequent, if at allpresent. The vertebral end plates are not fused, giving thespine the possibility of remodelling with restoration of almostnormal height, even following quite severe and deformingdisease.

The arterial supply to the vertebral body is through perios-teal branches, which penetrate the vertebral body at severalsites adjacent to each endplate, and the lumbar artery. Inaddition, there are posterior arterial branches that pass acrossthe posterior border of the vertebral body and anastomose nearthe midline [1, 2]. These are important to recognise as inflam-matory and vasculitic processes will be predisposed to arisenear the arterial vessels, increasing the destructive effects atthese sites.

The venous drainage is through small tributary vessels thatpass through the medulla to a central venous channel, the basi-vertebral vein, which drains from the dorsal aspect of thevertebral body. Here it merges with a large valveless

A. Saifuddin (*)Royal National Orthopaedic Hospital, Stanmore, UKe-mail: Asif.Saifuddin@rnoh.nhs.uk

H. HirjiNorth West London Hospitals NHS Trust Northwick Park Hospital,Harrow, UK

Skeletal Radiol (2014) 43:423–436DOI 10.1007/s00256-013-1792-3

venous plexus, which allows the ready passage of in-fection and metastatic seeding, particularly from thepelvis or perineum [1, 3].

At bir th, the marrow space is fi l led with red(haematopoietic) marrow. Over the first 5 years, this un-dergoes gradual conversion to yellow marrow. On MRI, redmarrow has T1W signal intensity (SI) greater than muscle, butless than fat. As the child moves towards maturity, the redmarrow is replaced by fatty, yellowmarrow. This has a shorterT1 relaxation time giving a higher T1W signal and a lowerT2W signal. A more detailed description of marrow signal in

children and its changes with maturation has been provided byFoster et al [4].

Imaging technique

Aswith the adult spine, AP and lateral radiographs should firstbe obtained, with standing radiographs, if possible, dependingupon the level of pain, allowing an assessment of overallspinal alignment. Following radiography, MRI will invariablybe the next investigation, and the majority of cases can be

Fig. 1 Pyogenic osteomyelitis ina 14-year-old girl. a Lateralradiograph of the thoraco-lumbarspine showing erosion of theanterior–superior corner of theL1 vertebra (arrow). b SagittalT1-weighted spin echo (T1W SE)and c T2-weighted fast spin echo(T2W FSE) MR images showingend-plate erosion, loss of discheight and extensive marrowoedema-like signal intensity(SI; arrows) with minor anteriorsub-ligamentous spread ofinfection. d Sagittal post-contrastfat-suppressed T1W SE MRimage showing enhancement ofthe anterior soft tissue extension(arrow) and also focal discenhancement. e Sagittal CTmultiplanar reconstruction (MPR)showing end-plate erosions andmedullary sclerosis (arrows)consistent with the relativelychronic nature of the diseaseprocess. f Sagittal short T1inversion recovery (STIR) MRimage shows normalisation of themarrow SI within T12 and L1,indicating healing with completeloss of the intervening disc

424 Skeletal Radiol (2014) 43:423–436

adequately diagnosed purely on this combination of imaging.In the setting of vertebral collapse, CT and scintigraphy addlittle further diagnostic information.

The MRI technique for assessing the paediatric spine doesnot differ from the adult spine, typically comprising a combi-nation of sagittal and axial T1W spin echo (SE) and T2W FSEsequences. The addition of a fat-suppressed sequence (STIRor fat-suppressed T2W FSE) is of value in the assessment ofsuspected neoplastic, infective or inflammatory disorders andimaging of the whole spine should always be performed tolook for multilevel non-contiguous disease, both for the

purpose of overall disease staging, but also to aid in differen-tial diagnosis. As in the adult spine, the administration of IVgadolinium is of particular value in suspected spinalosteomyelitis/discitis to aid in the differentiation of phlegmonfrom abscess. Depending upon the age of the child and/or thelevel of pain, general anaesthesia may be required. Diffusion-weighted imaging may be misleading since increased marrowSI may be seen in 48 % of normal children [5].

If required, staging of the remainder of the skeleton can beachieved with whole-body bone scintigraphy, or for someconditions, such as suspected Langerhans’ cell histiocytosis

Fig. 2 Pyogenic L2–L3 discitisin a 13-year-old boy. a SagittalT1W SE, b sagittal STIR andc)axial T2W FSE MR imagesshowing destruction of the disc,mild end-plate oedema andprominent posterior extensionof the phlegmon deep to theposterior longitudinal ligament(PLL; arrows)

Fig. 3 Tuberculosis (TB)osteomyelitis of T3 in a 10-year-old girl. aLateral plain radiographof the thoracic spine showsmarked anterior collapse andwedging of T3 (arrow) with focalkyphosis. b Sagittal T1W SE MRimage shows associated anteriorand posterior sub-ligamentousspread of infection with mildspinal cord compression (arrows)

Skeletal Radiol (2014) 43:423–436 425

(LCH), with radiographic skeletal survey. However, whole-bodyMRI using a combination of coronal T1W SE and STIRsequences is now becoming commonplace [6–8]

When a diagnosis cannot be achieved based on clinical andimaging features alone, then consideration will have to begiven to percutaneous needle biopsy, which is safelyperformed with CT guidance under general anaesthesia[9]

Acquired paediatric vertebral collapse

Vertebral collapse occurs secondary to a failure in the me-chanical integrity of the anterior and/or middle columns of thevertebral body. The vertebral body derives its strength from

the outer cortex and the integrity of the trabecular bone andmarrow within. Diseases causing destruction of either of thesecomponents may lead to a loss of vertebral height. Vertebraplana occurs when the vertebral body is reduced to a singledense sclerotic band, often with preservation of the discs oneither side.

Differentiating from trauma

It can be difficult to differentiate between a pathologicalfracture and a post-traumatic injury, but this can be of vitalimportance in determining further care and future prognosis.Traumatic injuries have a preponderance in the cervical spineof young children and for the thoracolumbar spine in older

Fig. 4 TB osteomyelitis of thelumbo-sacral junction in a16-year-old boy. a Sagittal T1WSE and b T2W FSE MR imagesshowing diffuse marrowoedema-like SI in the L5, S1,S2 and S3 vertebrae. Note theabsence of disc involvementand the extensive anterior sub-ligamentous extension (arrows).cCoronal T2W FSE MR imageshowing a large left psoas abscess(arrows). dAxial T2W FSE MRimage showing extension into theneural arch (arrows) as well as thelarge anterior sub-ligamentouscollection

426 Skeletal Radiol (2014) 43:423–436

children and teenagers [10]. The differentiation between avertebral fracture due to trauma and one caused by patholog-ical collapse will clearly depend upon the history of a signif-icant injury, as well as the imaging features. Simple vertebralfractures with no underlying cause will have the same imagingfeatures as wedge and burst fractures in adults. Features sug-gestive of simple trauma include marrow oedema involvingmainly the upper aspect of the vertebral body with someresidual fatty marrow, relative sparing of the pedicles,retropulsion of the posterior superior cortex and the absenceof a surrounding soft tissue mass.

The relative prevalence of the different causes of acquiredpaediatric vertebral collapse is difficult to determine, since itwill be highly dependent upon the specialist nature of thetreating centre. Our experience is in the setting of a tertiaryreferral spinal orthopaedic setting, in which case chronicrecurrent multifocal osteomyelitis (CRMO) is anecdotallythe commonest cause.

Infective causes

Pyogenic osteomyelitis

Pyogenic osteomyelitis is an uncommon presentation of boneinfection, which may be due to almost any infectious patho-gen. The most common agent is Staphylococcus sp., but othercommon organisms include H. influenza, Salmonella sp.,E. coli and Neisseria gonorrhoeae. The infection classicallyreaches the spine via haematogenous spread within the venousplexus, although direct inoculation, such as accidental expo-sure during surgery is also seen. Sites of infection are classi-cally in the lumbar spine, with the cervical and thoracicvertebrae less commonly affected.

Unlike adults, in which the vertebral end plate is closed, theend plate allows the passage of infection through to theadjacent disc space. As such, infection may be seen to passfrom an affected vertebral body to a contiguous vertebralbody.

Plain radiographs may show a reduction in vertebral bodyheight. The preponderance is for anterior vertebral body in-volvement (Fig. 1a). MRI shows variable fluid within the discspace, which may show loss of height, erosion of the end-plates and extensive marrow oedema-like SI within the adja-cent vertebral bodies (Fig. 1b, c). Disc and vertebral enhance-ment will be seen following contrast agent administration(Fig. 1d). Depending upon the chronicity of the lesion,there may also be associated sub-chondral marrow scle-rosis, best appreciated with CT (Fig. 1e). With adequateantibiotic therapy, marrow SI will return to normal(Fig. 1f). Other characteristic features of pyogenicdiscitis include epidural extension deep to the posteriorlongitudinal ligament (PLL; Fig. 2).

Tuberculous osteomyelitis

Tuberculous infection of the spine (Pott’s disease) is an un-common manifestation of tuberculosis (TB), but where theskeleton is affected, roughly half will have spinal disease [11].It is most commonly seen in children in endemic areas.Classically occurring through haematogenous spread, the or-igin is frequently unknown, but approximately 50 % of chil-dren will have co-existing pulmonary involvement.

Fig. 5 Ewing sarcoma of C4 in a 15-year-old boy. Lateral radiographshowing lytic collapse of C4 with mild focal kyphosis (arrow)

Fig. 6 Aneurysmal bone cyst (ABC) in a young boy. Antero-posterior(AP) radiograph of the lumbar spine showing collapse of the left side ofthe L3 vertebral body (arrows) with absence of the left pedicle

Skeletal Radiol (2014) 43:423–436 427

Infection typically affects the anterior vertebral endplatewith granulomatous disease predominating in the early phase.Expansion of the lesion destroys the trabeculae and may leadto anterior collapse and subsequent wedging. Infective focican erode through the cortex to form sub-ligamentous andsoft-tissue abscesses.

Kyphosis due to anterior wedging is a common feature(Fig. 3) [11]. The thoracic spine is the most common site ofdisease, followed by the lumbar and cervical spine. The diseaseis normally contiguous, but can occur at multiple non-contiguous levels, necessitating routine MR imaging of thewhole spine [8, 9].

Tuberculous lesions show low SI on T1W imaging(Fig. 4a) and appear hyperintense on T2W (Fig. 4b) and STIRsequences. Unlike other forms of discitis, disc spacenarrowing is uncommon and preservation aids diagnosis(Fig. 4a, b). Involved discs may show increased signal onT2W sequences, which can be difficult to identify in thepaediatric spine where the discs have a naturally high signal.

Differentiating between tuberculous and pyogenic infec-tions is aided by peripheral enhancement with gadolinium inTB, as opposed to more diffuse enhancement in pyogenicinfection. Other classical features include large anteriorsub-ligamentous and psoas abscesses (Fig. 4c, d). Fur-thermore, infection involving the posterior elements is a

characteristic feature of tuberculosis (Fig. 4d) [12, 13].TB may also produce vertebra plana [9]. Diffusion-weighted imaging has been found to have poor specificity(66 %) at distinguishing between tuberculosis and pyogenicosteomyelitis [14]

Neoplastic causes

Primary vertebral tumours are rare in children, with only onestudy demonstrating 8 affected children out of 1,971 cases ofmusculoskeletal tumours [15]. Nevertheless, it is important torecognise this group, as management is dependent on appro-priate diagnosis and referral.

Ewing sarcoma

Ewing sarcoma is the second most common malignant prima-ry spinal tumour in children, peaking at the age of 15 yearsand commonly presenting with a combination of pain andneurological deficit. The tumour typically arises unilaterallyin the neural arch with secondary involvement of the vertebralbody [16]. A large extra-osseous mass is usually seen at thetime of presentation, which may impinge on the canal causingneurological symptoms. Imaging features are of an expansile

Fig. 7 ABC of L2 in a 17-year-old girl. a AP radiograph shows lyticcollapse of the left side of the L2 vertebral body with absence of the leftpedicle (arrow) and a right thoraco-lumbar scoliosis. b Axial CT imageshowing the extensive vertebral destruction and the thinned expandedresidual vertebral and neural arch cortex (arrows). cCorresponding axial

T1W SE MR image shows a lesion with heterogeneous internal SIcharacteristics (arrows). d Axial T2W FSE MR image demonstrates alow SI rim (arrows) and multiple fluid–fluid levels (arrowheads). eAxialpost-contrast T1W SE MR image shows subtle septal enhancementthroughout the lesion

428 Skeletal Radiol (2014) 43:423–436

lesion with cortical thickening and destruction, which is pre-dominantly lytic, with a minority showing a mixed appear-ance. In rare cases the tumour may arise within the vertebralbody itself (Fig. 5). Tumour extension into the spinal canal iscommon with up to 91 % affected in some series [16, 17].Metastases are to the lungs, bones and regional lymph nodes,often at the time of diagnosis [18].

Aneurysmal bone cyst

Aneurysmal bone cysts (ABC) of the spine are benign, slowlygrowing, highly vascular lesions that predominantly affect theposterior elements unilaterally, but commonly extend into thevertebral body, where they may cause partial collapse or evenvertebra plana [19]. ABCs may be primary in origin or sec-ondary to other lesions such as osteoblastoma, giant celltumour, chondroblastoma or fibrous dysplasia. They are twiceas common in female subjects and usually present in thesecond decade of life. They are the second most commonvertebral neoplasm [19]. Radiographs classically demonstratea lytic lesion producing asymmetrical vertebral collapse

owing to extension of the lesion from the neural arch intoone side of the vertebral body, the absence of the ipsilateralpedicle being a classical feature (Fig. 6). The degree ofcollapse may be enough to produce a structural scoliosis(Fig. 7a). CT may show an “egg-shell” appearance ofthinned expanded vertebral cortex (Fig. 7b). MRI dem-onstrates a characteristic, multi-loculated lesion withheterogeneous internal SI on T1W (Fig. 7c). A sur-rounding low SI rim may be evident on T2W sequences(Fig. 7d), which also classically demonstrate multiplefluid–fluid levels (Fig. 7d), representing sedimentationof blood products from recurrent haemorrhage withinthe cysts. Enhancing septae may be seen separating thefluid-filled cavities (Fig. 7e) [19]. Neurological compromiseand cord compression have been associated with these lesions[20].

Fibrous dysplasia

Fibrous dysplasia of the spine may be monostotic orpolyostotic and can be seen as part of syndromes such as

Fig. 8 Polyostotic fibrousdysplasia of the spine in a13 year-old girl. aAP radiographof the thoraco-lumbar junction,which shows collapse of themildly sclerotic T11 vertebralbody. Note also the involvementof the adjacent left 11th rib(arrows). bAxial and c sagittalCT MPR showing medullarysclerosis due to the ground glassmatrix (arrows). A further lesionis seen in the inferior aspect of L1,which shows the classical thicksclerotic margin (short arrow),the “rind sign”

Skeletal Radiol (2014) 43:423–436 429

McCune–Albright. It most commonly affects thethoracolumbar region [21]. Spinal deformity is typically asso-ciated with the polyostotic form, which will also affect otherparts of the skeleton [22]. In particular, involvement of theadjacent rib is a relatively common feature, which shouldsuggest the diagnosis (Fig. 8a). The monostotic form is ex-tremely rare and not commonly associated with collapse [21,23]. Plain radiographs demonstrate a ground glass appearance,which may involve both the vertebral body and the posteriorelements, and is optimally demonstrated on CT (Fig. 8b, c).Focal lesions may have a thick sclerotic margin, the “rind-sign” (Fig. 8c). MRI shows reduced SI on both T1- and T2-weighted sequences representing the fibrous component, orareas of fluid SI when there is associated cystic degeneration.Technetium 99 m MDP bone scan will normally dem-onstrate increased uptake in the affected regions of the bone[21, 23–25].

Osteosarcoma

Osteosarcoma may develop as a primary lesion or secondaryto spinal irradiation. Primary osteosarcoma is the most

common primary malignant tumour of the spine in adults(excluding myeloma) and the eighth in children, with casesreported in children aged as young as 8 years [26]. The lesiontypically involves the posterior elements, extending second-arily into the posterior vertebral body causing collapse, al-though less typically the lesion may be confined to the poste-rior body alone. Radiography and CTwill frequently demon-strate mineralised matrix secondary to the deposition of oste-oid. This may be extensive, producing an “ivory vertebra”appearance. In approximately 20 % of cases, the process mayappear purely lytic (Fig. 9a). Extension into the vertebral canalis a common feature (Fig. 9b, c) [27].

Acute lymphoblastic leukaemia/lymphoma

Acute lymphoblastic leukaemia/lymphoma (ALL) is a veryrare cause of spinal collapse. Analysis by Meehan et al. foundonly 16 cases among 615 patients with leukaemia [28, 29].This is most commonly seen in children under 5 years old,although cases are seen up to the second decade. Features ofALL include osteopenia, which can result in vertebral collapsein the absence of malignant marrow infiltration, and therefore

Fig. 9 Osteosarcoma of T12 in a16-year-old boy. aCoronal CTMPR shows lytic destructionand collapse of the right side ofthe vertebral body with a smallparavertebral soft tissue mass(arrows). bSagittal T2W FSE andc axial T1W SE MR imagesdemonstrate diffuse vertebralmarrow infiltration, pathologicalcollapse and extra-osseousextension causing compressionof the conus

430 Skeletal Radiol (2014) 43:423–436

Fig. 10 B-cell lymphoma in a14-year-old boy. a Lateralradiograph of the lumbar spineshowing diffuse osteopenia andmild collapse of T12 (arrow).b Sagittal T1W SE MR imageof the cervico-thoracic spineshowing mildly hyperintenseintervertebral discs indicativeof diffuse vertebral marrowinfiltration and multilevelvertebral collapse (arrows). cCoronal STIR MR image of thesacrum showing diffuse marrowhyperintensity

a cbFig. 11 Osteosarcoma metastasisof L3 in a 17-year-old boy. aLateral radiograph showingdiffuse osteopenia mild anteriorwedging of the L3 vertebra(arrow). b Sagittal T1W SE and caxial T2W FSE MR imagesdemonstrate a hypointensemetastasis in the anterior half ofthe vertebral body (arrows)

Skeletal Radiol (2014) 43:423–436 431

no evident SI change in the vertebral bodies on MRI, whilemarrow involvement results in diffuse, homogenously lowT1Wand increased STIR SI 30–32]. There may be restorationof normal vertebral height through bone remodelling follow-ing treatment [32]. Similar appearances will be seen in child-hood lymphoma (Fig. 10).

Paediatric lymphomamay be primary or secondary. Primarylymphoma accounts for approximately 2–6 % of primary bonetumours in children, but more typically involves the longbones. Secondary lymphoma is more commonly non-Hodgkins than Hodgkins (20 % vs 1–5 %) [33, 34]. Lympho-ma may produce sclerotic or lytic lesions that may involve thewhole vertebra and may have low T1W SI on MRI with avariable appearance on T2W [35–37].

Metastases

Metastatic disease is less common in the paediatric populationthan in adults and has a different range of primary lesions, themost common being Ewing sarcoma and neuroblastoma,osteosarcoma, rhabdomyosarcoma, Hodgkin's disease, softtissue sarcoma and germ cell tumour. [38]

Vertebral metastases in children may derive from primarybone or soft tissue tumours. Secondary deposits from primaryspinal bone tumours are also well recognised with Ewingsarcoma among the most common. These are associated witha poorer prognosis [39]. Metastases from extraosseous pri-maries include neuroblastoma and astrocytoma. Metastasesare typically of low signal on T1W sequences replacingnormal marrow and can be variable on T2 and STIRsequences [40] (Fig. 11). Whole-body MRI has been

shown to have greater sensitivity than conventional imagingfor bony metastases [7].

Miscellaneous causes

Metabolic cause: idiopathic osteoporosis

Idiopathic osteoporosis is a rare condition that most common-ly presents in pre-pubescent children. It is characterised bybone demineralisation, but patients can be biochemically nor-mal. Clinical features include pain in the spine and limbs, gaitdisturbance and muscle weakness. Radiographs demonstrateosteopaenia and may show vertebral collapse at multiplelevels (Fig. 12). Metaphyseal compression in long bones hasalso been described and DEXA imaging will typically show

a b

Fig. 12 Idiopathic osteoporosis in a 16-year-old boy. a Lateral radio-graph of the lumbar spine showing generalised osteopaenia and severemultilevel vertebral collapse (arrows). b Sagittal T2W FSE MR imagedemonstrates multilevel vertebral collapse with biconcave end-plates andsecondary expansion of the intervertebral discs

a b

c dd

Fig. 13 Langerhans cell histiocytosis (LCH). A 14-year-old boy withbiopsy-proven LCH of C7. a Lateral cervical spine radiograph showingirregular, lytic vertebral collapse with anterior wedging and focal kypho-sis. b Follow-up lateral radiograph shows progression to the vertebraplana. c Sagittal T1W SE and d T2W FSE MR images showing reducedT1Wand increased T2Wmarrow SI. Note the maintenance of the corticalsignal void of the C7 vertebral end-plates, the preservation of the adjacentdiscs and the extra-osseous extension (arrows)

432 Skeletal Radiol (2014) 43:423–436

reduced Z scores [41]. It is a disease of exclusion once othermetabolic and genetic causes have been ruled out. Preventionof further vertebral fractures is the key to management, as thecondition typically resolves spontaneously with time [42].

Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) produces eosinophilicgranulomata within bone. These may be solitary or multiple,as in Hand–Schüller–Christian and Letterer–Siwe disease andmay involve the spine. It is more common in male subjectsand in particular in children under 5 years of age. The diseasecharacteristically affects the vertebral bodies, often singly, butsometimes at multiple levels, typically sparing the posterior

elements and may cause anterior wedging (Fig. 13a) [43]. It isone of the leading causes of pathological vertebral collapse inchildren, which may be so pronounced as to cause a vertebraplana (Fig. 13b). The disc spaces on either side of the col-lapsed vertebra are normally preserved, a feature seen clearlyon MRI. Resolution of the disease can lead to restoration ofvertebral body height [44]. Lesions are lytic and poorly de-fined on radiography and CT. MRI shows reduced marrow SIon T1W (Fig. 13c), increased SI on T2W (Fig. 13d) and STIR,and avid enhancement with gadolinium representing thehypervascular healing process. The T2/STIR hyperintensity

a b c

Fig. 14 Chronic recurrent multifocal osteomyelitis (CRMO). a Lateralradiograph of the thoracic spine in an 11-year-old girl showing collapse ofthe T8 vertebra (arrow). bLateral radiograph of the thoracic spine in a 16-year-old girl showing collapse of the T9 vertebra with marked loss of

vertebral height and diffuse medullary sclerosis (arrow). cAxial CT in thesame patient as in bconfirms the medullary sclerosis in the vertebral bodywith extension into both pedicles

a b

Fig. 15 CRMO in a 16-year-old girl. aSagittal T1W SE and bT2W FSEMR images showing severe collapse at the T9 level with marrow sclerosis(same case as in Fig. 2b and c), but also multilevel involvement manifestby the presence of oedema-like marrow SI (arrows). Note the absence ofany soft-tissue extension

a b

Fig. 16 CRMO in an 11-year-old girl. a Sagittal T1W SE and b T2WFSE MR images showing moderate collapse at the T8 level with mildreduction of marrow SI and bulging of the posterior vertebral body cortex(same case as in Fig. 2a), but also multilevel involvement manifest by thepresence of end-plate collapse and fatty marrow SI (arrows) indicatinghealed lesions

Skeletal Radiol (2014) 43:423–436 433

reduces as the lesion heals. Soft-tissue extension may also beseen on MRI [43, 44]. CT-guided biopsy is frequently used toaid diagnosis. Tc99m bone scans are helpful to identify mul-tilevel disease, but cannot reliably differentiate active fromhealing lesions, as both will show increased activity. There-fore, in the setting of multilevel disease, MRI can aid indeciding which vertebra to biopsy based on the presence ofmarrow oedema at an active level [8, 45].

Chronic recurrent multifocal osteomyelitis

Chronic recurrent multifocal osteomyelitis (CRMO) is a con-dition of undetermined aetiology characterised by areas ofsterile (non-bacterial) osteomyelitis [46]

Spinal CRMO is rare, representing approximately 3 % oflesions [16, 47]. Despite this, the spine is the most commonsite of pathological fracture, usually affecting the thoracicregion [16, 47, 48]. Lesions may range from lytic to scleroticand mixed lesions are not uncommon. Radiographs may showa lytic lesion with a sclerotic border (Fig. 14a) or a completelysclerotic lesion, possibly indicating an advanced stage of thedisease process (Fig. 14b). The sclerotic nature of a lesion willbe optimally demonstrated on CT (Fig. 14c) and may be

confined to the vertebral body or may extend into the posteriorelements. MRI shows altered marrow signal with reduced SIon T1W sequences (Fig. 15a). T2W hyperintensity is seen inacute disease (Fig. 15b). Typically, changes are confined toone or more vertebrae without crossing the adjacent discs orcontiguous vertebrae, a feature that differentiates from bacte-rial osteomyelitis. Vertebral collapse may lead to vertebraplana, but unlike LCH, this does not recover following treat-ment [48–51]. The absence of extra-osseous extension alsoaids in differentiation from LCH. Healed lesions show avariable degree of collapse, but normal marrow SI, althoughthe marrow may become hyperintense on T1W owing to thereplacement of red marrow with yellow marrow (Fig. 16).Depending upon the degree of collapse, significant kyphosiscan develop (Fig. 17) [51]. Multilevel disease is not uncom-mon, and the finding that healed lesions manifest by mildvertebral collapse and T1W hyperintensity at the time ofpresentation with a new vertebral collapse, is a very usefuldiagnostic clue.

Imaging with isotope bone scans or, where available,whole-body MRI is useful as it allows the identification offurther foci of disease, particularly since these may not besymptomatic on initial presentation. Whole-body MRI alsoaids follow-up and response to treatment [47, 48, 52]. Thecombination of clinical and imaging features usually allows aconfident diagnosis without the requirement for needlebiopsy.

Conclusion

Acquired vertebral collapse in the paediatric population rep-resents a short differential of conditions, which may appearvery similar on imaging, but have individual features that canaid differentiation, as described above. However, in manycases, biopsy may be required to confirm the diagnosis. Inall cases, referral for expert review in a specialist PaediatricOncology Centre is recommended.

Conflict of interest No conflict of interest.

References

1. Wiley AM, Trueta J. The vascular anatomy of the spine and itsrelationship to pyogenic vertebral osteomyelitis. J Bone Joint SurgBr. 1959;41:796–809.

2. Crock HV, Yoshizawa H. The blood supply of the lumbar vertebralcolumn. Clin Orthop Relat Res. 1976;115:6–21.

3. Coman DR, Delong R. The role of the vertebral venous system in themetastasis of cancer to the spinal column. Cancer. 1951;4:610.

4. Foster K, Chapman S, Johnson K. MRI of the marrow in the paedi-atric skeleton. Clin Radiol. 2004;59:651–7.

Fig. 17 CRMO in an 11-year-old girl. Sagittal T2W FSE MR imageshowing multilevel mid-thoracic involvement with severe collapse of T6resulting in marked focal kyphosis

434 Skeletal Radiol (2014) 43:423–436

5. OrdingMüller L, Avenarius D, Olsen O. High signal in bone marrowat diffusion-weighted imaging with body background suppression(DWIBS) in healthy children. Pediatr Radiol. 2011;41:221–6.

6. GooH, YangD, RaY, Song J, ImH, Seo J, et al. Whole-bodyMRI ofLangerhans cell histiocytosis: comparison with radiography and bonescintigraphy. Pediatr Radiol. 2006;36(10):1019–31.

7. Siegel M, Acharyya S, Hoffer F, Wyly J, Friedmann A, Snyder B,et al. Whole-body MR imaging for staging of malignant tumors inpediatric patients: results of the American College of RadiologyImaging Network 6660 Trial. Radiology. 2013;266(2):599–609.doi:10.1148/radiol.12112531.

8. Ferreira E, Brito C, Domingues R, Bernardes M, Marchiori E,Gasparetto E. Whole-body MR imaging for the evaluation ofMcCune-Albright syndrome. J Magn Reson Imaging. 2010;31(3):706–10.

9. Rajeswaran G, Malik Q, Saifuddin A. Image-guided percutaneousspinal biopsy. Skeletal Radiol. 2013;42(1):3–18. doi:10.1007/s00256-012-1437-y.

10. Curtis C, d'Hemecourt P. Diagnosis and management of back pain inadolescents. Adolesc Med State Art Rev. 2007;18:140–64.

11. Naim-ur-Rahman. Atypical forms of spinal tuberculosis. J Bone JointSurg Br. 1980;62:162–165.

12. Andronikou S, Jadwat S, Douis H. Patterns of disease on MRI in 53children with tuberculous spondylitis and the role of gadolinium.Pediatr Radiol. 2002;32:798–805.

13. Polley P, Dunn R. Noncontiguous spinal tuberculosis: incidence andmanagement. Eur Spine J. 2009;18:1096–101.

14. Pui MH, Mitha A, Rae WID, Corr P. Diffusion-weighted magneticresonance imaging of spinal infection and malignancy. JNeuroimaging. 2005;15:164–70. doi:10.1111/j.1552-6569.2005.tb00302.x.

15. Kaila R, Malhi AM, Mahmood B, Saifuddin A. The incidence ofmultiple level non-contiguous vertebral tuberculosis detected usingwhole spine MRI. J Spinal Disord Tech. 2007;20:78–81.

16. Koslowski K, Bellufi G, Masel J, Dosseur P, Labatut J. Primaryvertebral tumours in children: report of 20 cases with brief literaturereview. Paediatr Radiol. 1984;14:129–39.

17. Grubb M, Currier B, Prictard D. Primary Ewing's sarcoma of thespine. Spine. 1994;19:309–13.

18. Prichtard D, Dahlin D, Dauphine R. Ewing's sarcoma: a clinicopath-ologic analysis of patients surviving 5 years or longer. J Bone JointSurg. 1975;57A:10–6.

19. Caro RA, Mandell GA, Stanton RP. Aneurysmal bone cyst of thespine in children. MRI imaging at 0.5 tesla. Pediatr Radiol. 1990;21:114–6.

20. Chan M, Wong Y, Yuen M, Lam D. Spinal aneurysmal bone cystcausing acute cord compression without vertebral collapse, CT andMRI findings. Pediatr Radiol. 2002;32:601–4.

21. Troop JK, Herring JA. Monostotic fibrous dysplasia of the lumbarspine: case report and review of the literature. J Pediatr Orthop.1988;8:599–601.

22. Mancini F, Corsi A, De Maio F, Riminucci M, Ippolito E. Scoliosisand spine involvement in fibrous dysplasia of bone. Eur Spine J.2009;1:196–202.

23. Inamo Y, Hanawa Y, Kin H, Okuni M. Findings on magnetic reso-nance imaging of the spine and femur in a case of McCune-Albrightsyndrome. Pediatr Radiol. 1993;23:15–8.

24. Leet A, Magur E, Lee J, Wientroub S, Robey P, Collins M. Fibrousdysplasia in the spine: prevalence of lesions and association withscoliosis. J Bone Joint Surg Am. 2004;86-A:531–7.

25. Yang C, Zhu B, Chen A. Imaging diagnosis of monostotic fibrousdysplasia in thoracic and lumbar spine vertebrae. J Huazhong UnivSci Technolog Med Sci. 2007;27:684–6.

26. Ottaviani G, Jaffe N. The epidemiology of osteosarcoma. In: Jaffe Net al., editors. Pediatric and Adolescent Osteosarcoma. New York:Springer; 2009. doi:10.1007/978-1-4419-0284-9_1.

27. Ilaslan H, Sundaram M, et al. Primary vertebral osteosarcoma: im-aging findings. Radiology. 2004;230:697–702.

28. Meehan PL, Viroslav S, Schmitt Jr EW. Vertebral collapse in child-hood leukemia. J Pediatr Orthop. 1995;15(5):592–5.

29. Carrière B, Cummins-Mcmanus B. Vertebral fractures as initial signsfor acute lymphoblastic leukemia. Pediatr Emerg Care. 2001;17:258–61.

30. PandyaNA,Meller ST,MacVicar D, Atra A, Pinkerton CR. Vertebralcompression fractures in acute lymphoblastic leukaemia and remod-elling after treatment. Arch Dis Child. 2001;85:492–3.

31. El Saleeby CM, Grottkau BE, Friedmann AM, Westra SJ, SohaniAR. Case records of the Massachusetts general hospital. Case 4-2011. A 4-year-old boy with back pain and hypercalcemia. N EnglJ Med. 2010;364:552–62.

32. Parker BR, Castellino RA. Skeletal involvement in non-hodgkinslymphoma in paediatric oncologic radiology. St Louis: Mosby;1977. p. 200–2.

33. Parker BR, Castellino RA. Skeletal involvement in hodgkins diseasein paediatric oncologic radiology. St Louis: Mosby; 1977. p. 177–8.

34. Edeiken-Monroe B, Edeiken J, Kim EE. Radiologic concepts oflymphoma of bone. Radiol Clin North Am. 1990;28:841–64.

35. Hicks DG, Gokan T, O’Keefe RJ, et al. Primary lymphoma of bone:correlation of magnetic resonance imaging features with cytokineproduction by tumor cells. Cancer. 1995;75:973–80.

36. Stiglbauer R, Augustin I, Kramer J, Schurawitzki H, Imhof H,Radaszkiewicz T. MRI in the diagnosis of primary lymphoma ofbone: correlation with histopathology. J Comput Assist Tomogr.1992;16:248–53.

37. Traill Z, Richards MA, Moore NR. Magnetic resonance imaging ofmetastatic bone disease. Clin Orthop Relat Res. 1995;312:76–88.

38. Mukherjee D, Chaichana KL, Gokaslan ZL, Aaronson O, Cheng JS,McGirt MJ. Survival of patients with malignant primary osseousspinal neoplasms: results from the surveillance, epidemiology, andend results (SEER) database from 1973 to 2003. J Neurosurg Spine.2011;14:143–50.

39. Sinha AK, Seki JT, Moreau G, Ventureyra E, Letts RM. The man-agement of spinal metastasis in children. Can J Surg. 1997;40:218–26.

40. Chlebna-SokółD, Loba-Jakubowska E, Sikora A. Clinical evaluationof patients with idiopathic juvenile osteoporosis. J Pediatr Orthop B.2001;10:259–63.

41. Dimar JR, Campbell M, Glassman SD, Puno RM, Johnson JR.Idiopathic juvenile osteoporosis. An unusual cause of back pain inan adolescent. Am J Orthop (Belle Mead NJ). 1995;24:865–9.

42. Sartoris DJ, Parker BR. Histiocytosis X: rate and pattern of resolutionof osseous lesions. Radiology. 1984;152:679–84.

43. Bavbek M, Atalay B, Altinors N, et al. Spontaneous resolution oflumbar vertebral eosinophilic granuloma. Acta Neurochir (Wien).2004;146:165–7.

44. Kaplan GR, Saifuddin A, Pringle JA, Noordeen MH, Mehta MH.Langerhans’ cell histiocytosis of the spine: use of MRI in guidingbiopsy. Skeletal Radiol. 1998;27:673–6.

45. Gikas PD, Islam L, Aston W, et al. Nonbacterial osteitis: a clinical,histopathological, and imaging study with a proposal for protocol-based management of patients with this diagnosis. J Orthop Sci.2009;14:505–16.

46. Jurriaans E, Singh NP, Finlay K, Friedman L. Imaging of chronicrecurrent multifocal osteomyelitis. Radiol Clin North Am. 2001;39:305–27.

47. Langendoerfer M, von Kalle T, Maier J, Dannecker GE. Spinalinvolvement in chronic recurrent multifocal osteomyelitis (CRMO)in childhood and effect of pamidronate. Eur J Pediatr. 2010;169:1105–11.

48. Yu L, Kasser J, O’Rourke E, Kozakewich H. Chronic recurrentmultifocal osteomyelitis: association with vertebra plana. J BoneJoint Surg Am. 1989;71:305–6.

Skeletal Radiol (2014) 43:423–436 435

49. Jansson A, Renner ED, Ramser J, Mayer A, Haban M, Meindl A,et al. Classification of non-bacterial osteitis: retrospective study ofclinical, immunological and genetic aspects in 89 patients.Rheumatology (Oxford). 2007;46:154–60.

50. Khanna G, Sato T, Ferguson P, et al. Imaging of chronic recurrentmultifocal osteomyelitis1. Radiographics. 2009;29:1159–77.

51. Fritz J, TzaribatchevN, et al. Chronic recurrent multifocal osteomyelitis:comparison of whole-body MR imaging with radiography and correla-tion with clinical and laboratory Data1. Radiology. 2009;252:842–51.

52. Haydar AA, Gikas P, Saifuddin A. Case report: chronic recurrentmultifocal osteomyelitis: a case report and role of whole-body MRI.Clin Radiol. 2009;64:641–4.

436 Skeletal Radiol (2014) 43:423–436