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Abstract Adult scoliosis is definedas a spinal deformity in a skeletallymature patient with a Cobb angle ofmore than 10_ in the coronal plain.Adult scoliosis can be separatedinto four major groups: Type 1:Primary degenerative scoliosis,mostly on the basis of a disc and/orfacet joint arthritis, affecting thosestructures asymmetrically withpredominantly back pain symptoms,often accompanied either by signsof spinal stenosis (central as well aslateral stenosis) or without. Thesecurves are often classified as ‘‘denovo’’ scoliosis. Type 2: Idiopathicadolescent scoliosis of the thoracicand/or lumbar spine whichprogresses in adult life and isusually combined with secondarydegeneration and/or imbalance.Some patients had either no surgicaltreatment or a surgical correctionand fusion in adolescence in eitherthe thoracic or thoracolumbar spine.Those patients may developsecondary degeneration andprogression of the adjacent curve;in this case those curves belong tothe type 3a.Type 3: Secondary adultcurves: (a) In the context of anoblique pelvis, for instance, due to aleg length discrepancy or hippathology or as a secondary curvein idiopathic, neuromuscular andcongenital scoliosis, or asymmetricalanomalies at the lumbosacraljunction; (b) In the context of ametabolic bone disease (mostlyosteoporosis) combined withasymmetric arthritic disease and/orvertebral fractures. Sometimes it isdifficult to decide, what exactly theprimary cause of the curve was,

once it has significantly progressed.However, once an asymmetric loador degeneration occurs, thepathomorphology andpathomechanism in adult scoliosispredominantly located in the lumbaror thoracolumbar spine is quitepredictable. Asymmetricdegeneration leads to increasedasymmetric load and therefore to aprogression of the degeneration anddeformity, as either scoliosis and/orkyphosis. The progression of acurve is further supported byosteoporosis, particularly in post-menopausal female patients. Thedestruction of facet joints, jointcapsules, discs and ligaments maycreate mono- or multisegmentalinstability and finally spinalstenosis. These patients presentthemselves predominantly with backpain, then leg pain and claudicationsymptoms, rarely with neurologicaldeficit, and almost never withquestions related to cosmetics.The diagnostic evaluation includesstatic and dynamic imaging,myelo-CT, as well as invasive diagnosticprocedures like discograms,facetblocks, epidural and root blocks andimmobilization tests. These testsmay correlate with the clinical andthe pathomorphological findingsand may also offer the least invasiveand most rational treatment

REVIEWReprintEur Spine J (2005) 14: 925–948DOI 10.1007/s00586-005-1053-9

Max Aebi The adult scoliosisThe adult scoliosisThe adult scoliosisThe adult scoliosisThe adult scoliosis

Received: 26 October 2005Accepted: 26 October 2005Published online:18 November 2005Springer-Verlag 2005

Keywords Adult scoliosis .Degenerative scoliosis . Spinalstenosis . Adult deformity .Secondary scoliosis

M. AebiInstitute for Evaluative Research in OrthopaedicSurgery, University of Bern, Bern, Switzerland

M. AebiDepartment of Orthopaedics,Hirslanden-Salem Hospital,Stauffacherstrasse 78, 3014 Bern,SwitzerlandE-mail: max.aebi@MEMcenter.unibe.chTel.: +41-31-6315930Fax: +41-31-6315931

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Nota do Editor:Republicação autorizada peloEditor do European Spine Journal,em 08 de Junho de 2006.

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Introduction

Twenty-five years ago, a book chapter about scoliosis with specialemphasis on the adult and/or degenerative scoliosis wasrelatively small [5, 11, 20, 43, 53, 62, 64]. Most of the pages weredevoted to scoliosis in childhood and adolescence. Only theintroduction of spinali instrumentation, first Harrington rods andDwyer instrumentation,and later Zielke, and finally CD-instrumentation with all the following third generation pedicleinstrumentations,shifted the focus to the major problem of theadult scoliosis [1, 2, 8, 13, 23, 27, 32, 35, 38, 40, 45, 58, 59, 62]. Thisdisorder has been known for some time, but only a very fewsurgeons dealt with it. Patients were in an age group which wasconsidered to be too risky to undergo major spine surgery; thesurgical technical issues were widely unsolved due to the lack ofpowerful instrumentation; the bone stock was considered toopoor for a major corrective surgery; and the patients weregenerally made to believe that they had to live with this ailment.

Progress in surgical techniques and technology issignificantly supported by progress in anaesthesia for spinalsurgery and by more sophisticated and precise diagnosticimaging and differentiated application of invasive andfunctional diagnostic tests. Increased patient awareness,the patient’s unwillingness to accept their limitations andpains [54], and the gradual shift in the demographics towardsa ‘‘grey society’’, make adult scoliosis with all of its differentforms and clinical presentations,a much more frequentproblem in a general spine practice than the scoliosis ofchildren and adolescents. This trend is likely to continuewhen we consider the fact that in 25 years from now, asignificant part (more than 10%) of the population in theindustrialized societies will be over 65 years old.

Classification

A scoliosis is diagnosed in adult patients when it occursor becomes relevant after skeletal maturity with a Cobbangle of more than 10° in the frontal plain [1, 55].

Type 1: Primary degenerative scoliosis (‘‘de novo’’ form),mostly located in the thoracolumbar or lumbar spine [6,19,20 24, 25 27, 33, 43, 48, 52, 53].Type 2: Progressive idiopathic scoliosis in adult life ofthe thoracic, thoracolumbar, and/or lumbar spine [5, 8, 36,42, 46, 61, 71, 72].Type 3: Secondary degenerative scoliosis.

(a) Scoliosis following idiopathic or other forms of scoliosisor occurring in the context of a pelvic obliquity due to aleg length discrepancy, hip pathology or a lumbosacraltransitional anomaly, mostly located in thethoracolumbar, lumbar or lumbosacral spine [11, 24, 34,44, 50, 64].

(b) Scoliosis secondary to metabolic bone disease (mostlyosteoporosis) combined with asymmetric arthriticdisease and/or vertebral fractures [10, 15, 29, 51, 70].

Therefore, scoliosis can be present since childhood oradolescence and become progressive and/or symptomaticin adult life; or scoliosis may appear ‘‘de novo’’ in adult lifewithout any precedence in earlier life.

Clinically, the most prominent groups are secondary (type 3)and primary (type 1) degenerative adult scoliosis. In elderlypatients, both forms of scoliosis may be aggravated byosteoporosis, which also holds true for the type 2 scoliosis[24, 29, 70]. All three types of scoliosis may primarily appearat a certain stage as degenerative scoliosis, and degenerativescoliosis is therefore the main bulk of adult scoliosis.Beyond the proposed classification, the degenerative adultscoliosis could also be subdivided into scoliosis which havetheir aetiology in the spine itself and those scoliosis withthe aetiology beyond the spine (Table 1). Schwab etal.proposed recently a radiographic classification includingtype I–III scoliosis, characterized by the a/p and lateral viewin standing position. They correlated the classification I–IIIwith increasing severity of self-reported pain and disability[55]. Boachie-Adjei [8] considers specifically the idiopathicadult scoliosis (our type 2 scoliosis) and uses the age as aclassifying criteria combined with degenerated changes:patients with idiopathic adult scoliosis below and above 40years of age.

This review will concentrate on the forms of adultscoliosis which present themselves most frequently in aspinal practice and which are considered in theabovepresented classification.

Type 1 scoliosis: the primary degenerative scoliosis(‘‘de novo’’ scoliosis) (Fig. 1)

The primary degenerative curve develops mostly on thegrounds of primarily limited disc degeneration in one ormore motion segments. This curve also could be termed‘‘discogenic curve’’ and is basically the result of anasymmetric degenerative change of the disc with theconsecutive development of a frontal deviation andconcomitant rotation with the facet joints on one side as apivot (Fig. 1). The apex of this curve is usually between L3and L4 or L2 and L3 or, second most frequent,between L1and L2. These curves tend to go along with a significantrotational translation of the apical vertebra. In some casesthe primary cause of the degenerative process may belocalized in the facet joints where a wide variety of dystrophicformation, malformation, and misalignment can occur. Whenthis occurs at the lumbosacral junction, then the curvebelongs rather to the type 3a curves, following lumbosacralanomalies.

It is difficult to state whether some curves in this groupcould be considered as ‘‘resting’’ idiopathic scoliosis. Thereare obviously curves that only develop in adult life and mayappear like idiopathic scoliotic curves; however, upon closerlook, they rather may have developed on the basis of adegenerated disc. The primarily degenerative curves usuallyare less severe in terms of frontal angulation than the curves

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in secondary degenerative idiopathic scoliosis [24, 25].The primary degenerative scoliosis is therefore mostly alumbar or thoracolumbar curve consisting of a frontal aswell as a sagittal deviation in the form of mostly a flatback or lumbar kyphosis [5, 6, 8, 19,23, 31, 36]. The sagittalmalalignment is usually responsible for the severepostural back pain of the patients. These curves areshorter than the idiopathic curves and— at least in thebeginning—the deformity of the individual vertebral bodyis less expressed than in idiopathic scoliosis [24, 25]. Thisonly occurs as a consequence of erosion and destructionof the endplates and facet joints due to spondylosis and

spondylarthritis. Also, there is a difference in the bonedensity between the primary and secondary degenerativecurves. Spinal stenosis is more often seen in primarydegenerative scoliosis than in secondary degeneratedidiopathic curves [2, 6, 8, 21, 26, 44]. The disc degenerationends up with spondylosis, disc bulging, osteophytes,and facet joint arthritis with hypertrophic capsules,ligamentum flavum, and calcification of these structureswith osteophytes, all on the costs of the space in thespinal canal and foramina, thus contributing to theformation of spinal stenosis, be it a foraminal lateralstenosis or a central stenosis or both (Fig. 2).

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Fig. 1 Type 1 adult scoliosis: de novo scoliosis. a at 33years (8°), b at 50 years (25°), c at 55 years (40°) 928

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Type 2 scoliosis: progressive idiopathic scoliosisin adult life (Fig. 3)

The idiopathic curves and curves with other aetiology ofsecondary degeneration present themselves in a variety offorms, depending on whether these curves have been treatednon-surgically or not at all or whether they have had a fusion,with or without instrumentation, of the main thoracic andthoracolumbar curve [46]. In the latter case the degenerationappears in the adjacent curve and belongs to the type 3acurves (see below) (Figs. 3, 4). In both situations, however,there may be a significant degeneration and deformitypresent in the sagittal as well as in the frontal plain of theshort lumbar curve. The sagittal deformity is almost alwaysexclusively a flat back syndrome or a loss of physiologicallordosis and in extreme situations a real kyphosis. Thedegenerated idiopathic scoliosis mostly in the lumbar and/or thoracolumbar spine is quite frequently combined withspinal stenosis at a relatively young age, specifically in theadjacent lower segment after Harrington instrumentation.This adjacent segmental spinal stenosis, mostly below along fused idiopathic scoliosis, appears about 15–20 yearspost-surgical with Harrington rods(Fig. 5). There are notyet similar long-term results available for cases which havebeen treated with one of the CD-type third generationinstrumentation that allow superior restoration of the sagittalalignment, possibly protecting the spine from developingrapid adjacent segment degeneration [3, 4, 7, 21, 39, 56].

Type 3 scoliosis:

Secondary degenerative scoliosis (Fig. 6)

Adult secondary degenerative scoliosis is mostly locatedin the thoracolumbar and lumbar as well as lumbosacral spine.This scoliosis occurs either with its cause within the spineor outside the spine. Those scoliosis with the cause insideof the spine are either secondary to an adjacent curve, be itan idiopathic, neuromuscular or congenital curve, or it maybe the consequence of a lumbosacral anomaly, specificallywith a hemisacralization (Fig. 6). Scoliosis outside of the

spine is due to pelvic obliquity in the context of a hippathology or a leg length discrepancy (Fig. 7). Thesesecondary curves with the causes outside of the spineprimarily do not have a relevant rotation, however arebasically deviations in the frontal plain. Only over time thereis a translational displacement of vertebras close to the apex.

Adult scoliosis due to bone weakness (Fig. 8)

These deformities are mostly due to metabolic bone diseaseor diseases which have a secondary impact on the strengthof the bone (e.g. Morbus Adison) (Fig. 8). The most frequentcause for a secondary deformity due to metabolic bonedisease is osteoporosis. Owing to bone weakness, theremay be fractures, which create an asymmetric configurationwith expression of either kyphosis or scoliosis or bothtogether. It may also occur when a preexisting scoliosis,respectively kyphosis, is aggravated by an osteoporoticfracture [29, 65, 70].

Pathomorphology and pathomechanism in adultscoliosis

Degenerative adult scoliosis, specifically in the lumbarspine, is characterized by quite a uniform pathomorphologyand pathomechanism. The asymmetric degeneration of thedisc and/or the facet joints leads to an asymmetric loadingof the spinal segment and consequently of a whole spinalarea. This again leads to an asymmetric deformity, forexample, scoliosis and/or kyphosis. Such a deformity againtriggers asymmetric degeneration and induces asymmetricloading, creating a vicious circle (Fig. 9) and enhancing curveprogression. On the one hand, the curve progression isgiven by the pathomechanism of an adult degenerativecurve, and on the other hand by the pecific bone metabolismof the post-menopause female patients with a certain degreeof osteoporosis, who are most frequently affected by thedegenerative form of scoliosis. The potential of individualsymmetric deformation and collapse in the weak osteoporoticvertebra is clearly increased and contributes further to thecurve progression.

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Fig. 2 Secondary changes in degenetarivescoliosis: facet joint hypertrophy,recessal stenosis

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The destruction of discs, facet joints and joint capsulesusually ends in some form of uni- or multisegmental sagittaland/or frontal latent or obvious instability. There may benot only a spondylolisthesis, meaning a slip in the sagittalplain, but also translational dislocations in the frontal plainor rather threedimensionally when expressing itself in arotational dislocation (Figs. 1, 3, 6, 15). The biologicalreaction to an unstable joint or, in the case of the spine, anunstable segment, is the formation of osteophytes atthefacet joints (spondylarthritis) and at the vertebralendplates (spondylosis), both contributing totheincreasing narrowing of the spinal canaltogether with the hypertrophy and calcification ofthe ligamentum flavum and joint capsules, creatingcentral and recessal spinal stenosis [66] (see alsoFig. 2). These pathomorphological andpathomechanical relationships and theirsignificance for the clinical presentation of an adultdegenerative scoliosis are expressed schematicallyin Fig. 10. The osteophytes of the facet joints andthe spondylotic osteophytes, however, may notsufficiently stabilize a diseased spinal segment;such a condition leads to a dynamic, mostlyforaminal stenosis with radicular pain orclaudication type pain (e.g. Fig. 11).

Clinical presentation

Pain

The most frequent clinical problem of adultscoliosis is back pain [3, 6, 19, 31, 56, 73] andpresents itself with a multiform mosaic of

symptoms. Back pain at the site of thecurve can be localized either at the apexor in its concavity, and facet joint paincan be localized in the countercurvefrom below the curve to above the curve.The back pain can be combined withradicular leg pain. It can be theexpression of a muscular fatigue or of areal mechanical instability. Unbalanced,overloaded, and stressed, paravertebralback muscles may become very sore andin return will not contribute to balancethe muscle play, consequently becomingpart of a vicious circle. This is especiallytrue when the lumbar curve isaccompanied by the loss of lumbarlordosis [22]. This muscular pain is ratherdiffuse, distributed over the lower back,and often permanent at the insertion ofthe muscle tendons at the iliac crest,sacrum, os coccys, and bony processesof the spine.

The back pain can be constant andnon-specific, which is a bad prognostic

sign regarding the treatment outcome. The pain, however,can be present only when the patient is upright, especiallywhen standing and sitting, presenting as a so-called axialback pain, or only during certain movements or physicalactivities, pointing rather to a mechanically unstable segmentor a whole spinal region. The patients often indicate thatthey can control their pain well, when lying down flat or ontheir side and when the axial load is taken off the spine.

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Fig. 3 Young female teacher withprogressive idiopathic scoliosis. a At 35years, 62°, b at 38 years progressed to75°, c left bending with some correction, dsignificant lumbar kyphosis, e partialcorrection by extension, f flexion ofthoracic spine, g and h 18 monthspostoperatively after (1) anterior LISSrelease and fusion with beta-TCP(Chronos®) autologous bone mixture and(2) posterior correction and stabilizationand fusion with USS during the sameanaesthesia. Restoration of the lordosis

Fig. 4 a and b Double major idiopathic scoliosis in a 41-year-old femalepatient with increasing back pain in the last few years and subjectiveprogression of the curve. c Long fusion to the sacrum with development ofa non-union and loss of balance after a year postoperatively. d Osteotomyat L2/3, cut of the rod and correction of the malposition by reconnectingthe right cut rod and PLIF at the lumbosacral junction. Now with goodbalance and almost no pain

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Claudication

The second important symptom of adult degenerativescoliosis is radicular pain and claudication symptoms whenstanding or walking [57, 73]. The patient can have trueradicular pain due to a localized compression or root traction(root compromise is not necessarily on the concave sidewhere we may suppose narrowed foramens, but often onthe convex side, rather expressing a dynamic overstretch ofa root). There may, however, be a single- or multilevel spinalstenosis which can be central or more recessal, creatingclaudication symptoms [19, 24, 27, 53, 56]. Root compressions can occur at the bottom of the curve or at thetransition to the sacrum and can be linked to a hypermobilityof an overloaded bottom segment, especially in cases ofstiff curves. Short lumbosacral or lumbar curves ascountercurves to long fused thoracolumbar scoliosis oftenshow a severe spinal stenosis at the transition from the stiffupper spinal area to the lower lumbosacral area (Fig. 11).

Neurological deficit

The third important clinical presentation may manifest itself as a real neurological deficit, including individual roots,several roots, or the whole cauda equina with apparentbladder and rectal sphincter problems. An objective

neurological deficit, however, is rare and, when present, isdue to a significantly compromised space in the spinal canalwith a relatively acute aggravation and decompensation (Fig.12). A sequester of a severely degenerated and dried outdisc within the curve may be the cause of such an acuteneurological deficit (Fig. 13). It can be accentuated or onlybecome clinically relevant due to a latent or obvioussegmental instability.

Curve progression

The fourth relevant symptom or sign is curve progression.Curve progression may be an issue from the moment thecurve occurs in younger age. It may, however, only becomerelevant, when the curve has reached a certain amount ofdegrees and/or when osteoporotic asymmetric collapse maycontribute relevantly to the curve. Once a curve has reacheda certain extent of curve degrees, the progression willautomatically follow due to the axial mechanical overload ofindividual facet joints and/or osteoporotic vertebral bodies.

The progression of the curve may wellbe an indication for a surgical treatment.The surgeons have to be aware of theamount of aggravation which mayoccur, when nothing surgically is done.Patients do get older with all the medicalconsequences, which raiseautomatically the risk for a surgicalintervention. Therefore, a surgicalintervention may occasionally beindicated in order to avoid a furtherprogression and degeneration in apatient with potential medical risks [3,4. 7].

Cosmesis

In contrast to the adolescent idiopathicscoliosis, cosmesis almost never playsa role; patients see the orthopaedicsurgeon because of a simple or morecomplex pain syndrome and/orneurological deficit. Cosmesis mayoccasionally play a role in youngerpatients below 40 years with an earlysecondary degenerated idiopathicthoracolumbar or lumbar scoliosis.

Diagnostic evaluations

In addition to the standard clinicalexamination, patients with symptomatic adult scoliosis needprecise conventional imaging and often requireinterventional radiological procedures, such as sequentialdiscograms, facet blocks, epidural blocks and preferentially,a myelogram combined with a CT scan [24, 25, 34, 36, 57]. Aspiral CT is very useful in rapid reconstruction of the spine

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Fig. 5 a and b A 28-year-oldfemale patient 15 years afterHarrington correction and fusionto L5. Flat back, spinal stenosisat L5/S1 and disconnection ofthe rod off the hook seating onthe arch of L5 (arrow). c and dDecompression and attempt tocorrect in L4/5 and L5/S1,refusion with pedicle screws andanterior cage at L5/S1.Remaining flat back andconsecutive back pain. e and fThree years after rebalancing thespine by posterior wedgeosteotomy at L3/4 andrestabilization and fusion 4 yearsafter second surgery. Back painalmost entirely disappeared

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vertically and, in conjunction with the myelogram, obtaininga clear understanding of the pathology. MR imaging ofdegenerative scoliosis is often very polymorphic due to thecomplex pathology, parts of which may still be difficult tounderstand and may leave us uncertain as to the leadingpathology. For example, deformity may be interpreted onone of the MR-cuts as spinal stenosis, since the wholedeformity is not in the same plain.

In the context of the evaluation of the pain source,discograms and facet blocks are especially helpful sincetheir findings may change the therapeutic approach. It isimportant, e.g., in lumbar curves, to find out whether thepain occurs within the curve, below the main curve (usuallyinvolving L4/5 and/or L5/S1), or rarely, above the curve atthe thoracolumbar junction. Since the pain can be generated

in one or several segments, it isrecommended to perform the discogramssequentially in order to isolate the reallypainful segment. In addition, the discogramcan be used as a provocative pain test, aswell as a form of local anaesthesia in thedisc, usually combined with some cortisoneinjection. Therefore, the discogram servesto provide both direct pain provocation andlocalization and as a double test for painevaluation, when the pain disappears afterthe intradiscal depot of medication. Facetblocks should also be performedsequentially to catch the most probable paingenerator level.

If, despite all these tests, the painremains unexplained, it may be helpful inrare cases to put on a temporary cast in theform of a thoracolumbar orthosis (TLO) orthoracolumbosacral orthosis (TLSO) to seewhether an overall stabilization and fusionof the whole scoliotic spinal area could bebeneficial for the patient, specifically incases of an overall tendency of the spine tostatically collapse.

In elderly people with degenerativescoliosis displaying predominantsymptoms of claudication, leg pain, andmultilevel stenotic segments in the imaging,motor evoked potentials (MEP) may behelpful to identify the level responsible forthe clinical presentation. A cleartopographic diagnosis would certainly helpto minimize the surgery in these patients.

The selective use of epidural blocks atstenotic levels or selective nerve root blocksis another helpful tool to identify the levelclinically relevant to the symptomatologyon the one side and as a therapeutic tool ontheother side, in case surgery is not feasibleor is decided to be delayed [67].

Therapeutic decision (Fig. 14)

The indication for or against surgery and, more specifically,the type of surgery to be performed, involves complexdecision-making. Certainly, surgery is only an option whenthe non-surgical measures have no effect or do not promiseany relevant long-term help.

The non-surgical treatment options [9, 20, 34, 46, 67] consistbasically of non-steroid anti-inflammatory medication, musclerelaxants, pain medication, muscle exercises, swimming andoccasionally gentle traction, while avoiding manipulations andphysical activation that may increase the pain. Therapeutic epiduraland selective nerve root blocks as well as facet joint blocks mayhelp to control the pain temporarily. Sometimes a well-fitted braceto support the painful spine area may be necessary.

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Fig. 6 Type 3a scoliosis in a 25-year-old female patient. Arrows (1) transitionalanomaly; (2) spondylolysis L5. *Plomb line from the centre of the head

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In order to plan the most promising surgical procedurefor each patient, a clear understanding of theprominentsymptoms or clinical signs is mandatory. The symptomsand clinical signs, which can be addressed surgically, eitherindividually or in concert are represented in Table 2 (seealso Fig. 10).

The specific surgical answer to each of these signs orsymptoms may vary depending on whether they occurindividually or in the context of another. The surgicaldecision is also influenced by the patient’s general health,age, condition of bone quality, and the patient’sexpectations [3, 15].

The surgical concept involves three basic procedureswhich can be performed separately or together, dependingon the patient’s overall symptomatology.

Surgical procedures

A surgical approach to degenerative adult scoliosis is obviouslycomplex in terms of decision making, i.e., ascertaining thesurgical indication and choosing the patient and the procedureappropriately. The technical difficulties, however, are equallyelaborated. The aggravating factors and difficulties for thistype of surgery are manifold. Curve magnitude and age of thepatients are, e.g., significant predictors of curve flexibility. Theunderstanding of this association allows to better addresstreatment options over time [14].

The possible surgical technique can be divided inposterior, anterior or combined procedures. In all theseprocedures a simple decompression or stabilization can bedone, or both can be combined [2, 9, 16, 32, 38, 41, 56, 68, 69].In some cases, additional correction may be considered,

either by clearly defined osteotomiesor by sequential segmental correctionthrough instrumentation.

This is particularly of interest incombined sagittal–frontal rigiddeformities.

Decompression procedures

Decompression may be done as astand-alone procedure in cases ofcentral or lateral stenosis andsymptomatology limited to the legs,without relevant back pain, or togetherwith instrumentation and fusion incases of additional obvious instability,segmental dysfunction, or progressivedeformity. Stand-alone decompressionprocedures in deformities are rarelyindicated, although it may be attractive,particularly in elderly frail people. The

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Fig. 7 Secondary degenerative scoliosis due to a hip arthrodesisfor a posttraumatic damage of the left hip: left convex, longthoracolumbar curve with secondary rotational deformity

Fig. 8 Adult scoliosis in a 55-year-old malepatient with M. Addison: osteoporose, flatback and spinal stenosis. Decompression,stabilization and correction in the frontalplane, distraction, but not reestablishinglordosis. However, 2 years postoperatively,patient had no relevant back pain

Fig. 9 Pathomechanism of adult degenerative scoliosis as a‘‘vicious circle’’

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decision exactly when a decompression alone is possible, isdifficult to make, since a definite judgement about the degreeof stability or instability is sometimes not possible onobjective, scientific grounds alone. In clinical practice onelearns regularly that the judgement was wrong and thechosen procedure not sufficient. It is easier sometimes toadd a limited fusion and stabilization in the same sessionthan to go back to do a second surgery in these oftenmedically unstable patients (Fig. 11). The outcome isfrequently complicated by either further collapse of thecurve, or by segmental instability, with symptoms ofcontinuous back and/or leg pain, mostly when in an uprightposition with axial loading of the spine, and asymptomaticwhen lying down and resting. If decompression is performedat the apex of the degenerative curve, progression of thecurve is very likely. This means that decompressioncontributes to destabilization of a fragile balance. Thisprocedure can be detrimental and completely disable apatient (Fig. 15).

The same may be true when an isolated decompressionis done at the bottom of a rigid curve, i.e., at the transition tothe mobile part of the lumbar spine, usually L4/5 or L5/S1.The rigid curve above may decompensate these segmentsin translation, and the spine may fall off the balance. In mostof these cases, therefore, a decompression should beaccompanied by a fusion with or without stabilization insitu (Fig. 11).

In younger patients, in whom a partial correction isplanned, a decompression may be achieved by the correctionprocedure alone, and a formal decompressionmay not benecessary (Figs. 4, 5, 6).

Correction procedures

If the balance in the frontal plane is achieved and there is aproper sagittal alignment, there is no need to look for a

correction. Stabilization and fusion in situ may be more thanappropriate in which case overall decompensation of thespine can usually then be avoided. Often it is tempting tocorrect the lumbar curve and/or thoracolumbar deformitysince this is almost always possible when a rigorousposterior release and pedicular fixation is done.

In cases where the lumbar and/or thoracolumbar curveis a degenerated countercurve to an idiopathic thoraciccurve which is either completely rigid or has been fused inadolescence, a correction needs to be carefully thoughtabout, since the thoracic curve cannot adapt to the correctedcurve below, resulting in a decompensation of the thoracicspine, imbalance, and a chronic pain syndrome in the lowerthoracic or thoracolumbar spine (Fig. 16).

In cases of apparent flat back syndrome (primarydegenerative lumbar scoliosis are usually combined with aloss of lumbar lordosis), correction is mostly necessary ifthe chronic back pain should be addressed. This needs anextensive release of the posterior elements (facets, facetjoint capsules, ligamentous components, and sometimesfacet joint osteotomies), and mostly the anterior elements(disc calcified, posterior longitudinal ligament calcified). Ifnecessary, the disc can be released through the sameposterior approach at L2 and below. An anterior release isindeed rarely needed, unless there is a relevantly rigidscoliosis component present with translational dislocations,or significant anterior osteophytes bridging the segmentssolidly, where an anterior release may help to balance thefrontal and sagittal plane (Fig. 17). Such a combinedprocedure, however, may not be well tolerated by moreelderly patients over 60 years or in reduced general health.Selective segmental spinal osteotomies, mostlytranspedicular reduction osteotomies, may be necessary tobalance the spine.

In addition to the important release in the case of aplanned correction, it may also be necessary to extendthe

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Fig. 10 Pathophysiology of adult,degenerative scoliosis with its clinicalpresentation

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fixation and fusion beyond the major curve into thethoraciccountercurve, to guarantee appropriate sagittal as well asfrontal overall balance of the spine. If that is not considered,the patient may end up with a collapse of the cranial segmentadjacent to the fixation, or with a secondary instability bothmost probably due to an overloaded segment in anunbalanced correction. It is clear that such a magnitude ofsurgery is only possible in patients with a robust generalcondition, since the surgery may take several hours and theblood loss may be significant. For these reasons, werecommend that surgeryis considered as early as possible,when unfavourable developments and progression can beforeseen. This is a particularly important consideration infemale patients entering menopause who regularly have backand/or leg pain with beginning osteoporosis and significantdegeneration and tendency of flat back.

If an anterior release is necessary, the surgery can bestaged or done in the same surgical session. Anteriorinstrumentation in patients who need a secondary posteriorprocedure is not necessary or even contraindicated, since

the anterior instrumentation may limit posterior correction.

The posterior pedicular systems nowadays allow a powerfulmanipulation, correction, and stabilization of the lumbarspine, as long as a proper posterior release precedes thecorrective and stabilization procedure. The anteriorlyreleased segments, however, may be prepared for anteriorinterbody fusion by removing the disc, freshening theendplates and filling the intervertebral space with cancellousbone or even a solid tricortical spacer.

The correction of a deformity is therefore achieved afteran appropriate release either by stepwise correctionthrough segmental instrumentation or by one or moresegmental osteotomies for the frontal or sagittalrealignment of the spine.

In case a lumbar curve is still flexible, which can beassessed by sidebending and flexion/extension views, anda certain compensation of the thoracic countercurve can beanticipated, a posterior correction, stabilization and fusionwith or without decompression are sufficient. This is alsodone when a curve is clearly progressive.

Stabilization and fusion procedures

If back pain is a leading symptom, with or without leg pain,a fusion is usually indicated. The levels to be included inthe fusion can be difficult to determine.

Generally speaking, it is unfavourable to stop a fusion atL1 or even L2, i.e., below the thoracolumbar junction, becauseit may easily lead to decompensation above the fusion, withlocalized disc degeneration, segmental collapse,translational instability and secondary kyphosis [28].

The most critical segment to consider whether or not toinclude in a fusion is the lumbosacral junction. It takes allthe movement off of the lumbar spine and is the mostdifficult fusion to be achieved. A high percentage may remainwith a non-union due to the unfavourable mechanicalconditions of this junction between the two major lever armsof the fused spine and the rigid pelvis [8, 12, 17, 26, 30, 37,57, 68]. The incidence of the nonunion varies quiteremarkably in the literature (5–30%) [12, 18]. Various typesof instrumentation have been designed to enhance thefusion healing to the sacrum [12,30, 37, 60]. They are mostlybased on an increasingly more solid anchorage in the sacrum,or in the sacrum and iliac wings at the same time. None ofthese instrumentations have been clinically demonstratedto significantly overcome the problem of non-union in thecomplex pathology of degenerative scoliosis. The mostcertain approach to eliminate the problem of non-union is a360° circumferential fusion at the lumbosacral junction. Inorder to avoid the anterior approach, unless needed for anextensive release, the refinement and standardization ofposterior lumbar interbody fusion (PLIF) technique usingspecifically designed cages has become a well-controlledprocedure (Fig. 18). Controlled clinical studies need to showwhether the clinical reality will support the theoretically validconcept of load sharing at the lumbosacral junction in orderto avoid non-unions.

Obviously, it would be preferable to omit the lumbosacral

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Fig. 11 A 79-year-old female patient, known to have scoliosissince late twenties. Persistent L5 radiculopathy after selectiveforaminal decompression 4 years ago. Now again decompressionon the left with a local stabilization and fusion in order toprevent progression of the curve. Disappearance of the

Fig. 12 A 75-year-old actress with subacute paraparesis, norelevant back pain: decompression, stabilization, and fusion insitu. Almost complete recovery

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junction during the surgery for lumbar scoliosis correctionand stabilization. However, depending on the cause of thecurve, the lumbosacral junction usually is degenerated: discspace narrowing, facet joint arthritis, vertebral obliquity andpossibly rotational deformity and sometimes evenspontaneous fusion of L5 to S1 might be a consequence ofa lumbosacral transitional anomaly or a progresseddegeneration. In such cases, if there is no certainty aboutthe rigidity of L5/S1 it is better to include it in the fusion/stabilization since a stop of the fusion at L5 puts a lot ofstress on the lumbosacral junction and is able to ‘‘loosen’’ itup with a secondary pain syndrome. A fusion to L5 even incase of a ‘‘healthy’’ L5/S1 segment may overload that andinduce a secondary degeneration with loss of disc height,facet joint incongruency and arthritis and possibly discherniation (Fig. 19) [31, 37, 56, 63].

A fusion to the sacrum may not onlyhave an impact on the iliosacral jointsbut also on the hip joints specificallywhen there is already osteoarthritis ofthe hip. The disease process may beaccelerated by the change of the loadto the hip joint.

For posterior surgery, pedicularand/or translaminar screw fixation maybe considered [2, 4, 7, 40, 41]. In casecorrection is not the goal of the surgery,but towards stabilization andimmobilization, respectively, of thearthritic, often hypertrophic facetjoints, then the translaminar screwfixation may be a simple, little traumaticand minimally invasive procedure toachieve such a goal (Fig. 20). For a longfixation and correction, a pedicularsystem is the indication of choice.

Problems encountered in case of surgical treatment

Length of fusion

It is increasingly understood today that the fusion shouldbe avoided whenever possible. Unfortunately, up to now,we do not have better established treatment modalitiesavailable in severely degenerated and deformed spines.Therefore in severe adult degenerative scoliosis cases, thefusion is an integral part of a treatment plan. It is anticipatedthat within the lumbar spine, every segment should be sparedif possible, in order not just to maintain some lumbarmovement but also to spare further segments fromdegenerating. Discography and facet joint infiltrations aswell as temporary immobilization in a body cast may help todetermine the extent of the fusion. The radiologically

moderately degenerated motion segmentis not enough to be included in thefusion, as long as this segment does notreally contribute to the pain suffered bythe patient. It is, however, necessary toinform the patient that this concept ofrestricting the extent of the fusion maynecessitate further surgery in case aspared segment may degenerate furtherand become symptomatic. The mostproblem segments to be included in afusion are those below a curve in thelower lumbar spine.

Whether the emerging dynamicfixation devices or even discarthroplasty will be an option in thesurgical treatment of adult degeneratedscoliosis remains to be considered asmore experience is acquired with thatkind of implant.

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Fig. 13 A 79-year-old hard-working male farmer withrelatively mild back pain,however, severe left leg pain,corresponding L3 (4) root, withproven root compression:Microsurgical decompression only,no attempt to touch the severelydegenerated kyphoscoliotic spine

Fig. 14 Treatment options

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Inclusion of the lumbosacral segment

Patients with a lumbar or thoracolumbar curve have at leastsome degree of degeneration at L4/5 and/or L5/S1 segments.Although these segments may not be painful at the time ofthe decision-making, they may soon become painful after along-distance fusion has been performed above them. A rapiddeterioration is then possible, which may necessitate a furtherextension of the fusion down to the sacrum. The expert’sopinion is divided, whether in such cases a lumbosacral fusionshould be included right from the beginning, or whether await and see attitude should be taken and only fuse thelumbosacral junction in case there are significant clinicalproblems from this area [8, 10, 12, 15, 30, 37].

Spinal segments adjacent to the fusion/level of fixation

A long fusion, which is often necessary in scoliosis, acts as alever arm against the adjacent segments above and below. If thespine above (in adult lumbar scoliosis mostly the thoracic spineor the thoracolumbar junction) is rigid, either physiologicallydeveloped over the years, or acquired by a previous fusion of athoracic curve, it may not follow the correction executed in thelumbar spine. This may result in a decompensation above thelumbar curve with an unbalanced overall spine, which may bethe cause of further clinical problems. It is therefore crucial tounderstand through preoperative planning on whole spine X-rays as to how far a correction can be carried out without creatinga secondary problem in the spine above (see also Fig. 16).

Many mid-lumbar and thoracolumbar curves have a short andsharp countercurve at the lumbosacral junction either includingS1 and L5 only or also L4 with an oblique take off. This short curveis usually seen in the pelvis and leads to an overload of L3/4 or L4/5 [37]. These curves usually do not compensate a corrected curveabove and have a bad prognosis [34, 37]. They may even progressand contribute significantly to the clinical syndrome of the patient.In most cases, it is therefore recommended to consider inclusionof this lumbosacral junction into the fusion.

Previous scoliosis surgery

More than 25 years after Harrington instrumentation becameincreasingly popular for idiopathic scoliosis surgery, we seea growing number of patients who had such a surgery in theiradolescence. These patients, who are now between their latetwenties and early forties, are sometimes presentingthemselves with severe secondary problems below theirthoracolumbar fusion and fixation. This problem is particularlysevere in patients who were left with a flat lumbar spine becauseof the distractive-kyphotic effect of the Harringtoninstrumentation in the sagittal plain (Fig. 5).

They may have degenerated adjacent segments below,along with spinal stenosis, and chronic low back pain due tothe overstressed paraspinal muscles [23, 69].

These problems are not unique to patients who hadHarrington instrumentation. They can also be found in patientswho had non-instrumented fusions with long-term body castimmobilization, or who had not been treated at all for a relevant

thoracolumbar idiopathic scoliosis.In the majority of cases, a simple correction,

decompression, and instrumented fusion doesnot remedy the problem. These patients need acomplete overall rebalancing of the spine by oneor multiple osteotomies. This problem is socomplex and multifactorial that it needsconsideration in a separate review and can onlybe alluded to in this article.

Age and general medical condition of thepatient

The age of the patient—most of the patients withscoliosis and significant, symptomatic

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Fig. 15 A 76-year-old female patient with back pain and claudicationsymptoms in both legs. a When patient presented herself to a neurosurgicalservice, a decompression of the stenosis close to the apex of the curve wasdone. b 8 months later, progression of the curve, significant claudicationsymptoms, severe back pain and patient practically bedridden

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degenerative alterations of the curve or counter curve, are olderthan 50 years and often have systemic diseases such as cardiacor vascular disease or diabetes, and are very demanding forthe responsible anaesthesiologist who must also consider thepossible aggravating significant blood loss and surgical timein this procedure. The periand post-operative management ofsuch a patient may need an interdisciplinary approach amongthe surgeon, the anaesthesiologist, and an internist whoaccompany this patient, until his/her general condition post-

operatively has stabilized and returned to the pre-operativesituation. Also, the elderly and frail patients need a longerand more intensive rehabilitation time in order to recoveroptimally [49].

Osteoporosis

Osteoporosis is a major concern in the treatment of adultscoliosis. The majority of elderly patients with degenerativescoliosis are female, and osteoporosis may become an issueat the time of the menopause. This is the time whendegenerative scoliosis may become increasingly symptomaticbecause the curve may progress due to the asymptomaticload on weakened vertebrae, which get more wedged anddeformed. With the progression of the curve, the patient mayget more symptomatic in terms of back pain as well as of theclaudication pain.

The surgical treatment is complicated by the weak bonewhere implants are more difficult to be anchored and fixed,making the instrumented fusion prone to instrumentation-related complications. The industry has offered all kinds ofinstrumentations with big diameter screws and adaptedthreads to improve bone purchase. Cement reinforcement ofthe screw anchorage is another alternative which has beenadvocated [8, 37]. In our experience, instrumentation successis not really so dependent on the size of the screw. The wholeconstruct must respect a principle of balance as demonstratedin the Asian world where bamboo is used in the constructionof gigantic buildings. Each anchorage is loaded one againstthe other in an overall construct, creating some sort of elasticstability. This concept may be considered in spine stabilization

as well.

Outcome and complications

Complications may result from indicationand misjudgement of the case, non-suitable patients, wrong technicalperformance, implant failure, a lack ofachieving balance in the sagittal andfrontal planes, and complications whichcannot necessarily be explained [3, 4, 15,17, 21, 26, 47].

We have performed more than 250surgeries in adult scoliosis, and thecomplication rate has improved since wedid a retrospective study almost 10 yearsago on our first 75 adult scoliosis cases.

In the first series, the complication rate was comparable to thosein other studies and the overall results were satisfactory [56, 59,61, 63]. With the present expertise in this field and the referral ofincreasingly complex cases, the complications tencounter in thissurgery are problems of proper balance and non-union at thelumbosacral junction, as well as limitations of the surgical optionsin medically frail patients, resulting in only partial improvement.The nonunion problem has lost its significance in the last 3years since we systematically do circumferential fusions at the

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Fig. 16 A 54-year-old female patient who has been fused in lateadolescence for a idiopathic right convex thoracic curve(uninstrumented fusion); a secondary lumbar curve withdegenerative changes, b instrumented correction and fusion withdecompensation of balance because of the rigidity of thethoracic curve due to the fusion and secondary back pain in themid- and lower thoracic spinal area

Fig. 17 a A 71-year-old male physician with severe degenerative scoliosis and withspinal stenosis and flat back. b Decompression and posterior stabilization, however,correction of the lumbar kyphosis absent, because no anterior release was done andthe posterior release was also insufficient. Postoperatively no claudicationsymptoms anymore, however, persistent back pain

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lumbosacral junction, using either PLIF or ALIFcages in combination with posterior pedicularinstrumentation.

There is a quite substantial risk for mild orsevere post-operative nerve root paresis [47];some of them recover spontaneously.

The outcome needs to be age matched sincethe demands of daily life, professionalperformance, and leisure activities varysubstantially in the different age groups. Mostof the patients who are still professionally activedo not return to their previous work if it was aphysically demanding job, but almost all ofthose had already stopped working before thesurgery, because of pain limitations. Whenanalyzed, regarding their overall daily activityby different questionnaires [50], most of thesepatients irrespective of age have improved inalmost all categories of quality of life, and theuse of regular pain medication is reducedsubstantially in more than 70% of these patients.The assumption that this surgery may be toomuch for elderly patients could not besubstantiated, at least not in our institution,where we have done a comparative study ofgeneral complications in an age and comorbiditymatched cohort of patients undergoing hiparthroplasty surgery—an accepted surgery inelderly patients—and patients undergoing amajor spine surgery like degenerative lumbarscoliosis surgery [49]. The major problem ofthese patients after surgery, once surgicalcomplications and implant failures have notoccurred, is the residual back pain mostly as anexpression of muscular spasms and pain dueto unbalanced or chronic contractures of theparavertebral muscles.

Conclusion

The complexity of the relationship betweenclinical signs, symptoms, pathomorphology,and pathophysiology of adult—mostlylumbar, degenerative scoliosis—remains oneof the big challenges in spinal surgery. Thecombination of the relatively uniform majorsigns and symptoms, namely instability,stenosis, unbalanced deformity, and possiblecurve progression on one side, and the complexpain pattern, the claudication, and possibleneurological signs and symptoms on the otherside allow a variety of clinical patterns, whichneed to be analysed both, systematically andindividually in order to find concrete solutionsfor each of them, and to tailor a surgicalprocedure which best serves the rationalexpectations of the patient and surgeon.

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Fig. 18 Type 2 scoliosis (progressive idiopathic scoliosis in adult life): A 39-year-old female patient with AIS, significant loss of lumbar lordosispreoperatively. Postoperative restoration of lordosis and circumferential fusionwith PLIF at the lumbosacral junction in order to avoid non-union

Fig. 20 a A 73-year-old female patient with progressive degeneration scoliosiswith a foot drop and claidication symptoms as well as moderate back pain withwich the patient can live (a and b) c 10 months postoperatively. After a minimalsurgery for decompression and stabilization in situ, partial recovery of the footdrop. Back pain irrelevant, although the flat back is unchanged

Fig. 19 a A 61-year-old female patient with back pain and claudication symptoms. bDecompression, stabilization and fusion, while omitting L5/S1; 1 yearpostoperatively disc space L5/S1 still quite high, c 28 months postoperatively,collapsed disc space L5/S1 with L5 radicular syndrome due to foraminal stenosis

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