Ltt CHAPTER 22 Disorders of the Leg Chapter Outline Genu Varum (Bowiegs) 713 Genu Valgum (Knock-Knees) 733 Tibial Torsion 739 Bowing of the Tibia 741 ‘Congenital Pseudarthrosis of the Fibula 757 Deformities of the tibia and fibula are probably the most common and obvious abnormalities that affect a child's Tower extremity. They can be congenital or acquired, physiologic or pathologic, but all draw immediate attention to themselves because of the real or apparent detrimental effect they have on gait and upright activity. Most lower leg "deformities" are in fact physiologic and resolve sponta- neously, so early recognition of the benign nature of such deformities is as important as the correct diagnosis of true pathologic conditions. This can reassure parents, avoid lunnecessary treatment, and minimize excessive attention ‘0 a nonpathologic problem, The various conditions affect ing the lower part of the leg are discussed in this chapter ‘elation to their anatomic occurrence, proceeding from proximal to distal. Genu Varum (Bowlegs) Genu varum (bowlegs) is an extremely common pediatric deformity, and parents uniformly seck evaluation even though itis rarcly symptomatic in the age group (younger than 2 years) in which it is most common. Determining whether the condition represents physiologic genu varum or a pathologic process, such as infantile tibia vara, ds ertical because the prognosis and treatment differ profoundly Physiologic Genu Varum Physiologic genu varum is ¢ deformity with a tibiolemoral angle of at least 10 degrees of varus, a radiographically normal physs, and apex lateral bowing of the proximal end of the tibia and often the distal end of the femur!" The legs of most newborns are typically bowed, with 10 to 1S degrees of varus angulation. When the infant begins to stand and walk, the bowing may appear more prominent and often seems to involve both the tibia and the distal part of the femur. Concomitant internal ubial torsion may exacer- bate the deformity (Fig. 22-1, A). Children with physiologic genu varum and internal tibial torsion typically come to medical attention after standing age (between 12 and 24 Charles E. Johnston Megan Young months), usually because of parents! concem about the appearance of the legs. These children have no other signi ‘ant findings on clinical examination, In the typical mani- festation, radiographs generally are not needed to determine the physiologic nature of the deformity. Although radio- graphs at this time may show an apparent delay in ossifica- tion of the medial side of the distal femoral and proximal bial epiphyses (see Fig. 22-1, B) or flaring of the medial distal femoral metaphysis, the physes have a normal appearance. Clinical measurements of the tibiofemoral angle and intercondylar distance in normal children show maximal vvarus at 6 to 12 months old, neutral alignment by 18 to 24 ‘months, maximal genu valgum at 4 years (8 degrees of tibiofemoral valgus), and a gradual decrease in genu valgum toa mean of 6 degrees by the age of 1] years." The pres- cence of genu varum in children older than 2 years ean be considered abnormal, but this “expected! pattern of change ‘overtime from genu varum to genu valgum isa generalized standard, and variations may be observed (Fig. 22-2).!"" A lstinct subset of patients with more severe varus angulation at initial evaluation, slower resolution to neutral alignment bby 3 to 4 years old, and radiographic femora vara have been described.” Spontaneous resolution of the varus to neutral tibio- femoral alignment by 24 months old and to an adult valgus alignment after 3 years old is well documented (Fis. 22-3), as is the variation just noted.” Patients can be formally observed to ensure that the varus resolves. Parents| are reassured that the condition is usually self-correcting but also advised that reevaluation with radiographs may be warranted if the varus deformity persists oF progresses beyond 24 months old. Nonresolving, asymmetric defor- rity is the main indication for radiographs. ‘The differential diagnosis of persistent genu_varum still includes physiologic gemu varum, which remains the ‘most common etiology, even in a deformity that is slow to resolve and appeats to be pathologic (see Fig. 22-2). ‘One must also consider infantile tibia vara, physeal distur- bance secondary to trauma or infection, metabolic bone disease, generalized skeletal dysplasia, and foeal Sbrocarti- laginous dysplasia." All these conditions are diagnosed radiographically. Tibia Vara Tibia vara is defined as growth retardation at the medial aspect of the proximal tibial epiphysis and physis usually resulting in persistent or progressive bowleg. ‘Two forms of the deformity are recognized based on age at onset of the condition: infantile tibia vara, if younger than 3 years, and ferences 2,13, 24, 95, 113, 115,141, 158, ns714 SECTION! Anatomic Disorders [An 18-month-old child with bowlegs. A, Clinical appearance. Intoving exacerbates the angulation at the knee. B, Radiograph showing “delayed” ossicaton of the medial ital femoral epiphysis and, co a esse extent, the proximal tibial ‘epiphysis. The physes are normal cei ’An extrome case of physiologic varus in a 3.year-old child. A, Clinial appearance. epiphyseal ossification defects are more severe than in the eh in 5 years old, The condition resolved spontaneously. adolescent tibia vara, if 10 years or older. The two forms have distinctively different radiographic characteristics and ‘tment results, with infantile Ubia vara being more ‘Tibia vara has alternatively been classified as infantil, juvenile, and adolescent, with the juvenile form occurring between 4 and 10 years old and the adolescent form occur- ring after 10 years." The term late-onset tibia vara includes both the juvenile and the adolescent forms. jome authors have used late onset” to define tibia vara in ‘older children, but there are differences of opinion regard: ing whether late-onset tibia vara describes classic adolescent tubia vara or isa distinct entity ‘We have not found these distinctions useful and continue to classify tibia Radiographic appearance, The media but the physes are normal. €, Clinial appearance at vara as either infantile or adolescent, with the “juvenile” or undiagnosed manifestation of infantile a vara and “late-onset” tbia vara being synonymous with adolescent tibia vara form being a Infantile Tibia Vara (Blount Disease) Infantile ubia vara, frst described by Erlacher in 1922, is best known as Blount disease after the classic description by Blount in 1937." Blount characterized the deformity as an abrupt angulation just below the proximal physis, an irregular physeal line, and a wedge-shaped epiphysis with 2 “beak” at the medial metaphysis ( ). Apparent lateral subluxation of the proximal end of the tibia is often present.Varus 10 Vagus 10° - A ‘Age (yt) CHAPTER 22 Disorders of the Leg 715 FIGURE 22-3 A, Development of the tibiofemoral angle during growth (after Salenius). B, Serial radiographs demonstrating normal transtion from varus alignment at 14 months to neutral postion at 25 months to valgus tibiofemoral alignment at 39 months, A FIGURE 22-4 Blount disease in a S.year-old gi. A and B, Preoperative clinical appearance. Note the abrupt medial deviation ofthe tibia Just below the knee. Lateral “thru of the knee during weight bearing exacerbates the “limp.” €, Radiograph demonstrating abrupt angulation at the epiphyseal-metaphyseal junction ana medial metaphyseal radiolucency and beaking with apparent lateral subluxation of the proximal end ofthe tba, Etiology Several authors have reported a familial occurrence of che condition, "and one report of infantile tibia vara in a family suggested that the disease may be inherited as an autosomal dominant condition with variable penetrance.'" However, as noted by Langenskiold and Riska,'* because the radiographic features of infantile tibia vara have never been seen in patients younger than 1 year than 2 years, the condition is considered a developmental disorder and not 2 congenital fone. Other studies have found no evidence of an_inher- ited condition and have concluded that the etiology is multifactorial." Physiology Histologic evaluations of affected physis and the corre- sponding part of the metaphysis in infantile ibis vara have been conducted by a number of authors.” The general findings have included (1) islands of densely packed cari lage cells displaying greater hypertrophy than expected from their position in the physis, 2) islands of nearly acl lular fbrocartilage, and (3) exceptionally large clusters of capillary vessels. The physeal cell columns become irregular and disordered in arrangement and normal endochondral ossification is disrupted, both in the medial aspect of the ‘metaphysis and in the corresponding part of the phys. varus deformity progresses as long as ossification is defective and growth continues laterally.” In later stages of the deformity, an actual bony bridge may tether medial growth, and the medial tibial plateau may appear to be deficient posteromedially: However, actual depression of the postero- ‘medial tibial articular surface is probably not present, at least at the outset of the deformity. The "deficiency" is probably unossified abnormal fibrocartilage whose delay in‘ossification produces the appearance of a defect and is directly related to the underlying histopathology.” Liga- ‘mentous laxity on the lateral side of the knee frequently develops in a neglected or recurrent deformity. Clinical Features ‘The typical child with infantile tibia vara appears similar to «child with physiologic genu varum, with two major differ- ‘ences. First patients with tibia vara are often obese, exceed- ing the 95th percentile for weight. Significant differences in body mass index (BMD percentile have also been observed, for toddlers presenting at similar ages and timing of walking veho developed infantile tibia vara." Finite element analysis of the knee has shown that a compressive force sufficient to retard physeal growth by the Hueter-Volkmann principle is produced on the medial tibial plateau of a 2eyear-old in the 90th percentile for body weight and with 4 20-degree varus deformity during single-limb stance." Greater radiographic varus malalignment and procurvatur deformity has been correlated with higher BMI in infantile tibia vara.” This relationship between obesity and risk for Blount disease may warrant early intervention and nutrition counseling in young patients, Second, patients with infantile tibia vara often have a clinically apparent lateral thrust of the knee during the stance phase of gait that resembles a limp (see Fig. 22-4). ‘This sudden lateral knee movement with weight bearing is caused by varus instability at the joint line in concert with the angulation. In our practice, the presence of this thrust, though not pathognomonic for infantile tibia vara raises our level of suspicion and is an indication for radiography, regardless of the age of the patient Radiographic Findings Radiography is central to establishing the diagnosis of infan- tile tibia vara (Box 22-1). A standing anteroposterior view of the lower extremities from hip to ankle should be obtained. The diagnosis is based on familiar radiographic changes in the proximal end of the tibia: (1) a sharp varus angulation in the metaphysis, (2) a widened and irregular physeal line medially, (3) a medially sloped and irregularly. ‘ossified epiphysis, and (4) prominent beaking of the medi ‘metaphysis with lucent cartilage islands within the beak (see Fig, 22-4), Unequivocal radiographic changes diagnostic of infantile tibia vara are rarely observed before 18 months old (the youngest published case was radiographically diagnosed at 17 months old).'"* However, a normal knee radiograph in a toddler does not rule out infantile tibia vara. As an “+ Varus angulation at the epiphyseal-metaphyseal junction ‘+ Widened and irregular pnyseal line medially + Medialy sloped and irregularly ossiied epiphysis, Sometimes triangular ‘+ Prominent beaking of the medial metaphysis with lucent Cartage islands within the beak ‘+ Lateral subluxation ofthe proximal end of the ubia a aid to carly identification of toddlers who are at risk for the development of infantile tibia vara but who have no physeal or metaphyseal changes, Levine and Drennan mea- sured the tibial metaphyseal-diaphyseal angle (MDA), the angle created by the intersection of a line connecting the ‘most prominent medial portion of the proximal. tibial metaphysis (the “beak") and the most prominent lateral point of the metaphysis with a line drawn perpendicular ‘to the long axis of the tibial disphysis (Fig. 22-5). Blount lesions visible on radiographs subsequently developed in 29 of 30 patients whose MDA was greater than 11 degrees, whereas such changes developed in only 3 of 58 patients with an angulation of 11 degrees or less." However, sub- sequent studies measuring the MDA, the tibiofemoral angle, o the mechanical axis have not improved early detection of infantile tibia vara," nor have radiographic measurements been helpful in establishing the severity of disease once the condition is present. Any limb malrotation| during radiographic examination can affect the measured MDA’ and the tibiofemoral angle.""* Thus, although measurement of the MDA may have some prognostic accuracy,” it has not by itself been reliable to diagnose impending infantile tibia vara." The tibial epiphyseal-metaphyseal angle has been pro- posed as an adjunctive measurement to aid early disgnosis of infantile bia vara. An angle greater than 20 degeces in combination with an MDA greater than 10 degrees indicates a toddler at risk.” However, true infantile tibia vara cannot be diagnosed without the unequivocal presence of the cha acteristic lesion in the proximal medial tibial metaphysis. If this radiographic finding is not present, the patient by defi- nition has physiologic genu varum."* Although patients with large MDAs may be at risk for the development of infantile tibia vara and must be monitored, we currently 32) A FIGURE 22-5 Radiographic measurement of angular deformity of the lower limb. A, Tibiofemoral ange, B, Proximal metaphyseal- ciaphyseal angerecommend no treatment before the appearance of an tuneguivecal Blount lesion, which is often manifested between 18 and 24 months old. Attempts to use magnetic resonance imaging (MRI) to assess the growth disturbance in infantile tibia vara are ongoing.””" MRI characterizes the extent of the ossified. and cartilaginous epiphysis, along with any physeal anatomic disruption (Fig. 22-6). While not necessary to confirm the diagnosis, such imaging studies are useful preoperative tools to evaluate location and size of the physeal bridge (see Fig. 22-6, D and E), as well as the presence or absence of “true” intraarticular deformity (see Fig, 22-6, A to C), MRI assessment of physeal function to determine whether the physis is bridged or sufficiently disrupted, in order to CHAPTER 22 Disorders of the Leg 717 predict cessation of medial growth, is not yet proven to be In later evaluated or “relapsed” cases of infantile tibia vara, a more severe bony deformity, including depression and sloping of the posteromedial epiphyseal surface, has been identified by computed tomography (CT) (Fig 22-7). However, CT lacks the added benefit of visu alizing the cartilaginous articular surface, which may occupy most of the medial plateau. The occurrence of "true" intraarticular deformity in young patients is debatable Patients younger than 6 years old have not demonstrated such plateau depression as demonstrated by intraoperative arthrography showing preservation of a normal-appearing joint line despite the osseous defect (Fig. 22-8).”™ However, FIGURE 22-6 A, Late-presentng infantile tibia vara (6 years old). Radiograph is concerning for intraarticular deformity or depression of the medal joint ine because of irregular oifiation, B, Corresponding coronal magnetic resonance image (MR) of the proximal tibia shows the medial osstication defect of the anterior tibia epiphysis. C, A more posterior Section reveals futher defect in the metaphysis. Note the aticularsuface is maintained by notified cartilage. D and E, Axial MRI and corresponding radiograph of a female wna is 7 {years 11 months old with unteated uniateral disease. The MRI sic through the abnaemal [physic enable localization and quantticaton ofthe physeal bar (estimated 2: 6% of the {otal area in this case). Information was used to guide treatment with bar resection, fat graft interposition, and proximal bia osteotomy,718 SECTION It Anatomic Disorders Infant A. Preoperative knee radiograph showing significant downward sloping of the mesial epiphyseal ana metapnyseal regions. B, Intraoperative arthrogram iustates maintenance of the articular surlace despite the abnormal Dssfieaton defect obvious on the plain radiograph, the clinical finding of lateral thrust and the radiographic appearance of lateral tibial subluxation on the femur could, bbe explained by the presence of such defect from the outset Differential Diagnosis ‘The most common entity in the differential diagnosis is, physiologic genu varum, in which no Blount lesion is seen ‘ona radiograph; indeed, the physis and epiphysis are normal Although the deformity may be dramatic, especially in patients with femora vara (see ), the bowing is eften symmetric or nearly s, and the cil is otherwise ‘Nonphysiologic causes of genu varum, all demonstrated con radiographs, include skeletal dysplasias (e.g, m physeal chondrodysplasia, spondyloepiphyseal dysplasia, multiple epiphyseal dysplasia, achondroplasia), metabolic diseases (eg,, renal osteodystrophy, vitamin D-resistant rickets), posttraumatic deformity, postinfectious sequelae, and proximal focal fibrocartlaginous dysplasia, bia vara seen at a late age (6 years 016). A, Stage IV lesion previously untreated. B and C, Three-dimensional ‘computed tomographic reconstruction showing the severe epiphyseal and articular surface depression. Classification In 1952, Langenskidld classified infantile tibia vara accord- ing to the degree of metaphyseal-epiphyseal changes seen fon radiographs, with six stages varying with advancing age f H.* General prognostic guidelines were also provided. Restoration to normal was common in disease stages I and I] and possible in stages III and IV, whereas disease stages V and VI were associated with recurrence and permanent sequelae after treatment by mechanical realign: ‘ment (osteotomy) ‘Although Langenskidld’s classification was primar intended to be a radiographic description of infantile tibia vara, prognostic implications have gradually been derived from later studies In 1964, Langenskild and Risks reported that a simple osteotomy could cure the deformity Jn patients 8 years old or younger.’ In the few cases in which simple osteotomy failed, inadequate surgical correc- tion was implicated. Radiographic stage progression of the deformity was thought to he a consequence of skeletalStage i Stage i! ™ Complete restoration Stage iv CHAPTER 22 Disorders of the leg 719 Stage V Stage Vi 10-13 years | Restoration possible FIGURE 22.9 Langenskidl's classication of infantil tibia vara in si progresive stages with increasing age. The prognostic implications suggested by Langenskidd are that simple osteotomy can be effective up to stage IV. Redrawn from Langenskild A: Tibia var, Acta Chir 10839, 1952) FHGURE 22:10 A, A Syearold back gt with 2 25degree varus deformity ofthe let eg, B, Close-up vew o a stage lesion absence cof continuous epiphyseal bone sloping into the metaphyseal defect, although the lesion is progressing toward stage IV see Fig. 22-8) , Slight valgus overcorrectin with lateralization of maturation" rather than an indication of progressive inhibition of medial physcal growth and worsening of the condition." The premise that 8 years is the critical age up to which the condition is surgically curable has undoubtedly resulted ina certain complacency in treating young children, particularly those with demonstrable stage progression. A number of investigators have reported difficulty applying the Langenskidld classification to predict outcome in their town patients 028 The Langenskidld classification is inaccurate for progno- sis when applied to a predominantly nonwhite population in North America and the Caribbean." Major incon- sistencies are that (I) all stages can occur earlier than Langenskiéld deseribed (as young as 17 months old); (2) disease stages II and II] can progress to stage VI despite ‘treatment (Fig, 22-10), whereas previously it was thought that surgery cured the disease in these patients """"; (3) there is a marked tendency for progression of deformity in mechanical axis produced by a high tibial osteotomy, B, Radiograph obtained 2 years later showing obvious recurence and mecial physeal ares (stage VI) black girls and thus an even worse prognosis for these patients; and (4) predictably good results from a single tibial ‘osteotomy are achieved only ifthe surgery is performed by 4 years of age, a notable departure from the previous guide line of 8 years. ‘The unanticipated difficulty in curing stages UI and IV lesions by osteotomy alone has been confirmed by other investigators."""" In the non-Scandinavian patient population, infantile tibia vara proved to have a more malig nant course, and the results of treatment were poorer than Langenskisld's 1964 or 1981 guidelines suggested." * The poorer outcomes seemed to be, at least in part, due to the olay in surgical teeatment of younger patients. However, the poorer outcomes may also be attributable simply to a different type and severity of disease that is encountered in the nonwhite population, Reports from Scandinavian” and Japanese’ centers continue to attest to a relatively benign ‘ourse in more than 50% of their patients and to condition that will spontaneously resolve, with the varus deformitycorrecting without treatment in up to three fourths of patients—an experience diametrically opposite to the ones in the United States and the Caribbean,” From our experience, it appears that only stages I and TI lesions can predictably have full restoration (i.e., curc) with 4 single osteotomy or bracing. Definitive correction must be achieved by 4 years old. Stage IIL lesions may be restored, ‘whereas stages IV to VI lesions cannot be restored with a simple osteotomy and require complex reconstruction and. physeal procedures, with « guarded outcome at best. Treatment Untreated infantile tibia vara generally results in @ nonre- solving and sometimes progressive varus deformity. that produces joint deformity and growth retardation, which can then be corrected only with complex surgical procedures. Even when such surgery is performed, substantial articular sruption of both compartments of the knee may have already occurred.” Thus, once the radiographie diagnosis of infantile tibia vara is certain, the orthopaedist should rec~ ‘ommend treatment immediately because patients treated in the early stages of the disease have a better prognosis. ‘There is no justification for simply observing a patient with an unequivocal diagnosis. Treatment choices and prognosis depend greatly on the age of the patient at the time of «agnosis, which should be the same age at which treatment is recommended, Orthoses. Ifthe child is younger than 3 years old and the lesion is no greater than Langenski6ld stage Il, orthotic treatment is recommended because 50% or more of these patients can be successfully treated with braces, especially if they have only unilateral involvement. "2" There ‘may be an inclination to brace patients before a tue Blount lesion is visible on radiographs, particularly when the MDA is suggestive of varus progression.» Thus when evaluating the reported good outcomes from brace treatment, one ‘must realize that some patients probably had physiologic sgenu varum rather than true infantile tibia vara. Neverthe- less, orthotic treatment appears to affect the natual history favorably.7!°2" ‘The type of orthosis prescribed and the length of time that the orthosis is worn during a 24-hour period vary. Raney and associates'”” used a knee-ankle-foot orthosis (KAFO) that produced a valgus force by three-point pres- sure in 60 tibiae (38 patients), with lesions in 54 whiae (90%) resolving without surgery. Significant risks for failure included ligamentous instability, patient weight greater than the 90th percentile, and late initiation of bracing. OF the 54 tibial lesions that resolved, 27 were treated by full-time orthotic use, 23 by nighttime use only, and 4 by daytime use. Three of the six tibiae requiring surgery had been treated with full-time orthotic use and three with nighttime use only. Based on these findings, the authors conjectured that nighttime-only bracing might be as efficacious as full- time bracing, although they acknowledged that one inher- ently would expect daytime use (Le, during weight bearing) to be the most important factor in suceessful orthotic treat~ ‘ment. On the other hand, Zionts and Shean’” reported FIGURE 22-14 A and B, Preoperative plain radiograph and Corresponding MRI with a line marking the area of planned metaphyseal resection. Intraaperative fluoroscopic image Showing the path ofthe curved osteotome, B, Radiograph following resection of the metaphyseal “beak” and careful ‘curettage of the physis.E, Resection was continued laterally unt the epiphyseal bone, phys, and metaphyseal bone were uuncoveted. The Cobb is elevating the joint surface under the medial meniscus. F,Fuoroscopic shot after corrective osteotomy performed below the tibial tubercle, A fibular segment ‘was resected at the same level, Note the Bovie cord was used to [assess the mechanical axis intraoperatively, G and H, Another case ‘example toilusvate the Craniopast interposition material pinned 10 the epiphysis Subsequent follow-up showed persistent growth disturbance noted by the asymmetsic Harris growth ins. in retrospect, the metaphysealphyseal resection may not have ‘extended far enough lateral. congruity and removal of varus instability provide rationale for this treatment. Remember that the intraarticular oste- ‘tomy represents one component of the procedure and that 1 separate proximal tibia osteotomy is required to correct the mechanical axis, the so-called double level osteotomy. (Fig, 22-16). The realignment component can be achieved acutely or gradually,” which has the advantage of concur- rent deformity correction and lengthening because compl tion of the lateral proximal tibia and fibula epiphysiodesis is also performed. ‘Although the plateau elevation procedure has been per formed for more than 20 yeats,"*"™ long-term outcome studies are not available. Short-term results are genet encouraging with reported decrease in pain and instabil- “ satisfactory healing, and reconstitution of the tibial "© The ability of the proposed procedure to main- {ain joint congruity and mechanical alignment over time and prevent carly medial compartment, degenerative disease, characteristic of stage VI deformity,” remains to be proven Furthermore, no comparative studies of single proximal anddouble evel tibia osteotomies exist. The intraarticular oste- tomy must be considered a final salvage procedure in older patients with severe joint deformity secondary to inade- quate carly treatment of infantile tibia vara Technique, Fhuoroscopic visualization of the proximal end of the tibia is essential to perform the osteotomy without intraarticular displacement of the medial plateau. A straight anterior incision is made to expose the medial plateau proxi- ral to the tibial tubercle." The osteotomy begins distal to the insertion of the medial collateral ligaments. A series of holes (see Fig. 22-16, A) traving the curve of the osteotomy and stopping just short of the subchondral bone, just lateral to the tbial spine, are drilled in an anterior-to-posterior direction (with the popliteal structures protected). The drill holes are then connected with a curved osteotome (see Fig, 22-16, B and C), and the medial plateau fragment is gradually hinged proximally while maintaining the articular surface intact. With the knee in maximal passive valgus stress, the medial fragment is hinged upward to close the “gap” under the medial femoral condyle. Once the maximal clevation desired is achieved, provisional xation with inter- fragmentary pins or screws is then followed by a buttress CHAPTER 22 FIGURE 22-15 Aand B, Schematic correction of the media joint line depression by an intaarcular osteotomy to elevate medial plateau, combined with angular Correction of tibia vara. €, Sioping ofthe ‘medial plateau in severe Blount disease D, The extent of instability and the amount of desired plateau elevation are estimated from varuswvalgus stress fhuorascapy,, plate to maintain the opening wedge, and the defect under the medial plateau is filled with iliac crest bone graft 4 structural strut graft (autograft or allograft), oF both (see Fig. 22-16, D to F). Completion of the lateral bial and fibular epiphysiodesis is important to prevent further asymmetric growth, and corrective high tibial osteotomy is necessary to restore the mechanical axis. These components of the procedure can be performed simultaneously of in a staged fashion after ensuring consolidation of the plateau elevation. ‘Complications of Surgery. Complications of proximal tibial ‘osteotomy in a growing child can be numerous. The oste- ‘otomy must be performed distal to the tibial tubercle to avoid growth arrest. Injury to the proximal tibial physis at, the level of the tibial tubercle produces proximal tibial recurvatum, with resulting hyperextension instability of the knee, The optimal site of the osteotomy, distal to the tuber~ cle, isnear the level ofthe trifurcation of the popliteal artery. ‘The anterior tibial artery, which passes through the interos- seous membrane and enters the anterior compartment, can, be injured in as many as 29% of osteotomy procedures."726 SECTION I Anatomic Disorders FIGURE 22-16 Medial plateau elevation technique. A, A series of anterir-to-posterior dr ‘osteotomy, B and €, The ail holes are connected by a curved osteatome, withthe medal plateau segment gradually hinged upward, , Provisional stabilzation of the elevated segment is accomplished withthe knee in maximum valgus stress. Eand F, The medial plateau is stabilized and butressed with appropriate intemal fixation and bane grafting ofthe defect creates under the plateay, (Courtesy ) Fie Gordon, MD.) Prophylactic fasciotomy of all the compartments should be performed during all osteotomy procedures, with appropri- ate postoperative neurovascular surveillance for the first 48, hours. "Other reported complications include peroneal nerve palsy, deep and superficial infections, iatrogenic frac- tures, and loss of correction.” Unexpected recurrence of varus deformity in early-stage Langenskiold lesions may be due to inadequate correction ‘or loss of correction, with subsequent progression of the Langenskiold stage and early asymmetric physeal closure. IF such recurrence happens within | or 2 years of the oste- ‘tomy, repeat osteotomy and medial epiphysiolysis with placement of interposition material may correct the problem, particularly in a skeletally immature patient.” Failure of physeal bridge resection to at least maintain align- ‘ment is usually an indication for epiphysiodesis of the lateral half of the proximal tibial physi, with later limb-lengthening ‘equalization procedures used as necessary. Summary. As can be readily discerned from the complex treatment options and numerous complications discussed "References 95,127, 153, 166, 171, 197,205 holes are made to outline the path of the under the treatment of Langenskiald stages IV to VI lesions and the risks involved in general for any osteotomy or repeat procedure, early treatment aimed at curing infantile tibia vara is far more attractive and more likely to produce @ good outcome than later treatment of the more advanced condition.”?**""* Early diagnosis plus corrective treatment (orthotic or osteotomy) by 4 years old is the most reliable ‘way to avoid a poor outcome in both joint and leg function and cannot be overemphasized. Adolescent Tibia Vara The adolescent form of tibia vara, less common than the infantile form, isa distinctly different entity because of the later age at onsct and, consequently, the more mature physis and more osslied chondroepiphysis, which are more resis- tant to mechanical compression and disruption. In the original description by Blount," the adolescent form was defined as occurring after 6 years of age, and Langenskiéld!”""" used the term adolescent to describe partial premature closure as a result of trauma or infection Jn patients between 6 and 13 years old, A mote widely accepted definition used at our institution deseribesincreas- ing tibia vara alter 8 years of age in a patient who is usuallymale, morbidly obese, and without a history of treuma, infection, or other physeal insult to explain the proximal medial tibial physeal inhibition, ‘Although some authors have subdivided patients with onset after 3 years of age into a juvenile group (4 t0 10 years old) and an adolescent group (11 years or older)" it ean arguably be determined that in most cases the “juvenile” onset merely represents the lack of definitive radiographic diagnosis before age 4, perhaps in the setting of milder clinical deformity. Furthermore, the adolescent form has litle in common with the so-called juvenile tibia vara with ‘the response to osteotomy generally being more favorable inthe adolescent form and recurrence more common in the juvenile form, as would be predicted fora child with infan- tile tibia vara treated after 4 years old.*° =" Finally, some authors have used the term late-onset tibia ara to inchide both the juvenile and the adolescent types," whereas others use late-onset tibia vara inter- changeably with what is normally understood to be adolescent tibia vara."”**! For this discussion the term adolescent is used exclusively to refer to those with onset after age 8." Etiology Adolescent tibia vars is frequently observed in patients who had a mild degree of physiologic genu varum as younger children that never completely resolved to neutral align- ment or physiologic valgus." Concurrent with the adole cent growth spurt in children who are significantly obese, a gradual varus of the proximal end of the tibia develops because of growth suppression from mechanical causes.” ‘Although such occult varus can be confirmed by the history in many cases (family photographs are often available), not all investigators have been able to determine either that a mild varus preexisted' or that it was required for adolescent tibia vara to develop..”*" Trauma and infection, sometimes described as etiologic factors and known to produce physeal arrest," are not considered factors in the development of adolescent tibia vara (which technically is idiopathic) unless one wishes to define chronic growth suppression secondary to obesity as trauma. Histopathologically, biopsy specimens of the medial physis show evidence of injury, with fissuring and clefts in the physis, fbyovascular and cartilaginous repair tissue at ‘he physeal-metaphyseal junction, and disorganization and sequestered islands of hypertrophic chondrocytes." Although these findings cannot be considered pathogno- monic of repetitive trauma, in the absence of a history of significant trauma or infection they are consistent with rieroscopic damage secondary to mechanical compression according to the HuetesVolkmann principle.” True bony bridges have rarely been demonstrated in specimens from adolescent tibia vara, suggesting that the onset of the repetitive “trauma” occurs once the physis and epiphysis are much more developed than in the infantile form. Furthermore, mechanical realignment to unload the compressed medial physis is usually successful in curing adolescent tibia vara, a is gradual mechanical realignment produced by a lateral epiphysiodesis. Consequently, even ‘hough there may be marked clinical deformity and signifi- cant radiographic physeal widening (evidence of disrup- tion), the actual histologic insult to the physis must be CHAPTER 22 Disorders of the Leg 727 relatively moderate, as evidenced by its acute or gradual response to mechanical unloading ‘The additive effect of vitamin D deficiency on a growth plate susceptible to high mechanical loads is under invest- ation. A retrospective analysis of obese children identified «relationship between vitamin D deficiency and likelihood of adolescent tibia vara."" However, confirmation. of low vitamin D levels as an independent risk factor for develop- ing Blount disease is not yet determined. Glinical Features ‘The typical patient with adolescent tibia vara is a male teenager, often black, whose body weight greatly exceeds 2 standard deviations (SD) greater than the mean (Fig. 22-17), At our institution, patients weighing up to 200 kg in the early teenage years have been treated. The average weight of patients with adolescent tibia vara_has been reported to exceed the 95th percentile for age by a mean (of 43 kg.” Involvement is frequently unilateral, ut bilateral «cases are also seen. The preponderance of male patients has not been explained. Patients may seek medical care either because of the deformity itself oF because of the deformity with symp- ‘toms. Many patients are essentially asymptomatic but, on close questioning, may describe an aching in the medial or anteromedial portion ofthe knee associated with activity or ‘occurring toward the end of the day. An area of tenderness along the medial joint line is almost universal, and occas ally there are patellofemoral_ complaints. Internal tibial torsion is frequently present but variable in severity. In unilateral cases, limb length discrepancy is generally present, and the choice of treatment may be influenced by a discrep: ancy greater than 2.5 cm, Investigation of other conditions associated with obesity completes the evaluation of an adolescent with tibia vara, Because of the concomitant external rotation of the thigh in obese patients, slipped capital femoral epiphysis right be expected. Simultaneous occurrence of these con itions has been reported but rarely. OF greater concern, 4 high incidence (61%) of obstructive sleep apnea among adolescents with tibia vara has been recognized at one institution, warranting thorough preoperative evaluation to avoid potential perioperative airway complications in these patients.” Radiographic Findings Radiographiclly, the shape of the tibial physis is relatively normal, without the depression and beaking in the metapi- ysis that are typical of infantile tibia vara (Box 22-2). The sine qua non of diagnosis s widening ofthe proximal medial phys. This widening may be restricted to the medial fourth ‘of the physis, or it may extend completely across the proxi- ‘mal ubial physis, suggestive of epiphysolysts, as might occur ‘with slipped eapital femoral epiphysis (see Fig. 22-17).The widening of the medial tial physs is sigaiicanly: more than that on the lateral side of the physis or the physcal width in the normal, contralateral knec.'* The lack of sloping or inferior beaking of the medial proximal aspect of the tia (with absence of medial articular depression) sug- gests that the proximal tibial physis and chondroepiphysis formed and ossiied normally for a number of years before being compressed later in childhood, coincident with the i728 SECTION It Anatomic Disorders wb {A and B, Clinical appearance ofa 13-year-old, 170-4g child with adolescent tibia vara. € and B, Radiographs ofthe right and let knees showing (ypical widening of the medial iblal physis and generalized epiohysolysis across the enlie proximal growth plate. ‘Abrupt angulation at the epiphyseal metaphyseal junction is evident. E, Radiographic appearance after bilateral high tial osteotomies with correction toa neutral mechanical ais. F, Close-up view of the knees T-year postoperation. Both proximal tiblal physes ae closed, ‘wnereas both distal femoral physes remain open. G and H, Postoperative clinical appearance. Despite attainment of a radiographic neviral axis, valgus “overcorrection” is evident. Box 22-2 Radiographic Features of Adolescent Tibia Vara ‘+ Shape of the epiphysis relatively normal * Lack of beaking ofthe medial tibial metaphysis “+ Widening ofthe proximal medial physeal plate, sometimes extending across to the lateral side of the physis “Widening ofthe lateral distal femoral physs in comparison to either the medial femoral physis ofthe same knee or the distal femoral physs of the normal knee weight gain and adolescent growth spurt in a susceptible individual, In addition, there may be widening of the lateral distal femoral physis when compared with either the medial femoral physis of the same knec or the distal femoral physis ‘of the normal knee. *"* This traction widening on the lateral side of the varus deformity of the femur would appear to bbe consistent with Wolff's law (that bone remodels accord. ing to the stress placed on it), although interestingly, there is usually no localized widening of the lateral proximal tibial physis. Distal femoral varus, described in several studies, probably results from the tension over growth suggested by localized widening of the lateral proxi ‘mal tibial physis and can be detected by measuring lateral distal femoral angle (normal, 88 degrees; range, 86 90 degrees). As opposed to the situation in infantile tibia vara, where femoral varus is not routinely present, the femoral vatus in an adolescent exceeds 10 degrees beyond normal in nearly 20% of patients. In one report, correction of femoral varus was necessary in nearly two irds of patients undergoing simultaneous proximal tibial correction, Distal tibial valgus—presumably a physiologic adaptation, the proximal tibial varus in the same bone—may also develop in a significant number of patients,” although this finding has not been consistently present in other series, Treatment ‘Treatment is predominantly surgical. Orthotic management in patients with this degree of obesity is impossible and ineffective. Weight loss is undoubtedly desirable and should be recommended. Howe is probably not curative once 1¢ deformity is established and, because of the morbid obesity of most patients, is unrealistic to expect, The goal of surgical treatment is to correct the m at physeal growth, if any remains, is restored and dep erative arthritis of the medial compartment of the knee can be avoided, Long-term studies correlating various radio- graphic parameters with clinical outcome scores and pain scales are currently lackingOsteotomy. High tibial osteotomy is the standard and most direct method to correct adolescent tibia vara. Correction to a neutral mechanical axis is sufficient to restore growth, from the medial physis and thus prevent recurrence, although premature closure of the entire physis has been observed after high tbial osteotomy (see Fig, 22-17). Over- correction in adolescent tibia vara is contraindicated," as, opposed to the recommended intentional overcorrection into valgus for infantile tibia vara ‘There is an interesting dilemma when contemplating correction of the mechanical axis in these patients, in that correction to a neutral mechanical axis in patients with morbidly obese thighs actually produces an unsightly cos- metic result that appears to be excessive valgus. In addition, when these patients undergo correction to neuteal align- ment, they may have difficulty ambulating because their ‘highs impinge during normal gait after mechanical realign~ ment (see Fig. 22-17, G and H). Although undercorrection| may invite recurrence of the deformity, it has been our cbservation that a final femorotbial angle of 0 to 5 degrees ‘varusis probably the best compromise between the mechan~ teal correction desired and the problem posed by the massive proximal thigh girth ‘Valgus-producing high tibial osteotomy with rigid inter- nal fixation for acute correction of the mechanical mallign- ‘ment is probably the most commonly used approach. Again, because of the massive thigh girth, external immobilization with casts is not effective in maintaining alignment of the osteotomy, and rigid internal fixation is far superior. The obvious challenge with this approach is that correct align- ment must be achieved at surgery because it cannot be changed postoperatively without reoperation. Loss of fixa- ‘ion and inappropriate intraoperative alignment are causes of postoperative undercorrection or overcorrection."" "| In addition, the well-known complications of high tibial osteotomy (eg, nervepalsy,compartmentsyndrome,'"”"* infection, delayed union or mahunion, and in this patient population, the possibility of deep vein thrombosis) make acute correction a formidable surgical task. Special large- circumference tourniquets are required for intraoperative hemostasis, and the logistics of performing high tibial osteotomy in a very obese patient must be taken into account before bringing such a patient into a pediatric operating room. Emphasis on recognition and treatment of distal femoral varus has added a further dimension of complexity to treat- ment of adolescent tibia vara. Femoral varus of more than 5 degrees beyond the normal femoral. mechanical axis {lateral distal femoral angle >93 degrees) has been reported in more than SO% of patients, with 19% having deformities of more than 10 degrees of varus.” Distal femoral varus was corrected acutely in two thirds of limbs undergoing simul- ‘ancous proximal tibial osteotomy with correction by exter- ral fixation, the indication being mechanical femoral varus preater than S degrees beyond normal.” The rationale for correction by external fixation (see "Realignment by Exter nal Fixation’) isthe adjustability of the postoperative limb position to avoid malcorrection in these obese patients, in whom intraoperative control of alignment is challenging Pain-free and clinically stable knees were reflective of the short-term results studying this comprehensive approach, but avoidance of early degenerative joint disease remains CHAPTER 22 Disorders of the leg 729 unproven."* Because there are no reports comparing these results with proximal ubia osteotomy alone, the value of the additional levels of correction remains uncertain Realignment by External Fixation. The problem of achiew= ing the desired alignment intraoperatively in this patient population is more challenging than is often acknowledged, and the inability to postoperatively adjust intemal fixation is a significant disadvantage." In addition, limb length inequality may exist in unilateral cases, and the ability to achieve equalization by lengthening after angular realign- ment makes external fixation all the more attractive. A direct retrospective comparison of acute versus gradual cor- rection in a small series of patients using external fixation suggested a higher frequency of accurate angulation and ‘mechanical axis correction with the gradual approach, but the clinical relevance of such small differences in radio- graphic measurements is unclear.” The major disadvantages ‘of external fixation are pin or wire complications, which can produce loosening, sepsis, or nerve palsy, and joint stiffness and muscle weakness, which can complicate prolonged treatments. Because the total treatment time with external fixation is definitely longer than the 6 to 8 weeks until union by conventional osteotomy, these disadvantages may have a significant effect on the ultimate outcome. ‘Three methods of external fixation have been reported, (1) External fixation can be used to align an extremity acutely after complete tibial and fibular osteotomy.” (2) Alternatively, a corticotomy technique with gradual corrce~ tion (distraction osteogenesis) can be performed with either circular or monolateral external fixators (Fig. 22-18).' (3) Hemichondrodiastasis, or asymmetric physeal distraction, has also becn used, with mixed results, depending on the rapidity and strength of bony consolidation in the physeal istraction gap."”"""” Because of the almost certain physeal closure after physeal distraction methods, use of asym- ‘metric physeal distraction is limited to patients nearing skeletal maturity. The time to consolidation with physeal distraction can be prolonged,'” and thus this approach appears to offer litle advantage over conventional metaphy- seal corticotomy, ‘The trade-off for the postoperative adjustability of exter- nal fixators isa prolonged treatment time that averages 12 ‘weeks to union or frame removal and not infrequently takes up to 6 months in the frame.” "=" Obese patients may have difficulty engaging in postoperative rehabilitation activities with a circular frame and walker or crutches, thus leading to increased reliance on non-weight-bearing ‘mobility. This, in turn, can slow bony consolidation, Knee discomfort as'a result of fixation wires near the joint may compound the immobility, consequently, half-pin_ tech- niques that involve more anterior pin placement are better tolerated, Although correction by external fixation has the advan- tage of postoperative adjustability, the technique is just formidable as traditional osteotomy. Gradual deformity correction carries a different set of problems related to ‘maintaining function and tolerating longer treatment periods with potential fixation and pin complications "References 38, 49,50, 51, 73, 78, 203 i730 SECTION I Anatomic Disorders FIGURE 22-18 A and B, Clinical and radiographic appearance of a 13-year-old with adolescent tia vara, There fsa 2-cm shortening on the left €, Radiograph cbtained after gradual correction by extemal fixation. The regenerated bone was allowed to consolidate. Total time inthe fame was 14 weeks D, Final result after frame removal 1 an FIGURE 22-19 A, Radiographic appearance of a 13¥%syear-old boy with adolescent tibia vara on the lft. Only the medial fourth of the proximal physs appears widened. Excessve physiologic genu vaigum is present onthe right. B, "Windswept" clinical appearance ofthe Combination of deformities. €, Fuoroscopie view of lateral proximal tibial epiphysiodesis performed va a miniincsion. The peripheral fourth of the physs is treated by curettage (depth, 1.5 cm). D, Clinical appearance 1 year after left lateral tibial and right medial distal femoral epiphysiodesi Lateral Epiphysiodesis. Because of the potential complica tons associated with proximal tibial osteotomy and cither acute or gradual correction, correction by lateral proximal tubial epiphysiodesis is an attractive alternative procedure. ‘The technique is significantly simpler and associated with ‘minimal morbidity, the only real complication being that angular correction is at times not realized. Assuming that the epiphysiodesis is technically adequate, incomplete or inadequate correction may be due to a medial physis that is simply too suppressed to respond to the tethering effect, ‘of the lateral epiphysiodesis. ‘The main disadvantage of lateral epiphysiodesis is that rotational deformity cannot be addressed, Patients treated with this technique have a 50% to 87% response rate in which correction of the varus malalignment, is judged to be satisfactory at maturity and no further treat ment is necessary (Fig, 22-19). Our institution ported a failure rate, defined by need for osteotomy or mechanical axis deviation exceeding 40 mm, of 65% follow- ing lateral hemiepiphysiodesis. Factors associated with a higher risk of failure included an age older than 14, BMI greater than 45 kg/m’, and more severe varus deformity. While variable amounts of correction can be expected, per- forming a lateral epiphysiodesis in no way complicates sub- sequent correction by high tibial osteotomy and remains 8 viable option for initial treatment, particularly in younger patients with mild-moderate deformity. Surgical decision making should weigh the benefits of a less morbid proce- dlure against 2 more extensive but predictable realignment achieved with an osteotomy:FIGURE 22-20 Cavitation of the peripheral physi including ‘metaphyseal and epiphyseal extension, to ensure adequate ablation for epiphysiodess A lateral cpiphysiodesis can be performed either as an ‘open procedure or percutaneously (Fig. 22-20). The pereu- taneous technique probably has a higher rate of failure ‘because of technical inadequacy of the actual curettage of the physis."”’ In addition, the main advantage of the percu- tancous technique, cosmesis, may not be a critical factor in treatment of this patient population. We have found that an ‘open epiphy'siodesis performed through a miniincision under Auoroscopic control is reliable in terms of ensuring that the lateral physis is obliterated by curettage under direct vision ‘Allocal bone graft from the metaphysis and epiphysis imme- diately adjacent to the physis is packed into the physeal excavation. In selected patients, a distal lateral femoral ep- physiodesis can also be performed if there is radiographic evidence of distal femoral varus. We have not performed proximal fibular epiphysiodesis as part of the treatment of adolescent tibia vara in the 13- to 15-year-old patient population. A significant difference in fibular overgrowtls ‘was reported when proximal tibia epiphysiodesis was per- formed alone compared with combined fibular epiphysiode- sisand controls, with an estimated rate of 3 mm/yr reported from one institution.’ Unless expected overgrowth exceeds 2 em, benelits of this added procedure are unlikely because symptomatic fibular overgrowth is not anticipated. Hemiepiphyseal Stapling. Lateral hemicpiphysiodesis of the proximal end of the tibia (and distal end of the femut) can also be produced by insertion of Vitallium staples span- ning the physis. This procedure, introduced by Blount and Clarke in 1949," theoretically ereates the possibility of a CHAPTER 22 Disorders ofthe Leg 731 transient hemsiepiphysiodesis and should be used in younger patients in whom eventual removal is planned when the deformity has corrected, or slightly overcorrected, and growth remains. Stapling can be effective in patients with late-onset tibia vara with mild to moderate deformity and chronologic age of 12 years or younger." In this setting, 27 of 29 patients obtained a successful or partially success- ful outcome from stapling, with minimal morbidity from the procedure itself or from a second procedure to remove oor revise staples, required in approximately a third of patients. Severe preoperative varus (zone 4 medial mechan- ical axis deviation in the Mielke-Stevens classification’) and older adolescents with less growth remaining will not benefit from stapling," just as a permanent hemiepiphys- iodesis by curettage might also be ineffective. The timing of the stapling procedure is not as crucial in voiding overcorrection as it may be in permanent hemiepi- physiodesis, although patient compliance with follow-up is ‘mandatory with either procedure. We have observed that most patients with adolescent tibia vara have advanced bone age, and thus there isa lessened risk for valgus overcorrec- tion with permanent hemiepiphysiodesis in patients older than 11 yeats. Stapling has its greatest indication in patients ‘younger than 11, in whom valgus overcorrection isa distinct possibilty with any form of hemiepiphysiodesis, inchiding stapling,” and thus the transient method with appropriate follow-up is more useful Hemiepiphyseal Plating. The advent of extraperiosteal plate and screw systems introduced an alternative method to nonpermanent deformity correction in patients with ‘open physes (Fig. 22-21). The technique employs 2 tension- band concept as opposed to the physcal-compressive effect ‘exerted by stapling and has proven to be equally effective in restoring mechanical alignment." However, awareness of potential technical pitfalls when using these devices is essential as more mechanical failures have been reported in ‘obese patients characteristic of adolescent tibia vara’? (see Fig, 22-21). The intersection of the screw shank at the lateral cortex in the metaphyseal region accounted for the failures in nearly al cases. Biomechanical testing of differ- tent guided growth constructs suggests fatigue strength is superior for solid compared with cannulated screws with stainless steel screws yielding the highest number of cycles to faire” When using extraperiosteal plating in this high- risk population, consider adding s second parallel plate or 2 single four-hole plate, noncannulated screws, and possibly stainless steel implants to avoid potential screw breakage and the need for revision surgery. Tibia Vara Secondary to Focal Fibrocartilaginous Dysplasia ‘Occasionally, an infant or toddler is seen with unilateral varus of the tibia, the deformity appearing slightly more distal than the knee joint itself If the child has reached standing age, hyperextension of the knee may also be noted (Fig, 22-22). This latter finding is not usually present in a toddler with infantile tibia vara, although the lateral thrust seen in stance phase can mimi¢ this deformity. The radio- _aphic finding of focal fibrocartilaginous dysplasia separates this entity from infantile tibia vara, as do the pathologic Findings discovered during surgical treatment. i732 SECTION I Anatomic Disorders FIGURE 22:21 Case example of guided growth using Bplates and faire. A, A 10-year-old male with adolescent tibia vara. B, A lateral “8 plate™ was implanted to tet the proximal Iateral tibial physis. Nine months later, minimal correction of the tibia vara is noted, along with | broken distal serew. D, Close-up of broken implant. E, Revision age 11. F, Final result folowing plate removal Radiographic Findings Radiographs of focal fibrocartilaginous dysplasia (Box 22-3) show a characteristic abrupt varus at the metaphys iaphyseal junction of the tbia, clearly not involving physis (see Fig, 22-22, C). There is cortical sclerosis in and around the arca of the abrupt varus on the medial cortex. ‘A radiolucency may appear just proximal to this area of cortical sclerosis, which probably corresponds to the fibro- cartilaginous tissue found at surgery in the area of insertion ‘of the pes anserine tendons. The etiology of this defect and. the path sgenesis of the deformity are unknown, Box 22-3 Radiographic Features of Tibia Vara Secondary to Focal Fibrocartilaginous Dysplasia “Abrupt varus at the metaphyseal-iaphyseal junction of the tibia not involving the physis + Cortical sclerosis in and around an area of abrupt varus on the medial cortex + falueny poss appeatng jus proximal tothe ea