Coxa Vara PDF

CONGENITAL COXA VARA

BY FRANK SHALEEN BABB, M.D., ROCHESTER, MINNESOTA

Fellow in Orthopedic Surgery, Mayo Foundation

RALPH K. GHORMLEY, M.D., ROCHESTER, MINNESOTA

Section on Orthopedic Surgery, Mayo Clinic

AND CARL C. CHATTERTON, M.D., 5T. PAUL, MINNESOTA

Chief of Staff, Gillette State Hospital, St. Paul

Any deciease in the angle formed by the femoral neck with time fenmolal shaft is re-feired to as coxa varatm4. Acquired coxa vara is a common deformity and may be dime to avariety of causes. Congenital coxa vara is relatively infrequent and of unknown etiology.It occurs sufficiently often, however, to make its recognition as a distinct clinical entitymost important. The authors propose, therefore, to summarize the current concepts of

this deformity, to differentiate it from multiple congenital deformities, and to repoitfifteen cases.

Some confusion has arisen regarding congenital coxa vara, as evidenced by the follow-ing titles under which it has been described: so-called congenital coxa vara 8,44 infantile coxa vara 1.2, 7,32,44 and developmental coxa vara . These authors are allreferring to a lesion best defined by Fairbank as a form of coxa vara occurring in chil-dren and associated with radiographic changes in the neck of the femur which are suffi-ciently characteristic to distinguish it from all the other types of this deformity . It is thislesion, characterized by a vertical fissure in the femoral neck H, with which this paper isconcerned.

REVIEW OF THE LITERATURE

In time year 1881, disabilities of the hip were still being considei-ed as tuberculous ornon-tuberculous, and congenital dislocation was one of the more popular non-tuberculousdiagnoses. Fiorani, in that year, found fifteen cases in which previously a diagnosis ofcongenital dislocation had been made, and concluded that this rare form of limping wasin effect due to a bending of the femoral neck. Little attention w-as attracted by his article,but MUller, in 1888, gave the first full anatomical description of coxa vara 12; however,the condition he described was apparently an epiphyseal separation 42#{149}Hofmeister, in1894, is generally given credit for coining the term coxa vara 41,42#{149} Finally, in 1896,Kredel gave the first detailed description of congenital coxa vara 3,26,44#{149}Zadek imas givena careful review of the early articles describing this condition.

In 1899, Whitman made an impassioned plea for a moie careful classification of eoxavara on time basis of etiology. As though in answer to this paper, Hoffa, in 1905, publishedhis now famous monograph in which he reported two cases, undoubtedly congenitalcoxa vara, and included the first report of the microscopic pathological findings of thelesion in the femoral neck.

The period from 1905 to 1913 is characterized by a series of isolated case reports andtwo outstanding papers. Delitala reported one case and included a detailed account of themicroscopic pathological findings. This was the second such report. Fairbank 14 statesthat Elmslie, in reporting on two gross specimens, was the first to recommend designatingcongenital coxa vara as infantile coxa vara . Although this term has much to recom-mend it, it unfoi-tunately has not been universally a(lopted.

8 Abridgmimenmt of timesis submitted by Dr. Babh to time faculty of the Graduate School of time University ofMinnesota, in partial fulfillment of the requirements for the degree of Master of Science in OrthopedicSurgery.

VOL. 31-A NO. 1. JANUARY 1949 115

116 F. S. BABB, R. K. 6HORMLEY, AND C. C. CHATTERTON

From 1913 to 1924, very little was written on this subject. Then, in 1924, Nilsonnereviewed the literature and reported five cases; he was the first to suggest an embryonicvascular disorder as the etiological factor in congenital coxa vara.

In 1926, Noble and Hauser, in a very comprehensive paper on coxa vara in general,gave an excellent description of congenital coxa vara. They attempted to solve the con-fusion on the subject by including all varieties of coxa vara present at birth under timeterm congenital, and then dividing them into four types on time basis of additionalaplasia of the femur and the presence or absence of othei congenital deformities.

Fairbank, in 1927, had the misfortune to have a patient die on the operating table,of a massive pulmonary thrombosis. Subsequent examination of the hip (Iisclosed thatwhereas the femoral neck was not visible on the roentgenogram, it was, in fact, intact butcartilaginous. In the following year, Fairbank contributed to time Robert Jones BirthdayVolume a comprehensive article on infantile coxa vara, including a discussion of the roent-genographic recognition of the deformity.

In the years from 1927 through 1946, approximately seventy references to congenitalcoxa vara have appeared in the literature. Twenty of these are in English. Among thelatter might be mentioned Barrs paper in 1929, in which he reported five cases; an cx-cellent and comprehensive article by Zadek, in 1935; Ollerenshaws presidential addressto the Royal Society of Medicine, in 1938; a paper by Duncan, in 1938, in which he differ-entiated between congenital and developmental coxa vara and reported thirty-one cases;and Goldings paper, in 1939, in which he pointed out that the nine descriptions of micro-scopic pathological findings in the literature up to that date revealed nothing character-istic about this lesion. Of the foreign literature, Camitz in 1934, Pouzet and Tavernierand Pouzet, in 1934, have contributed substantially; reference to them will be made later,under the discussions of etiology and treatment . Among the other ai-ticles w-ritt-en duringthis period were interesting case reports 1,IO,1319,27,28,41,43 and papers on coxa vara in gen-eral 36,38#{149}Very little has been written on this subject- since 1939, which is analogous tothe situation that occurred during and immediately after World War I.

ETIOLOGY

Fimere is still no universal agreement as to the cause of congenital coxa vai-a. Kredelaimd Hoffa expressed a belief that intra-uterine pressure mi-as responsible. Pouzet and1)uncan held that the condition is the result of a developmental error. Such theoriescan neither be proved nor criticized 29 J suffices to say that no hereditary factor hasbeen demonstrated.

Then there are theories that have not withstood investigation. Rickets, which hasnever been shown to be coexistent with congenital coxa vara, can imaidly be responsible.Trauma can be excluded, according to Hoffa, because of the unusual number of cases of

bilateral lesions. B#{246}hmsuggested an atavistic theory, but Herz denied this because anexamination showed that the angle of inclination of simians mi-as not much less than thatof man.

Walmsley, in a careful investigation of the uppei femoral epiphysis, proposed thetheory of a separately ossified diaphyseal spur. There is no doubt but that such a theorywould account for the characteristic triangular fragment, to be described later; but howthis theory would explain the more severe cases, in whicim time fenmoral neck is almost com-pletely cartilagiimous, is hard to understand.

Nilsonne, iii 1924, presented what is perhaps the most attractive etiological theoi-y,-----that of an embryoimic vascular disturbance. Camitz, in 1934, after exanuning inicioscopicsections, concluded that it was impossible histologically to (listingwsh between osteo-chondritis juvenilis (coxa plana) and so-called congeimital coxa vaia. Nilsonnes theoryand Camitz observations would seem to coincide in the postulation of a plausible explana-tion of the phenomena observed ill this lesion. Since time fenmoral neck is not completely

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(ONGENITAL COXA \AlIA I IT

VOL. 31-A, NO. 1, JANUARY 1949

ossified until time cimild is at least four yeai-s of age l2 even a postnatal vascular distuibancemvould explain the subsequent findings. Piergrossi, in 1939, in support of this hypothesis,demonstrated Bertolottis met-aphysitis as an evolutionary stage in congenital coxa vara.I)uncan #{176}Supporte(l timis timeory, and many of those who advocated the term infantile01 developmental instead of congenital 1,2,39,44 appeared to be in favor of it..

This mo(lern teimdeimcy to explain congenital coxa vara as an aseptic necrosis iS makesit all time more essential that the condition be separated from otimer obvious congenitaldeformities. An attempt will also be made to demonstrate pathologically that the coxavara occurring as one of multiple congenital deformities is not time same as this infantilelesion, the cause of which may even be postnatal.

1ATHOLOGICAL CHANGES

Noble and Hauser statethat, at birtim, the upper endof time femur is a mass of carti-

lage. Timere is a single, trans-verse, ascending edge of ossifi-

cation mvimicim, during timefourth year, reaches the upper1)older of time neck. The imeck,being completely ossified atthe foiiitim yeam, sepaiates thecapital epiphysis appearing attwelve months from timat for

time greater trochantei, whichbegins to ossify only in thefourth year. Any disturbanceof ossification of the neck,therefore, must occur in time

first four years of life.. FIG. 1-A

I he earliest stages of coim- . . . . .. Illustrative case of multiple conmgemmital (lefornmlt-les of i)ommes, in-

genital coxa vara are seldonm cludinmg (OXR vara. Coronmal section througim hip joint.seen. Ollenenshaw was simownby Herzog roentgeimogm-ams ilhistiating snmall areas of iarefact-ion in time femoral neckwhich appareimt-ly coalesced to pm-odiice time typical fissuie, Sel(loITl evi(lent before twoyeans of age. By time fifth oi- sixth year of life, the femoral head imas definitely slipped(low-n, and the vertical fissui-e latemal to time epiphysis separates it from time remainder oftime neck. What appears to be time Imead is in reality the anatomical head, the epipimysealcartilage, and a triangular fm-agment of time neck, as will be demonstrated i-oentgenograpimi-

cally.This descemmt of time Imead, theim, due to a defect in time femoral imeck, comistitutes the

gross patimological change, except for the more than occasional coexistence of a short femur.Shortening of time femur occurs at the upper end and appears to he a manifestation of anaplasia timat is the result of some common underlying disturbance in ossification, as dis-(usse(1 jim a preceding part of timis paper. Such clmaiacterist-ics as a vertically (lisplacedepiphyseal line and anteversion of the neck are roentgenograpimic features, not so eVi(lPfltin gross pathological specimens.

1)escriptions of the microscopic pathological appearance of time defect in the femoralneck have been given by Hoffa, Elmslie, 1)elitala, Nilsonne, Camitz, Barr, Zadek, andDuncan Timere is nothing characteristic in the microscopic appearance of tissue re-move(1 fmonm the femoral neck #{176}-8, 11 Barr reported finding the inclusion of embryonic cart-i-lage in normal bone, and the occurrence of non-calcified osteoid tissue has been reported

FIG. 1-B FIG. 1-CSection through epiphysis (X 35).

118 F. 5. BABB, R. K. GHORMLEY, AND C. C. CHATTERTON


Section through mid-cervical region (X 6).

by Noble and Hauser. Otimervise, little can apparently be said about time microscopicpathology. Therefore, in an attempt to arrive at a fundamental patimological criterion forcongenital coxa vara as herein defined, we can say that it is a lesion of the upper end ofthe femur witim an incompletely ossified segment in the neck ; it is composed of cartilageand osteoid tissue ; and it sometimes includes gross aplasia of time upper femoral diaphysis.

The coexistence of multiple congenital deformities of other bones and coxa vara con-stitutes the differential problem. In an attempt to determine whether or not coxa varain this relationship satisfies the pathological criterion laid (10mm previously, such a casewas invest-igate(I.

A white girl, aged ciglmt s-ears, msa.s first seen at time Mayo Clinic in Septenmber 1928. Examination re-veaie(l flmUltil)le (-ongemmital deformimities, including club-feet, flexion (leformities of botim knees and 1)0th hips,ami(I I)ilibteritl COX1t vara. Spina bifida occuita was also present. During a nmanipulation under anaesthesia mm.Jamiuary 1929, tue ciiiid suddenly experiermced severe respiratory distress and (lied shortly afterward. Atnecropsy, (heath m-a.s found to have been due to fat embolisnm.

The resected s)ec-inmens included one hip joint, consisting of part of time inmnormminate hone and the head,neck, and upper l)art of time femoral shaft. This specimen had i)een sectioned in the coronal arid horizontall)laneS through time middle of time head anmd neck. Accurate reconstruction was inmipossible, i)ut moderatecoxa vara appeared to be present, with a coxofenioral angle of about 1 10 degrees. No gross (lefect or triangu-lar fragment was noted in the neck of the femur (Fig. 1-A).

Two blocks were cut , omme to include epipimyseal cartilage amid time other fronmi time mmmid-cervical region,imicludinig the inferior cortex and extending almost across time width of time available neck. Both blocks were(lecalcified before they were sectioned, stained with imenimatoxylin an(l eosirm, and mounted. Examination re-Veale(i a nornmal epipimysis with nornmmal compact and cancellous bone in the cervical region (Figs. 1-B and 1-C).

It must be concluded, therefore, that since the condition (lid not satisfy the patho-logical criterion out-lined previously, the case was not one of congenital coxa vara asherein defined. Sucim an observation, if made on otimer sucim specimens whenever they areavailable, may help to prove that there are two types of congenital coxa vara in inf ants,-

CONGENITAL COXA VARA 119

first, a true congenital lesion accompanied by multiple other deformities, and, second,a disturbance in ossification confined to the upper portion of one or both femora.

CLINICAL FINDINGS

Congenital coxa vara is usually discovered when the child starts to walk . If no de-formity, such as a short leg, had been previously noted, the parents then become alarmedat the appearance of a painless limp. Occasionally the limp is ignored and medical aid isnot requested until adolescence. By this time the presenting complaint is a painful limp 42,with undue fatigue.

Physical examination typically reveals an otherwise normal child. The limp is easily(lemonstrated when the child walks, and resembles that of congenital dislocation of thehip. In fact, in cases of bilateral congenital coxa vara, the waddling gait and time markedlordosis are identical with those of congenital dislocation.

Examination of the hip reveals measured shortening, which is considerable in those

patients vith associated shortness of the femur. There is no spasm or tenderness, althoughal)duction, inmterimal rotation, and sometimes extension are mechanically limited 42#{149} Timegreater trocimanter is elevated and prominent , and the Trendelenburg test is positive onthe involved side 42 The examining physician, suspecting a congenital dislocation, is timensurprised to find that there is no telescoping of the femur and that the femoral head isstill palpable beneath the pulsations of the femoral artery.

As mentioned previously, no other congenital deformities are present in a typical case.

ROENTGENOGRAPHIC EXAMINATION

Roentgenographic examination offers the most readily available means of studyingtimis deformity. Unless one is familiar with the condition, the correct diagnosis is usuallynot made. The femoral neck is at once observed to be bent, so that the head is depressedand the distal part of the limb is thereby adducted 26#{149}The epiphyseal line for the head ismore vertical than it is normally, and it appears to be branched like an inverted V 42#{149}Brailsford states that this abnormal branch runs from the superior medial to the inferiorlateral extremity of the neck, and isolates a triangular fragment in the inferior medialquadrant. It- is not, however, a branching of the epiphyseal line, but a disorganisedsegment , sometimes referred to as the vertical fissure, in which an abnormal ossificationprocess has resulted in a defect- not unlike that seen in aseptic necrosis 18 or localizedosteochondritis 6

The greater trochanter is elevated and may have a peculiar beaked appearance. Thefemoral head is comparatively large, somewhat translucent, and lies in the bottom of theacetabulum. The acetabuhim may be (leformed in outline, and shallow and (lefective in-feriorly ; shadows resembling those seen in osteochondritis have been not-ed 42#{149}

At first glance, such a roentgenogram suggests a fracture of the femoral neck, i)utZadek has stated that such fractures are extremely rare in children. When closer scrutinydiscloses a short, imperfectly formed neck, with a zone of rarefaction which containsosseous nuclei and cuts off a triangular fragment 25,43, one should have the necessary en-teria for a correct diagnosis. When, a-s occasionally happens, the femur is short, owingapparently to an extensive failure of ossification at the upper end, the diagnosis is obvious.It is felt 14 that any such lesion w-ith roentgenographic features so characteristic needs amore specific designation.

The untreated lesion that is seen during adolescence or later is much more deservingof the diagnosis, ununited fracture. In effect, this is usually the state of affairs: Whatwas originally a femoral defect has now become a complete dissolution of continuity.The trochanter may be riding much higher than at first, the deficient neck may have beencompletely absorbed, and true telescoping of the femur may now be present. The femoralhead, strangely enough, is usually present and viable. Not only does this late lesion re-


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semble an ununited fracture, but also, as will be shown later, it should probably 1)e treatedas such.

DIAGNOSIS

1mma cimild, therefore, a diagnosis of congenital coxa vara should be considered for any

l)aiIiless limp resembling a congenital dislocation of the hip, if the femoral head is stillpalpable beneath the femoral vessels and telescoping of the femur is not present. If theroent-genograms suggest a fracture of the femoral neck, the criteria outlined should beconsidered before diagnosis of such a rare condition is made. No particular harm is doneif the lesion in an adolescent person is mistaken for an ununited fracture, since that is infact what is present, even though the etiological agent is non-traumatic.

TREATMENT

Congeimital coxa vara was originally considered hopeless. If it is untreated, nonmalO55it1CLt-iOfl rarely occurs, and the condition proceeds to an established non-union withthe usual sequelae resultant upon a disruption of the hip joint, including arthritis, pain,and increasing disability.

A conservative attitude has been recommended by Barr and by Nilsonne. They haveadvocated non-weight-bearing with the limb in wide abduction and under traction, andBarr has suggested that a Brackett type of reconstruction be done after puberty. Nilsonneagreed that osteotomy is necessary for the more marked deformities.

The aim of treatment, as recommended by Pouzet n, is twofold : first, to promoteossification of the cartilaginous neck, and, second, to correct the deformity. It is generallyagreed timat surgical intervention is necessary to accomplish both of these aims. Surgicalprocedures on the neck of the femur have proved universally unsuccessful In thechild, subtrochantotic osteotomy has by far the greatest number of supporters II, 14, 17,23,27.29,31,33, 11,43,44 In correcting the deformity, this procedure converts what was a vertical(lefect-, across wimich played a sheering force, into a horizontal defect with a compressionforce. This has almost universally proved successful in promoting ossification. AlthougimZadek, in addition to the osteotomy, drills the neck, such a procedure does not appear toi)e necessary. Neither is a bone graft required . The objection was raised by Noble andHauser that subtroehanteric osteotomy offers only temporary benefit. This seems to bewhat Duncan had in mind when he urged extreme abduction of the distal part of thelimb at the time of osteotomy. Peabody 31, in fact, suggested placing the limb in adduc-tion before the osteotomy is done. He claimed that only in this way could the distal seg-ment be brought into line with the proximal portion of the neck, an essential if maximalcorrection is to be maintained. With the advent of internal fixation, such as that securedby Blount with the blade-plate, such procedures as subtroehanterie osteotomy may be(Xpecte(l to yiel(l even i)ett-er results.

In time adolescent or a(hllt group of patients, time ploblem is essentially that of treat-ilig aim tiimtiiiited fracture of time femoral neck. Of the reconst-I-uction operations available,it Brackett type of repair is particularly in favor because of the high incidence of viablefemoral heads found at operation. Should this type of repair prove impossible, selectionof time alternative is obviously governed by the conditions encountered by the operatingsurgeon.

PROGNOSIS

If congenital coxa vat-a remains untreated, the head slips farther and farther downthe shaft of the femur until it comes to lie on the floor of the acet-abulum. To state it morecorrectly, the greater trochanter, atop the femoral shaft, goes higher and higher untilILh)dtlctiOfl is impossii)le, owing to impingement of the trochanter agaitmst time ilium. The

l)mLti(11ts gait i)((01fl(s \VO1S(, iLIl(I l)aitm atRi fatigue add to thu (usability.viii; J()(RNAL O1 hONE ANI) JOINr I4IJRGERY

FIG. 2-A

Case 15. Roentgenogram of hip.

CONGENITAL COXA IARA 123

VOL. 31-A. NO. 1, JANUARY 1919

FIG. 2-B Fn;. 2-C

5(ctionIs fnonnm (listal (-(1gm ( )f lnnimonal hi(a( I . No (-nhl)1-yonlal (ant i lag(- on ( )st-noi( I I ISSU( is visit ,l-.I niset shovs gross aI)l)eananire of fenmonal h(-a( I.

JIG. 3-A

(use I . 11)1(itl ((mmIg(-niital C(IXIL vara of right trip, at tlnn-e yettis of age.

I 24 P. 5. BABE, II. K. GHORMLEY, ANt) C. C. CHATTERION

-ilii; JOURNAl. ()I 1l(NI-. AND .O)1NT SI

It has been said that with efficient immobilization, Imealmg occur-s vithuim about t-voyears, just as in Legg-Perthes disease. Early invest-igatoi-s who tried to accomplish timisresult with complete rest and the linmb in abductioim and ti-action were (lisappointed.4Such treatment does not constitute adequate immobilization of time defect-, eit-Imer in coavara or in fracture of time femoral neck. Until the vertical (lefect imas been conTecte(1 into ahorizontal defect witim a compression force a-cross it, healing will not take Place. Subt-no-chanteric osteotomy accomplisimes this effect, and the entimusiasnm for this form of treat-ment, p1115 the results in this series and otimers, bears out the claims ma(le for it.

The prognosis in adolescents and adult-s is obviously not so good as in the childtreated by subtrochanteric osteotomy at about six years of age. Some of time latter caneven expect a imip functionally as good as on the normal side. In older l)eisons, iiomvever,irreversible traumatic arthritis may already have been established. Hecoimstiuction opela-

lions on adult Imips are not likely to restore a hip to imormal. Therefore, tue ptogimosis ittthese hips mi-ill vary with the condition, time patient, and time sut-geon.

THE CASES iN TI-lB PRESENT STUDY

Of time fifteen cases reported here, all of the patieimts imave beeim seetm or treated sinmce1927, ten since 1937. Cases 1 to 10, itmclusive, were treated at Gillette State Hospital;Cases 11 to 15, inclusive, were tteated at the Mayo Clinic (Table I). The case numi)ersreferred to are timose given in Tainle I.

This series included seveim males and eight females ; onie gii-l was a imegro. Time rigimthipalone was involved in nine cases, time left imip alone in two cases, an(l botim hips in timeother four cases. Time aenage age at time time of a(lmissiolm was 8.8 years, although themajority of patient-s had ima(l symptoms ft-urn infancy. live of time l)mmtielmts vere 1)011m wit-h

Fn;. 3-B FIG. 3-CAl)1)CLrLm1CC at age of eight years. Inimnl((hiately after operationi.

FIG. 3-D

Three years after operation.

CONGENITAL COXA VARA 1 2?


the involved exttemity obviously slmott, and in each case time shot-tening was (Inc primarilyto hypoplasia of time upper end of the femur. Seven patients complained of a painless limpor wad(lling gait, witim onset shortly after they had begun to walk. In time other threecases the limp (hi(l not (levelop until the ages of five years, twelve years, and thirteeny(ans, iPSj)PCti\el. The last two patients Imad only minor (iegnees of this (leformity. lime

TUE JOURNAL OF BONE AND JOINT SURGERY

I 26 F. 5. BABB, B. K. (HORMLI\, ANI) C. C. CHATTI-IITON

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FIG. 4-D

Eighteen months after last operation.

CONGENITAL COXA VARA I 27

VOL. 31-A. NO. 1, JANUARY 1949

average age at operation was 1 1 .5 years. The family histories, birtim histories, and pimysicalexaminations were not remarkable, except that only two patients were smaller in stature

FIG. 5-A

Case 1 1 . lvpical congemutal coxa yam mma young a(lult , at age of t-wemity-tvo years.

FIG. 5-B FIG. 5-CImmne(Iiat,eiy after subtrochanteric osteotomy (Brackett reconstruction was technically

I 2 F. S. IIABI4, 11. K. CHORMLEY, AND C. C. CILATTERTON

THE JOURNAl. OF BONE ANt) JOINT SURGERY

th1LI1 imornmal (Cases 1 and 10). Apait fiom shortening of the femur-, imo congenital nie-formities were noted except in Cases 8, 10, 14, and 15, to which special reference will bemade.

No Surgical tteatment was carried out in Cases 8, 10, and 14. In Case 8 the patienthad a congenitally short tibia on the left, and the coxa vara of the right hip was an mci-dental finding. The roentgenogram in this case did not reveal a vertical fissure, and timeflattening of the head mi-as more character-ist-ic of coxa plana than of congenital coxa vara.

Fig. 5-B:inmipossible).

Fig. 5-C: Three months after operation.

CONGENITAL COXA VARA I 29

In Case 10 there were multiple congenital deformities and a history of birth injury andrickets. The roentgenogtams in timis case are not typical, and perhaps time case does notbelong in this series. This case is illustrative, as is also Case 14, of time type of congenitalhip deformity from wimich congeimital coxa aia, as imeiein defined, 5110111(1 i)e distinguished.Case 15 is of panticulat intetest and a detailed report follows.

CASE 15. A wimite i)oy, aged niinme years, was first seen at the Mayo Clinic iii March 1947. His complainton admission was timat a simort right lower extremity had been present sirmce birtim. Birth and family historieswere not renmiarkable. Exanmination revealed an otimerwise normal nine-year-old boy, whose right lower ex-tremity wa-s seven inches (17.8 centinmeters) shorter than the left, the shortening all being confined to timefemur. Time trochanter wa-s prominent and higher on the right than on the left; the Trendelenburg test onthe right wa-s positive. Since the femoral head could not definitely be felt beneath the femoral ves.sels andtelescoping of the fermi ur was present, congenital dislocation of the hip was suspected. Rocntgenograms re-vealed the faint outline of a fennoral head still presenmt in the acetabulum, but the femoral neck was not visible;gross aplasia of the upper portion of the femoral (llaphysLs was evident, with time epiphysis for the greatertrochanter i)einig present and riding high on the ilium (Fig. 2-A). The inferior ischial and pubic rami wereununited, and the upper end of the right fibula was absent.

Upon surgical exploration of the hip, a small, incompletely ossified head wa-s found, stuck to time floorof the acetabulunm. It was removed (Fig. 2-B, inset) and a modified Colonna reconstruction of the imip wasperformed.

No fenmoral neck, cartilagitmous or osteoid, was demonstrated grossly. A block was cut from the distaledge of time head, decalcified, sectioned, stained with hematoxylin and eosin, arid mounted. No embryonalcartilage or osteoid tissue, suggestive of a remnant of the neck, could be found.

In this case the lesion resembled clinically that of a congenital dislocation. Roent-genograms suggested a congenital coxa vara with a cartilaginous neck, as in Fairbankscase 13 Surgical exploration, however, revealed that the femur had no neck, a fact whichexplains the telescoping observed on clinical examination. This should probably be classi-fled as a coxa vara associated with multiple congenital deformities, including aplasia oftime right innominate bone, right femur, and right fibula. Such a differentiation, of course,is of more than academic interest, as the treatment is obviously different.

In the eleven remaining cases (exclusive of Cases 8, 10, 14, and 15) the patients wereoperated upon; ten of the patients had osteotomy. In six cases (Cases 5, 6, 7, 9, 11, and12) osteotomy w-as of the subtrochanteric vai-iety and, except for a wire in Case 7, nointernal fixation was required. Uniformly good results were obtained in these cases, cor-rection of the deformity and the promotion of ossification being secured in every instance.It is felt that in the child of six to eight years of age this procedure is the one of choice,and that wide abduction of the limb after osteotomy is essential . In time next higher agegroup, wide abduction is more difficult to obtain. Four such patients (Cases 1, 2, 4, an(113) , whose average age was thirteen years, iequired high osteotomy, and internal fixationof the fragments mi-as used to advantage.

One patient (Case 3) underwent a reconstruction operation. This patient was sixteenyeais of age at the time of operation and imad what amounted to non-union of the femoralneck, with a viable and movable head. A Brackett type of reconstruction, followed bytransplantation of time greater trochanter, proved successful.

The ioentgenogtaphic appearance in Cases 1, 2, and 11 is shown in Figures 3-A to5-C, inclusive.

SUMMARY AND CONCLUSIONS

Congenital coxa vara is an infrequent deformity of the femoral neck in children, rec-ognized since 1896 and characterized by a defect which pathologically and roentgeno-graphically is not unlike that seen in aseptic necrosis. Except for its frequent associationwith a short femur, congenital coxa vara seems to occur entirely apart from other con-genital deformities and to possess sufficiently distinguishing characteristics to warrantrecognition of it as a separate entity.

The lesion is to be suspected in any cimild w-ho is born with an obviously short leg or

VOL. 31-A, NO. 1. JANUARY 1949

130 F. S. BABB, It. K. (tHORMLEY, AND C. C. CHAI1ERTON

who limps wheim walkirmg is first attempted. The findings on physical examination suggesta congenital dislocatioim of time Imip, with shortening, high position of time trochanter, and

a positive Treimdelenburg test. However, there is no telescoping and the femoral head isstill palpable beneath the femoral vessels at the groin. The roentgenogram will confirmthe diagnosis if the lesion is not mistaken for an ununited fracture of the femoral head,a rare condition in children. The aim of treatment is to promote ossification in the neck of the femur and to cor-rect any deformity already present. Subtrochanteric osteotomy with wide abduction ofthe distal part of the limb will correct the coxa vara, and conversion of the sheering strainacross the defect into a compression force along the axis of the neck promotes completeossification in a high proportion of cases.

Adolescent and adult patients with untreated lesions exhibit what amounts to non-union of the femoral neck. Of the reconstructions available for such lesions, the Brackettoperation is particularly suitable because of the high percentage of viable heads found atoperation.

The series of fifteen cases reported here is fairly representative and purposely in-eludes four examples of unusual coxa vara (Cases 8, 10, 14, and 15), from which it wouldseem advisable to differentiate the specific lesion defined in this paper.

It) can be said, then, that congenital coxa vara as herein defined is not a proved con-geimital deformity. The frequent association of this lesion with a short femur is probablydue to a common etiological factor, possibly avascular necrosis. Coxa vara associatedwitim multiple congenital deformities is not the same lesion as that with which this paperis conceined. Subtrochanteric osteotomy at about six to eight years of age, with wideabduction of the distal part of the limb, is the treatment of choice.

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