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Jurnal CDH 1
Jurnal CDH 1
PROGRESS
"There are persons,. who, from birth or from dis- either a complete dislocation of the head of the
ease, have dislocations outward of both the thighs; femur from the acetabulum or a partial displace-
in them, then, ihe bones are affected in like man- ment (subluxation) from the socket. Inadequate
ner, but the fleshy parts in their case lose their development of the roof of the socket (dysplasia)
strength less; . . . They have the equal use of and laxity or elongation of the ligaments and
both their legs, for in walking they totter equally capsule of the joint may be primary pathogenic
to this side and that."' Thus Hippocrates, in features; both will be discussed in greater detail
About Articulations described hip dislocations in later. True congenital dislocation of the hip must
the second century B.C. be distinguished from teratologic dislocations such
CONGENITAL DISLOCATION of the hip is a geneti- as may occur in arythrogryposis multiplex con-
cally influenced condition in which there is a genita, diastrophic dwarfism, Larsen's syndrome
disturbance of the normal anatomy of the hip, and other rare generalized mesenchymal dis-
orders. It should also be distinguished from the
From the Division of Orthopedics and Rehabilitation, University paralytic dislocations which may occur in cerebral
of Oregon Health Sciences Center, Portland.
Reprint requests to: E. E. Specht, MD, Division of Orthopedics
and Rehabilitation, University of Oregon Health Sciences Center,
palsy, myelomeningocele, and following paralytic
3181 SW Sani Jackson Park Road, Portland, OR 97201. anterior poliomyelitis. In true congenital disloca-
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Figure 1.-Barlow's maneuver. With one hand grasping Figure 2.-Ortolani's maneuver (sign of jerking into
the symphysis in front and the sacrum in back, lateral place). After provocation of a dislocation by Barlow's
pressure is applied to the medial thigh with the thumb maneuver, as shown in Figure 1, the hip should be ab-
of the other hand while longitudinal pressure is applied ducted to about 80 degrees while the proximal femur
with the palm to the knee on the side being examined. is lifted anteriorly with the fingers placed along the
The hip, which has been flexed 90 degrees, is then lateral thigh. A positive sign is a sensation of a jerk or
adducted. A positive sign is a sensation of abnormal snap with reduction into the socket. A click is not
movement, indicating dislocation of the femoral head necessarily heard and a click wihout a sensation of ab-
from the acetabulum. The hands are reversed for ex- normal motion is probably not significant. (Reproduced
amining the other hip. This sign and Ortolani's sign by permission from Specht EE: Am Fam Physician 9:
may be found only in the first few weeks of life. (Re- 88-96, Feb 74)
produced by permission from Specht EE: Am Fam
Physician 9:88-96, Feb 74)
to drain respiratory secretions, is deleterious, at therefore giving an incidence of joint laxity in 50
least in the presence of laxity of the capsule and percent in the offspring of an affected person.
ligamentum teres. Obviously this far exceeds the risk factor in con-
It has long been recognized that females are genital dislocation, but environmental factors
about five times more commonly affected than vary, and necessarily modify the genetic predispo-
males, and a hormonal effect has been inferred. sition in such families.
Some observers14 have in fact reported that estro- Results in other familial studies"')"7 indicate
gen excretion levels are higher in affected infants that defective development of the acetabulum,
than in normals, but others15 have failed to con- with a resulting shallow socket (primary acetabu-
firm this. The belief, however, that female infants lar dysplasia), a concept popularized by Hart,'8
are more sensitive to the hormones of pregnancy may be polygenically inherited, and that those
and that these hormones cause greater ligamen- cases diagnosed after the neonatal period may
tous laxity than in the male, holds some currency. represent the dysplastic rather than the ligamen-
Findings in several studies', have shown that both tous laxity type.'9'20 It is thus possible that two
patients and first degree relatives of patients have genetic systems, one autosomal dominant and the
an unusual degree of joint laxity. This is prob- other polygenic, may interact and in turn be
ably inherited as an autosomal dominant trait, acted upon by environmental factors.
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CONGENITAL DISLOCATION OF THE HIP
late in the game to make a diagnosis, the char- quency of dislocation and subluxation combined
acteristic painless abductor lurch (Trendelenberg was 20 percent in one series of infants who had
gait) toward the affected side, and an external presented for torticollis. The diagnostic inference
rotation attitude of the involved leg may be is clear; all such infants should be carefully ex-
noted. amined, and probably x-ray studies should be
We still find late cases and the question done as well.
has been raised20 if they represent a different
group of "stiff hips" than those which present Roentgenographic Features
early with instability. This would fit nicely with The diagnosis of congenital dislocation is essen-
the thesis of two genetic entities'6 with a common tially a clinical one; however, roentgenograms
end result, but as yet the proof is lacking.20 In can be of considerable help-especially in fol-
any case, it should be reiterated that one normal lowing progress under treatment. Efforts have
examination in the nursery does not relieve the been made6 28,13 to use roentgenograms in the
physician of further diagnostic efforts. newborn period, but opinions vary considerably
A significantly higher incidence of congenital as to their efficacy,",2 and most authorities agree
dislocation and subluxation of the hip in infants that normal findings on x-ray films do not exclude
affected by congenital muscular torticollis has the possibility that a dislocatable hip was x-rayed
been noted by two groups of authors.'2 The fre- while in the reduced position31 and the finding
therefore gave a false sense of security (Figure
5). After the age of 4 to 6 months, at which time
the capital femoral epiphyses ossify, roentgeno-
grams in the anteroposterior and "frog-leg" lateral
positions will satisfactorily delineate the relation-
ships between the femoral head and the acetabu-
i-
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lum. A horizontal line drawn transversely across
II A, 1-.i
I
the pelvis through the centers of both triradiate
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Figure 4.-Asymmetry of the thigh and inguinal Figure 5.-This roentgenogram shows the difficulty in
creases. Bilateral asymmetry of the thigh creases alone evaluating x-ray studies of the hips in newborns. Al-
is an unreliable sign but asymmetry of the inguinal or though the hips appear symmetric and are not obvi-
gluteal creases, especially with apparent shortness of ously displaced superiorly, there is a suggestion of
the thigh, is a good indication of hip dislocation. Note lateral subluxation bilaterally. The acetabulums appear
that the infant's left knee is higher than the right and normal. This infant had positive Barlow and Ortolani
the prominence of the greater trochanter on the left is signs and was treated with a splint because of the
more pronounced and more proximal than on the right. positive clinical findings, in spite of the equivocal x-ray
The left hip is dislocated. (Reproduced by permission findings. (Reproduced by permission from Specht EE:
from Specht EE: Am Fam Physician 9:88-96, Feb 74) Am Fam Physician 9:88-96, Feb 74)
cartilages in the acetabula and a vertical line epiphyses. In a normal hip, the ossific nucleus of
dropped from the lateral margin of the bony roof the femoral head lies below the horizontal line
of the socket to intersect the horizontal line at and medial to the vertical line. As subluxation
right angles is useful in detecting abnormally occurs the ossific nucleus is either bisected by
lateral and superior position of the capital femoral the vertical line or lies lateral to it. Similarly,
proximal migration of the femoral head, which
ultimately comes to rest superior to the horizontal
line, occurs.
Another useful measurement in following prog-
ress in treatment, and in diagnosis in late cases
at least, is the so-called acetabular index34 (Fig-
ures 6 and 7) which might better be called the
roof-slope angle. Lines drawn along the acetabu-
lar roof and extended to intersect the horizontal slope of the acetabular roof, adductor tightness
line will form an acute angle which is easily meas- and actual dislocation of the head) simple abduc-
ured, and which is increased in dysplasia and dis- tion splinting by any of several devices6" 1'24'31'38
location. This angle normally becomes more will almost invariably result in a normal
acute with increasing age. The normal values for hip.6"'1124"28 In fact such treatment is probably best
this angle have been described27 and can be help- considered prophylactic.
ful in diagnosing dysplasia, particularly if there Following the first few weeks of life, however,
is a pronounced discrepancy between the two the difficulty in attaining normal clinical and
sides. With sufficiently early and successful treat- roentgenographic findings is greatly increased38-40
ment the acetabular index declines to normal, hence the considerable efforts expanded on early
whereas the slope increases and assumes a bi- diagnosis and treatment.
labiate appearance if dislocation persists (Figures Although there are exceptions,41 most ortho-
8A and 8B). paedic surgeons42'43 believe that nonoperative
A line can be projected along the medial por- management, as propounded by Putti22 some 40
tion of the femoral neck, the calcar femorale, and years ago, is best until the child begins to walk,
extended medially to intersect the margins of the and perhaps later. Opinions vary on details and a
obturator foramen in the anteroposterior projec- review of the literature suggests that there are no
tion (Figure 6). Normally this medial projection hard data on precisely when conservative man-
will correspond exactly with the superior margin agement ceases to be appropriate. Most would
of the foramen; if there has been proximal dis- agree that up to the age of 6 months or so, abduc-
placement of the head, however, the line will be tion splinting, or the Pavlik harness44 currently
disrupted and will pass more toward the triradi- gaining favor, is indicated until the clinical and
ate cartilage. An additional measurement of roentgenographic findings revert to normal in
value in appraising adequacy of reduction and both dysplasia without frank dislocation and in
coverage of the head by the roof is the center- dislocation. This may be a matter of some months,
edge angle of Wiberg35 in which an angle is con- and a period of immobilization equal to the
structed by a line drawn from the center of the elapsed time between birth and initiation of treat-
head through the lateral margin of the acetabu- ment has been suggested as a guideline to dura-
lum, intersecting at that point-with the vertical tion. Demonstration, by x-ray films taken in the
line described above. This "CE angle" is normally abduction device, that the hip is in fact reduced
more than 20 degrees, and declines to zero as the is essential. Between the ages of 6 and 12 months,
center of the laterally-displaced head comes to gentle manipulation under anesthesia may be re-
lie on the vertical line. quired to attain reduction. This should be fol-
Arthrography36 is used by some surgeons to lowed by a period of immobilization in a cast in
evaluate adequacy of reduction and to delineate the position of maximal stability. In recent years
the condition and position of the acetabular there has been increasing awareness of the danger
labrum (the limbus) and other soft tissues inter- of prolonged immobilization in either the frog-leg
posed between the head and the medial wall of (Lorenz) position of extreme abduction45 or the
the acetabulum, but its usefulness is limited. In internal rotation position of Lange-both of
our hands tomography37 has been useful to ascer- which subject the posterior retinacular vessels,
tain that a concentric reduction, without posterior which supply the capital femoral epiphysis, to ex-
displacement, has been obtained when the patient treme mechanical distortion. It is commonly be-
is in a cast or splint. lieved, and probably correctly so, that these
bizarre positions may contribute to the develop-
Treatment ment of avascular necrosis of the femoral head
(osteochondrosis), and instances have been re-
Treatment of congenital dislocation of the hip ported in which the previously normal hip in uni-
is perhaps more clearly age-related than any other lateral cases developed avascular necrosis during
condition in medicine, a fact that reflects progres- treatment.4'; It should also be noted that this conm-
sion of secondary pathologic changes. In an infant plication is never seen in untreated patients, and
with dislocatable hips due to ligamentous laxity can fairly be said to be iatrogenic. In an effort
(a primary change occurring before the develop- to avoid this catastrophe, a position involving
ment of secondary changes, such as increased somewhat more flexion but considerably less ab-
pending on the tension of the muscles about the otomies,71 in which the proximal femur is tran-
hip as seen at the operating table. One of the sected, the proximal fragment is adducted against
remaining problems in open reduction and in- the pelvis and the distal fragment is abducted
nominate osteotomy is the pernicious tendency relative to the proximal. It is held that the dis-
of the head to redislocate while in the cast, or placed proximal fragment helps to support the
perhaps even while the cast is being applied. This pelvis, minimizing the abductor lurch which oc-
is a consequence of two factors, persistent femoral curs during gait. Early postoperative results have
anteversion and tension on the muscles crossing been described as encouraging, but there are no
the hip joint. Both of these factors can be dealt series of significant size over a period of years,
with at one sitting by subtrochanteric resection and some question must arise as to the effect on
and derotation osteotomy combined with open the knee of such realignments of the proximal
reduction. The concept of derotation osteotomy femur.
in the management of femoral anteversion is not
new, 9'59-61 and while some contend it is unneces- Complications and Prognosis
sary, others are equally convinced it is on occa-
sion critical to obtaining the best result. The The most common complications of treatment
question of future leg-length discrepancy must are failure to obtain concentric reduction, loss of
inevitably arise when bone is resected from one motion from operative intervention, the possi-
leg, and no long-term results are yet available; bility of postoperative infection of the joint and
however, it should be noted that sacrifice of 2 cm avascular necrosis of either the affected or the
or so of bone, the average necessary, will likely normal hip (Figure 9). Premature epiphyseal
eventuate in only a 1/2 cm discrepancy because fusion of the knee has also been described,72
of the well-known tendency of the adjacent Sequellae of failure to obtain reduction, or of no
epiphyses to overgrow following fracture and treatment, are the development of premature de-
other insults. A 1/2 cm, or even a 1 cm disparity generative joint disease.36'3 We have seen symp-
in leg lengths is negligible compared to the long- toms of easy fatigability and discomfort occur
term sequellae of persistent hip dislocation or in adolescence; however, most patients do remark-
repeated operative intrusion and resultant loss of ably well at this age in spite of an unsightly lurch-
motion. ing gait, in which the center of gravity is shifted
In children over the age of 7 or 8 who first toward the affected hip during the stance phase
appear for treatment, and perhaps in some in of gait, thus causing the trunk to sway laterally.
which there has been failure of previous treat- This occurs because the lever arm of the abductor
ment, the advisability of salvage procedures arises. muscles of the hip, which must maintain the
In times gone by the Hey-Groves-Colonna cap- pelvis level, is mechanically deficient. This allows
sular arthroplasty62-64 and various shelf pro- the pelvis to tilt abnormally away from the af-
cedures65,6' were widely used and still enjoy some fected hip when it is bearing weight. The effort to
support,67 but other authors68'69 contend that at compensate for this deficiency and prevent falling
least in the bilateral cases nothing should be done results in the shift of the center of gravity de-
after the age of 7 or so. Another procedure gain- scribed above.
ing advocacy in older children and young adults The usual course of a dislocated hip is one of
with persistent dislocations is the innominate increasing pain and disability during early middle
osteotomy of Chiari, which should be viewed as age, a time when most women need to remain
a salvage procedure when traction and reduction active in the care of their families. During this
do not appear feasible. In this procedure the ilium period many will require reconstructive arthro-
is cut through above the femoral head, which is plasties74,75- a subject in itself and beyond the
then displaced medially under the buttress of the scope of this paper. Thus one can see the crucial
osteotomy. Preliminary results are encouraging, need for early diagnosis and skillful management.
and the provision of bony stock above the femoral With the virtual disappearance of poliomyelitis
head will undoubtedly be helpful should subse- as a crippler of young people, there is probably
quent reconstruction be necessary. no single area in which the primary care physician
Other procedures which should be mentioned can be so effective in the prevention of late
for completeness, but which appear to have fallen crippling. Nowhere else is the proverbial ounce
from favor are the so-called pelvic support oste- of prevention (in this context a high index of
suspicion and a willingness to examine carefully 32. Hummer CD, MacEwen GD: The coexistence of torticollis
and congenital dysplasia of the hip. J Bone Joint Surg 54A:1255-
hundreds of babies in order to identify the one at 1256, 1972
33. Andren L, Von Rosen S: The diagnosis of dislocation of
risk) of greater curative value to the patient. To the hip in newborns and the primary results of immediate treat-
ment. Acta Radiol 49:89-95, 1958
reiterate the words of Putti "La cura preambula- 34. Caffey J, Ames R, Silverman WA, et al: Contradiction of
toria non e piu una vuota aspirazione." congenital dysplasia-Predislocation hypothesis of congenital dis-
location of the hip through study of normal variation in acetabular
angles at successive periods in infancy. Pediatrics 17:632-640, 1956
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31. MacKenzie IG: Congenital dislocation of the hip-The dc- 64. Buehler CE, Coleman SS: Colonna arthroplasty for dislo-
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