OCCULT SPINAL DYSRAPHISM IN ESTABLISHED CONGENITAL DISLOCATION OF THE HIP 745

achieved stability, but within one year the same femoral

heads were found to have rotated laterally and drifted out
of the surgically deepened acetabula. Each child had
further open reductions and pelvic osteotomies in
attempts to stabilise the affected hips. The prolonged
surgical programmes were thought to be the cause of the
spontaneous supracondylar fractures that allthree
sustained after the removal of their plasters. At this
stage, the wasting of the affected buttock and to a lesser
degree the calf (associated with a smaller foot on the
same side), as well as nocturnal enuresis and constipa-
tion, were considered to be secondary to their prolonged
treatment. The outcome was always poor in the affected
hips (Fig. 2).
Occult cases. The other five children who had failed to
respond to treatment had no sacral stigmata of spinal
dysraphism, but developed physical signs similar to
After repeated open reduction, pelvic osteotomy and femoral shorten-
ing, the right congenital dislocation has still not stabilised. those seen in the first group. Radiologically, they had
spina bifida occulta, though of a lesser degree than the
previous group. Two had bilateral dislocations and one
of the three unilateral dislocations was right sided. All
had a normal response to frame reduction and posterior
Table I. Results in 1 17 children with established congenital dislocation
arthrotomy in each case revealed an inverted limbus.
of the hip at five- to 20-year follow-up
Six months later, they were allowed to walk freely,
MOdified but the radiograph taken one year after reduction usually
Severin Number
revealed some drifting of the femoral head out of a
grade of hips Result Per cent
dysplastic acetabulum. Only one child sustained a
I 55
Success 76 spontaneous fracture after pelvic osteotomy, but in all
2 42
cases, when these children stood and walked, the
3 18 Intermediate 13
affected limb rotated laterally (Fig. 3). At first this
4 8
Failure 11
posture could be corrected passively, but later it became
5 7
fixed, and the Trendelenburg test also became positive
S 83#{176}c
were acceptable, I7#{176}
required further surgery on the affected side (Figs 4 and 5). All five children

\ I
I ‘

1”:..

Fig. 3 Fig. 4 Fig. 5

Figure 3 - Lateral rotation of the left leg, persisting after completion of surgical management of CDH . Figures 4 and 5 - The
right hip is stable but the Trendelenburg test is positive on the left.

VOL. 70-B. No. 5. NOVEMBER 1988

746 J. A. WILKINSON, E. M. SEDGWICK

developed the same degree and distribution of muscle right angle and in neutral rotation. This Lorenz position
wasting as the first group, and the foot on the side of has been shown to produce, experimentally, congruity of
dislocation usually failed to grow as much as the opposite the concentrically reduced femoral head within the
foot. Most suffered from nocturnal enuresis. All these acetabulum (Wilkinson 1962).
children had failed to respond to primary surgery in the After the period of splinting, the child is encouraged
same way as those with overt spinal dysraphism. to kick freely and walk, allowing the hips to be extended
and medially rotated actively, bringing the knees into the
sagittal plane. This postural adaptation depends on the
CLINICAL FEATURES
development of normal power in the hip extensors and
Foot inequality. In our total series of 250 cases of medial rotators; any selective weakness of these muscle
established congenital dislocation of the hip, born groups will prevent this natural recovery and the child
between 1965 and 1985, 30% were diagnosed at birth will be left with persistent lateral rotation. This might
and at that time appeared to have normal feet of well be the mechanism of the lateral or outward drift of
equal length. Subsequently, no measurable degree of the femoral head out of the acetabulum at this stage of
inequality developed in the first year of life. treatment in a minority of the patients in our series
At two years ofage, the time ofassessment for pelvic (Fig. 7).
osteotomy, the majority were walking with a normal gait Pelvic osteotomy is usually performed at two years
and their feet remained equal in length. In 7% of them, of age and postoperatively the legs are splinted for eight
however, one foot failed to grow to its full length; this weeks in a plaster spica with the hip in full extension
was usually on the side of dislocation. Measurement of with minimal abduction and medial rotation. After
the feet from heel to the tip of the longest toe proved to be removal of the plaster, it is necessary to carry out both
difficult at this age as the inequality was rarely more than passive and active medial rotation exercises to maintain
0.1 or 0.2cm. a normal gait. If there is any selective weakness of the
Within the next three years, however, the inequality medial rotators, the child will fail to respond to this
of foot length in these patients increased to 0.5 cm or treatment and once again develop lateral rotation of the
more (Fig. 6) and there was greater inequality in those affected leg with recurrence of the lateral drift of the
who had developed unilateral metatarsus varus or cavus femoral head out of the surgically deepened and
deformities of the affected feet. The group of patients anteverted acetabulum (Fig. 8).
with such foot shortening included the children with Thus, a persistent lateral rotation posture of one or
overt and occult spinal dysraphism. occasionally both legs, signifies a persistence of muscle
Lateral rotation posture of the leg. In our treatment of imbalance with lateral rotator dominance ; this is usually
established congenital dislocation of the hip, after associated with a smaller foot on the affected side
posterior arthrotomy and concentric reduction, the (Fig. 9).
children are splinted in Lorenz plasters and then in Denis Stress fractures. Spontaneous or stress fractures are most
Browne abduction harnesses for a total of six months. commonly seen in children with meningomyelocele who
During this time, both legs are held in a position above a have extensive sensory and motor denervation of the legs

Fig. 6 Fig. 7 Fig. 8

The same child as shown in Figs 3 to 5. Figure 6 - Foot inequality. The left foot is 0.5 cm shorter,
and there is slight cavus deformity. Figure 7 -
Radiograph showing left CDH one year after posterior arthrotomy and excision of the limbus. There is lateral drift of the femoral head, spreading
of the “tear drop” and an increased gap between the tear drop and the calcar. Figure 8 - Same case one year after plication of capsule and pelvic
osteotomy, there is lateral rotation of the femoral head with drift out of the acetabulum.

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 OCCULT SPINAL DYSRAPHISM IN ESTABLISHED CONGENITAL DISLOCATION OF THE HIP 747

Strong Weak Somatosensory evoked potentials. Somatosensory evoked

medial rotation medial rotation
potentials can be recorded from the scalp following an
electrical stimulus delivered to the posterior tibial nerve
at the ankle (Fig. 10). The latency of the response
depends on the height of the subject and normal data is
available from our own and other studies (Gilmore et al.
1985 ; Katifi and Sedgwick 1986 ; Zhu, Georgesco and

Inequality
““ :T11:: Selective weakness of
Cadilhac
dysfunction
1987).
of the
Abnormalities
dorsal column
of the
and
response
medial lemniscus
reflect

medial hip rotators sensory pathway but not the spinothalamic system.
Fig. 9 Recordings were made from 96 children, 54 of
whom had CDH alone with no stigmata of spinal
Two physical signs in overt and occult forms of neuromuscular
hypoplasia resulting from spinal dysraphism. dysraphism. The results are summarised in Table II and
a preliminary report has been published (Sedgwick et al.
and the most common sites are the distal and proximal 1988). The difference in incidence of abnormal findings
thirds of the femur. The same distribution of fractures in the various groups is shown.
has been observed in children with overt spinal
dysraphism associated with congenital hip dislocation,
but they do not display the excessive callus formation in DISCUSSION
healing that is seen in the former condition. Such
fractures were rare in our cases of occult spinal Stability ofthe structurally normal hip is dependent upon
dysraphism, only being seen in one of the five patients. the balance between antagonist and protagonist muscle
In our series of 300 infants treated for CDH, only groups acting in three primary planes (Steindler 1973).
one who had no clinical and neurological evidence of The flexor and extensor muscle groups are strong and
spinal dysraphism sustained a similar fracture, and this well balanced, and the same can almost be said for the
resulted from a fall out of her cot. She recovered and abductor and adductor muscles. Yet in the horizontal
responded well to her surgical treatment for the plane, the two opposing rotatory muscle groups are not
dislocation. well balanced, because the lateral rotators (including the
Generally, stress fractures are very uncommon iliopsoas, gluteus maximus and the short rotators) are
complications of the management of CDH, but they three times stronger than the medial rotators (including
have a reputation of ominous significance concerning the gluteus medius, gluteus minimus and tensor fascia
the outcome of treatment. Such a long-term effect is lata).
unlikely to be due to the simple fracture, but may well be Thus before birth, this natural dominance of lateral
caused by an underlying neurogenic defect, such as rotators can produce subluxation and even dislocation of
spinal dysraphism. the flexed hip joint in the presence of hormonal joint

idealised potential recordings

Fig. 10

Ground

Stimulation of rosterior tibial nerves

Somatosensory evoked potential tests, diagram showing levels of stimulation and typical
recordings.

VOL. 70-B, No. 5, NOVEMBER 1988

748 J. A. WILKINSON, E. M. SEDG WICK

laxity (Wilkinson 1985). The ability to rotate the lower

limbs medially is fundamental to the development of
normal function in the hip and knee and is responsible
for the final fetal posture in which the flexed hips are
held in inward rotation. This position accounts for the
normal prenatal development of acetabular and femoral
anteversion (Wilkinson 1985).
Newborn infants are thus capable of rotating their
legs inward beyond the sagittal plane, without any risk of
hip displacement when they first extend their thighs. At
this time, the natural dominance of lateral rotation can
be increased by gravity, if the child lies supine with hips
extended, since this encourages lateral rotation of the
legs and favours the lateral rotator group.
In paralytic infants, it has been suggested previously
that an imbalance between the abductor and adductor
groups is responsible for the spontaneous dislocation of
affected hips, with selective weakness of the abductors in
the presence of strong adductors reinforced by strong
flexors in the presence ofweak extensors (Mustard 1952;
Sharrard 1964). In occult spinal dysraphism, however,
Fig. 11
there appears to be a weakness of the medial rotators,
which usually affects one leg more than the other. Muscle imbalance in spinal dysraphism.
Iliopsoas is the stronger lateral rotator of
The clinical and radiological observations in our the femur, as compared to gluteus medius
patients have reaffirmed Steindler’s original concept, the and minimus which are weaker medial
rotators in the normal child. This differ-
natural muscle imbalance in the horizontal plane being ence is greater when there is selective
weakness of the medial rotators, as in
exaggerated by a selective weakness of the medial
spinal dysraphism.
rotators of the hip (Fig. 1 1), even in the presence of
weakness and wasting of the gluteus maximus. Thus
subluxation leading to redislocation is due to recurrent or
persistent posture of lateral rotation of the leg, which months the foot has grown to half its adult size (from
causes a drift of the femoral head out of the acetabulum 8.0 cm at birth to 12.75 cm) and at four years the typical
with the development of a secondary mechanical value is 16.0 cm.
dysplasia. The only reference to inequality of foot length in
This lateral rotation posture of the affected leg has CDH patients was made by Fairbank (1936), who noted
been found to be closely associated with a failure of the that “in older children the foot on the affected side is
ipsilateral foot to grow to its full extent when compared often smaller than its opposite number”. In our series,
to the opposite limb. Extensive surveys ofthe human foot the majority of patients had equal feet, but in a minority
length have been undertaken at all ages (Meredith 1944). an inequality of foot length was first detected at two
It is interesting to note from these studies that at 18 years and was no more than 0.2 cm. Further inequality

Table II. The incidence of abnormal somatosensory evoked potentials in patients with
congenital dislocation of the hip alone and those who also had spinal dysraphism, unequal
feet or an unsatisfactory response to surgery

With abnormal SEP

Total
Group of patients number Number Per cent

CDH only 54 17 31

CDH and occult spinal dysraphism 34 19 56

CDI-I and overt spinal dysraphism 8 5 63

CDH and unequal feet 52 27 52

CDH not responding to surgery 41 23 56

CDH responding to surgery 47 11 23

* differences in somatosensory evoked potential latency figures were taken as statistically

significant if they exceeded 2 s.d.

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