Hip Disorders in Children

295 Pages·1978·15.93 MB·English

by  G.C. Lloyd-Roberts

POSTGRADUATE ORTHOPAEDICS SERIES
under the General Editorship of
A. Graham Apley, MB, BS, FRCS
Honorary Director, Department of Orthopaedics, St. Thomas' Hospital, London;
Consultant Orthopaedic Surgeon, The Rowley Bristow Orthopaedic Hospital, Pyrford and
St. Peter's Hospital, Chertsey, Surrey
Hip Disorders
in Children
G.C.LLOYD-ROBERTS
MChir(Cantab), FRCS(England)
Orthopaedic Surgeon, The Hospital for Sick Children,
Great Ormond Street, London
A.H.C. RATLIFF
ChM(Bristol), FRCS(England)
Consultant Orthopaedic Surgeon, United Bristol Hospitals
Butterworths
LONDON-BOSTON
Sydney-Wellington-Durban-Toronto
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First Published 1978
Reprinted 1981
©Butterworths & Co (Publishers) Ltd. 1978
British Library Cataloguing in Publication Data
Lloyd - Roberts, George Charles
Hip disorders in children - (Postgraduate
orthopaedics series).
1. Hip joint - Diseases
2. Children — Diseases
I. Title II. Series III. Ratliff, A H C
617\58 RJ480 77-30339
ISBN 0 407 00132 8
Typeset by Butterworths Litho Preparation Department
Printed in Great Britain by Hartnoll Print Ltd., Bodmin, Cornwall
Editor's Foreword
The traditional tome is ill-suited to dealing with those aspects of orthopaedics
which are controversial and advancing rapidly. However massive the manual, the
space available for detailed appraisal of individual topics can never be enough.
The Postgraduate Orthopaedics Series aims to cover such topics in individual
books — monographs of modest size, published as and when the need becomes
apparent. For each subject one or two authorities are invited to survey the
recent literature and to use it as a backcloth against which to present their
own views, based on personal observations and technical know-how.
In this, the first volume of the series, two writers of distinction and experience
have combined to describe disorders of the growing hip; each has extensive
knowledge in this important field and they have included an up-to-the-minute
account of their practice and results. Their constant collaboration was of course
encouraged and fostered, but each was asked to write his particular sections
single-handed, so that the individual flavour is retained. I enjoyed both and have
learned much from them.
A. G. A.
Acknowledgements
The authors are indebted to the Departments of Medical Illustration of Bristol
Royal Infirmary and the Hospital for Sick Children, London. They also thank
their secretaries Mrs S. McLennan, Mrs C. Calder, Mrs B. Warren and Mrs S. Miers
for their invaluable assistance in the preparation of this work.
Publishers' note
Owing to the age of some of the x-ray originals and the fact that the subjects are
children, the reproduction of certain of the illustrations is unavoidably imperfect.
The position of the right and left hips has been reversed in a few of the illustrations.
However, the 'message' they convey is in no instance ambiguous.
v
1
Congenital Femoral
Deficiency
G. C. Lloyd- Roberts
Femoral dysplasia is a maldevelopment of broad spectrum, including not only
deficiencies of the femur ranging from minor shortening to total absence, but
also modifications in form such as coxa vara, pseudarthrosis and hip dislocation.
Furthermore the dysplasia may not be restricted to the femur alone but be a
reflection of a generalized malformation of the whole limb so that abnormalities
of the leg and foot complicate the problems of management. Lastly, the presen
tation may be bilateral with differing patterns of deformity on the two sides.
In so complex a disorder accurate classification of the numerous variations is
essential, for the prognosis differs between one pattern and another. Denied this
knowledge we cannot plan our management in a rational manner.
Prostheses will be an essential adjuvant in most cases but in this chapter I will
consider only the role of surgery in preventing some predictable complications
which will emerge as growth proceeds and in alleviating their effects later, in
the hope that the problems facing the prosthetist will thereby be reduced.
Aetiology
A precise cause is not recognized and there is no genetic formula, but it seems
that some noxious physical, chemical or infective influence may modify normal
development at a vulnerable stage at some time before the ninth week of uterine
life when the hip joint and femur become fully differentiated. The sequence of
normal development has been well described and illustrated by King (1969).
Femoral dysplasia has been described following thalidomide poisoning
(Fixsen and Lloyd-Roberts, 1974) and as one of the syndromes associated with
maternal diabetes (Kucera, Lenz and Maier, 1965). It is therefore interesting to
note that femoral deficiency has followed insulin injections into the yolk sac of
developing chick embryos on the sixth day (Duraiswami, 1952). Morgan and
Somerville (1960) have proposed an alternative explanation for the more proximal
deficiencies postulating that the orderly progression of vascular invasion is
arrested late in foetal life so that ossification in the cartilaginous model is
delayed and the capacity for longitudinal growth greatly impaired.
1
CONGENITAL FEMORAL DEFICIENCY
Qinical features at birth
These vary with the degree of malformation. Those destined to develop simple
femoral shortening alone will show no abnormality, whereas those in whom the
hip joint and the greater part of the femur are absent will seem to have no femur
and lack stability between the lower leg and pelvis. Most, however, display
marked femoral shortening with flexion, abduction and external rotation defor
mity at the hip which is however stable and mobile from the position of fixed
deformity — Figure 1.1 — findings which may cause surprise when the radiograph
Figure 1.1. The leg is short, flexed, abducted and externally
rotated
is seen. These features are important for they indicate that continuity exists
between femur and pelvis in spite of radiological translucency in the area nor
mally occupied by the proximal femoral shaft {Figure 1.2).
Associated abnormalities when present may be local or general. Tibial shorten
ing with flbular dysplasia is more likely to be seen in company with either
severe femoral deficiency or in association with simple short femur when it
takes precedence and dominates the clinical problem (Hootnick, et al. 1976).
y
It would be surprising if an insult to the embryo during the stage of differen
tiation was localized in its effect and we must therefore be alert to the possibility
of developmental anomalies elsewhere, especially in the arms.
2
CLASSIFICATION OF FEMORAL DYSPLASIA
Difficulty in diagnosis is unlikely when the manifestation is florid but in
minor degrees, i.e. simple short femur and coxa vara, this is possible. A healed
birth fracture will not show evidence of distal femoral epiphyseal delay and the
shortening, if any, will not progress. Idiopathic hemiatrophy will declare itself
Figure 1.2. The proximal femur is translucent but the acet-
abulum is well formed. The femur has not moved proximally
and its tip is bulbous being situated at a greater distance
from the acetabulum than on the normal side
in later childhood and is not identifiable in infancy, but the shortening and loss
of girth characteristic of spinal dysraphism may be confusing as may unexplained
dystrophy of a limb without the stigmata of femoral dysplasia. Congenital coxa
vara must be distinguished from the infantile variety (see below).
CLASSIFICATION OF FEMORAL DYSPLASIA WITH SPECIAL REFERENCE
TO THE PROGNOSIS OF THE HIP
It has already been emphasized that classification is essential to prognosis and
prognosis is the key to management. Important contributions to the differen
tiation of types have been made by Aitken (1969) and Amstutz (1969) with
3
CONGENITAL FEMORAL DEFICIENCY
others in a Symposium devoted to this subject which is probably the most
significant publication on femoral dysplasia at the present time. Simple short
femur and congenital coxa vara have been separately studied by Ring (1959,
1961) and Amstutz and Wilson (1962). The following observations are largely
based upon the contributions of these authors.
Aitken's classification distinguishes four subdivisions of established deformity
of increasing severity with instability (A-D) (Figure 1.3). Amstutz provides
Figure 1.3. Aitken classification. (Reproduced with permission from
Proximal Femoral Focal Deficiency: A Congenital Anomaly. 1969.
Washington: National Academy of Sciences)
this in more detailed form, also arranged in terms of progressively severe dysplasia,
as Types I—V with subdivisions illustrating the initial state and the final outcome
in all, with alternatives in two of the Types described (Figure 1.4). Although
more complex, the Amstutz classification is more apposite to the practical
problems of management especially as applied to the hip and will therefore be
adopted in the following discussion. Congenital short femur (simple hypoplasia)
will be mentioned separately.
I will also incorporate certain observations which we have made with special
reference to the signs by which those destined to develop pelvifemoral instability
may be detected at a very early age (Fixsen and Lloyd-Roberts, 1974).
I intend, however, to make a further modification because this chapter deals
primarily with surgical management of the hip which will, in the early stages,
be concentrated upon the maintenance or restoration of stability with mobility
within this joint. Consequently I will consider the various Types in relation to
their influence upon the integrity of the hip joint, with special reference to the
4
CLASSIFICATION OF FEMORAL DYSPLASIA
Figure 1.4. Amstutz classification. (Reproduced with
permission from Proximal Femoral Focal Deficiency:
A Congenital Anomaly. 1969. Washington: National
Academy of Sciences)
indications for operations designed to preserve or restore this integrity. We may
classify the indications for such operations in relation to the type of dysplasia
as:
(a) Impossible;
(b) Indicated early;
(c) Indicated later;
(d) Unnecessary.
(a) Hip joint reconstruction impossible (Amstutz Type V)
The femur may very rarely be completely absent (Ring, 1961) but usually,
although apparently absent at birth, later ossification will disclose persistence of
CONGENITAL FEMORAL DEFICIENCY
the distal femoral epiphysis and the supracondylar area. The significant feature
is absence of both components of the hip joint, i.e. femoral head and acetabulum.
This can usually be recognized at an early stage for the acetabular concavity is
Figure 1.5. The acetabulum is flat indicating that the
femoral head is absent
non-existent or greatly reduced implying lack of the femoral head. The knee
joint, however is normal. The prospect for establishing a stable and mobile hip
is clearly non-existent (Figures 1.5 and 1.19).
(b) Hip joint reconstruction indicated early (Amstutz Type IV)
The femur is at first represented by opacity limited to the distal third but it will
ossify proximally with time. A concave acetabulum is present and the capital
epiphysis will appear later. The appearance and position of the most proximal
area of ossified femur is of importance. When this is pointed or irregular with
sclerosis of the femur immediately distal to it, disintegration of the junction
between bone and the cartilage model of the hip is imminent (Figure 1.6).
6