DISLOCATION OF THE HIP IN TRISOMY 21

Transcription

1 DISLOCATION O THE HIP IN TRISOY 21 GEORGE C. BENNET, ERCER RANG, DAVID P. ROYE, H. APRIN rom the Division oforthopaedic Surgery, The Hospita!for Sick Children, Toronto Almost one child in twenty with trisomy 21 will develop spontaneous of the hip between learning to walk and the age of 10 years. After the age of two years spontaneous habitual may occur. If left untreated, acute, subluxation and fixed follow in sequence. The natural history of the condition is described and the clinical and radiological features of 4 s in 28 patients are presented. Nineteen had received no treatment. The most effective treatment was found to be pelvic or femoral osteotomy, combined with capsular plication, earned out in the phase of habitual. Once subluxation or fixed was present, the results ofoperation were poor and it is not recommended. All patients, even if left untreated, remain mobile. Pain is not a prominent feature. Dislocation of the hip in trisomy 21 is not an everyday problem but its treatment can be a demoralising experience for the surgeon. Trisomy 21 is a common cause of handicap. Otherwise known as Down s syndrome (Down 1866) or mongolism, it occurs with a frequency of 1 in 700 births (Benda 1969). Orthopaedic problems are second only to congenital heart disease as a cause of morbidity in this condition (akley and Sliger, personal communication). Effective surgical treatment for congenital heart disease, coupled with the use of antibiotics, has drastically cut the mortality of these children in the first year of life. As their survival increases so too does the relative importance of their orthopaedic problems which may lead to a child becoming institutionalised because of impaired mobility (Diamond 1976). These problems include of the patella, genu valgum, pes planus, atlanto-axial and scoliosis. The cause is probably ligamentous laxity which is present in 88 per cent of these children at birth (Benda 1969). A direct correlation has been found in trisomy 21 between instability of the cervical spine and the degree of ligamentous laxity (Semine eta!. 1978). In normalnewborn infants instability of the hip has been correlated with joint laxity (Carter and Wilkinson 1964), but we can only conjecture that s ofthe hip in trisomy 21 are similarly related. Kaufmann and Taillard (1961) recorded three cases ofspontaneous ofthe hip in Down s syndrome occurring in children over the age of five years. The incidence ofthe problem was examined by Rang in Diamond (1976) noted that the treatment was difficult. Packer, Lefkowitz and Ryder (1972) described successful treatment in one case. In this paper we will review the incidence, natural history and response to treatment of spontaneous of the hip in trisomy 21. ATERIAL AND ETHODS All patients with Down s syndrome in two long-term care homes were examined. These 220 children and adults, aged S to 62 years (average 26 years 6 months), underwent an examination of gait and were tested for range of movement at the hip ; stability was tested by telescoping of the hip and attempting to produce an Ortolani sign. If any abnormality was suspected, radiographs were obtained. We looked for instability of the patella, signs of an upper motor neuron lesion suggesting C 1-2 subluxation, and we assessed the degree ofligamentous laxity using the criteria ofcarter and Wilkinson (1964). embers of the Paediatric Orthopaedic Society were circularised and provided details of patients with Down s syndrome with s of the hip whom they had treated. inally, those patients seen and treated for this condition at the Hospital for Sick Children, Toronto, were reviewed. Altogether 28 patients with 4 unstable or dislocated were studied (five right-sided, six left-sided and 1 7 bilateral). Their ages ranged from 2 to 36 years, with an average of I 8 years 3 months. Thirteen were resident in institutions. Twenty-six had been treated and 19 left untreated (Tables I and II); four patients with bilateral s had had only one side treated. Other stigmata of ligamentous laxity were present in seven of these patients : five had dislocating patellae, one had a congenital G. C. Bennet, RCS, Consultant Orthopaedic Surgeon Royal Hospital for Sick Children, Yorkhill, Glasgow G3 8J, Scotland.. Rang, RCS(C), Orthopaedic Surgeon The Hospital for Sick Children, University Avenue, Toronto, Ontario SG lx8, Canada. D. P. Roye, Jr. D, Paediatric Orthopaedic Surgeon The New York Orthopaedic Hospital, at the Columbia Presbyterian edical Center, New York 10032, USA. H. Aprin, D, Orthopaedic Surgeon Long Island Jewish Hillside edical Center, New Hyde Park, New York I 1042, USA. Requests for reprints should be sent to r G. C. Bennet British Editorial Society of Bone and Joint Surgery 030l-620X/82/ $2.00 VOL. 64-B, No

2 290 G. C. BENNET,. RANG, D. P. ROYE, H. APRIN Table I. Present state of those that were not treated Case Sex Present age (years) Side State of hip Acetabular angle (degrees) I 2 Habitual Habitual Habitual /subluxation I 2 Dislocatable 9 16 Dislocatable I 7 ixed s Subluxating Subluxating Subluxating Subluxating I I 24 Subluxating ixed ixed s ixed?? of the hip and one was tetraplegic secondary to Cl-2 subluxation. Treatment of the hip aimed at restoring congruity of the joint was judged accordingly and salvage procedures were judged by relief of pain and improvement in mobility. RESULTS Incidence. We examined 220 institutionalised patients, all of whom were able to walk; 114 were male and 106 female. Ten unstable or dislocated were found, an incidence of 4. per cent. Dislocatable patellae were found in 12 (. per cent). No signs of upper motor neuron lesions were elicited. Ligamentous laxity was common in the young, but less frequent with increasing age (ig. 1).. After the age of seven or eight years the child presents with a suddenly increased limp or with decreased activity. Pain is not a prominent feature. The hip is clinically dislocated but can easily be reduced under anaesthesia. Subluxation phase. Concentric reduction becomes rarer and progressive acetabular dysplasia develops (ig. 7). ixed phase. If untreated, the hip invariably develops a fixed by the time the patient is in his late teens or early twenties. All patients can walk without aids, though they have an obvious limp. ew have pain and none require analgesics. Radiographs show a fixed iliac with acetabular dysplasia and a false acetabulum (ig. 8). Natural history Initial phase. The are stable but hypermobile until the age of two years. Walking is usually delayed until about this time. The radiological appearance ofthe pelvis is characteristic (Kaufmann and Taillard 1961): the iliac crests appear broad, possibly as a result of recumbency during the prolonged pre-walking phase, and the acetabulum is well formed and deeper than that of a normal child. Only rarely does a child with Down s syndrome present with congenital of the hip and this generally responds to the usual treatment methods. Dislocation phase. Habitual. Between the ages of 2 and 10 the hip spontaneously begins to dislocate in a particular position without trauma and then to reduce itself. Children may be brought for treatment at this stage because of clicking, an increasing limp or a complaint of giving way (igs 2 to 6). ig. AGE (years) Joint laxity, judged by the criteria of Carter and Wilkinson ( I 964). The numbers in brackets refer to the number of patients in each group. I THE JOURNAL O BONE AND JOINT SURGERY

3 DISLOCATION O THE HIP IN TRISOY Case 1. Arthrograms of a three-year-old boy with habitual of the right hip. ig. Iig. 6 Case I. A two-year-old girl with Down s syndrome. igure ---Weight-bearing anteroposterior radiograph of the. igure 6--The patient is sitting demonstrating bilateral posterior s (the radiograph was taken with the beam aimed from behind the patient at an angle of 40 degrees from the horizontal). Case 7. A girl with trisomy 21 presented at the age of 14 years with an ig. 8 odd gait and pain in the right hip. Case I 3. Untreated bilateral s in a 29-year-old man. Treatment Treatment had been undertaken for 26 in 1 8 patients (Table II). A large variety of procedures had been performed and no conclusions can be drawn about the reasons for selecting a particular procedure in each case. Non-operative treatment. ive underwent closed reduction and immobilisation in a cast. Two of these had persistent subluxation, one two months and the other seven years after treatment. One hip redislocated after three months in a spica and subsequently had an innominate osteotomy. Another had three separate episodes of treated by hip spicas ; three years after the last episode this patient had a normal radiograph. In one case, the hip was clinically stable after one year but radiographs were not available. Operative treatment. In two capsular plication was VOL. 64-B, No. 3, 1982

4 C. BENNET,. RANG, D. P. ROYE, H. APRIN performed. In one case this was done as the primary procedure ; the hip remained stable and was normal radiographically seven years after operation. In the other, plication was performed after re in a hip previously treated by femoral varus derotation osteotomy ; this hip remained stable two years later. In a further two, capsular plication was performed at the time of open reduction. One redislocated and the other subluxated ; both were subsequently treated by femoral osteotomies. emoral osteotomy with plication ofthe capsule was performed on three. One patient had bilateral treatment and both remained stable three years after operation ; in the other patient the hip was stable five years later. An innominate osteotomy was combined with open reduction and capsular plication in four. In three of these osteotomy was of the Salter type : all had excellent clinical and radiographic results, one at six years and the others at nine years after operation (igs 9 and 10). One of the osteotomies was of the Sutherland Table II. Treated Age at presentadon (years) Side Presentation Treatment ollow-up Result 1 2 derotation femoral osteotomies Plication of right capsule- INECTED redislocated latercapsular plication years 2 years Both now stable 16 femoral derotation osteotomies, no plication 4 years Both stable 17 femoral derotation osteotomies and plication Both stable 18 innominate osteotomies and plication of capsule 9 years Stable, normal radiographs years later-chronic s and pain None initially Schantz osteotomies (12 years) No pain. Poor mobility because of dislocating patella 20 7 alling over on walking Capsular plication 7 years Stable hip, normal radiographs 21 7 while in long leg cast Spica 3 months-redislocated Innominate osteotomy and capsular plication- INECTED 6 years Stable hip, normal radiograph 22 8 Closed reduction and spica Redislocated 2 and later-same treatment Now stable Radiographs normal 9 Abnormal gait anipulation and spica 7 years Dislocatable, subluxated anipulation and spica 2 months Subluxated anipulation and spica 9 months No further s 2 12 Chiari and shelf arthroplasty- INECTED 9 months Redislocated Increasing limp open reductions femoral derotation osteotomies 6 years stable dislocated derotation osteotomies Both redislocated Pain Chiari aged 14 Chiariaged 17 6 years ly dislocated 7 14 Pain and odd gait Varus femoral and double innominate osteotomy 4 years Degenerative joint disease and subluxation (Total hip replacement) II 18 Pain Chiari- INECTED Subluxation and degenerative joint disease Pain femoral osteotomy- INECTED 2 years Dislocated THE JOURNAL O BONE AND JOINT SURGERY

5 DISLOCATION O THE HIP IN TRISOY ig. 9 ig. 10 Case 18. Radiographs taken nine years after bilateral innominate osteotomies and capsular plication at the age offive for habitual of the. i:y ig. I 1 ig. I 2 ig. I 3 Case 1 1. A girl with trisomy 21 who presented at the age of 18 with pain in the right hip. igure 1 I-ilm before operation. igure 12- Immediately after operation. igure 13-Three years later, showing marked degenerative disease. The left hip was not treated. type : two years after operation progressive subluxation and degenerative joint disease were present, and a total hip replacement was performed, giving a hip which was stable and functioning well one year later. Osteotomies without capsular plication were carried out on all the remaining. A Chiari osteotomy was carried out on four : three redislocated and one showed subluxation with degenerative joint disease. The follow-up ranged from nine months to six years (igs 11 to 1 3). Schantz osteotomies were performed bilaterally in one patient, the indication being painful fixed. Three years later neither was painful and he was mobile (ig. 14). emoral osteotomies were performed in nine, one in conjunction with a double pelvic osteotomy; five redislocated at periods of up to three years after operation. In the operated group five wound infections were known to have occurred. This gives a minimum known infection rate of 19 per cent. DISCUSSION ig. 14 Case I 9. This boy presented at the age of six because of failure to walk. ly dislocatable were noted. Subsequently he developed painful, fixed s. At the age of 1 bilateral Schantz osteotomies were performed. This follow-up radiograph was taken three years later. We have found that, in trisomy 21, about one child in 20 will spontaneously develop a dislocatable or dislocated hip between learning to walk and the age of 10 years. We acknowledge that this figure was obtained from the examination of institutionalised patients and it may be that the incidence is lower in patients in the community. Ifleft untreated, such instability may lead to subluxation and and produce a degree of disability comparable to untreated congenital of the hip. Presumably some stabilise in the phase of habitual but we have no way of knowing this VOL. 64-B, No

6 294 G. C. BENNET,. RANG, D. P. ROYE, H. APRIN at present (ig. 1). It is interesting to observe that habitual takes place in the age group of greatestligamentous laxity whereas the acute s occur subsequently when laxity is disappearing. Treatment has been successful in only half of the cases and infection has been a problem. This may lead some to suggest that s in mongols are best left alone. Successfully treated patients, however, are much better than those with an ignored. Our experience leads us to suggest that all children with trisomy 21 should be tested regularly during childhood for instability of the hip; that habitual requires operative treatment in the absence ofany contraindication ; and that an acute is probably best treated operatively, although closed reduction and immobilisation in a hip spica is occasionally successful. If the hip is still unstable after six weeks in a cast then an operative procedure should be undertaken. We consider that fixed and subluxation should be left alone. The results of an operation at this stage are poor. As the life expectancy of these children is less than normal and by the nature of their condition they do not need to be as mobile as the general population, symptoms ofdegenerativejoint disease are not so important, nor for that matter is the limitation oflife style that they impose. Bony operations alone seldom succeed. Capsular repair combined with femoral or pelvic osteotomy is usually successful. Hence we would recommend either of these as the treatment of choice. The best results can be 210 yrs 2 loyrs IGTh >2 ye ye STABLE 1 DISLOCATABLE HIP I HABITUAL DISLOCATION.NORAL ig. 1. ACUTE DISLOCATION I Osseotomy PI,c.t,on NORAL The natural history of of the hip in trisomy 21. G2 yrs 7 12 yrs expected ifthis is done in the phase ofhabitual before acetabular dysplasia develops. When fixed is present a Schantz osteotomy may be helpful for the treatment of pain and gross deformity. We would like to thank all the members of the Paediatric Orthopaedic Society for their response to our questionnaire and the following surgeons who supplied the details of cases included in the study:. Bell, W. P. Bobechko, R. L. Cristofaro, J. Gallagher,. Letts,. enelaus, R. orrissy, R. B. Salter, L. Staheli, D. H. Sutherland, H. Watts and J. Wiley. We should also like to thank Dr Elsie Crawford and Dr Tom Lennox and the staff of the Huronia Regional Centre, and Dr J. Stewart of Rideau Hospital School at Smith s alls, for providing the facilities for examining the children. igures and 6 are reproduced by kind permission of Dr R. L. Cristofaro. This work was supported by a grant from the Conn Smythe oundation. REERENCES Benda CE. Down s syndrome. New York : Grune and Stratton, Carter C, WilkinsOn J. Persistent joint laxity and congenital of the hip. J Bone Joint Surg [Br] 1964;46-B: 40-. Diamond ES. anagement of inherited disorders of the skeleton AAOS instructional course lectures 1976 ; 2 : Down JLH. Observations on an ethnic classification of idiots. London Hosp Clin Lect Rep 1866;3 : Kaufmann HJ, Tafflard W. Pelvic abnormalities in mongols. Br edj 1961 ;i: Packer JW, Lefkowitz LA, Ryder CT. Habitual of the hip treated by innominate osteotomy : a report of three cases. Clin Orthop 1972;83: Rang. Dislocation of the hip in Down s syndrome. J Bone Joint Surg [Br] 1972; 4-B : 770. Semine AA, Ertel AN, Goldberg J, Bull J. Cervical spine instability in children with Down syndrome (Trisomy 21). J Bone Joint Surg [Am] 1978 ;60-A : THE JOURNAL O BONE AND JOINT SURGERY