Congenital Dislocation or Developmental Dysplasia of the newborn hip?

Thomas R M, Urquhart D S, Wefers B.

Neonatal Intensive Care Unit, Northwick Park Hospital, Watford Rd, Harrow, Middlesex HA1 3UJ

SMJ 2006 51(1): 57

 

Abstract

The role of universal primary ultrasound screening for detection of developmental dysplasia of the hip of the newborn remains controversial in Britain. Two infants in whom ultrasonography was reported to show no evidence of developmental dysplasia in the newborn period, but who later presented with acetabular dysplasia and subluxation requiring surgery, are presented.

 

Case Reports

Case 1

A dislocatable left hip was detected on routine neonatal examination at 24 hours of age. A consultant orthopaedic surgeon confirmed that the hip was Barlow test positive on day 3 of life, but an ultrasound scan was reported to show no evidence of hip dysplasia. A paediatric radiologist experienced in the scanning of newborn hips performed the ultrasound scan, and a second experienced paediatric radiologist reviewed the scans. At 4 months of age, both hips were deemed to be clinically stable by the same orthopaedic surgeon, but left acetabular dysplasia with subluxation was diagnosed on dynamic ultrasonography. Adductor psoas tenotomy and closed reduction of the left hip was undertaken at 8 months of age. At 22 months, the child was walking and the radiograph showed that the acetabulum was developing normally.

 

Case 2

A dislocatable left hip was detected on routine neonatal examination at 28 hours of age. Both hips appeared normal on static sonographic assessment, performed by an experienced paediatric radiologist at 12 days of age, and reviewed by a second radiologist. The infant was lost to follow up and re-presented at 8 months of age when the mother observed reduced movement of the right leg. Dislocation of the right hip was confirmed radiologically. Adductor psoas tenotomy and open reduction of the right hip was undertaken at 11 months of age. At 20 months of age the child was walking, but there was some residual dysplasia of the right hip on radiograph.

 

Discussion

The success of routine clinical screening in reducing, but not abolishing late presentation of developmental dysplasia of the hip is well documented (1).  A Medical Research Council working party review of primary screening in the United Kingdom found that surgery was required for late diagnosed congenital dislocation of the hip in 0.78 per 1000 live births, which represented 70% children reported to the national surveillance programme (2). Other studies evaluating the role of clinical screening have shown low rates of late diagnosed congenital dislocation of the hip (CDH) requiring surgery and support continuation of clinical screening (3, 4). An Australian population-based study of clinical screening has shown a late detection rate of only 2 - 4 % (0.19 per 1000 live births) requiring surgery, and attribute their success to the quality of the training of the testers and the organisation of the screening programme (4).  Some European countries, notably Germany and Austria, have adopted primary neonatal ultrasound screening of the hip following the publications of Graf (5). The high treatment rates for hip dysplasia detected on routine sonographic screening in Europe have concerned orthopaedic surgeons in Britain and North America. Dynamic tests of hip instability increase the usefulness of ultrasound examination (6,7). Engesaeter et al found that even in a group of 100 infants at high risk of hip instability, only dynamic assessment of stability, but none of the standard sonographic measurements of acetabular depth and femoral head cover, correlated with outcome at 3 months of age (8).

 

There has been no published population-based study to allow epidemiological assessment including the incidence of late-presenting CDH, after apparently normal neonatal ultrasound screening.  Advocates of hospital-based primary neonatal ultrasound screening of the hip in the United Kingdom have demonstrated that late-presenting congenital dislocation of the hip can be virtually eliminated, but such a screening programme requires a highly dedicated team making extraordinary efforts to ensure that all neonates are screened and followed up (9). Recent publications from a Medical Research Council Working Party reporting on research initiated by the Department of Health to assess the current screening programme in the United Kingdom and the potential role of ultrasound screening, have explored various screening options focussing on health outcomes and cost (10, 11). The authors have concluded that it is not feasible to recommend a single national approach to this complicated medical problem, particularly when local resources are so variable.

 

Clinical guidelines and studies which aim to improve the outcome of this important and disabling childhood condition by developing a more scientific basis for primary screening should continue to include both clinical and sonographic evaluation. Our cases serve to remind clinicians that long term follow up of all infants with clinical signs of hip instability in the newborn period is mandatory, even if the initial ultrasound scan is considered to be normal.

 

References

1.       Macnicol MF. Results of a 25 year screening programme for neonatal hip instability. J Bone Joint Surg (Br) 1990; 72-B: 1057-1060.   

2.       Godward S, Dezateux C.  Surgery for congenital dislocation of the hip as a measure of outcome of screening. Lancet 1998; 351: 1149-1152.

3.       Sanfridsen J, Redlund-Johnell I, Uden A. Why is congenital dislocation of the hip still missed? Acta Orthop Scand 1991; 62: 87-91

4.       Chan A, Cundy PJ, Foster BK, Keane RJ, Byron-Scott R. Late diagnosis of congenital dislocation of the hip and presence of a screening programme: South Australian population-based study. Lancet 1999; 354: 1514-1516.

5.       Graf  R. Classification of hip joint dysplasia by means of sonography. Arch Orthop and Trauma Surg 1984; 102: 248 - 255.

6.       Clarke NMP, Harcke HT, McHugh P et al. Real-time ultrasound in the diagnosis of congenital dislocation and dysplasia of the hip. J Bone Joint Surg (Br) 1985; 67-B:406-412.

7.        Harcke HT, Grissom LE. Performing dynamic sonography of the infant hip.

AJR 1990; 155: 837-844. 

8.       Engesaeter LB, Wilson DJ, Nag D, Benson MK. Ultrasound and congenital dislocation of the hip. The importance of dynamic assessment. J Bone Joint Surg (Br) 1990; 72: 197-201

9.       Marks DS, Clegg J, Al-Chalabi AN. Routine Ultrasound for neonatal hip instability: Can it abolish late presenting congenital dislocation of the hip? J Bone Joint Surg (Br) 1994; 76-B: 534-538.

10.   Dezateux C, Brown J, Arthur R, Karnon J, Parnaby A. Performance, treatment pathways, and effects of alternative policy options for screening for developmental dysplasia of the hip in the United Kingdom. Arch Dis Child 2003;88:753-759.

11.   Brown J, Dezateux C, Karnon J, Parnaby A, Arthur A.. Efficiency of alternative policy options for screening for developmental dysplasia of the hip in the United Kingdom. Arch Dis Child 2003;88:760-766.

 

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