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CASE REPORT

A Rare Combination of Legg-Calve Perthes’ Disease and Slipped Capital Femoral Epiphysis in the Same Child: A Case Report and Literature Review

Annapureddy S R, Parkar A A H, Williamson D M
Colchester University Hospital
Colchester,  Essex

Address for Correspondence:
Asif A. H. Parkar
Flat 2 Lisle House, Charter Way
Colchester
,  CO4 5JL, U.K.
Phone:  
+44-7888662717
E-mail:  drasifparkar@rediffmail.com

Abstract:

Legg-Calvé Perthes’ disease and Slipped Capital Femoral Epiphysis (SCFE) in the same child occur very rarely. Only eight such cases have been reported in the literature. We report a case of SCFE occurring in a patient with Perthes’ disease who also had proximal femoral osteotomy for the correction of rotational mal-alignment.

Also, going through the literature it seems likely that slippage of proximal femoral epiphysis after previous Perthes’ disease in the contra lateral hip is most likely a coincidental occurrence.  We believe this case highlights a rare but important clinical scenario which can have serious consequences for the patient if not identified and treated appropriately.

J.Orthopaedics 2009;6(3)e9

Keywords:
Legg-Calvé Perthes; Disease, Slipped Capital Femoral Epiphysis; Osteotomy; Retroversion; Rotational mal-alignment.

 

Introduction:

Legg-Calvé Perthes’ disease is the name given to idiopathic osteonecrosis of the capital femoral epiphysis of the femoral head. It is caused by an interruption to the blood supply of the head of the femur and is typically found in young children aged between three to 12 years. They present with hip, knee or groin pain exacerbated by movements which can easily be dismissed as growing pains. Its treatment varies between careful monitoring to surgical intervention when necessary.

Slipped Capital Femoral Epiphysis (SCFE) is another condition where there is posterior and inferior slippage of the proximal femoral epiphysis on the metaphysic which occurs during the adolescent growth spurt and is most frequent in obese children. They too present with hip, knee or groin pain exacerbated by movements. Its management involves either pinning in situ or corrective osteotomy.

Although Perthes’ disease and SCFE are completely different entities they both can present with similar symptoms in a relatively similar age group. Diagnosing these diseases is often enigmatic and unfortunately in many instances it is initially missed. It is therefore important to have a clear understanding of their manifestations and management as delay in their treatment results in a less favourable long term prognosis.

Both of these are relatively unusual conditions in Children. The reported incidence in Caucasians is about one in 6500 for Perthes’ disease and only about one in 10000 for SCFE [1,2]. We report a case where both these conditions were diagnosed and treated effectively in the same child.

Case Report:

A six years old Caucasian male child presented with spontaneous onset of a painful right hip of a few days duration. Radiographs revealed changes consistent with Perthes’ disease with whole head involvement but without any head at risk signs (Fig-1). He was treated with analgesia and short periods of bed rest. Subsequent follow up radiographs showed re ossification of epiphysis.

Approximately two years later at the age of eight he was seen with an abnormal gait. On examination, he had an external rotation of 90° in extension at both hips with no internal rotation possible. He also had increased external torsion in both tibias. The child was mildly obese but did not have any clinical evidence of neuromuscular disease or hyper laxity of joints.  There was no evidence of any other coexisting abnormality, which could explain the rotational deformity of both hips. He underwent bilateral femoral rotational osteotomies with 45° of internal rotation at the level of lesser trochanter (Fig-2). He was allowed full weight bearing after radiological evidence of callus formation.

At the age of 10, he presented with a limp and painful left hip. On examination, he had flexion 100° abduction 35° internal rotation 20° and external rotation 35°. Radiographs suggested mild slipping of proximal femoral epiphysis (Fig-3). Bone scan showed increased uptake in the left hip. He underwent in situ pinning with one 6.5mm AO screw after the removal of blade plate (Fig-4). On subsequent follow up there was no evidence of further slipping or avascular necrosis of the femoral head. 

Fig1: Initial anteroposterior  radiograph of the pelvis showing the evidence of Perthes’ disease of right hip.

Fig2:Anteroposterior  radiograph of the pelvis after the corrective osteotomy of the proximal femur showing re-ossification of the right femoral head.

Fig3:Frog leg lateral radiograph of the pelvis showing mild slip of the left proximal femoral physis.

Fig4:Anteroposterior radiograph of the left hip after insitu pinning of the left proximal femoral epiphysis.

Discussion :

Slipped Capital Femoral Epiphysis and Legg-Calvé Perthes’ disease in the same child occur very rarely. Only eight such cases have been reported in the literature. Individual cases were reported by Crawford and Markheim [3,4]. Graziano et al in 1987 reported a series of three cases [5]. Sutro in 1935 in his series on the SCFE reported a patient whose radiograph showed previous contra lateral Perthes’ disease [6].  Crawford calculated the incidence of both occurring in the same child as 0.71-3.41/12000000. In all except one of the described cases in the literature SCFE occurred in the contra lateral hip of Perthes’ disease [3]. In 1992 Ruoff et al described one case of SCFE which occurred in the ipsilateral hip after cheilectomy for Perthes’ disease [7].

In all these cases no common risk factors were found. Also the treatment options for Perthes’ disease  were diverse and could not be attributed as etiological factors for subsequent SCFE (Table-1). Catterall et al in 1982 described the histological changes in the physis of hip affected by Perthes’ disease. They included a thinner physeal plate with irregular columns of cartilage cells and tongues of unossified cartilage extending into the metaphyseal region [8]. If these changes were to weaken the physis and contribute to slipping of proximal femoral epiphysis, the incidence of SCFE following Perthes’ disease would be expected to be much higher both in clinical practice as well as in the literature reported so far.

NUMBER

AUTHOR

AGE AT LCPD

MANAGEMENT OF LCPD

AGE AT SFCE

MANAGEMENT OF SFCE

1

Markhiem 1949

11

Skin traction

Ischial Calliper

Shoe Rise

13

Skin Traction

2

Crawford 1975

5

Split Russell Traction

Patten bottom brace

6

Split Russell Traction

3

Graziano 1987

9

Traction

Chielectomy

14

In situ Knowles pin fixation

4

Graziano 1987

4

Perthes sling

12

Closed Reduction

Knowles pin fixation

5

Graziano 1987

9

Contralateral hip showed old LCPD

9

Insitu Knowles pin fixation

6

Ruoff 1992

14

Chielectomy & greater trochanteric advancement

16

Insitu percutaneous pin fixation

7

Ruoff 1992

13

Toronto brace

Chielectomy

14

Percutaneous pin fixation

Table 1: Data On Patients With Perthes’ Disease And Slipped Capital Femoral Epiphysis

After three dimensional tomography in 22 hips with Perthes’ disease and epiphyseal dysplasia Kim et al in 1997 proposed functional retroversion and functional coxa vara [9]. In Perthes’ disease deformed femoral head consists of two portions, an antero-infero-lateral false head blocking the internal rotation and a postero-medial-superior portion representing the true articulating femoral head. The true head is retroverted in relation to the antero-lateral segment resulting in functional retroversion which causes an externally rotated gait [10]. Functional retroversion and functional coxa vara explain the paradox of supposed increased antiversion on radiograph in a child who walks with the externally rotated limb.

Retroversion of the proximal femoral epiphysis was supposed to predispose to SCFE by altering the biomechanics across the proximal femur. However if the functional retroversion in healed Perthes’ disease is a contributing factor to SCFE their coexistence should be a lot higher.

To determine the rotational profile of the lower limb a CT scan was performed and it showed a 25º retroverted femoral head. A 45º de rotation osteotomy corrected the rotational profile of the proximal femur from 25º retroversion to 25º anteversion.

Several ultra structural alterations of the physis were documented in both Perthes’ disease and SCFE [10]. These include reduction in the number of chondrocytes, chondrocyte degeneration and haphazardly arranged collagen. The significance of these changes in the physis is unknown and they may have a causative role. Obesity predisposes to SCFE by two different mechanisms, increased stresses across the physis and decreased anteversion of femur.

In our patient, we used one 6.5 mm cannulated screw as that is the standard practice and we did not have any further complications. However, there have been concerns expressed in the past about using this technique in this age group due to growth disturbance of the proximal femur and premature physeal closure which has led to the consideration of alternative methods.

Segal et al in 1991 presented a series of 21 patients (33 hips) where pinning  for SCFE resulted into growth disturbances including greater trochanteric overgrowth, coxa vara, and coxa breva in 64% of the hips [11]. In 1988 Huggland et al presented six boys with SCFE who were successfully treated using a hook pin. The advantages of this technique are that it causes minimal trauma, cannot slide back therefore does not loose epiphyseal grip resulting into normal hip growth [12]. Guzzanti et al in 2004 presented 10 skeletally immature patients with SCFE who were treated by using a modified cannulated screw. The threaded portion of the screw was placed entirely within the epiphysis and the screw head was allowed to remain two to three cm lateral to the lateral femoral cortex. The screw used by them allowed continued growth of the femoral neck, epiphyseal/physeal complex and remodelling of the epiphysis and metaphysic [13]. 

Altered biomechanics following the de-roatation osteotomy and obesity are two possible mechanisms which might have a contributory role in the SCFE in our patient.

We agree with the other authors who have reported similar cases that there is no common aetiological factor for the causation of the Perthes’ disease and SCFE in the same child. It seems likely that slippage of proximal femoral epiphysis after previous Perthes’ disease in the contra lateral hip is most likely a coincidental occurrence.

Reference :

  1. Pillai A, Atiya S, Costigan PS (2005) The Incidence of Parthes’ Disease in Southwest     Scotland. J Bone Joint Surgery.Br. 87-B(11):1531-1535.

  2. Murray AW, Wilson NIL (2008) Changing Incidence of Slipped Capital Femoral Epiphysis. J Bone Joint Surgery.Br. 90-B(1):92-94.

  3. Crawford A H (1975) Legg-Calve-Perthes’ Disease Coexistent with Slipped Capital Femoral Epiphysis. J Bone Joint Surgery.Am. 57-A(2):280-281.

  4. Markheim HH (1949) Legg–Perthes’ Disease and Slipped Epiphysis in the Same Patient: A Case Report.  J Bone Joint Surgery. Am. 31-A(3):666-668.

  5. Graziano GP, Kernek CB, Derosa GP (1987) Coexistant Legg-Calve-Perthes’ Disease and Slipped Femoral Epiphysis in the Same Child. J Pediatr Orthop. 7(1):61-62.

  6. Sutro CJ (1935) Slipping of the Capital Epiphysis of the Femur in Adolescence. Arch Surg  31(3):345-360.

  7. Ruoff MJ, Schwentker EP (1992) Legg-Calve-Perthes’ Disease and Slipping of the Capital Femoral Epiphysis in the Same Child. Orthopaedics. 15(9):1070-1072.

  8. Catterall.A, Pringle J, Byers PD, Fulford GE, Kemp HB (1982) Is the Epiphyseal Infarction Complete?  J Bone Joint Surgery. Br. 64(3):276-281.

  9. Kim HT, Wegner DR (1997)  Functional Retroversion of the Femoral head in Legg-Calve-Perthes’ Disease and Epiphyseal Dysplasia: Analysis of head –neck deformity and its effects on limb position using three dimensional computed tomography. J of Pediatr  Orthop. 17(2):240-246.

  10. Aronsson DD, Loder RT, Breur GJ, Weinstein SL (2006) Slipped Capital Femoral Epiphysis: Current Concepts. J Am Acad Orthop Surg. 14(12):666-679.

  11. Segal LS, Davidson RS, Robertson WW, Drummond DS (1991) Growth Disturbances of the Proximal Femur after  Pinning of Juvenile Slipped Capital Femoral Epiphysis. J Pediatr Orthop. 11(5):631-637.

  12. Huggland G, Bylander B, Hansson L I, Selvik G (1988) Bone Growth After Fixing Slipped Femoral Epiphysis : Brief Report .  J Bone Joint Surgery.Br. 70-B(5):845-846  

  13. Guzzanti V, Falciglia F, Stanitski CL (2004) Slipped Capital Femoral Epiphysis in Skeletally Immature Patients. J Bone Joint Surgery.Br. 86-B(5):731-736

 

This is a peer reviewed paper 

Please cite as: Asif A. H. Parkar: A Rare Combination of Legg-Calve Perthes’ Disease and Slipped Capital Femoral Epiphysis in the Same Child: A Case Report and Literature Review.

J.Orthopaedics 2009;6(3)e9

URL: http://www.jortho.org/2009/6/3/e9

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