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CASE REPORT
Year : 2014  |  Volume : 2  |  Issue : 2  |  Page : 31-32

Bilateral ankylosed hip joint in a sickle cell disease patient: A case report


1 Assistant Professor, Department of Orthopaedics, SBKSMIRC, Sumandeep Vidyapeeth, Piparia, Waghodia, Dist, Vadodara - 391760, India
2 Resident, Department of Orthopaedics, SBKSMIRC, Sumandeep Vidyapeeth, Piparia, Waghodia, Dist, Vadodara - 391760, India
3 Professor & HOD, Department of Orthopaedics, SBKSMIRC, Sumandeep Vidyapeeth, Piparia, Waghodia, Dist, Vadodara - 391760, India

Date of Web Publication24-Aug-2018

Correspondence Address:
C S Kapoor
Resident, Department of Orthopaedics, SBKSMIRC, Sumandeep Vidyapeeth, Piparia, Waghodia, Dist, Vadodara - 391760
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2347-6486.239546

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  Abstract 


Sickle cell disease affects 10% of tribal population of India. These patients have multiple orthopaedic complications like AVN (avascular necrosis) of femoral and humeral head, osteomyelitis, etc. One such patient having an atypical bilateral hip ankylosis has been reported.
A 17 year female, known case of sickle cell disease, came to our hospital with complains of pain in bilateral hip since five years, both hips showed ankylosis and both knees showed flexion deformities.
Ideal treatment for ankylosed hip in modern era is total hip replacement. However in sickle cell disease patients belonging to tribal population requiring squatting ability and having financial constraints, alternatives like RAO (resection angulation osteotomy) and arthrodesis for mobility as well as stability respectively should be considered inspite of inferior outcomes.

Keywords: Sickle cell disease; Ankylosis; Tribal; RAO; Arthrodesis


How to cite this article:
Shah S N, Kapoor C S, Merh A A, Golwala P P. Bilateral ankylosed hip joint in a sickle cell disease patient: A case report. J Integr Health Sci 2014;2:31-2

How to cite this URL:
Shah S N, Kapoor C S, Merh A A, Golwala P P. Bilateral ankylosed hip joint in a sickle cell disease patient: A case report. J Integr Health Sci [serial online] 2014 [cited 2022 Jan 22];2:31-2. Available from: https://www.jihs.in/text.asp?2014/2/2/31/239546




  Introduction Top


Hb S (sickle cell gene) has been reported from various Indian states, communities and ethnic groups with an average frequency of 4.3% (range: 0-44%). Hb S is predominantly found in central India i.e. Vidarbh in Maharashtra, Madhya Pradesh, Orissa, Andhra Pradesh, Gujarat [1]. These patients have multiple orthopaedic complications like AVN femoral and humeral head, osteomyelitis, etc. due to higher chances of vaso-occlusive haemoglobin-opathies[2]. In places where sickle cell disease is more prevalent, progression of collapse in five years following diagnosis is seen in more than 90% of patients with AVN of femoral head[3]. One such patient having bilateral hip ankylosis with sickle cell disease has been reported.

Case Report

A 17 year old female, known case of sickle cell disease, came to our hospital with complains of insidious onset bilateral hip pain since five years which gradually increased over period of time. She had inability to walk and carry out daily activity since one year. She had no history of acute joint pain or abdominal crisis.

On examination both hips showed ankylosis with right hip fused in 10° of flexion and no rotations whereas left hip fused in 20° flexion and 10° adduction with no further movements possible. Both knees also had flexion deformities. Hip radiograph confirmed bony ankylosis (L>R).

Knee deformities were corrected by skeletal traction for two weeks. Left hip was treated with subtrochanteric valgus osteotomy and DHS to achieve 20° abduction whereas in right hip resection angulation osteotomy (RAO) was done after two weeks followed by bilateral skeletal traction for six weeks till adequate callous was seen. Gradual muscle strengthening was encouraged with weight bearing at eight weeks post operatively.

Pre-operative X-ray



Post-operative X-ray




  Discussion Top


Osteomyelitis, septic arthritis and avascular necrosis are very common orthopaedic complications encountered in sickle cell disease patients. Most of the times ankylosis can ensue septic arthritis resulting into complete resorption of the articular cartilage. Also in tribal population due to lack of literacy and health care, patient might not get adequate medical treatment and can have inadequate compliance leading to poorer outcomes.

Ankylosis of hip can lead to abnormal gait as well as difficulty in carrying out day to day activities, especially sitting cross legged and squatting (a necessity of tribal population). However if both the hips are ankylosed as in the given case, life becomes miserable as the patient cannot address even the toilet needs.

Ideal treatment for ankylosed hip in modern era is total hip replacement with acceptable outcomes [4]. However in sickle cell disease patients as stated earlier, as ankylosis usually follows infection, total hip replacement may have higher chances of complications giving inferior results.

Also for patients belonging to tribal population who require squatting ability and have financial constraints, alternatives like RAO and arthrodesis for mobility as well as stability respectively should be considered inspite of inferior outcomes. In this case, as the patient had bilateral involvement, RAO if done bilaterally can result in significant instability whereas bilateral arthrodesis is not feasible. Thus in this case, more ankylosed hip (left) was treated with subtrochanteric valgus osteotomy and DHS, to achieve 20 degree abduction which is necessary to address toilet as well as sexual needs. However on other side, RAO was done to provide mobility to the joint and aide movement. This treatment may not provide cosmetic and functionally excellent movements, but definitely helps to improve the quality of life by addressing the basic needs of the patient.



 
  References Top

1.
Patel AB, Athavale AM. 2004. Sickle cell disease in Central India. Ind. J. Paediatr. Vol.71.pp789-793  Back to cited text no. 1
    
2.
Platt, O.S., Thorington, B.D., Brambilla, D.J., Milner, P.F., Rosse, W.F.,Vichinsky, E. & Kinney, T.R. (1991) Pain in sickle cell disease. Rates and risk factors. New England Journal of Medicine, 325, 11-16.  Back to cited text no. 2
    
3.
Neumayr LD, Aguilar C, Earles AN, Jergesen HE, Haberkern CM, Kammen BF, et al. National Osteonecrosis Trial in Sickle Cell Anemia Study Group. Physical therapy alone compared with core decompression and physical therapy for femoral head osteonecrosis in sickle cell disease. Results of a multicenter study at a mean of three years after treatment. J Bone Joint Surgery 2006; 88: 2573-82.  Back to cited text no. 3
    
4.
Al-Mousawi F1, Malki A, Al-Aradi A, Al-Bagali M, Al-Sadadi A, Booz MM. Total hip replacement in sickle cell disease. Int Orthop. 2002; 26(3):157-61. Epub 2002 Apr 5.  Back to cited text no. 4
    




 

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