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SICKLE CELL ANAEMIA - An Interesting Outcome of a Difficult Fracture
(Intracapsular Fracture Neck Femur)


Harish S Hosalkar*, AD Kulkarni**, MG Yagnik***, Hetal Shah****, Priti Gujar*****
*Senior Registrar; **Intern; ***Consultant Hon H.O.D. Peadiatric Orthopaedics; ****Lecturer, Department of Anesthesia; *****Lecturer, Department of Radiology. Bai Jerbai Wadia Hospital for Children, Parel, Mumbai.

An interesting case of a fracture neck femur in sickle cell anaemia patient is presented. After good immobilisation the fracture united without the surgical intervention.

INTRODUCTION

Pathological fractures occur spontaneously or following minimal injury in long bones and vertebrae, commonly because of diminished strength of osteoporotic bone or occasionally through an infarcted area.

CASE REPORT

We present a case of 6 year old female child with sickle cell anaemia who presented with severe pain in hip following a trivial trauma and developed fever later.

Examination revealed painfully restricted range of movements of hip joint and radiological evidence of undisplaced transcervical fracture neck femur (Fig. 1). MRI was done to ascertain infection/infarction aetiology in view of haemoglobin of 6 gm%, polymorphonucleocytosis, 70% sickling on peripheral smear. In view of high risk for anaesthesia and increased incidence of infection (80% with respect to 70% sickling) it was decided to conserve the fracture. The patient was immobilized in a Thomas’ splint and medical line of management was carried out. The fracture completely united in 3 months period although with slight coxa vara and at present child is independently mobile although walking with slight limp. The child can squat, sit, crosslegged and run. This 2 years follow up has not shown increase in coxa vara.

DISCUSSION

Bone and joint crisis are common in sickle cell anaemia after 2 or 3 years of age as sludging of blood occurs in larger bones of extremities, spine, rib cage and periarticular structures. [1-4] Bone ischaemia is most frequent in humerus, tibia and femur. [2]

Osteomyelitis often involving multiple sites occurs with increased frequency at all ages, over 50% of these being caused by salmonella, [5,6] staphylococcal and pneumococcal also with increased frequency. [7,8] Provided that infection and vasoocclusive crisis is taken care of, diaphyseal fracture are known to have a normal course of healing.

In our case intra-capsular trans-cervical fracture neck of femur (which has high incidence of non-union) inspite of sickle cell anaemia (with sickling of 70%) and associated ischaemic changes without any surgical fixation did progress to union.

Fig 1: Clinicoradiological sequence of transcenical fracture neckfemur in case of sickle cell anaemia
Fig 1
Clinicoradiological sequence of transcenical fracture neckfemur in case of sickle cell anaemia


CONCLUSION

It is worth while noting the fact that even intra capsular fracture neck of femur can unite in sickle cell haemoglobinopathy provided the medical management is duly taken care of and adequate immobilization offered thus avoiding need for surgical intervention.

ACKNOWLEDGEMENTS

Dr. AR Bacha Consultant Peadiatric Orthopaedics. Dr. AN Johari Consultant Peadiatric Orthopaedics. Dean Dr. NC Joshi for having permitted to carry out this analysis and presentation.

    REFERENCES

    1. Diggs LW. Sickle cell crisis. Am J Clin Patho 1965; 44 : 1.
    2. Keeley K, Buchanan GR. Acute infarction of long bones in children with sickle cell anaemia. J Pediatr 1982; 101 : 170.
    3. Konotey - Ahulu FID. Clinical manifestation of sickle cell disease including the sickle cell crisis.
    4. Milner PF, Brown TA. Bone marrow infarction in sickle cell anaemia : Correlation with hematologic profiles. Blood 1982; 60 : 1411.
    5. Constant E, et al. Salmonella osteomyelitis of both hands and hand foot syndrome. Arch Surg 1971; 102 : 148.
    6. Diggs LW, Williams DL. Treatment of painful sickle cell crisis with papaverine.
    7. Barret Connor E. Acute Pulmonary diseases and sickle cell anaemia. Am Rev Respire Dis 1971; 104 : 159.
    8. Engh CA, et al. Osteomyelitis in patient with sickle cell disease. J Bone Joint Surg (Am) 1971; 53 : 1.

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