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Year : 2003  |  Volume : 13  |  Issue : 2  |  Page : 161-163
Sickling as a cause of bilateral avascular necrosis of hip with autoinfarction of spleen

Department of radiology, E.S.I.S, General Hospital, Bapunagar, B.J. Medical College, Ahmedabad-380024, India

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Keywords: Sickle cell disease, Avascular Necrosis, hip joint, Splenic infarct

How to cite this article:
Shash H R, Patwa P C, Tannk A V, Jindal N, Hans D, Nayak C. Sickling as a cause of bilateral avascular necrosis of hip with autoinfarction of spleen. Indian J Radiol Imaging 2003;13:161-3

How to cite this URL:
Shash H R, Patwa P C, Tannk A V, Jindal N, Hans D, Nayak C. Sickling as a cause of bilateral avascular necrosis of hip with autoinfarction of spleen. Indian J Radiol Imaging [serial online] 2003 [cited 2020 Dec 4];13:161-3. Available from:

   Introduction Top

We wish to report a case of "Sickling as a cause of bilateral avascular necrosis of hip with autoinfarction of spleen", diagnosed by using various radiological modalities and confirmed by blood investigation.

   Case report Top

A 37 yr old male presented with pain in the right hip joint more so on walking and in the squatting position for 1 year. There was history of slipping on the floor 1 year back. Patient gave a history of limp during walking. There was history of pain in the elbows, knees, shoulder and wrist joint on exertion and in winter & he took analgesics for it. There was history of Jaundice two months back.

There was no history of corticosteroids taken in the past by patient. Patient was operated for piles 12 years back. There was history of scrotal swelling predominantly left sided for 3 years, when patient had trauma to that region. The swelling was not increasing in size and was not associated with pain. No history of diabetes mellitus, hypertension or pulmonary tuberculosis.

`X-ray pelvis with both hips' (AP view) [Figure - 1] was done, which revealed grade V avascular necrosis on right hip and grade III avascular necrosis on left hip. Preoperative chest X-ray was advised which showed dense Radio-opaque shadow in left hypochondriac region. USG was advised which showed calcified spleen. Ct abdomen [Figure - 2] showed, shrunkened calcified spleen measuring 47.02 x 18.26 mm.

Peripheral smear for sickling of RBC's was advised, which proved to be positive. MRI of pelvis with both hips [Figure - 3] A & B showed grade IV avasular necrosis on right hip and grade IV avasular necrosis on left hip.

Skeletal survey was performed which showed : X-ray Dl spine [Figure - 4] :- Increased bone density with kyphosis with coarsened trabecular pattern. X-ray shoulder joint [Figure - 5] :- Areas of lucency and sclerosis producing `snow-capped' appearance.

Blood investigations revealed :

  • Serum Bilirubin - 2.1 mg%
  • Serum alkaline phosphate - 560 KA
  • Haemoglobin - 9.6 gm%
  • Peripheral smear - RBC's are moderately hypochromic predominantly microcytic, moderate degree of Anisopoikilocytosis, Tear drop cells, Acanthosis and sickle cells present. Total count within normal limits. Platelets are adequate. No premature cells or parasites seen.

   Discussion Top

The chronic, congenital and hereditary hemolytic anaemia occur exclusively in Negros who are homozygous for sickle trait. The sickle cell disease is transmitted as a Mendelian dominant.

The basic pathogenesis of sickle crisis is the abnormal erythrocytes when deprived of oxygen become long and slender in shape which causes vasocclusion and tissue death. These infarcts may occur in any tissue system but skeleton, kidneys, Lungs, spleen, brain are common sites. The areas of skeletal avascularity resulting from infarction are due to systemic disease.

Two groups have been defined :

1. Heterozygous : Which is milder, with normal survival rate.

2. Homozygous : In which patient is symptomatic and survival after 30 years in rare.

Appearance depends upon the

1. Duration

2. Homozygosity or Heterozygosity

The splenic changes in sickling are :

  • Splenomegaly (less than 10 years)
  • Calcification and progressive shrinkage of the spleen [2],[3]
  • Repeated infraction of the spleen [1]
  • Acute splenic sequestration crisis [1],[2]

1) Due to venous drainage obstruction of small intrasplenic veins or within sinusoids.

2) Sudden splenic enlargement.

  • Splenic rupture as a complication [2]
  • Rarely massive splenic infarction [6]

The bony changes in sickling are :

  • Deossification due to marrow hyperplasia [8]
  • Thrombosis and infarction leading to osteonecrosis of ends of long bones particularly head of femur and humerus. [5],[8]
  • Secondary osteomyelitis [8],[4]
  • Growth effects.

One study reveals that the sickle cell disease is most common cause of Avascular Necrosis in childhood and those having Hbs-s genotype are at highest risk.

   References Top

1.S.A. Sohaib and Rodney H. Reznek; Reticulo endothelial disorders : the spleen. In R.G. Grainger, D.J. Allision, A. Adam, A.K. Dixon, Diagnostic Radiology (Vol-2) fourth edition, Churchill Livingstone. Edinburgh, PP 1433-1445.  Back to cited text no. 1    
2.Warshauer DM, Koehler RE 1998 Spleen. In : Lee JK Sagel SS, Stanley RJ, Heiken JP (eds) CT with MRI corelation, 3rd edition. Lippincott, Raven, Philadelphia PP (845-872)  Back to cited text no. 2    
3.Christian Gorg; The Spleen : In Cosgrove DO, Miere, HB, Dewbury KCD (eds), Farrant pat; Clinical ultrasound (2nd edition) Churchill livingstone, Edinburgh pp 381-445.  Back to cited text no. 3    
4.Terry R. Yochum, Lindsay J. Rowe : Hematologic and vascular disorders; Essentials of skeletal radiology (2nd edition) William & Wilkins, Philadelphia pp (1234-1326).  Back to cited text no. 4    
5.Milner PF, Kraus AP, Seber JI, Sleeper LA, Dukes KA, Embury SH, et al. Sickle cell disease as a cause of osteonecrosis of femoral head. New England Journal of Medicine 1991; 325(21) : 1476-81.  Back to cited text no. 5    
6.Jama AH, Salem AH, Dabbous IA, Massive splenic infarction in Saudi patients with sickle cell anemia : a unique manifastation. American journal hematoly 2002 Mar; 69(3):205-9.  Back to cited text no. 6    
7.Styles CA, Vichinsky EP. Core depression in AVN in sickle cell disease. American Journal of hematology 1996; 52(2): 103-7.  Back to cited text no. 7    
8.D.J. Stoker, A Saifuddin; Myeloproliferative and similar disorders in R.G. Grainjer, D.J. Allison, A Adam, A.K. Dixon Diagnostic Radiology Volume 3 4th edition. Churchill Livingstone Edinburgh pp (1901-24)  Back to cited text no. 8    

Correspondence Address:
P C Patwa
87/708, Pushpak Apartments, Mirambika Road, Naranpur, Ahmedabad 380 063
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Source of Support: None, Conflict of Interest: None

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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]


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