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ABDOMINAL IMAGING Table of Contents   
Year : 2004  |  Volume : 14  |  Issue : 3  |  Page : 247-251
Sonological evaluation of abdominal organs in sickle cell crisis in Western Orissa


Department of Radiology, V.S.S. Medical College and Hospital, Burla, Sambalpur, Orissa, India

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   Abstract 

OBJECTIVE : Sickle cell disease (SCD), being prevalent in the western belt of Orissa, the following study was undertaken to evaluate the various spectrum of abdominal sonographic findings involving the liver, spleen, gall bladder and kidneys. MATERIALS AND METHODS : The study included fifty patients of SCD presented with acute symptoms of sickle cell crisis. RESULTS : The most frequent US findings in the study were: hepatomegaly (72 percent), splenomegaly (64 percent) and cholelithiasis (22 percent). 22 percent of patients presented with splenic infarction, 10 percent patients showed splenic calcification. Increased bilateral renal echogenicity was observed in 16 percent of patients. CONCLUSIONS : To conclude ultrasonography is a readily accessible and non invasive method for the evaluation of different abdominal organs.

Keywords: Abdominal ultrasonography, sickle cell disease, sickle cell crisis

How to cite this article:
Mohanty J, Narayan J, Bhagat S, Panda B B, Satpathi G, Saha N. Sonological evaluation of abdominal organs in sickle cell crisis in Western Orissa. Indian J Radiol Imaging 2004;14:247-51

How to cite this URL:
Mohanty J, Narayan J, Bhagat S, Panda B B, Satpathi G, Saha N. Sonological evaluation of abdominal organs in sickle cell crisis in Western Orissa. Indian J Radiol Imaging [serial online] 2004 [cited 2019 Nov 19];14:247-51. Available from: http://www.ijri.org/text.asp?2004/14/3/247/28595

   Introduction Top


In Orissa since the first case of sickle cell disease (SCD) reported by Nanda et al in 1967, considerable efforts have been made to study the various aspects of the disease. A very high prevalence (2-12%) of sickle cell disease is seen in western belt of Orissa particularly in - Sambalpur, Sundergarh, Jharsuguda and Bolangir districts.

The term "sickle cell crisis" refers to acute onset of symptoms like - fever, abdominal pain, joint and muscle pain, jaundice, oliguria etc. in SCD patients generally due to invivo sickling of red blood cells affecting various organs of the body. Among the different types of sickle cell syndromes mainly homozygous sickle cell anemia patients and rarely S/B thalassaemia or hemoglobin SC patients suffer from sickle cell crisis [1]. In vivo sickling results in increased blood viscosity which leads to stasis of blood leading to hypoxia followed by infarction of the tissues. The commonest organs involved in crisis are spleen, liver, biliary tract and kidneys. Though GI tract and adrenals may sometimes be affected, the percentage is very less.


   Materials and methods Top


Fifty patients of sickle cell disease belonging to either sex (36 males and 14 females) and different age groups (three to 48 years) were included in the present study.

Selection of patients was based on following criteria -

  1. All the cases taken for study were sickling positive.
  2. By Hb-electrophoresis method, out of 50 patients, 46 patients were SS type (homozygous SCD) and four patients were FSA2 type (S-B0 thalassaemia).
  3. All the patients had positive family history and sibling history of SCD in homozygous and heterozygous state.
  4. These patients presented with acute symptoms and signs of sickle cell crisis like - pallor, generalized weakness, upper abdominal pain, jaundice, joint pain and muscle pain, hepatomegaly, splenomegaly and oliguria.


By the time of examination, none of the patients had undergone any operation like splenectomy or cholecystectomy.

Seimenssonoline Adara and L and T Sigma ultrasound machines were used for the study. Standard views were obtained in transverse and sagittal plains with the patients in supine, right lateral and left lateral decubitus positions.


   Results Top


Changes found in liver,spleen and GB are shown in [Table - 1] and those of kidneys in [Table 2].

In the liver, hepatomegaly was seen in 36 patients with gross hepatomegaly in four patients. Two patients showed altered coarse parenchymal liver echo texture with enlarged diameter of portal and splenic veins at the porta hepatis and associated ascites. One patient had multiple liver abscesses.

Splenomegaly was the commonest finding in spleen found in 32 patients with five patients having spleen > 17cm. Four patients had very small shrunken spleen. Eleven patients had splenic infarction [Figure - 1], [Figure - 2] and five patients showed splenic calcification [Figure - 3]. One of the patients having splenic infarction developed a large abscess with in the spleen [Figure - 4], [Figure - 5]. In the GB, 11 patients showed calculi [Figure - 6] and four patients had biliary sludge in the lumen. Five of them showed thickened GB wall (> 3 mm.). Dilated CD (>6 mm) and IHBR were detected in one patients with features of obstructive jaundice.

[Table 2] shows that, 86% of patients in the present study had normal sized kidneys on both sides. Six patients showed small size kidneys and only one patient had enlarged kidneys. Renal cortical echogenicity was increased in eight patients [Figure - 7]. All of them had either normal or reduced size and three of them showed loss of cortico-medullary differentiation. No one of the patients showed renal calculus.


   Discussion Top


Sickle cell disease particularly in homozygous state has a significant contribution to the morbidity and mortality of this disorder during crisis. The various types of crisis cause changes in different organs of the body.

The present study includes 50 patients of SCD in crisis with special reference to the abdominal organs evaluated sonologically. The age distribution ranged from 3 to 48 years. Highest number of patients were in the age group of 11-20 years (44%) followed by 21-30 years (30%). Only seven patients were above 30years of age. The sex distribution of the disease revealed that there were 72% of male and 28% of female patients. This discrepancy was due to disproportional hospital admission rate between male and female patients.

In the studied group, we found 72% of patients with hepatomegaly. Among them gross hepatomegaly was seen in 8% patients. This is in accordance with the findings of Papadaki MG et al (2003) [2].The cause of hepatomegaly has been attributed to kupffer cell erythrophagocytosis and engorgement of sinusoids by aggregates of sickle cells. The patients with gross hepatomegaly showed elevated levels of bilirubin with clinical jaundice and associated hepatic tenderness, suggestive of painful crisis. Features of hepatic cirrhosis with ascitis found in two patients (4%). However Bauer et al (1980) and Green et al (1953) found cirrhosis in almost 20% of patients with sickle cell crisis [3]. Cirrhosis in SCD may be attributed to associated viral hepatitis, hemochromatosis, cardiac cirrhosis and due to some unknown cause. Rare focal anomalies like liver infarction and liver abscess have been described in SCD patients.

Though we could not find any case of liver infarction we got one patient with ameobic liver abscess in our series.

Splenomegaly was present in 64% of patients in our study,with 10% of patients having a spleen more than 17cm. of size. It is evident that mild to gross splenomegaly was seen in patients in their second (64%), third (53%), even fourth (60%) decade of life. This is in contrast to study of Sergeant et al (1985) who showed that after 30 years as the age advances spleen undergoes progressive fibrosis and shrinkage and only 6% had splenomegaly beyond 30 years [4]. Kar et al in 1986, attributed this persistence of splenomegaly in later age to HbF and association with alpha thalassaemia. In our study we also found small shrunken spleen in 8% of patients due to autosplenectomy. In the present study 22% of patients showed hypoechoic focal areas suggestive of splenic infarction which were both solitary and multiple in nature and were commonly spherical [Figure - 1] or wedge shaped [Figure - 2]. Only one of them presented with splenic abscess. This varies with findings of Ki-Yia Yeung et al (1974) and M.G.Papadaki et al(2001) who found Splenic infarction in 14.5% and 15% of patients respectively [5]. 10% of patients also had Splenic calcification which were both punctuate and amorphous in type. However Mac Call et al in 1981 reported that 31% of patients had Splenic calcification in his study on Jamaican patients [6].

In the present study gallstones were detected in 22% of patients and sludge in 8% of patients. The stones were multiple with posterior acoustic shadowing. This is in accordance with the findings of Lachman BS et al (1979) and Karayalcin G et al (1979),who detected stone in GB in SCD patients in 29% and 17% patients respectively [7]. 16.6% of males and 35.7% of females showed evidence of gall stones in our series. There is a progressive age related increase in prevalence of gall (7) stones which is more pronounced in third (33%) and fourth (40%) decades. One patient had CBD and IHBR dilatation but the cause could not be established.

The present study indicates that most of the kidney sizes were within normal limit. Only one patient showed increased size of kidneys (>12 cm.) probably due to acute papillary necrosis. This finding is not matching with that of Mac Call et al (1978) and I.S.Kaskarelis et al (2001) who showed enlargement of kidneys in 10% and 15.4% of patients respectively [8]. As much as 12% of patients had decreased size of kidneys with thinning out of cortices and 16% of patients had increased renal cortical echogenicity, all of them showed abnormally increased BUN and creatinine levels, indicating medical renal disease. It also shows loss of cortico-medullary differentiation in 6% of patients. Focal pyramidal changes due to ischemia in SCD patients may account for this change.


   Conclusion Top


It is therefore concluded that real time ultrasonography is a standard screening procedure in all cases of sickle cell disease patients for the assessment of the pathological changes occurring in the abdominal organs, specially liver, gall bladder, spleen and kidneys. The extent and changes in the various organs can accurately be observed and thereby help the clinicians to modulate their mode of treatment. In acute crisis, the patients can be divided as to needing whether surgical or medical intervention. Therefore abdominal sonography is an easy, cheap, accurate method for diagnosis, management and follow up of patients suffering from SCD and can be carried out as an out patient diagnostic procedure.

 
   References Top

1.Edward J.Benz, Jr. Harrison's principles of internal medicine, 15th ed. Mc Graw- Hill, 2001. Volume 1; 669-671  Back to cited text no. 1    
2.Papadaki MG et al. Abdominal ultrasonographic findings in patients with sickle cell anemia and thalassaemia intermedia. Pediatr Radiol 2003Aug; 33(8): 515-21  Back to cited text no. 2    
3.Baur TW, Moore GW, Hutchins GM. The liver in sickle cell disease. A clinicopathological study of 70 patients. Am J Med. 1980 Dec; 69(6): 833-7  Back to cited text no. 3    
4.Sergeant GR. Sickle cell disease: the spleen, 1st ed, New York:Oxford University press, 1985:109 -123  Back to cited text no. 4    
5.Papadaki MG et al. Ultrasonographic findings of the spleen in patients with sickle cell disease. ECR 2001- Presentation B-0785  Back to cited text no. 5    
6.McCall IW,Vaidya S, Sergeant GR. Splenic opacification in homozygous sickle disease, Clinical Radiology 1981; 32(611-15)  Back to cited text no. 6    
7.Karayalcin G, Hassani N, Abrams M, Lanzkowsky P. Cholelithiasis in children with sickle cell disease. Am J Dis Child. 1979 Mar; 133(3):306-7  Back to cited text no. 7    
8.Mac Call et al, 1978. Urographic findings in homozygous sickle cell disease. Radiology; 126(99-104)   Back to cited text no. 8    

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Correspondence Address:
J Mohanty
Department of Radiology, V.S.S. Medical College and Hospital, Burla, Sambalpur, Orissa
India
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Source of Support: None, Conflict of Interest: None


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    Figures

[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7]

    Tables

[Table - 1]

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