British Journal of Radiology (2004) 77, 698-700
© 2004 British Institute of Radiology
doi: 10.1259/bjr/14109917
Selective spleen scintigraphy in the diagnosis of polysplenia syndrome
E Abut, MD1,
L Akkaya, MD2,
U Uysal, MD2,
A Arman, MD3,
H Güveli, MD1,
C Bölükbas, MD1 and
O Ö Kurdas, MD1
Departments of 1 Gastroenterohepatology and 3 Radiology, Haydarpasa Numune Training and Research Hospital, Tibbiye caddesi Üsküdar 34668, Istanbul and 2 Department of Nuclear Medicine, Polis Hospital, Altunizade Üsküdar 81190, I·stanbul, Turkey
 |
Abstract
|
|---|
This report describes a case of polysplenia syndrome diagnosed by selective spleen scintigraphy. This syndrome is rarely encountered in the elderly. It is characterized by multiple splenuncules with a number of associated congenital anomalies involving the cardiovascular system and the viscera. The differential diagnosis of these intra-abdominal masses can sometimes be difficult and can be confused with lymphadenopathy or metastases. Furthermore, biopsy of the splenuncules may be hazardous. The diagnosis of polysplenia can be established in most instances by 99Tcm labelled heat-denatured red blood cell selective spleen scintigraphy without biopsy.
|
Introduction
|
|---|
Polysplenia syndrome is a rarely encountered condition in the elderly age, consisting of multiple small spleens (splenuncules) in the abdominal cavity and congenital anomalies of the cardiovascular system and viscera [1]. This syndrome is often identified incidentally on ultrasound examination [2]. The multiple small spleens seen on ultrasound may mimic lymphadenopathy or neoplasia and may be a diagnostic problem for radiologists and clinicians. Needle biopsy may not be always diagnostic and sometimes may be hazardous causing haemorrhage or rupture. However, the use of 99Tcm heat-denatured red blood cell (RBC) selective spleen scintigraphy enables the correct diagnosis to be made by confirming the splenic nature of these masses [3].
In this report, we present a case with multiple intra-abdominal masses that were erroneously diagnosed as intra-abdominal lymphadenopathy or metastases on initial ultrasound examination, but 99Tcm labelled heat-denatured RBC selective spleen scintigraphy showed the patient had polysplenia syndrome.
|
Case report
|
|---|
A 62-year-old female patient was admitted to our gastroenterohepatology department in April 2002 with complaints of fatigue and abdominal discomfort. On her past medical history, she had an atrial septal defect operation 20 years ago. Physical examination revealed an enlarged liver, other findings were unremarkable. Laboratory findings showed elevated transaminases and HCV-RNA positivity. Abdominal ultrasound examination revealed hepatomegaly with left lobe hypertrophy and para-aortic round solid multiple masses. These masses were initially reported as either lympadenopathy or metastatic lesions. However, abdominal CT scan, performed to deliniate the masses, demonstrated an enlarged and centrally located liver and prominent left lobe hypertrophy, but interestingly the spleen was not present in its normal location (Figure 1
). Furthermore, in the para-aortic region, there were multiple round solid masses (Figures 2, 3). In addition to these findings, absence of the subhepatic portion of the inferior vena cava (IVC) and the presence of an azygos/hemiazygos continuation anomaly (azygos/hemiazygos vein replaced IVC and drained directly to superior vena cava) were noted.
View larger version (103K):
[in this window]
[in a new window]
|
Figure 1. Contrast-enhanced CT scan at the level of upper abdomen demonstrates centrally located liver with absent spleen and right-sided stomach (S). Notice absence of inferior vena cava, it is replaced by dilated azygos vein (Az). Aorta was in the normal position (Ao).
|
|
View larger version (128K):
[in this window]
[in a new window]
|
Figure 2. Contrast-enhanced CT scan at the level of the kidneys demonstrates a centrally located and multilobated mass (arrows).
|
|
View larger version (133K):
[in this window]
[in a new window]
|
Figure 3. Contrast-enhanced CT scan caudal to Figure 2 demonstrates two separate centrally located masses (arrows).
|
|
A centrally located liver with absent spleen and azygos/hemiazygos continuation anomaly with absent intrahepatic portion of the IVC are usually associated with polysplenia syndrome. The multiple para-aortic solid masses that were demonstrated on ultrasound and CT scan were thought to represent multiple splenuncules, suggesting that the patient had polysplenia syndrome. To exclude the possibility of para-aortic lymphadenopathy or metastases and to verify the splenic nature of these masses, selective spleen scintigraphy using 99Tcm labelled denatured autolog erythrocytes (99Tcm heat-denatured RBC selective splenic scintigraphy) was performed [4]. On this examination, multiple round to ovoid hyperactive regions with the same appearance and shape of the masses seen on the CT scan were demonstrated (Figure 4). These findings confirmed the masses were multiple splenuncules, confirming the diagnosis of polysplenia syndrome.
View larger version (125K):
[in this window]
[in a new window]
|
Figure 4. 99Tcm heat-denatured red blood cell selective splenic scintigraphy; anterior, posterior, and right–left oblique projections. Notice midline hyperactive round foci compatible with the splenuncules seen on the ultrasound and CT scan.
|
|
Thoracic CT and echocardiography were performed to detect other anomalies related to this syndrome. It was found that each lung had two lobes on each side representing left sided isomerism. The hepatic veins drained directly into the right atrium. The patient's echocardiographic examination showed only mild pulmonary hypertension and there was no atrial abnormality.
Because the patient's complaints were not associated with polysplenia syndrome, and the transaminases were elevated with HCV-RNA positivity, a percutaneous liver biopsy was performed and revealed chronic hepatitis C infection. Combination therapy with interferon alpha and ribavirin was started.
|
Discussion
|
|---|
Polysplenia syndrome is a form of situs ambiguous anomaly (disturbing the physiological asymmetry of the viscera). This congenital anomaly is rarely encountered in the elderly age (about 2.5:100 000 live births but only 5% of patients survive beyond 5 years of life) [5, 6]. The typical feature of this syndrome is the existence of the multiple small spleens in the abdominal cavity with absence of the normally located spleen. Furthermore, associated congenital anomalies of the cardiovascular system and viscera are often present (such as atrial septal defect, ventricular septal defect, pulmonary stenosis, bilateral superior vena cavae, azygos/hemiazygos continuation of the IVC, malrotation of gut, centrally located liver, pulmonary isomerism, biliary atresia, absent gallbladder and short pancreas) [6].
This syndrome is generally diagnosed in the newborn or infant period, because the majority of patients have severe congenital cardiac anomalies. Most of these patients have severe cyanosis and most of them die before the age of 2 years. Less than 5% of patients survive beyond 5 years and these patients can be incidentally detected on routine radiological examination [7].
There are no clinical abdominal diagnostic features of the multiple spleens, but when they are detected incidentally during investigation, the absence of the intrahepatic portion of the IVC on ultrasound or CT scans, enables a correct diagnosis to be made. This can be confirmed using 99Tcm labelled heat-denatured RBC splenic scintigraphy. With this examination, 99Tcm labelled heat-denatured autolog erythrocytes are vigorously taken up by the splenic tissues and the splenunculi can be easily visualized [8]. When the multiple hyperactive regions are found in the abdomen, they should not be confused with accessory spleens or splenosis. In the cases of the accessory spleen, the small splenic masses are located besides a normal spleen without the multisystem involvement characteristics of polysplenia [9]. Splenosis represents heterotopic autotransplantation and implantation of splenic tissue, most commonly within the peritoneal cavity after splenic trauma or surgery, and the patient should have a history of abdominal trauma or surgery [10].
|
Conclusion
|
|---|
The existence of intra-abdominal undiagnosed masses on ultrasound or CT scan examination together with cardiac anomalies and lack of the intrahepatic portion of the IVC (azygos/hemiazygos continuation anomaly) should alert the radiologists and clinicians to the possibility of polysplenia syndrome. The diagnosis may be confirmed if necessary by 99Tcm labelled heat-denatured RBC selective spleen scintigraphy.
|
Footnotes
|
|---|
Address correspondence to Dr Evren Abut, Nuzhetiye Caddesi Deniz Apartmani No: 38–40 Daire: 15, 80690, Besiktas-Istanbul, Turkey. 
Received for publication November 19, 2002.
Accepted for publication September 22, 2003.
|
References
|
|---|
- Hernanz-Schulman M, Ambrosino MM, Genieser NB, Friedman D, Banner H, Rumancik W, et al. Pictorial essay. Current evaluation of the patient with abdominal visceral situs. AJR Am J Roentgenol 1990;154:797–802.[Abstract/Free Full Text]
- Sener RN, Alper H. Polysplenia syndrome: a case associated with transhepatic portal vein, short pancreas, and left inferior vena cava with hemiazygous continuation. Abdom Imaging 1994;19:64–6.[CrossRef][Medline]
- Derbekyan VA, Pham C, Emond C, Novales Diaz JA, Lisbona R. Scintigraphic diagnosis of polysplenia in the adult. Clin Nucl Med 1999;24:161–3.[CrossRef][Medline]
- Shimizu M, Seto H, Kageyama M, Wu YW, Nagayoshi T, Kamisaki Y, et al. The value of combined 99mTc-Sn-colloid and 99mTc-RBC scintigraphy in the evaluation of a wandering spleen. Ann Nucl Med 1995;9:145–7.[Medline]
- Winer-Muram HT. Adult presentation of heterotaxic syndromes and related complexes. J Thorac Imaging 1995;10:43–57.[Medline]
- Rose V, Izukawa T, Moes CA. Syndromes of asplenia and polysplenia: a review of cardiac and non-cardiac malformations in 60 cases with special reference to diagnosis and prognosis. Br Heart J 1975;37:840–52.[Abstract/Free Full Text]
- Peoples WM, Moller JH, Edwards JE. Polysplenia: a review of 146 cases. Pediatr Cardiol 1983;4:129–37.[CrossRef][Medline]
- Gunes I, Yilmazlar T, Sarikaya I, Akbunar T, Irgil C. Scintigraphic detection of splenosis: superiority of tomographic selective spleen scintigraphy. Clin Radiol 1994;49:115–7.[CrossRef][Medline]
- Andrews MW. Ultrasound of the spleen. World J Surg 2000;24:183–7.[CrossRef][Medline]
- Logan H, Harding B, Gaffney R, Mooney E, Masterson J, Duffy G. Detection of splenosis: 99Tcm tin colloid scanning and computed transmission tomography compared. Nucl Med Commun 1988;9:993–7.[Medline]
Top
Abstract
Introduction
