|Year : 2020 | Volume
| Issue : 3 | Page : 288-291
Isolated splenic peliosis
Rait Patel, Jay Kantilal Satapara, Nandini Bahri
Department of Radiodiagnosis, Shri M. P. Shah Government Medical College and Shri Guru Gobind Singh Government Hospital, Jamnagar, Gujarat, India
|Date of Submission||26-Mar-2019|
|Date of Acceptance||09-Sep-2019|
|Date of Web Publication||3-Jun-2020|
Jay Kantilal Satapara
Department of Radiodiagnosis, Shri M. P. Shah Government Medical College and Shri Gurugobind Singh Government Hospital, P. N. Marg, Jamnagar - 361 008, Gujarat
Source of Support: None, Conflict of Interest: None
Peliosis a rare condition characterized by multiple blood-filled cavities within the parenchyma of solid organs mainly organs of mononuclear phagocytic system. Peliosis is associated with tuberculosis, human immunodeficiency virus, disseminated cancer, previous thorium dioxide contrast injection, chronic hematological disorders, and many other conditions. Peliosis is usually asymptomatic and diagnosed as an incidental finding. Imaging modalities such as ultrasonography, computed tomography (CT), and magnetic resonance imaging are useful for diagnosis. On CT, hypoattenuating nodules with fluid-fluid levels are a unique feature for diagnosing peliosis. Although rare and asymptomatic most of the time, peliosis is an important condition to be aware of because rupture of the blood-filled cysts can lead to hemoperitoneum and ultimately be fatal. We reported a case of a 35-year-old male with isolated splenic peliosis with its rupture and resultant hemoperitoneum.
Keywords: Computed tomography, hemoperitoneum, magnetic resonance imaging, peliosis, splenic peliosis, ultrasonography
|How to cite this article:|
Patel R, Satapara JK, Bahri N. Isolated splenic peliosis. Med J DY Patil Vidyapeeth 2020;13:288-91
| Introduction|| |
Peliosis is characterized by multiple blood-filled cavities within the parenchyma of solid organs. It can affect organs of mononuclear phagocytic system, especially liver; however, isolated splenic peliosis is a unique phenomenon. Patients are usually asymptomatic. However, this condition can be potentially lethal if spontaneous organ rupture occurs. We reported a case of a 35-year-old male with isolated splenic peliosis with its rupture and resultant hemoperitoneum.
| Case Report|| |
A 35-year-old male patient presented to the emergency department with acute abdominal pain and abdominal distension since 1 day. The patient had no history of any trauma or fever and had no associated altered bowel or bladder habit. The patient had a history of similar complaints 5 years back for which he had undergone emergency exploratory laparotomy and was diagnosed to have an intestinal perforation which was repaired at that time.
On per abdominal examination, tenderness and guarding was present with increased abdominal girth; however, bowel sounds were normal. Per rectal examination was unremarkable.
Routine blood investigations such as complete blood count, liver function test, and renal function test were performed all of which were within the normal limits except for hemoglobin, which was decreased (7.5 mg/dl); however, the patient was hemodynamically stable.
Ultrasonography (USG) was performed which revealed few well-defined heterogeneously hypoechoic lesions with high-level internal echoes in interpolar region and upper pole of spleen [Figure 1]a. On color Doppler study, the lesions showed no significant internal vascularity [Figure 1]b. Gross amount of fluid with moving internal echoes and internal echogenic content was noted within the peritoneal cavity [Figure 2]. As the patient had a history of intestinal perforation, USG-guided aspiration was done to differentiate causes of ascites with echoes, aspiration showed frank blood suggestive of hemoperitoneum with clot formation. Multiple dilated anechoic vascular channels were noted replacing main portal vein showing color flow on Doppler study suggestive of cavernous transformation of the portal vein. Multiple dilated anechoic channels were noted in gastrosplenic, splenic hilar, peripancreatic, and periportal region suggestive of varices formation.
|Figure 1: Ultrasonography showing (a) well-defined heterogeneously hypoechoic lesion with internal echoes in interpolar region of spleen. (b) Color Doppler study showing no significant internal vascularity within lesion|
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|Figure 2: Ultrasonography showing gross ascites with moving internal echoes within peritoneal cavity (hemoperitoneum)|
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On contrast-enhanced computed tomography (CT), the lesions in spleen appeared to be heterogeneous and nonenhancing with approximate HU: 55. The larger one communicated with the peritoneal cavity through a capsular breech with resultant moderate-to-gross hemoperitoneum. Cavernous transformation of portal vein associated with multiple varices formation seen in ultrasound was confirmed in CT [Figure 3].
|Figure 3: Contrast-enhanced computed tomography showing (a) Nonenhancing heterogeneous lesion in spleen (vertical arrow) and hemoperitoneum (horizontal arrow). (b) Cavernous transformation of portal vein (horizontal arrow) and multiple varices (vertical arrow)|
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On noncontrast magnetic resonance imaging (MRI), splenic lesions appeared heterogeneous (Iso to slightly hyperintense) with internal hypointense content on T1-weighted imaging (T1WI) and hyperintense with peripheral hypointense rim on T2WI and showed internal fluid–fluid level suggestive of recent and old internal hemorrhage. Gross hemoperitoneum with multiple internal clot formation was confirmed [Figure 4] and [Figure 5].
|Figure 4: Noncontrast magnetic resonance imaging, T2-weighted image showing hyperintense lesion in spleen with peripheral hypointense rim and internal fluid-fluid level suggestive of recent and old internal hemorrhage. (a) Axial scan (b) coronal scan|
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|Figure 5: Noncontrast magnetic resonance imaging, T1-weighted image showing heterogeneous (Iso to slightly hyperintense) lesion in spleen with internal hypointense content|
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The patient was operated, in which imaging findings were confirmed, and specimen was sent for histopathological examination. On macroscopic examination, spleen measured (100 mm × 75 mm × 40 mm) weighing 175 g with subcapsular hemorrhagic nodules, one of which showed capsular tear of size 80 mm [Figure 6]. On sectioning, multiple blood-filled spaces up to 15 mm diameter were noted throughout the parenchyma. Histology showed unlined cystic spaces filled with blood. The features were consistent with splenic peliosis.
|Figure 6: Postoperative specimen of removed spleen showing subcapsular hemorrhagic nodules with capsular tear|
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| Discussion|| |
Peliosis is a rare pathological entity characterized by multiple blood-filled cavities within parenchyma of solid organs. Previously, it was thought that peliosis exclusively develops in organs of mononuclear phagocytic system including liver, spleen, bone marrow, and lymph nodes. However, few studies indicate the involvement of other organs too, such as lungs, parathyroid glands, and kidneys., Splenic peliosis was thought to be occurring only in association with more common peliosis hepatis, and isolated case of splenic peliosis had not been reported before 1978.
Peliosis has been associated with the oral contraceptive pill, anabolic steroids, advanced tuberculosis, human immunodeficiency virus (HIV), disseminated cancer, previous thorium dioxide contrast injection, and chronic hematological disorders such as Hodgkin's disease, myeloma, and aplastic anemia. In all these cases, both the liver and the spleen are usually involved. Isolated splenic peliosis is usually asymptomatic and detected incidentally on imaging for other indications or at autopsy.
Peliosis in patients with AIDS is due to secondary infection with Bartonella henselae and Rochalimaea henselae. Other infective agents which are thought to be associated include hepatitis B and C, Staphylococcus aureus, and tuberculosis.,
Increased serum vascular endothelial growth factor has also been linked. It may be seen in patients with diabetes mellitus, end-stage renal disease, and renal transplantation.
The exact etiology of peliosis hepatis is not clear, but postulated hypothesis suggests initial focal cell necrosis leading to the destruction of the reticulum framework and cyst formation from the inflow of blood from adjacent sinusoids.
The complications of splenic peliosis include rupture of the hemorrhagic cysts with life-threatening intraperitoneal hemorrhage either spontaneously or secondary to minimal trauma.
Pathologically, the surface of the spleen shows nodular areas on macroscopic examination. On cut surface, numerous cyst-like blood-filled cavities are seen. These cavities are round to oval dilated sinuses in the parafollicular location of the red pulp.
On USG, multiple hypoechoic or hyperechoic lesions with poorly demarcated borders are seen; sometimes, entire spleen may be involved. On CT, multiple low-attenuation foci are seen. The enhancement pattern is similar to that of hemangiomas, showing eventual slow centripetal enhancement. Others have reported multiple hypoattenuating nodules with fluid-fluid levels due to hematocrit effect and show enhancement in their dependent portions which is unique for peliosis. Usually, extracapsular extension is not seen. However, if lesions rupture, subcapsular hematoma, and intraperitoneal hemorrhage may be seen on CT.
On MRI, lesions show mixed signal on T2-weighted sequences due to the presence of deoxyhemoglobin and methemoglobin. Peliosis hepatis show increased signal on T2-weighted sequences and variable signal on T1-weighted and proton-density sequences, reflecting various stages of hemorrhage. On postcontrast MRI, none of the lesions enhances.
The differential diagnosis of peliosis includes hemangiomatosis, lymphangioma, and angiosarcoma.
Definitive management is done by splenectomy.
| Conclusion|| |
Although splenic peliosis is a rare benign disorder, it is usually associated with other chronic contributing factors (HIV, hematological factors, steroids, and the oral contraceptive pill) so search for the other disease should be done. As life-threatening hemorrhage can occur spontaneously or as a result of minor trauma from the rupture of surface lesions, an early diagnosis of this rare anomaly and definite management can prevent fatal outcome, and imaging modalities are helpful in making the diagnosis.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.]
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]