American Journal of Emergency Medicine 32 (2014) 194.e3–194.e4
Contents lists available at ScienceDirect
American Journal of Emergency Medicine journal homepage: www.elsevier.com/locate/ajem
Case Report
Isolated splenic peliosis with spontaneous rupture after a viperine bite☆,☆☆ Abstract Isolated splenic peliosis is an extremely uncommon condition that can present with atraumatic rupture and potential fatal outcome. We here report 1 such case that developed after a viperine bite in a 21-year-old woman. The case highlights the diagnostic findings on computed tomographic (CT) scan and its potential complications. Peliosis is a rare entity of unknown etiopathogenesis characterized by multiple cyst-like, blood-filled cavities predominantly in the mononuclear phagocytic system (liver, spleen, bone marrow, and lymph nodes) [1]. Splenic peliosis usually occurs in conjunction with peliosis hepatis [2,3]. Isolated cases of peliosis spleen are extremely uncommon, and until yet, only 41 cases have been reported in literature [4], of which only 10 patients presented with spontaneous rupture [2,59]. Here, we report a case of isolated peliosis spleen apparently due to a viperine bite leading to spontaneous rupture. To the best of our knowledge, this association has never been reported in the past. A 21-year-old unmarried woman, a household worker with history of viperine bite (the diagnosis of viperine bite was established based on clinical presentation and the morphological description of the snake), 5 days back was referred to our institute with severe pain and swelling of the right lower limb associated with painful abdominal distension. On clinical examination, she had marked pallor and ecchymotic patches on the right lower limb, around the left breast and left shoulder. The patient had marked pallor, but there was no cyanosis, clubbing, or lymphadenopathy. Blood pressure was 100/70 mm Hg; pulse rate, 106 per minute; and respiratory rate, 28 per minute. The chest radiograph showed a few patches of peripheral consolidations in both lungs. Laboratory investigations revealed hemoglobin 5.5 gm%, TLC 7000/mm 3, DLC N58L35M4E3B0, platelet counts 37 000/mm 3, random blood sugar 184 mg%, blood urea 33 mg%, serum creatinine1.0 mg%, serum Na 135, serum K 4.4, bilirubin 1.4 (conjugated 0.7); coagulation profile revealed prothrombin time of 25 seconds (control, 10 seconds) and activated partial thromboplastin time of 29 seconds (control, 25 seconds). Fibrin degradation products were found positive. Patient was managed with intravenous fluids, blood, antibiotics, and polyvalent antisnake venom. Ultrasound abdomen revealed splenomegaly with multiple illdefined hypoechoic lesions and presence of fluid in perisplenic area and in pelvis. A contrast-enhanced CT (CECT) (4-Slice Light Speed QX/I Plus; GE Healthcare, Milwaukee, WI) of abdomen was performed. The data acquisition was done in the porto-venous phase (65-second delay), which revealed enlarged spleen with
☆ Grant support: Nil. ☆☆ Conflicts of interest: Nil. 0735-6757/$ – see front matter © 2013 Elsevier Inc. All rights reserved.
multiple intrasplenic cysts of varying sizes with some of them showing high-density fluid levels suggestive of hemorrhage (Figs. 1 and 2). There was suggestion of rupture along the posterolateral surface of the spleen (Fig. 3) with high attenuation fluid in the perisplenic region and pelvic cavity suggestive of hemoperitoneum, which was subsequently confirmed on abdominocentesis. The radiologic features were consistent with peliosis spleen with rupture leading to hemoperitoneum. Liver and other abdominal viscera were normal. The patient was put on ventilator as she developed type 1 respiratory failure due to adult respiratory distress syndrome, which progressively worsened, and she died the next day of admission. Autopsy was denied by the relatives. Peliosis is a rare entity of unknown etiopathogenesis characterized by multiple cyst-like, blood-filled cavities predominantly in the organs of mononuclear phagocytic system (liver, spleen, bone marrow, and lymph nodes) [1]. Splenic peliosis usually occurs in conjunction with peliosis hepatis [2,3], and only 41 cases of isolated splenic peliosis have been reported in literature [4] with 10 cases of spontaneous rupture [2,5-9]. The peliosis is known to be associated with tuberculosis; hematological malignancies such as leukemia and lymphoma, AIDS, and posttransplant immunodeficiency; intravenous drug abuse; and chronic alcoholism and in conjunction with the intake of oral contraceptives or steroid [1,8,10]. The precise mechanism in development of peliosis is still unknown and hypothesized to be related to damage to the sinusoidal barrier, which may be postinfection, humoral immunity mediated or increased circulating levels of
Fig. 1. Axial CECT image showing multiple round to oval hypodense lesions in the spleen (arrows) with perisplenic fluid (hemoperitoneum).
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A. Lal et al. / American Journal of Emergency Medicine 32 (2014) 194.e3–194.e4
Fig. 2. Magnified CECT axial view of the spleen showing fluid-fluid levels (arrows) within the splenic lesions. Note dependent high density in most of the lesions due to hematocrit effect.
vascular endothelial growth factor [10-12]. The vascular endothelial growth factor levels are well known to be raised post viperine bite [13] and, in the indexed case, might have resulted in peliosis. Peliosis, otherwise, is a clinically silent condition until it ruptures where it manifests as an acute abdomen and life-threatening hemorrhage [7,8]. The clinical significance of this entity is as follows: (a) propensity to rupture spontaneously or with trivial trauma; (b) identification of underlying cause; and (c) resolution, although rare, after discontinuation of culprit medication/condition. Diagnosis of peliosis is usually established at histopathologic examination of resected organ or at autopsy; however, if imaging finding is typical, then preoperative or premorbid diagnosis can be made [3,10,14]. Ultrasound feature of peliosis spleen are nonspecific; however, on CT scans, it manifests as multiple small, well-defined hypoattenuating cyst-like lesions that demonstrate significant enhancement with loss of definition of the lobules and septa and do not exert mass effect on the splenic vessels coursing through them [8,10,14-16]. The nodules may show fluid-fluid levels, which are thought to reflect a hematocrit effect that shows enhancement in their dependent portions [3,15]. Calcification or extracapsular extension is not seen. However, if lesions rupture, subcapsular hematoma and intraperitoneal hemorrhage may be evident at CT [14,15]. Once a diagnosis of peliosis spleen is established on imaging, then one should search for its presence in other organs especially the liver and avoid interventional procedures
Fig. 3. Axial CECT at the level of superior pole of left kidney shows rupture of spleen along the posterolateral surface (arrow). Note the high density perisplenic fluid (asterisk), which was confirmed on abdominocentesis to be hemoperitoneum.
such as biopsy, which can precipitate iatrogenic rupture and lifethreatening hemorrhage [8]. The treatment of ruptured peliosis spleen is splenectomy if recognized promptly; however, the role of splenectomy in otherwise preserved patients is not known [8,17]. Our case was unique because association of peliosis with snake bite has not been reported previously. The temporal course of events in our case was consistent with the diagnosis of disseminated intravascular coagulation leading to adult respiratory distress syndrome due to hematotoxic snake bite. The present case lacks the histologic evidence, as the autopsy was not performed; however, the CT imaging findings of blood-filled cystic spaces (fluid-fluid levels and hematocrit effect) within the spleen were consistent with the diagnosis of peliosis. There is unquestionable evidence of splenic rupture on CT scan, which has been corroborated with abdominocentesis. In conclusion, peliosis spleen, although rare, should be included in the list of differential diagnosis of cystic lesions of spleen in appropriate clinical setting. Its clinical significance lies in the potential of peliotic lesions on the surface of the involved organs to rupture and cause life-threatening hemorrhage. Anupam Lal MD Manphool Singhal MD Navneet Sharma MD 1 Ashish Bhalla MD 1 Niranjan Khandelwal MD Departments of Radio Diagnosis and Internal Medicine Post Graduate Institute of Medical Education and Research Chandigarh 160012, India E-mail address: [email protected] 1 Departments of Radio Diagnosis and Internal Medicine http://dx.doi.org/10.1016/j.ajem.2013.09.021 References [1] Tsokos M, Erbersdobler A. Pathology of peliosis. Forensic Sci Int 2005;149:25–33. [2] Celebrezze Jr JP, Cottrell DJ, Williams GB. Spontaneous splenic rupture due to isolated splenic peliosis. South Med J 1998;91:763–4. [3] Tada T, Wakabayashi T, Kishimoto H. Peliosis of the spleen. Am J Clin Pathol 1983;79:708–13. [4] Abo D, Terae S, Fukasawa Y, Seki H, Hasegawa Y, Sakuhara Y, et al. Splenic peliosis mimicking a solid tumor: finding in multimodalities. J Comput Assist Tomogr 2008;32:890–2. [5] Etzion Y, Benharroch D, Saidel M, Riesenberg K, Gilad J, Schlaeffer F. Atraumatic rupture of the spleen associated with hemophagocytic syndrome and isolated splenic peliosis. Case report. APMIS 2005;113:555–7. [6] Tsokos M, Puschel K. Isolated peliosis of the spleen: report of 2 autopsy cases. Am J Forensic Med Pathol 2004;25:251–4. [7] Lo CM. A case of splenic peliosis associated with pathologic rupture. Hong Kong J Emerg Med 2007;14:174–8. [8] Lashbrook DJ, James RW, Phillips AJ, Holbrook AG, Agombar AC. Splenic peliosis with spontaneous splenic rupture: report of two cases. BMC Surg 2006;26(6):9. [9] Davidson J, Tung K. Splenic peliosis: an unusual entity. Br J Radiol 2010;83:e126–8. [10] Arai S, Asai T, Uozaki H, Hangaishi A, Kanda Y, Motokura T, et al. Int J Hematol 2007;86:329–32. [11] Leong SS, Cazen RA, Yu GS, LeFevre L, Carson JW. Abdominal visceral peliosis associated with bacillary angiomatosis. Ultrastructural evidence of endothelial destruction by bacilli. Arch Pathol Lab Med 1992;116:866–71. [12] Gugger M, Gebbers JO. Peliosis of the spleen: an immune-complex disease? Histopathology 1998;33:387–9. [13] de Azevedo IL Junqueira, Farsky SH, Oliveira ML, Ho PL. Molecular cloning and expression of a functional snake venom vascular endothelium growth factor (VEGF) from the Bothrops insularis pit viper. A new member of the VEGF family of proteins. J Biol Chem 2001;276:39836–42. [14] Abbot R, Levy A, Aguilera N, Luis G, Thompson W. Primary vascular neoplasms of the spleen: radiologic-pathologic correlation. Radiographics 2004;24:1137–63. [15] Cochrane LB, Freson M. Peliosis of the spleen. Gastrointest Radiol 1991;16:83–4. [16] Shimono T, Yamaoka T, Nishimura K, et al. Peliosis of the spleen: splenic rupture with intraperitoneal hemorrhage. Abdom Imaging 1998;23:201–2. [17] Kohr RM, Haendiges M, Taube RR. Peliosis of the spleen: a rare cause of spontaneous splenic rupture with surgical implications. Am Surg 1993;59:197–9.
Contents lists available at ScienceDirect
American Journal of Emergency Medicine journal homepage: www.elsevier.com/locate/ajem
Case Report
Isolated splenic peliosis with spontaneous rupture after a viperine bite☆,☆☆ Abstract Isolated splenic peliosis is an extremely uncommon condition that can present with atraumatic rupture and potential fatal outcome. We here report 1 such case that developed after a viperine bite in a 21-year-old woman. The case highlights the diagnostic findings on computed tomographic (CT) scan and its potential complications. Peliosis is a rare entity of unknown etiopathogenesis characterized by multiple cyst-like, blood-filled cavities predominantly in the mononuclear phagocytic system (liver, spleen, bone marrow, and lymph nodes) [1]. Splenic peliosis usually occurs in conjunction with peliosis hepatis [2,3]. Isolated cases of peliosis spleen are extremely uncommon, and until yet, only 41 cases have been reported in literature [4], of which only 10 patients presented with spontaneous rupture [2,59]. Here, we report a case of isolated peliosis spleen apparently due to a viperine bite leading to spontaneous rupture. To the best of our knowledge, this association has never been reported in the past. A 21-year-old unmarried woman, a household worker with history of viperine bite (the diagnosis of viperine bite was established based on clinical presentation and the morphological description of the snake), 5 days back was referred to our institute with severe pain and swelling of the right lower limb associated with painful abdominal distension. On clinical examination, she had marked pallor and ecchymotic patches on the right lower limb, around the left breast and left shoulder. The patient had marked pallor, but there was no cyanosis, clubbing, or lymphadenopathy. Blood pressure was 100/70 mm Hg; pulse rate, 106 per minute; and respiratory rate, 28 per minute. The chest radiograph showed a few patches of peripheral consolidations in both lungs. Laboratory investigations revealed hemoglobin 5.5 gm%, TLC 7000/mm 3, DLC N58L35M4E3B0, platelet counts 37 000/mm 3, random blood sugar 184 mg%, blood urea 33 mg%, serum creatinine1.0 mg%, serum Na 135, serum K 4.4, bilirubin 1.4 (conjugated 0.7); coagulation profile revealed prothrombin time of 25 seconds (control, 10 seconds) and activated partial thromboplastin time of 29 seconds (control, 25 seconds). Fibrin degradation products were found positive. Patient was managed with intravenous fluids, blood, antibiotics, and polyvalent antisnake venom. Ultrasound abdomen revealed splenomegaly with multiple illdefined hypoechoic lesions and presence of fluid in perisplenic area and in pelvis. A contrast-enhanced CT (CECT) (4-Slice Light Speed QX/I Plus; GE Healthcare, Milwaukee, WI) of abdomen was performed. The data acquisition was done in the porto-venous phase (65-second delay), which revealed enlarged spleen with
☆ Grant support: Nil. ☆☆ Conflicts of interest: Nil. 0735-6757/$ – see front matter © 2013 Elsevier Inc. All rights reserved.
multiple intrasplenic cysts of varying sizes with some of them showing high-density fluid levels suggestive of hemorrhage (Figs. 1 and 2). There was suggestion of rupture along the posterolateral surface of the spleen (Fig. 3) with high attenuation fluid in the perisplenic region and pelvic cavity suggestive of hemoperitoneum, which was subsequently confirmed on abdominocentesis. The radiologic features were consistent with peliosis spleen with rupture leading to hemoperitoneum. Liver and other abdominal viscera were normal. The patient was put on ventilator as she developed type 1 respiratory failure due to adult respiratory distress syndrome, which progressively worsened, and she died the next day of admission. Autopsy was denied by the relatives. Peliosis is a rare entity of unknown etiopathogenesis characterized by multiple cyst-like, blood-filled cavities predominantly in the organs of mononuclear phagocytic system (liver, spleen, bone marrow, and lymph nodes) [1]. Splenic peliosis usually occurs in conjunction with peliosis hepatis [2,3], and only 41 cases of isolated splenic peliosis have been reported in literature [4] with 10 cases of spontaneous rupture [2,5-9]. The peliosis is known to be associated with tuberculosis; hematological malignancies such as leukemia and lymphoma, AIDS, and posttransplant immunodeficiency; intravenous drug abuse; and chronic alcoholism and in conjunction with the intake of oral contraceptives or steroid [1,8,10]. The precise mechanism in development of peliosis is still unknown and hypothesized to be related to damage to the sinusoidal barrier, which may be postinfection, humoral immunity mediated or increased circulating levels of
Fig. 1. Axial CECT image showing multiple round to oval hypodense lesions in the spleen (arrows) with perisplenic fluid (hemoperitoneum).
194.e4
A. Lal et al. / American Journal of Emergency Medicine 32 (2014) 194.e3–194.e4
Fig. 2. Magnified CECT axial view of the spleen showing fluid-fluid levels (arrows) within the splenic lesions. Note dependent high density in most of the lesions due to hematocrit effect.
vascular endothelial growth factor [10-12]. The vascular endothelial growth factor levels are well known to be raised post viperine bite [13] and, in the indexed case, might have resulted in peliosis. Peliosis, otherwise, is a clinically silent condition until it ruptures where it manifests as an acute abdomen and life-threatening hemorrhage [7,8]. The clinical significance of this entity is as follows: (a) propensity to rupture spontaneously or with trivial trauma; (b) identification of underlying cause; and (c) resolution, although rare, after discontinuation of culprit medication/condition. Diagnosis of peliosis is usually established at histopathologic examination of resected organ or at autopsy; however, if imaging finding is typical, then preoperative or premorbid diagnosis can be made [3,10,14]. Ultrasound feature of peliosis spleen are nonspecific; however, on CT scans, it manifests as multiple small, well-defined hypoattenuating cyst-like lesions that demonstrate significant enhancement with loss of definition of the lobules and septa and do not exert mass effect on the splenic vessels coursing through them [8,10,14-16]. The nodules may show fluid-fluid levels, which are thought to reflect a hematocrit effect that shows enhancement in their dependent portions [3,15]. Calcification or extracapsular extension is not seen. However, if lesions rupture, subcapsular hematoma and intraperitoneal hemorrhage may be evident at CT [14,15]. Once a diagnosis of peliosis spleen is established on imaging, then one should search for its presence in other organs especially the liver and avoid interventional procedures
Fig. 3. Axial CECT at the level of superior pole of left kidney shows rupture of spleen along the posterolateral surface (arrow). Note the high density perisplenic fluid (asterisk), which was confirmed on abdominocentesis to be hemoperitoneum.
such as biopsy, which can precipitate iatrogenic rupture and lifethreatening hemorrhage [8]. The treatment of ruptured peliosis spleen is splenectomy if recognized promptly; however, the role of splenectomy in otherwise preserved patients is not known [8,17]. Our case was unique because association of peliosis with snake bite has not been reported previously. The temporal course of events in our case was consistent with the diagnosis of disseminated intravascular coagulation leading to adult respiratory distress syndrome due to hematotoxic snake bite. The present case lacks the histologic evidence, as the autopsy was not performed; however, the CT imaging findings of blood-filled cystic spaces (fluid-fluid levels and hematocrit effect) within the spleen were consistent with the diagnosis of peliosis. There is unquestionable evidence of splenic rupture on CT scan, which has been corroborated with abdominocentesis. In conclusion, peliosis spleen, although rare, should be included in the list of differential diagnosis of cystic lesions of spleen in appropriate clinical setting. Its clinical significance lies in the potential of peliotic lesions on the surface of the involved organs to rupture and cause life-threatening hemorrhage. Anupam Lal MD Manphool Singhal MD Navneet Sharma MD 1 Ashish Bhalla MD 1 Niranjan Khandelwal MD Departments of Radio Diagnosis and Internal Medicine Post Graduate Institute of Medical Education and Research Chandigarh 160012, India E-mail address: [email protected] 1 Departments of Radio Diagnosis and Internal Medicine http://dx.doi.org/10.1016/j.ajem.2013.09.021 References [1] Tsokos M, Erbersdobler A. Pathology of peliosis. Forensic Sci Int 2005;149:25–33. [2] Celebrezze Jr JP, Cottrell DJ, Williams GB. Spontaneous splenic rupture due to isolated splenic peliosis. South Med J 1998;91:763–4. [3] Tada T, Wakabayashi T, Kishimoto H. Peliosis of the spleen. Am J Clin Pathol 1983;79:708–13. [4] Abo D, Terae S, Fukasawa Y, Seki H, Hasegawa Y, Sakuhara Y, et al. Splenic peliosis mimicking a solid tumor: finding in multimodalities. J Comput Assist Tomogr 2008;32:890–2. [5] Etzion Y, Benharroch D, Saidel M, Riesenberg K, Gilad J, Schlaeffer F. Atraumatic rupture of the spleen associated with hemophagocytic syndrome and isolated splenic peliosis. Case report. APMIS 2005;113:555–7. [6] Tsokos M, Puschel K. Isolated peliosis of the spleen: report of 2 autopsy cases. Am J Forensic Med Pathol 2004;25:251–4. [7] Lo CM. A case of splenic peliosis associated with pathologic rupture. Hong Kong J Emerg Med 2007;14:174–8. [8] Lashbrook DJ, James RW, Phillips AJ, Holbrook AG, Agombar AC. Splenic peliosis with spontaneous splenic rupture: report of two cases. BMC Surg 2006;26(6):9. [9] Davidson J, Tung K. Splenic peliosis: an unusual entity. Br J Radiol 2010;83:e126–8. [10] Arai S, Asai T, Uozaki H, Hangaishi A, Kanda Y, Motokura T, et al. Int J Hematol 2007;86:329–32. [11] Leong SS, Cazen RA, Yu GS, LeFevre L, Carson JW. Abdominal visceral peliosis associated with bacillary angiomatosis. Ultrastructural evidence of endothelial destruction by bacilli. Arch Pathol Lab Med 1992;116:866–71. [12] Gugger M, Gebbers JO. Peliosis of the spleen: an immune-complex disease? Histopathology 1998;33:387–9. [13] de Azevedo IL Junqueira, Farsky SH, Oliveira ML, Ho PL. Molecular cloning and expression of a functional snake venom vascular endothelium growth factor (VEGF) from the Bothrops insularis pit viper. A new member of the VEGF family of proteins. J Biol Chem 2001;276:39836–42. [14] Abbot R, Levy A, Aguilera N, Luis G, Thompson W. Primary vascular neoplasms of the spleen: radiologic-pathologic correlation. Radiographics 2004;24:1137–63. [15] Cochrane LB, Freson M. Peliosis of the spleen. Gastrointest Radiol 1991;16:83–4. [16] Shimono T, Yamaoka T, Nishimura K, et al. Peliosis of the spleen: splenic rupture with intraperitoneal hemorrhage. Abdom Imaging 1998;23:201–2. [17] Kohr RM, Haendiges M, Taube RR. Peliosis of the spleen: a rare cause of spontaneous splenic rupture with surgical implications. Am Surg 1993;59:197–9.
