TRAUMATIC PSEUDOCYST OF THE SPLEEN Timothy C. Simmons, MD Inglewood, California
A rare case of a patient with a large multiloculated symptomatic pseudocyst of the spleen is presented. The splenic pseudocyst was diagnosed preoperatively by ultrasonography and computerized tomography of the abdomen. Pathologic spleen sections demonstrated benign splenic parenchyma overlying a fibrous cyst wall, the inner lining of which contained no epithelial membrane. Key words * splenic cysts * splenectomy * echinococcal infection * hydatid cyst * abdominal trauma
Splenic cysts are rare compared with cysts involving other abdominal viscera. Pemberton, in a discussion of a paper by Roberson,l noted a 0.5% incidence of splenic cysts in 800 splenectomies. We report a patient with a splenic cyst referred to us for consultation in August 1987. A review of the literature is also presented.
CASE REPORT A 29-year-old woman with a history of blunt midabdomen trauma (sustained during an automobile accident in June 1983), peptic ulcer disease, and functional bowel syndrome was referred for gastrointestinal consultation on August 11, 1987. Her initial symptoms were increasing right and left upper quadrant abdominal pain and low back pain. She had no
From the West Gastroenterology Group, Centinela Valley Hospital, Inglewood, California. Requests for reprints should be addressed to Dr TC. Simmons, 301 North Prairie Ave, Suite 415, Inglewood, CA 90301. JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 82, NO. 10
complaints of nausea, vomiting, dysphagia, diarrhea, or constipation. The physical examination disclosed an easily palpable spleen edge 7 cm below the left costal margin. The spleen was not tender to palpation. The complete blood count, platelet count, prothrombin time, partial thromboplastin time, chemistry panel, and amylase were within normal limits. Upper gastrointestinal endoscopy revealed a small hiatal hernia and extrinsic pressure at the greater curvature of the stomach body and fundus. No gastric or duodenal mucosal defects were detected. An abdominal ultrasound demonstrated a 13.2 x 12.5 x 10.8 cm multiseptated, cystic mass in the left upper quadrant of the abdomen, which appeared intimately related to the spleen. The mass also caused inferior left kidney displacement. An abdominal computerized tomography (CT) scan (Figure 1) confirmed the ultrasonographic suspicion of a large, septated splenic cyst displacing the left kidney and pancreas and compressing the greater curvature of the stomach. Recommended surgical excision of the splenic cyst was postponed because cyst maturity was not established, and the patient's gastrointestinal symptoms were not suggestive of recent cyst enlargement. During February 1988, marked exacerbation of left upper quadrant abdominal pain and low back pain led to hospital admission. An easily palpable, tender, left upper quadrant mass was noted. Serologic markers for echinococcal infection were negative. A second abdominal CT scan showed minimal splenic cyst enlargement compared with that of August 1987. Splenectomy was performed in February 1988. A 658 gm, 17 x 13 x 10 cm spleen was removed. The splenic parenchyma was distorted by a large fluid-filled 727
PSEUDOCYST OF THE SPLEEN
.. S
~~~~~~~:r
W_ v
I,'v Figure 1. Computerized tomography scan of the abdomen demonstrating a large muktisep. tated splenic cyst.
Figure 3. Photomicrograph of a pathologic section of the spleen demonstrating benign splenic parenchyma (S) overlying a cyst wall consisiting of a thickened fibrous plaque material (arrow). The cyst wall is lacking an epithe.. lial membrane. fibrinous debris containing numerous cholesterol clefts.
graph of spleen (surgical speciFigure 2. men) showing a 10 cm cyst at its inferior border.
cyst measuring 10 cm in its greatest dimension (Figure 2). The cyst had a smooth wall, contained 100 gm of brown fibrinous debris, and pushed the normalappearing splenic parenchyma to one side. On sectioning, the inner cyst wall appeared relatively smooth, except for adherent fibrinous clot material. Rep'resentative pathologic spleen sections (Figure 3) demonstrated benign splenic parenchyma, overlying a cyst wall fibrous plaque material, the inner of a thickmacriophgs consisting andiaglrge a)dmounstraof hiemptoosiein-sladen lining of which contained no epithelial membrane. The luminal surface of the splenic cyst was lined by
DISCUSSION Splenic cysts are classified in two main categories2: true cysts which contain an epithelial lining and false cysts (pseudocysts) which lack an epithelial lining. Excluding echinococcal (hydatid) cysts, true splenic cysts are further divided into congenital and neoplastic categories. Congenital splenic cysts evolve as a result of peritoneal endothelial cell entrapment during early development. The entrapped cells proliferate; secretions of serous cell fluid form the contents of congenital splenic cysts.2 The diverse group of benign neoplastic splenic cyst include hemangiomas, dermoid, epidermoid, and lymphangiomatous tumors.3 Malignant splenic cysts have not been reported. Splenic pseudocysts develop almost exclusively as a direct, usually delayed, complication of splenic trauma. The time between splenic trauma and pseudocyst diagnosis can vary from a few months to several
years.4 Thus, the 4 year time interval between 728
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 82, NO. 10
PSEUDOCYST OF THE SPLEEN
abdominal trauma and pseudocyst discovery in the patient reported here is not uncommon. During the period between trauma and cyst development, an unruptured splenic hematoma goes through phases of encapsulation, liquefaction, and absorption.5 After these processes are complete, a residue of serous fluid, cholesterol crystals, and fat-laden macrophages form the contents of the pseudocyst. The pseudocyst wall is composed of thick, fibrous plaque-like material, which has no epithelial membrane lining its inner surface. The symptoms of splenic cysts are protean. Small splenic cysts are usually asymptomatic. Pain at the left hypochondrium is the most frequent symptom of large splenic cysts, occurring in 40% of patients with this anomaly.6 Large splenic cysts produce symptoms by displacing or compressing adjacent intra-abdominal organs; for example, stomach compression can produce abdominal pain, nausea, and vomiting. Displacement of the left hemidiaphragm by a large splenic cyst can result in dyspnea, atelectasis of the left lower lobe of the lungs, recurrent pneumonia, and pleurisy.2 In addition, Garvey et a14 noted that extrinsic pressure on the intra-abdominal esophagus and esophagogastric junction by an enlarging splenic cyst can produce dysphagia. Extrinsic compression of the splenic flexure of the colon by a large splenic cyst can produce constipation and a dragging sensation in the left hypogastrium. A presumptive diagnosis of splenic pseudocyst can be made when a patient with a remote history of blunt abdominal trauma presents with a palpable left upper quadrant mass. However, distinguishing splenic cysts from cystic tumors of other intra-abdominal viscera can be difficult. Calcified splenic cysts are, on occasion, demonstrated on plain radiographs of the abdomen. An upper gastrointestinal series shows anterior gastric displacement and a greater curvature extrinsic pressure stomach defect in the majority of patients with large splenic cysts. Abdominal ultrasound is a noninvasive diagnostic test which is sensitive in separating cystic from solid intra-abdominal masses. However, as shown in the current case, ultrasound is not as specific as the CT
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 82, NO. 10
scan in discerning the abdominal viscera from which the cyst arises. Hydatid cysts (Echinococcus granulosus) are rare.7 A history of exposure to definitive animal hosts, typically in areas endemic for echinococcosis, is noted in the majority of cases.8 In some cases, cyst wall calcification can be seen on plain abdomen radiographs. Most patients with spleen hydatid disease develop positive serologic markers for echinococcal infection. Serologic tests for echinococcal infection were negative in the patient reported here. As minimal abdominal trauma can result in spontaneous rupture of a large splenic cyst, surgical intervention is usually warranted, especially when symptoms are present. Splenectomy is the procedure of choice. However, with resurgence of interest in the role of the spleen in immunology, spleen salvage operations have also become popular.9 Nonetheless, because most of the spleen had been replaced by the cyst, salvage was not possible in the patient reported here. Following total splenectomy, she had an uneventful course and remained asymptomatic at a 6 month follow-up. Literature Cited 1. Roberson F. Solitary cysts of the spleen. Ann Surg. 1940;1 1 1:848-850. 2. Martin JW. Congenital splenic cysts. Am J Surg. 1 958;96:302-308. 3. Fowler RH. Collective review: nonparasitic benign cystic tumors of the spleen. Int Abst Surg. 1953;96:209-227. 4. Garvey JW, Peyser I, Delany HM. Traumatic cyst of the spleen: an unusual presentation. J Trauma. 1974;1 1:974-979. 5. Langley JR, Poole RW, Jackson N. Splenic cyst: report of a case with review of the literature. American Surgeon. 1977;43:171-174. 6. Qureshi MA, Hafner CD. Clinical manifestations of splenic cysts: study of 75 cases. American Surgeon. 1965;31:605-608. 7. Amir-Jahed AK, Fardin R, Farzad A, Bakshandeh K. Clinical echinococcosis. Ann Surg. 1975;1 82:541-546. 8. Schantz PM. Larval cestodiasis: cystic hydatid disease (Echinococcus granulosus). In: Hoeprich PD, ed. Infectious diseases. 3rd ed. Philadelphia, PA: Harper & Row; 1983:769779. 9. Brown JJ, Bynoe RP, Greene FL, Burke ML. Splenic salvage techniques in the management of pseudocysts of the spleen. South Med J. 1986;79:710-71 1.
729
A rare case of a patient with a large multiloculated symptomatic pseudocyst of the spleen is presented. The splenic pseudocyst was diagnosed preoperatively by ultrasonography and computerized tomography of the abdomen. Pathologic spleen sections demonstrated benign splenic parenchyma overlying a fibrous cyst wall, the inner lining of which contained no epithelial membrane. Key words * splenic cysts * splenectomy * echinococcal infection * hydatid cyst * abdominal trauma
Splenic cysts are rare compared with cysts involving other abdominal viscera. Pemberton, in a discussion of a paper by Roberson,l noted a 0.5% incidence of splenic cysts in 800 splenectomies. We report a patient with a splenic cyst referred to us for consultation in August 1987. A review of the literature is also presented.
CASE REPORT A 29-year-old woman with a history of blunt midabdomen trauma (sustained during an automobile accident in June 1983), peptic ulcer disease, and functional bowel syndrome was referred for gastrointestinal consultation on August 11, 1987. Her initial symptoms were increasing right and left upper quadrant abdominal pain and low back pain. She had no
From the West Gastroenterology Group, Centinela Valley Hospital, Inglewood, California. Requests for reprints should be addressed to Dr TC. Simmons, 301 North Prairie Ave, Suite 415, Inglewood, CA 90301. JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 82, NO. 10
complaints of nausea, vomiting, dysphagia, diarrhea, or constipation. The physical examination disclosed an easily palpable spleen edge 7 cm below the left costal margin. The spleen was not tender to palpation. The complete blood count, platelet count, prothrombin time, partial thromboplastin time, chemistry panel, and amylase were within normal limits. Upper gastrointestinal endoscopy revealed a small hiatal hernia and extrinsic pressure at the greater curvature of the stomach body and fundus. No gastric or duodenal mucosal defects were detected. An abdominal ultrasound demonstrated a 13.2 x 12.5 x 10.8 cm multiseptated, cystic mass in the left upper quadrant of the abdomen, which appeared intimately related to the spleen. The mass also caused inferior left kidney displacement. An abdominal computerized tomography (CT) scan (Figure 1) confirmed the ultrasonographic suspicion of a large, septated splenic cyst displacing the left kidney and pancreas and compressing the greater curvature of the stomach. Recommended surgical excision of the splenic cyst was postponed because cyst maturity was not established, and the patient's gastrointestinal symptoms were not suggestive of recent cyst enlargement. During February 1988, marked exacerbation of left upper quadrant abdominal pain and low back pain led to hospital admission. An easily palpable, tender, left upper quadrant mass was noted. Serologic markers for echinococcal infection were negative. A second abdominal CT scan showed minimal splenic cyst enlargement compared with that of August 1987. Splenectomy was performed in February 1988. A 658 gm, 17 x 13 x 10 cm spleen was removed. The splenic parenchyma was distorted by a large fluid-filled 727
PSEUDOCYST OF THE SPLEEN
.. S
~~~~~~~:r
W_ v
I,'v Figure 1. Computerized tomography scan of the abdomen demonstrating a large muktisep. tated splenic cyst.
Figure 3. Photomicrograph of a pathologic section of the spleen demonstrating benign splenic parenchyma (S) overlying a cyst wall consisiting of a thickened fibrous plaque material (arrow). The cyst wall is lacking an epithe.. lial membrane. fibrinous debris containing numerous cholesterol clefts.
graph of spleen (surgical speciFigure 2. men) showing a 10 cm cyst at its inferior border.
cyst measuring 10 cm in its greatest dimension (Figure 2). The cyst had a smooth wall, contained 100 gm of brown fibrinous debris, and pushed the normalappearing splenic parenchyma to one side. On sectioning, the inner cyst wall appeared relatively smooth, except for adherent fibrinous clot material. Rep'resentative pathologic spleen sections (Figure 3) demonstrated benign splenic parenchyma, overlying a cyst wall fibrous plaque material, the inner of a thickmacriophgs consisting andiaglrge a)dmounstraof hiemptoosiein-sladen lining of which contained no epithelial membrane. The luminal surface of the splenic cyst was lined by
DISCUSSION Splenic cysts are classified in two main categories2: true cysts which contain an epithelial lining and false cysts (pseudocysts) which lack an epithelial lining. Excluding echinococcal (hydatid) cysts, true splenic cysts are further divided into congenital and neoplastic categories. Congenital splenic cysts evolve as a result of peritoneal endothelial cell entrapment during early development. The entrapped cells proliferate; secretions of serous cell fluid form the contents of congenital splenic cysts.2 The diverse group of benign neoplastic splenic cyst include hemangiomas, dermoid, epidermoid, and lymphangiomatous tumors.3 Malignant splenic cysts have not been reported. Splenic pseudocysts develop almost exclusively as a direct, usually delayed, complication of splenic trauma. The time between splenic trauma and pseudocyst diagnosis can vary from a few months to several
years.4 Thus, the 4 year time interval between 728
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 82, NO. 10
PSEUDOCYST OF THE SPLEEN
abdominal trauma and pseudocyst discovery in the patient reported here is not uncommon. During the period between trauma and cyst development, an unruptured splenic hematoma goes through phases of encapsulation, liquefaction, and absorption.5 After these processes are complete, a residue of serous fluid, cholesterol crystals, and fat-laden macrophages form the contents of the pseudocyst. The pseudocyst wall is composed of thick, fibrous plaque-like material, which has no epithelial membrane lining its inner surface. The symptoms of splenic cysts are protean. Small splenic cysts are usually asymptomatic. Pain at the left hypochondrium is the most frequent symptom of large splenic cysts, occurring in 40% of patients with this anomaly.6 Large splenic cysts produce symptoms by displacing or compressing adjacent intra-abdominal organs; for example, stomach compression can produce abdominal pain, nausea, and vomiting. Displacement of the left hemidiaphragm by a large splenic cyst can result in dyspnea, atelectasis of the left lower lobe of the lungs, recurrent pneumonia, and pleurisy.2 In addition, Garvey et a14 noted that extrinsic pressure on the intra-abdominal esophagus and esophagogastric junction by an enlarging splenic cyst can produce dysphagia. Extrinsic compression of the splenic flexure of the colon by a large splenic cyst can produce constipation and a dragging sensation in the left hypogastrium. A presumptive diagnosis of splenic pseudocyst can be made when a patient with a remote history of blunt abdominal trauma presents with a palpable left upper quadrant mass. However, distinguishing splenic cysts from cystic tumors of other intra-abdominal viscera can be difficult. Calcified splenic cysts are, on occasion, demonstrated on plain radiographs of the abdomen. An upper gastrointestinal series shows anterior gastric displacement and a greater curvature extrinsic pressure stomach defect in the majority of patients with large splenic cysts. Abdominal ultrasound is a noninvasive diagnostic test which is sensitive in separating cystic from solid intra-abdominal masses. However, as shown in the current case, ultrasound is not as specific as the CT
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 82, NO. 10
scan in discerning the abdominal viscera from which the cyst arises. Hydatid cysts (Echinococcus granulosus) are rare.7 A history of exposure to definitive animal hosts, typically in areas endemic for echinococcosis, is noted in the majority of cases.8 In some cases, cyst wall calcification can be seen on plain abdomen radiographs. Most patients with spleen hydatid disease develop positive serologic markers for echinococcal infection. Serologic tests for echinococcal infection were negative in the patient reported here. As minimal abdominal trauma can result in spontaneous rupture of a large splenic cyst, surgical intervention is usually warranted, especially when symptoms are present. Splenectomy is the procedure of choice. However, with resurgence of interest in the role of the spleen in immunology, spleen salvage operations have also become popular.9 Nonetheless, because most of the spleen had been replaced by the cyst, salvage was not possible in the patient reported here. Following total splenectomy, she had an uneventful course and remained asymptomatic at a 6 month follow-up. Literature Cited 1. Roberson F. Solitary cysts of the spleen. Ann Surg. 1940;1 1 1:848-850. 2. Martin JW. Congenital splenic cysts. Am J Surg. 1 958;96:302-308. 3. Fowler RH. Collective review: nonparasitic benign cystic tumors of the spleen. Int Abst Surg. 1953;96:209-227. 4. Garvey JW, Peyser I, Delany HM. Traumatic cyst of the spleen: an unusual presentation. J Trauma. 1974;1 1:974-979. 5. Langley JR, Poole RW, Jackson N. Splenic cyst: report of a case with review of the literature. American Surgeon. 1977;43:171-174. 6. Qureshi MA, Hafner CD. Clinical manifestations of splenic cysts: study of 75 cases. American Surgeon. 1965;31:605-608. 7. Amir-Jahed AK, Fardin R, Farzad A, Bakshandeh K. Clinical echinococcosis. Ann Surg. 1975;1 82:541-546. 8. Schantz PM. Larval cestodiasis: cystic hydatid disease (Echinococcus granulosus). In: Hoeprich PD, ed. Infectious diseases. 3rd ed. Philadelphia, PA: Harper & Row; 1983:769779. 9. Brown JJ, Bynoe RP, Greene FL, Burke ML. Splenic salvage techniques in the management of pseudocysts of the spleen. South Med J. 1986;79:710-71 1.
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