Clinical Review & Education
JAMA Surgery Clinical Challenge
Splenic Cyst Carlos A. Pelaez, MD; Meaghan F. Dehning, BS
Figure 1. A large splenic cyst with calcified capsule is present in the anterior aspect of the spleen, with a smaller cyst in the posterior aspect of the spleen, also with calcified capsule. Arrowheads indicate the location of the cysts. Under higher resolution, debris is noted within the larger cyst.
A woman in her 30s was referred for evaluation of a large splenic cyst identified on a computed tomographic (CT) scan. She had previously lived in Beijing, China. In 2011, she was evaluated in a Chinese clinic for abdominal bloating and moderate left upper quadrant pain; she was offered a splenectomy, but she declined and pursued a second opinion at a hospital in the United States in 2012. During that evaluation, the patient reported that she had run in areas with dog feces on the ground and had eaten sheep meat (notably, not organ meat); she denied consuming canine meat. Results of serologic tests for Echinococcus antibody IgG were negative. Results of a CT scan were remarkable for a 9.7 × 9.0 × 10.5-cm anterior cyst and a smaller posterior cyst measuring 4.6 × 4.7 × 3.8 cm, both with rim calcifications (Figure 1).
jamasurgery.com
WHAT IS YOUR DIAGNOSIS?
A. Congenital cyst B. Splenic abscess C. Cystic metastasis to the spleen D. Echinococcal disease
(Reprinted) JAMA Surgery April 2015 Volume 150, Number 4
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archsurg.jamanetwork.com/ by a Yale University User on 05/17/2015
365
Clinical Review & Education JAMA Surgery Clinical Challenge
Diagnosis D. Echinococcal disease
Discussion Echinococcal disease is seen in areas in which the intermediate host— sheep— and the definitive host— canines— are in close proximity. The highest prevalence of the disease is seen in South America, Asia, and parts of Africa; in the United States, it is most commonly found in immigrants. Echinococcus granulosus is a cestode that survives within an accidental host—humans, who become hosts through contact with infected canine feces or ingesting infected sheep organ meat. The cestode forms cysts with large fluid collections and septations; daughter cysts may also be present. The liver is the most common site of infestation (55%-70%), followed by the lungs and spleen (2.5%).1 The diseaseisoftenasymptomatic,andwhileinfectioninchildhoodiscommon,theslow-growingcestodeisoftendiagnosedinadulthood.When symptoms manifest, they are commonly caused by compression of adjacent structures; the presenting symptom is frequently a dull ache. Patients may also experience dyspepsia or constipation.1 Initial workup should include CT scan and serologic tests for echinococcal titers. Findings on CT scan supportive of echinococcal disease include a nonenhancing mass that may be cystic or solid. Following the death of the helminths, a calcified wall may develop (Figure 1). Serologic studies have a relatively high sensitivity and remain positive until 1 year after the organism has been cleared. However, there is a high false-negative rate in extrahepatic disease.2 Despite the availability of CT imaging and serologic studies, Moro and Schantz3 suggested that calcification, presence of daughter cysts, and patient history are useful in establishing a diagnosis. The importance of diagnosing and treating echinococcal disease is because of the possible complications, which include infection, rupture, and fistulization. Broadly, rupture of the cyst leads to potentially fatal anaphylaxis. Specifically, of concern in a large splenic cyst is the likelihood of traumatic rupture. Splenectomy has therefore become the criterion standard of treatment4; however, antihelminthic drugs are used as adjunctive therapy. Recently, spleen-preserving therapy has become increasingly common, especially in children. Spleen-preserving operations are reserved for solitary, small, and superficial cysts.5 While isolated splenic hydatidosis is uncommon, the spleen is the third most common location of echinococcal cysts, and recognizing the risk factors and presentation are paramount to diagnosing this disease and preventing potentially fatal complications (Figure 2). ARTICLE INFORMATION
Corresponding Author: Carlos A. Pelaez, MD, Department of Surgery, University of Iowa, 200 Hawkins Dr, 0091 RCP, Iowa City, IA 52242 ([email protected]). Section Editor: Pamela A. Lipsett, MD, MHPE.
366
In this patient, a CT scan revealed multiple cysts, which the literature reports are generally not amenable to laparoscopic removal because laparoscopic removal is reserved for small superficial cysts. 6 Similarly, the puncture, aspiration, injection, and reaspiration technique was not indicated because the serologic test results for echinococcal titers were negative. However, the US Centers for Disease Control and Prevention reports that false positives are common, especially if the cyst is located within the lungs, brain, or spleen.5 Simple antihelminthic treatment was deemed insufficient owing to the possibility of traumatic rupture and subsequent anaphylaxis. Because of strong suspicion for echinococcal disease and location and size of the cysts, the decision was made to proceed with open splenectomy. The patient was treated preoperatively with albendazole and praziquantel. Diphenhydramine and corticosteroids were in the operating room during the procedure in the event of rupture. The smaller cyst seen on CT scan was found to be an accessory spleen; this was also removed. Pathologic examination of the cysts showed no evidence of echinococcal disease; hence, the cyst was determined to be a primary epithelial cyst. The presence of a cystic structure within the accessory spleen, patient’s travel history, and echinococcal false-positive rate make inactive echinococcal disease a likely diagnosis.
REFERENCES
4. Makkar M, Gupta C, Singh DP, Kaur S, Mahajan N. Giant isolated splenic hydatidosis. Trop Parasitol. 2012;2(1):74-76.
1. Bhuiyan MJH, Siddiq AKMZ, Akhter L. Hydatid cyst of spleen. JAFMC Bangladesh. 2009;5(2):4445. doi:10.3329/jafmc.v5i2.4584.
5. Ammann RW, Eckert J. Cestodes: echinocococcus. Gastroenterol Clin North Am. 1996; 25(3):655-689.
2. Lightowlers MW, Gottstein B. Echinococcosis/ hydatidosis: antigens, immunological and molecular diagnosis. In: Thompson RCA, Lymbery AJ, eds. Echinococcus and Hydatid Disease. Wallingford, UK: CAB International; 1995:355-410.
6. Polat FR. Hydatid cyst: open or laproscopic approach? a retrospective analysis. Surg Laparosc Endosc Percutan Tech. 2012;22(3):264-266.
Conflict of Interest Disclosures: None reported.
Author Affiliations: Department of Surgery, University of Iowa, Iowa City (Pelaez); Roy J. and Lucille A. Carver College of Medicine, Iowa City, Iowa (Dehning).
Published Online: February 25, 2015. doi:10.1001/jamasurg.2014.852.
Figure 2. Spleen in situ after mobilization and lysis of adhesion. The white area over the anterior aspect of the spleen is the capsule of the larger cyst. The posterior cyst was identified after the vessels were divided. The spleen and cysts were removed en bloc without violating their capsule.
3. Moro P, Schantz PM. Echinococcosis: a review. Int J Infect Dis. 2009;13(2):125-133.
JAMA Surgery April 2015 Volume 150, Number 4 (Reprinted)
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archsurg.jamanetwork.com/ by a Yale University User on 05/17/2015
jamasurgery.com
JAMA Surgery Clinical Challenge
Splenic Cyst Carlos A. Pelaez, MD; Meaghan F. Dehning, BS
Figure 1. A large splenic cyst with calcified capsule is present in the anterior aspect of the spleen, with a smaller cyst in the posterior aspect of the spleen, also with calcified capsule. Arrowheads indicate the location of the cysts. Under higher resolution, debris is noted within the larger cyst.
A woman in her 30s was referred for evaluation of a large splenic cyst identified on a computed tomographic (CT) scan. She had previously lived in Beijing, China. In 2011, she was evaluated in a Chinese clinic for abdominal bloating and moderate left upper quadrant pain; she was offered a splenectomy, but she declined and pursued a second opinion at a hospital in the United States in 2012. During that evaluation, the patient reported that she had run in areas with dog feces on the ground and had eaten sheep meat (notably, not organ meat); she denied consuming canine meat. Results of serologic tests for Echinococcus antibody IgG were negative. Results of a CT scan were remarkable for a 9.7 × 9.0 × 10.5-cm anterior cyst and a smaller posterior cyst measuring 4.6 × 4.7 × 3.8 cm, both with rim calcifications (Figure 1).
jamasurgery.com
WHAT IS YOUR DIAGNOSIS?
A. Congenital cyst B. Splenic abscess C. Cystic metastasis to the spleen D. Echinococcal disease
(Reprinted) JAMA Surgery April 2015 Volume 150, Number 4
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archsurg.jamanetwork.com/ by a Yale University User on 05/17/2015
365
Clinical Review & Education JAMA Surgery Clinical Challenge
Diagnosis D. Echinococcal disease
Discussion Echinococcal disease is seen in areas in which the intermediate host— sheep— and the definitive host— canines— are in close proximity. The highest prevalence of the disease is seen in South America, Asia, and parts of Africa; in the United States, it is most commonly found in immigrants. Echinococcus granulosus is a cestode that survives within an accidental host—humans, who become hosts through contact with infected canine feces or ingesting infected sheep organ meat. The cestode forms cysts with large fluid collections and septations; daughter cysts may also be present. The liver is the most common site of infestation (55%-70%), followed by the lungs and spleen (2.5%).1 The diseaseisoftenasymptomatic,andwhileinfectioninchildhoodiscommon,theslow-growingcestodeisoftendiagnosedinadulthood.When symptoms manifest, they are commonly caused by compression of adjacent structures; the presenting symptom is frequently a dull ache. Patients may also experience dyspepsia or constipation.1 Initial workup should include CT scan and serologic tests for echinococcal titers. Findings on CT scan supportive of echinococcal disease include a nonenhancing mass that may be cystic or solid. Following the death of the helminths, a calcified wall may develop (Figure 1). Serologic studies have a relatively high sensitivity and remain positive until 1 year after the organism has been cleared. However, there is a high false-negative rate in extrahepatic disease.2 Despite the availability of CT imaging and serologic studies, Moro and Schantz3 suggested that calcification, presence of daughter cysts, and patient history are useful in establishing a diagnosis. The importance of diagnosing and treating echinococcal disease is because of the possible complications, which include infection, rupture, and fistulization. Broadly, rupture of the cyst leads to potentially fatal anaphylaxis. Specifically, of concern in a large splenic cyst is the likelihood of traumatic rupture. Splenectomy has therefore become the criterion standard of treatment4; however, antihelminthic drugs are used as adjunctive therapy. Recently, spleen-preserving therapy has become increasingly common, especially in children. Spleen-preserving operations are reserved for solitary, small, and superficial cysts.5 While isolated splenic hydatidosis is uncommon, the spleen is the third most common location of echinococcal cysts, and recognizing the risk factors and presentation are paramount to diagnosing this disease and preventing potentially fatal complications (Figure 2). ARTICLE INFORMATION
Corresponding Author: Carlos A. Pelaez, MD, Department of Surgery, University of Iowa, 200 Hawkins Dr, 0091 RCP, Iowa City, IA 52242 ([email protected]). Section Editor: Pamela A. Lipsett, MD, MHPE.
366
In this patient, a CT scan revealed multiple cysts, which the literature reports are generally not amenable to laparoscopic removal because laparoscopic removal is reserved for small superficial cysts. 6 Similarly, the puncture, aspiration, injection, and reaspiration technique was not indicated because the serologic test results for echinococcal titers were negative. However, the US Centers for Disease Control and Prevention reports that false positives are common, especially if the cyst is located within the lungs, brain, or spleen.5 Simple antihelminthic treatment was deemed insufficient owing to the possibility of traumatic rupture and subsequent anaphylaxis. Because of strong suspicion for echinococcal disease and location and size of the cysts, the decision was made to proceed with open splenectomy. The patient was treated preoperatively with albendazole and praziquantel. Diphenhydramine and corticosteroids were in the operating room during the procedure in the event of rupture. The smaller cyst seen on CT scan was found to be an accessory spleen; this was also removed. Pathologic examination of the cysts showed no evidence of echinococcal disease; hence, the cyst was determined to be a primary epithelial cyst. The presence of a cystic structure within the accessory spleen, patient’s travel history, and echinococcal false-positive rate make inactive echinococcal disease a likely diagnosis.
REFERENCES
4. Makkar M, Gupta C, Singh DP, Kaur S, Mahajan N. Giant isolated splenic hydatidosis. Trop Parasitol. 2012;2(1):74-76.
1. Bhuiyan MJH, Siddiq AKMZ, Akhter L. Hydatid cyst of spleen. JAFMC Bangladesh. 2009;5(2):4445. doi:10.3329/jafmc.v5i2.4584.
5. Ammann RW, Eckert J. Cestodes: echinocococcus. Gastroenterol Clin North Am. 1996; 25(3):655-689.
2. Lightowlers MW, Gottstein B. Echinococcosis/ hydatidosis: antigens, immunological and molecular diagnosis. In: Thompson RCA, Lymbery AJ, eds. Echinococcus and Hydatid Disease. Wallingford, UK: CAB International; 1995:355-410.
6. Polat FR. Hydatid cyst: open or laproscopic approach? a retrospective analysis. Surg Laparosc Endosc Percutan Tech. 2012;22(3):264-266.
Conflict of Interest Disclosures: None reported.
Author Affiliations: Department of Surgery, University of Iowa, Iowa City (Pelaez); Roy J. and Lucille A. Carver College of Medicine, Iowa City, Iowa (Dehning).
Published Online: February 25, 2015. doi:10.1001/jamasurg.2014.852.
Figure 2. Spleen in situ after mobilization and lysis of adhesion. The white area over the anterior aspect of the spleen is the capsule of the larger cyst. The posterior cyst was identified after the vessels were divided. The spleen and cysts were removed en bloc without violating their capsule.
3. Moro P, Schantz PM. Echinococcosis: a review. Int J Infect Dis. 2009;13(2):125-133.
JAMA Surgery April 2015 Volume 150, Number 4 (Reprinted)
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archsurg.jamanetwork.com/ by a Yale University User on 05/17/2015
jamasurgery.com
![[Primary splenic cyst].](/assets/img/hold.png)
