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Year : 2015  |  Volume : 1  |  Issue : 3  |  Page : 157-159

Preoperatively diagnosed isolated hydatid cyst of pancreatic head with triad of obstructive jaundice, left-sided portal hypertension and chronic pancreatitis

1 Department of Radiodiagnosis, Government Medical College, Kota, Rajasthan, India
2 Department of Radiology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India

Date of Web Publication30-Sep-2015

Correspondence Address:
Pradeep Choudhary
Department of Radiodiagnosis, Government Medical College, Kota, Rajasthan
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Source of Support: Nil., Conflict of Interest: There are no conflicts of interest.

DOI: 10.4103/2394-7438.166297

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We report a rare case of young female with isolated pancreatic head hydatid cyst causing obstructive jaundice, left-sided portal hypertension, and chronic pancreatitis.

Keywords: Hydatid cyst, jaundice, pancreatitis, splenomegaly

How to cite this article:
Choudhary P, Khokhar HV, Aswani Y, Saxena S. Preoperatively diagnosed isolated hydatid cyst of pancreatic head with triad of obstructive jaundice, left-sided portal hypertension and chronic pancreatitis . MAMC J Med Sci 2015;1:157-9

How to cite this URL:
Choudhary P, Khokhar HV, Aswani Y, Saxena S. Preoperatively diagnosed isolated hydatid cyst of pancreatic head with triad of obstructive jaundice, left-sided portal hypertension and chronic pancreatitis . MAMC J Med Sci [serial online] 2015 [cited 2021 Sep 16];1:157-9. Available from: https://www.mamcjms.in/text.asp?2015/1/3/157/166297

  Introduction Top

Echinococcosis (hydatid disease) is one of the World Health Organization's neglected zoonotic diseases that continues to be a major public health issue in many regions of the world where agriculture and stockbreeding are primary sources of income. Liver followed by lungs are the most commonly involved organs in the body. Pancreatic hydatid cyst (PHC) disease is rare, even in endemic regions.[1]

  Case Report Top

A 30-year-old female presented with dull aching epigastric pain followed by gradually progressive yellowish discoloration of the eyes and urine along with pruritus for last 1-month. No history of previous trauma, abnormal bowel habits, weight loss, anorexia, or vomiting, was noted. Abdominal palpation revealed an epigastric lump in lesser sac and splenomegaly. On physical examination, icterus was seen. Rest of the general physical examination was normal. The liver function tests showed raised aspartate aminotransferase: 250 U/L; alanine aminotransferase: 500 U/L; total bilirubin: 8.2 mg/dL; direct bilirubin: 7.5 mg/dL., serum amylase: 500 U/L, and serum lipase: 300 U/L. Total blood count and hemoglobin level were normal. Ultrasonography (USG) showed a large cystic lesion with internal daughter cysts and floating membrane in pancreatic head region [Figure 1]. Gall bladder was overdistended with dilated common bile duct (CBD) and intrahepatic bile ducts. Splenomegaly with prominent splenic vein was also seen. Contrast enhanced computed tomography (CECT) abdomen also showed 81 mm × 96 mm × 97 mm sized complex cystic lesion in pancreatic head region with internal daughter cysts and enhancing curvilinear internal membrane [Figure 1]. The cyst was showing communication with dilated main pancreatic duct (6.4 mm). CBD was compressed by the cyst in its distal part with dilated proximal part (17 mm). Pancreatic body and tail were showing parenchymal atrophy. Portal confluence was compressed by the cyst with dilated splenic vein (10.5 mm at hilum) and splenomegaly (size - 12.5 cm craniocaudally). Rest of the abdominal viscera including liver were normal. The magnetic resonance imaging (MRI) of abdomen including magnetic resonance cholangiopancreatography (MRCP) revealed similar findings as of CECT abdomen [Figure 1] and [Figure 2]. The true membrane of the cyst including the detached membrane appeared hyperintense on T1-weighted and hypointense on T2-weighted. Based on imaging and biochemical analysis, preoperative diagnosis of isolated hydatid cyst (HC) of the pancreatic head with a triad of obstructive jaundice, left-sided portal hypertension, and chronic pancreatitis, was made. The patient was operated by median laparotomy and cystectomy was performed removing the germinal layer and daughter cysts without spillage. Intraoperatively fistulous communication with main pancreatic duct (MPD) was noted, so cystogastric anastomosis was performed to avoid postoperative pancreatic fistula formation. Macroscopic examination of cyst content showed fluid, small daughter cysts, and pale yellow membrane [Figure 2]. Histopathological examination showed outermost layer formed by the compressed pancreatic tissue, laminated middle layer, and an innermost germinal layer. Follow-up abdominal USG after 6 months of postoperative albendazole therapy revealed no abdominal cyst. Follow-up chest X-ray was also normal.
Figure 1: (a) Ultrasound abdomen shows multiloculated cystic lesion. (b) Postcontrast axial computed tomography scan shows cystic lesion in the pancreatic head having communication with dilated main pancreatic duct (arrow in b) with the curvilinear internal membrane. (c) T1-.weighted axial magnetic resonance imaging image. (d) Internal daughter cysts having hyperintense cyst wall and central hyperintense floating membrane

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Figure 2: (a) Coronal T2-.weighted magnetic resonance imaging showing hydatid cyst with dilated common bile duct (arrow) and overdistended gall bladder (arrowhead) with splenomegaly. (b) Magnetic resonance cholangiopancreatography image. (c) Cyst communication with dilated main pancreatic duct (vertical arrow) and compression of lower common bile duct with dilated upper common bile duct and intrahepatic biliary radicals dilatation (horizontal arrow). (d) Gross examination of cyst component. Daughter cysts and membrane (curved arrow)

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  Discussion Top

Hydatid disease is a widely prevalent neglected zoonosis of major public health importance in India.[2] It is produced by the larval stage of the Echinococcus tapeworm mostly of Echinococcus granulosus or Echinococcus multilocularis.[3] The disease mostly affects liver, lung, spleen, and kidney in decreasing order, although it can be found in almost any tissue or organ of the human body. PHC disease is rare, even in regions where hydatidosis is endemic with a reported prevalence of 2–2%. In a recent literature review of reported cases of PHCs between January 2010 and April 2014, isolated PHC was detected in the majority of patients and the pancreatic head was the most common location.[1] Possible routes of primary hydatid disease of pancreas include hematogenous dissemination, through biliary system, through lymphatic channels, direct passage of larvae into pancreatic tissue, and retroperitoneal dissemination.[1]

The patient can be asymptomatic or symptomatic depending upon the cyst size and location.[1] HCs of pancreatic head may cause obstructive jaundice, acute pancreatitis, cholangitis, duodenal stenosis, or duodenal fistula.[3] The present patient had obstructive jaundice and left-sided portal hypertension due to compression of CBD and splenic vein by the cyst and chronic pancreatitis due to direct fistulization of the cyst in the MPD.

HCs are composed of outermost pericyst formed by compressed host tissue and inner true cyst wall derived from the parasite. The true cyst wall is divided into acellular outer laminated (ectocyst) and inner one-cell-thick germinal membrane (endocyst). Endocyst forms the daughter cysts.[4]

Preoperative diagnosis of PHC is difficult due to its rarity and resemblance to other common pancreatic cysts and pseudocyst.[5] Appearance of hydatid is variable on USG, computed tomography, and MRI depending on the stage of the disease. HCs are classified into four types on the basis of their appearance.[6] Type I HCs appear as a well-defined anechoic mass with or without hydatid sand and septa. These are considered to be an initial stage in the development of the parasite. Rolling the patient during evaluation disperses the sand and produces small echogenic foci, or falling snowflakes. Type I HC appears as a well-defined, water-attenuation mass on computed tomography (CT). On MRI these cysts appear hypointense on T1-weighted images and hyperintense on T2-weighted images, similar to a simple cyst. A characteristic low-signal intensity rim ("rim sign") is described on T2-weighted MRI. This finding represents the parasitic membranes and pericyst. Frequently, postcontrast enhancement of the septa and cyst wall is seen on CT and MRI. Our cyst was type II cyst in which daughter cysts were seen inside the mother cyst. Matrix, floating membranes or vesicles can also be seen in the cyst. Sometimes multiple cysts and echogenic areas that are enclosed together within a single capsule give rise to a"racemose"or "wheel spoke"appearance on USG. On CT, type II HCs can be visualized in three stages depending on the age, number, and arrangement of the daughter cysts. Type IIA lesions contain round daughter cysts arranged at the periphery. Type IIB lesions contain larger, irregularly shaped daughter cysts that occupy almost the entire volume of the mother cyst as seen in the present patient. The high-attenuation fluid that surrounds the daughter cysts within the mother cyst looks like septa, creating a"rosette"appearance. Type IIC lesions appear on CT as relatively high-attenuation round or oval masses with scattered calcifications due to degeneration of old cysts and contain occasional daughter cysts. On MRI, daughter cysts may appear hypointense or isointense relative to the maternal matrix on T1-and T2-weighted images. The "serpent sign," or "snake sign," which represents collapsed parasitic membranes secondary to damage or degeneration of an HC, is another MRI manifestation of an HC. These membranes have low signal intensity with all sequences. In the present patient, daughter cyst wall and detached internal membrane appeared hyperintense on T1-weighted images and hypointense on T2-weighted images.

Type III lesions are totally calcified dead cysts, which show strong posterior shadowing on USG, manifest as round, hyperattenuating areas on CT and hypointense areas on MRI. Type IV HCs (complicated HC) are type I or type II cysts with rupture and superinfection. MRCP can show the communication of the cystic lesion with the pancreatic duct and helps in defining the type of surgical treatment.[1] A bacterial superinfection of HC is always secondary to rupture. Numerous nonspecific signs of cyst infection have been described, including poor delimitation, mixed internal echoes, and air-fluid or fluid-fluid levels.[6]

  Conclusions Top

HC is an important differential diagnosis of pancreatic cyst in endemic areas and characteristic imaging features like the presence of daughter cysts and detached membrane can help in accurate preoperative diagnosis and better surgical planning.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Akbulut S, Yavuz R, Sogutcu N, Kaya B, Hatipoglu S, Senol A, et al. Hydatid cyst of the pancreas: Report of an undiagnosed case of pancreatic hydatid cyst and brief literature review. World J Gastrointest Surg 2014;6:190-200.  Back to cited text no. 1
John TJ, Dandona L, Sharma VP, Kakkar M. Continuing challenge of infectious diseases in India. Lancet 2011;377:252-69.  Back to cited text no. 2
Pedrosa I, Saíz A, Arrazola J, Ferreirós J, Pedrosa CS. Hydatid disease: Radiologic and pathologic features and complications. Radiographics 2000;20:795-817.  Back to cited text no. 3
Ishimitsu DN, Saouaf R, Kallman C, Balzer BL. Best cases from the AFIP: Renal hydatid disease. Radiographics 2010;30:334-7.  Back to cited text no. 4
Turkyilmaz Z, Kapisiz A, Sonmez K, Karabulut R, Basaklar A. Obstructive jaundice resulted from hydatid cyst of pancreatic head. Hippokratia 2013;17:378-9.  Back to cited text no. 5
Polat P, Kantarci M, Alper F, Suma S, Koruyucu MB, Okur A. Hydatid disease from head to toe. Radiographics 2003;23:475-94.  Back to cited text no. 6


  [Figure 1], [Figure 2]


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