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CASE REPORT
Year : 2023  |  Volume : 28  |  Issue : 3  |  Page : 263-265
 

A rare case of true pancreatic cyst


Department of Paediatric Surgery, Lilavati Hospital and Research Centre, Mumbai, Maharashtra, India

Date of Submission22-Oct-2022
Date of Decision28-Jan-2023
Date of Acceptance22-Feb-2023
Date of Web Publication02-May-2023

Correspondence Address:
Rajeev Redkar
Department of Paediatric Surgery, Lilavati Hospital and Research Centre, Bandra West, Mumbai - 400 050, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaps.jiaps_150_22

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   Abstract 


Pancreatic cysts can be true or pseudocysts. True pancreatic cysts in children are rare clinical entities. We present a 23-month-old boy with a cystic lesion in the distal body and tail of the pancreas which on histopathology was found to be a rare true congenital simple cyst of the pancreas.


Keywords: Amylase, intestinal duplication cyst, lipase, pseudocyst of the pancreas, true congenital pancreatic cyst


How to cite this article:
Achugatla S, Singh S, Sharma RD, Redkar R, Tampi CS. A rare case of true pancreatic cyst. J Indian Assoc Pediatr Surg 2023;28:263-5

How to cite this URL:
Achugatla S, Singh S, Sharma RD, Redkar R, Tampi CS. A rare case of true pancreatic cyst. J Indian Assoc Pediatr Surg [serial online] 2023 [cited 2023 Jun 9];28:263-5. Available from: https://www.jiaps.com/text.asp?2023/28/3/263/375521





   Introduction Top


Congenital pancreatic cyst is very rare in infants and children. The majority of pancreatic cysts in children are pseudocysts resulting from trauma, acute pancreatitis, or infection. True congenital pancreatic cysts, on the other hand, are extremely rare, and they account for <1% of all pancreatic cysts.[1] To the best of our knowledge, only 40 cases have been reported in English literature below the age of 2 years.[2] Although rare, true pancreatic cyst should be considered in the differential diagnosis of a child of <2 years with a cystic lesion of the pancreas.


   Case Report Top


A previously healthy 23-month-old boy presented with pain in the abdomen and a fever for 7 days. Abdominal pain was continuous, localised to left upper quadrant, transiently relieved with analgesics. It was also associated with a high-grade fever, continuous, reaching maximum of 103°F. He also had a mild cough for 1 day. On examination, the patient was sick with tachycardia and tachypnea. He had fullness in the left lumbar region with a palpable soft cystic mass. Blood tests were normal except raised serum amylase (172 U/L) and serum lipase (670 U/L). Abdominal ultrasound (USG) showed a well-defined anechoic thick-walled cystic mass of 9 cm × 8 cm × 6 cm, arising from the body and tail of the pancreas, located in the left abdomen extending to the left iliac fossa, displacing the bowel loops, and containing hyperechoic cystic fluid [Figure 1]. It was associated with multiple gallbladder calculi. Computed tomography (CT) abdomen and pelvis confirmed a thick-walled bilobed cystic lesion arising from the distal body to the tail of the pancreas, with no dilatation of the pancreatic duct, and was suggestive of a pancreatic pseudocyst. Magnetic resonance cholangiopancreatography (MRCP) suggested a possibility of intestinal duplication cyst or pseudocyst of the pancreas [Figure 2]a. Conservative management was started with intravenous fluids and antibiotics. A percutaneous decompression of the cyst followed by open excision of the cyst was carried out. USG-guided drainage of the cyst drained around 200 ml of brownish fluid. Check USG confirmed no vessel involvement, then the pigtail catheter was fixed. Cystic fluid showed raised amylase (10,648 U/L) and lipase levels (42,200 U/L). Cultures showed no growth. As the cystic fluid stopped draining, catheter displacement with no bowel wall perforation was confirmed on USG and CT, then a laparotomy was performed with a left upper quadrant incision. The cyst was partially excised with marsupialization. Cystic fluid routine microscopy showed more than 1 lakh red blood cells and neutrophils rich with raised amylase levels (46,155 U/L) and lipase levels (46,100 U/L). Postoperatively, the patient remained stable and recovery was smooth.
Figure 1: Abdominal USG showed a well-defined anechoic thick-walled cystic bilobed mass (white arrow). USG: Ultrasound

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Figure 2: (a) MRCP showed a large bilobed pancreatic cyst (black arrow), (b) Computed tomography of the abdomen and pelvis showing catheter in the right iliac fossa (white arrow). MRCP: Magnetic resonance cholangiopancreatography

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Grossly, the specimen consisted of multiple congested flat whitish pieces aggregating to 7.0 cm × 6 cm, with thickness ranging from 0.4 to 0.1 cm. The largest piece showed pancreatic tissue at one end measuring 1 cm. Microscopically, the cyst wall was lined by single-layered duct-type columnar epithelium, with large areas of denudation [Figure 3]a. No atypia was seen. The cyst wall also shows scattered residual pancreatic acini and ducts through its length [Figure 3]b. Some of the acini and ductules were compressed in the fibrous stroma of the wall. Few areas of granulation tissue composed of proliferating vessels are also seen. The presence of cyst lining excluded a pseudocyst. No epithelial papillary tufting was seen. Based on the morphology, an enterogenous cyst was excluded and a diagnosis of a true congenital simple pancreatic cyst was given. On follow-up, after 8 months, the patient is healthy and asymptomatic.
Figure 3: (a) H and E stain (×400) – simple-layered duct-type epithelial lining, (b) H and E stain (×100) – cyst showed simple-layered epithelial lining with pancreatic acini in its wall

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   Discussion Top


The first pancreatic cyst was described by Jean Cruveilhier in 1816, whereas the first malignant pancreatic cystic tumor was described by Luecke in 1867.[3] In the pediatric age group, cystic lesions of the pancreas are rare. Pancreatic cysts in children are classified into congenital – developmental, retention, duplication, parasitic pseudocysts, and neoplastic. True cysts (i.e. congenital, duplicated, and retention) are very rare and have an actual epithelium on their walls.[4] Most of the reported cases of cysts within the pancreas are confirmed by intraoperative findings only, as it is not easy to diagnose and confirm them preoperatively. They rarely present with symptoms. Our patient has presented with persistent abdominal pain and high-grade fever. They can present with abdominal pain, abdominal mass, ascites, fever in case of infected cysts, and vomiting. Although USG of the abdomen is a quick and reliable first-line investigation to detect pancreatic cysts, it cannot accurately determine the origin of the cyst. Magnetic resonance imaging is better for assessment of the origin of the giant cysts than CT. Hence, in our case, MRCP was done for better delineation of the cyst anatomy and its relations. Raised serum amylase and lipase levels suggested acute pancreatitis in the background of a cyst with pancreatic origin.

The presence of epithelial cyst lining excluded a pseudocyst. The absence of gastric/intestinal-type epithelium and smooth muscle in the wall excludes the enterogenous cyst. The presence of pancreatic tissue all around the cyst wall and high amylase levels within the cystic fluid, and pancreatic ductal-type epithelium confirms the congenital true pancreatic cyst in this case.

Congenital pancreatic cysts are managed surgically, depending on the size and location of the cyst. Complete resection is done in case of small cyst in the trunk and tail of the pancreas, which may require the removal of the entire distal region.[5] It is important that the spleen is to be preserved in case of distal cysts, especially in children. Cysts arising from the head and body of the pancreas are difficult to resect completely and are managed by internal drainage of the cyst by Roux-en-Y anastomosis.[4] Alternately, percutaneous decompression of the cyst followed by laparoscopic excision of the cyst is also done. In our case, we went ahead with percutaneous decompression in the first stage to relieve fever and abdominal pain. Subsequently, we planned for the cystic excision. As the cystic fluid stopped draining all of a sudden, USG was done, which showed a displaced catheter from the cyst with cystic fluid reduced moderately. Then, CT abdomen and pelvis showed a catheter in the right iliac region [Figure 2]b, with no perforation of the bowel wall. As the catheter displacement was an unforeseen event, we went ahead with open technique. Intraoperatively, the cyst was thin walled with dark brownish fluid within , was seen arising from the distal body and tail of the pancreas. The cyst was marsupialized and excised partially, the mucosa of the edges was burnt with underrunning of the cyst wall, and the tail of the pancreas and spleen was preserved [Figure 4]. The pigtail catheter was removed, and an abdominal drain was placed. Partial excision of the cyst was done in this case as it was adherent to the superior mesenteric artery, so the adherent part of the cyst was left unresected. Internal drainage of the cyst was not considered in our case, because the cyst wall was thin and not matured for anastomosis, besides the cystic fluid was infected. A distal pancreatectomy was not performed as the pancreatic ductal anatomy was normal with no stricture or chronic pancreatitis. As the cyst was large arising from the tail and body as well, and the removal of too much of the pancreatic tissue has its own consequences in a small child, hence pancreatectomy was way far in this case. To minimize the risk of recurrence, the mucosa was cauterized all throughout. The follow-up was uneventful. USG done after 2 months showed no recurrence of the cyst. A successful outcome results in resolution of symptoms and decrease in the size of the cyst, documented by radiological diagnosis as in our case.
Figure 4: Black arrow signifies underrunning sutures of the edges (post marsupialisation of the cyst and partial excision, underrunning of the edges was shown with black arrows)

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   Conclusion Top


A preoperative diagnosis is difficult to establish in a true pancreatic cyst since this is usually asymptomatic. The final confirmation is histopathological with the presence of pancreatic ductal-type epithelium and pancreatic tissue all around the cyst wall. Percutaneous decompression of the cyst followed by laparoscopic or open excision of the cyst is a safer alternative. If the cyst is adherent to the vessels or the base of the mesentery, then a partial excision with underrunning of the edges with cauterization of the mucosal lining should suffice.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given consent for the patient's images and other clinical information to be reported in the journal. The guardian understands that the patient's name and initials will not be published and due efforts will be made to conceal the patient's identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Bawazir OA, Al-Salem AH, Bawazir AO. Congenital pancreatic cyst: Preoperative diagnosis and management. J Pediatr Surg Case Rep 2017;25:16-21.  Back to cited text no. 1
    
2.
Gentimi FE, Papandreou E, Tzovaras AA, Antoniou D. Pancreatic cystic lesion in an infant. J Indian Assoc Pediatr Surg 2011;16:72-4.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Zamora JL. Cystic neoplasms of the pancreas. Evolution of a concept. Am J Surg 1985;149:819-23.  Back to cited text no. 3
    
4.
Auringer ST, Ulmer JL, Sumner TE, Turner CS. Congenital cyst of the pancreas. J Pediatr Surg 1993;28:1570-1.  Back to cited text no. 4
    
5.
Lau ST, Kim SS, Lee SL, Schaller RT. Mucinous cystadenoma of the pancreas in a one-year-old child. J Pediatr Surg 2004;39:1574-5.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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