K.E.M.
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Department of Radiology
Seth G.S. Medical College and K.E.M. Hospital, Mumbai , India
Case of the Month
Case of the Month
Gastric duplication cyst with accessory pancreatic lobe
Gastric duplication cyst with accessory pancreatic lobe
Contributed by : Kritika Bhatia
Contributed by : Kritika Bhatia
Introduction :
Introduction :
Accessory pancreatic lobe is an extremely rare congenital anomaly defined as an accessory lobe of pancreatic tissue originating from the main pancreatic gland and containing an aberrant duct (1). This anomaly is usually associated with a gastric duplication cyst. The aberrant duct communicates with the main pancreatic duct and the duplication cyst. The gastric duplication cyst also contains ectopic pancreatic tissue or gastric mucosa which is prone to haemorrhage. The aberrant duct is prone to blockage leading to pancreatitis.
Accessory pancreatic lobe is an extremely rare congenital anomaly defined as an accessory lobe of pancreatic tissue originating from the main pancreatic gland and containing an aberrant duct (1). This anomaly is usually associated with a gastric duplication cyst. The aberrant duct communicates with the main pancreatic duct and the duplication cyst. The gastric duplication cyst also contains ectopic pancreatic tissue or gastric mucosa which is prone to haemorrhage. The aberrant duct is prone to blockage leading to pancreatitis.
Clinical Profile:
Clinical Profile:
A 19-year-old woman, four weeks post partum, came with acute onset pain in the abdomen for two days. She had had a similar complaint in her seventh antepartum month for which she had undergone an MRI examination elsewhere , Ultrasonography, contrast-enhanced CT and MRI examination were performed during the present hospital visit.
A 19-year-old woman, four weeks post partum, came with acute onset pain in the abdomen for two days. She had had a similar complaint in her seventh antepartum month for which she had undergone an MRI examination elsewhere , Ultrasonography, contrast-enhanced CT and MRI examination were performed during the present hospital visit.
Laboratory findings:
Laboratory findings:
Increased levels of serum and ascitic fluid amylase. Ascitic fluid amylase increased upto 25,000 IU- thus, confirming the diagnosis of pancreatitis.
Increased levels of serum and ascitic fluid amylase. Ascitic fluid amylase increased upto 25,000 IU- thus, confirming the diagnosis of pancreatitis.
Radiological findings :
Radiological findings :
1. Ultrasonogram of the epigastric region (Fig. 1) shows a cystic lesion with echogenic content. The cyst wall shows gut signature. Adjacent to it, is the accessory pancreatic lobe with a prominent ductal system.
1. Ultrasonogram of the epigastric region (Fig. 1) shows a cystic lesion with echogenic content. The cyst wall shows gut signature. Adjacent to it, is the accessory pancreatic lobe with a prominent ductal system.
Fig 1
Ultrasound images: A cystic lesion with echogenic content and wall showing the gut signature.
2. Contrast-enhanced CT scan of the abdomen (Fig. 2) reveals a well-defined cystic lesion in the greater omentum with a multi-layered wall. It has an intensely enhancing inner wall with a breech in its superolateral aspect. On plain scan, there is a hyperdensity within the cystic lesion- suggestive of blood products. The cyst shares a wall with the greater curvature of the stomach. The pancreatic body and tail are bulky with a prominent main pancreatic duct. There is gross ascites. The accessory pancreatic lobe communicates with the main pancreas. It contains an aberrant duct which communicates with the main pancreatic duct and the cystic lesion.
2. Contrast-enhanced CT scan of the abdomen (Fig. 2) reveals a well-defined cystic lesion in the greater omentum with a multi-layered wall. It has an intensely enhancing inner wall with a breech in its superolateral aspect. On plain scan, there is a hyperdensity within the cystic lesion- suggestive of blood products. The cyst shares a wall with the greater curvature of the stomach. The pancreatic body and tail are bulky with a prominent main pancreatic duct. There is gross ascites. The accessory pancreatic lobe communicates with the main pancreas. It contains an aberrant duct which communicates with the main pancreatic duct and the cystic lesion.
Fig 2
Contrast-enhanced CT scan of the abdomen: (Fig 2A, B, D, E) A well-defined cystic lesion with a multi-layered wall in the greater omentum. It has an intensely enhancing inner wall with the breech in its superolateral aspect. (Fig 2C) On a plain scan, there is a hyperdense blood clot in the cystic lesion. The pancreatic body and tail are bulky with prominent main pancreatic duct and ascites. (Fig 2 D, E) Accessory pancreatic lobe is seen communicating with the main pancreas.
3. T2 weighted MRI images of the abdomen (Fig. 3) confirm the presence of a T2 hyperintense cystic lesion with a multi-layered wall. There is a breach in its superolateral aspect. The 3D image shows the aberrant duct. There is T2 hypointense content within the cyst suggestive of a blood clot.
3. T2 weighted MRI images of the abdomen (Fig. 3) confirm the presence of a T2 hyperintense cystic lesion with a multi-layered wall. There is a breach in its superolateral aspect. The 3D image shows the aberrant duct. There is T2 hypointense content within the cyst suggestive of a blood clot.
Fig 3
T2 weighted MRI images of the abdomen: (Fig 3A, C) T2 hyperintense cystic lesion with a multi-layered wall and a breach (fig 3B) in its superolateral aspect.
(Fig 3D) 3D image shows the aberrant duct. There is a T2 hypointense content within the cyst suggestive of a blood clot.
Radiological diagnosis:
Radiological diagnosis:
Differential diagnosis:
Differential diagnosis:
Pancreatitis with pseudocyst
Pancreatitis with pseudocyst
Gastric duplication cyst
Gastric duplication cyst
The wall of the pseudocyst will show delayed enhancement as it is made of fibrous tissue . The inner mucosal wall shows hyperenhancement. The size will decrease on a follow up scan.
The wall of the pseudocyst will show delayed enhancement as it is made of fibrous tissue . The inner mucosal wall shows hyperenhancement. The size will decrease on a follow up scan.
As compared to the MRI of the abdomen performed during the seventh antenatal month, there is no change in the size of the cyst and no T2 hypointense blood clot within.
As compared to the MRI of the abdomen performed during the seventh antenatal month, there is no change in the size of the cyst and no T2 hypointense blood clot within.
Accessory pancreatic lobe and gastric duplication cyst with mucosal breach and intracystic haemorrhage leading to pancreatitis.
Accessory pancreatic lobe and gastric duplication cyst with mucosal breach and intracystic haemorrhage leading to pancreatitis.
Management:
Management:
The patient had a poor nutritional build and unstable hemodynamics, so surgery was deferred. Instead, she was taken up for ERCP and PD stenting during which she succumbed. The postmortem examination revealed a ruptured duplication cyst with an accessory pancreatic lobe. Histopathology images are not available as the patient’s relative did not conset.
The patient had a poor nutritional build and unstable hemodynamics, so surgery was deferred. Instead, she was taken up for ERCP and PD stenting during which she succumbed. The postmortem examination revealed a ruptured duplication cyst with an accessory pancreatic lobe. Histopathology images are not available as the patient’s relative did not conset.
Timeline:
Timeline:
Discussion
Discussion
Duplications of gastrointestinal organs are very rare congenital abnormalities (2). This gastric anomaly in association with an accessory pancreas and pancreatic duplications is rarely reported (3).
Duplications of gastrointestinal organs are very rare congenital abnormalities (2). This gastric anomaly in association with an accessory pancreas and pancreatic duplications is rarely reported (3).
1. Embryology –
1. Embryology –
The hypothesis states that there is failure of endodermal-ectodermal separation in the 3rd fetal week (4). This leads to adhesions that persist as a ‘neurenteric band’. This band causes traction along the dorsal part of the foregut which leads to the formation of duplication cysts. As the pancreas also develops from a bud from the foregut, traction along this bud due to the neurenteric band causes extension of the pancreatic tissue and gives rise to an accessory pancreatic lobe.
The hypothesis states that there is failure of endodermal-ectodermal separation in the 3rd fetal week (4). This leads to adhesions that persist as a ‘neurenteric band’. This band causes traction along the dorsal part of the foregut which leads to the formation of duplication cysts. As the pancreas also develops from a bud from the foregut, traction along this bud due to the neurenteric band causes extension of the pancreatic tissue and gives rise to an accessory pancreatic lobe.
2. Anatomy
2. Anatomy
The gastric duplication cyst usually arises from the greater curvature or the posterior wall of the stomach. The accessory pancreatic lobe usually stems from the neck or the body of pancreas and follows a ventral direction towards the gastric duplication. (5)
The gastric duplication cyst usually arises from the greater curvature or the posterior wall of the stomach. The accessory pancreatic lobe usually stems from the neck or the body of pancreas and follows a ventral direction towards the gastric duplication. (5)
The gastric duplication cyst comprises of an inner epithelial lining and an outer smooth muscle coat, blood supply originating from gastric vessels, and continuity with the gastric wall. However, luminal continuity may or may not be present (6).
The gastric duplication cyst comprises of an inner epithelial lining and an outer smooth muscle coat, blood supply originating from gastric vessels, and continuity with the gastric wall. However, luminal continuity may or may not be present (6).
The cyst mucosa can be gastric mucosa or ectopic pancreatic tissue. This makes it prone to ulceration and bleeding.
The cyst mucosa can be gastric mucosa or ectopic pancreatic tissue. This makes it prone to ulceration and bleeding.
3. Presentation
3. Presentation
Recurrent acute pancreatitis at a younger age is the typical presentation, precipitated by ;
Recurrent acute pancreatitis at a younger age is the typical presentation, precipitated by ;
a. Obstruction of the pancreatic duct by the blood of intracystic haemorrhage with subsequent hemoductal pancreatitis (7).
a. Obstruction of the pancreatic duct by the blood of intracystic haemorrhage with subsequent hemoductal pancreatitis (7).
b. Mucoid secretions from the cyst with subsequent stasis of pancreatic secretions (8).
b. Mucoid secretions from the cyst with subsequent stasis of pancreatic secretions (8).
4. Treatment –
4. Treatment –
Surgical resection of cyst with accessory pancreatic tissue is the treatment of choice.
Surgical resection of cyst with accessory pancreatic tissue is the treatment of choice.
Conclusion:
Conclusion:
This case teaches us the possibility of rare congenital causes of pancreatitis. However, such rare causes are to be considered after ruling out the common causes like history of alcoholism, gall stones, etc. Evaluation of the ductal system is extremely important in pancreatitis using MRCP to look for congenital anomalies of the ductal system.
This case teaches us the possibility of rare congenital causes of pancreatitis. However, such rare causes are to be considered after ruling out the common causes like history of alcoholism, gall stones, etc. Evaluation of the ductal system is extremely important in pancreatitis using MRCP to look for congenital anomalies of the ductal system.
References:
References:
1. Rousek M, Kachlik D, Nikov A, Pintova J, Ryska M. Gastric duplication cyst communicating to accessory pancreatic lobe: A case report and review of the literature. World J Clin Cases [Internet]. 2018 Dec 12 [cited 2022 Dec 19];6(16):1182.
1. Rousek M, Kachlik D, Nikov A, Pintova J, Ryska M. Gastric duplication cyst communicating to accessory pancreatic lobe: A case report and review of the literature. World J Clin Cases [Internet]. 2018 Dec 12 [cited 2022 Dec 19];6(16):1182.
2. Izumi H, Yoshii H, Abe R, Mukai M, Nomura E, Ito H, et al. Successful laparoscopic resection for gastric duplication cyst: a case report. J Med Case Rep [Internet]. 2019 Jul 19 [cited 2022 Dec 19];13(1):240. Available from: https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-019-2129-1
2. Izumi H, Yoshii H, Abe R, Mukai M, Nomura E, Ito H, et al. Successful laparoscopic resection for gastric duplication cyst: a case report. J Med Case Rep [Internet]. 2019 Jul 19 [cited 2022 Dec 19];13(1):240. Available from: https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-019-2129-1
3. Chattopadhyay A, Mitra SK, Dutta S, Chakraborty H. Gastric, pancreatic, and ureteric duplication. J Indian Assoc Pediatr Surg [Internet]. 2010 Jan 1 [cited 2022 Dec 19];15(1):25–7. Available from: https://pubmed.ncbi.nlm.nih.gov/21180501/
3. Chattopadhyay A, Mitra SK, Dutta S, Chakraborty H. Gastric, pancreatic, and ureteric duplication. J Indian Assoc Pediatr Surg [Internet]. 2010 Jan 1 [cited 2022 Dec 19];15(1):25–7. Available from: https://pubmed.ncbi.nlm.nih.gov/21180501/
4. ROSSLE R. [Rudolf Virchows lecture on general pathological anatomy and general pathology in the year 1852; in commemoration of Virchow’s death 50 years ago (5 Sept. 1902); comments on the true history of disease]. Virchows Arch Pathol Anat Physiol Klin Med [Internet]. 1952 [cited 2022 Dec 19];322(3):233–9. Available from: https://pubmed.ncbi.nlm.nih.gov/13006016/
4. ROSSLE R. [Rudolf Virchows lecture on general pathological anatomy and general pathology in the year 1852; in commemoration of Virchow’s death 50 years ago (5 Sept. 1902); comments on the true history of disease]. Virchows Arch Pathol Anat Physiol Klin Med [Internet]. 1952 [cited 2022 Dec 19];322(3):233–9. Available from: https://pubmed.ncbi.nlm.nih.gov/13006016/
5. Christians KK, Pappas S, Pilgrim C, Tsai S, Quebbeman E. Duplicate pancreas meets gastric duplication cyst: A tale of two anomalies. Int J Surg Case Rep. 2013 Jan 1;4(8):735–9.
5. Christians KK, Pappas S, Pilgrim C, Tsai S, Quebbeman E. Duplicate pancreas meets gastric duplication cyst: A tale of two anomalies. Int J Surg Case Rep. 2013 Jan 1;4(8):735–9.
6. Rousek M, Kachlik D, Nikov A, Pintova J, Ryska M. Gastric duplication cyst communicating to accessory pancreatic lobe: A case report and review of the literature. World J Clin Cases [Internet]. 2018 Dec 26 [cited 2022 Dec 19];6(16):1182–8. Available from: http://www.ncbi.nlm.nih.gov/pubmed/30613679
6. Rousek M, Kachlik D, Nikov A, Pintova J, Ryska M. Gastric duplication cyst communicating to accessory pancreatic lobe: A case report and review of the literature. World J Clin Cases [Internet]. 2018 Dec 26 [cited 2022 Dec 19];6(16):1182–8. Available from: http://www.ncbi.nlm.nih.gov/pubmed/30613679
7. Longmire WP, Rose AS. Hemoductal pancreatitis. Surg Gynecol Obstet. 1973 Feb;136(2):246–50.
7. Longmire WP, Rose AS. Hemoductal pancreatitis. Surg Gynecol Obstet. 1973 Feb;136(2):246–50.
8. Pancreatitis of unusual origin - PubMed [Internet]. [cited 2022 Dec 19]. Available from: https://pubmed.ncbi.nlm.nih.gov/1162566/
8. Pancreatitis of unusual origin - PubMed [Internet]. [cited 2022 Dec 19]. Available from: https://pubmed.ncbi.nlm.nih.gov/1162566/