Acute peritonitis caused by the acute pancreatitis of an ectopic pancreas in a jejunal duplication, in an adult with intestinal malrotation: a case report
Surgical Case Reports volume 9, Article number: 150 (2023)
Intestinal duplication and ectopic pancreas are two rare independent congenital anomalies. Few reports describe cases of patients with ectopic pancreas in an intestinal duplication causing acute peritonitis.
A 31-year-old man was admitted to the hospital for epigastric pain. The patient was diagnosed with acute peritonitis caused by the acute pancreatitis of an ectopic pancreas in a jejunal duplication, with intestinal malrotation. The patient underwent the partial resection of the jejunum and Ladd’s procedure. The histopathological findings indicated ectopic pancreatitis in the jejunal duplication.
We presented the case of acute peritonitis caused by the acute pancreatitis of an ectopic pancreas in a jejunal duplication in an adult with intestinal malrotation. Surgery is the primary treatment and is necessary for a definitive diagnosis.
Intestinal duplication is a rare congenital anomaly, which can be localized anywhere from the mouth to the anus . Ectopic pancreatic tissue is commonly present in the gastrointestinal tract. Only 10 cases of an ectopic pancreas in an intestinal duplication have been reported. Herein, we reported the case of acute peritonitis caused by the acute pancreatitis of ectopic pancreas in a jejunal duplication in an adult with intestinal malrotation.
A 31-year-old man with no related medical history visited his general practitioner because of epigastric pain. Acute cholecystitis was suspected, and the patient was referred to the surgical department of our hospital. On admission, the vital signs were as follows: blood pressure, 124/70 mmHg; pulse, 70 beats/min; and temperature, 36.5 °C. The physical findings included right upper quadrant pain, negative Murphy sign, and no rebound pain. Blood tests revealed the following elevated inflammatory markers; white blood cell count, 9300/μL (3100–8400/μL); C-reactive protein level, 12.1 mg/dL (< 0.3 mg/dL); total bilirubin level, 2.2 mg/dL (0.4–1.5 mg/dL), and gamma glutaryl transferase level, 87 U/L (13–64 U/L). Contrast-enhanced abdominopelvic computed tomography (CT) revealed a 30-mm oval soft tissue mass with microlobulated margins below the gallbladder. The density of the mass was similar to the pancreas (Fig. 1a). In addition, CT revealed a cystic lesion adjacent to the mass with a thick enhanced wall, which communicated with the jejunum (Fig. 1b). These findings suggested an ectopic pancreas and a jejunal duplication. CT also showed that the ascending colon ran up the midline, suggesting that the patient had intestinal malrotation (Fig. 1c). Magnetic resonance cholangiopancreatography (MRCP) revealed an ectopic pancreatic duct without communication to the normal pancreatic duct draining into the jejunal duplication (Fig. 1d). Based on these findings, the patient was diagnosed with acute peritonitis due to the acute pancreatitis of ectopic pancreas in a jejunal duplication and intestinal malrotation. The acute pancreatitis was classified as mild according to the revised Atlanta classification of acute pancreatitis; the patient exhibited neither organ failure nor local complication .
The patient was treated with antibiotics his symptoms and blood test parameters improved in 5 days. However, contrast-enhanced CT revealed indistinct margins and increased fat density around the ectopic pancreas and jejunal duplication, suggesting worsening peritonitis. Thus, the patient was scheduled for surgery. Although the surgery indicated as a laparoscopy, it was converted to open laparotomy because of inflammation and adhesions in the right upper quadrant of the abdominal cavity. An intra-abdominal abscess was observed between the tip of duplication and the gallbladder. Adhesions between the gallbladder and small intestine were dissected, and the abscess cavity was opened. A 12-cm jejunal duplication was observed on the opposite side of the mesentery, located 100 cm distal to the descending part of the duodenum. The tip of the duplication was swollen. Additionally, cord-like tissue was found between the duodenum and the ascending colon. The ligament of Treitz was absent, and the cecum and ascending colon were not fixed to the retroperitoneum (Fig. 2a). Partial resection of the jejunum, including the jejunal duplication, was performed. The Ladd’s procedure, including release of the Ladd’s band, repositioning of the duodenal flexure, and appendectomy, was performed (Fig. 2b). The operation time was 176 min and the intraoperative blood loss was 5 mL. The postoperative course was uneventful, and the patient was discharged on postoperative day 11.
The macroscopic findings of the resected specimen revealed jejunal duplication bifurcated from the jejunum in a T-shaped manner and communicating with the lumen of the normal jejunum (Fig. 3). Microscopically, intestinal mucosa, submucosa, and muscular and serosal layers were detected in the jejunal duplication (Fig. 4a). Ectopic pancreatic tissue with acini and ducts was observed in the adipose tissue between the muscular and serosal layers at the tip of the duplication (Figs. 3b, 4b). Part of the ectopic pancreatic tissue exhibited chronic pancreatitis with the proliferation of fibroblasts and lymphocytic infiltration (Fig. 4c). Hemorrhagic necrosis was observed in the serosa at the tip of the duplication, and pancreatic tissue was not identified, probably because of the strong inflammation (Fig. 3d).
We reported an extremely rare case of acute peritonitis caused by the acute pancreatitis of an ectopic pancreas in a jejunal duplication with intestinal malrotation. Intestinal duplication is a rare congenital anomaly that can affect any part of the gastrointestinal tract. The most frequent location of intestinal duplication is the ileum (31.5%), followed by the ileocecal valve (30.2%), duodenum (9.6%), stomach (8.2%), jejunum (8.2%), colon (6.8%), and rectum (5.5%) . An ectopic pancreas, which is a relatively rare congenital condition, involves pancreatic tissue lacking anatomical or vascular continuity with the normal pancreas. Ectopic pancreas can occur in the gastrointestinal tract, biliary system, liver, spleen, lung, mediastinum, and brain; the most common location is the stomach followed by the duodenum (25%–35%) and jejunum (16%) [4,5,6,7]. Most cases of ectopic pancreas are identified incidentally during surgery or upon autopsy. Autopsy reports indicate that the prevalence of ectopic pancreas range from 0.6% to 13.7% .
Table 1 summarizes 11 cases of ectopic pancreas in intestinal duplication, including the present case [9,10,11,12,13,14,15,16,17,18]. The median age of the eight male patients and three female patients was 35 (15–69) years. The intestinal duplications were located in the jejunum in five cases, the stomach in three cases, the ileum in two cases, and other in one case. Patients with intestinal duplications exhibit various symptoms, including abdominal distension, abdominal pain, hematemesis, and an abdominal painless mass. The symptoms are related to the location, size, and shape of the intestinal duplication. The acute complications of intestinal duplications, such as bleeding, perforation, intestinal obstruction, and intussusception, are rare . An ectopic pancreas is usually asymptomatic but can exhibit complications similar to normal pancreatic tissue, such as pancreatitis, abscess and pseudocyst formation, and malignant transformation [7, 8, 20, 21]. The most common symptoms in patients with ectopic pancreas include epigastric pain, abdominal fullness, and hematochezia . Table 1 shows that 10 (90.9%) patients exhibited symptoms and jejunal duplication with ectopic pancreas was incidentally detected intraoperatively in 1 patient. Three patients (Patients 1, 2, and 8) exhibited symptoms related to the inflammation of the intestinal duplication, and three patients (Patients 4, 5 and 11) exhibited symptoms related to the inflammation of the ectopic pancreas. The inflammation of the intestinal duplication may be due to the stagnation in the blind pouch variety of duplication. However, the causes of inflammation in the ectopic pancreas are unknown. In our patient, the chief complaint was epigastric pain. The pathological findings revealed the highest inflammation in the serosal and the normal mucosal surfaces of the intestinal duplication. Hemorrhagic necrosis was observed in the serosa at the tip of the duplication. The pancreatic tissue could not be identified in the area where hemorrhagic necrosis was observed, possibly because the inflammation was sufficiently strong to cause the disappearance of the pancreatic tissue. The pathological findings suggested that this symptom was caused by the inflammation of the ectopic pancreas, although the patient had no episodes causing pancreatitis.
The preoperative diagnosis of intestinal duplication and ectopic pancreas may be difficult owing the rarity and absence of specific findings. CT may provide some informative findings, and endoscopic ultrasonography (EUS) may be useful for the intestinal segments that can be reached by an endoscope. CT typically shows a spherical duplication cyst as a thick-walled cystic lesion with enhancement of the inner lining and tubular duplication as an intestinal structure with a blind end . The ectopic pancreas appears as a round or oval intramural soft tissue mass with smooth or microlobulated margins on CT . On MRI, the ectopic pancreas generally appears isointense to the orthotopic pancreas in all the sequences, and MRCP may show a duct-like structure inside the lesion. MRI may help distinguish the ectopic pancreas from other submucosal lesions to help diagnose ectopic pancreas [24, 25]. Ectopic pancreas in intestinal duplications were diagnosed preoperatively in only three cases (27.3%), including the present case (Table 1); ectopic pancreas was diagnosed via CT in two patients and via EUS in one patient. In our patient, ectopic pancreas in intestinal duplications was diagnosed preoperatively based on the typical CT and MRI findings. The CT performed immediately after the appearance of symptoms may facilitate accurate diagnosis because the pancreatic structure in the ectopic pancreas can be preserved.
In the reported cases, the ectopic pancreas in the intestinal duplication was resected using endoscopic submucosal dissection (ESD), apart from l the duplication muscle layer in one case. ESD is a treatment option if the lesion is amenable to endoscopic resection and is adequately deep to be safely removed. In other cases, including the present case, small bowel resection or partial gastrectomy, including the intestinal duplication and the cyst, were performed. In general, complete resection is the treatment of choice for symptomatic, possibly malignant, and incidental cases of ectopic pancreas to prevent future complications.
Our patient also had intestinal malrotation. The intestinal malrotation was most likely not associated with the peritonitis in this case. The coexistence of intestinal malrotation with ectopic pancreas and intestinal duplication is extremely rare, and this was the first case to be reported to the best of our knowledge.
We present a case of acute peritonitis caused by the acute pancreatitis of an ectopic pancreas in a jejunal duplication in an adult with intestinal malrotation. CT and MRI may be useful for the preoperative diagnosis of ectopic pancreas. Surgery is the primary treatment and is necessary for a definitive diagnosis. This report aimed to raise awareness and provide information regarding this rare case to help clinical management.
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Endoscopic submucosal dissection
Magnetic resonance cholangiopancreatography
Liu R, Adler DG. Duplication cysts: diagnosis, management, and the role of endoscopic ultrasound. Endosc Ultrasound. 2014;3:152–60.
Banks PA, Bollen TL, Dervenis C, Gooszen HG, Johnson CD, Sarr MG, Pancreatitis Classification Working Group, et al. Classification of acute pancreatitis-2012: revision of the Atlanta classification and definitions by international consensus. Gut. 2013;62:102–11.
Puligandla PS, Nguyen LT, St-Vil D, Flageole H, Bensoussan AL, Nguyen VH, Laberge JM. Gastrointestinal duplications. J Pediatr Surg. 2003;38:740–4.
Christodoulidis G, Zacharoulis D, Barbanis S, Katsogridakis E, Hatzitheofilou K. Heterotopic pancreas in the stomach: a case report and literature review. World J Gastroenterol. 2007;13:6098–100.
Filip R, Walczak E, Huk J, Radzki RP, Bieńko M. Heterotopic pancreatic tissue in the gastric cardia: a case report and literature review. World J Gastroenterol. 2014;20:16779–81.
Kim JY, Lee JM, Kim KW, Park HS, Choi JY, Kim SH, et al. Ectopic pancreas: CT findings with emphasis on differentiation from small gastrointestinal stromal tumor and leiomyoma. Radiology. 2009;252:92–100.
Cazacu IM, Luzuriaga Chavez AA, Nogueras Gonzalez GM, Saftoiu A, Bhutani MS. Malignant transformation of ectopic pancreas. Dig Dis Sci. 2019;64:655–68.
Gokhale UA, Nanda A, Pillai R, Al-Layla D. Heterotopic pancreas in the stomach: a case report and a brief review of the literature. JOP. 2010;11:255–7.
Mizumoto T, Abe S, Okuma T, Mita S, Tomiyasu S, Kanemitsu K. Duplication of intestine presented with an intra-abdominal abscess. J Jpn Surg Assoc. 2009;70:1717–21.
Asano F, Nagamine K, Kaneda K, Yamamoto S, Yoshida K, Kubo A, Takekawa Y. A case of adult ileal duplication with heterotopic pancreas presenting with peritonitis. J Jpn Surg Assoc. 2009;70:2008–12.
Sinha A, Saluja SS, Gamanagatti S. Gastric duplication cyst with macroscopic serosal heterotopic pancreas. JOP J Pancreas. 2010;11:470–3.
Emoto K, Mukai M, Kawachi S, Kitagawa Y, Sakamoto M. Duplication of the digestive organs in the retroperitoneum: a case report with reference to the importance of a standardized nomenclature and definition. Pathol Int. 2011;61:430–4.
Seita M, Jikuhara A, Ogawa R, Nakagawa K, Hashimoto M. A case of jejunal duplication with an ectopic pancreas presented with peritoneal symptoms in an adult. J Jpn Surg Assoc. 2014;75:3289–93.
Gruzu S, Bara T Jr, Bara T, Fetyko A, Jung L. Cystic jejunal duplication with Heinrich’s type I ectopic pancreas, incidentally discovered in a patient with pancreatic tail neoplasm. World J Clin Case. 2016;4:281–4.
Passos ID, Chatzoulis G, Milias K, Tzoi E, Christoforakis C, Spyridopoulos P. Gastric duplication cyst (gdc) associated with ectopic pancreas: case report and review of the literature. Int J Surg Case Rep. 2017;31:109–13.
Ito Y, Kajikawa M, Okano K, Asada T, Okumura N, Watanabe T. A case of jejunal duplication with ectopic pancreatic tissue diagnosed following surgery. J Jpn Surg Assoc. 2020;81:486–92.
Wang TL, Gong XS, Wang J, Long CY. Intestinal intussusception caused by intestinal duplication and ectopic pancreas: a case report and review of literature. World J Clin Cases. 2022;10:2261–7.
Ye X, Wang M, Wang Y, Lin D, Wang X. Gastric duplication cyst with ectopic pancreas in a teenager successfully resected by endoscopic submucosal dissection. BMC Surg. 2022;22:381.
Martini C, Pagano P, Perrone G, Bresciani P, Dell’Abate P. Intestinal duplications: incidentally ileum duplication cyst in young female. BJR Case Rep. 2019;5:20180077.
Emerson L, Layfield LJ, Rohr LR, Dayton MT. Adenocarcinoma arising in association with gastric heterotopic pancreas: a case report and review of the literature. J Surg Oncol. 2004;87:53–7.
Papaziogas B, Koutelidakis I, Tsiaousis P, Panagiotopoulou K, Paraskevas G, Argiriadou H, Atmatzidis S, Atmatzidis KL. Carcinoma developing in ectopic pancreatic tissue in the stomach: a case report. Cases J. 2008;1:249.
Hsia CY, Wu CW, Lui WY. Heterotopic pancreas: a difficult diagnosis. J Clin Gastroenterol. 1999;28:144–7.
Kim DU, Lubner MG, Mellnick VM, Joshi G, Pickhardt PJ. Heterotopic pancreatic rests: imaging features, complications, and unifying concepts. Abdom Radiol. 2017;42:216–25.
Okuhata Y, Maebayashi T, Furuhashi S, Abe K, Takahashi M, Kanamori N, Inoue K, Takayama T. Characteristics of ectopic pancreas in dynamic gadolinium-enhanced MRI. Abdom Imaging. 2010;35:85–7.
Kung JW, Brown A, Kruskal JB, Goldsmith JD, Pedrosa I. Heterotopic pancreas: typical and atypical imaging findings. Clin Radiol. 2010;65:403–7.
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Yoshikawa, C., Migita, K., Yamato, I. et al. Acute peritonitis caused by the acute pancreatitis of an ectopic pancreas in a jejunal duplication, in an adult with intestinal malrotation: a case report. surg case rep 9, 150 (2023). https://doi.org/10.1186/s40792-023-01736-2