• Case report
• Intestinal atresia
• Intestinal duplication
• Stapled anastomosis
• Visceral inversion

• Case report
• Duodenal duplication cyst
• Neonate
• Pediatric surgery

• Duodenum
• duodenal duplication cyst
• neonatal abdominal lump
• neonate
• paediatric surgery

Intraluminal duodenal diverticulum (IDD) is a rare congenital abnormality, consisting of a sac-like mucosal lesion in the duodenum. Cases of IDD can present with gastrointestinal bleeding, duodenal obstruction, or pancreatitis. Here, we report a rare case of a 25-year-old female presenting with IDD complicated by duodeno-duodenal intussusception and recurrent pancreatitis. The diagnosis was based on findings from radiologic examinations (CT and MRI), upper gastrointestinal series (barium swallow), and gastroduodenofiberscopy. Laparoscopic excision of the presumed duodenal duplication was performed. The subsequent histopathologic evaluation of the excised sac revealed normal mucosa on both sides, but the absence of a proper muscle layer confirmed the diagnosis of IDD. Radiologic detection of a saccular structure in the second portion of the duodenum can indicate IDD with duodeno-duodenal intussusception as the lead point.

Duodenal duplications are rare congenital anomalies of the gastrointestinal tract. As the periampullary variant is much rarer, literature is scant and only few authors have reported their experience in diagnosis and treatment, particularly with operative endoscopy.

To report our experience with the endoscopic treatment in a series of children with periampullary duodenal duplication cysts, focusing on the importance of obtaining an accurate preoperative anatomic assessment of the malformations. The pediatric periampullary duodenal duplication cyst literature is reviewed. We conducted a systematic review according to the PRISMA guidelines. The PubMed database was searched for original studies on “duodenal duplication”, “periampullary duplication” or “endoscopic management” published since 1990, involving patients younger than 18 years of age. Eligible study designs were case report, case series and reviews. We analyzed the data and reported the results in table and text. Fifteen eligible articles met the inclusion criteria with 16 patients, and analysis was extended to our additional 4 cases. Median age at diagnosis was 13.5 years. Endoscopic treatment was performed in 10 (50%) patients, with only 2 registered complications.

Periampullary duodenal duplication cysts in pediatric patients are very rare. Our experience suggests that an accurate preoperative assessment is critical. In the presence of sludge or stones inside the duplication, endoscopic retrograde cholangio-pancreatography is mandatory to demonstrate a communication with the biliary tree. Endoscopic treatment resulted in a safe, minimally invasive and effective treatment. In periampullary duodenal duplication cyst endoscopically treated children, long-term follow-up is still necessary considering the potential malignant transformation at the duplication site.

• Periampullary duodenal duplication cyst
• Duodenal duplication
• Endoscopic ultrasound
• Endoscopic treatment
• Double wall sign
• Case report

Duodenal duplication cysts (DDCs) are rare congenital anomalies typically manifesting during childhood. Clinical manifestations are uncommon in adulthood. DDCs were classically treated surgically, but endoscopic treatment has been increasingly reported. Endoscopic cyst marsupialization establishes a communication between the cyst cavity and the duodenal lumen so that the cystic content can be drained continuously into the duodenum. We herein describe two cases of symptomatic DDCs diagnosed in adulthood and submitted to endoscopic marsupialization using different techniques and devices.

Case 1: A 23-year-old female patient was admitted with the diagnosis of acute pancreatitis. Endoscopic ultrasound revealed a 35-mm duodenal subepithelial lesion whose proximal limit was immediately distal to the ampulla of Vater and filled with fluid and calcifications. Using a duodenoscope, deroofing of the lesion was made with a diathermic snare. Pathology confirmed the diagnosis of DDC. Case 2: A 41-year-old female, submitted to laparoscopic cholecystectomy 1 month earlier due to suspected lithiasic acute pancreatitis, was admitted due to suspicion of iatrogenic biliary fistula. An endoscopic retrograde cholangiopancreatography was performed and the bile leak was treated. Immediately distal to the papillary orifice, a 20-mm subepithelial lesion was also detected. A biopsy forceps was used to fenestrate its wall, allowing the exit of mucous fluid and stones, and a sphincterotome was used to expand the incision. No recurrence was documented in both cases.

These cases highlight DDC as a potential cause for acute pancreatitis in adults and endoscopy as an easy treatment option.

• Duodenal duplication cyst
• Endoscopy
• Imaging
• Pediatrics
• Surgery

Duodenal duplication cysts in adults are rare, and a preoperative diagnosis remains difficult because clinical manifestations are nonspecific and variable. We describe a case of a duodenal duplication cyst mimicking a pancreatic pseudocyst with repeated intracystic hemorrhage.

A 47-year-old male who complained of upper abdominal pain and vomiting was referred to our hospital. He was a heavy drinker and had a past history of hospitalization for alcoholic chronic pancreatitis. Plain abdominal computed tomography (CT) showed a cystic lesion of 7 cm in size in the lumen near the second part of the duodenum. The cystic lesion showed high density inside. Gastrointestinal endoscopy revealed that the lumen of the duodenum was deformed by a submucosal tumor-like mass and the endoscope could not pass through it, but active bleeding was not seen in the lumen of the duodenum. On the fourth day of hospitalization, anemia progressed and contrast-enhanced CT demonstrated a high-density spot on the wall of the cystic lesion. A pancreatic pseudocyst complicated with intracystic hemorrhage was preliminarily considered. Angiography was immediately performed, and a pseudoaneurysm was identified in the branch of the anterior superior pancreaticoduodenal artery (ASPDA). Transcatheter arterial embolization (TAE) was performed. Anemia did not progress after that, and follow-up CT showed reduction in the size of the cystic lesion. Afterward, the same symptoms recurred twice and surgical treatment was performed for the pancreatic pseudocyst with repeated intracystic hemorrhage. Macroscopically, a cystic mass of 5 cm in size was adjacent to the second part of the duodenum on the pancreas side. A pinhole-sized communication was identified between the cyst and the duodenum lumen. Microscopically, the cyst wall was composed of gastric mucosa and shared a common proper muscle layer with the duodenum. Chronic ulcers and erosions were seen in the cyst. Based on these findings, a diagnosis of duodenal duplication cyst was made.

Duodenal duplication cysts in adults have seldom been reported and should be considered as a differential diagnosis for a patient with a cystic lesion adjoining the duodenum.

• Duodenum duplication cyst
• Intracystic hemorrhage
• Pancreatic pseudocyst

• DDC, duodenal duplication cyst

• Contrast Media
• Endosonography/methods
• Gallbladder Diseases/diagnostic imaging
• Humans
• Lymph Nodes/diagnostic imaging
• Pancreatic Cyst/diagnostic imaging
• Pancreatic Neoplasms/diagnostic imaging
• Sensitivity and Specificity
• Stomach Neoplasms/diagnostic imaging

• Adult
• Cysts/diagnosis
• Cysts/surgery
• Duodenal Diseases/diagnosis
• Duodenal Diseases/surgery
• Endoscopy/methods
• Humans
• Male

• Duodenal duplication
• abdominal pain
• bowl obstruction
• congenital anomaly
• duodenum
• internal derivation
• subtotal excision

A 15-year-old domestic shorthair cat presented for lethargy, vomiting and anorexia. Abdominal ultrasound showed a bi-lobed cystic duodenal mass. Based on ultrasonographic features, malignant transformation of a duodenal duplication cyst was suspected. A resection and anastomosis was performed. Histology of the mass was consistent with carcinoma and an intestinal mucosa was present along the inner surface of the mass, suggestive of a duplication cyst. The patient returned 3 months postsurgery with recurrence of clinical signs. Abdominal ultrasound showed a recurrent duodenal mass at the surgery site and body wall nodules. Fine-needle aspirates of these lesions showed epithelial neoplasia. Owing to the poor prognosis, the owners elected euthanasia.

Malignant transformation of duplication cysts in cats is rare but can be detected on ultrasound. The described ultrasonographic features can aid in prioritization of malignant transformation of duplication cysts as a differential diagnosis.

• Cyst
• Nonmucinous
• Pancreas
• Pseudocyst
• Serous cystic neoplasm

• Abdominal Pain/diagnosis
• Abdominal Pain/etiology
• Adolescent
• Adult
• Aged
• Child
• Cysts/complications
• Cysts/diagnosis
• Cysts/surgery
• Diagnosis, Differential
• Duodenal Diseases/complications
• Duodenal Diseases/diagnosis
• Duodenal Diseases/surgery
• Duodenum/abnormalities
• Duodenum/surgery
• Early Diagnosis
• Female
• Humans
• Infant, Newborn
• Intussusception/diagnosis
• Intussusception/etiology
• Male
• Middle Aged
• Nausea/diagnosis
• Nausea/etiology
• Pancreatitis/diagnosis
• Pancreatitis/etiology
• Tomography, X-Ray Computed
• Treatment Outcome
• Vomiting/diagnosis
• Vomiting/etiology

Duodenal duplication, a rare congenital malformation, can also be observed in adulthood. Although it can be cystic or tubular, communicating or non-communicating, cystic and non-communicating forms are the most common. Several complications, such as obstruction, bleeding, perforation and pancreatitis, may result. Optimal treatment is total excision, although endoscopic procedures have also been described in appropriate cases. If total excision is not possible, subtotal excision and internal derivation can be performed. The 38-year-old woman presented here had occasional attacks of abdominal pain and obstruction, and we considered the diagnosis of duodenal duplication by abdominal computerized tomography. As we confirmed the diagnosis with operative findings and histopathological signs, we treated her with subtotal excision and intraduodenal cystoduodenostomy.

• Duodenum
• Duplication
• Subtotal excision
• Intraduodenal cystoduodenostomy