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Background: Gastrointestinal duplication is a rare developmental anomaly that can be present anywhere along the GI tract, most often being found in ileum. The purpose of this study is to share our experience in evaluation of the presentation, investigations, management challenges and complications of patients with this very rare condition. Methods: This descriptive case series was conducted at the Department of Paediatric Surgery, National Institute of Child Health Karachi, Pakistan, from April 2018 to October 2019. Data was analysed with regard to age, clinical presentation, investigations, surgical procedures, site and type of lesion, histopathology, complications and outcomes. Results: A total of five patients were managed in one and half year. The patients' ages ranged from antenatally diagnosed foetus to 12 years old child. New-born who presented with antenatal diagnosis of abdominal cyst turned out to have duodenal duplication cyst. Among other four were thoracoabdominal duplication cyst, gastric duplication, jejunal duplication and ileal duplication, last two presented with perforation. Other presentations were abdominal pain, swelling and vomiting. Diagnosis was made on clinical ground, x-ray of abdomen, ultrasound and computed tomography. All cysts were resected successfully and patients remained asymptomatic till one year follow up except one patient who expired postoperatively due to liver failure. Conclusion: Enteric duplication can present in variety of ways depending on anatomical location. Prompt diagnosis and complete excision of cyst is the aim of treatment. However, these rare types of duplication are a challenge to operating surgeons. [ABSTRACT FROM AUTHOR]

Duodenal duplication cyst is rarely located inside the liver. We present an extremely rare case of a 12-year-old girl with a large intrahepatic duodenal duplication cyst and a preduodenal portal vein. She was incidentally diagnosed with a large liver cyst (6.5 × 6.1 × 3.6 cm) containing air and fluid with a preduodenal portal vein after a systemic computed tomography scan for suspected internal injuries because of a traffic accident. The upper gastrointestinal series revealed that the cyst communicated with the duodenal bulb via a wide stalk. Upper gastrointestinal endoscopy revealed a large entry hole to the cyst at the duodenal bulb. Biopsy of the inner surface of the cyst revealed that the alimentary mucosa resembled that of the duodenum. She had no symptoms; therefore, continued observation was recommended as the sole management option. This is the third case of this extremely rare condition reported in the literature. This report describes and compares the common features of the three cases.

Wang and colleagues report a rare anatomic anomaly consisting of an accessory pancreatic lobe which originated from the main pancreatic gland with duodenal duplication in a girl with relapsing pancreatitis. The anomaly was visualized on magnetic resonance cholangiopancreatography before being confirmed on endoscopic retrograde cholangiopancreatography and surgery., (© 2022 Japanese Society of Hepato-Biliary-Pancreatic Surgery.)

Duodenal duplication cyst is rarely located inside the liver. We present an extremely rare case of a 12-year-old girl with a large intrahepatic duodenal duplication cyst and a preduodenal portal vein. She was incidentally diagnosed with a large liver cyst (6.5 × 6.1 × 3.6 cm) containing air and fluid with a preduodenal portal vein after a systemic computed tomography scan for suspected internal injuries because of a traffic accident. The upper gastrointestinal series revealed that the cyst communicated with the duodenal bulb via a wide stalk. Upper gastrointestinal endoscopy revealed a large entry hole to the cyst at the duodenal bulb. Biopsy of the inner surface of the cyst revealed that the alimentary mucosa resembled that of the duodenum. She had no symptoms; therefore, continued observation was recommended as the sole management option. This is the third case of this extremely rare condition reported in the literature. This report describes and compares the common features of the three cases. • Duodenal duplication cyst is rarely located inside the liver. • A girl had a large intrahepatic duodenal duplication cyst and preduodenal portal vein. • Only three cases of intrahepatic duodenal duplication cysts have been reported. • There are some common features between these cases. • These features should serve as identification patterns. [ABSTRACT FROM AUTHOR]