Clinical features and treatment of heterotopic pancreas in children: a multi-center retrospective study

Abstract

Objective: Heterotopic pancreas, an uncommon condition in children, can present with diagnostic and treatment challenges. This study aimed to evaluate the clinical features and treatment options for this disorder in pediatric patients.

Methods: We conducted a retrospective analysis, including patients diagnosed with heterotopic pancreas at four tertiary hospitals between January 2000 and June 2022. Patients were categorized into symptomatic and asymptomatic groups based on clinical presentation. Clinical parameters, including age at surgery, lesion size and site, surgical or endoscopic approach, pathological findings, and outcome, were statistically analyzed.

Results: The study included 88 patients with heterotopic pancreas. Among them, 22 were symptomatic, and 41 were aged one year or younger. The heterotopic pancreas was commonly located in Meckel's diverticulum (46.59%), jejunum (20.45%), umbilicus (10.23%),ileum (7.95%), and stomach (6.82%). Sixty-six patients had concomitant diseases. Thirty-three patients had heterotopic pancreas located in the Meckel's diverticulum, with 80.49% of cases accompanied by gastric mucosa heterotopia (GMH). Patients without accompanying GMH had a higher prevalence of heterotopic pancreas-related symptoms (75%). Treatment modalities included removal of the lesions by open surgery, laparoscopic or laparoscopic assisted surgery, or endoscopic surgery based on patient's age, the lesion site and size, and coexisting diseases.

Conclusions: Only one-fourth of the patients with heterotopic pancreas presented with symptoms. Those located in the Meckel's diverticulum have commonly accompanying GMH. Open surgical, laparoscopic surgical or endoscopic resection of the heterotopic pancreas is recommended due to potential complications. Future prospective multicenter studies are warranted to establish rational treatment options.

Keywords: Children; Ectopic pancreas; Heterotopic pancreas; Meckel’s diverticulum; Pancreatic rest; Treatment.

Fig. 1

A 2-day-old boy with malrotation complicating midgut volvulus. (A) intraoperative findings showing a mass at the jejunal mesentery (yellow arrow) (B) gross pathologic appearance revealing an irregular mass along the mesenteric border (red arrow) compressing the adjacent jejunal wall (green arrows) (C) pathologic findings confirming heterotopic pancreas (Heinrich type I), including pancreatic acini (blue arrow), duct (white arrow), and islet cells (black arrow). (Hematoxylin–eosin stain, original magnification, 200 ×)

Fig. 2

An 18-year-old boy with recurrent epigastric pain and non-bilious vomiting (A) gastroscopic view of a polypoid submucosal mass (gray arrow) with intact overlying mucosa located in the greater curvature of the stomach fundus (B) endoscopic ultrasonography (EUS) showing a submucosal mass heterogeneous and hypoechoic (pink arrow), 2.5 cm × 2.0 cm in size, being suspicious for stromal tumor (C) ESD procedure, dissecting the mucosa to expose the submucosal mass (yellow arrow), removing the slight yellow-colored multilobulated massclip closure (green arrow) (E) ESD resected sample(F) pathological findings confirming heterotopic pancreas (Heinrich type I), including pancreatic acini (white arrow), duct (black arrow), and islet cells (blue arrow), (hematoxylin–eosin, original magnification, 200 ×)

Fig. 3

A 13-year-old boy with chronic epigastric pain. (A) an antral umbilicated submucosal nodule (white arrow) protruding into the gastric lumen under gastroscopy, suggestive of submucosal heterotopic pancreas (B) ESD technique (pink arrow) (C) defect after removal of the nodule (yellow arrow)clip closure post-ESD referring to Fig. 1D (D) ESD resected sample (E) pathological findings confirming heterotopic pancreas (Heinrich type II), including pancreatic acini (red arrow) and ducts (gray arrow). (Hematoxylin–eosin stain, original magnification, 200 ×). (F) appearance of endoscopic follow up 11 weeks after ESD (green arrow)