Small intestinal hamartomatous polyp due to Peutz-Jeghers syndrome in middle childhood.

Small-bowel surveillance is recommended from the age of 8 years in asymptomatic individuals with Peutz-Jeghers syndrome. Because intussusception and risk of strangulated ileus were noted in an 11-year-old patient, small-bowel surveillance should be reliably performed from the age of 8 years to avoid urgent surgery.

CASE DESCRIPTION

An 11‐year‐old boy with Peutz–Jeghers syndrome (PJS) was referred to our hospital. Because his mother also had PJS and black pigmentation was noted on his lips, mouth, and limbs (Figure 1), he underwent upper and lower gastrointestinal endoscopy at the age of 8 years at a previous hospital. Endoscopic mucosal resection for duodenal polyp was performed, and the pathological finding was hamartoma. At that hospital, the small intestine could not be evaluated and was left unattended for 3 years. At our hospital, small intestine fluoroscopy revealed a polyp in the jejunum, and abdominal computed tomography showed two polyps and intussusception (Figure 2), but no gastrointestinal symptom was observed. In double‐balloon enteroscopy, the resected polyps were hamartoma with diameters of 20 mm and 30 mm (Figure 3).

FIGURE 1

Patient had black pigmentation on the lips (A), oral cavity (B), and palm and sole of the foot (D)

FIGURE 2

Small intestine fluoroscopy revealed a polyp in the jejunum (A), and abdominal computed tomography showed two polyps (B, C) and intussusception (C)

FIGURE 3

Double‐balloon enteroscopy revealed two pedunculated polyps in the jejunum (A, B). Pathologically, the muscularis mucosae were dendritic and proliferated, and the small intestinal epithelium was hyperplasia‐proliferated, which was a consistent finding as Peutz–Jeghers syndrome (C, D). Black arrows: muscularis mucosae and white arrows: small intestinal epithelium

Small‐bowel surveillance is recommended from the age of 8 years in asymptomatic individuals with PJS, and polypectomy should be performed for small‐bowel polyps with diameters >15–20 mm to prevent intussusception. In PJS, the risk of intussusception is approximately 44% by the age of 10 years. Because this patient had risk of ileus by intussusception, small‐bowel surveillance for PJS should be reliably performed from the age of 8 years.

AUTHOR CONTRIBUTIONS

T.K. was involved in all stages of patient management and wrote the manuscript. T.A. performed the endoscopic treatment and analyzed the data and collaborated as a reviewer. All authors read and approved the final manuscript.

CONFLICT OF INTEREST

None.

ETHICAL APPROVAL

Written informed consent was obtained from the patient's parents. This report is exempt from ethical approval because it is an observational report after the current care.

CONSENT

Parental/guardian consent was obtained.