Introduction
Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant hereditary disorder characterized by mucocutaneous melanin pigmentation and multiple gastrointestinal (GI) hamartomatous polyps[1]. Hamartomatous polyps can be detected in the small bowel, most commonly in the jejunum[2]. These hamartomatous polyps in the small bowel can lead to bleeding, intussusception and obstruction[3,4]. Consequently, patients often undergo multiple laparotomies with intestinal resection, which can ultimately result in short-bowel syndrome and/or severe adhesions[4,5]. It is well acknowledged that large polyps (10-15mm) or symptomatic or rapidly growing polyps should be removed[2,6,7]. In addition, the risks of gastrointestinal and non-gastrointestinal malignancies significantly increase in patients with PJS[8,9].
Double balloon enteroscopy (DBE) was first introduced by Yamamoto et al . in 2001. The major advantage of DBE is that a wide variety of therapeutic interventions can be performed during the examination procedure[10-12]. Over the last decade, DBE has been reported to be useful for treatment of small bowel polyps in patients with PJS[13-16]. The data on the usefulness of DBE for treatment of polyps were focus on adult. However, the diagnosis and treatment procedures in children (aged 0-17 years old) with PJS may be difficult. The key reasons are as follows: One is narrow intestinal lumen, another is thinner intestinal wall, and the third is sharper angle of enteroscopy[17]. The evidence in regards to the role of enteroscopy in children with PJS is limited. Single balloon enteroscopy (SBE) was first introduced in 2007. The main advantages of SBE compared with DBE are ease of setup, shorter procedure time and lower operative cost[18,19].
The aim of this study was to evaluate the efficacy and safety of SBE in patients with PJS. In detail, the aims were: 1) to evaluate the efficacy and safety of SBE in patients with PJS; 2) to evaluate the influence of laparotomy on SBE procedure; 3) to evaluate the efficacy and safety of SBE in children.