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.