ABSTRACT
INTRODUCTION AND AIMS: Peutz-Jeghers syndrome is an autosomal dominant inherited pathology characterized by gastrointestinal hamartomatous polyps, predominantly in the small bowel, and pigmented mucocutaneous lesions. Guidelines suggest polypectomy with a balloon-assisted enteroscope when polyps are larger than 10mm. Complications in adults can be as high as 6.8%, but there is little information on pediatric populations. Our aim was to describe the safety and efficacy of polypectomy in a group of pediatric patients with Peutz-Jeghers syndrome using balloon-assisted enteroscopy. MATERIALS AND METHODS: A retrospective study was conducted at the Hospital de Especialidades del Centro Médico Nacional Siglo XXI on pediatric patients with Peutz-Jeghers syndrome that required balloon-assisted enteroscopy and polypectomy within the time frame of January 2010 and December 2015. Patients that underwent polypectomy with a push enteroscope were excluded from the study. RESULTS: A total of 35 polypectomies were performed on 4 patients (female/male: 3/1). The mean age of the patients was 13.7 years (range:11-16). Twelve enteroscopies were carried out, 8 of which were anterograde. A single-balloon enteroscope was used in 7 procedures and a double-balloon enteroscope in 5. The mean size of the polyps was 1.6cm (range: 1-4cm). A major complication (acute pancreatitis) presented in only one case (8.3%). No other major complications associated with the procedures were observed. CONCLUSION: Balloon-assisted enteroscopy with polypectomy in children is a safe and effective procedure, with complications similar to those reported in adults.
Subject(s)
Digestive System Surgical Procedures/methods , Double-Balloon Enteroscopy/methods , Intestinal Polyps/surgery , Intestine, Small/surgery , Peutz-Jeghers Syndrome/surgery , Adolescent , Child , Digestive System Surgical Procedures/adverse effects , Female , Humans , Intestine, Small/diagnostic imaging , Male , Peutz-Jeghers Syndrome/diagnostic imaging , Retrospective StudiesSubject(s)
Peutz-Jeghers Syndrome/diagnosis , AMP-Activated Protein Kinase Kinases , Adolescent , Endoscopy, Gastrointestinal , Hamartoma/diagnosis , Hamartoma/diagnostic imaging , Hamartoma/surgery , Humans , Intestinal Polyps/diagnosis , Intestinal Polyps/diagnostic imaging , Intestinal Polyps/surgery , Male , Peutz-Jeghers Syndrome/diagnostic imaging , Peutz-Jeghers Syndrome/genetics , Protein Serine-Threonine Kinases/geneticsABSTRACT
The authors review a single case history of intestinal polyposis (Peutz-Jeghers syndrome) of a patient followed by them for eleven years. The paper includes a review of the pertinent literature. The present case was diagnosed as a result of the clinical observation of pigmented spots in the oral mucosa and crises of spasmodic abdominal pain accompanied by bowel movements containing mucus and blood. In other members of the family only a younger sister had slightly pigmented spots in the oral mucosa. None had radiologic findings suggestive of the syndrome of Peutz-Jeghers. Recently the patient under study suffered an intestinal intussuception which led to the surgical resection of a segment of small intestine and permitted pathologic confirmation of the diagnosis. Surgical resection of nodules in both breasts resulted in diagnoses of sclerosing adenosis in the left and an epidermoid cyst in the right.