Affiliation:
1. National Medical Research Center for Children’s Health
2. National Medical Research Center for Children’s Health; I.M. Sechenov First Moscow State Medical University (Sechenov University)
Abstract
Introduction. Peutz–Jeghers syndrome (PJS) and juvenile polyposis (JP) are congenital, inherited polyposis of the gastrointestinal tract. Both diseases are rare and characterized by the development of polyps in the small intestine, which can cause small intestine intussusception, obstruction of the gastrointestinal tract, and perforation of the intestinal wall. The main method of treatment is surgical. Polyps are formed throughout the life, and most patients undergo many surgical interventions, but despite this, there is currently no unequivocal opinion on the effectiveness of diagnostic and monitoring methods.
The purpose of this study is to determine the effectiveness of balloon enteroscopy, ultrasound examination of the abdominal cavity and video capsule enteroscopy as methods for diagnosing hamartoma polyps of the deep sections of the small intestine in children with Peutz–Jeghers syndrome and juvenile polyposis.
Materials and methods. Twenty seven SPE and JP patients were examined. Over the period from 2018 to 2022, this group of patients was hospitalized 67 times. Each hospitalization was assessed as a separate case. The average age at the first visit to medical care was 11 years 3 months. (min — 3 years, 6 months, max — 17 years 10 months). All patients underwent abdominal ultrasound and balloon enteroscopy (BE), 7 patients underwent video capsule enteroscopy (VCE).
Results. In 24 cases, ultrasound revealed intussusception of the small intestine. BE revealed 236 polyps in the small intestine, including 29 giant ones. 7 VCE revealed 57 polyps, while BE revealed 30 polyps in the same patients.
Conclusion. Ultrasound of the abdominal cavity does not reveal polyps of the small intestine, but is able to determine the presence of intussusceptum. VCE is the most effective method for detecting polyps, however, BE allows not only searching, but also radical removing.
Publisher
National Medical Research Center for Childrens Health
Reference24 articles.
1. Beggs A.D., Latchford A.R., Vasen H.F., Moslein G., Alonso A., Aretz S., et al. Peutz-Jeghers syndrome: a systematic review and recommendations for management. Gut. 2010; 59(7): 975–86. https://doi.org/10.1136/gut.2009.198499
2. Xu Z.X., Jiang L.X., Chen Y.R., Zhang Y.H., Zhang Z., Yu P.F., et al. Clinical features, diagnosis, and treatment of Peutz-Jeghers syndrome: Experience with 566 Chinese cases. World J. Gastroenterol. 2023; 29(10): 1627–37. https://doi.org/10.3748/wjg.v29.i10.1627
3. Singh A.D., Gupta A., Mehta N., Heald B., Macaron C., Liska D., et al. Occurrence of gastric cancer in patients with juvenile polyposis syndrome: a systematic review and meta-analysis. Gastrointest. Endosc. 2023; 97(3): 407–14.e1. https://doi.org/10.1016/j.gie.2022.10.026
4. Pérez-Castilla A., Peñailillo P., Oksenberg D. Juvenile polyposis syndrome: A case report. Int. J. Surg. Case Rep. 2019; 59: 73–5. https://doi.org/10.1016/j.ijscr.2019.04.041
5. Peutz J.L. A very remarkable case of familial polyposis of the mucous membrane of the intestinal tract and nasopharynx accompanied by peculiar pigmentation of the skin and mucous membrane. Ned. Tijdschr. Geneeskd. 1921; 10: 134–46.