Continuously Recurring Entero-Enteric Intussusception in a Child with Peutz‒Jeghers Syndrome
https://doi.org/10.46563/2026-1-2-2044
Abstract
Peutz‒Jeghers syndrome is a rare hereditary disorder characterized by hamartomatous gastrointestinal polyps and mucocutaneous pigmentation. In childhood, the most clinically significant complications are intussusception and chronic blood loss leading to anemia.
We present a case of a 12-year-old boy with clinically verified Peutz‒Jeghers syndrome, burdened family history, marked pigmentation of the lips and oral mucosa, multiple hamartomatous gastrointestinal polyps, and mild anemia. During planned hospitalization, despite the absence of clinical signs of acute intestinal obstruction, abdominal ultrasound revealed intussusception associated with a polyp. At the first stage, retrograde balloon enteroscopy and endoscopic polypectomy were performed. Follow-up ultrasound demonstrated persistent entero-enteric intussusception, which determined the need for urgent surgical treatment. Laparoscopic desinvagination, enterotomy and polypectomy without bowel resection were performed. Histological examination confirmed hamartomatous polyps. The postoperative period was uneventful.
This case demonstrates that even in minimally symptomatic Peutz‒Jeghers syndrome, ultrasound monitoring plays a key role in detecting continuously recurring intussusception and in choosing a stage-based treatment strategy.
About the Authors
E. Yu. DyakonovaRussian Federation
Elena Yu. Dyakonova, MD, Dr. Sci. (Medicine), Head and Professor of the Department of Pediatric Surgery with a course in Anesthesiology and Intensive Care at the Institute of Additional Professional Education; Head of the Research Institute of Pediatric Surgery, Head of the Department of General and Planned Surgery
Moscow
M. M. Lokhmatov
Russian Federation
Maksim M. Lokhmatov, MD, Dr. Sci. (Medicine), Professor, Senior Researcher, Endoscopist, Head of the Department of Endoscopic Research
Moscow
A. A. Gusev
Russian Federation
Aleksey A. Gusev, MD, Cand. Sci. (Medicine), Acting Deputy Chief Physician for Surgery, Leading Researcher, pediatric surgeon at the Department of General and Planned Surgery
Moscow
A. E. Myagkov
Russian Federation
Aleksandr E. Myagkov, Pediatric surgeon at the Department of General and Elective Surgery
Moscow
A. S. Bekin
Russian Federation
Aleksandr S. Bekin, MD, Cand. Sci. (Medicine), Senior Researcher, Pediatric Surgeon, Department of General and Elective Surgery
119991, Moscow
References
1. Kirakosyan EV, Lokhmatov MM, Dyakonova EYu. High-tech diagnostic methods and an enteroscopic treatment of children with PeutzJeghers syndrome. M.Ya. Studenikin Russian Pediatric Journal. 2019;22(1):17–22. doi: 10.18821/1560-9561-2019-22-1-17-22 EDN: QFYEFZ
2. Amru RL, Dhok A. Peutz-Jeghers syndrome: a comprehensive review of genetics, clinical features, and management approaches. Cureus. 2024;16(4):e58887. doi: 10.7759/cureus.58887
3. Beggs AD, Latchford AR, Vasen HF, et al. Peutz-Jeghers syndrome: a systematic review and recommendations for management. Gut. 2010;59(7):975–986. doi: 10.1136/gut.2009.198499
4. Latchford A, Cohen S, Auth M, et al. Management of Peutz-Jeghers syndrome in children and adolescents: a position paper from the ESPGHAN Polyposis Working Group. J Pediatr Gastroenterol Nutr. 2019;68(3):442–452. doi: 10.1097/MPG.0000000000002248
5. Rachkov VE, Zakuev RI, Kutskaya SV, et al. Peitz-Jaegers syndrome as a cause of intussusception in children. Russian Journal of Pediatric Surgery. 2023;27(6):452–461. doi: 10.17816/ps584 EDN: XDMZXL
6. Van Lier MG, Mathus-Vliegen EM, Wagner A, et al. High cumulative risk of intussusception in patients with PeutzJeghers syndrome: time to update surveillance guidelines? Am J Gastroenterol. 2011;106(5):940–945. doi: 10.1038/ajg.2010.473
7. Korolev GA, Lokhmatov MM, Dyakonova EYu, et al. Tactics of surgical treatment of children with small intestinal polyps with Peutz-Jeghers syndrome and juvenile polyposis. Pediatrics. Consilium medicum. 2024;(1):82–86. doi: 10.26442/26586630.2024.1.202687 EDN: HWOBLD
8. Verma A, Kanneganti P, Kumar B, et al. Peutz-Jeghers syndrome: management for recurrent intussusceptions. Pediatr Surg Int. 2024;40(1):148. doi: 10.1007/s00383-024-05723-y
Review
For citations:
Dyakonova E.Yu., Lokhmatov M.M., Gusev A.A., Myagkov A.E., Bekin A.S. Continuously Recurring Entero-Enteric Intussusception in a Child with Peutz‒Jeghers Syndrome. M.Ya. Studenikin Russian Pediatric Journal. 2026;1(2):140-144. (In Russ.) https://doi.org/10.46563/2026-1-2-2044
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