Abstract
Familial adenomatous polyposis (FAP) is a well-described inherited syndrome, which presents with thousands of adenomas. Polyps appear in childhood or adolescence. Colectomy is the treatment of choice to prevent colorectal cancer. Decision on the timing for colectomy should be determined by polyp burden and characteristics of colonic adenomas in the context of social, personal, and educational factors. A panproctocolectomy and Ileal J-pouch-anal anastomosis for FAP in a 9-year-old girl was done jointly by the adult and paediatric colorectal team. The national multidisciplinary meeting recommended to proceed with a laparoscopic proctocolectomy and J-pouch reconstruction. The aim was to perform the procedure jointly by the adult and paediatric surgery team in a high-volume centre, to cover the pouch with a temporary ileostomy and do a hand-sewn anastomosis. Laparoscopically, the colon was mobilised and the proctectomy was performed. In lithotomy position, the incision was made on the dentate, a 5 cm circular dissection of a mucosal cuff, and a full thickness dissection of the rectum was performed. The pouch was created, a hand-sewn ileoanal anastomosis was performed. A joint paediatric-adult MDT approach is essential for any decision making, providing expertise-sharing cornerstone of the best outcome for the patient.
This is a preview of subscription content, log in via an institution to check access.
Data availability
Data available on request.
References
Wennstrom J, Pierce ER, McKusick VA (1974) Hereditary benign and malignant lesions of the large bowel. Cancer 34:850–857. https://doi.org/10.1002/1097-0142(197409)34:3+%3c850::AID-CNCR2820340711%3e3.0.CO;2-S
Hyer W, Cohen S, Attard T, Vila-Miravet V, Pienar C, Auth M et al (2019) Management of familial adenomatous polyposis in children and adolescents: Position paper from the espghan polyposis working group. J Pediatr Gastroenterol Nutr 68:428–441. https://doi.org/10.1097/MPG.0000000000002247
Distante S, Nasioulas S, Somers GR, Cameron DJS, Young MA, Forrest SM et al (1996) Familial adenomatous polyposis in a 5 year old child: A clinical, pathological, and molecular genetic study. J Med Genet 33:157–160. https://doi.org/10.1136/jmg.33.2.157
Vasudevan SA, Patel JC, Wesson DE, Plon SE, Finegold MJ, Nuchtern JG (2006) Severe dysplasia in children with familial adenomatous polyposis: Rare or simply overlooked? J Pediatr Surg 41:658–661. https://doi.org/10.1016/j.jpedsurg.2005.12.005
Rogers FB, Horst MA, Morgan ME, Vernon TM, Gaines BA, Rogers AT et al (2020) A comparison of adolescent penetrating trauma patients managed at pediatric versus adult trauma centers in a mature trauma system. J Trauma Acute Care Surg 88:725–733. https://doi.org/10.1097/TA.0000000000002643
Somani BK, Griffin S (2018) Ureteroscopy for paediatric calculi: the twin-surgeon model. J Pediatr Urol 14:73–74. https://doi.org/10.1016/j.jpurol.2017.10.004
Marcell AV, Burstein GR (2017) Sexual and reproductive health care services in the pediatric setting. Pediatrics 140:e20172858. https://doi.org/10.1542/peds.2017-2858
Funding
None.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors declare no conflict of interest.
Ethical approval
Hospital-approved protocols were followed.
Consent to publish
Consent was obtained.
Rights and permissions
About this article
Cite this article
Budzanowski, A., Manzo, C.A., Wills, D. et al. Proctocolectomy and ileal J-pouch anastomosis for familial adenomatous polyposis in a paediatric patient. J Ped Endosc Surg 6, 25–27 (2024). https://doi.org/10.1007/s42804-024-00215-1
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s42804-024-00215-1