Erculiani Marta, Zanatta Cinzia, Vidal Enrico, Martelossi Stefano, Midrio Paola
Division of Pediatric Surgery, Presidio Ospedaliero di Treviso, Treviso, Veneto, Italy.
Division of Pediatrics, Presidio Ospedaliero Universitario Santa Maria della Misericordia, Udine, Friuli-Venezia Giulia, Italy.
European J Pediatr Surg Rep. 2021 Jan;9(1):e33-e36. doi: 10.1055/s-0041-1726868. Epub 2021 May 18.
The case of a toddler with long-channel cloaca, mild chronic kidney disease (CKD) due to renal dysplasia, and early onset of ulcerative colitis (UC) is herein reported. The patient underwent definitive repair of cloaca, that included vaginal elongation with colon, at 5 months of age and was admitted for episodes of vaginal bleeding at 22 months of age. A vaginoscopy revealed a severe inflammation of the colonic neovagina. As rectal bleeding was also noticed, she underwent a colonscopy that showed the same macroscopic inflammatory picture. Neovaginal and colonic biopsies confirmed UC. The mother turned out to be affected by UC since adolescence. The patient is now on oral therapy with mesalazine and topical steroid and mesalazine in the neovagina. The association between cloaca and inflammatory bowel disease (IBD) is anecdotal, but the family history of IBD should be considered when planning the surgical reconstruction of patients with cloaca. In this patient, the occurrence of UC may require a new neovagina in the future and the concomitance of CKD may complicate the overall management due to the potential nephrotoxicity of drugs used for UC therapy.
本文报告了一名患有长通道泄殖腔、因肾发育不良导致轻度慢性肾病(CKD)以及溃疡性结肠炎(UC)早发的幼儿病例。该患者在5个月大时接受了泄殖腔的确定性修复,包括用结肠延长阴道,并在22个月大时因阴道出血发作入院。阴道镜检查显示结肠新阴道严重炎症。由于还发现有直肠出血,她接受了结肠镜检查,显示出相同的宏观炎症表现。新阴道和结肠活检证实为UC。结果发现母亲自青春期起就患有UC。该患者目前正在接受美沙拉嗪口服治疗以及新阴道内局部使用类固醇和美沙拉嗪。泄殖腔与炎症性肠病(IBD)之间的关联鲜有报道,但在为泄殖腔患者规划手术重建时应考虑IBD家族史。在该患者中,UC的发生可能在未来需要一个新的阴道,而CKD的并存可能会因用于UC治疗的药物潜在的肾毒性而使整体管理复杂化。