Pescatori Lorenzo C, Villanacci Vincenzo, Pescatori Mario
Lorenzo C Pescatori, Mario Pescatori, Coloproctology Unit, Clinica Parioli, 00100 Rome, Italy.
World J Gastroenterol. 2014 Apr 21;20(15):4462-6. doi: 10.3748/wjg.v20.i15.4462.
A rare case of a severely constipated patient with rectal aganglionosis is herein reported. The patient, who had no megacolon/megarectum, underwent a STARR, i.e., stapled transanal rectal resection, for obstructed defecation, but her symptoms were not relieved. She started suffering from severe chronic proctalgia possibly due to peri-retained staples fibrosis. Intestinal transit times were normal and no megarectum/megacolon was found at barium enema. A diverting sigmoidostomy was then carried out, which was complicated by an early parastomal hernia, which affected stoma emptying. She also had a severe diverting proctitis, causing rectal bleeding, and still complained of both proctalgia and tenesmus. A deep rectal biopsy under anesthesia showed no ganglia in the rectum, whereas ganglia were present and normal in the sigmoid at the stoma site. As she refused a Duhamel procedure, an intersphincteric rectal resection and a refashioning of the stoma was scheduled. This case report shows that a complete assessment of the potential causes of constipation should be carried out prior to any surgical procedure.
本文报告了一例罕见的患有直肠神经节细胞缺乏症的严重便秘患者。该患者无巨结肠/巨直肠,因排便梗阻接受了经肛门吻合器直肠切除术(STARR),但症状未缓解。她开始遭受严重的慢性直肠疼痛,可能是由于残留吻合钉周围纤维化所致。肠道传输时间正常,钡灌肠未发现巨直肠/巨结肠。随后进行了乙状结肠造口术,但出现了早期造口旁疝并发症,影响了造口排空。她还患有严重的造口旁直肠炎,导致直肠出血,仍诉说直肠疼痛和里急后重。麻醉下的直肠深部活检显示直肠无神经节,而造口部位的乙状结肠神经节存在且正常。由于她拒绝接受杜哈梅尔手术,计划进行括约肌间直肠切除术并重新塑形造口。本病例报告表明,在进行任何外科手术之前,都应对便秘的潜在原因进行全面评估。