Published online Apr 21, 2014. doi: 10.3748/wjg.v20.i15.4462
Revised: January 7, 2014
Accepted: February 17, 2014
Published online: April 21, 2014
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.
Core tip: A patient with persisting constipation following STARR or transanal stapled rectal resection, carried out for rectal internal prolapse, needed a diverting sigmoidostomy. She also had proctalgia due to retained staples. Despite normal manometry and intestinal transit times, a deep rectal biopsy showed marked alterations of the intrinsic plexus, which was the main cause of symptoms. Both morphology and function of the anorectum should be carefully investigated prior to indicate surgery. Obstructed defecation may be considered an “Iceberg syndrome”: the rectal internal prolapse is just the tip of the iceberg, and occult underlying lesions should be properly diagnosed and cured.