title: Biofeedback therapy for chronic constipation in a patient with Prader-Willi syndrome.
abstract: Constipation is a common feature of Prader-Willi syndrome. Research exploring the prevalence, cause and treatment options for constipation is limited and lacks objective measurements such as anorectal manometry. We report a case of a 16-year-old lady with Prader-Willi syndrome presenting with rectal pain and constipation for 2 years despite multiple medications and weekly enemas. She also noted passive fecal incontinence that required frequent manual disimpactions. Anorectal manometry revealed an abnormal relaxation of the puborectalis and external sphincter muscles on push maneuvers suggesting dyssynergic defecation and rectal hypersensitivity. Contraction and relaxation of her pelvic muscles were recorded with electromyography. Relaxation of the puborectalis muscle improved significantly after three biofeedback sessions. Patient was successfully tapered off laxatives and has been maintained on linaclotide only. Dyssynergic defecation may be a common finding in Prader-Willi syndrome. In selected cases we recommend anorectal manometry to identify neuromuscular dysfunction and subsequent biofeedback therapy depending on the degree of mental retardation to minimize overuse of laxatives.
authors.affiliation: Department of Medicine (Juan E. Corral, Rahul Kataria), University of Miami Miller School of Medicine, Miami, Florida, USA.
authors.forename: Juan E
authors.initials: JE
authors.lastname: Corral
authors.affiliation: Department of Medicine (Juan E. Corral, Rahul Kataria), University of Miami Miller School of Medicine, Miami, Florida, USA.
authors.forename: Rahul
authors.initials: R
authors.lastname: Kataria
authors.affiliation: Division of Gastroenterology (Dawn Vickers, Baharak Moshiree), University of Miami Miller School of Medicine, Miami, Florida, USA.
authors.forename: Dawn
authors.initials: D
authors.lastname: Vickers
authors.affiliation: Division of Pediatric Gastroenterology (Rahgad Koutouby), University of Miami Miller School of Medicine, Miami, Florida, USA.
authors.forename: Raghad
authors.initials: R
authors.lastname: Koutouby
authors.affiliation: Division of Gastroenterology (Dawn Vickers, Baharak Moshiree), University of Miami Miller School of Medicine, Miami, Florida, USA.
authors.forename: Baharak
authors.initials: B
authors.lastname: Moshiree
keywords: Prader-Willi syndrome; constipation; pelvic floor disorders