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Biofeedback therapy for chronic constipation in a patient with Prader-Willi syndrome.

Corral JE, Kataria R, Vickers D, Koutouby R, Moshiree B - Ann Gastroenterol (2015 Oct-Dec)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine (Juan E. Corral, Rahul Kataria), University of Miami Miller School of Medicine, Miami, Florida, USA.

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.

No MeSH data available.


Related in: MedlinePlus

(A) Anorectal manometry, four view measurements: Dyssynergic defecation is seen in Valsalva as puborectalis pressure (internal sphincter) increased in push maneuver (arrow heads). (B) Normal Wireless Motility Capsule (SmartPill®) study: red are pressure, blue temperature and green pH readings. First tracing is oro-gastric transit, yellow box is duodenal, and remaining tracing is small and large bowel transit
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Figure 1: (A) Anorectal manometry, four view measurements: Dyssynergic defecation is seen in Valsalva as puborectalis pressure (internal sphincter) increased in push maneuver (arrow heads). (B) Normal Wireless Motility Capsule (SmartPill®) study: red are pressure, blue temperature and green pH readings. First tracing is oro-gastric transit, yellow box is duodenal, and remaining tracing is small and large bowel transit

Mentions: We present a case of a patient with PWS who was referred to our tertiary motility clinic for further evaluation of constipation. PWS was diagnosed in her second month of life after developing four major Holm criteria (neonatal hypotonia, feeding problems, facial features, and intellectual disability) and confirmatory genetic testing in an outside institution [4]. Patient was a 16-year-old female presenting with rectal pain and constipation of 2-year duration. Prior to that, she was having one bowel movement daily. She noted having one soft bowel movement every 5 days with a feeling of incomplete evacuation. Prior medications included polyethylene glycol, lubiprostone, milk of magnesia, and mineral oil leading to mild relief. Additionally, she used bisacodyl, magnesium citrate and enemas at least once weekly in order to have a bowel movement. Patient was not receiving any opioids, thyroid replacement therapy, or antipsychotics. She did not have any other past medical history and specifically no rectal or pelvic surgeries. She noted passive fecal incontinence and had to use manual disimpaction to aid with having a bowel movement. Patient‘s caretakers had not attempted any behavioral treatments like scheduled toilet visits. On physical examination, she was noted to have a body mass index of 30.4 Kg/m2 (height 1.57 m and weight 75.3 Kg). Abdominal examination was unremarkable other than generalized fullness of abdomen with minimal distension on palpation. On digital rectal exam she had a normal anocutaneous reflex with normal sphincter tone but an abnormal relaxation of puborectalis muscle on Valsalva maneuvers. Fasting glucose, celiac serology and thyroid testing were normal. A colonoscopy was performed with no abnormalities found. Poor preparation precluded ileal intubation. Colonic and rectal mucosae were unremarkable. For further evaluation of anorectal function, an anorectal manometry with endorectal surface probe (MMS® EMG & CC-Simulator probe) was preformed showing the following: decreased resting pressure (52 mmHg, normal range 59-74 mmHg), normal squeeze pressure (120 mmHg), normal recto-anal inhibitory response and normal balloon expulsion with ability to expel a 50 cc balloon at <1 min. Rectal sensation testing was normal except for finding of rectal hypersensitivity (maximal tolerable volume of 160 mL, normal range 218-266 mL). An abnormal relaxation of the puborectalis and external sphincter muscles on push maneuvers was seen suggesting DD (Fig. 1A).


Biofeedback therapy for chronic constipation in a patient with Prader-Willi syndrome.

Corral JE, Kataria R, Vickers D, Koutouby R, Moshiree B - Ann Gastroenterol (2015 Oct-Dec)

(A) Anorectal manometry, four view measurements: Dyssynergic defecation is seen in Valsalva as puborectalis pressure (internal sphincter) increased in push maneuver (arrow heads). (B) Normal Wireless Motility Capsule (SmartPill®) study: red are pressure, blue temperature and green pH readings. First tracing is oro-gastric transit, yellow box is duodenal, and remaining tracing is small and large bowel transit
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4585403&req=5

Figure 1: (A) Anorectal manometry, four view measurements: Dyssynergic defecation is seen in Valsalva as puborectalis pressure (internal sphincter) increased in push maneuver (arrow heads). (B) Normal Wireless Motility Capsule (SmartPill®) study: red are pressure, blue temperature and green pH readings. First tracing is oro-gastric transit, yellow box is duodenal, and remaining tracing is small and large bowel transit
Mentions: We present a case of a patient with PWS who was referred to our tertiary motility clinic for further evaluation of constipation. PWS was diagnosed in her second month of life after developing four major Holm criteria (neonatal hypotonia, feeding problems, facial features, and intellectual disability) and confirmatory genetic testing in an outside institution [4]. Patient was a 16-year-old female presenting with rectal pain and constipation of 2-year duration. Prior to that, she was having one bowel movement daily. She noted having one soft bowel movement every 5 days with a feeling of incomplete evacuation. Prior medications included polyethylene glycol, lubiprostone, milk of magnesia, and mineral oil leading to mild relief. Additionally, she used bisacodyl, magnesium citrate and enemas at least once weekly in order to have a bowel movement. Patient was not receiving any opioids, thyroid replacement therapy, or antipsychotics. She did not have any other past medical history and specifically no rectal or pelvic surgeries. She noted passive fecal incontinence and had to use manual disimpaction to aid with having a bowel movement. Patient‘s caretakers had not attempted any behavioral treatments like scheduled toilet visits. On physical examination, she was noted to have a body mass index of 30.4 Kg/m2 (height 1.57 m and weight 75.3 Kg). Abdominal examination was unremarkable other than generalized fullness of abdomen with minimal distension on palpation. On digital rectal exam she had a normal anocutaneous reflex with normal sphincter tone but an abnormal relaxation of puborectalis muscle on Valsalva maneuvers. Fasting glucose, celiac serology and thyroid testing were normal. A colonoscopy was performed with no abnormalities found. Poor preparation precluded ileal intubation. Colonic and rectal mucosae were unremarkable. For further evaluation of anorectal function, an anorectal manometry with endorectal surface probe (MMS® EMG & CC-Simulator probe) was preformed showing the following: decreased resting pressure (52 mmHg, normal range 59-74 mmHg), normal squeeze pressure (120 mmHg), normal recto-anal inhibitory response and normal balloon expulsion with ability to expel a 50 cc balloon at <1 min. Rectal sensation testing was normal except for finding of rectal hypersensitivity (maximal tolerable volume of 160 mL, normal range 218-266 mL). An abnormal relaxation of the puborectalis and external sphincter muscles on push maneuvers was seen suggesting DD (Fig. 1A).

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine (Juan E. Corral, Rahul Kataria), University of Miami Miller School of Medicine, Miami, Florida, USA.

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.

No MeSH data available.


Related in: MedlinePlus