NASPGHAN Postgraduate Course 2017 (Part 5): Refractory constipation, Extraesophageal GERD, POTS, Recurrent Abdominal Pain

This blog entry has abbreviated/summarized these presentations. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well.

Here is a link to postgraduate course syllabus: NASPGHAN PG Syllabus – 2017

The child with refractory constipation

Jose Garza   GI Care for Kids & Children’s Healthcare of Atlanta

Key points:

  • Polyethylene glycol is a first-line agent and many patients require cleanout at start of therapy
  • Adequate dose of laxative is needed for sure regular painless stools
  • Don’t stop medicines before toilet training and until pattern of regular stooling established. “All symptoms of constipation should resolve for at least one month before discontinuation of treatment”
  • Gradually reduce laxatives when improved
  • An abdominal xray is NOT recommended to make the diagnosis of constipation
  • Do you have the right diagnosis? Irritable bowel is often confused with constipation.  With constipation, the pain is relieved after resolution of constipation.
  • Outlet dysfunction. Stimulant laxatives (eg. Senna) are probably underutilized. Biofeedback may help in older children.
  • Slow transit constipation. Newer prosecretory agents may be helpful –lubiprostone and linaclotide.
  • Organic constipation. Hirschsprung’s, Spina bifida, anorectal malformations etc. Testing: anorectal manometry, rectal biopsy (for Hirschsprung’s)
  • For refractory disease, consider rectal therapy –suppositories, transanal irritagations/enemas (~78% success for fecal incontinence/constipation). These treatments should be used prior to surgical therapy (eg. Malone antegrade continence enema/cecostomy)

 

 

The quest for the holy grail: Accurately diagnosing and treating extraesophageal reflux

Rachel Rosen   Boston Children’s Hospital

Key points:

  • It is frequent that EGD or impedance study will be abnormal, though this may not be causally-related.
  • No correlation with ENT exams/red airways and reflux parameters
  • No correlation with lipid laden macrophages and reflux parameters
  • No correlation with salivary pepsin and reflux parameters

Treatment:

  • Lansoprazole was not effective for colic or extraesophageal symptoms (Orenstein et al J Pediatrics 2009)
  • PPIs can increase risk of pneumonia/respiratory infections
  • Macrolides have been associated with increased risk of asthma but may be helpful for pulmonary symptoms
  • Fundoplication has not been shown to be effective for reducing aspiration pneumonia.  Fundoplication could increase risk due to worsened esophageal drainage.
  • ALTEs (BRUEs -brief resolved undefined events) need swallow study NOT PPIs

POTS and Joint Hypermobility: what do they have to do with functional abdominal pain?

Miguel Saps  University of Miami

Key points:

  • Patients with POTS and joint hypermobility have frequent functional abdominal pain as well as other comorbidities
  • Beighton Score can determine if joint hypermobility is present
  • Brighton Score determines if hypermobile Ehlers-Danlos syndrome is present
  • Patients with frequent fatigue.  Gradual progressive and regular exercise is important part of therapy.  Can start with recombant exercise – training bicycle exercise, swimming
  • Need to push salt intake and fluds

Do I need to test that C.R.A.P.?

Rina Sanghavi   Children’s Medical Center Dallas

This basic talk reviewed a broad range of issues related to functional abdominal pain.

Key points:

  • Carnett’s sign can help establish abdominal pain as due to abdominal wall pain rather than visceral pain
  • What is an appropriate evaluation?  Limited diagnostic testing for most patients.
  • Alarm symptoms include: Fevers, Nocturnal diarrhea, Dysphagia, Significant vomiting, Weight Loss/poor growth, Delayed Puberty, and Family history of IBD

Updated Pediatric Expert Constipation Guidelines

Updated guidelines for the diagnosis and treatment of constipation by NASPGHAN and ESPGHAN have undergone formal peer review are likely to be published soon (available online with the following link: bit.ly/1geLxrk).  “Evidence-Based Recommendations from ESPGHAN and NASPGHAN for Evaluation and Treatment of Functional Constipation in Infants and Children” by Merti Tabbers, Carlo DiLorenzo et al. The following are some of their recommendations.

Diagnostic recommendations:

  • The ROME III criteria are recommended for the definition of functional constipation for all age  groups.
  • Diagnosis of functional constipation is based on history and physical examination.
  • There is no role for the routine use of an abdominal X-ray to diagnose functional constipation.
  • A plain abdominal  radiography may be used in a child in whom fecal impaction is  suspected but in whom physical examination is unreliable/not possible.
  • Based on expert opinion, a 2-4 weeks trial of avoidance of cow’s milk protein may be indicated in the child with intractable constipation.
  • Routine laboratory testing to screen for hypothyroidism, celiac disease and hypercalcemia is not recommended in children with constipation in the absence of alarm symptoms. 

Treatment Recommendations:

  • We do not recommend the use of biofeedback as additional treatment in childhood constipation.
  • Polyethylene glycol (PEG) with or without electrolytes orally 1-1.5 gr/kg/day for 3-6 days is recommended as first-line treatment for children presenting with fecal impaction
  • An enema once a day for 3-6 days is recommended for children with fecal impaction if PEG is not available.
  • PEG with or without electrolytes is recommended as first-line maintenance treatment. A starting dose of 0.4 gr/kg/day is recommended and the dose should be adjusted according to the clinical response.
  • Addition of enemas to the chronic use of PEG is not recommended.
  • Based on expert opinion, use of milk of magnesia, mineral oil and stimulant laxatives may be considered as additional or second line treatment.
  • Antegrade enemas are recommended in the treatment of selected children with intractable constipation.

Related blog posts:

ACE report -10 year effectiveness

More data is now available on the use of antegrade continence enema (ACE) for difficult-to-treat defecation disorders (J Pediatr 2012; 161: 700-4).

This study reports the 10-year (retrospective) experience of a single center.  In total there were 99 patients, median age 8 years.  Mean time for followup was 46 months.  Most procedures were undertaken by interventional radiology with temporary  8.5 Fr Dawson-Mueller catheter which was changed to a Chait Trapdoor catheter after six weeks.  All patients received triple antibiotics for 48 hours and then oral metronidazole for 7 days.  While ACE were not started for 10-14 days, the tube was irrigated with 10 mL of normal saline BID after placement.

Key findings:

  • 71% became symptom-free, and an additional 20% improved significantly
  • Patient population: 35 with functional constipation, 29 with spinal abnormalities, 8 with cerebral palsy, 8 with Hirschsprung’s, 7 with imperforate anus, 7 with combined imperforate anus/tethered cord, and 5 with other causes
  • Irrigations with a stimulant seemed to be more effective.  Specific stimulants included bisacodyl (5 mg if patient <10 years and 7.5 mg if >10 years) and glycerine (median dose 37.5 mL).
  • In the 7 patients without improvement, the stoma was closed after a median time of 7 months & 5 ultimately underwent colostomy.
  • Risk factors for poor outcome: younger age, shorter duration of symptoms, Hirschsprung’s disease, cerebral palsy, previous abdominal surgery, and abnormal colonic manometry (Previous studies have not shown that preop manometry helpful in predicting outcome of ACE).
  • 13% of patients were able to discontinue ACE without recurrence of symptoms
  • Major complications occurred in 12 patients.  12 patients had infections, 4 had abscess, and 3 had peritonitis.  Minor complications were commonplace including leakage in 21 and granulation tissue in 41 patients.

I think this report does provide a balanced view of cecostomy/appendicostomy placement; this will be helpful in counseling families.  While the majority of children will be able to irrigate their colon and thus minimize their intractable constipation, many will have issues with the tube site and particularly early on there is a risk of serious infections.

Related blog entries:

Clues about constipation and more than 2.5 million views

Stimulants for constipation | gutsandgrowth

It’s worth the cost | gutsandgrowth

thyroid | gutsandgrowth

Additional references:

  • Percutaneous Endoscopic Colostomy (PEC) – YouTube (LEFT-SIDED PROCEDURE)

  • -J Pediatr Surg 2010; 45: 213-9.  ACE highly effective for intractable constipation
  • -JPGN 2011;52: 574.  n=117. 69% success with antegrade enemas.
  • -JPS 2002; 37: 348-51.  sigmoid irrigation.
  • -Lancet 1990; 336: 1217. Malone’s initial description.