Category Archives: Pediatric Gastroenterology Intestinal Disorder

Chronic Care Mode: GJ tube Data

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A lot has changed in the field of pediatric gastroenterology since I completed my training 16 years ago.  One technology that is used frequently now is the gastrojejunal (GJ) tube for feeding neurologically-impaired children.  Previously, GJ tubes were used as a temporary solution.  Part of the rational for short-term usage was that these tubes were often difficult to maintain; they could easily become dislodged or clogged.

A recent study documents the more frequent usage of GJ tubes and their indefinite usage to treat complex feeding issues (JPGN 2013; 56: 523-27).

This retrospective chart review encompassed a 10 year period (1999-2009) at a single academic center.  In total 33 patients were treated with GJ tubes with 160 placements.  The mean age at initial placement was 6 years and the mean weight 19.4 kg. 76% of the patients had cerebral palsy/neurologic disorder, 21% had congenital heart disease, and 9% had chronic lung disease.

Common indications for replacement: dislodgment, obstruction, coiling into stomach, and broken tubing.

Three techniques were used:

  1. Fluoroscopy with guide wire and subsequent GJ
  2. Gastroduodenscopy via gastrostomy site to place guidewire for GJ placement
  3. Tube placement during esophagogastroduodenoscopy

Most procedures (85%) did not require sedation.

Outcomes:

  • 13 (39%) maintained on GJ throughout study period
  • 10 (30%) converted to gastric or oral feeds
  • 5 (15%) surgical intervention
  • 5 (15%) deceased
  • Duration of tube survival: mean 91 days for Mic-Key GJ (low profile) and 177 days for  coaxial PEG-PEJ (e.g. 16 French Corflo gastric tube with 6 French jejunal tube)

When reading the study, it is hard to ignore Figure 3 which shows more than 30 placements per year after 2007 whereas the number was about two per year before 2001. In the discussion, the authors do not focus on how this technology has been embraced so widely.  It is mostly a discussion on the indications, methods, and complications.  Indications included high aspiration risk, intractable vomiting, failed Nissen fundoplication, and gastroparesis.  “Our study showed that long-term jejunal feeding via GJ tubes is possible and safe.”

My preference is generally to avoid GJ feedings as a primary intervention for long-term feeding problems.  That is, when patients need gastrostomy tube feeds but are prone to vomiting, most often a fundoplication is worthwhile.  When a patient has had a fundoplication that is no longer effective, a GJ tube should be considered.

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Spina Bifida, Humility, and Quality of Life

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While a recent article (J Pediatr 2013; 162: 993-98) on spina bifida and its effect on quality of life provide useful information, the accompanying editorial by John Freeman (J Pediatr 2013; 162: 894-95) had much broader implications for me.

The referenced article provides data showing the effects of spina bifida on quality of life QOL), even in those who reported their current health as good.  The tools that the investigators used for 40 youth and 13 young adults were the HUI-3 and AQoL.  Both of these tools correlated health-related QOL (HR-QOL) and the level of the anatomic lesion; those with thoracic/higher lesions had worse HR-QOL.

Some of the points made by Dr. Freeman:

  • Quote at start: “Health: A state of complete physical, mental, and social well-being and not just the absence of sickness or frailty.” –World Health Organization
  • In 2004, he followed up with two patients who he had presumed to be quite successful.  Both “were living at home, wheelchair-bound, with high paraplegias.  Both were community college graduates and employed….When I asked them what I should tell parents delivering a newborn with spina bifida, each replied, ‘I wish I had never been born.’ I was shocked.”
  • He notes that the referenced article had a low participation rate (39%) which could introduce significant biases.  “Were the respondents those with greater or less disability?”
  • Conclusion: “I will not presume to have answers to these questions but propose further discussion of the quality-of-life issues…on decisions regarding early treatment of spina bifida.”

These studies make it clear that some children with spina bifida, particularly those with higher lesions, have poor HR-QOL.  The broader implication is that many children/adults who we think are doing pretty well do not see it that way.  Trying to determine what the future holds for children with chronic health problems in infancy is difficult.  Even in the most severe diseases, most parents want everything done.  Will this type of information change anything?

“Family Feud” for Pediatric Crohn’s Abscess Management

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Initially, this blog entry was titled “What is the best way to manage an intra-abdominal abscess in pediatric Crohn’s disease?”  My answer was simple: it depends on who you ask (Inflamm Bowel Dis 2013; 818-25).  As I thought about this study, the responses reminded me of “Family Feud” minus Richard Dawson.

This survey examined the responses of NASPGHAN members.  Of the initial 1608 emails which were delivered, 248 fully completed surveys.  25% of respondents were in practice for >20 years and 26% treated >50 patients with Crohn’s disease (CD). 87% of respondents were from U.S.

  • What is the best way to image initially?  52% recommended CT scan, 26% MRI, and 21% ultrasound.
  • What is the best modality for followup imaging? 47% ultrasound, 33% MRI, and 13% CT
  • Antibiotics or drainage for abscess <2 cm? 61% recommended antibiotics; 51% would treat for 3-4 weeks, whereas 19% for 1-2 weeks.
  • Antibiotics or drainage for abscess >2 cm? 28% would attempt antibiotics alone
  • When is surgery indicated? 75% said only in select cases after completing antibiotics and interventional radiology drainage.
  • Anti-TNFα therapy?  The survey also questioned the shortest preoperative interval one would prescribe anti-TNFα therapy.  The results ranged from 12% for <1 week to 45% who would not give anti-TNFα therapy at all.

The authors note that there is “a paucity of research and practice guidelines for the optimal management of children with intra-abdominal abscess.”  There were no trends in management identified based on practitioner level of experience.  Some answers to the questions are alluded to by the authors but not expressed definitively. For example, “several studies have reported a lack of association between infliximab and an increased rate of postoperative complications.” “Most infections that occur while on anti-TNFα therapy tend to be opportunistic, not bacterial.”

The study’s conclusions are limited by the low participation rate.  In addition, when physicians are confronted with a specific situation, their response in practice may be different than in a theoretical scenario.  However, it appears that the answers to these important questions are closer to guesses on a game show rather than best care.  More research and collaboration is needed to reduce this highly variable care and determine the most effective approach.

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Norovirus Impact on Young Children

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As noted in a previous blog (Norovirus -now more important than rotavirus | gutsandgrowth), Norovirus has become the most important cause of gastroenteritis in children younger than 5 years.  More data to back up that claim has been published (NEJM 2013; 368: 1121-30).

The authors examined laboratory-confirmed cases of norovirus in children younger than 5 years with acute gastroenteritis in hospitals, emergency departments, and outpatient clinical settings during the years 2009 and 2010.  Using the New Vaccine Surveillance Network (NVSN), the authors undertook a 2-year prospective population-based survey with a catchment population of more than 141,000.  The specific sites included county populations around the University of Rochester, Vanderbilt University, and Cincinnati Children’s.

Results:

  • Norovirus was detected in 21% of children with acute gastroenteritis (2009-2010); it was also detected in 4% of healthy controls.
  • The age group with the highest rates of norovirus infection in this study were 6-18 months of age.
  • The GII.4 Minerva strain was most predominant strain in 2009 and GII.4 New Orleans in 2010. (In 2012, a novel GII.4 Sydney variant has emerged).
  • Rotavirus was identified in 12% of children with acute gastroenteritis (2009-2010).
  • Using this data, the authors extrapolated national estimates (for norovirus) of 14,000  hospitalizations per year (in this age group), 281,000 emergency room visits, and 627,000 outpatient visits.
  • The estimated costs exceed more than $273 million.

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Predicting duration of reflux symptoms in babies

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A recent study identified two factors on multichannel intraluminal impedance pH monitoring (MII/pH) that correlated with the duration of gastroesophageal reflux symptoms in newborns (J Pediatr 2013; 162: 770-5).

This study examined 64 newborns who underwent MII/pH in the first weeks of life and then were enrolled in followup at 1, 3, 6, 9, 12, 18. 24, and 36 months. 53 patients completed the three-year study.

These patients were enrolled consecutively.  All preterm infants had to have a minimum postmenstrual age of 36 weeks.  Other criteria included a MII/pH study with a minimum duration of 19 hours, absence of GERD pharmacology for at least 1 week, and absence of infection, metabolic disease or central nervous system disease.

Results:

  • Impedance bolus exposure index (IBEI) and proximal reflux frequency positively correlated with duration of GERD symptoms.
  • IBEI was 1.45 in the short duration group (0-3 months), 1.85 in the medium duration group (4-9 months) and 2.46 in the long duration group (> 9 months).
  • Proximal reflux frequency (events/hour) was 1.56 in the short duration group (0-3 months), 1.95 in the medium duration group (4-9 months) and 2.38 in the long duration group (> 9 months).
  • Overall, one-half of patients were asymptomatic within the fifth month of age and the vast majority were asymptomatic by one year of age.
  • Weakly acidic events but not acid reflux events were significant in determining the differences in IBEI and proximal reflux.  As such, this study adds weight to the idea that acid blockers have little benefit in the first months of life.

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Global increases in IBD incidence

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Two more studies have shown increasing incidence of pediatric inflammatory bowel disease.

First in Victoria, Australia (mostly Melbourne) (Inflamm Bowel Dis 2013; 19: 1-6).

Over a 60-year span (1950-2009), a retrospective review was undertaken of ulcerative colitis (UC) pediatric patients. In total, 342 children were diagnosed with UC. Key finding: The number of reported cases increased by 11-fold during the study period with a marked increase since 1990 (0.15 –>1.61/100,000).  In addition, recently diagnosed children have had more extensive disease.

Next in Spain (Inflamm Bowel Dis 2013; 19: 73-80).

This retrospective study from hospitals’ databases looked at the incidence between 1996-2009 in the pediatric population (<18 years).  A total of 2107 patients were identified: 1165 with Crohn’s disease, 788 ulcerative colitis, and 154 IBD unclassified. Median age at diagnosis was 12.3 years.  Key finding: in the last 14 years, pediatric IBD incidence has almost tripled (0.9 –>2.8/100,000).

Both of these studies have limitations related to large retrospective reviews in terms of potential problems with capturing all of the patients and the potential for misdiagnosis.  However, the trend is clear.  In addition, these studies show incidence rates comparable to several other Western studies.  The increasing incidence of IBD ‘argue for a common environmental factor in their pathogenesis.’  While interest has focused on microbial factors, the basis for this increased incidence currently remains elusive.

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Nuance in Celiac Serology Interpretation

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A recent study adds additional nuance to the interpretation of celiac serology (Clin Gastroenterol Hepatol 2013; 11: 398-403).

In this study the authors analyzed anti-TTG IgA levels from 104 consecutive pediatric and adult patients who were not IgA deficient.  The study took place between 2000-2009.  In addition, samples from 537 consecutive controls were available for comparison.

The study determined the likelihood of having celiac disease based on antibody level from four different companies and pre-test clinical factors.  The general population pretest probability was 1%; the pretest probability for their population was 6% if their were gastrointestinal complaints, 14% if weight loss/small stature was present, 11% for patients with anemia/iron deficiency, and 9% for patients with malabsorption.

Key findings:

Even in those with high antibody titers (>10-fold normal), if they were asymptomatic, only 53%-75% had celiac disease (depending on the individual assay).  That is, >10-fold elevation with some commercial assays did not correspond to >10-fold elevation in all of the assays leading to variable probabilities.

In patients with low level elevations (1-3 fold times the cut-off level), the probability of having celiac disease in asymptomatic individuals varied from 1% to 7%.  Thus, mild elevations in TTG IgA are not highly predictive in this asymptomatic population.  However, in those with pretest probability of 14%, the frequency of celiac disease varied among the four assays from 14% to 56%.

Take home message:  Not all assays for celiac disease are comparable.  While very high serology levels (>10 fold) are associated with celiac disease, in asymptomatic patients as few as 53% may have celiac disease.

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Button Battery Algorithm Link

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The following website has a useful button battery algorithm (thanks to JL for forwarding this link):

One important point is that in an asymptomatic patient, a button battery in the stomach can be left alone if no coingestion with a magnet.  If gastric battery is > 15 mm and child is less than 6 year,  the recommendation is to check xray in 4 days and remove if still in stomach or whenever there are symptoms.
Of course, a button battery in the esophagus is a true endoscopic emergency. Recognizing that it is a button battery and not a coin can alter the outcome.
Related blog links:
Another link on foreign bodies, 2011: Postgraduate Course Syllabus – Slides, only – NASPGHAN.org (Look for the slides from Marsha Kay)

Fish Oil, IFALD, and Liver Fibrosis

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While there has been a lot of enthusiasm for the use of fish oil as a potential breakthrough for intestinal failure-associated liver disease, this has been based largely on the use of surrogate markers of liver disease and based on comparisons with the historical use of conventional intravenous lipids.  The latter problem has been discussed before on this blog (see links below).

A recent study begins to address the issue of surrogate markers by reinforcing the viewpoint that improvements in bilirubin and aminotransferases may not translate into improvement in liver fibrosis or other clinically-meaningful outcomes (JPGN 2013; 56: 364-69).  A previous pediatric study (J Pediatr 2010; 156: 327-31) showed failure of regression of hepatic fibrosis in 2 children receiving FOE therapy.

In this study, the authors sequentially examined 6 children on fish-oil lipid emulsions (FOE) who underwent multiple liver biopsies.  In this cohort, 5 of 6 children had gastroschisis and the mean gestational age was 35 weeks.  Median intestinal (small bowel) length beyond the ligament of Treitz was 26 cm and most children retained about 2/3rds of their colon.  Liver biopsies were obtained at the time of other open abdominal operations (eg. serial transverse enteroplasty, stoma takedown).

Key results:

  • Liver fibrosis persisted in 2 cases, progressed in 3 cases, and regressed in 1 case.
  • Histology and biochemistries indicated improvement in cholestasis and inflammation.
  • One patient has weaned off parenteral nutrition, two patients underwent isolated small bowel transplantation due to recurrent line infections, and three patients receive 25-40% of their calories parenterally.

The biggest limitation of this study besides the small number of enrolled patients was the relatively short  time period that was studied.  Only one patient who was studied had data reported for FOE more than 36 weeks.  The oldest age of any patient at the time of their last biopsy was 131 weeks old.

Take-home points:

  • “There is no direct evidence to support any one [proposed theoretical benefit of FOE] as clinically meaningful as yet.”
  • “Lipid minimization strategies are also effective in reducing cholestasis.”
  • “Many of the biopsies taken right at the time of FOE initiation” showed significant fibrosis which “speaks to how quickly fibrosis can develop.” One child had stage 2 fibrosis at 14 weeks of life.
  • “The biochemical resolution of cholestasis is at best a weak surrogate marker ..for…enteral independence and overall survival.”  “These findings make a strong case for early referral of children with short bowel syndrome to specialized intestinal rehabilitation centers.”

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True red flags in recurrent abdominal pain

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For pediatricians and pediatric gastroenterologists alike, identifying which children need additional workup for recurrent abdominal pain (RAP) is facilitated by recognizing “red flags.”  “Red flags” are clinical features that indicate a higher likelihood of a nonfunctional disorder.  A recent study notes that reports of waking from sleep and joint pains do not distinguish functional from nonfunctional causes of RAP (J Pediatr 2013; 162: 783-7).

This study, performed between 2005 to 2008, had patients presenting to an outpatient pediatric gastroenterology clinic for RAP prospectively complete a detailed questionnaire. Data, though, was extracted retrospectively. In this population (n=606), 85% were Caucasian.  After their evaluation, patients with functional GI diseases (FGID, n=478) were compared with patients confirmed with Crohn’s disease (CD, n=128).  All FGIDs underwent biochemical testing, 41% had upper endoscopy, and 32% underwent colonoscopy.

Additional key findings:

  • Using a tree analysis, the cumulative sensitivity for Crohn’s disease was 54% with the presence of anemia, 78% when blood in stool was added to anemia, and 94% when weight loss was added as well.
  • FGID patients were more likely to report stress and headaches, more likely to have family history of FGID, and less likely to have anemia, hematochezia, or growth issues.
  • FGID patients were more likely to experience vomiting.

The sensitivity and specificity of these symptoms/signs will vary based on the population.  For a general pediatric clinic, it is likely that the sensitivity of these red flags would remain high; the specificity would likely be lower than in a pediatric gastroenterology office due to the increased prevalence of functional diseases in the general pediatric setting.

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