Long-term Outcomes with Pediatric PEG Placement

As noted about a week ago in this blog, gastrostomy tube (gtube) placement in children is much different from gtube placement in adults.

A retrospective study from Boston Children’s followed 138 patients who had PEG tube placed between 1999-2000 (JPGN 2013; 57: 663-67).  The median followup was approximately 5 years.

Results:

  • Median time to elective tube removal was 10.2 years.
  • ~50% of patients continued with gastrostomy tube 10 years after placement.
  • 11% (n=15) had at least 1 major complication related to gastrostomy placement.  Major complication was defined as any unplanned adverse events requiring hospitalization, surgery (eg. fundoplication) or interventional radiology (eg. gastrojejunal tube placement). Most major complications occurred during the first 6-12 months following placement with the most common being cellulitis (n=10).
  • 18% of the cohort died during the 10-year study period because of non-gastrostomy-related issues.  No deaths were attributed to gastrostomy tube placement.

Bottomline: The need for gastrostomy tube placement is associated with frequent comorbidities.  A significant number of patients undergoing gastrostomy tube placement experience major complications.

Also noted:

JPGN 2013; 57: 659-62. This prospective study of 69 patients showed that early reintroduction of feedings after gastrostomy placement, 4 hours postoperatively, was safe and compared favorably to those fed 12 hours postoperatively.  Early feedings were associated with hospital duration, on average, of 6.7 hours. At this center, prophylactic antibiotics were not administered without apparent increase in infections.

JPGN 2013; 57: 668-72. This retrospective study of 77 children with feeding disorders showed that inpatient behavioral interventions are effective in transitioning children from gastrostomy tube feeding to oral feeding.

Related blog entries:

Microscopic, Lymphocytic and Collagenous Colitis

Microscopic Colitis (MC) is a rare pediatric problems and occurs when chronic diarrhea occurs in the presence of a normal-appearing endoscopic exam but with abnormal histology.  In adult populations, microscopic colitis is seen more frequently and can be confused with irritable bowel syndrome.  The two subtypes:

  • Lymphocytic Colitis (LC):  >20 intraepithelial lymphocytes/100 colonocytes
  • Collagenous Colitis (CC): thickened subeptihelial collagen band in addition to changes seen with LC

In a recent study (JPGN 2013; 57: 557-61), 27 MC cases were identified from a pathology database between 1995-2011.  5 were excluded due to an enteric infection.  Among the 22 other cases, 19 had LC and 3 had CC.  Association with celiac disease was evident in 4 patients and many had preceding drug exposures.

Treatment included steroids, melamine, an bismuth.

Additional references:

  • -JPGN 2011; 53: 579. lymphocytic colitis case report
  • -Clincal Gastro & Hep 2011; 9: 13.  Celaic with persistent symptoms: consider poor adherence**, SBBO*, pancreatic insufficiency*, refractory celiac (rare), PLE, giardia, malignancy, lactose intolerance, functional d/o*, microscopic colitis, Crohn’s*, NSAIDs
  • -Gut 2009; 58: 68-72. Collagenous colitis: Budesonide at 6mg/day maintained remission in ~25%.
  • Gastro 2008; 135: 1510.  Budesonide effective for collagenous colitis; n=48, 9mg/day.
  • -Gastro 2011; 140: 1155. Review of microscopic colitis/collagenous colitis.
  • -Am J Gastroenterol 2010; 105: 859-865.  n=466 & 451 controls.  Microscopic colitis present in 1.5% of IBS patients.  IBS pts with lower incidence of adenomas (7.7.% vs 26%).  9% had diverticulosis (lower).
  • -Clin Gastro & hep 2009; 7: 1210. 4.3% of pts w microscopic colitis had celiac. 44/1009.

Family Resource for IBD

In cooperation with ImproveCareNow, EmpoweredByKids.com, which was started by a group of parents, developed ‘The Book of Hope.’ This book “contains stories of hope and inspiration from parents and patients of Inflammatory Bowel Disease.  These are encouraging stories to let families know someone else has been there too and you are not alone.”

You can download a copy of the IBD Book of Hope here.

I read through the downloaded version.  In my view, the general theme was of perseverance.  Given some of the difficulties shared in the book, this book might be best when someone is hospitalized and/or very sick rather than at the onset of diagnosis.

What do you think?

Additional family resource:

Free Self Management Handbook endorsed by ImproveCareNow:

https://improvecarenow.org/patients/self-management-handbook

Related Blog Posts:

Predicting Remission in Pediatric Ulcerative Colitis

Trying to offer realistic information to families on the long-term success of infliximab (Remicade) for pediatric ulcerative colitis has been difficult due to a limited amount of data.  In addition, many studies have a number of limitations which can make it difficult to extrapolate to a less-selected population (According to the study which you would never qualify for …).

A recent post hoc analysis (Clin Gastroenterol Hepatol 2013; 11: 1460-65) from 51 children (age 6-17 years) provides some insight into this issue.  The authors used data collected prospectively during the T72 clinical trial (Clin Gastroenterol Hepatol; 10: 391-99 -reviewed in blog post: Infliximab for children with Ulcerative Colitis | gutsandgrowth).

Results:

  • Week 8 PUCAI scores best predicted which patients would be in steroid-free remission after 1 year of treatment: 9 of 17 (53%) with scores <10 points were in sustained remission compare with 4 of 20 (25%) with scores ≥10.
  • Week 8 PUCAI outperformed mucosal healing in predicting remission.

Key points:

  • PUCAI may have outperformed mucosal healing because the latter can lag behind clinical response.  Furthermore, mucosal healing is more subjective with less interobserver reliability.
  • In patients with known inflammatory bowel disease, endoscopic assessment remains important in several situations: condition or reason for symptoms is in question (eg. exclude irritable bowel symptoms or medication-induced symptoms), acute severe colitis not responding to 3-day treatment with intravenous steroids to exclude superinfection, and to assess mucosa before major treatment changes “such as when starting or stopping biologics and before referral for colectomy.”

Related blog posts:

Trends in Clostridium difficile Infection

Many recent reports have provided conflicting data with regard to Clostridium difficile infection (CDI) epidemiology.  Some of the newest data needs to be interpreted with caution due to the adoption of PCR technology.  Previously, CDI was difficult to culture and identify.  The problem now is proving causation when C diff is identified.

J Pediatr 2013; 163: 699-705.  This study, using an administrative database, analyzed 33,095 first pediatric hospitalizations for malignancy from 43 pediatric hospitals between 1999 and 2011.  A total of 1736 admissions with CDI were identified; 380 were considered hospital-acquired.  The authors noted an apparent decrease in CDI incidence between 2006-2010.  Exposure to chemotherapy, proton pump inhibitors and certain antibiotics were independent risk factors for hospital-acquired CDI.

JPGN 2013; 57: 487-88. New-onset patients with IBD cases were retrospectively reviewed from 2010-2012.  10 cases (8.1%) of 124 were positive for CDI within the first two months of diagnosis.  Only 42% of the total 290 new IBD cases had documented testing for CDI.  The prevalence of CDI without obvious preceding antibiotic exposure was 2.4%.

JPGN 2013; 57: 293-97. Between 2006-2012, stool samples were prospectively obtained from children with IBD (UC, n=76, Crohn, n=69) and controls with other noninflammatory GI conditions (n=51).

Key points:

  • The prevalence of positive PCR results were 11.6% in patients with Crohn disease, 18.4% in patients with UC, and 11.8% in controls.  No significant difference.
  • CDI as identified by PCR may be an incidental finding.
  • Only test diarrheal stools.  Testing for cure is not recommended.
  • Asymptomatic colonization with C diff is frequent in patients with and without IBD

Related blog posts:

“Poo in You” Video

New “Poo in You” education video for constipation / encopresis available through YouTube channel: http://www.youtube.com/NASPGHANencopresis

I took a look at this 5 minute video and it explains why kids soil and the basics of treatment; in addition, there is a “wah-wah-wah” sound effect at 2:29 in video when soiling occurs.  Probably worth including this link on an after visit summary:

Related blog posts:

Constipation Guidelines:

Endoscopy Module -Postgraduate Course Notes

Advances in Hemostasis for Upper GI Bleeding —Brad Barth, MD, MPH  (page 77)

Upper GI Bleeding

Effect of IV PPI on patients with UGI bleeding PRIOR to EGD

  • 6 trials including 2223 patients
  • No significant difference in mortality, rebleeding or need for surgery compared to controls
  • DID significantly reduce rates of high risk stigmata identified on EGD
  • DID significantly decrease the need for endoscopic therapy
  • Reference: Sreedharan A, Martin J, Leontiadis G, et al. Proton pump inhibitor treatment initiated prior to endoscopic diagnosis in upper gastrointestinal bleeding. Cochrane Database of Systematic Reviews 2010

 

Upper GI Bleeding – Proton Pump Inhibitors/Prokinetics

  • Omeprazole 1 mg/kg q 12 hours  (Solana, et al. J Pediatr 2013:162:776-82)
  • Proposed PPI drip dose: 1 mg/kg bolus followed by 0.1 mg/kg/hour infusion
  • IV erythromycin or metoclopramide; infuse 20-120 minutes prior to endoscopy in patients with acute UGIB; decreased need for repeat endoscopy to determine cause and site of bleeding. Prokinetic did NOT affect transfusion requirements, duration of stay, need for surgery. Reference: Barkun et al. Prokinetics in acute upper GI bleeding:a metaanalysis. GIE 2010;17:126-132

Upper GI Bleeding —Other points:

  • Epinephrine alone is RARELY enough
  • Non bleeding adherent clot has 8-35% chance of rebleeding in adults. Consider removing it CAREFULLY!
  • A conservative transfusion strategy is usually appropriate

Useful References

Surveillance Endoscopies: The established, the debated, and the unknown –Mitchell Shub, M.D. (page 85)

“Beware of false knowledge; it is more dangerous than ignorance.” —George Bernard Shaw

Familial Adenomatous Polyposis

Surveillance protocol: Age of initial evaluation/Type of procedure/Frequency

  • Colon 10 – 12 y of age (Sooner: family h/o aggressive disease) -Flex sig or Colonoscopy, 1 – 2 y
  • Upper GI tract 20 – 25 y or at initial colonoscopy
  • EGD and side viewing scope, 1 – 3 y
  • Post-colectomy (pouch) 6 – 12 mo. after surgery, Flex sig 1 y (6 mo. If retained rectum)
  • Small bowel: capsule or MRI, frequency unknown

Peutz-Jeghers Syndrome: begin screening at age 8 years or when symptomatic with colonoscopy, EGD, and small bowel imaging (?capsule vs alternatives); then every 2-3 years

Juvenile Polyposis Syndrome: begin screening at age 10-15 years or when symptomatic with colonoscopy, EGD, and  possibly small bowel imaging (?capsule vs alternatives); then every 1-3 years

Discussed guidelines for IBD cancer surveillance and for Barrett’s esophagus

  • For UC, start surveillance 8-10 years after diagnosis.
  • For Crohn’s with ~1/2 colon (or more) involvement, follow same guidelines
  • For coexisting PSC, annual surveillance
  • Barrett’s esophagus in children: adenocarcinoma very rare, evidence lacking to develop surveillance schedule

Expanding the view: Update on Upper GI Strictures —Mark A. Gilger, M.D. (page 95)

Why balloons for kids (for dilatation)?

You can see what you’re doing

  • Blind pouches
  • Abnormal mucosa
  • Caustic injury
  • Epidermolysis bullosa
  • Already requires general anesthesia
  • Ability to wire through narrow strictures
  • Ability to use radiographic assistance

Tip: Can use vegetable spray (eg. Pam) to make advancement of balloon catheter easy

How to do balloon dilation

  • Inflate balloon to ½ desired initial atmospheres & re‐check placement
  • Begin dilation at to 1‐2 mm more than initial estimated stricture diameter
  • Hold for 1 minute/dilation
  • •ove balloon catheter in and out during dilation;  if balloon moves freely, increase diameter by 1mm.  If stricture moves with the balloon, hold x 1 minute, then done
  • Oh, oh, there’s blood! Good! No blood, no dilation.
  • After dilation, carefully advance endoscope through the stricture; if resistance stop, can try cork‐screw maneuver
  • Document everything; especially stricture location (CM from incisors), dilation diameters (to help you next time)

Adjunct therapy for recalcitrant strictures  –adjunct therapy to sustain dilation needs further study

  • Oral & intravenous corticosteriods
  • Injectable corticosteroids – Thins the mucosa, OK 1‐2 times, but not repeated
  • Mitomycin C
  • Acid reduction
  • Stents

Endoscopy in the high‐risk patient: Keeping your patient safe —Jenifer R. Lightdale, MD, MPH (page 63)

Safety of Pediatric GI Procedures

  • Peds‐CORI data from >10,000 procedures
  • Overall rate of complications 2.3%: risk of hypoxia 1.5%; risk of bleeding 0.3%

Examples of pediatric populations at increased risk for perforation

  • History of caustic ingestion
  • Esophageal atresia/tracheo‐esophageal fistula
  • Severe duodenitis
  • Severe ulcerative colitis
  • Patients with multiple co‐morbidities (i.e. Type I diabetes, cerbrovascular disease, peripheral vascular disease, renal insufficiency, liver disease)
  • Ehlers‐Danlos Syndrome (Vascular Type)

Pre‐procedure Assessment –lends itself to a checklist 

Thrombocytopenia -Current recommendations

  • EGD ok if platelets >20,000/mL
  • Biopsies ok if platelets >50,000/mL

Bleeding –discussed high risk conditions

Decreasing Risk of Perforation:

  • Avoiding excessive pressure
  • Avoiding premature cutting of a polyp – Coagulate before cutting
  • Avoiding blind intubation of the lumen

Decreasing Risks of Infection

  • SBE Prophylaxis– generally NOT indicated in diagnostic procedures. Congenital heart disease is complex & may be needed on a case‐by‐case basis
  • Single‐dose cephalexin has been shown to decrease peristomal infection during PEG placement
  • Prophylactic antibiotics recommended for cirrhotic patients admitted with GI hemorrhage

Postgraduate Course Syllabus (posted with permission) with complete slides of above lectures: PG Syllabus

Related blog references:

Disclaimer: These blog posts are for educational purposes only. Specific dosing of medications (along with potential adverse effects) and specific medical management interventions should be confirmed by prescribing physician.  Application of the information in a particular situation remains the professional responsibility of the practitioner.

Postgraduate Course Notes -Pancreatitis Module

When and how to assess pancreatic function: an update for clinicians –Sohail Z. Husain, MD (page 31)

Reviewed methods of detecting pancreatic insufficiency

Indirect (non-stimulatory) Methods

Stool:

  • Fecal Fat Analysis: Coefficient of fat absorption (CFA): (fat intake – fat in stool / fat intake) * 100
  • Normal > 93% (> 85% in less than 6 mo.
  • old)
  • 72 hr collection gold standard

ELASTASE-1: Stable, specific for human pancreas

  • Normal > 200 μg elastase/g stool
  • Particularly good for monitoring the development of PI in patients with CF
  • Low levels (false-positive) with diarrhea
  • Only detects severe PI

Other tests

  • Chymotrypsin: less sensitive; requires discontinuation of enzymes
  • Steatocrit: cheap; has low sensitivity
  • Serum
  • Breath
  • Direct (stimulatory)
  • Dreiling tube
  • Endoscopic pancreatic function testing (ePFT)
  • Secretin-enhanced MRCP (sMRCP)

Causes of Pancreatic Insufficiency

-85% of patients with Cystic Fibrosis have pancreatic insufficiency

Shwachman-Diamond Syndrome

  • Mutation in SBDS, found in ~90% of SDS patients
  • PI affects almost all SDS pts

Johanson-Blizzard syndrome (JBS): Key findings

  • PI
  • Severe developmental delay
  • Hypoplasia or aplasia of the nasal wings

Pearson marrow pancreas syndrome Key findings: Severe hypoplastic,macrocytic anemia,  Pancreatic insufficiency (due to pancreatic fibrosis)

Diagnosis: Clinical picture, High serum lactate/pyruvate,  Southern blot for mtDNA rearrangements

Other causes of pancreatic insufficiency

  • Chronic pancreatitis
  • Pancreatic obliteration after severe, acute pancreatitis
  • Pancreatic tumors
  • Celiac disease
  • Diabetes
  • IBD

Managing nutrition in cystic fibrosis: the role of the pediatric gastroenterologist — Sarah Jane Schwarzenberg, M.D. (page 41)

Good nutrition status correlates with better heights, better lung function, and better survival.  (Presentation did not delve into the issue of potential reverse causation.)

  • Patients with a Weight-for-Age percentile >50% at age 4 years reached a much higher height-for-age early in life and maintained this advantage into adulthood
  • Pulmonary function (FEV1%predicted) was much lower in CF patients with WAP<10% at age 4 years. This finding tracked through age 18 years.
  • Small bowel overgrowth is common in CF
  • Small bowel bacterial overgrowth contributes to poor nutritional intake and increased nutrient losses

Options to improve nutrition in CF

  • Review and optimize enzyme dose and adherence
  • Review patient’s diet with an experienced CF dietician
  • Consider adding a PPI to improve intestinal pH
  • Consider confounding disease
  • Evaluate for signs and symptoms of small bowel overgrowth and consider trial of metronidazole or rifaximin
  • Ask patient about abdominal pain
  • Evaluate for gastroparesis
  • Evaluate for DIOS
  • Consider non-CF gastrointestinal disease
  • Consider oral glucose tolerance test

Therapy to improve nutrition

  • Time-limited interventions
  • Behavior therapy to improve intake
  • Offer oral supplements
  • Consider cyproheptadine as an appetite stimulant
  • Consider a G-tube for nocturnal feeds
  • Consider Endocrinology consult

Beyond the Basics in the Management of Pancreatitis  –Aliye Uc, M.D. (page 51)

INSPPIRE To Study Acute Recurrent and Chronic Pancreatitis in Children-180 children from 14 centers enrolled to study the etiologies, epidemiology, natural history and outcome.

Pediatric Acute Recurrent and Chronic Pancreatitis-etiologies

  • Genetic (49%) (61 of 91 tested)
  • PRSS1-30%, CFTR-22%, SPINK1-14%, CTRC-3%
  • Obstructive (34%)
  • Idiopathic (20%)
  • Toxic-Metabolic (17%)
  • Autoimmune (3%)

Genetics of Pancreatitis:

1. PRSS1 (cationic trypsinogen): Autosomal dominant, 80% penetrance, Mutations are due to increased activation or decreased inactivation of trypsin (i.e. R122H, N29I).

2. SPINK1 (trypsin inhibitor): Autosomal recessive/complex inheritance, 2% have mutation, <1% have pancreatitis (i.e. N34S), Pancreatitis is dose-related (homozygous>>>het), Associated with other mutations (CFTR)

3. CFTR (>1700 mutations):

  • 2 Severe mutations = Cystic Fibrosis
  • 1 severe, 1 mild mutation = mild or atypical CF, ARP, CP
  • CF carriers = 3-4 fold increase risk in pancreatitis.
  • 1 any +SPINK1 = CFTR-associated pancreatitis
  • 1 any +divisum = CFTR-associated pancreatitis

4.New Modifier Genes in ARP and CP

  • CTRC (trypsin degrading enzyme)
  • CASR (a calcium-sensing receptor)
  • CLDN2 (tight junction protein on X chromosome)
  • CPA1 (Carboxypeptidase 1)  increased riskf for CP in younger patients

Management:

  • Unclear if antioxidants helpful for pain.
  • The role of pancreatic enzymes in CP is equivocal.

Diet

  • When to start feeds? depends on the severity of AP, OK to start early; correlate with clinical readiness, abd pain
  • What mode of nutrition? prefer enteral over TPN, NG vs. NJ
  • What to feed?  recent studies in adults with mild AP support full diet

(Moraes JM et al. J Clin Gastroenterol 2010 44:517)

No evidence that low-fat diet is helpful

IV Fluids: With acute presentation, Lactated Ringer’s preferred over Normal saline.

NG Suction

  • Not shown to decrease symptoms,mortality or hospital stay.
  • May be useful if: severe gastric distention, refractory nausea and vomiting, or obstruction seen on abdominal x-ray

IBD References 10/13

Recent useful references:

Inflamm Bowel Dis 2013; 19: 2490-2500.  “Endemic Fungal Infections in Inflammatory Bowel Disease Associated with Anti-TNF Antibody Therapy”

  • Reviews histoplasmosis, blastomycosis, & coccidioidomycosis. Provides endemic maps (which are available at CDC website), diagnostic tips, and treatment recommendations.  Of these three infections, blastomycosis is endemic in Northern Georgia.
  • Histoplasmosis can be diagnosed with urinary antigen, Blastomycosis is most commonly diagnosed with sputum cultures or bronchial washings for cytology, and coccidioidomycosis can be identified with serology (Coccidioides immittis)
  • Generally a good idea to get a chest radiograph in patients with respiratory symptoms, fever, chills, myalgias, and headaches.
  • CDC Fact Sheet – Centers for Disease Control and Prevention  Map for several endemic fungal diseases, including histoplasmosis and blastomycosis.
  • CDC Features – Valley Fever: Awareness is Key Map for endemic coccidiomycosis.

Inflamm Bowel Dis 2013; 19: 2457-2463. “Efficacy and Safety of Natalizumab in Crohn’s Disease Patients Treated at 6 Boston Academic Hospitals”

  • 44 of 64 with adequate evaluation had either a partial or complete clinical response.  In this select group of complicated patients, about one-third had clinical improvement for more than a year.
  • No cases of PML noted in this cohort.

Inflamm Bowel Dis 2013; 19: 2433-2439. “Serum IL-17A in Newly Diagnosed Treatment-Naive Patients with Ulcerative Colitis Reflects Clinical Disease Severity and Predicts the Course of Disease”

  • Mucosal mRNA expression of IL-17A was 99.8 times higher in ulcerative colitis patients compared to controls.
  • Serum IL-17A correlated with clinical disease severity and was a marker for disease course over the following 3 years.

Inflamm Bowel Dis 2013; 19: 2440-2443. “Assessment of the Relationship Between Quality of Sleep and Disease Activity in Inflammatory Bowel Disease Patients”

  • Data found an association between poor sleep quality and disease activity.  Furthermore, patients in clinical remission with abnormal sleep have a high likelihood of subclinical disease activity (another question for the EPIC smartform?).

Inflamm Bowel Dis 2013; 19: 2423-2432. “Nationwide Temporal Trends in Incidence of Hospitalization and Surgical Intestinal Resection in Pediatric Inflammatory Bowel Diseases in the United States from 1997-2009”

  • Annual percent increase (API) of 2.1% noted in incidence of intestinal resection for Crohn’s disease.  Stable colectomy rate for ulcerative colitis during this period.
  • Annual incidence of hospitalization was 5.7 per 100,000 for Crohn’s and 3.5 per 100,000 for ulcerative colitis; there was a significant increases during study period: 3.8% API for Crohn’s and 4.5% for ulcerative colitis.