A recent study (C Columba et al. Full text: Intestinal Involvement in Kawasaki Disease J Pediatr 2018; 202: 186-93) indicates that when patients with Kawaski’s present with predominantly intestinal symptoms that this may lead to a delay in diagnosis and potential cardiac complications. In this systematic review, 33 articles reporting 48 cases of Kawasaki disease with intestinal involvement were considered.
Some key points:
- In this study, abdominal pain and vomiting were the most frequently reported symptoms, followed by diarrhea. Fever was typically present but other features of Kawaski disease were not apparent at presentation. Presentation can include dilated bowel loops suggestive of obstruction (pseudo-obstruction), pancreatitis, and intestinal vasculitis/bowel wall thickening.
- “The prevalence of gastrointestinal involvement in Kawasaki disease is unknown as available data can only be derived from single reports and few case series. Miyake et al in their retrospective case series of 310 children with Kawasaki disease reported gastrointestinal involvement in only 7 cases (2.3%).”
- “Abdominal symptoms in Kawasaki disease are more often due to hydrops of the gallbladder. In Taiwan, a routine abdominal ultrasound showed hydrops of the gallbladder in 21% of patients with Kawasaki disease.”
My take: Since early treatment of Kawasaki disease may improve outcomes, it is worthwhile to consider this in the differential diagnosis of patients presenting with possible obstruction and in those who have fever in addition to pain/emesis.
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A new report from an ESPGHAN-Led Expert Group (N Thapar et al. JPGN 2018; 66: 9991-1019) provides detailed recommendations for pediatric intestinal pseudo-obstruction (PIPO). In addition, this report serves as an excellent self-assessment of your vision. If you can read figure 1, which has some incredibly tiny font size, then your vision is fantastic.
Full Link: “Paediatric Intestinal Pseudo-obstruction: Evidence and Consensus-based Recommendations From an ESPGHAN -Led Expert Group”
Aside from that snarky comment, the report offers a great deal of useful advice.
- After obstruction has been excluded, the authors recommend that patients should undergo a basic laboratory evaluation (including CBC, CMP, ESR/CRP, Celiac serology, Cortisol, Thyroid testing, Metabolic tests [urine organic acids, ammonia, lactate]) and to consider more extensive evaluation.
- If primary, rather than secondary, PIPO is suspected, the authors recommend neurogastroenterology evaluation.
Subsequently, the authors review management: potential medications (Table 6), enteral feeds, gastrostomy and ileostomy, and in more than 80% then need for parenteral nutrition. At the time of therapeutic procedures, it is recommended to obtain full-thickness biopsies to further characterize the PIPO.
Clinical features which distinguish pediatric chronic intestinal pseudo-obstruction (CIPO) from adult CIPO are listed in Table 2. These include the following:
- Frequent urologic involvement in pediatric CIPO which is rare in adults with CIPO.
- Dilated bowel loops are commonly absent (~40%) in pediatric CIPO in the neonatal period and universal in adult cases.
- Unlike in adults, there is a high risk of colonic and small bowel volvulus in pediatric CIPO and malrotation is evident in ~30% of pediatric CIPO (rarely seen in adults).
- Also, in pediatrics, fabricated cases are more commonly encountered.
Intestinal transplantation should be considered in patients with PIPO who develop life-threatening complications associated with TPN or poor quality of life/high morbidity.
Pictures below from yesterday’s Peachtree Road Race and previous T-shirts from previous years.
Disclaimer: These blog posts are for educational purposes only. Specific dosing of medications/diets (along with potential adverse effects) should be confirmed by prescribing physician/nutritionist. This content is not a substitute for medical advice, diagnosis or treatment provided by a qualified healthcare provider. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a condition.