BSPGHAN 2021 Virtual Annual Meeting

27- 29 April 2021

CHARACTERISTICS OF CHILDREN WITH INTUSSUSCEPTION IN PEUTZ-JEGHERS SYNDROME IN A SPECIALIST CENTRE OVER A 10 YEAR PERIOD

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Christine Spray
Christine Spray
23 days ago

Thank you for your poster. Patient C presented at diagnosis and therefore patient C does not really support your conclusion for annual screening. Can you explain a VCE performed 8m prior to presentation has reported faecal loading? is this correct? Would the author like to suggest their preference of screening for these patients especially as patient A had undergone VCE 4 months prior to presentation with intussusception. Is there a size of polyp if identified on screening which determines the next step in management

Vaia Zouzo
Vaia Zouzo
20 days ago

Thank you for your comments. Unfortunately, as this is a virtual meeting, I did not have the chance to present those cases and give you more clinical information.

All three cases presented in our centre before the implementation of the current guidelines.

Patient C was not known to the gastro team and presented in the district hospital in 2010 with a 3-month history of anaemia, acute abdominal pain and vomiting. Despite known family history of PJS, predictive genetic test has not been performed earlier because the family did not attend the local genetic clinics against our recommendations. If a diagnosis of PJS had achieved earlier, maybe GI surveillance would have started before the presentation with intussusception, especially with the history of anaemia.

Patient B: The VCE was limited due to significant faecal matter and air bubbles – poor quality of imaging. Adult WCE guidelines have abandoned bowel prep due to poor patient adherence. We evaluated and audited the quality of WCE in children and have returned to strict bowel prep in all children, preferably we aim to combined OGD+Colo+WCE under one bowel prep procedure to improve compliance and quality of imaging.

Patient A: The VCE 4m prior to intussusception has shown multiple small intestinal polyps and therefore she has been referred for DBE. This was a case of de novo presentation, diagnosed 1.5 years ago and maybe the devastating complications were not clear enough to the family, as they have not adhered to the referral for removal of the polyps.

We reinforce the current guidelines for GI surveillance no later than the age of 8 years and repeat every 3 years in asymptomatic patient. We recommend annual screening in case of known small intestinal polyps, under optimal imaging conditions, or immediately when children are symptomatic, however the guidelines should always be individualized.
Our recommendation for annual screening regards the cases with small intestinal polyps, not significant to be removed by DBE.

As per the current ESPGHAN guideline polyps > 1.5 cm are at higher risk of causing intussusception and subsequent small bowel resection, so should be referred for polypectomy. Polyps < 1 cm should be screened every 3 years with VCE, individualizing frequency depending on patient’s symptoms. Based on our audit data, we have returned tour previous arrangements to screen patients even with small polyps of less than 1cm annually. 

Christine Spray
Christine Spray
20 days ago

Thank you for your reply

Khaled Abdelaal
Khaled Abdelaal
18 days ago

Thank you for the author