Constipation When to consider Organic Disease?2015.cipediatrics.org/wp-content/uploads/2015/03/2-Sigalet.W.U.h... · Doha Qatar Professor of Surgery ... most of us are ‘familiar’

Consensus in Pediatrics 2015

David Sigalet MD PhD FRCSC FACS

Chief of Surgery Sidra Medical and Research Center,

Doha Qatar Professor of Surgery

Weil Cornell Medical College, Doha/New York

Constipation When to consider Organic Disease?

Objectives

Constipation Differential Diagnosis When/Who to Investigate Work up Surgery for Hirshprungs Long Term Follow-up Primary Pediatrician

Presenter

Presentation Notes

Thank you Mr Chairman: I am delighted to be here with you to day, to discuss the problem of intestinal failure. This is an overview of what we will discuss today; I would like you all to take a minute an just consider these points about Intestinal failure; most of us are ‘familiar’ with this topic, but I’d like to ask you to consider each of these questions before we proceed; What is intestinal failure;? What is intestinal adaptation?, how can we stimuate it? How do these new lipid preperations work? When should we do the step procedure? What are the results of best practices treatment today? What are the possible new therapies for the future?

Differential Diagnosis Hirschprung’s Disease Ganglia

Absent Enteric Neuronal Ganglia

= Absent peristalsis

Neuronal Intestinal Dysplasia

4

Abnormal number and neurotransmitter subtype of enteric neurons Ineffective Peristalsis Often associated with CNS neuronal or behavioral abnormalities

Strictures, Metabolic Causes

Anatomic/Congenital Strictures Hypothyroidism Meconium Ileus/Cystic Fibrosis

5

Evaluation of Constipation: Who Needs Biopsy?

6

All Constipation patients?

7

Critical Symptoms

Normal time to passage of meconium (>99% passage is within 24 hrs of birth)

Failure to thrive Intermittent diarrhea

(enterocolitis)

Critical Signs No fecal impaction on PR

Goulet J Ped Surg. 61: 723-8

Presenter

Presentation Notes

To begin with, definitions: Intestinal failure is the ‘new’ term, for what we used to call Short bowel syndrome, which is still valid, but we also see many patients with longer lengths of intestine, which have a functional lack of gut mass, requiring PN.

Investigation:

Contrast Enema For all

Hirschprungs Biopsy

Neuronal Intestinal Dysplasia

Biopsy + Manometry

Lanager, Curr Opin Peds, 2013

Presenter

Presentation Notes

While the overall incidence is only 1200/100,000 live births, the survival rates range from 73-89% making pediatric SBS one of the most lethal conditions in infancy and childhood

Delayed Presentation

10

Operative Procedures for Hirschprung’s

11

What’s new? Transanal Approach

12

Long Term Follow-up: Partnership Pediatrician: Surgeon

13

Birth Diagnosis Surgery Early follow-up

infancy

Age: 0 3 6 12 months

Incontinence Enterocolitis ( Rx:

Metronidazole) Constipation

Long Term Complications

Incontinence Enterocolitis ( Rx: Metronidazole) Constipation Sexual dysfunction Long term follow-up essential Yearly Rectal!!

14

Long Term Follow-up: Partnership Pediatrician: Surgeon

15

Childhood Adult

Age: 0 1 2 6 12 24 40+ years Incontinence Enterocolitis ( Rx: Metronidazole) Constipation Socialization, Sports Sexual dysfunction Long term follow-up essential Yearly Rectal!!

Conclusions

Constipation: common Investigate if:

FTT, Diarrhea, no response to conventional therapy

Work up: Bx, Contrast, Manometry ↑ MIS surgery Long Term Follow-up Essential

Presenter

Presentation Notes

Thank you Mr Chairman: I am delighted to be here with you to day, to discuss the problem of intestinal failure. This is an overview of what we will discuss today; I would like you all to take a minute an just consider these points about Intestinal failure; most of us are ‘familiar’ with this topic, but I’d like to ask you to consider each of these questions before we proceed; What is intestinal failure;? What is intestinal adaptation?, how can we stimuate it? How do these new lipid preperations work? When should we do the step procedure? What are the results of best practices treatment today? What are the possible new therapies for the future?

Documents

Constipation When to consider Organic Disease?2015.cipediatrics.org/wp-content/uploads/2015/03/2-Sigalet.W.U.h... · Doha Qatar Professor of Surgery ... most of us are ‘familiar’