diagnosis and treatment of functional constipation in infants and children

➡️Introduction

Functional constipation is a common problem in children. Although some guidelines exist for management of childhood constipation (1) there are no such guidelines for Egyptian children.

Functional constipation constituted 30% of pediatric gastroenterology outpatient practice, 4-5% of all referrals to pediatric gastroenterology tertiary care centers and 0.8-1% of all pediatric cases in medical colleges (1).

Normal stool frequency is; <1-month age: 3-4 times/day; 1 month to 1-year age: 1.5-2 times/day; 1 to 2-year age: 1-2 times/day, mostly formed; older than 2-year age: 1 time/day. While constipation is a delay or difficulty in defecation sufficient to cause significant distress to the patient (3,4).

When the duration of constipation is less than 4 weeks, it is considered as acute constipation and when the duration is more, it is chronic constipation; in addition to two or more of the following criteria: (a) defecation frequency ≤2 times per week, (b) fecal incontinence ≥1 times per week after the acquisition of toileting skills, (c) history of excessive stool retention, (d) history of painful or hard bowel movements, (e) presence of a large mass in the rectum or on per abdomen examination, (f) history of large-diameter stools that may obstruct the toilet (7).

The term “Fecal Incontinence”, currently used instead of the terms soiling or encopresis, is defined as passage of stools in the undergarment and is classified as: (a) Constipation-associated fecal incontinence and (b) non-retentive fecal incontinence: diagnosed only if there is no constipation and normal anal sphincter tone, and symptoms last for more than 2 months in a child with a developmental age of ≥4 years (1).

The term “Refractory constipation” is defined as constipation not responding to optimal conventional treatment for at least 3 months, despite good compliance. These patients should be referred to a pediatric gastroenterologist for evaluation (2).

History and examination are relevant in making a diagnosis of constipation and differentiating functional and organic constipation as follows:  Dietary history; like intake of fruits and vegetables and refined foods (e.g., bakery), beverages etc. in older children, nature of feeds (breast vs top feeds) and details of supplementary feeds in younger babies, exclusive and prolonged milk intake with minimal solids in young infants, In addition to evidence of important precipitating factors of functional constipation;, (a) premature initiation of toilet training (normally toilet training should start not before 24 months in a developmentally normal child) (b) drugs and inter-current illnesses, (c) quick and abrupt transition of diet e.g. liquid to solid, breastfeeding to bottle feeding and (d) change in local environment (start of schooling) and psychosocial factors (3,5,6).

Characteristics of functional constipation in infants up to 4 years must include 2 or fewer defecations per week or 1 month of at least 2 of the following: (a) History of excessive stool retention, (b) History of painful or hard bowel movements, (c) History of large-diameter stools, (d) Presence of a large fecal mass in the rectum. While in toilet-trained children, the following additional criteria may be used: (a) At least 1 episode/week of incontinence after the acquisition of toileting skills, (b) History of large-diameter stools that may obstruct the toilet. Children greater than four years must have 2 or more of the following occurring at least once per week for a minimum of 1 month with insufficient criteria for a diagnosis of irritable bowel syndrome: (a) 2 or fewer defecations in the toilet per week in a child of a developmental age of at least 4 years, (b) At least 1 episode of fecal incontinence per week, (c) History of retentive posturing or excessive volitional stool retention, (d) History of painful or hard bowel movements, (e) Presence of a large fecal mass in the rectum, (f) History of large diameter stools that can obstruct the toilet (7).

Organic causes of constipation include; Abnormalities of the colon and rectum (e.g., chronic intestinal pseudo obstruction, anal stenosis, anal/colonic stricture, post NEC/IBD and ectopic anus), Neuropathic lesions (e.g., Hirschsprung disease and intestinal neuronal dysplasia), Spinal cord lesions (e.g., spina bifida, meningomyelocele,  sacral agenesis, tethered cord and tumors), Metabolic (e.g., hypothyroidism, hypo/hyper-calcemia, hypokalemia and uremia), Systemic disorders (e.g.,  celiac disease, cystic fibrosis and diabetes mellitus) and Drugs (e.g., analgesics, anticholinergics, iron, sympathomimetics, psychotropics, NSAIDs and antacids) (6 ).

The aim of this adapted clinical practice guideline (CPG) is to provide evidence-based recommendations for diagnosis and treatment of functional constipation in infants and children. Thise recommendations were adapted from the relevant evaluation and treatment of functional constipation in infants and children: evidence-is based recommendations from ESPGHAN and NASPGHAN: JPGN 2014;58: 258–274 CPG(s) using a formal methodology for CPG adaptation: the Adapted-ADAPTE.

This guideline focuses on diagnosis and treatment of functional constipation.

Disease/Condition:

Guideline Objective(s)

Diagnosis and treatment of functional constipation in infants and children

Health / Clinical Question (PIPOH)

P: Patient (Target Population):

Gender: Both genders

Age group: infants and children from 1-18 years

Disease/condition: functional constipation in infants and children

I: Interventions and Practices Considered / CPG Category:

Clinical: history taking and examination

Laboratory investigations:

•        laboratory investigations to diagnose (cow’s milk) allergy, celiac disease, hypothyroidism, and hypercalcemia

•        abdominal radiography (use of barium enema to diagnose organic causes such as hd

•        colonic transit time (ctt)

•        transabdominal rectal ultrasonography

•        magnetic resonance imaging (mri) of the spine

•        colonic manometry

•        anorectal manometry (arm) or rectal suction biopsy to diagnose hd

•        colonic full-thickness biopsies

•        treatment

P: Professionals (Intended / Target Users or Stakeholders)        :

Primary health care physician

General practitioners

Family physician

Pediatrician

Gastroenterologist

O: Major Outcomes Considered:

1. Primary outcome: proper diagnosis of functional constipation
2. Secondary outcome: proper treatment of functional constipation

H: Healthcare Settings:

Types:

primary, secondary and tertiary healthcare centers.

•governmental healthcare sector:

moh, university, military, health insurance organization

•non-governmental healthcare sector:

private and NGO healthcare centers

Guideline development process and methods

After reviewing all the inclusion and exclusion criteria and quality appraisal results, the GDG/ GAG recommended using the following source original clinical practice guidelines (CPGs):

1- Tabbers MM, DiLorenzo C, Berger MY, Faure C, Langendam MW, Nurko S, Staiano A, Vandenplas Y, and Benninga MA, evaluation and Treatment of Functional Constipation in Infants and Children: Evidence-Based Recommendations from ESPGHAN and NASPGHAN: JPGN 2014;58: 258–274.

We conducted Adolopment for these guidelines: (Adoption, Adaptation, and Development)

      -  Adoption for most of the guideline recommendations.

      -   Development of Good Practice Statements

 Recommendations and Good Practice Statements (GPS)

This version of the CPG includes recommendations and good practice statements on the following four sub-sections:

A. Diagnosis of Functional Constipation

The guideline covers 1 to 18 years old

This guideline emphasis on

B. Management of Functional Constipation

This section includes recommendations and good practice statements on

We can summarize the guidelines’ recommendations for Functional Constipation in the following:

·Based on expert opinion, we recommend the Rome III criteria for the definition of functional constipation for all age groups. • Quality of evidence: Low • Strength of recommendation: Conditional

·  Based on expert opinion, the diagnosis of functional constipation should be based on history and physical examination. • Quality of evidence: Low • Strength of recommendation: Conditional

· Based on expert opinion, use the following alarm signs and symptoms to identify an underlying disease-causing constipation (e.g., onset < 48 h, family history of Hirschsprung disease, ribbon stools, blood without fissures, failure to thrive, bilious vomiting, abnormal anorectal or neurologic findings, sacral dimple, extreme fear during examination, etc.). • Quality of evidence: Very Low • Strength of recommendation: Conditional

· Based on expert opinion, if only one Rome III criterion is met and diagnosis is uncertain—or if alarm signs or intractable constipation are present—a digital rectal examination is recommended to exclude underlying conditions. • Quality of evidence: Very Low • Strength of recommendation: Conditional

· Based on expert opinion, abdominal radiography is not indicated routinely but may be used when fecal impaction is suspected and physical examination is unreliable or impossible. • Quality of evidence: Very Low • Strength of recommendation: Conditional

·  Based on expert opinion, rectal ultrasound is not recommended to diagnose functional constipation. • Quality of evidence: Very Low • Strength of recommendation: Conditional

·  Based on expert opinion, routine laboratory testing for cow’s-milk allergy, celiac disease, hypothyroidism, and hypercalcemia is not recommended in children with functional constipation; a 2–4 weeks trial of cow’s-milk protein avoidance may be considered in intractable cases. • Quality of evidence: Very Low • Strength of recommendation: Conditional

·  Based on expert opinion, barium enema should not be used as an initial diagnostic tool for organic causes of constipation. • Quality of evidence: Very Low • Strength of recommendation: Conditional

·  Based on expert opinion, routine spinal MRI is not recommended in patients with intractable constipation without neurologic abnormalities. • Quality of evidence: Very Low • Strength of recommendation: Conditional

·  Based on expert opinion, full-thickness colonic biopsies are not recommended to diagnose colonic neuromuscular disorders in intractable constipation. • Quality of evidence: Very Low • Strength of recommendation: Conditional

·  Based on expert opinion, routine colonic scintigraphy is not recommended in children with intractable constipation. • Quality of evidence: Low • Strength of recommendation: Conditional

·  Based on expert opinion, children with functional constipation should have a normal fiber intake. • Quality of evidence: Very Low • Strength of recommendation: Conditional

·  Based on expert opinion, children with functional constipation should have a normal fluid intake. • Quality of evidence: Low • Strength of recommendation: Conditional

· Based on expert opinion, children with functional constipation should engage in normal physical activity. • Quality of evidence: Low • Strength of recommendation: Conditional

·  Based on expert opinion, routine use of prebiotics and probiotics is not recommended in the treatment of childhood constipation. • Quality of evidence: Very Low • Strength of recommendation: Conditional

· Based on expert opinion, intensive behavioral therapy protocols are not recommended; rather, provide demystification, explanation, and toilet-training guidance (for developmental age ≥ 4 years). • Quality of evidence: Low • Strength of recommendation: Conditional

·  Based on expert opinion, biofeedback is not recommended as an adjunctive treatment in childhood constipation. • Quality of evidence: Low • Strength of recommendation: Conditional

· Based on expert opinion, routine multidisciplinary treatment is not recommended for childhood constipation. • Quality of evidence: Low • Strength of recommendation: Conditional

·  Based on expert opinion, alternative medicine (acupuncture, homeopathy, mind-body therapy, osteopathic/chiropractic manipulations, yoga) is not recommended in childhood constipation. • Quality of evidence: Low • Strength of recommendation: Conditional

·  Based on expert opinion, oral polyethylene glycol (PEG) 1–1.5 g/kg/day for 3–6 days is first-line for fecal disimpaction; if PEG is unavailable, daily enemas for 3–6 days are recommended. • Quality of evidence: Very Low • Strength of recommendation: Conditional

· Based on expert opinion, PEG (0.4 g/kg/day, titrate to response) with or without electrolytes is first-line maintenance; lactulose is an alternative if PEG is unavailable; routine chronic enemas are not recommended. • Quality of evidence: Very Low • Strength of recommendation: Conditional

·  Based on expert opinion, maintenance therapy should continue for at least 2 months, with all symptoms resolved for ≥ 1 month before tapering; stop medication only after toilet training is achieved. • Quality of evidence: Low • Strength of recommendation: Conditional

·  Based on expert opinion, transcutaneous nerve stimulation (TNS) is not supported for children with intractable constipation. • Quality of evidence: Low • Strength of recommendation: Conditional

· Based on expert opinion, antegrade continence enemas (ACE) are recommended in selected children with intractable constipation. • Quality of evidence: Very Low • Strength of recommendation: Conditional

·  Based on expert opinion, approximately 50% of referred children recover without laxatives by 6–12 months, 10% remain well on laxatives, and 40% stay symptomatic; 50% recover by 5 years and 80% by 10 years; delayed treatment (> 3 months) correlates with longer symptom duration. • Quality of evidence: Low • Strength of recommendation: Conditional

· Based on expert opinion, use demographics, clinical history, physical exam, and select ancillary tests to identify prognostic factors for functional constipation—most evidence is limited, though strong evidence exists that sex and family history have no prognostic value. • Quality of evidence: Limited to Strong • Strength of recommendation: Conditiona

➡️Guideline Registration

PREPARE (Practice guideline REgistration for transPAREncy), WHO Collaborating Center for Guideline Implementation and Knowledge Translation, EBM Center, University of Lanzhou, Lanzhou, China. Registration Number: IPGRP-2021CNXXXXXXX. Link: http://www.guidelines-registry.org/