Abstract
Constipation is defined as a stool frequency of less than 3 times per week. These patients will have bulky, hard, dry bowel movements that can be painful to pass. The age range for this diagnosis can obviously be from a newborn infant to an older child, though the most common age of presentation is between 1 and 5 years. The work up and management is often dependent on the age of the patient. Most children with constipation are well managed by pediatricians and pediatric gastroenterologists. The patients that are referred to the pediatric surgeon are often those who rarely or never have bowel movements without some mechanical assistance, either orally or per rectum. They may have had difficulty since birth or gradually developed worsening constipation as they transitioned off breast milk or formula and onto cow’s milk. Sometimes they will have a diagnosis that might indicate the possibility of a functional disorder of the colon, such as gastroschisis, Hirschsprung disease or cystic fibrosis. Rectal sphincter disorders, such as imperforate anus, cloacal anomalies and myelomeningocele also contribute to constipation issues. There are children who have conditions such as trisomy 21 and cerebral palsy and who are more likely to be constipated. Finally, children with complex neuromuscular disorders (muscular dystrophy, spinal muscular atrophy, intestinal pseudo-obstruction), might require surgical assistance with the problems of constipation.
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Appendices
Summary Points
A variety of organic, functional and anatomical problems can lead to constipation. These must be thoroughly evaluated and addressed before the symptom of constipation can be treated.
A thorough history and physical exam will help the clinician decide which studies might be helpful.
Contrast studies are indicated in most patients for diagnosis, treatment and predictive purposes.
Evaluation of difficult patients can be enhanced by motility studies.
Sitzmark study often add information regarding colonic motility.
Dietary, medical and behavioral therapy is indicated in most situations.
The ACE procedure can be very helpful in difficult cases or those with neurological problems that cannot be addressed any other way.
There is a relatively high minor complication rate for these procedures.
If an ACE is performed, dealing with the complications of the surgery and dedication to finding irrigation solutions that are effective takes time and patience.
A dedicated bowel management expert as a resource for patients with chronic constipation is invaluable.
Editor’s Comment
Patients with constipation are referred to the pediatric surgeon for one of three indications: for fecal disimpaction, for consideration of an antegrade enema procedure, and for “rule out Hirshsprung’s disease.” Though unpleasant and sometimes tedious, fecal disimpaction is often quite rewarding. It should be performed with an aggressive but careful bimanual technique and accompanied by saline irrigation of the left colon through a red-rubber catheter. The anal dilatation that invariably occurs can be helpful for a time after the procedure but deliberate forceful dilatation of the anal sphincter is no longer recommended because of the risk of incontinence.
Antegrade continent enemas do nothing to treat the underlying disorder, but they can help patients with intractable constipation and encopresis stay clean for up to 24 h. The procedure should be done laparoscopically whenever possible and, to make it truly continent, some form of an antireflux valve must be created at the base of the conduit. This procedure is clearly undervalued and offered not nearly as often as it should be for patients who could clearly benefit. This might be due to the fact that it can be technically challenging, the postoperative care is laborious and fraught with frequent minor complications (recurrent stenosis, stool leakage), and perhaps because some are uneasy recommending a procedure that treats a symptom rather than an underlying disease.
Hirschsprung disease can generally be excluded by a careful history, but once the possibility has been raised it is often impossible to disprove without a rectal biopsy. In children older than about 4 months, this needs to be done as an open procedure under general anesthesia. A contrast enema should also be performed, but the sensitivity of this test for Hirschsprung disease is disappointingly low.
Be very careful to avoid stating or even implying that a child’s symptoms are due to “just constipation,” as this can provoke anger and resentment in parents, many of whom interpret this as evidence of a lack of empathy or an overt criticism of their parenting. Severe constipation can be a serious issue that causes a great deal of anxiety and physical discomfort. I am confident that most cases of “functional” constipation will someday prove to have a genuine underlying pathophysiologic mechanism that we simply have yet to appreciate. Finally, the traditional and sometimes self-righteous recommendations that clinicians frequently give (drink more water, increase dietary fiber intake, avoid “constipating” foods) are almost never enough for children with severe or symptomatic constipation; laxatives or cathartics are almost always necessary in these cases.
Differential Diagnosis
Newborns
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Imperforate anus
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Anal stenosis
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Meconium ileus
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Hirschsprung disease
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Ileal atresia
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Meconium plug syndrome
Infants
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Hirschsprung disease
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Anal stenosis
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Functional/familial
Toddlers
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Environment/psychological
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Functional constipation
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Hirschsprung disease
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Ectopic anus
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Neurologic dysmotility (cerebral palsy)
School age
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Environment/psychological
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Functional constipation
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Tethered spinal cord/occult spina bifida
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Neurologic dysmotility
Diagnostic Studies
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Abdominal radiograph
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Contrast enema
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Suction rectal biopsy or full thickness rectal biopsy
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Anal manometry
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Colonic manometry
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Sitzmark study
Parental Preparation
Education about diet, medication, and behavior modification programs.
Finding the right combination takes patience and a great deal of trial and error.
If surgery is an option, the minor complication rate is high but overall patient satisfaction is high.
Finding the right irrigation recipe can take time and creativity.
Preoperative Preparation
Bowel preparation
Prophylactic antibiotics
Technical Points
All anastomoses should be tension-free.
Always handle the mesentery carefully to avoid ischemia and subsequent stricture.
If the umbilicus is chosen for the site of an appendicostomy, remove the thickened skin at base of umbilicus prior to creating the stoma.
If a RLQ site is chosen, use a technique that creates a skin tunnel with generous skin flaps.
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Nicolette, L. (2011). Constipation. In: Mattei, P. (eds) Fundamentals of Pediatric Surgery. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-6643-8_58
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DOI: https://doi.org/10.1007/978-1-4419-6643-8_58
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