Clinical presentation

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A child may present with either the primary or the secondary effects of the underlying disease.

However, the clinical symptoms are variable and often non-specific. The location of the affected bowel, diffuse or regional, seems a more important determinant of the clinical presentation than the underlying disease. A child may be obstructed or complain of severe constipation depending upon whether the small intestine and colon or just the colon is affected. In addition, those with urinary tract involvement may initially present with acute or chronic urinary retention. These effects quite clearly are the result of the ability of the underlying disease of the neuromusculature of the gut to produce ordered motor activity. However, the effects of the underlying disease may also be secondary, such as the consequences of bacterial overgrowth, fecal impaction or adhe-sional obstruction associated with previous surgery. In neuropathic disorders the consequences of denervation may not only be on motor activity but also on secretomotor control and sensation. These effects may result in diarrhea or visceral hyperalgesia.

Although initial reports of CIP were in adults, it is now apparent that it probably occurs more commonly in infants. Indeed, when the presentation and mortality of cases published in the literature are considered, it is found to occur most commonly in children, with high morbidity and mortality. Considering these data5 together with a large series from one center,1 the conditions in childhood present most commonly during infancy, either in the neonatal period, or under the age of 1 year, during which time the highest mortality rates are suffered. In most cases an underlying abnormality of either smooth muscle or of enteric nerves is found when adequately sought. The clinical manifestation at presentation depends on both age and the type and extent of the condition affecting the neuromusculature of the gut.

Before birth

CIP may first be recognized before birth, either as part of a routine antenatal ultrasound scan or in the investigation of a mother with polyhydramnios. On abdominal ultrasound examination the fetus may have either dilated loops of bowel or a distended bladder, or both.

Birth and the neonatal period

The majority of children with CIP present either at birth or in the neonatal period.1 In all series approximately half the infants have symptoms at birth or within the first few days of life. In those who present at birth, the labor and delivery are frequently difficult, owing to an already distended abdomen. After birth there is abdominal distension, failure to pass meconium and bilious vomiting. The abdominal distension is due to swallowed air, which distends and dilates the small bowel, but is not passed further through the gut. Contrast studies may show the presence of a microcolon or a short small intestine or, in approximately 34%, a malrotation. In some there may be a specific clinical syndrome of a congenitally short small intestine, pyloric stenosis and a mal-rotation.6,7 In addition to gastrointestinal symptoms, there may also be failure to pass urine, mega-cystis and hydroureter or hydronephrosis. Incomplete bladder emptying often results in recurrent urinary tract infection, and this may completely overshadow the gastrointestinal symptoms.

Later infancy

After the neonatal period, although infants may present with an acute obstruction, the symptoms are often intermittent or slowly progressive. Initially some infants may appear completely healthy, taking breast feeds normally, but then suddenly develop an episode of obstruction following what appears to be an intercurrent enteric infection. In these infants persistence of vomiting for more than 7 days warrants further investigation in a patient who has been thought to have had acute gastroenteritis, as they may have developed an acute myositis, which is potentially treatable.8 In other such infants, who have fed normally from birth, episodes of obstruction begin only when more complex foods are introduced into the diet and attempts are made at weaning. Urinary symptoms due to involvement of the urinary tract may continue to present for the first time during infancy.


In later childhood the initial presentation may continue to indicate obstruction, but may simply be constipation, which becomes intractable. Severe abdominal pain may also occur, owing to distension of the bowel or as part of visceral hyperalgesia. In those with a distended abdomen, bowel sounds may be totally absent or very markedly reduced. If high-pitched bowel sounds are present, or if there is visible peristalsis, the distension of the gut is more likely to be secondary to a mechanical rather than a functional obstruction.

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Constipation Prescription

Constipation Prescription

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