Rare. Signs and symptoms of permanent or recurrent intestinal obstruction of the small bowell (more rarely the colon) without any mechanical cause. Vomiting, constipation, intestinal dilation is often accompanied by abdominal pain. The first signs appear in the neonatal period or in early childhood. Typical X-ray pictures: important duodenal dilation, few contractions, loss of colic haustrations . The impairment may be diffuse or localized.

CIPO can be primary or secondary. In children, the most common secondary causes are post-operative ileus, hypokalemic ileus and post-infectious ileus of autoimmune origin. In adults the causes are: multiple sclerosis, diabetes mellitus, scleroderma, amyloidosis, Chagas’ disease.

The causes of primary CIPO are neuropathic (more frequent) or myopathic:

• neuropathic: detectable abnormalities of intrinsic or extrinsic intestinal innervation at optical or electron microscopy.

    

1) developmental abnormalities:

-        aganglionosis: Hirschsprung's disease (mutation of cRET gene)

-        hypoganglionosis: similar in appearance to the transition zone in Hirschsprung's disease

-        hyperplasia of the myenteric plexus (mutation of cRET gene): often more localized (" neuronal intestinal dysplasia"); in case of complete form, often associated with multiple endocrine neoplasia (MEN) type IIb with multiple mucosal neuromas and a marfanoid phenotype

-        NID A (Neuronal Intestinal Dysplasia) [MIM 243 180]: hypoplasia or aplasia of the sympathetic bowel system, with abnormalities of the argyrophilic cells; there is also ataxia, dysarthria, low-reflexive pupils, dysautonomy (abnormal response to Valsalva's manoeuvre)

-        NID B [MIM 601 223]: involvement of the submucous parasympathetic plexus

-        X-linked form [MIM 300 048]: X-linked recessive transmission of a mutation of the FLNA gene (Xq28): central nervous system involvement and facial dysmorphism (wide jaw, hypertelorism, low set ears), persistence of the ductus arteriosus

2) delayed maturation: positive evolution over a few weeks time

3) secondary involvement: for example in case of vincristine treatment or CMV infection

• myopathic: generally autosomal recessive transmission; 5 different histological phenotypes have been described: alterations in the structure of the muscular wall of the small intestine, myocytes or the extracellular matrix abnormalities (see congenital visceral myopathy abnormalities).

*        isolated forms

-        visceral myopathy: vacuolar degeneration  of smooth muscle cells as in the non-familial visceral leiomyopathy known as the Bantu disease, that begins with the colon and could be due to the toxicity of some traditional enemas

-        microcolon, megabladder, hypoperistalsis syndrome

-        lately expressed visceral myopathy, associated with a mitochondrial cytopathy (see MNGIE).

*        forms secondary to a disease of the striated muscle : e.g., Steinert's disease and Duchenne muscular dystrophy.

There are CIPO cases without any histological abnormalities: some cases of deficiency in α-actin have been described.

Treatment is essentially symptomatic . Prokinetics: cisapride. Parenteral or enteral nutrition (by gastrostomy or jejunostomy feeding),  digestive derivations to decompress the intestine and prevent bacterial overgrowth. In case of associated megabladder, a urinary derivation is often necessary. Liver transplantation is often proposed.

Anesthetic implications:

check the electrolytes and protein levels, liver function (risk of liver disease secondary to parenteral nutrition); difficult peripheral or central venous access (thrombosis complicating chronic or repeated central venous access). Delayed gastric emptying. Risk of latex allergy, the primary prevention of which is recommended.

References : 

-         Burt N, Williams AR. 
A pseudosyndrome, a real risk : aspiration on induction in a child with chronic idiopathic pseudoobstruction. 
Am J Anesthesiol 1999; 26: 223-5.

-         Moore SW, Schneider JM, Kaschula RDC. 
Non familial visceral myopathy: clinical and pathological features of degenerative leiomyopathy.
Pediatr Surg Int 2002; 18: 6-12.

Updated: August 2020