Pneumatosis cystoides intestinalis


Pneumatosis cystoides intestinalis

Description, Causes and Risk Factors:

A condition of unknown cause characterized by the occurrence of gas cysts in the intestinal mucous membrane; may produce intestinal obstruction.

Pneumatosis cystoides intestinal is of the small bowel are conditions in which multiple gas filled cysts are seen in the gut without any definite evidence of infection. This condition is known to occur both in infants as well as in the adults. In infants it occurs as a component of necrotizing enterocolitis and often has a fatal outcome, whereas in adults, it usually presents as an idiopathic finding.

It is a relatively rare disease, with an estimated 410 cases having been described in the lietrature by 1973. The cysts are more common in men than women, with an approximate ration of 3.5:1. It has been recorded in all age groups, but is most common in the age group of 25-55 years.

Numerous etiologies of pneumatosis cystoides intestinalis have been described in the literature. In general, these can be divided into either infectious or mechanical.

    Infectious pneumatosis cystoides intestinalis is usually caused by gas-forming bacteria. This is a relatively rare entity characterized by mucosal and submucosal inflammation and bacterial infiltration of most of the gastric wall layers on autopsy specimens. These patients present with leukocytosis, high grade fevers, and other signs and symptoms of sepsis. Infectious pneumatosis cystoides intestinalis carries a very poor prognosis, with an overall mortality rate as high as 60% to 70%.

  • Pneumatosis cystoides intestinalis secondary to mechanical etiologies is far more common than the infectious pneumatosis cystoides intestinalis. Here, causes include obstruction, trauma, rupture of pulmonary bullae, enteric tube placement and upper endoscopic procedures. In these cases, the intraluminal gas dissects into the gastric wall through a mucosal tear or defect. The tear usually results from increased intraluminal pressure secondary to an obstruction or as a direct result of trauma. The reported mortality in this group, although lower than mortality associated with gastric emphysema, is still high at 6% to 41%. While other authors describe pneumatosis cystoides intestinalis secondary to bowel or gastric outlet obstruction.

A person who shows signs of pneumatosis cystoides intestinalis is likely to have an inflammatory gastrointestinal disorder or a pulmonary obstruction. Bacterial infections, Crohn's disease, and ulcerative colitis can all lead to inflammation, irritation, and gas buildup. In addition, blood flow to the bowels can be disrupted in a condition called ischemia. A lack of oxygenated blood causes hydrogen gas buildups in the intestinal walls and eventually results in necrosis of intestinal tissue.

Symptoms:

Clinically the patients present with variety of signs and symptoms including crampy abdominal pain, diarrhea, constipation, and abdominal distension. Pneumatosis cystoides intestinal is usually discovered on radiological examination, during endoscopy or at laparotomy.

Diagnosis:

Abdominal radiography and CT are the most frequently used techniques for diagnosis of pneumatosis cystoides intestinalis. CT has been shown to be more sensitive than radiography at detecting pneumatosis cystoides intestinalis. CT has also been shown to be more sensitive than radiography at detection of hepatic portal and portomesenteric venous gas, the presence of which increases the possibility of pneumatosis cystoides intestinalis due to life-threatening causes. Advances in CT may further improve the detection of PI and hepatic portal and portomesenteric venous gas; 16-and 64-MDCT scanners are capable of generating isotropic data sets that allow multiplanar reformations with a spatial resolution similar to or even greater than the axial plane. The ability to study the bowel wall in the coronal, sagittal, and axial planes may allow a more confident diagnosis of pneumatosis cystoides intestinalis and portal venous gas.

Microscopically, sessile or pedunculated, single or multiple gas cysts are visible in the serosal and mucosal surface of the bowel. They may vary in size from a few millimeters to severe centimeters. These cysts have also been noted in the submucosa, with the same range of characteristics. Palpation reveals a spongy crepitant cushion-like consistency.

Microscopically, the cysts are found in order of decreasing frequency in the serosa and subserosa, the submucosa, and the muscularis layers. The cysts are lined by endothelial cells with eosinophilic protoplasm and small, dark round nuclei. Large multinucleated giant cells are seen in close proximity to the lining.

Treatment:

The use of high concentration of oxygen in the treatment of gas containing cavities was first proposed in 1935. Successful acceleration of absorption of gas from body cavities has been reported for pneumatosis cystoides intestinalis. The rationale for this type of treatment depends upon two facts. The first is that the net movement of a dissolved gas follows the direction of the partial pressure gradient between the cavity and the bloodstream. The second involves the fact that the cysts contain gases other than oxygen. Researchers postulated that the use of hyperbaric oxygen results in an increased partial pressure of non-oxygen gases within the cysts, and washing out of all non-oxygen gases from bloodstream. This results in a steeper diffusion gradient which accelerates the reabsorption of the gases within the cyst. The oxygen which equilibrates within the cyst during treatment is absorbed and utilized for cellular metabolism.

Surgical resection of involved bowel, with its attendant morbidity and mortality has been complicated by recurrence of the disease. Normobaric oxygen treatment lasting six to ten days, has the advantage of being noninvasive, but does expose the patient to pulmonary oxygen toxicity. Recurrence following treatment is positive.

Hyperbaric oxygen therapy at 2.5 ATA has the advantage of requiring only two to three treatments. It is also noninvasive, and does not cause pulmonary oxygen toxicity. The incidence of clinically significantly pulmonary or CNS oxygen toxicity is very low, and has proven reversible on discontinuation of therapy.

NOTE: The above information is for processing purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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