GASTROINTESTINAL COMPLICATIONS OF PXE

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By:  Dr. Kenneth Neldner

The major gastrointestinal (GI) complications of PXE thus far reported have been primarily gastrointestinal hemorrhages. A rare complication, gastrointestinal hemorrhages occur in only about 10% of those with PXE. When they do occur, they occur more frequently in the stomach than in any other area of the GI tract.

Most gastric stomach hemorrhages will stop over a period of several days with conservative management and blood replacement if necessary. For those few patients who continue to bleed despite medical management, surgical intervention may become necessary. The surgeon should be aware of the fact that the stomach seldom shows a specific bleeding spot or vessel that can be ligated as in most other causes of gastric hemorrhages. A general oozing of blood from the stomach wall has been most commonly reported, so the decision must be made whether to do essentially nothing to the stomach or to do a partial gastrectomy. In most such instances, a partial gastrectomy has been performed. Decision making is often complicated by the fact that, at least in many of the reported cases, the diagnosis of PXE was unknown or unrecognized at the time of the hemorrhage, so many other causes are considered, such as gastric or duodenal ulcers, acute gastritis, or malignancies of the stomach (none of which are related to PXE).

The PXE patient with acute sudden vomiting of blood should be hospitalized and placed at rest with sedation, cimetadine, antacids, and preferably no milk products because of the known ability of calcium to induce further gastric hyperacidity. Frequent ingestion of antacids at hourly intervals and little to no food during the acute stage is recommended. Careful attention to hemoglobin, hematocrit, and fluids and electrolytes is mandatory, with intravenous replacement when indicated.

Gastric hemorrhage has received considerable attention in the medical literature because many of the cases occurred in younger patients or in pregnant women whose underlying PXE was not recognized or was belatedly recognized and then considered to be unusual enough to warrant a case report in a medical journal. The youngest case on record, and probably the youngest ever, was in a three-year-old with PXE.

Gastric hemorrhage during pregnancy is uncommon, but when it does occur, it is believed to be related to pressure on the stomach from the enlarging fetus which interferes with normal peristaltic movements and prevents the stomach from assuming a normal, relaxed position between meals, causing increased mechanical stress on the stomach. Another possible mechanism not previously suggested relates to fats that the average pregnant woman ingests through dairy products and calcium supplements. Calcium stimulates gastric acidity and stomach irritation and may enhance local absorption of calcium into the gastric mucosae and vasculature resulting in an increased tendency to rupture and bleed. Until more definitive data are available, ingestion of calcium greater than 1200mg/day, which is the RDA during pregnancy, should not be exceeded in PXE, and it may be prudent to limit to 1000 mg/day.

It should be reemphasized that most of the common pain-relieving drugs should not be taken throughout pregnancy. The nonsteroidal anti-inflammatory drugs (NSAIDs) should be avoided by everyone with PXE because they are well known to cause irritation and mild bleeding in anyone who takes them regularly. This is in addition to their anticoagulant properties which enhance bleeding at any site. An occasional aspirin or NSAID for a headache is acceptable.

By:  Dr. Kenneth Neldner 3:3 (Summer 1995)