Successful management of late-onset primary lymphatic hypoplasia.

Primary lymphedema of the extremities, abdomen, or chest is an unusual and difficult clinical problem with few guidelines for management. A case is reported of lymphedema acquired at the age of 61 years, with associated massive chylous ascites and chylothorax. No underlying condition was discovered and the patient was found to have hypoplastic lymphatics by lymphangiography. Initial management consisted of extremity elevation, diuresis, and repeated paracenteses and thoracenteses. A peritoneojugular shunt provided temporary relief. Surgical pleurodesis combined with intensive diuresis has given prolonged relief of his symptoms allowing him to return to a functional life. Diagnostic and therapeutic guidelines for the management of this unusual condition are suggested.

Chronic primary intestinal pseudo-obstruction.

Chronic primary intestinal pseudo-obstruction (CPIP) has received attention despite of its unclear etiology and infrequent occurrence. Recently a patient with this disorder had evidence of a primary visceral neuropathy. Reviewing the literature, we found 30 case reports of CPIP and evaluated their clinicopathologic findings. Presenting symptoms and radiologic findings were nonspecific. Esophageal motility was abnormal in 12 of 14 reports. Intestinal histopathology revealed normal muscle wall, mucosa, and ganglion cells in over 50% of reports. Only 48% of cases demonstrated clinical improvement. Thirty percent (8 of 30) ultimately died. We conclude that CPIP is a perplexing, often fatal entity that can mimic mechanical obstruction in the absence of definite etiology. Primary neurologic or muscular disease may be a possible explanation, but, as yet, definite documentation does not exist.