Congenital pleuroperitoneal communication in a patient with pseudomyxoma peritonei

Background and Objectives: Pseudomyxoma peritonei syndrome is a rare diseas e arising from a perforated appendiceal adenoma. The syndrome is characteri zed by progressive accumulation of mucinous ascites and tumor within the pe ritoneal cavity. Direct extension of pseudomyxoma peritonei to the pleural cavity is uncommon and has been associated with surgical penetration of the diaphragm at the time of cytoreduction. Methods: We review the case of a patient who presented with mucoid peritone al and pleural fluid consistent with spontaneous pleural spread of pseudomy xoma peritonei. Results: Surgical exploration confirmed direct pleuroperitoneal communicati on by macroscopic diaphragmatic fenestration. Conclusions: This is a rare phenomenon. We outline a therapeutic approach t o be applied when pleural involvement is suspected in patients with pseudom yxoma peritonei syndrome. (C) 2000 Wiley-Liss, Ins.