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Congenital pleuroperitoneal communication in a patient with pseudomyxoma peritonei
S R Pestieau1, R Wolk, P H Sugarbaker
1The Washington Cancer Institute, Washington Hospital Center, Washington, DC 20010, USA.
Background And Objectives:
Pseudomyxoma peritonei syndrome is a rare disease arising from a perforated appendiceal adenoma. The syndrome is characterized by progressive accumulation of mucinous ascites and tumor within the peritoneal 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 peritoneal and pleural fluid consistent with spontaneous pleural spread of pseudomyxoma peritonei.
Results:
Surgical exploration confirmed direct pleuroperitoneal communication by macroscopic diaphragmatic fenestration.
Conclusions:
This is a rare phenomenon. We outline a therapeutic approach to be applied when pleural involvement is suspected in patients with pseudomyxoma peritonei syndrome.
Insights
Pseudomyxoma peritonei syndrome can rarely spread to the pleural cavity. This case highlights spontaneous pleuroperitoneal communication, offering insights into managing this rare phenomenon.
Area of Science:
- Oncology
- Gastroenterology
- Thoracic Surgery
Background:
- Pseudomyxoma peritonei syndrome (PMP) originates from perforated appendiceal adenomas, causing mucinous ascites and peritoneal tumors.
- Direct pleural extension of PMP is rare, often linked to diaphragmatic surgical penetration during cytoreduction.
Observation:
- A patient presented with mucoid peritoneal and pleural fluid, suggesting spontaneous pleural spread of PMP.
- Surgical evaluation revealed a direct pleuroperitoneal communication via diaphragmatic fenestration.
Findings:
- The case confirms spontaneous diaphragmatic fenestration as a rare route for PMP pleural metastasis.
- This finding challenges the assumption that surgical intervention is the sole cause of diaphragmatic PMP spread.
Implications:
- This case underscores the importance of considering spontaneous pleural involvement in PMP.
- A therapeutic strategy is proposed for suspected pleural involvement in PMP patients.
- Further research into the mechanisms of spontaneous PMP spread is warranted.