J. Esquivel et Ph. Sugarbaker, Pseudomyxoma peritonei in a hernia sac: analysis of 20 patients in whom mucoid fluid was found during a hernia repair, EUR J SUR O, 27(1), 2001, pp. 54-58
Introduction: Pseudomyxoma peritonei syndrome is a rare disease that causes
extensive accumulation of noninvasive mucinous tumour and mucinous fluid t
hroughout the abdomen and pelvis, The primary tumour is a perforated append
iceal adenoma or mucinous adenocarcinoma. In patients with an open processu
s vaginalis or other peritoneal defect, the adenomatous epithelial cells ma
y accumulate within the hernia sac, causing mucoid fluid to be present at t
he time of hernia repair.
Materials and methods: From February 1991 to September 1997, the diagnosis
of pseudomyxoma peritonei syndrome was established in 20 patients in whom m
ucoid material was found in a hernia sac during a hernia repair. Clinical f
eatures of these patients were critically evaluated using the completeness
of cytoreduction as an endpoint.
Results: The treatments delivered at the time of hernia repair had no impac
t on the outcome, A lack of interim events vs the use of interim treatments
between hernia repair and definitive cytoreduction was significant in rela
tion to a complete vs incomplete cytoreduction. Non-aggressive histology an
d an interval of less than I year was statistically significantly associate
d with a favourable outcome from surgical intervention,
Conclusion: When mucoid fluid is encountered at the time of a hernia repair
, recovery of that fluid and the hernia sac for histologic study is importa
nt. When adenomatous epithelial cells are present, a CT scan should be perf
ormed to look for the typical distribution of mucinous tumour and fluid tha
t suggests the pseudomyxoma peritonei syndrome. If the diagnosis can be con
firmed, referral to a centre experienced in the treatment of this rare dise
ase is suggested, (C) 2001 Harcourt Publishers Ltd.