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Abdominal complications

Perihepatitis: Fitz - Hugh - Curtis syndrome

Once chlamydiae gain access to the peritoneal cavity they may infect a number of the internal organs, of which the liver is the commonest. 

Curtis, a Chicago gynaecologist, noted in 1930 an association between the presence of "violin string" adhesions between the liver and the abdominal wall and evidence of upper genital tract infection due to gonorrhoea. Subsequently Fitz-Hugh in 1934 described the early manifestations of perihepatitis [see the excellent review of perihepatitis by Bolton & Darougar 1983].

Fitz - Hugh - Curtis syndrome [perihepatitis] due to chlamydiae or gonococci occurs almost entirely in women.  It is the result of ascending infection from the lower genital tract via the fallopian tubes and paracolic gutters to the subphrenic space where it involves the liver capsule. It may also involve the spleen [perisplenitis],  the kidney [perinephritis] [Gatt & Jantet, 1987] or the appendix [periappendicitis].  Very rarely infection may spread through the blood stream, perhaps explaining rare cases of apparent perihepatitis in men [Bolton & Darougar, 1983]. 

The syndrome presents as a sudden onset of frequently severe pain in the right upper quadrant of the abdomen. The pain is typical of peritoneal inflammation, being made worse by movement, deep breathing and abdominal palpation. It may be accompanied by nausea and anorexia.  Frequently the pain extends to the back and the right shoulder. There may be right upper quadrant tenderness, guarding and a hepatic friction rub may be heard over the right anterior costal margin. Roughly half the patients have a low grade pyrexia, but jaundice and signs of generalized peritonitis are usually absent. However, signs of pelvic peritonitis may be found if carefully sought.  Most patients are initially suspected of suffering from biliary diseae. Other possibilities in the differential diagnosis include pleurisy, pneumonia, pulmonary embolism or, rarely, perforation of an intra-abdominal viscus.  Failure to make the correct diagnosis may lead to extensive and unnecessary investigations and treatment, with neglect of the underlying genital infection. However, careful questioning will elicit a history of genital infection or pelvic inflammatory disease in at least two thirds of patients [Bolton & Darougar, 1983]. Symptoms from the right upper abdomen in a young woman should be considered a possible  indirect sign of a genital infection [Wolner-Hannsen et al., 1980]. However, perihepatitis may also occur in the absence of liver symptoms [Wolner-Hannsen et al., 1980]. 

At laparoscopy, "violin-string" adhesions between the liver surface and the abdominal wall can often be seen [van Dongen et al., 1993]. These adhesions frequently give rise to pain. The adhesions are fragile and friable and may be broken at laparoscopy giving rise to an appearance of white fibrous plaques and hemorrhagic spots. The perihepatitis may be associated with ascites [abdominal fluid; Haight & Ockner, 1988; van der Laan et al., 1995] which, together with the adhesions, may be detectable by ultrasound examination [van Dongen et al., 1993]. Thickening of the right anterior extra-renal tissue in perihepatitis has also been observed using  ultrasound [Schoenfeld et al., 1992]. Ultrasound can be a useful tool to diagnose  Fitz - Hugh - Curtis syndrome or pelvic inflammatory disease  in a timely , non-invasive way [van Dongen et al., 1993; Schoenfeld et al., 1992]. In disease of chlamydial origin, serology generally shows high levels of antibody to C. trachomatis [Puolakkainen et al., 1985], although these should not be considered diagnostic.

Perihepatitis occurs with or without clinically evident cervicitis or salpingitis. Compared with women with salpingitis alone, a prospective study found that patients with perihepatitis plus salpingitis did not have distinctive clinical or microbiologic findings from those with salpingitis alone, but they did have a higher prevalence of moderate-to-severe pelvic adhesions [70% against 35%; p=.003] and of antibody to chlamydial heat shock protein [67% versus 28%, p=0.005]. Interestingly a history of current use or ever having used an oral contraceptive mitigated against perihepatitis [p=0.05 and p=0.008 respectively; Money et al., 1997], as previously reported in the study of Wolner-Hannsen, 1986.

Experimental studies of chlamydial genital tract infection in female mice suggest that cell mediated immune responses are important for preventing severe upper genital tract infection and perihepatitis  [Thoma-Uszynski et al., 1998].

Treatment guidelines are provided by Ross, 2001.

[MEW] Updated March 2002

NEXT: Prevention of chlamydial genital tract infections.

References

Anonymous (1999). National guideline for the management of pelvic infection and perihepatitis. Clinical Effectiveness Group (Association of Genitourinary Medicine and the Medical Society for the Study of Venereal Diseases). Sexually Transmitted Infections 75, Suppl 1; S54 - S56.

Bolton, J. P., Darougar, S. (1983). Perihepatitis. British Medical Bulletin 39, 159 - 162.

Garcia Compean, D., Blanc, P., d'Abrigeon, G., Larrey, D. & Michel H. (1995). Fitz-Hugh and Curtis syndrome. Presse Medicale 24, 1348 - 1351. [Review based on around 100 cases. In French].

van der Laan, N.  E., Voerman, B. J., Rustemeijer, C. & van der Hoeven, K. J. (1995). Peritonitis, moderate ascites and hepatitis due to infection with Chlamydia trachomatis and Epstein-Barr virus in a young woman. Diagnosis by polymerase chain reaction from peritoneal tissue. Netherlands Journal of Medicine 46, 41 - 43.

van Dongen, P. W. (1993). Diagnosis of Fitz-Hugh-Curtis syndrome by ultrasound. European Journal of Obstetrics Gynecology &  Reproductive Biology 50, 159 - 162.

Gatt, D. & Jantet, G. (1987). Perisplenitis and perinephritis in the Curtis-Fitz-Hugh syndrome. British Journal of Surgery 74, 110 - 112.

Haight, J. B. & Ockner, S. A. (1988). Chlamydia trachomatis perihepatitis with ascites. American Journal of Gastroenterology 83, 323 -325.

Money, D. M., Hawes, S. E., Eschenbach, D. A., Peeling, R. W., Brunham, R., Wolner-Hanssen, P& Stamm, W. E. (1997). Antibodies to the chlamydial 60 kd heat-shock protein are associated with laparoscopically confirmed perihepatitis. American Journal of Obstetrics and Gynecology 176, 870 - 877.

Puolakkainen, M., Saikku, P., Leinonen, M., Nurminen, M., Vaananen, P. & Makela, P. H. (1985). Comparative sensitivity of different serological tests for detecting chlamydial antibodies in perihepatitis. Journal of Clinical Pathology 38, 929 - 932.

Ross, J. D. (2001). European guideline for the management of pelvic inflammatory disease and perihepatitis. International Journal of STD and AIDS. 12 Suppl 3,  84 - 87.

Schoenfeld A, Fisch B, Cohen M, Vardy M, Ovadia J. (1992). Ultrasound findings in perihepatitis associated with pelvic inflammatory disease. Journal of Clinical Ultrasound 20, 339 - 342.

Thoma-Uszynski S, Simnacher U, Marre R, Essig A. (1998). Clearance of Chlamydia trachomatis-induced polyserositis in SCID mice requires both CD4+ and CD8+ cells. Medical Microbiology and Immunology (Berlin) 187, 71 - 78.

Wolner-Hanssen, P. (1986). Oral contraceptive use modifies the manifestations of pelvic inflammatory disease. British Journal of Obstetrics & Gynaecology 93, 619 - 624.

Wolner-Hanssen, P., Westrom, L. & Mardh, P. A. (1980). Perihepatitis and chlamydial salpingitis. Lancet. 1 (8174), 901 - 903.

NEXT Prevention of chlamydial genital tract infection