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Clinical diagnosis

Case 262

4. Fitz Hugh Curtis syndrome


【Discussion】
 Pelvic inflammatory disease (PID) so called pelvic peritonitis occurs exclusively in baby-gifted aging female. The pathogen enters from vagina to peritoneal cavity through uterus lumen and Fallopian tube. The main pathogens are gonococcus causing gonorrhea and chlamydia trachomatis causing chlamydia. Incidence of Chlamydia trachomatis increases year by year. It is reported that PID occurs approximately 4 million females annually and approximately a half of them arises from chlamydia trachomatis in USA (1, 2). When PID reaches to liver capsule, it is called Fitz-Hugh Curtis syndrome. Two gynecologists, Fitz-Hugh and Curtis found this disease 1930ies (3, 4).
 Chlamydia trachomatis is gram negative bacillus with 0.3 to 0.4 μm in size whose size is less than a half as common bacteria of 1 μm and three or four-folds as virus of 0.1 μm (2). Chlamydia trachomatis infects epithelial cell of vagina, uterus, Fallopian tube, ovary and peritoneum, causing vaginitis (discharge), endometritis, tubo-ovaritis (salpingitis) and peritonitis, respectively.
 When chlamydia trachomatis contact to epithelium, epithelium cell engulfs chlamydia trachomatis of elementary body with potency of infection. When elementary body is engulfed in the cell, it forms cyst where elementary body turns reticulate body with potency of multiply to create new elementary bodies. Proliferated elementary bodies are released accompanied with lysis of the host cell, extending infection to the upper genital tract, finally reaching peritoneal cavity and liver capsule (2).
 It is thought that when chlamydia infects, macrophages and lymphocytes work at first inducing to create antibody, and then, neutrophils are called to control because chlamydia trachomatis is one of the bacilli. Most patients with Chlamydia are asymptomatic due to that they are left under control of immune system (5-8). However, the number of chlamydia trachomatis increase and excel the immune potency, symptoms emerge such as slight fever, CRP elevation, increase of white blood cells, Murphy symptom (right hypochondoralgia, often experienced in cholecystitis), and abdominal muscle tension pain (5-8).
 Futz-Hugh Curtis syndrome which indicates liver capsule inflammation indicate a relatively advanced stage of Chlamydia trachomatis. Violin string sign found on laparoscopy and liver capsule attenuation on arterial phase of contrast-enhanced CT are characteristics of this syndrome. Violin string sign is fibrous tissue indicative of absorption process of fibrocytes and liver capsule attenuation only demonstrated on arterial phase but not on portal phase and delayed phase indicative of permeability of capillaries by neutrophils infiltration.
 In our case, she experienced abdominal muscle tension pain, slight fever, and liver capsule enhancement was demonstrated on arterial phase of contrast-enhanced CT. Thread between liver capsule and stomach wall was also demonstrated on CT on contrast-enhanced CT. Laboratory test revealed positive chlamydia trachomatis Ig A and Ig G. These are characteristic of Fitz-Huge Curtis syndrome.


【Summary】
 We presented a twenty-three-year-old female for epigastric pain and slight fever. She worked for sex industry for a month. Laboratory test revealed CRP 0.79mg/dL, Chlamydia trachomatis IgA 7.66 μg/mL, IgG 17.7μg/mL and liver capsule enhancement was demonstrated on arterial phase of contrast-enhanced CT, indicative of diagnosis of Fitz-Hugh Curtis syndrome. It is borne in mind that Fitz-Hugh Curtis syndrome is a relatively advanced stage of pelvic inflammatory disease caused by Chlamydia trachomatis. Chlamydia trachomatis is gram-negative bacillus and its size is the medium (0.3 to 0.4 μm ) between common bacillus and viruses. Liver capsule enhancement on arterial phase of contrast-enhanced CT is due to that infiltration of neutrophils facilitates permeability of capillaries in the liver capsule and violine string sign on laparoscopy is due to fibrous proliferation produced by fibroblasts. In our case, liver capsule enhancement and violin string-like fibrous component were demonstrated on arterial phase of contrast-enhanced CT.


【References】
1.Wang PY, et al. Fitz-Hugh-Curtis syndrome: clinical diagnostic value of dynamic enhanced MSCT. J Phys Ther Sci. 2015 Jun; 27(6): 1641–1644.
2.Patel LA, et al. Prevalence of Chlamydial infection among women visiting a gynaecology outpatient department: evaluation of an in-house PCR assay for detection of Chlamydia trachomatis. Ann Clin Microbiol Antimicrob. 2010;9:24–33
3.Curtis AH. A case of adhesionin the right upper quadrant. J Am Med Assoc, 1930, 94: 1221–1222.
4.Fitz-Hugh T Acute gonococcic peritonitis of the right upper quadrant in women. J Am Med Assoc, 1934, 102: 2094–2096.
5.McCormick M, et al. An atypical presentation of the Fitz-Hugh-Curtis syndrome. J Emerg Med, 1990, 8: 55–58.
6.Khine H, et al. Fitz-Hugh-Curtis Syndrome in Adolescent Females: A Diagnostic Dilemma. Pediatr Emerg Care. 2019 Jul;35(7):e121-e123.
7.Kimball MW, et al. Gonococcal perihepatitis in a male. The Fitz-Hugh--Curtis syndrome. N Engl J Med. 1970 May 07;282(19):1082-4.
8.Wølner-Hanssen P, et al. Perihepatitis and chlamydial salpingitis. Lancet. 1980 Apr 26;1(8174):901-3.

2022.4.4



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