Fitz-Hugh-Curtis Syndrome

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Diagnosis and reasoning

This patient has presented with a perplexing combination of symptoms : pain in the right upper quadrant AND the pelvis. Examination shows her to be markedly febrile, with right hypochondriac (RHC) tenderness but no hepatomegaly. More importantly though, it also reveals cervical motion tenderness and bilateral adnexal tenderness - a pair of signs found in only a few conditions : pelvic inflammatory disease (PID), endometriosis and very rarely, ectopic pregnancy (in which unilateral adnexal tenderness is more usual). Ectopic pregnancy can be excluded by virtue of her history. While endometriosis can cause right upper quadrant pain (due to diaphragmatic endometriosis), this is rare - and does not explain her fever. Note also that the pain of endometriosis is often cyclic and typically follows a more protracted course. PID is a definite possibility - in which case the RHC tenderness might be due to inflammation of the liver capsule (the Fitz-Hugh-Curtis syndrome - FHCS). It is also possible that the pelvic pain and RHC pain are completely unrelated - in which case the RHC pain might be due to acute hepatitis or biliary tract disease. Her liver function tests rule out acute hepatitis, while the ultrasound findings do not favor biliary tract pathology. While the free fluid in the pelvis is supportive of PID, this is too non-specific a finding. A multiphase CT scan of the abdomen and pelvis is a good second line imaging investigation. In this patient, the thickened fallopian tubes and uterosacral ligaments indicate the presence of pelvic inflammation. In addition, the enhancement during the arterial phase is strongly suggestive of hepatic capsular inflammation. The final step is to establish the etiological agent of the pelvic inflammation. The nucleic acid amplification tests for gonococcus and chlamydia (GC/CT NAAT) implicate Chlamydia trachomatis as the causative organism. When taken together, these findings are suggestive of FHCS syndrome secondary to chlamydial PID. Note that definitive diagnosis of FHCS requires direct visualization of the liver via laparoscopy or laparotomy. However, these invasive procedures are usually unnecessary if characteristic imaging features are present and a supportive etiological agent is isolated. Antibiotics are the mainstay of therapy and should be commenced immediately. In addition, as chlamydia is a sexually transmitted infection (STI), her partner should be screened and treated as necessary. Steroids are not indicated in the management.


Discussion

FHCS is a rare complication of pelvic inflammatory disease (PID) where there is inflammation of the liver capsule (perihepatitis). While this almost exclusively affects females, a few male cases have also been reported. Both Neisseria gonorrhoeae and Chlamydia trachomatis have been shown to cause FHCS. The incidence varies from 4% to 14% in women with PID, with a relatively higher incidence in adolescents than older women. The risk factors are the same as for PID, i.e. multiple sexual partners, unprotected sexual intercourse, age less than 25 years, and a history of STIs. The pathogenesis of perihepatitis in FHCS is still poorly understood, with several mechanisms postulated. One such mechanism is direct infection of liver capsule by bacteria which travel from the genital tract to the liver capsule via the paracolic gutters. Other suggestions include hematogenous and lymphatic spread of organisms; or a hyperimmune response to C. trachomatis giving rise to non-infectious perihepatitis. The perihepatitis typically gives rise to a sharp pain in the right upper abdomen, which may sometimes be referred to the right shoulder or arm due to accompanying inflammation of the underside of the diaphragm. Fever, malaise, hiccups, nausea and vomiting are also frequently present. Symptoms of PID (such as lower abdominal pain and vaginal discharge) are almost universal - and may either accompany the upper abdominal pain, or precede it by several days. FHCS is often a very tricky diagnosis, as it may mimic several other conditions. Imaging studies are invaluable in establishing the presence of perihepatitis and excluding other differentials. Note that endocervical screening is negative in some patients - this does not exclude FHCS. The treatment of FHCS is similar to that of PID, and includes antibiotics effective against N. gonorrhoeae and C. trachomatis. The symptoms usually resolve quickly after initiation of therapy. In a rare few patients, symptoms persist, potentially necessitating surgical exploration and adhesiolysis. The overall prognosis is good, with most patients recovering completely following antibiotic therapy.


Take home messages

  1. FHCS is a rare complication of PID, and is often a tricky diagnosis.
  2. While direct inspection of the liver is required for definitive diagnosis, supportive imaging findings and isolation of a likely etiological agent is usually adequate.
  3. A negative endocervical screen does not exclude FHCS - but does make the diagnosis harder.
  4. FHCS usually resolves completely following antibiotic therapy.

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  1. Centers for Disease Control and Prevention: Sexually Transmitted Diseases Treatment Guidelines 2010: Pelvic Inflammatory Disease
  2. Green Top Guideline No 32: Management of Acute Pelvic Inflammatory Disease (Nov 2008)
  3. UK National Guideline for the Management of Pelvic Inflammatory Disease(2011)
  4. World Journal of Gastroenterology: Clinical outcome of Fitz-Hugh-Curtis syndrome mimicking acute biliary disease (2008)