Fitz Hugh Curtis syndrome

Fitz-Hugh-Curtis syndrome or perihepatitis, is a chronic manifestation of pelvic inflammatory disease (PID) in which a woman has swelling of the tissue covering the liver ¹. Fitz-Hugh-Curtis syndrome is described as an inflammation of the liver capsule, without the involvement of the liver parenchyma, with adhesion formation accompanied by right upper quadrant pain ². Fitz-Hugh-Curtis syndrome symptoms most often include pain in the upper right abdomen just below the ribs, fever, nausea, or vomiting. The symptoms of pelvic inflammatory disease (PID) is pain in the lower abdomen and vaginal discharge – are often present as well.

Fitz-Hugh-Curtis syndrome is usually caused by an infection of chlamydia or gonorrhea that leads to pelvic inflammatory disease; it is not known why pelvic inflammatory disease progresses to Fitz-Hugh-Curtis syndrome in some women ³.

A final diagnosis can be made through laparoscopy or laparotomy via direct visualization of violin string-like adhesions or through hepatic capsular biopsy and culture.

Fitz-Hugh-Curtis syndrome is treated with antibiotics, given by intravenous (IV) injection or as medication taken by mouth ⁴. The specific antibiotic medication is determined by the type of underlying infection; that is, treatment depends on whether the infection is chlamydia or gonorrhea. If pain continues after treatment with antibiotics, surgery (laparoscopy) may be done to remove bands of tissue (adhesions) that connect the liver to the abdominal wall and cause pain in individuals with Fitz-Hugh-Curtis syndrome ³.

How long does it take pelvic inflammatory disease to develop into Fitz-Hugh-Curtis syndrome?

Fitz-Hugh-Curtis syndrome develops in up to one fourth of individuals of pelvic inflammatory disease (PID). However, it is not yet known exactly how or why PID progresses to Fitz-Hugh-Curtis syndrome ⁴. The time it takes for PID to develop into Fitz-Hugh-Curtis syndrome is also unknown.

If left untreated, what symptoms does Fitz-Hugh-Curtis syndrome cause?

Because Fitz-Hugh-Curtis syndrome is usually cured with antibiotics, long-term effects of this disease are uncommon. Rare long-term complications are thought to be related to pelvic inflammatory disease rather than Fitz-Hugh-Curtis syndrome and may include persistent pain, bowel obstruction, or infertility ⁴.

Fitz-Hugh-Curtis syndrome cause

Fitz-Hugh-Curtis syndrome is a complication of pelvic inflammatory disease (PID). Microorganisms associated with pelvic inflammatory disease are thought to spread through one of three ways:

1. Spontaneous ascending infection whereby microbes from the cervix or vagina travel to the endometrium, through the fallopian tubes, and into the peritoneal cavity. Complications include endometritis, salpingitis, tubo-ovarian abscess, pelvic peritonitis, and Fitz-Hugh-Curtis syndrome ⁵.
2. Lymphatic spread, such as infection of the parametrium from an intrauterine device
3. Hematogenous spread, such as with tuberculosis ⁶.

Pelvic inflammatory disease (PID) is an ascending microbial infection involving the genital tract that affects sexually active women between 15 to 30 years of age. The United States experiences 750,000 cases of pelvic inflammatory disease (PID) each year. Fitz-Hugh-Curtis syndrome is an uncommon manifestation of pelvic inflammatory disease (PID) involving around 4% of adolescents. While many organisms are associated with Fitz-Hugh-Curtis syndrome, Chlamydia trachomatis and Neisseria gonorrhoeae are the most common pathogens involved ⁷.

Fitz-Hugh-Curtis syndrome symptoms

The most common symptom that patients with Fitz-Hugh–Curtis syndrome who visit the emergency rooms experience is usually acute abdominal pain. Typically, patients with Fitz-Hugh-Curtis syndrome are women of childbearing age who visit a hospital with complaints of acute pain or chronic tenderness in the right upper abdomen. A thorough history and a high index of suspicion are necessary to reach an appropriate diagnosis. Right upper quadrant abdominal pain is a symptom of myriad pathologies including, but not exclusive to, biliary disease like gall bladder stones or cholecystitis, pleurisy, right pyelonephritis, subphrenic abscess, or herpes zoster infection and clinicians have to consider also potential duodenal ulcers, liver abscess, subphrenic abscess, and herpes zoster infectionmaking an assessment for Fitz-Hugh-Curtis syndrome particularly difficult ⁸.

The evaluating physician who suspects Fitz-Hugh-Curtis syndrome should focus on high-risk behaviors and symptoms in the appropriate patient population. Risk factors to consider are an age less than 25 years, age at first sexual encounter less than 15 years, history of pelvic inflammatory disease (PID), use of IUD or oral contraceptives, recent IUD insertions, and vaginal douching. Investigating a patient’s exposure to new, multiple, or symptomatic sex partners is also of paramount importance. Obtaining a complete past medical and past surgical history also may help narrow the differential further.

Right upper quadrant abdominal pain is caused by perihepatic inflammation and adhesion formation between the anterior surface of the liver and the abdominal wall. The pain is usually worse with movement and breathing, thereby mimicking other acute abdominal pathologies. Patients also may complain of lower abdominal, pelvic, or back pain with varying degrees of severity. Other symptoms may include fevers, chills, nausea, vomiting, vaginal discharge, dyspareunia, dysuria, cramping, and postcoital bleeding ⁶.

The inflammation of the upper side of the diaphragm usually causes a sharp right upper quadrant pain; pain can also be spotted at the right shoulder or the inside of the right arm, accompanied by nausea, vomiting, night sweats, headache, and malaise. Additionally, pain usually becomes stronger upon movement ⁹. A pleuritic right-sided chest pain could also occur, mimicking pleurisy, pneumonia, and pulmonary embolism ¹⁰. Nevertheless, there have been reports in the literature about Fitz-Hugh–Curtis syndrome patients complaining about left upper quadrant pain posing as perisplenitis, thus, with no participation of the liver ¹¹.

Physical exam findings may reveal the following:

• Vital signs: Fever greater than 100.9 °F (>38.3 °C).
• Abdominal exam: Right upper quadrant tenderness, rebound tenderness, guarding, or a silent abdomen.
• Pelvic exam: Cervical motion tenderness, adnexal tenderness, uterine compression tenderness on bimanual examinations. Look for signs of lower genital tract infection such as cervical mucopus and cervical friability on speculum examination ⁷.

Fitz-Hugh-Curtis syndrome diagnosis

Fitz-Hugh–Curtis syndrome poses a diagnostic puzzle, as it mimics many known pathologic tracks. Though it is usually set off by PID, it can be portrayed as left renal colic, urinary tract infection, acute appendicitis, pulmonary embolism, and acute or chronic cholecystitis ¹².

Differential diagnosis of Fitz-Hugh–Curtis syndrome:

• Appendicitis
• Cholelithiasis
• Cholecystitis
• Enteroviral epidemic pleurodynia (Bornholm disease)
• Hepatitis
• Herpes zoster
• Nephrolithiasis
• Pancreatitis
• Pleurisy
• Pneumonia
• Pulmonary embolism
• Rib fracture
• Pyelonephritis
• Perforated ulcer
• Subphrenic abscess

The following are helpful in the evaluation of Fitz-Hugh-Curtis syndrome and PID.

Lab tests

• Performing a pregnancy test will not only guide the choice of antibiotic therapy but also address the possibility of an ectopic pregnancy.
• Complete blood count (CBC) to assess for leukocytosis. Know however that only up to 50% of women with PID have a clinically significant leukocytosis. Blood cultures can vary and are generally negative in the setting of PID.
• Complete metabolic panel to assess for any electrolyte, renal, or hepatic derangements.
• Vaginal secretions can be assessed for leukorrhea.
• Quantitative culture for chlamydia along with gonorrhea and chlamydial DNA probes can aid in diagnosis.
• Other tests to consider include RPR, Hepatitis B and C, HIV, and urinalysis.

Radiological findings

• CT scan will show increased perihepatic enhancement in the arterial phase with a majority of patients also showing pelvic fat infiltration. Other findings associated with PID can be found: pyosalpinx, tubo-ovarian abscess, and fluid collection in the pelvic cavity.
• Transvaginal ultrasonographic scanning is a favorable option for cases in which a clinical picture of PID may be unclear. Findings can include hydrosalpinx, pyosalpinx endometritis, tubo-ovarian abscess, oophoritis, and ectopic pregnancy.
• MRI can show tubo-ovarian abscess, edematous tubes, or free pelvic fluid collections.

Procedural findings

• Laparoscopy is the gold standard for diagnosing Fitz-Hugh-Curtis syndrome and PID. In the setting of PID, laparoscopy can show edema with exudates on tubal surfaces, ectopic pregnancy, or tubo-ovarian abscess. Fitz-Hugh-Curtis syndrome can be diagnosed directly via visualization of adhesions between the diaphragm and liver or liver and the anterior abdominal wall.
• An endometrial biopsy can show endometritis.

Fitz-Hugh-Curtis syndrome treatment

Treatment of Fitz-Hugh-Curtis syndrome coincides with the management of PID. Goals of treatment are to relieve symptoms, eradicate the infection, and minimize risks of long-term complications (infertility or ectopic pregnancy). As the diagnosis of PID may be challenging and the potential for serious complications is great, the Centers for Disease Prevention and Control (CDC) advises that physicians maintain a low threshold for aggressive treatment. Antibiotics are successful in up to 75% of cases and most patients with PID can be managed as outpatients. Antibiotic therapy should be geared at covering the most common organisms, C. trachomatis, and N. gonorrhea, as well as gram-negative organisms, anaerobes, and streptococci ¹³.

Depending on the degree of suspicion, antibiotics regimens can be tailored for each patient. Most commonly, ceftriaxone and azithromycin are adequate for the control of gonococcal and chlamydial infections ¹⁴. Current recommendations for complicated pelvic inflammatory disease include ceftriaxone, doxycycline, and metronidazole ¹⁵.

Hospitalization should be considered for patients with the following conditions:

• Uncertain diagnosis
• Pregnancy
• Severe illness
• Pelvic abscess on imaging
• Inability to tolerate anything by mouth
• Immunodeficiency
• Failure to improve after 72 hours of therapy

Patients with persistent symptoms of fever, chills, or cervical motion tenderness after 72 hours of treatment should be reevaluated for possible surgical intervention. Diagnostic laparoscopy is warranted in the setting of Fitz-Hugh-Curtis syndrome for symptomatic adhesiolysis and PID with goals of conserving reproductive potential with abscess drainage or unilateral adnexectomy if necessary. Laparotomy is usually reserved for patients experiencing surgical emergencies (ruptured abscesses) and for patients who are not candidates for laparoscopic intervention.

Fitz-Hugh-Curtis syndrome prognosis

There is insufficient data documenting the prognosis of Fitz-Hugh-Curtis syndrome as it usually responds to antibiotics very well. In one trial of triple therapy (penicillin-gentamicin-metronidazole) versus augmentin for non-chlamydial salpingitis, only one patient in each treatment group had treatment failure ¹⁶.

References

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