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Fitz-Hugh-Curtis Syndrome

Valerie Lewis MD, MPH


Nadja Peter MD
Basics
Description
Fitz-Hugh-Curtis (FHC) is the syndrome (FHCS) of perihepatitis, an
inflammation of the liver capsule, associated with PID in the reproductiveaged female.
Characterized laparoscopically by violin-string adhesions between the liver
capsule and abdominal wall.

Characterized clinically by acute or subacute onset of RUQ pain at the time of


genital tract infection (may be asymptomatic) with Chlamydia trachomatis or
Neisseria Gonorrhea.

RUQ pain can be persistent in the chronic phase of the disorder.

Age-Related Factors
Rates of FHCS are higher among adolescent females than adult women with
PID.
The higher rates among adolescents may be related to their immature cervical
ectropion (presence of columnar cells on the ectocervix), making them
biologically more susceptible to cervicitis.
Epidemiology
The incidence of FHCS (perihepatitis in presence of PID) is higher among adolescent
girls (27%) when compared to adult women (413.8%).
Risk Factors
Those factors which place an individual at risk for acquiring a genital infection with
Chlamydia or Gonorrhea resulting in pelvic inflammation:
Unprotected intercourse
Early sexual debut

Multiple sexual partners

In adult women, recent IUD placement may be a risk factor in the


development of PID.

Pathophysiology
The pathophysiology of this disorder is poorly understood, but the following
mechanisms have been proposed (some with conflicting or controversial
evidence, and maybe with different mechanisms in operation for Chlamydia
vs. Gonorrhea):
o Direct bacterial spread from the fallopian tubes to the liver via the
pericolic gutters:

Although it is possible that pelvic fluid can be tracked to the


RUQ, the causative bacteria has seldom been isolated from the
liver surface.

Hematogenous or lymphatic spread:

Some evidence for these mechanisms for Gonorrhea but very


little for Chlamydia.

Exaggerated immune response to C. trachomatis is the most likely


explanation, given what is known to date.
o

Higher anti-Chlamydial IgG titers have been found in those with both
perihepatitis and salpingitis than with salpingitis alone.

Antigenic proteins from Chlamydia may cross-react with host tissue


proteins, resulting in an exaggerated inflammatory response.

Associated Conditions
PID
Salpingitis
Diagnosis
Signs and Symptoms
History
The acute phase is characterized by:
o Sharp, pleuritic pain located in the RUQ of the abdomen, or pain
referred to the ipsilateral shoulder
o

Symptoms are usually associated with acute salpingitis, but signs or


symptoms of PID may be absent, especially with Chlamydia.

The chronic phase is characterized by persistent RUQ pain.

Pericapsular adhesions can occur in the absence of RUQ pain or salpingitis,


which can make the diagnosis challenging.

Review of Systems
May be associated with nausea, vomiting, hiccups, fever, chills, malaise, or signs of
salpingitis (lower abdominal pain and abnormal vaginal discharge)
Physical Exam
No pathognomonic exam findings, making FHCS a diagnosis of exclusion
Examination will demonstrate RUQ tenderness, or rarely a friction rub is
heard over the same area.

In patients with concurrent salpingitis, cervical motion or adnexal tenderness


may be present.

Tests
Laboratory tests and imaging studies are often nonspecific.
Labs
Liver enzyme levels: Typically, normal, but may be elevated. (Transaminase
elevations are more likely in the setting of gonococcal rather than Chlamydial
perihepatitis.)
ESR and/or C-reactive protein may be elevated.

WBC count may be normal or elevated.

Isolation of either Chlamydia and/or Gonorrhea from the cervix using a


variety of testing techniques including culture, direct immunofluorescent
smears, enzyme immunoassays, DNA probes, or nucleic acid amplification
tests.

Chlamydia-specific serology tests

If high clinical suspicion for STDs exists, samples should also be obtained
from the rectum, urethra, and pharynx.

Obtain studies to rule out other gastrointestinal or renal causes of RUQ pain:
Amylase, lipase, stool guaiac, and urinalysis/urine culture.

Imaging
Imaging studies are useful in ruling out or eliminating other potential causes of
RUQ pain.
Chest and abdominal radiographs to evaluate for pneumonia or
subdiaphragmatic free air:
o

US to evaluate the liver, the gallbladder for cholelithiasis or cholecystitis, or


the ovaries for a tubo-ovarian abscess or other signs of PID:
o

A right-sided hemidiaphragm elevation or small reactive pleural


effusion may be seen in FHCS.

Rarely, ascites in the hepatorenal space, loculated fluid in the pelvis or


abdomen or adhesions between the liver and abdominal wall may be
seen in FHCS.

CT occasionally demonstrates liver capsule enhancement.

Differential Diagnosis
FHCS can mimic other conditions making the diagnosis difficult.
Cholelithiasis/cholecystitis

Pneumonia

Pulmonary embolism

Rib fracture/abdominal trauma

Pyelonephritis

Hepatitis/Peritonitis

Nephrolithiasis

Subphrenic abscess

Pancreatitis

Appendicitis

Herpes zoster

Enteroviral epidemic pleurodynia (Burnhold disease)/pleurisy

Infection
C. trachomatis is more likely to be the etiologic agent than N. gonorrhea, although
both have been implicated in FHCS.
Treatment
General Measures
Antibiotic therapy is indicated in the management of FHCS.
The same agents directed against Chlamydia and Gonorrhea are used to treat
PID and FHCS.

One should not await confirmatory lab results to treat if clinical suspicion is
high.

Pain control can be achieved with NSAIDs or narcotic agents.

Sexual activity should be avoided until 1 week after both the patient and
partner(s) have completed treatment.

P.109
Medication (Drugs)
Treatment regimen as per CDC Guidelines for treatment of PID
Oral regimens:

Ceftriaxone 250 mg IM in a single dose PLUS doxycycline** 100 mg


PO b.i.d. for 14 days WITH OR WITHOUT Metronidazole 500 mg PO
b.i.d. for 14 days

Alternative: OR cefoxitin 2 g IM in a single dose and probenecid 1 g


PO administered concurrently in a single dose, OR other parenteral
3rd-generation cephalosporin PLUS doxycycline 100 mg PO b.i.d. for
14 days WITH OR WITHOUT metronidazole 500 mg PO b.i.d. for 14
days

Parenteral regimens:
o

Cefotetan 2 g IV q12h OR cefoxitin 2 g IV q6h PLUS doxycycline**


100 mg PO or IV q12h

Clindamycin 900 mg IV q8h PLUS gentamicin loading dose IV or IM


(2 mg/kg of body weight), followed by a maintenance dose (1.5
mg/kg) q8h. Single daily dosing may be substituted.

Alternative: OR ampicillin/sulbactam 3 g IV q6h PLUS doxycycline**


100 mg PO or IV q12h

Fluoroquinolones should only be used in persons with intolerance to


other aforementioned antibiotics and with low individual risk and low
community prevalence of Gonorrhea. If testing for Gonorrhea is

positive, the antibiotic should be changed or susceptibility testing


done.
Alert
Pediatric Considerations
Fluoroquinolones should be used sparingly in those younger than 18 years secondary
to concerns about interference with bone development.
Pregnancy Considerations
Fluoroquinolones and doxycycline should be avoided during pregnancy.
Surgery
Laparoscopic exploration can be used to assist in making the diagnosis of PID
and FHCS.
Surgical intervention is indicated only in the event that symptoms do not
resolve after antibiotic therapy:
o

Laparoscopy can be used to lyse perihepatic adhesions in the case of


chronic RUQ pain following an episode of FHCS.

Followup
Disposition
Hospital admission criteria for FHCS are similar to those for PID and include:
o Inability to rule out a surgical emergency

Inability to tolerate an oral regimen

The presence of a complication of PID such as tubo-ovarian abscess

Pregnancy

Discharge when the patient's symptoms of salpingitis have resolved.

Issues for Referral


Consider gynecologic surgical referral for lysis of perihepatic adhesions in individuals
with chronic RUQ pain.
Prognosis
Excellent
Patient Monitoring
Monitor for resolution of RUQ pain, as well as signs and symptoms of
salpingitis.
An association may exist between FHCS and fallopian tube dysfunction that
results in infertility. Severity of the tubal abnormality is determined by the
host's reactivity to Chlamydia.
Bibliography
Centers for Disease Control and Prevention, Updated recommended treatment
regimens for gonococcal infections and associated conditions United States, April
2007. http//www.cdc.gov/std/treatment/2006/updated-regimens.htm
Kobayashi Y, et al. Pathological study of Fitz-Hugh-Curtis syndrome evaluated from
fallopian tube damage. J Obstet Gynaecol. 2006;32(3):280285.
Litt IF, et al. Perihepatitis associated with salpingitis in adolescents. JAMA.
1978;240:12531254.

Peter NG, et al. Fitz-Hugh-Curtis syndrome: A diagnosis to consider in women with


right upper quadrant pain. Cleve Clin J Med. 2004;71(3):233239.
Tsubuku M, et al. Fitz-Hugh-Curtis syndrome: Linear contrast enhancement of the
surface of the liver on CT. J Comput Assist Tomogr. 2002;26:456458.
Miscellaneous
Synonym(s)
Gonococcal perihepatitis
Perihepatitis syndrome
Clinical Pearls
C. trachomatis and N. gonorrhea are the main causative agents implicated in FHCS.
Among those with PID, FHCS occurs more commonly among adolescent females
(27%) than adult women (414%).
FHCS is usually a clinical diagnosis based on exclusion of other causes of RUQ pain
and isolation of the bacterial pathogen.
Treatment is with antibiotics directed against C. trachomatis and N. gonorrhea. Can
consider lysis of perihepatic adhesions if RUQ pain persists.
Abbreviations
FHCSFitz-Hugh Curtis syndrome
PIDPelvic inflammatory disease
RUQRight upper quadrant
STD/STISexually transmitted disease/infection
Codes
ICD9-CM
99.56 Perihepatitis
614.9 Pelvic inflammatory disease
Patient Teaching
Educate about safer sex practices including consistent condom use.
Encourage testing for other STIs, including HIV and syphilis.
Counsel about the importance of partner notification to prevent spread or
reinfection.
Frequent STI screening and testing among sexually active populations
Prevention
Reduction of high-risk sexual behaviors