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eMedicine - Fitz-Hugh-Curtis Syndrome : Article by

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Author: Michael M Frumovitz, MD, Fellow, Department of Gynecologic Oncology, The University of Texas M.D. Anderson Cancer Center

Michael M Frumovitz is a member of the following medical societies: American College of Obstetricians and Gynecologists

Coauthor(s): Charles J Ascher-Walsh, MD, Clinical Assistant Professor, Department of Obstetrics and Gynecology, New York-Presbyterian Medical Center, Columbia University

Editors: Gerard S Letterie, DO, Associate Clinical Professor, Medical Director of In-vitro Fertilization Lab, Department of Obstetrics and Gynecology, Virginia Mason Medical Center, University of Washington; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; A David Barnes, MD, PhD, MPH, FACOG, Consulting Staff, Department of Obstetrics and Gynecology, Mammoth Hospital, Mammoth Lakes, California, Pioneer Valley Hospital, Salt Lake City, Utah, Warren General Hospital, Warren, Pennsylvania and Mountain West Hospital, Tooele, Utah; Frederick B Gaupp, MD, Consulting Staff, Department of Family Practice, Assumption Community Hospital; Lee P Shulman, MD, Professor of Obstetrics and Gynecology, Feinberg School of Medicine, Northwestern University; Chief, Division of Reproductive Genetics, Department of Obstetrics and Gynecology, Prentice Women's Hospital, Northwestern Memorial Hospital

Author and Editor Disclosure

Synonyms and related keywords: Fitz-Hugh-Curtis syndrome, FHC syndrome, Fitz-Hugh and Curtis syndrome, infectious perihepatitis, liver infection, pelvic infection, pelvic inflammation, diaphragm inflammation, acute salpingitis, pelvic inflammatory disease, PID, gonorrhea, chlamydia, Neisseria gonorrhoeae, N gonorrhoeae, Chlamydia trachomatis, C trachomatis

Background

Originally described in 1920, Fitz-Hugh-Curtis (FHC) syndrome (formally known as Fitz-Hugh and Curtis syndrome) consists of right upper quadrant pain resulting from ascending pelvic infection and inflammation of the liver capsule or diaphragm. Although it is typically associated with acute salpingitis, it can exist without signs of acute pelvic inflammatory disease (PID). In that respect, FHC syndrome can mimic other abdominal emergencies and is often a diagnosis of exclusion.

Pathophysiology

FHC is an extrapelvic manifestation of PID. It is associated with right upper quadrant pain that likely results from inflammation of the liver capsule and diaphragm. Previously, Neisseria gonorrhoeae was thought to be the main causative agent. However, recent studies have shown that cases of FHC due to Chlamydia trachomatis infection outnumber those due to N gonorrhoeae infection by almost 5 to 1. The spread of bacteria from the pelvis to the liver capsule likely results from the circulation of abdominal fluid over the right paracolic gutter to the subphrenic space and hepatic surface. However, lymphatic and hematogenous spread have not been excluded, and these probably play a role in the dissemination of the disease.

Frequency

International

FHC syndrome occurs in 15-30% of women with PID.

Sex

  • It is a disease that overwhelmingly affects females, although a few cases have been reported in males.

Age

  • FHC occurs in reproductive-aged women.



History

Fitz-Hugh-Curtis (FHC) syndrome consists of 2 phases, termed acute and chronic.

  • Acute phase
    • Acute onset of excruciating sharp pain over the area of the gallbladder
    • Possible referred pain to the right shoulder
    • Pleuritic pain that increases with Valsalva (ie, any maneuver that increases intra-abdominal pressure, eg, cough, sneeze) or movement
    • Occasional nausea, vomiting, hiccups, chills, fever, night sweats, headaches, or general malaise
    • Most often associated with acute salpingitis but symptoms of FHC without signs of PID are possible
  • Chronic phase - Characterized by persistent right upper quadrant pain or relief of symptoms altogether

Physical

Without a diagnosis of PID, FHC is most often a diagnosis of exclusion.

  • Typically, no pathognomonic signs are present upon physical examination.
  • The diagnosis is inferred from symptoms and positive culture findings for gonorrheal or chlamydial organisms.
  • Listening at the anterior costal margin may reveal a finding described as a "walking-in-new-snow" type of crunching friction rub.

Causes

FHC is caused by infection with C trachomatis or N gonorrhoeae.



Abdominal Trauma, Blunt
Adrenal Carcinoma
Appendicitis
Cholecystitis
Cholelithiasis
Hepatitis, Viral
Nephrolithiasis
Pancreatitis, Acute
Pancreatitis, Chronic
Peptic Ulcer Disease
Pneumonia, Bacterial
Pneumonia, Fungal
Pneumonia, Viral
Pulmonary Embolism

Other Problems to be Considered

Ectopic pregnancy
Pyelitis
Pyelonephritis
Pylephlebitis
Peritonitis
Subphrenic abscess



Lab Studies

  • Lab test findings are consistent with those of acute PID.
    • Cervical cultures for gonorrhea and chlamydia
    • Elevated WBC count and erythrocyte sedimentation rate
  • Because FHC rarely affects liver parenchyma, LFT results are rarely affected.
  • Rule out other disease.
    • Amylase or lipase to help exclude gallbladder disease
    • LFTs to help exclude hepatitis
    • Urinalysis or urine culture to help exclude pyelonephritis or kidney stones
    • Stool guaiac to help exclude perforated ulcer

Imaging Studies

  • Ultrasonography
    • Case reports exist that indicate visualizing perihepatic adhesions may be possible, especially when fluid is present in the abdominal cavity.
    • One study found an increase in the width of anterior extrarenal tissue due to inflammation.
    • Ultrasonographic findings help exclude the presence of gallstones.
  • CT scan
    • CT scan findings may help delineate a loculated perihepatic peritoneal collection.
    • Findings help exclude the presence of other diseases.
  • Chest radiograph
    • The right hemidiaphragm may be elevated.
    • Findings help exclude the presence of pneumonia.
    • Check for free air to help rule out perforation.

Procedures

  • Diagnostic laparoscopy
    • This is the criterion standard procedure for diagnosis.
    • Most diagnoses are made with after direct visualization of the liver capsule.
    • During the acute phase, inflammation of the peritoneum and anterior liver capsule is present and exudate that is gray and flaky or granular appears. The exudate has been described as looking like salt sprinkled on a moist surface.
    • During the chronic phase, the classic "violin-string" adhesions of the anterior liver capsule to the anterior abdominal wall or diaphragm are present (see Image 1).



Medical Care

  • Antibiotics are the mainstay of therapy for Fitz-Hugh-Curtis (FHC) syndrome.
  • Treatment is the same as for PID.
  • Patients may be treated in an outpatient setting unless they meet one of following criteria:
    • Positive for human immunodeficiency virus infection
    • Unilateral or bilateral tubo-ovarian abscess
    • Oral intake not possible due to secondary nausea or vomiting
    • Outpatient treatment has failed
    • Pregnant

Surgical Care

  • Laparoscopy is the criterion standard for diagnosis.
  • Relief of symptoms with lysis of adhesions is of questionable benefit.

Consultations

  • Consultation with a gynecologist may be indicated, especially if considering admission.

Activity

  • Sexual activity should be restricted until the patient's partner is treated.



Antibiotics and pain control agents are the medications of choice to treat Fitz-Hugh-Curtis (FHC) syndrome. Use the same antibiotics as would be used to treat PID. See Treatment for criteria regarding outpatient treatment regimens versus inpatient treatment regimens. Pain control may be achieved with NSAIDs or acetaminophen with codeine (Tylenol #3).

Drug Category: Outpatient antibiotic regimen #1

Includes antibiotics to treat the common causes of PID. This regimen includes both ceftriaxone and doxycycline.

Drug NameCeftriaxone (Rocephin)
DescriptionSingle-dose therapy. Third-generation cephalosporin with broad-spectrum, gram-negative activity; lower efficacy against gram-positive organisms; higher efficacy against resistant organisms. Arrests bacterial growth by binding to one or more penicillin-binding proteins.
Adult Dose250 mg IV/IM once
Pediatric Dose250 mg IV/IM once
ContraindicationsDocumented hypersensitivity
InteractionsProbenecid may increase levels; coadministration with ethacrynic acid, furosemide, and aminoglycosides may increase nephrotoxicity
PregnancyB - Usually safe but benefits must outweigh the risks.
PrecautionsAdjust dose in renal impairment; caution in breastfeeding and penicillin allergy

Drug NameDoxycycline (Doryx, Vibramycin)
DescriptionBroad-spectrum antibiotic. Inhibits protein synthesis and thus bacterial growth by binding to 30S and possibly 50S ribosomal subunits of susceptible bacteria.
Adult Dose100 mg PO bid for 14 d
Pediatric Dose<100 lb: 2 mg/lb PO divided bid for 14 d
>100 lb: Administer as in adults
ContraindicationsDocumented hypersensitivity; severe hepatic dysfunction
InteractionsBioavailability decreases with antacids containing aluminum, calcium, magnesium, iron, or bismuth subsalicylate; tetracyclines can increase hypoprothrombinemic effects of anticoagulants; tetracyclines can decrease effects of oral contraceptives, causing breakthrough bleeding and increased risk of pregnancy
PregnancyD - Unsafe in pregnancy
PrecautionsPhotosensitivity may occur with prolonged exposure to sunlight or tanning equipment; reduce dose in renal impairment; consider drug serum level determinations in prolonged therapy; tetracycline use during tooth development (last half of pregnancy through 8 y) can cause permanent discoloration of teeth; Fanconilike syndrome may occur with outdated tetracyclines

Drug Category: Outpatient antibiotic regimen #2

Includes antibiotics used to treat the common causes of PID. Regimen includes both ofloxacin and metronidazole (Flagyl)

Drug NameOfloxacin (Floxin)
DescriptionBroad-spectrum antibiotic. Penetrates prostate well and is effective against N gonorrhoeae and C trachomatis. A derivative of pyridine carboxylic acid with broad-spectrum bactericidal effect.
Adult Dose400 mg PO bid for 14 d
Pediatric DoseNot established
ContraindicationsDocumented hypersensitivity
InteractionsAntacids, iron salts, and zinc salts may reduce serum levels; administer antacids 2-4 h before or after taking fluoroquinolones; cimetidine may interfere with metabolism of fluoroquinolones; ciprofloxacin reduces therapeutic effects of phenytoin; probenecid may increase ciprofloxacin serum concentrations; may increase toxicity of theophylline, caffeine, cyclosporine, and digoxin (monitor digoxin levels); may increase effects of anticoagulants (monitor PT)
PregnancyC - Safety for use during pregnancy has not been established.
PrecautionsIn prolonged therapy, perform periodic evaluations of organ system functions (eg, renal, hepatic, hematopoietic); adjust dose in renal function impairment; superinfections may occur with prolonged or repeated antibiotic therapy

Drug NameMetronidazole (Flagyl)
DescriptionIn prolonged therapy, perform periodic evaluations of organ system functions (eg, renal, hepatic, hematopoietic); adjust dose in renal function impairment; superinfections may occur with prolonged or repeated antibiotic therapy
Adult Dose500 mg PO bid for 14 d
Pediatric DoseNot established
ContraindicationsDocumented hypersensitivity
InteractionsMay increase toxicity of anticoagulants, lithium, and phenytoin; cimetidine may increase toxicity; disulfiramlike reaction may occur with orally ingested ethanol
PregnancyB - Usually safe but benefits must outweigh the risks.
PrecautionsAdjust dose in hepatic disease; monitor for seizures and development of peripheral neuropathy

Drug Category: Inpatient antibiotic regimen #1

Includes antibiotics used to treat the common causes of PID. This regimen includes both cefotetan and doxycycline.

Drug NameCefotetan (Cefotan)
DescriptionWide range of aerobic and anaerobic gram-positive and gram-negative organisms. No activity against chlamydial species
Adult Dose2 g IV q12h
Pediatric DoseNot established
ContraindicationsDocumented hypersensitivity
InteractionsConsumption of alcohol within 72 h may produce disulfiramlike reactions; may increase hypoprothrombinemic effects of anticoagulants; coadministration with potent diuretics (eg, loop diuretics) or aminoglycosides may increase nephrotoxicity
PregnancyB - Usually safe but benefits must outweigh the risks.
PrecautionsReduce dosage by half if CrCl <10-30 mL/min and by one quarter if <10 mL/min; bacterial or fungal overgrowth of nonsusceptible organisms may occur with prolonged or repeated therapy

Drug NameDoxycycline (Doryx, Vibramycin)
DescriptionBroad-spectrum antibiotic. Inhibits protein synthesis and thus bacterial growth by binding to 30S and possibly 50S ribosomal subunits of susceptible bacteria.
Adult Dose100 mg PO/IV q12h
Pediatric DoseNot established
ContraindicationsDocumented hypersensitivity; severe hepatic dysfunction
InteractionsBioavailability decreases with antacids containing aluminum, calcium, magnesium, iron, or bismuth subsalicylate; tetracyclines can increase hypoprothrombinemic effects of anticoagulants; tetracyclines can decrease effects of oral contraceptives, causing breakthrough bleeding and increased risk of pregnancy
PregnancyD - Unsafe in pregnancy
PrecautionsPhotosensitivity may occur with prolonged exposure to sunlight or tanning equipment; reduce dose in renal impairment; consider drug serum level determinations in prolonged therapy; tetracycline use during tooth development (last half of pregnancy through 8 y) can cause permanent discoloration of teeth; Fanconilike syndrome may occur with outdated tetracyclines

Drug Category: Inpatient antibiotic regimen #2

Includes antibiotics to treat the common causes of PID. This regimen includes both clindamycin and gentamicin.

Drug NameClindamycin (Cleocin)
DescriptionBroad-spectrum antibiotic. Lincosamide for treatment of serious skin and soft-tissue staphylococcal infections. Also effective against aerobic and anaerobic streptococci (except enterococci). Inhibits bacterial growth, possibly by blocking dissociation of peptidyl t-RNA from ribosomes, causing RNA-dependent protein synthesis to arrest.
Adult Dose900 mg IV q8h
Pediatric Dose20-40 mg/kg/d IV divided tid/qid
ContraindicationsDocumented hypersensitivity; regional enteritis, ulcerative colitis, hepatic impairment, antibiotic-associated colitis
InteractionsIncreases duration of neuromuscular blockade induced by tubocurarine and pancuronium; erythromycin may antagonize effects; antidiarrheals may delay absorption
PregnancyB - Usually safe but benefits must outweigh the risks.
PrecautionsAdjust dose in severe hepatic dysfunction; no adjustment necessary in renal insufficiency; associated with severe and possibly fatal colitis by allowing overgrowth of Clostridium difficile

Drug NameGentamicin (Gentacidin, Garamycin)
DescriptionGood aerobic coverage. Aminoglycoside antibiotic for gram-negative coverage. Used in combination with both an agent against gram-positive organisms and one that covers anaerobes. Not the DOC. Consider using if penicillins or other less toxic drugs are contraindicated, when clinically indicated, and in mixed infections caused by susceptible staphylococci and gram-negative organisms.
Adult Dose2 mg/kg loading dose IV, then 1.5 mg/kg q8h
Pediatric Dose6-7.5 mg/kg/d (2-2.5 mg/kg q8h) IV
ContraindicationsDocumented hypersensitivity; renal insufficiency not on dialysis
InteractionsCoadministration with other aminoglycosides, cephalosporins, penicillins, and amphotericin B may increase nephrotoxicity; aminoglycosides enhance effects of neuromuscular-blocking agents, thus, prolonged respiratory depression may occur; coadministration with loop diuretics may increase auditory toxicity of aminoglycosides; possible irreversible hearing loss of varying degrees may occur (monitor regularly)
PregnancyD - Unsafe in pregnancy
PrecautionsNarrow therapeutic index (not intended for long-term therapy); caution in renal failure (not on dialysis), myasthenia gravis, hypocalcemia, and conditions that depress neuromuscular transmission; adjust dose in renal impairment

Drug Category: Analgesics

Pain control is essential to quality patient care. Analgesics ensure patient comfort and have sedating properties, which are beneficial for patients who experience pain.

Drug NameAcetaminophen and codeine (Tylenol #3)
DescriptionIndicated for the treatment of mild to moderate pain.
Adult Dose30-60 mg/dose based on codeine content PO q4-6h or 1-2 tab q4h; not to exceed 4 g/d of acetaminophen
Pediatric Dose0.5-1 mg/kg/dose based on codeine content PO q4-6h; 10-15 mg/kg/dose based on acetaminophen content; not to exceed 2.6 g/d of acetaminophen
ContraindicationsDocumented hypersensitivity
InteractionsToxicity increases with CNS depressants or tricyclic antidepressants
PregnancyC - Safety for use during pregnancy has not been established.
PrecautionsCaution in patients dependent on opiates because this substitution may result in acute opiate-withdrawal symptoms; caution in severe renal or hepatic dysfunction



Further Inpatient Care

  • Continue inpatient antibiotics until the patient is able to tolerate oral intake, is afebrile, and has improvement in abdominal pain.
  • Switch to an oral regimen to complete the 14-day antibiotic course.

Further Outpatient Care

  • Perform a follow-up examination in 48-72 hours to document improvement of symptoms.
  • Treat the patient's sexual partner for gonorrhea and chlamydia.

Deterrence/Prevention

  • Use condoms.
  • Limit the number of sexual partners.

Prognosis

  • Prognosis is excellent.
  • Most cases are asymptomatic (ie, difficult to diagnose clinically) and are diagnosed only at the time of surgery, when Fitz-Hugh-Curtis syndrome is in the chronic stage.



Media file 1:  "Violin-string" adhesions of chronic Fitz-Hugh-Curtis syndrome.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  Photo



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Fitz-Hugh-Curtis Syndrome excerpt

Article Last Updated: Aug 28, 2006