Fitz-Hugh-Curtis Syndrome

Fitz-Hugh-Curtis Syndrome
Last Updated: October 16, 2002
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Synonyms and related keywords: 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


AUTHOR INFORMATION
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Author: Michael M Frumovitz, MD, Fellow, Department of Gynecologic Oncology, The University of Texas M.D. Anderson Cancer Center
Coauthor(s): Charles J Ascher-Walsh, MD, Clinical Assistant Professor, Department of Obstetrics and Gynecology, New York-Presbyterian Medical Center, Columbia University
Michael M Frumovitz, MD, is a member of the following medical societies: American College of Obstetricians and Gynecologists
Editor(s): Gerard S Letterie, MD, Medical Director of In-vitro Fertilization Lab, Associate Clinical Professor, Department of Obstetrics and Gynecology, Virginia Mason Medical Center, University of Washington; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, Pharmacy, eMedicine; A David Barnes, MD, PhD, Clinical Assistant Professor, Department of Obstetrics and Gynecology, Jackson Memorial Hospital, University of Miami; Frederick B Gaupp, MD, Consulting Staff, Department of Family Practice, Lake Hospital; and Lee P Shulman, MD, Professor of Obstetrics and Gynecology, Head, Section of Reproductive Genetics, Feinberg School of Medicine, Northwestern University; Distinguished Physician, Department of Obstetrics and Gynecology, Northwestern Memorial Hospital

INTRODUCTION
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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 typically is 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 often is 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:
Internationally: 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.

CLINICAL
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History: FHC 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 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 most often is 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.

DIFFERENTIALS
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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 pregnancyPyelitisPyelonephritisPylephlebitisPeritonitisSubphrenic abscess
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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
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WORKUP
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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 rarely are 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:
Ultrasound
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.
Ultrasound 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).

TREATMENT
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Medical Care:
Antibiotics are the mainstay of therapy.
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 tuboovarian 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.