Fitz-Hugh-Curtis Syndrome

It is also known as Curtis-Fitz-Hugh Syndrome, FMC syndrome and the Fitz-Hugh and Curtis syndrome. The last is sensible as, like the Paterson-Brown-Kelly syndrome, it is named after two people, not three.

The Fitz-Hugh-Curtis syndrome originates as pelvic inflammatory disease but results in upper quadrant pain due to inflammation of the capsule of the liver and the diaphragm. During the chronic phase, adhesions form between the anterior liver capsule to the anterior abdominal wall or diaphragm and they are classically descried as like a "violin string".
Traditional teaching is that Neisseria gonorrhoeae is the main causative agent but Chlamydia trachomatis is found 5 times as often. This may represent a true change in epidemiology or it may be that culture techniques for chlamydia are now more readily available.¹
The spread of disease from the pelvis to the liver may be due to circulation of fluid along the paracolic gutter, it may be due to lymphatic drainage or the spread may be haematogenous.

This is said to affect between 4 and 14% of women who have pelvic inflammatory disease but the immature anatomy of adolescents is thought to make them more susceptible and a figure of 27% is given.² The epidemiology tends to mimic that of PID, affecting women of reproductive age and often younger women but the condition has been reported in the absence of PID and in men.

There is both an acute and chronic phase. In the acute phase the following features are often found:

• Acute onset of severe, sharp pain in the right upper quadrant and especially over the area of the gallbladder.³
• Pain may be referred to the right shoulder.
• Pain is pleuritic in nature and anything that increases intra-abdominal pressure, such as a cough, sneeze or movement, is associated with a sharp aggravation of the pain.
• There may possibly be
  • nausea
  • vomiting
  • hiccups
  • chills
  • fever
  • night sweats
  • headaches
  • general malaise
• There are often features of acute salpingitis but this is not invariable.

The chronic phase may show persistent, dull pain in the right upper quadrant or the pain may subside.

Examination

• There may be typical features of PID with lower abdominal tenderness, cervical excitation pain and tender adnexa
• Auscultation over the anterior costal margin may show a friction rub described as walking in new snow. This is similar to the sound of acute pericarditis.
• There may be no abnormalities on examination

The differential diagnosis includes that for pelvic pain and right upper quadrant pain. The presentation of this disease may mimic a number of others.⁴ The most important include:

• Ectopic pregnancy
• Pyelonephritis
• Cholecystitis
• Viral hepatitis
• Pulmonary embolism
• Renal colic
• Appendicitis

Often it is a diagnosis of exclusion.

• Swabs should be taken for gonorrhoea and chlamydia
• Other techniques to diagnose genitourinary chlamydia infection are described in the article on chlamydial genital infection.
• FBC may show an elevated white count and ESR may be raised
• LFTs should be normal as the parenchyma of the liver is not involved
• Microscopy and culture of urine
• Abdominal ultrasound to exclude renal or biliary stones. Diagnosis by ultrasound showing the "violin string" and ascites has been reported.⁵ Enhanced CT may also be of value.⁶
• CXR may be helpful to exclude pneumonia, pulmonary embolism and air under the diaphragm

Laparoscopy is often required for final diagnosis. Abnormality of the fallopian tubes may be seen with possible adhesions. During the acute phase, inflammation of the peritoneum and anterior liver capsule is seen and there may be an exudate that is grey and flaky or granular in appearance. The exudate has been described as like salt sprinkled on a moist surface.
In the chronic phase, the classical "violin-string" adhesions of the anterior liver capsule to the anterior abdominal wall or diaphragm may be seen.

• The main component of treatment is appropriate antibiotics of appropriate duration to treat the PID. This may well be dependent on the results of culture but the matter is discussed more fully in the article about PID.
• Analgesia may be required.
• It may be possible to divide some adhesions at laparoscopy.

There may be complications of PID such as tubo-ovarian abscess. Future fertility may be impaired or there may be a predisposition to ectopic pregnancy.

Prognosis is generally as for PID. There may be no symptoms of Fitz-Hugh-Curtis disease and it is found incidentally at operation at a later date. It may also be found as an incidental finding when investigating infertility and, as such, it may also indicate tubal damage.⁷

Prevention is as for PID.

Thomas Fitz-Hugh Jr was born in Maryland in 1894 but grew up in Charlottesville, Virginia, where his father was Professor of Latin. For the duration of the First World War, he served at the Base Hospital at St. Denis, France. After the war he studied medicine at the University of Pennsylvania, where he became chief of the haematological section of the University Hospital in 1929. During the Second World War, now as assistant professor of clinical medicine, he served with the Pennsylvania University unit as Chief of Medical Service in Assam, India. Having spent the First World War as a private, he was promoted to the rank of colonel. He retired in 1955 and died in 1963.
Arthur H. Curtis was born in 1881 in Wisconcin and graduated in medicine in, Chicago, in 1905. He subsequently went overseas to undertake postgraduate work and served with the American forces overseas in World War I. He later became Professor of Obstetrics and Gynecology at the Northwestern Medical School, Chicago and died in 1955. The two eponymous doctors do not seem ever to have worked together and their papers were published independently.^{8 9 10}
Fitz-Hugh and Curtis were not the first to describe the condition. In 1920 it was described by Stajano from Uraguay in an article in Spanish.¹¹ Carlos Stajano was born in 1891 and died in 1976. He worked in Montevideo and became Professor of Surgery in 1925. If he had published in English, we might be calling it Stajano's syndrome.