Introduction

Fitz-Hugh-Curtis syndrome or perhepatitis is a rare disorder that occurs predominantly in women. It is a complication of pelvic inflammatory disease that causes the inflammation of the liver capsule, leading to adhesions. The disease was first described by Carlos Stajano in 1920 but was later also described by Thomas Fitz-Hugh, Jr and Arthur Hale Curtis in the 1930s. It is commonly caused by the organisms Chlamydia trachomatis and Neisseria gonorrhoea

How does Fitz-Hugh-Curtis syndrome develop?

Fitz-Hugh-Curtis Syndrome(FHCS) is associated with pelvic inflammatory disease(PID). PID is an ascending infection of the upper genital tract.¹ It is usually associated with sexually transmitted organisms, especially Neisseria gonorrhoeae and Chlamydia. trachomatis. Some microorganisms that live in the vagina such as Gardnarella vaginalis and Mycoplasma hominis have also been linked with PID, though these have not been known to cause FHCS. PID is a common problem in sexually active women of reproductive age. 

The disease was initially thought to be exclusively due to N. gonorrhoea but recent evidence shows that it is more commonly associated with C.trachomatis, than N.gonorrhoea.²

The exact mechanism by which the pelvic infection spreads to the liver is not known.^2,3 However, some mechanisms have been suggested, these include:

• Direct spread to the liver
• Spread through the bloodstream
• Spread through the lymphatic channels
• Exaggerated response of the immune system to Chlamydia. Higher quantities of antibodies against Chlamydia trachomatis have been found in patients with the disease compared to those without the disease⁴

The disease is characterised by the inflammation of the liver capsule and overlying lining(peritoneum). This does not involve the tissues of the liver, so the liver function is not affected. The capsular inflammation leads to the development of violin string-like fibrous tissue(adhesion bands) between the liver capsule and the abdominal wall or diaphragm(the muscle between the chest and abdomen).²

What is the affected population?

The disease usually affects women, though it has rarely been reported in men.^5,6

Up to 4-14% of people with PID have been reported to have the disease. 

Women younger than 25 years, who have multiple sex partners, do not use contraception, and who live in an area with a high prevalence of sexually transmitted diseases are at high risk of the disease.⁷

Diagnosis of Fitz-Hugh-Curtis syndrome

The disease can be difficult to diagnose as it mimics many other diseases.⁴

The diagnosis is suspected through history and examination but confirmation is on direct visualisation on laparoscopy or laparotomy.^2,3

History

Pain in the right upper abdomen, just below the ribs is the major symptom.2,3 The pain is usually worsened by breathing or movement. It may radiate to the right shoulder tip or the inner surface of the right arm.

Other symptoms include:

• Chills
• Nausea and vomiting
• Headache
• Hiccups
• A feeling of being unwell(malaise)

The upper abdominal pain may be preceded by symptoms of pelvic inflammatory disease(fever, abdominal pain, vaginal discharge). However, some patients may present with upper abdominal pain without prior pelvic symptoms.

Examination

The major examination finding is pain in touching the right upper abdomen.

Examination of the lower abdomen and pelvis would reveal pain on touching the lower abdomen, the uterus(womb) or the cervix(neck of the womb)

Investigations

Investigations would involve:

• Laboratory tests
• Imaging studies 
• Diagnostic operative procedure

Laboratory tests

• Full blood count; To check for evidence of raised white blood cells, which is indicative of infection
• Erythrocyte sedimentation rate: This is usually raised
• C-reactive protein; This is usually raised
• Swabs from the vagina and cervix for culture
• Urine tests for the detection of Chlamydia and Gonorrhoea

Radiological investigations 

They include:

• Pelvic ultrasound: This shows evidence of fluid-filled fallopian tubes or an abscess in the pelvis
• Abdominal ultrasound: This helps to exclude other diseases like cholecystitis(inflammation of the gallbladder) and cholelithiasis(stone in the gallbladder). These may mimic Fitz Hugh Curtis syndrome. Pockets of free fluid around the liver may suggest the presence of the disease
• Computed Tomography(CT) scan- May show enhancement around the liver capsule. It may also show signs of PID, like the presence of abscesses and fluid in the pelvic cavity
• Magnetic Resonance Imaging(MRI): This may show abscesses around the fallopian tubes and ovaries; it may also show swollen and fluid-filled tubes

Surgical procedures

The diagnosis of FHCS is confirmed on direct visualisation.

Laparoscopy is the gold standard for diagnosis. Laparoscopy is the visualisation of the internal abdominal organs through a small camera inserted through a small hole in the abdomen. 

Findings include:

• Evidence of a band of adhesions between the liver capsule and the diaphragm or abdominal wall
• The band adhesions have the look of violin strings
• Evidence of swelling and redness of the fallopian tubes
• Pus and fluid discharge from the fallopian tubes may also be observed

In addition, a sample taken from the lining of the womb will show signs of inflammation(endometritis)

Other diseases mimic FHCS and should be excluded by the time the investigations are concluded. 

These diseases include:

• Hepatitis
• Cholecystitis
• Cholelithiasis
• Pancreatitis 
• Pyelonephritis
• Appendicitis
• Ectopic pregnancy

Treatment of Fitz-Hugh-Curtis syndrome

Most patients with FHCS are managed as outpatients, but occasionally there may be a need for admission.

The indications for admission include

• No improvement after 72 hours of oral antibiotic therapy
• If the exact diagnosis is uncertain
• If there is suspicion of a surgical emergency like appendicitis
• If the symptoms are severe i,e, temperature is more than 38.5 degrees centigrade
• If there is an abscess 
• If there is an inability to tolerate anything by mouth
• In a patient with a deficiency of the immune system
• Pregnancy

Medications

The use of antibiotics is the first line of treatment for FHCS.

Treatment is the same as for pelvic inflammatory diseases. The Centres for Disease Control recommends that antibiotic treatment should be commenced once there is a suspicion of pelvic inflammatory disease.¹ This is to prevent long-term sequelae like infertility and ectopic pregnancy.

The recommended antibiotics are those effective against Chlamydia trachomatis and Neisseria gonorrhoea. The addition of antibiotics against anaerobic organisms like Bacteroides fragilis also reduces the risk of long-term sequelae. The commonly recommended regimen is Ceftriaxone plus doxycycline and metronidazole.

Supportive management

For patients who are acutely ill, supportive management would be needed. This involves the administration of:

• Intravenous fluids
• Analgesics: to manage the pain
• Antipyretics: to manage fever

Surgical management

This is indicated in patients with symptoms persisting after 72 hours.

Laparoscopy is the preferred mode of management. This helps to confirm the diagnosis and exclude other diseases.

Interventions at laparoscopy include:

• Drainage of abscess
• Adhesiolysis(breakdown of the band of adhesions)

Laparotomy(open surgical operation) is reserved for patients who are not good candidates for laparoscopy or those with emergencies like ruptured abscess.

Other management considerations

• Advice to women to avoid sexual intercourse while treatment is completed and symptoms have resolved
• Contact tracing and treatment of sexual partners within the past 60 days or the most recent sexual partner
• Screening for other sexually transmitted infections like HIV, hepatitis B and syphilis

Prevention of Fitz-Hugh-Curtis syndrome

Because of the long-term sequelae of PID and FHCS, preventive measures need to be instituted.⁷

These include:

• Educational programs on strategies to prevent PID i.e
  • Delaying the onset of sexual intercourse in adolescents
  • Avoiding multiple sexual partners
  • Using barrier contraception
  • Avoiding sexual activity/use of barrier contraception by those with symptoms of PID until symptoms resolve
  • Compliance with medications by those on treatment for PID
  • Notification of sexual partners by those diagnosed with PID

• Screening for chlamydial infection for women up to 25 years who are at high risk
• Early diagnosis and treatment of PID

FAQs

Can men have Fitz-Hugh-Curtis syndrome?

The disease is almost always seen in females, there have however been occasional occurrences in men. The mechanism of spread in men is thought to be due to the blood and lymphatic system instead of through direct spread. 

Can one get pregnant with Fitz-Hugh-Curtis Syndrome?

The disease affects the reproductive organs so one of the complications of the disease is infertility. However, if treated on time, this complication can be averted.

Are there other sexually transmitted infections that affect the liver?

Hepatitis B is a sexually transmitted infection that can cause infection and inflammation of the liver. 

Summary

Fitz-Hugh-Curtis Syndrome is a rare but significant complication of pelvic inflammatory disease. It presents a diagnostic challenge due to its similarity to other abdominal conditions, so a high index of suspicion is needed. Early diagnosis through a combination of history, examination, advanced imaging techniques and laparoscopy is essential for effective management. Antibiotic treatment and sometimes surgical management may be needed. Understanding and treatment of the disease is essential for preventing long-term complications such as infertility and ectopic pregnancy.