Introduction

What is Fitz-Hugh-Curtis syndrome?

Fitz-Hugh-Curtis syndrome (FHCS), also known as perihepatitis, is a rare chronic manifestation and complication of pelvic inflammatory disease.¹ It occurs mostly in females, typically 15-30 years old, and is often caused by a sexually transmitted infection (STIs). It is said that there are 750,000 cases annually seen in the United States.⁴ It causes adhesions of the liver and results in pain in the right upper quadrant.

It is thought that microorganisms that are associated with pelvic inflammatory disease (PID) spread through one of three ways:¹

1. Spontaneous ascending of the infection, where microbes from the cervix travel upwards into the peritoneal cavity towards the liver to cause FHCS
2. Spread via the lymphatic system during infection
3. Spread via blood, such as during tuberculosis

Causes and key risk factors

As mentioned before, FHCS primarily is associated with STIs, like Chlamydia and Gonorrhoea.² STIs can lead to a cervical infection, which then spreads bacteria into the upper reproductive tract, which leads to pelvic inflammatory disease (PID).¹ Environmental factors like the lack of healthcare access and poor hygiene can also increase the risk of developing STIs and PIDs.

 Lifestyle choices such as smoking, excessive alcohol consumption, and unprotected sexual intercourse increase the risk for STIs.¹ This means that there is an increased likelihood of developing FHCS. Risk factors such as the following must be considered due to their association with FHCS:

1. Age: reproductive females between the ages of 15 and 30 are most at risk
2. Gender: Primarily females due to association with PID
3. Geographical locations: associated with poor or limited healthcare access
4. Underlying conditions: Women with a history of STIs, PID, or other healthcare issues have an increased risk

Symptoms and signs of FHCS 

As mentioned before, FHCS commonly affects women between 15 and 30, or of childbearing age.¹ They present with either acute or chronic pain and tenderness in the right upper quadrant of the abdomen, where the liver is. The reason why there is localised tenderness to the liver is due to the perihepatic inflammation and the formation of adhesions between the anterior surface of the liver and the abdominal wall. Therefore, other symptoms like worsening pain with movement, coughing, or deep breathing are also seen. This pain might also radiate in some individuals to the upper right shoulder, which is known as referred pain. This is caused by the irritation of the diaphragm with movement, which triggers the phrenic nerve supplying the diaphragm. Therefore, the impulses are transferred upwards where the phrenic nerve passes the right shoulder, causing the referred pain.³

Other symptoms related to pelvic inflammatory disease, such as lower abdominal or pelvic pain, abnormal vaginal discharge, dyspareunia (pain during sex), post-coital or intermenstrual bleeding, low-grade fever, or even dysuria, may present themselves as well.¹ Moreover, physical examination of patients will often reveal other signs such as:

1. Fever ≥ 38.3º C
2. Right upper quadrant pain of the abdomen with rebound tenderness
3. Cervical motion tenderness upon pelvic examination¹

Diagnosing Fitz-Hugh-Curtis syndrome?

Whilst the diagnosis of FHCS is primarily based on clinical expertise and judgement, the symptoms that FHCS presents in people are not specific to FHCS. This means that symptoms like right upper quadrant pain, shoulder irritation, pelvic pain, and tenderness can present with other conditions such as acute cholecystitis, choledocholithiasis, hepatitis, liver abscess, and ectopic pregnancies.⁴

To rule out FHCS, doctors often test other potential causes of pains by:

1. Pregnancy tests: To rule out ectopic pregnancies
2. Blood test: To ensure biliary blockage, inflammation, and hepatic damage aren’t present
3. Sexual screenings: To check for STIs and other potential conditions

Other physical tests, such as a CT scan, may be used to diagnose FHCS. Ultrasounds are also used to diagnose FHCS and rule out certain conditions, such as:

1. Cholecystitis
2. Biliary colic
3. Liver abscess. 

A pelvic ultrasound can show signs of pelvic inflammatory diseases, but this might not present in all individuals.² A CT scan might show the structures inside the body, which might show an increased blood flow in the liver capsule due to inflammation. Both of these imaging techniques are helpful when diagnosing for FHCS, as they are often able to identify ‘violin-string adhesions’, which are a sign of FHCS.⁴

Figure1. Laparoscopic imaging showing ‘violin-string adhesions’ between the anterior surface of the liver and the abdominal wall. 

How is Fitz-Hugh-Curtis syndrome treated?

It should be mentioned that FHCS is treatable. The treatment is usually administered alongside pelvic inflammatory disease.⁴ Therefore, antibiotics are a crucial step in management.

Medical management

Antibiotics are the main treatment methods for FHCS infections, and have been proven to be 75% effective.¹ The goal of management and treatment is to reduce pain and the likelihood of long-term complications, like ectopic pregnancies or infertility.²

Medical management options for FHCS:

1. Antibiotics: a course of antibiotic options for two weeks, depending on the individual’s medical history. Most commonly, ceftriaxone or azithromycin are the primary choices. The goal is to treat sexually transmitted illnesses like gonorrhoea and chlamydia, which cause PID²
2. Pain management: to relieve pain and inflammation
3. Surgical options: In severe cases, surgical interventions are necessary for the removal of infected tissue. This is done by surgical laparoscopy^4,1

Additional management and prevention management

Non-pharmacological treatments are also crucial for FHCS. These include:

1. Lifestyle modifications: this encourages safe sexual practice by using protective measures like condoms or alternative contraceptives
2. Dietary changes: this can be helpful in supporting an individual’s immune system, which can fight off infections and maintain overall health
3. Vaccinations: vaccines for hepatitis, PHV, and other conditions can help prevent and decrease the risk of infections that might lead to PID
4. Good Hygiene: This will decrease the likelihood of catching sexually transmitted illnesses and, therefore, decrease the risk for PID
5. Regular health check-ups: all sexually active individuals are recommended to have regular screening for sexually transmitted illnesses. This will ensure that infections are picked up early

Summary

Fitz-Hugh-Curtis syndrome is a pelvic inflammatory disease that typically affects individuals who are sexually active between 15 and 30 years of age, and has a higher risk in those with multiple sexual partners. The liver in FHCS forms adhesions known as ‘violin string adhesions’ between the anterior surface of the liver and the peritoneal cavity of the abdominal wall. Common symptoms include liver tenderness, upper right quadrant pain, and shoulder tip pain. Other possible symptoms that can persist include cervical motion tenderness, dyspareunia, and vaginal discharge. In addition, individuals with FHCS may continue to experience systemic symptoms such as fever, chills, headaches, nausea, and vomiting.

The diagnosis of FHCS is typically clinical. Imaging methods like CT scans can aid in early detection and the exclusion of other alternatives, like hepatitis, cholecystitis, etc. Fitz-Hugh-Curtis syndrome is treatable with antibiotics, pain management, and patient education, i.e, sexual health resources, vaccinations.