Overview

Fitz-Hugh-Curtis Syndrome (FHCS) is a rare condition characterised by chronic pain of the upper right stomach due to inflammation of the liver (perihepatitis) - specifically the capsule, which is the connective tissue layer surrounding the liver.¹ This severe pain is caused by string-like adhesions or scar tissue forming between the liver capsule, abdominal wall and/or the diaphragm. Those affected tend to only be sexually active women or people assigned female at birth (AFAB) between 15 and 30 years old, who have developed a pelvic inflammatory disease (PID), which is the umbrella term for upper genital tract infections.² PID can result from sexually transmitted infections (STIs) caused by bacteria such as gonorrhoea or chlamydia.³ When the reproductive tract becomes infected, there is a risk that the infection spreads to the liver and surrounding tissues causing FHCS causing pain and swelling.

How infection occurs in FHCS

How PID leads to the development of FHCS

There are 3 ways bacteria are thought to infect the liver capsule and cause FHCS:

1. The bacteria infecting the upper genital tract may travel from the vagina, through the cervix, to the lining of the womb, up the fallopian tubes (the tubes connecting your ovaries to your vagina) and into a small, fluid-filled cavity containing the stomach, intestines and the liver¹ 
2. The layer of fatty and fibrous connective tissue surrounding the uterus may become infected as a result of the coil or an intrauterine device (IUD).¹ Infection may reach the liver via the lymphatic system which is a network of tubes that keep body fluid levels balanced and carry immune cells around the body to fight infections
3. Bacteria may reach the liver and surrounding tissue via the bloodstream¹ 

Impact on the liver capsule 

Development of "violin string" adhesions and their significance

Adhesions describe the formation of scar tissue from one tissue to another across a cavity, in this case, the peritoneal cavity containing the liver, stomach and diaphragm. The gonorrhoea-causing bacteria (Neisseria gonorrhoeae), for example, infects the liver capsule and surrounding tissue causing the immune system to be activated, causing inflammation. As part of the body’s healing process, a fibrinous exudate is produced, which is a thick yellow fluid containing fibrin (a fibrous protein involved in blood clotting) and inflammatory cells. This fluid is deposited on the adjacent injured liver capsule and abdominal cavity surfaces. This results in a thickening of the liver capsule. The fibrin deposits also form numerous sticky violin string-like structures that can be inches wide, connecting the liver capsule with the abdominal wall or diaphragm. Inflammatory signals interfere with enzymes that would normally reduce or even break down these adhesions. Once the violin string adhesions are formed, tissue repair cells deposit collagen amongst other matrix substances into the adhesions to form permanent fibrous adhesions.

Functional implications

Violin string adhesions cause pulling on the liver capsule and the diaphragm causing discomfort, and sharp pain in the upper right quadrant of the abdomen which gets worse with breathing. Other implications of these adhesions include limiting the normal range of movement of the liver and the diaphragm affecting normal breathing activity. This may cause breathing to feel difficult, especially deep breaths, and the chest to feel tight but does not impair the primary function of the liver. In rare cases, gastrointestinal problems may occur due to restricted liver movement.⁴ 

Clinical presentation

Symptoms specific to liver capsule involvement

The adhesions and inflammation may result in chronic pain in the upper right abdomen, which can persist even when the initial infection has been resolved. This chronic pain can impact the patient's quality of life and may require long-term management. Pain radiating down the right shoulder and back may occur via a phenomenon called referred pain due to their shared nerve pathways with the liver capsule. Those with FHCS may also experience stiffness and a limited range of motion, particularly on the right side.

Other symptoms:²

• Lower stomach or pelvic pain 
• Fever
• Nausea and vomiting 
• Vaginal discharge 
• Painful sexual intercourse 
• Painful urination 
• Headaches, hiccups, overall feeling of poor health

Differential diagnosis: distinguishing FHCS from other hepatic and biliary conditions

Those with FHCS will produce normal liver function tests as only the liver capsule is affected and not the liver cells (parenchyma cells). A history of PID, an elevated white blood cell count and positive inflammatory markers would be present in someone with FHCS, as well as a positive cervical swab for chlamydia or gonorrhoea. There is liver tenderness without enlargement of the liver which occurs in other hepatic conditions and a lack of gastrointestinal problems is observed in FHCS. By considering clinical presentation, patient history, lab findings, imaging studies, and response to treatment - clinicians can differentiate FHCS from other hepatic or biliary conditions. 

Other complications

If left untreated, FHCS can cause infertility as PID can cause the formation of scar tissue in the fallopian tubes, blocking the tube, and preventing fertilisation of the sperm and egg.¹

Management and treatment

Treatment of FHCS involves prompt antibiotic therapy to eradicate the underlying infection, pain management, possible surgical intervention for adhesions, and preventive measures to avoid recurrence and protect sexual partners. Regular follow-up is important to ensure full recovery and to address any complications that may arise.

Antibiotic therapy

The treatment protocol for FHCS is antibiotic therapy, which is successful in three-quarters of cases.¹ Due to FHCS being caused most commonly by chlamydia or gonorrhoea, antibiotics for these specific bacteria are administered (Chlamydia: Doxycycline and Gonorrhea: Ceftriaxone). When the specific pathogen is not immediately identified, a combination of 2 antibiotics may be used. After antibiotics, tests for STIs are usually carried out to ensure the elimination of infection.

Pain management strategies and lifestyle modifications

Pain medications called analgesics such as acetaminophen and codeine may be used in some cases. Heat application to the affected area may also aid pain management.³

Surgical interventions

If antibiotics do not alleviate the right upper quadrant pain, if there is uncertainty that FHCS is the cause, or if symptoms are recurrent, a surgical procedure called a laparoscopy may be undertaken. During this surgery, if adhesions are present, they can be visualised and cut using a small instrument inserted into the abdominal cavity through a hole made in the abdomen. ³

Preventing recurrence 

Reducing FHCS risk:²

• Condom use 
• Regular STI testing 
• Avoiding douching 

Those experiencing FHCS may benefit from lifestyle modifications including resting, and avoiding activities worsening pain and may need additional emotional support, especially in the context of sexually transmitted infections requiring sensitivity. 

Summary

Fitz-Hugh-Curtis Syndrome is the chronic manifestation of pelvic inflammatory disease resulting in the liver capsule and its surrounding tissues becoming inflamed.¹ Around 4-14% of women with PID develop Fitz-Hugh-Curtis syndrome with a greater frequency occurring in adolescents with PID due to their increased susceptibility to infection. Whilst this is a very rare condition, some important risk factors should be observed to prevent the escalation of genital tract infections into FHCS.