Fitz-Hugh-Curtis Syndrome (FHCS) is a rare complication of Pelvic Inflammatory Disease (PID), an infection of the female reproductive system;  pathogens infiltrate the connective tissue surrounding the liver, resulting in inflammation and formation of scar tissue that can lead to adhesion of the liver capsule to the diaphragm or abdominal wall. FHCS typically occurs in women of childbearing age, with rare cases reported in males.^1,4 It presents with pain in the upper right abdomen, which is worsened by breathing, coughing and laughing. In addition, it can also cause fever, pelvic pain, vaginal discharge and pain upon urination or sexual intercourse.¹

Introduction

Fitz-Hugh-Curtis Syndrome (FHCS) is a medical condition characterised by inflammation of the liver capsule, resulting from Pelvic Inflammatory Disease (PID). PID is an infection of the female reproductive system, particularly the uterus, ovaries and fallopian tubes. It is believed that pathogens can spread through the blood, lymphatic system and fallopian tubes to infiltrate the Glisson capsule surrounding the liver, which results in inflammation of the connective tissue with potential adhesion to the abdominal wall or diaphragm, and characteristic upper abdominal pain. Other symptoms include fever, pelvic pain, vaginal discharge and dysuria (painful urination).¹ FHCS occurs in 5-15% of people with PID and is most frequently associated with PID due to sexually transmitted bacteria, particularly Neisseria Gonorrhea and Chlamydia trachomatis.⁵  

Diagnosis of Fitz-Hugh-Curtis Syndrome involves history taking and clinical examination, often revealing pain and tenderness upon palpation of the upper right abdomen. Abdominal ultrasound, computed tomography (CT) scanning and laparoscopic examination of the abdomen can provide a definitive diagnosis, typically showing thickening of the hepatic capsule and violin-string-like connective tissue adhesions around the liver. 

The main treatment for FHCS is antibiotic therapy, usually Ceftriaxone and Azitromycin, targeting the most common pathogens involved. In severe cases, laparoscopic lysis of connective tissue lesions may be necessary. Most patients recover with appropriate treatment. However, without timely diagnosis and therapy, complications such as endometriosis, salpingitis, small bowel obstruction, infertility and chronic abdominal pain can develop.⁶ 

What is FHCS

FHCS is a complication of PID, in which pathogens can spread to the abdomen, resulting in inflammation, adhesions and scar formation in the connective tissue surrounding the liver. FHCS is relatively uncommon, affecting 750.000 people every year in the United States; patients are almost exclusively females. Historically, it has been associated with gonorrheal infections; however, research now indicates Chlamydia trichomatis is the most common causative agent. Bacteria can spread directly from the fallopian tubes or through the bloodstream or lymphatic system.¹

Symptoms of FHCS

The hallmark feature of FHCS is severe, sharp pain in the right upper abdomen, which can radiate to the right shoulder or back. Pain may worsen with deep breathing, coughing, laughing or movement. Clinical examination typically reveals pain and tenderness upon palpation of the right upper abdomen. Other symptoms that may be associated with FHCS include fevers, chills, nausea/vomiting, vaginal discharge, dyspareunia, dysuria, cramping, and postcoital bleeding.¹ 

Diagnosis of FHCS

Clinical history in FHCS typically reveals right upper abdominal pain with preceding symptoms of PID, such as lower abdominal or pelvic pain, vaginal discharge, dyspareunia, and/or abnormal vaginal bleeding. Risk factors for sexually transmitted diseases, such as multiple sexual partners, unprotected intercourse, previous history of PID and use of an intrauterine device, can also be present. (6) Upon palpation, the right abdomen is often tender and painful.¹ 

Laboratory blood tests can be useful in the diagnosis of FHCS, as they often show elevated inflammatory markers such as C-reactive protein and white blood cell count. In addition, normal or very slightly elevated levels of liver enzymes can help differentiate it from other conditions such as cholecystitis and hepatitis. Due to clinical similarities, particularly pain aggravation with breathing and right shoulder radiation, pneumonia and pleurisy (infection of the lungs and pleura, respectively) can be ruled out through chest X-ray. 

Definitive diagnosis of FHCS is through imaging of the abdomen, particularly abdominal ultrasound or CT scan, or laparoscopic examination. These tests typically show thickening of the connective tissue forming the liver capsule, with the presence of violin-string-like adhesions to the abdominal wall or diaphragm. Laparoscopy with biopsy involves removing a piece of tissue from the liver capsule, which can be put through PCR testing to reveal the causative pathogen and provide insight into the appropriate antimicrobial treatment. In some cases, several different bacteria can be involved.⁷ 

Prevention and treatment of FHCS

The most important aspect of treating FHCS is antibiotic therapy. Azitromycin and Cefetriaxone are effective against chlamydial and gonorrheal infections. Depending on the microbes identified in the tissue culture and clinical judgement, Doxycycline and Metronidazole can also be used.  Severe pelvic or abdominal adhesions or the presence of abscesses can warrant surgical lysis of the lesions through laparotomy.¹ 

In addition, minimising risk factors can help prevent future instances of PID. Doctors may recommend the use of protection during intercourse with non-regular partners, as well as regular sexually transmitted Infections (STIs) following high-risk sexual encounters. 

Prognosis and complications of FHCS

The prognosis of FHCS is usually good and, in most patients, symptoms resolve within 72 hours of outpatient antibiotic treatment. Hospitalisation is, however, recommended in high-risk cases, such as patients with severe illness, pelvic abscesses, inability to tolerate oral antibiotics or certain comorbid conditions, including pregnancy and immunodeficiency. 

On the other hand, if left untreated for long periods, FHCS can cause serious complications, such as abdominal/pelvic abscess, infertility, small bowel obstruction due to adhesions, chronic pelvic pain and ectopic pregnancy.¹ 

Summary

Fitz-Hugh-Curtis Syndrome (FHCS) is a rare complication of Pelvic Inflammatory Disease, affecting 750.000 people every year in the United States, the vast majority of whom are women of childbearing age. It results from the spread of bacteria, most commonly Chlamydia trachomatis or Neisseria Gonorrhea, from the reproductive system to the connective tissue capsule surrounding the liver, causing inflammation and formation of adhesions between the hepatic capsule and the abdominal wall or diaphragm. Patients typically present with upper right abdominal pain and tenderness upon palpation. Pain is usually aggravated by  breathing and movement and may be associated with fever, vaginal discharge, pain upon urination or sexual intercourse and nausea/vomiting.¹

FHCS is a serious condition which leads to debilitating pain and potential complications, including infertility and small bowel obstruction. Therefore, prompt and appropriate diagnosis and treatment are fundamental. This involves detailed clinical history and examination, laboratory tests and, most importantly, abdominal imaging or laparoscopy, which can reveal a thickened hepatic capsule with violin-string-like adhesions. A tissue biopsy is often performed to identify the causative agent or agents and help health professionals choose the appropriate antibiotic treatment.⁷ The disease typically resolves within 72 hours of starting an outpatient antibiotic regimen; however, severe cases may require hospitalisation and/or surgical lysis of adhesions. In addition, prevention of future sexually transmitted Infections (STIs) may be recommended.¹ Overall, correctly identifying, diagnosing, treating and preventing FHCS can improve patient outcomes and prevent potential debilitating complications.