Overview 

Fitz-Hugh-Curtis Syndrome (FHCS) - also known as perihepatitis - is the most extreme and uncommon chronic complication of pelvic inflammatory disease (PID).¹

PID is an inflammation of the upper genital tract (fallopian tubes, uterus, and ovaries) that spreads from the lower genital tract often as a result of exposure to a sexually transmitted infection. It’s estimated that 10-15% of PID cases are linked to bacterial STIs caused by the microbial agents Neisseria gonorrhoeae and Chlamydia trachomatis.² PID then develops into FHCS through spontaneous ascending infection with microbes travelling from the lower genital tract to the peritoneal activity through the upper genital tract. 

FHHCS is described by the inflammation of the Glisson’s capsule (a layer of interstitial tissue surrounding the liver) with adhesion formation between the liver capsule and abdominal wall which often results in acute pain in the upper quadrant of the abdomen that can radiate to the right shoulder and is worsened by deep breathing and body movements.¹

PID typically affects sexually active individuals assigned female at birth (AFAB) of childbearing age, more specifically those aged between 15 and 30.¹ FHCS is an uncommon manifestation resulting in complications in individuals with PID and incidence is said to vary depending on the diagnostic criteria used. It’s estimated that 2-5% of PID patients will develop FHCS; these figures are often higher in adolescents and individuals who have intrauterine devices.³ In individuals with intrauterine devices, an infection of the parametrium causes microbes to spread through lymphatic channels eventually leading to FHCS.¹ There have also been reported cases of hematogenous spread in individuals that have been infected by Mycobacterium tuberculosis.³

Importance of understanding severe cases

Clinical significance

Typically, FHCS patients visit doctors complaining of sharp acute pains or chronic tenderness in the right upper quadrant of the abdomen, occasionally the patient may also complain of pain in the right shoulder. This is a non-specific symptom that can mimic a myriad of other pathologies which makes assessing for FHCS challenging. Therefore, careful differential diagnosis is required, usually necessitating the use of imaging techniques and careful consideration of a patient’s history and high-risk behaviours.⁴ It is important to diagnose FHCS accurately and early in order to minimise risks of long-term complications such as infertility or ectopic pregnancies (pregnancies that occur outside the uterus).¹

Diagnostic evaluation

Risk factors

The attending physician who suspects a diagnosis of FHCS must focus on high-risk behaviour and confirm the symptoms via physical examination in an individual of the appropriate patient group. There are risk factors that can help narrow the differential further: ¹

• Aged between 15-30 and AFAB
• First sexual encounter happened before 15 years of age
• History of PID
• Use of an intrauterine device (IUD) or oral contraceptives
• Recent IUD insertions
• Vaginal douching
• Exposure to new, multiple or symptomatic sexual partners

Obtaining medical and surgical history could also be beneficial to the attending doctor in diagnosis.⁵

Symptoms of FHCS

Though abdominal pain in the right upper quadrant is the main symptom associated with FHCS as a result of adhesion formation, there are several other symptoms that the attending doctor should consider: ¹

• Lower abdominal, pelvic, or back pain (that may vary in degrees of severity)
• Fever (>38.3 C)
• Chills
• Nausea
• Vomiting
• Abnormal vaginal discharge
• Dyspareunia (pain during intercourse)
• Dysuria (pain during urination)
• Cramping
• Postcoital bleeding (bleeding after sex)

To further rule out other conditions that share symptoms with FHCS, the doctor may run radiological exams including:

• Transvaginal ultrasound - a scan carried out using a probe that is inserted into the vagina to indicate abnormalities in the lower genital tract; these are favoured when it is unclear if the patient has suffered/is suffering from PID¹
• Ultrasound - to check for the presence of peritoneal fluid around the spleen and liver⁶
• CT scan - to check for increased enhancement along the hepatic surface⁷
• Magnetic resonance imaging (MRI) - rarely used as its radiological features are poorly described in research⁸

Some laboratory tests may also be run including:

• Full panel blood tests
• Pregnancy test
• STI tests to check for evidence of microbiological pathogens

The gold standard for diagnosing both FHCS and PID is laparoscopy. In the case of FHCS, a direct diagnosis can be made using this technique as it allows for the visualisation of adhesions that have formed between the liver and anterior abdominal wall or the liver and diaphragm.¹

Indications for surgical intervention

Failure of conservative treatments

Usually, the treatment of FHCS coincides with the management of the underlying issue, PID. In the case of PID, the goal of treatment is to get rid of the infection, provide the patient with symptom relief and minimise risks of the development of long-term complications. In 75% of cases, treatment using antibiotics is successful and most PID patients can be treated outpatient. The antibiotics used are often those most successful at clearing the most common offending pathogens N. gonorrhoeae and C. trachomatis.¹ The most commonly used antibiotics are azithromycin and ceftriaxone. However, in cases of complicated PID, current recommendations for antibiotic therapies include ceftriaxone, doxycycline, and metronidazole.⁹ Antibiotic regimens must also be tailored to each patient based on the degree of suspicion as assessed by the evaluating doctor.¹⁰

If symptoms persist, past 72 hours of treatment, the patient should be evaluated for surgical intervention. It is recommended that laparoscopy is used at this stage as it is less invasive than exploratory laparotomy. This surgical intervention allows doctors to make an official diagnosis of FHCS as they can visualise the adhesions, understand the extent of liver capsule involvement, and scope out the severity of adhesions and the impact on adjacent abdominal structures.¹¹

Surgical techniques and approaches

Laparoscopy

Laparoscopy is a keyhole surgery that is used for the lysing (cutting) of adhesions formed in the abdominal cavity; this surgery is also referred to as laparoscopic adhesiolysis. The surgery involves making small incisions and inserting a camera (a laparoscope) into the patient’s stomach that has been inflated with air to visualise the adhesions that are then cut to alleviate the unfavourable symptoms that occur as a result of their formation. It is a minimally invasive surgery with a faster recovery time than other types of surgery.¹² This method also conserves reproductive potential as the surgeon can perform abscess drainage and unilateral adnexectomy where necessary. 

Laparoscopies are common surgeries, thus, complications are rare but some that may arise are:¹³

• Organ damage 
• Blood vessel damage
• Severe allergic reaction to general anaesthesia
• Development of blood clots
• Development of a hernia
• Requiring further surgical intervention (open surgery) 

Open surgery

Indications for open surgery over laparoscopy

Open surgeries (also known as conventional surgeries or laparotomies) differ from laparoscopies as they require larger incisions to be made to offer direct access to the site of surgery. This procedure affords the surgeon more liberty as it offers increased visualisation however it comes at the cost of a longer recovery time and increased tissue damage. This surgery is often preferred for difficult/complicated cases.¹⁴

There are complications associated with this type of surgery including:¹⁵ 

• Haemorrhage
• Infection 
• Hematoma
• Tissue necrosis
• Damage to underlying structures
• Incisional hernia
• Skin numbness
• Wound dehiscence
• Internal scar tissue formation

Emerging surgical interventions

The aforementioned surgical interventions are the most commonly used in the treatment of FHCS however, as the medical field continues to evolve and transform, there is a need for new methods/advancements in the surgical management of this condition. Robotic-assisted laparoscopies have been used in the management of a few cases of FHCS - this method offers more precision lessening the chances of organ or blood vessel damage during the procedure.¹⁶

Routine postoperative management similar to that of any other condition is required to minimise complications and optimise outcomes. In patients with other comorbidities or those who had severe cases of adhesion thus a complicated surgery, they require extra care and observation. Patients may be given painkillers such as paracetamol or ibuprofen to manage pain.¹²

Summary

Fitz-Hugh-Curtis syndrome is an uncommon complication of pelvic inflammatory disease. Therefore to understand how to treat and manage FHCS we must first understand what causes PID and how to treat and manage that first. PID is often caused by ascending infection from the lower genital tract into the upper genital tract; this condition is often caused by bacterial STIs caused by the bacteria Neisseria gonorrhoeae and Chlamydia trachomatis. FHCS has also been linked to Mycobacterium tuberculosis. Thus the risk population for FHCS is individuals that were assigned female at birth (AFAB), aged between 15 and 30 years old with a history of PID or infections caused by the offending microbial pathogens associated with the development of PID or individuals who were recently fitted with an intrauterine device (IUD). 

Patients often present to hospitals with complaints of acute pain in the right upper quadrant of their abdomen or chronic tenderness in that same area that is worsened by body movements and deep breathing. Thorough patient history checks, physical examination and laboratory tests are often ordered to establish a PID diagnosis; following this, they will be treated with antibiotics that are commonly used to treat chlamydia and gonorrhoea.

If patients have already been given antibiotic treatment and present with the aforementioned abdominal pain, they may be referred for a CT scan, MRI or ultrasound that will help doctors further diagnose. If FHCS is suspected or diagnosis is still unclear following lab and imaging tests, patients will then undergo a laparoscopy or laparotomy dependent on the severity of the case; these procedures help doctors visualise if there is adhesion formation between the liver capsule and abdominal wall, a key characteristic of FHCS. If these adhesions are seen they will be cut during this procedure and patients will then be given postoperative care. Complications from surgery are possible but rare.