Portions of this study have been presented as a poster abstract at the Case Western Reserve University 2021 by Martha L. Lepow MD, and Irwin H. Lepow, MD, PhD, during the Virtual Medical Student Research Day.

Case

A 26-year-old male with a past medical history of human immunodeficiency virus (HIV) (CD4 count of 981) and hepatitis C virus infections presented to the emergency department for abdominal pain. His pain started six days prior, originally located in the lower left quadrant. The pain was described as cramping and worsened with having a bowel movement. The patient also noted intermittent fevers and chills.

He had been seen two days prior to our emergency department visit at an outside hospital. The previous work-up included a computed tomography (CT) scan of the abdomen and pelvis, which showed perirectal inflammation concerning for proctitis. Rectal swabs for gonorrhea and chlamydia were performed; however, results were still pending. Labs revealed a mild transaminitis (alanine transaminase (ALT) 53, aspartate transaminase (AST) 43). Otherwise, complete blood count (CBC), basic metabolic panel (BMP), and lipase levels were within normal limits. The patient was treated with an intramuscular dose of ceftriaxone 250 mg and prescribed seven days of doxycycline, which he had been taking as prescribed.

In our emergency department (ED), blood pressure was 115/67, pulse 65, temperature 98.3 F, respiratory rate 16, SpO2 99 percent. Physical exam was significant for lower left quadrant abdominal tenderness without peritoneal signs. The patient also had a new right upper quadrant (RUQ) abdominal tenderness. BMP, CBC, and lipase were within normal limits. LFTs showed improving AST of 41 and ALT of 37. Due to the acute worsening of his abdominal pain and new RUQ abdominal pain on exam, a CT scan of the abdomen and pelvis was ordered. CT scan confirmed known rectal wall thickening concerning for proctitis. However, new findings included liver surface enhancement concerning for perihepatitis (see Image 1). Given the high likelihood of gonorrhea or chlamydial origin of his proctitis, there was concern that this may be Fitz-Hugh-Curtis Syndrome (FHCS). Rectal swabs were again performed. Intravenous (IV) Cefoxitin and Doxycycline were administered. The patient was admitted to the hospital, where his pain improved. Rectal cultures were positive for Chlamydia trachomatis. An infectious disease physician was consulted and recommended a 21-day course of doxycycline. The patient was discharged two days later.

Pathophysiology

FHCS, also known as perihepatitis, is a rare complication of chronic pelvic inflammatory disease (PID). It is characterized by inflammation of the liver capsule and surrounding peritoneum with “violin-string” adhesion formation. FHCS is usually found in sexually active women between 15–30, with incidence ranging from four to 14 percent in individuals with PID. The condition was first reported in the English language in 1930 by Dr. Curtis, who noted extensive adhesions between the anterior surface of the liver and anterior abdominal wall in a female patient presenting with gonococcal salpingitis.¹ In 1934, Dr. Fitz-Hugh reported similar cases in which three women presented with RUQ abdominal pain due to acute gonococcal peritonitis that showed liver capsule adhesions.² Although Neisseria gonorrhoeae was initially thought to be the primary cause of FHCS, Chlamydia trachomatis has also been shown to be a frequent causative agent.³ Infectious agents associated with PID are thought to be disseminated either through direct spread from reproductive organs to the peritoneal cavity, hematogenous spread, or lymphatic spread. Some studies have also hypothesized that FHCS may be the result of an exaggerated immune response to C. trachomatis due to increased serum titers of antichlamydial immunoglobulin G antibodies.⁴