Introduction

Fitz-Hugh-Curtis syndrome (FHC) and acute appendicitis pose a diagnostic challenge due to overlapping clinical manifestations, especially in young female patients with acute abdominal pain [1]. Acute appendicitis, which peaks in incidence among individuals aged 10–19, typically progresses from upper abdominal to right lower quadrant pain, resembling gynecologic conditions such as pelvic inflammatory disease (PID) and its severe complication, tubo-ovarian abscess [1, 2]. Symptoms such as right lower quadrant pain associated with fever and leukocytosis, and imaging findings such as appendiceal dilation are indicative of acute appendicitis [2]. Conversely, PID may manifest as vaginal discharge, pelvic tenderness, and urinary symptoms [2, 3]. FHC is often triggered by pathogens similar to those causing PID such as Chlamydia trachomatis or Neisseria gonorrhoeæ, is an inflammation of the liver capsule with adhesion formation accompanied by right upper quadrant pain [2, 3]. Laparoscopy has improved diagnostic accuracy among these conditions, further supported by imaging techniques such as computed tomography (CT) scans, which enhance diagnostic accuracy for both FHC and appendicitis [4].

Herein, we describe a case of FHC mimicking acute appendicitis in a female patient, followed by a comprehensive review of the available literature on diagnosis and appropriate therapeutic management.

Case Report

A 34-year-old woman presented to the emergency department with symptoms of upper abdominal pain, accompanied by nausea and fever (38.7 °C). The symptoms, which had lasted for 3 days, were only partially relieved by symptomatic therapy and, over the following days, the pain extended to the right iliac fossa. Her medical history included a previous episode of left pyelonephritis during pregnancy, two full-term pregnancies, no known drug allergies, and no previous surgical procedures. Her body mass index was 20.

No abdominal tenderness apart from the right lower quadrant was present, where Mc Burney's sign was strongly present. Rovsing's and iliopsoas tests were negative, with no signs of localized or diffuse peritonitis, and peristalsis was present. An abdominal ultrasound indicated a thickened appendiceal wall (Fig. 1A) with a 3-mm intraluminal fecalith and a small perivisceral fluid collection (Fig. 1B), suggesting acute appendicitis. Laboratory findings included a normal white blood cell count, a hemoglobin level of 12.0 g/dL, a CRP level of 21.39 mg/L, and an ALT level of 125 U/L. The Alvarado and AIR-Anderson scores were 6 and 3, respectively, indicating a low risk of appendicitis.

Fig. 1
figure 1

Abdominal ultrasound showing a thickened appendix wall (A) with a 3-mm intraluminal coprolite (B)

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Due to the suspected diagnosis of acute appendicitis, an exploratory laparoscopy was performed, showing PID and extensive perihepatitis suggestive of FHC (Fig. 2A–D). Notably the 'violin string' scarring and adhesions were observed on the liver capsule (Fig. 2B, C) and were carefully detached by laparoscopic adhesiolysis. The appendix showed signs of mild inflammation as shown in Fig. 3; laparoscopic appendectomy was performed due to the presence of the fecalith and periappendiceal fluid. A vaginal swab was positive for Chlamydia trachomatis and negative for Neisseria gonorrhoeæ. Tests for hepatitis B and C were negative.

Fig. 2
figure 2

Exploratory laparoscopy revealed pelvic inflammatory disease (PANEL A), with diffuse perihepatitis, suggesting the diagnosis of Fitz-Hugh Curtis-Stajano syndrome (PANEL B–D). The initial formation of classic 'violin string' adhesions was observed along with liver capsule scarring and inflammation (PANEL B-C)

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Fig. 3
figure 3

The appendix showed signs of mild inflammation

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The patient was treated with a 10-day course of intravenous antibiotics (piperacillin–tazobactam and doxycycline) with gradual normalization of her inflammatory markers and liver function tests. Histological analysis of the appendix confirmed chronic appendicitis. She was discharged in good health on the tenth postoperative day.

Discussion

FHC was first reported by Dr Carlos Stajano during the 1920 meeting of the Society of Obstetricians and Gynecologists in Montevideo, Uruguay [5]. A decade later, in 1930, Dr Thomas Fitz-Hugh and Dr Arthur Curtis detailed the syndrome’s hallmark “violin string” adhesions, which uniquely link the liver to the abdominal wall in women with previous salpingitis, distinguishing it from other forms of peritonitis [6, 7]. Initially it was linked exclusively to Neisseria gonorrhoeæ due to findings by Fitz-Hugh on liver capsule smears [6]. The etiological perspective widened in 1978 when Müller-Schoop et al. identified Chlamydia trachomatis in similar cases [8]. This finding was supported by subsequent studies by Wang et al. and Paavonen et al., establishing C. trachomatis as a prevalent cause of FHC, which mainly affects women of reproductive age [9, 10].

The symptomatic interplay between FHC and acute appendicitis often poses a significant diagnostic challenge due to their overlapping presentations but distinct underlying pathologies [11]. FHC, a complication of pelvic inflammatory disease, involves inflammation of the liver capsule (perihepatitis) without affecting the liver parenchyma, leading to right upper quadrant pain that can mimic hepatic, biliary, or gastrointestinal disorders, including appendicitis [11,12,13]. Pathophysiologically, FHC is characterized by the spread of the infection from the pelvic region to the liver capsule via the right paracolic gutter, often resulting in the formation of “violin string” adhesions [2].

Clinically, acute appendicitis typically presents with periumbilical pain that localizes to the right lower quadrant, often accompanied by anorexia, nausea, and fever [14]. Laboratory findings in acute appendicitis frequently include elevated white blood cell counts and C-reactive protein levels, indicating systemic inflammation [14]. Radiologic features such as a thickened appendiceal wall and the presence of an appendicolith on ultrasound or CT scan are characteristic of acute appendicitis [15, 16].

Conversely, FHC primarily involves right upper quadrant pain due to perihepatitis and can be mistaken for hepatic or biliary pathology [17]. Clinical features distinguishing FHC from acute appendicitis include a history of sexually transmitted infections, pelvic pain, and symptoms of PID such as vaginal discharge [2]. Radiologically, CT scans of FHC reveal hepatic capsular enhancement and perihepatic fluid [18, 19]. Biochemically, FHC is accompanied by mild elevations in liver enzymes such as ALT and AST, and slight alterations in other markers such as bilirubin, alkaline phosphatase (ALP), and γ-glutamyl transferase (GGT) all supporting the diagnosis of FHC over more worrisome hepatic conditions [20].

The patient's medical history plays a crucial role in differentiating between these conditions. A thorough history that includes sexual activity, previous PID episodes, and any related symptoms such as vaginal discharge or dyspareunia can point towards FHC [2]. In contrast, a lack of these historical features and a more straightforward presentation of acute right lower quadrant pain may lean towards a diagnosis of acute appendicitis. Thus, combining clinical presentation, laboratory results, radiologic findings, and a detailed patient history is essential for the effective differentiation and management of these conditions. A comprehensive sexual history and gynecological examination are essential in distinguishing among these conditions.

We conducted a comprehensive literature review through PubMed and Scopus, using the keywords “Fitz-Hugh Curtis,” “appendicitis,” “appendectomy,” and “appendix” to collect relevant studies. The reference lists of identified articles were examined to uncover additional relevant publications. The selected publications, which included case reports and review articles, underwent a thorough analysis by the authors. Data from these sources were extracted using a standardized data collection tool. Table 1 provides a summary of the reviewed articles [21,22,23,24,25,26].

Table 1 Summary of retrieved studies on FHC and acute appendicitis
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From the data analysis, the average patient age ranged from 15–40, with a predominance of females who were notably young (mean age 24 years). These patients were often sexually active with multiple partners, aligning with the transmission dynamics of Chlamydia trachomatis, the most commonly identified pathogen. Common clinical features included lower abdominal pain, primarily in the right lower quadrant, abdominal guarding, varying degrees of fever, and a history of malodorous vaginal discharge, which was reported by the majority of the cases. Imaging studies such as CT scan and abdominal ultrasound typically showed appendiceal enlargement, hepatic capsular enhancement, or peritoneal inflammation; laboratory findings often indicated elevated systemic inflammatory markers such as CRP and white blood cell count. Treatments largely consisted of combinations of broad-spectrum antibiotics like azithromycin, ceftriaxone, and doxycycline, with some cases requiring surgical interventions such as appendectomy and adhesiolysis. The presence of vaginal discharge underscores the importance of considering a sexually transmitted etiology in young females presenting with right upper quadrant pain and other related symptoms. Post-operative follow-up was generally around 42–90 days, with no reported recurrences, highlighting the importance of an effective management of the initial conditions.

The management of FHC entails surgical laparoscopy not only as a diagnostic tool but also for its contribution to treating the characteristic “violin string” adhesions that define the condition [1]. By carefully detaching the liver from these adhesions, laparoscopy alleviates the pain and prevents the sequelae associated with perihepatic inflammation [1]. Pharmacological treatment is essential for managing the underlying PID that gives rise to FHC, with a regimen of antibiotics tailored to treat Chlamydia trachomatis and Neisseria gonorrhoeae infections [22]. The successful resolution of symptoms and the absence of recurrence in follow-up underscore the efficacy of combining surgical treatment with targeted antibiotic therapy [27]. This double strategy ensures comprehensive management of both the mechanical and infectious symptoms of FHC, promoting full recovery and minimizing the risk of chronic complications [27].

Conclusion

The complexity of differentiating FHC from acute appendicitis highlights the necessity for precise diagnostic protocols, especially in female patients presenting with acute abdominal symptoms, including the combination of laparoscopy and imaging techniques.

Key Messages

  • FHC may present symptoms similar to acute appendicitis, particularly in young women, necessitating a precise and thorough diagnostic approach for accurate differentiation and management.

  • Laparoscopy is essential in the diagnosis and management of FHC, resolving symptoms and preventing recurrence by allowing direct visualization and treatment of the characteristic “violin string” perihepatic adhesions.

  • A tailored antibiotic regimen is critical in managing the underlying pelvic inflammatory disease associated with FHC.