Chilaiditi Syndrome: A Case Report, Literature Review, and Proposition of a Novel Management Staging System

Chilaiditi's sign refers to colonic interposition between the liver and the diaphragm in the right subphrenic space secondary to the relaxation of the suspensory ligaments of the right colic flexure. The diagnosis of Chilaiditi's sign is based on radiological findings with the following three criteria: 1) The right hemidiaphragm must be adequately elevated above the liver by the intestine, 2) the bowel must be distended by air to illustrate pseudo-pneumoperitoneum, and 3) the superior margin of the liver must be depressed below the level of the left hemidiaphragm. In this report, we present the case of a 49-year-old female presenting with signs and symptoms suggestive of Chilaiditi syndrome managed with laparoscopic surgery. We also present a literature review with a summary of previous studies and propose a novel management staging system for this syndrome.


Introduction
Chilaiditi's sign, also known as the interposition of the colon above the liver, refers to the presence of the colon in the right subphrenic space secondary to the relaxation of the suspensory ligaments of the right colic flexure [1].This condition was first documented in 1865 by Demetrius Chilaiditi, who reported three cases of patients with incidental benign intra-abdominal free air on routine X-rays [2,3].The sign is relatively rare, with an incidence of 0.025%-0.28% on abdominal or chest X-rays and 1.18%-2.4% on abdominal CT scans and with a marked 4:1 male-to-female predominance [3].Chilaiditi's sign is usually asymptomatic but can also present as Chilaiditi syndrome, which presents with respiratory distress, substernal pain, and cardiac arrhythmias [4].Chilaiditi syndrome typically requires prompt medical intervention, though surgical management may be considered in cases where medical treatment is unsuccessful or if the patient shows evidence of bowel obstruction/ischemia or worsening symptoms [1].To diagnose Chilaiditi's sign, several criteria must be met [5]: 1) the elevation of the right hemidiaphragm above the liver by the intestine, 2) the distended colon by air to illustrate pseudo-pneumoperitoneum, and 3) the depression of the superior margin of the liver below the level of the left hemidiaphragm.
While there are several cases of Chilaiditi syndrome reported in the literature, there is a paucity of data with regard to its management.In this study, we present a case of Chilaiditi syndrome in a 49-year-old female.Furthermore, we propose a novel staging system for patients with Chilaiditi syndrome based on radiological and clinical findings, with the aim of streamlining future medical management.

Case Presentation
A 49-year-old female with no pertinent past medical history presented to the emergency department with a chief complaint of sharp chest pain for 3-4 hours.The pain was localized to the right upper quadrant (RUQ) and epigastric regions, with radiation to the right shoulder and chest.The patient reported an increased frequency of intermittent pain associated with nausea, which was exacerbated by deep breathing and oral intake.The patient also endorsed occasional dysphagia, globus sensation, palpitations, and long-standing chronic constipation.She had no relief with over-the-counter antacids and laxatives for many years and did report a family history of constipation and colonic perforation.Surgical history was significant for appendectomy and cholecystectomy.Physical examination revealed marked abdominal distention and mild epigastric pain with decreased bowel sounds.A CT scan of the abdomen and pelvis revealed a diffusely hyperlucent diaphragm with a lobular appearance, consistent with bowel gas and Chilaiditi syndrome (Figure 1).Follow-up abdominal imaging showed gaseous loops of bowel present throughout the gastrointestinal tract with distention extending down to the rectum.Distended loops of small bowel were noted, with most lying within the left side of the abdomen, necessitating a surgical consultation.

FIGURE 1: CT of the abdomen revealed a hyperlucent appearance involving the diaphragm diffusely with a lobular appearance, consistent with bowel gas and Chilaiditi syndrome.
Despite implementing a combination of medical management, lifestyle modifications, the use of multiple laxatives, and changes in diet, her bowel movements continued to be infrequent, occurring every 2-3 weeks.Further evaluation with a colonoscopy, a sitz marker study, and a balloon expulsion test in the absence of anorectal abnormalities excluded other conditions including inflammatory bowel disease, constipation, perforation, and abdominal or pelvic floor dysfunction.After a thorough discussion with the patient regarding the risks and benefits of surgical management, a laparoscopic-assisted subtotal colectomy with ileorectal anastomosis was performed.The patient was later successfully discharged on postoperative day 6.
Postoperatively, the patient's recovery was complicated by the development of a pelvic abscess, which was successfully treated with percutaneous drainage.Subsequently, she reported no nausea or vomiting and was tolerating a diverse diet with an average of 3-5 bowel movements per day.

Discussion
Upon initial presentation, our patient displayed signs and symptoms consistent with a potential diagnosis of Chilaiditi syndrome including sharp, localized pain in the chest and RUQ of the abdomen accompanied by nausea.The pain was not worse with breathing but may have been triggered by oral intake.Treatment through medical management including oral antacids was unsuccessful, and she required surgical intervention.While our patient's case was successfully managed with surgery, this may not be the case for all patients with this syndrome.Table 1 highlights a case series of varying outcomes in patients managed conservatively and patients who underwent surgical interventions currently present in published literature.Currently, there is no widely accepted standard method for classifying or managing patients with Chilaiditi syndrome.Given the varying responses to treatment, it is crucial that a standardized protocol be developed to aid clinicians with classification and management.We propose a three-stage grading system for Chilaiditi syndrome patients.This system is based on radiological and clinical findings with the goal of simplifying future medical management (Table 2).The proposed grading system includes symptoms such as abdominal pain (which can be diffuse or localized to the RUQ), difficulty tolerating oral intake, chest pain, and vasovagal reactions.The proposed grading system will provide a clear and consistent approach to evaluating and managing Chilaiditi syndrome patients.With this new system, clinicians can make well-informed decisions to improve patient outcomes.We also propose the use of our treatment algorithm (Figure 2) to guide management for Chilaiditi syndrome.
Following the classifications in Table 2, mild cases of Chilaiditi syndrome (stage 1) should be managed medically with a high-fiber diet, laxatives or stool softeners, and antispasmodics [14].Additionally, physical activity has been shown to be beneficial by promoting intestinal peristalsis [15].However, moderate to severe cases (stages 2 and 3) should be worked up further with imaging scans such as gastrografin enemas, CT scans with endoluminal contrast, and colonoscopy.In cases where these imaging studies provide equivocal or normal findings, additional diagnostic tests such as sitz marker studies, balloon expulsion studies, and anal manometry studies should be performed.These studies will help differentiate Chilaiditi syndrome from other benign anorectal maladies.For patients who test positive on these additional diagnostic tests, surgical intervention may be considered as the most suitable treatment option.

Conclusions
Chilaiditi syndrome is a complex condition that can manifest in various complications, depending on the underlying causes.This report highlights and describes a standardized staging system to guide the medical management of Chilaiditi syndrome patients.This will help ensure that patients receive the most appropriate treatment for their condition and that treatment is tailored to their level of severity.This will ultimately lead to improved outcomes for patients with Chilaiditi syndrome.

Additional Information Disclosures
Human subjects: Consent was obtained or waived by all participants in this study.Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work.Financial relationships: All authors have declared that they have no financial Pain resolved with supportive treatment, and he was stable before discharge.Oneyear follow-up: asymptomatic and no complications Bin US of the Aborted initial laparoscopy for conservative management.Gradually 2023 Adu et al.Cureus 15(10): e46688.DOI 10.7759/cureus.days of hospitalization with close follow-up.Four-week follow-up period: reported complete resolution of abdominal pain, distension, and defecation difficulties.Nine-month followup: asymptomatic and no complications Yin et al. follow-up: appendectomy for persistent symptoms.Three-week followup: reported complete resolution of abdominal pain with improved regularity of bowel movements.Two-month follow-up:

FIGURE 2 :
FIGURE 2: Proposed treatment algorithm to Chilaiditi syndrome to guide optimal management.