Last updated: January 28, 2013
Years published: 2010, 2013
NORD gratefully acknowledges Michael J Boros, MD, Munson Medical Center, for assistance in the preparation of this report.
Chilaiditiโs syndrome is a rare condition in which a portion of the colon is abnormally located (interposed) in between the liver and the diaphragm. The diaphragm is the muscle that separates the chest cavity from the abdomen. Chilaiditiโs syndrome can cause a variety of symptoms including abdominal pain, nausea, vomiting, and small bowel obstruction. The specific symptoms and presentation of Chilaiditiโs syndrome can vary greatly from one person to another. The cause of Chilaiditiโs syndrome is not fully understood.
In most cases, interposition of a portion of colon between the liver and diaphragm does not cause symptoms (asymptomatic) and is often an incidental finding in the elderly. When no symptoms are present, this clinical finding is referred to as Chilaiditiโs sign. In rare cases, symptoms do develop; these cases are referred to as Chilaiditiโs syndrome.
The symptoms of Chilaiditiโs syndrome may vary from one person to another. The presentation and specific symptoms that develop can be significantly different. Chronic, recurrent episodes of abdominal pain are a common finding. Abdominal pain may be mild and come and go (intermittent). However, abdominal pain can be severe and force affected individuals to go to the emergency room.
Additional symptoms can occur with Chilaiditiโs syndrome. These symptoms can occur together in a wide variety of different combinations. Affected individuals will not have all of these symptoms and some affected individuals will not have any of these symptoms.
Additional symptoms that have been reported in Chilaiditiโs syndrome include nausea, vomiting, constipation, indigestion (dyspepsia), abnormal twisting of the intestines (volvulus) causing obstruction, abdominal swelling (distention), difficulty swallowing (dysphagia), and tenderness in the upper, central area of the abdomen (epigastric region). In some cases, Chilaiditiโs syndrome has been associated with breathing problems (respiratory distress).
The exact cause of Chilaiditiโs syndrome is unknown. The condition occurs with greater frequency in individuals with chronic lung disease, scarring of the liver (cirrhosis), and the accumulation of fluid within the abdominal cavity (ascites). Ascites can be associated with a wide variety of medical conditions.
Additional predisposing factors include abnormal elongation of the colon or abnormal looseness (laxity) of certain ligaments of the colon and liver. Ligaments are tough, fibrous bands of tissue that primarily serve to connect or support structures within the body.
According to the medical literature, other factors may play a role in the development of Chilaiditiโs syndrome in some cases including reduced liver volume, paralysis of the motor nerve of the diaphragm (phrenic nerve palsy) and obesity.
Chilaiditiโs syndrome affects males and females in equal numbers. It is more common in older adults, but can occur at any age and has been reported in children. The incidence of Chilaiditiโs syndrome is unknown. One published report estimated the incidence at .025-.28 percent of the general population. Chilaiditiโs syndrome was first described in the medical literature in 1910 by a Greek radiologist named Demetrius Chilaiditi.
A diagnosis of Chilaiditi's syndrome is made based upon imaging (radiographic) confirmation of the abnormal positioning of the colon and the occurrence of associated symptoms. Such imaging techniques may include chest and abdominal x- rays, ultrasounds, or computerized tomography (CT) scanning.
Treatment
The treatment of Chilaiditi's syndrome is directed toward the specific symptoms that are apparent in each individual. Some affected individuals may not require any therapy. The removal of pressure from the intestines (bowl decompression) has alleviated symptoms in some cases.
In some cases, surgical intervention may be required. Surgical techniques that have been used to treat individuals with Chilaiditi's syndrome include the removal of a portion of the colon (transverse colectomy or right hemicolectomy) or the anchoring of a displaced liver to the abdominal wall (hepatopexy).
IInformation on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Toll-free: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]
For information about clinical trials sponsored by private sources, in the main, contact:
www.centerwatch.com
For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/
Contact for additional information about Chilaiditiโs syndrome:
Michael J Boros, M.D.
Munson Medical Center
Traverse City, MI 49684
Michael J Boros, M.D.
JOURNAL ARTICLES
Glatter RD, April RS, Miskovitz P, Neistadt LD. Severe recurrent abdominal pain: an anatomical variant of Chilaiditiโs syndrome. MedGenMed. 2007;9:67.
Keles S, Artac H, Reisli I, Alp H, Koc O. Chilaiditi syndrome as a cause of respiratory distress. Eur J Pediatr. 2006;165:367-369.
Saber AA, Boros MJ. Chilaiditiโs syndrome: what should every surgeon know? Am Surg. 2005;71:261-263.
Kulkarni N, Dadayal G, Yeluri S, Kapoor A, Gupta A. Medical image. Chilaiditiโs syndrome. N Z Med J. 2004;117:U1092.
Barroso Jornet JM, Balaguer A, Escribano J, et al. Chilaiditi syndrome associated with transverse colon volvulus: first report in a paediatric patient and review of the literature. Eur J Pediatr Surg. 2003;13:425-428.
Plorde JJ, Raker EJ. Transverse colon vovulus and associated Chilaiditiโs syndrome: case report and literature review. Am J Gastroenterol. 1996;9
NORD strives to open new assistance programs as funding allows. If we donโt have a program for you now, please continue to check back with us.
NORD and MedicAlert Foundation have teamed up on a new program to provide protection to rare disease patients in emergency situations.
Learn more https://rarediseases.org/patient-assistance-programs/medicalert-assistance-program/Ensuring that patients and caregivers are armed with the tools they need to live their best lives while managing their rare condition is a vital part of NORDโs mission.
Learn more https://rarediseases.org/patient-assistance-programs/rare-disease-educational-support/This first-of-its-kind assistance program is designed for caregivers of a child or adult diagnosed with a rare disorder.
Learn more https://rarediseases.org/patient-assistance-programs/caregiver-respite/