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Turkish Journal of Cancer
2007, Volume 37, Number 3, Page(s) 117-119
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Chilaiditiís syndrome with pancreatic malignancy
SEZA› DEM›RBAř, YAVUZ KURT, M. LEVH› AKIN, AHMET ÷ZT‹RK, TUNCAY «ELENK
GATA HaydarpaĢa Teaching Hospital, Department of General Surgery, ›stanbul-Turkey
Keywords: Chilaiditiís syndrome, subdiaphragmatic colonic interposition, pancreatic carcinoma
Summary
Hepatodiaphragmatic interposition of the intestine is a rare anomaly. It was first described by Demetrius Chilaiditi. This disorder may be associated with a variety of disorders. Though it is frequently asymptomatic with incidental radiographic sign Chilaiditiís syndrome may combine with imperative clinical problems requiring surgical treatment. Some symptoms mimicking the one occurred in pancreatic malignancy which was defined in this report may be the presenting finding in the aged patient with this syndrome. Even though Chilaiditiís syndrome is not listed among causes of pancreatic cancer, we present the first known report in the literature about the Chilaiditiís syndrome associated with pancreatic malignancy. [Turk J Cancer 2007;37(3):117-119]
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  • Introduction
    Chilaiditiís syndrome known as the interposition of the intestine between liver and right diaphragm (Hepatodiaphragmatic interposition, HDI) is a rare entity. It is frequently experienced in aged people, particularly in men. The incidence in the general population ranges from 0.025 to 0.28). An increased prevalence in elderly suggests that the disease is an acquired condition. Chilaiditiís syndrome is usually asymptomatic. In a clinical condition this disorder can be encountered with an incidental radiographic entity. However several cases with acute pain in right upper abdomen which was misdiagnosed with subphrenic abscess and/or pneumoperitoneum were reported [1-3]. In English literature, several clinical conditions associated with the Chilaiditiís syndrome including colonic and gastric cancer, colonic volvulus and obesity were reported [2-7]. No literature data exists regarding the association of the Chilaiditiís syndrome along with pancreatic malignancy. In this report the coexistence of the Chilaiditiís syndrome with pancreatic cancer was evaluated.
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  • Material and Methods
    During the retrospective analysis of the database of the Department of General Surgery from 1985 to 2002 4 cases of Chilaiditiís syndrome (3 male and 1 female) have been revealed. The patients all had abdominal pain, digestive complaints such as bloating, flatulence, obstructive jaundice and significant weight loss. The patients were all classified as ASA III in relation to ASA classification. Chilaiditiís syndrome was detected incidentally throughout the evaluation of patients for possible causative reason of jaundice by abdominal computed tomography (CT). Plain X-rays also revealed remarkably dilated colonic loops with air-fluid levels locating transversally below the diaphragm extending up to the right (Figures 1 and 2). In all patients a pancreatic mass causing to jaundice was found on the CT. Preoperative fine needle aspiration cytology of the mass revealed an adenocarcinoma of the pancreas. Gastroduodenopancreatectomy (Whippleís procedure) was performed in three patients. Hepaticojejunostomy + T tube bile diversion was made in the remaining one due to the mass invaded to portal vasculatures and adjacent tissues with proved lymphatic invasion. During the surgery of these patients hepatodiaphragmatic interposition of the proximal transverse colon was detected. Hepatopexy was performed by suturing the falciforme ligament on the anterior margin of liver up to the diaphragm to prevent further interposition of the colon. One of the patients undergoing Whipple procedure died on postoperative 3rd day. The patient undergoing hepaticojejunostomy also died three months after surgery owing to the complications of the unresolved jaundice. The remaining two patients with Whipple procedure have been still doing well in follow up.

    Fig 1: CT feature of the pancreatic mass and HDI

    Fig 2: Plain roentgenogram of the chest shows hepatodiaphragmatic interposition of the colon; elevation of the right hemidiaphragm and distended, fixed interposed colon

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  • Discussion
    Hepatodiaphragmatic interposition of the bowel is frequently an asymptomatic and rare clinical condition which remains as an undiagnosed entity during whole life time. In a period of time when the literature was reviewed, we have found 110 articles reporting approximately 150 cases of Chilaiditiís syndrome since 1965. Most of them associated with various disorders including the colonic volvulus, supra-hepatic appendicitis, scleroderma, congenital hypothyroidism, melanosis coli, salmonellosis and obesity, which seem to be reasonably related or without clear relationship to the disease. Also there were a few articles about Chilaiditiís syndrome associated with mammarian, colonic, gastric and pulmonary malignancies [2-4,8].

    Although frequently an asymptomatic clinical event Chilaiditiís syndrome combine with symptoms such as abdominal pain, nausea, vomiting, distention, flatulence, substernal pain, incomplete intestinal obstruction, and the condition consisting with cardiac arrhythmias and even difficult respiration [7,9]. In plain X-ray of the chest, the appearance of air collection marking with haustral signs in the subdiaphragmatic area gives a strong hint to diagnose. However subdiaphragmatic abscess show characteristics similar to the hepatodiaphragmatic interposition of the colon. If doubts still remain after plain X-ray of the chest CT combined with radio contrast media will be suggested to make a certain diagnosis [10]. If diagnosed in first step required treatment is usually a conservative one with bed-rest and nasogastric decompression. When the symptoms give a processing course to acute intestinal obstruction, surgical treatment then can be a requisite [11]. In addition, a few articles about colonic volvulus with Chilaiditiís syndrome were found in literature [10,12,13]. One case was about gastric volvulus and the other was about recurrent colonic volvulus [2,14].

    Some intestinal, diaphragmatic and hepatic factors induce progression of Chilaiditiís syndrome. Absence of peritoneal attachments and redundant colon with a long mesentery, abnormal colonic motility are the intestinal factors. A possible diaphragmatic factor is the location of abnormal upright position of diaphragm due to muscular degeneration of phrenic nerve injury. Hepatic factors incorporate small liver (cirrhosis), relaxation of suspensor ligaments.

    In our four cases of Chilaiditiís syndrome, we realized the colonic elongation and indulgent suspensory ligament of the colon as predisposing factors. But we could not find any relationship between the factors and pancreatic malignancy. The accompaniment of the pancreatic cancer with the Chilaiditiís syndrome in those cases may possibly be coincidental, but the similar dyspeptic symptoms of each disease especially in the early stage of pancreatic cancer may mimic each other.

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  • References

    1) Orangio GR, Fazio VW, Winkelman E, et al. The Chilaiditi syndrome and associated volvulus of the transverse colon. An indication for surgical therapy. Dis Colon Rectum 1986;29:653-6.

    2) Takagi Y, Abe T, Nakada T, et al. A case of Chilaiditiís syndrome associated with strangulated volvulus of the sigmoid colon. Am J Gastroenterol 1995;90:1905.

    3) Vessal K, Borhanmanesh F. Hepatodiaphragmatic interposition of the intestine (Chilaidaitiís syndrome). Clin Radiol 1976; 27: 113-6.

    4) Melester T, Burt ME. Chilaiditiís syndrome. Report of three cases. JAMA 1985;254:944-5.

    5) Risaliti A, De Anna D, Terrosu G, et al. Chilaiditiís syndrome as surgical and nonsurgical problem. Surg J Gyn Obst 1993;176:5-58.

    6) Havenstrite KA, Harris JA, Rivera DE. Splenic flexura volvulus in association with Chilaiditiís syndrome: Report of a case. Am Surg 1999;65:874-8.

    7) Murphy JM, Maibaum A, Alexander G, et al. Chilaiditiís syndrome and obesity. Clin Anat 2000;13:181-4.

    8) Sendon JL. Primary lung cancer and the Chilaiditi syndrome. Chest 1975,67:130.

    9) Schubert SR. Chilaiditiís syndrome: an unusual cause of the chest or abdominal pain. Geriatrics 1998;53:85-8

    10) Plorde JJ, Raker EJ. Transverse colon volvulus and associated Chilaiditiís syndrome: case report and literature review. Am J Gastroenterol 1996;91:2613-6

    11) Altomare DF, Rinaldi M, Petrolino M, et al. Chilaiditiís syndrome. Successful surgical correction by colopexy. Tech Coloproctol 2001;5:173-5.

    12) Gumbs MA, Kashan F, Shumofsky E, et al. Volvulus of the transverse colon: Report of cases and review of the literature. Dis Colon Rectum 1983;26:825-8.

    13) Javors BR, Sorkin NS, Flint GW. Transverse colon volvulus: A case report. Am J Gastroenterol 1986;81:708-10.

    14) Matthews J, Beck GW, Bowley DM, et al, Chilaiditiís syndrome and recurrent colonic volvulus: a case report. J R Nav Med Serv 2001;87:111-2.

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