chilaiditi's syndrome. successful surgical correction by colopexy

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D.F. Altomare () M. Rinaldi M. Petrolino P.L. Sallustio A. Guglielmi O.C. Pannarale Department of Emergency and Organ Transplantation, Section of General Surgery and Liver Transplantation, Coloproctology Unit, Policlinico, Piazza G. Cesare 11, I-70124 Bari, Italy Introduction Chilaiditi’s syndrome consists of interposition of the small or large bowel between the inferior face of the diaphragm and the liver. This condition was identified for the first time by Demetrio Chilaiditi [1] in 1910 and is reported to have an incidence ranging between 0.025% and 0.28% in the gener- al population [2]. It is a rare anomaly, often just a radiologi- cal finding (Chilaiditi’s sign), but can sometimes be compli- cated by severe and unresponsive abdominal pain, intestinal volvulus, bowel ischaemia and obstruction (Chilaiditi’s syn- drome) [3]. In the medical literature, two types of this syn- drome are described: anterior and posterior types according to the position of the bowel and the liver [4]. We present a case of Chilaiditi’s syndrome observed and treated surgical- ly in our department. Case report A 50-year-old man came to our outpatients clinic in January 2001 complaining of faecal incontinence. In 1986 he had undergone surgical implantation of a spinal electrode con- nected to an implantable pulse generator to control severe pain after spinal trauma. Since this trauma he had been affected by urinary and faecal incontinence. In addition, he complained of long-lasting right abdominal pain, resistant to the usual analgesics, which normally improved spontaneous- ly during the night. For this abdominal pain he had already had two colonoscopies but both were uncompleted because of the impossibility of passing through the right transverse colon; a barium enema was then performed but only sigmoid diverticulitis was diagnosed. The correct diagnosis was made some months later during an abdominal X-ray performed before treatment of the faecal incontinence (Fig. 1). The abdominal radiographs showed interposition of the large Tech Coloproctol (2001) 5:173–175 © Springer-Verlag 2001 D.F. Altomare M. Rinaldi M. Petrolino P.L. Sallustio A. Guglielmi O.C. Pannarale Chilaiditi’s syndrome. Successful surgical correction by colopexy Received: 10 August 2001 / Accepted in revised form: 29 September 2001 CASE REPORT Abstract We describe the case of a patient affected by the Chilaiditi’s syndrome, the interposition of the small or large bowel between the inferior face of the diaphragm and the liver, a rare anomaly, often asymptomatic. A 50-year-old man came to our outpatients clinic because of persistent pain in the right abdominal region. Two attempts to perform colonoscopy failed because of the impossibility of passing through the transverse colon. A double contrast enema indi- cated only sigmoid diverticulitis. Only after abdominal ra- diography was the abnormal position of the right colon noted. At surgery the right colon was totally intraperitoneal and positioned between the diaphragm and liver, which was smaller than normal. The right colon was repositioned and fixed to the anterior abdominal wall. The postoperative course was uneventful and the right abdominal pain disap- peared completely. In conclusion, surgical treatment of Chilaiditi’s syndrome may be required in cases of persistent abdominal pain. Key words Chilaiditi Hepatodiaphragmatic herna Colopexy

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Page 1: Chilaiditi's syndrome. Successful surgical correction by colopexy

D.F. Altomare (�) • M. Rinaldi • M. Petrolino • P.L. SallustioA. Guglielmi • O.C. PannaraleDepartment of Emergency and Organ Transplantation,Section of General Surgery and Liver Transplantation,Coloproctology Unit, Policlinico,Piazza G. Cesare 11, I-70124 Bari, Italy

Introduction

Chilaiditi’s syndrome consists of interposition of the small orlarge bowel between the inferior face of the diaphragm andthe liver. This condition was identified for the first time byDemetrio Chilaiditi [1] in 1910 and is reported to have anincidence ranging between 0.025% and 0.28% in the gener-al population [2]. It is a rare anomaly, often just a radiologi-cal finding (Chilaiditi’s sign), but can sometimes be compli-cated by severe and unresponsive abdominal pain, intestinalvolvulus, bowel ischaemia and obstruction (Chilaiditi’s syn-drome) [3]. In the medical literature, two types of this syn-drome are described: anterior and posterior types accordingto the position of the bowel and the liver [4]. We present acase of Chilaiditi’s syndrome observed and treated surgical-ly in our department.

Case report

A 50-year-old man came to our outpatients clinic in January2001 complaining of faecal incontinence. In 1986 he hadundergone surgical implantation of a spinal electrode con-nected to an implantable pulse generator to control severepain after spinal trauma. Since this trauma he had beenaffected by urinary and faecal incontinence. In addition, hecomplained of long-lasting right abdominal pain, resistant tothe usual analgesics, which normally improved spontaneous-ly during the night. For this abdominal pain he had alreadyhad two colonoscopies but both were uncompleted becauseof the impossibility of passing through the right transversecolon; a barium enema was then performed but only sigmoiddiverticulitis was diagnosed. The correct diagnosis was madesome months later during an abdominal X-ray performedbefore treatment of the faecal incontinence (Fig. 1). Theabdominal radiographs showed interposition of the large

Tech Coloproctol (2001) 5:173–175 © Springer-Verlag 2001

D.F. Altomare • M. Rinaldi • M. Petrolino • P.L. Sallustio • A. Guglielmi • O.C. Pannarale

Chilaiditi’s syndrome. Successful surgical correction by colopexy

Received: 10 August 2001 / Accepted in revised form: 29 September 2001

C A S E R E P O R T

Abstract We describe the case of a patient affected by theChilaiditi’s syndrome, the interposition of the small or largebowel between the inferior face of the diaphragm and theliver, a rare anomaly, often asymptomatic. A 50-year-oldman came to our outpatients clinic because of persistent painin the right abdominal region. Two attempts to performcolonoscopy failed because of the impossibility of passingthrough the transverse colon. A double contrast enema indi-cated only sigmoid diverticulitis. Only after abdominal ra-diography was the abnormal position of the right colonnoted. At surgery the right colon was totally intraperitonealand positioned between the diaphragm and liver, which wassmaller than normal. The right colon was repositioned andfixed to the anterior abdominal wall. The postoperativecourse was uneventful and the right abdominal pain disap-peared completely. In conclusion, surgical treatment ofChilaiditi’s syndrome may be required in cases of persistentabdominal pain.

Key words Chilaiditi • Hepatodiaphragmatic herna • Colopexy

Page 2: Chilaiditi's syndrome. Successful surgical correction by colopexy

bowel between the lower face of the diaphragm and theupper face of the liver. Due to persistence of the rightabdominal pain, we decided to operate the patient in order toreposition the bowel. At laparotomy, the patient was found tohave a small liver and the entire right colon, the hepatic flex-ure and the proximal transverse colon incarcerated in theright subphrenic space between the liver and the diaphragm(Fig. 2). There was also malrotation of the sigmoid anddescending colon which was retroperitoneally positioned inthe right side and a Meckel’s diverticulum. The bowel was

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repositioned in the abdominal cavity and a colopexy wasperformed by suturing the right colonic flexure and half ofthe transverse colon to the anterior abdominal wall withinterrupted Vicryl 4/0 sutures. The postoperative course wasuneventful. The patient was discharged after 2 days.Postoperative abdominal radiography performed two weekslater showed normal location of the bowel. Notably, the rightabdominal pain disappeared completely immediately aftersurgery. At the 9-month follow-up, the clinical conditionsremained unchanged.

D.F. Altomare et al.: Chilaiditi’s syndrome

Fig. 1 Lateral chest X-Ray showing thecolonic interposition between liver anddiaphragm

Fig. 2 Intraoperative finding with the rightand transverse colon dislocated under theright diaphragm

Page 3: Chilaiditi's syndrome. Successful surgical correction by colopexy

Discussion

Hepatodiaphragmatic interposition of the bowel wasdescribed for the first time in 1865 [5] but only in 1910 wasthis particular abdominal complaint named Chilaiditi’s syn-drome, after Demetrio Chilaiditi, a German radiologist [1].In the medical literature, about 110 cases of this syndromehave been described, but the true prevalence of this conditionin the population cannot be precisely estimated becausesometimes there are no clinical signs and these may not beabdominal, as shown by a report of heart arrhythmia and res-piratory symptoms [6]. There are many contributing factorsin the pathogenesis of this syndrome such as reduction of theliver volume (cirrhosis) and laxity of the hepatic suspensoryligaments, colonic redundancy, malrotation or congenitalmalpositioning of the bowel; it also has been reported to beassociated with clinical conditions such as pneumatosis cys-toides intestinalis, scleroderma, melanosis coli, duplicationof the small intestine and pregnancy [7]. Finally, a relation-ship with mental retardation and use of antipsychotic drugshas recently been documented [8]. Only in a few cases, how-ever, does a surgical approach become necessary, occasion-ally as an emergency due to bowel ischaemia or intestinalobstruction by volvulus. The most frequently preferred sur-gical treatment is extraperitoneoplasty [6] that elides theenlarged subphrenic space, but right hemicolectomy hassometimes been indicated in cases of volvulus [2]. Bothoperations are quite complex and risky compared to thebenign nature of the syndrome. For that reason we preferredto perform anterior colopexy of the right flexure and trans-verse colon to the anterior abdominal wall. This procedureprevents the colon itself and the small bowel to dislocateupward between the liver and the diaphragm without risk.Another possibile low-risk and easy surgical option is theomentum interposition to obliterate the right subphrenicspace. Clearly, control studies comparing different surgicaloptions are not feasible in the case of this rare disease, butthis operation also has the advantages to be quick and easy

to perform. Although Chilaiditi’s syndrome can usually bemanaged conservatively, a surgical procedure is necessarywhen severe symptoms unresponsive to medical therapy orcomplications are present. Although a longer follow-up iscertainly necessary for a definitive conclusion, in our expe-rience peritoneal colopexy is a low-risk, effective procedurefor preventing further colonic dislocations.

Acknowledgements We thank Mary Victoria Pragnell for review-ing the manuscript.

References

1. Chilaiditi D (1910) Zur Frage der Hapatoptose und Ptose inallgemeinen im Auschluss an drei Falle von temporaerer par-tiellaer Leberverlagerung. Fortschritte auf dem Gebiete derRoentgenstrahlen 11:173–208

2. Orangio GR, Fazio VW, Winkelman E, McGonagle BA(1986) The Chilaiditi syndrome and associated volvulus of thetransverse colon. An indication for surgical therapy. DisColon Rectum 29:653–656

3. Plorde JJ, Raker EJ (1996) Transverse colon volvulus andassociated Chilaiditi’s syndrome: case report and literaturereview. Am J Gastroenterology 91:2613–2616

4. Oancea T, Bocaneala O, Popescu P (1968) L’interpositionhepato-diaphragmatique postérieure du colon. J Chir96:547–552

5. Rogers JCT (1935) Hepatodiaphragmatic interposition ofcolon: report of a case. Ill Med J 68:264–268

6. Gullino D, Cardino L, Giordano O, Ghione S, Rachetta A(1985) Trattamento chirurgico della sindrome di Chilaiditi ointerposizione viscero-epato-diaframmatica. Min Chir40:983–989

7. Risaliti A, De Anna D, Terrosu G, Uzzau A, Carcoforo P,Bresadola F (1993) Chilaiditi’s syndrome as a surgical andnonsurgical problem. Surg Gyn Obstet 176:55–58

8. Miyaoka T, Seno H, Itoga M et al (2001) Chilaiditi’s syn-drome associated schizophrenia: 3 case reports. J ClinPsychiatry 62:58–59

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