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 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 4  |  Issue : 3  |  Page : 180-182

Laparoscopic cholecystectomy in situs ambiguous


1 Department of Surgery, Government Medical College, Patiala, Punjab, India
2 Department of Radiodiagnosis, Government Medical College, Patiala, Punjab, India

Date of Web Publication8-Dec-2014

Correspondence Address:
Dr. Anoop Varma
Room no 56, Doctors PG Hostel, Rajendra Hospital, Government Medical College, Patiala - 147 001, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-9596.146438

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  Abstract 

A twenty seven year old female patient presented to the hospital with recurrent pain in the right hypochondrium of five years duration. She had a history of failed cholecystectomy four years back. A CT and MRI scan, revealed splenenculi, midline liver, cholelithiasis, truncated pancreas and dextrocardia. These symptoms were attributed to the calculous cholecystitis. Laparoscopic cholecystectomy was done and patient recovered uneventfully. Here we are presenting the first instance of successful laparoscopic cholecystectomy in a patient with heterotaxy syndrome, as it is a unique challenge for operating surgeons. The purpose of this report is to encourage further research to establish a protocol for the management of these patients with a rare congenital anomaly. This will help us establish a protocol for such patients so as to minimize complications intra operatively.

Keywords: Laparoscopic cholecystectomy, situs ambiguous, splenenculi


How to cite this article:
Varma A, Mahajan A, Singh M, Sandhu GS, Kaur N. Laparoscopic cholecystectomy in situs ambiguous. Arch Int Surg 2014;4:180-2

How to cite this URL:
Varma A, Mahajan A, Singh M, Sandhu GS, Kaur N. Laparoscopic cholecystectomy in situs ambiguous. Arch Int Surg [serial online] 2014 [cited 2022 Sep 29];4:180-2. Available from: https://www.archintsurg.org/text.asp?2014/4/3/180/146438


  Introduction Top


Situs anomalies are rare, complex, and confusing with an incidence of 1/8000-1/25 000 live births. [1] Situs ambiguous or heterotaxy implies a disordered organ arrangement in the chest or abdomen. This unpredictable anatomy differs from the orderly arrangement of the truncal organs in either the typical anatomy (situs solitus) or the mirror image of it (situsInversus). Situs solitus is the usual arrangement of organs and vessels within the body. The systemic atrium is on the right with a right-sided trilobed lung, liver, gallbladder, and inferior vena cava. The pulmonary atrium is on the left with a left-sided bilobed lung, stomach, single spleen, and aorta. The incidence of congenital heart disease in patients with situs solitus and levocardia is only 0.6%-0.8% [2] Situs inversus refers to an anatomic arrangement that is the mirror image of situs solitus. It is seen in 0.01% of the population. [2]

Situs ambiguous also known as heterotaxy syndrome can be classified into situs ambiguous with polysplenia or asplenia. Situs ambiguous with polysplenia (SAP) also known as classic left isomerism or bilateral left sidedness, implies that patients have bilateral bilobed lungs, bilateral pulmonary atria, a centrally located liver, a stomach in indeterminate position, and multiple spleens. However, no single anomaly was detected that is pathognomonic for this condition. [3] Interruption of the inferior vena cava with azygous or hemiazygous continuation is the most consistent finding seen in heterotaxy with polysplenia.

In this report, we present a case of calculus cholecystitis in a patient with situs ambiguous and the challenge of laparoscopic cholecystectomy in these patients.


  Case Report Top


A 27-year-old female patient was admitted to our hospital with diagnosis of cholelithiasis with cholecystitis. She was scheduled for open cholecystectomy four years prior to presentation in a peripheral hospital, but the surgeon was unable to locate the gall bladder. Later she had multiple episodes of pain in the epigastrium with radiation to the right hypochondrium and right scapular region that resulted in multiple hospital admissions. There were no significant findings on abdominal examination. On perabdomen examination no significant findings were present. On cardiovascular examination apex beat was found to be in 4 th intercostalsspace in midclavicular line on the right side.

On ultrasonography, cholelithiasis with chronic cholecystitis and mild hepatomegaly was noted. On contrast enhanced computed tomography (CECT) of abdomen and chest, liver was found to be enlarged in size, hypo dense, extra hepatic gallbladder, splenenculi, normal pancreas and dextrocardia was found. The gallbladder neck was interlobar, fundus pointing towards midline and was with multiple gall stones. On magnetic resonance imaging (MRI) scan, abdomen and liver were seen in midline and the extra and intra hepatic ducts were normal [Figure 1]. Gall bladder was seen in midline with a calculi and cystic duct opening in to hepatic duct bifurcation [Figure 2]. Multiple spleen of variable sizes and shapes were seen in left hypochondrium. Pancreas was small with non-visualization of part of head and tail suggestive of truncated pancreas [Figure 3]. Patient had gestational hypertension and was diagnosed to have type 1 diabetes mellitus three months back and is on insulin therapy.
Figure 1: MRI image showing liver and gall bladder in mid line

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Figure 2: MRCP image showing intra and extra hepatic biliary radicals

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Figure 3: MRI showing truncated pancreas

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Cholecystectomy was done under general anesthesia with four port technique. First port was placed just below umbilicus, second in epigastrium, third in right hypochondrium on right mid axillary line and the fourth one in left hypochondrium on left mid axillary line. Gall bladder was found to be in midline with adhesions to omentum and falciform ligament. pancreas was found to be in midline and the lower portion of cystic duct was found to attach to common bile duct just above pancreas. Areas of inflamed tissue were present around gallbladder, pancreas. Multiple splenenculi were present in left hypochondrium. Gall bladder was freed from adhesions; cystic artery was placed posteriorly to cystic duct which was clipped and ligated. Cystic duct was ligated with vicryl 1-0. Specimen was extracted out. Post operative recovery was uneventful and patient was discharged on the 3 rd day. Patient was followed up for one month and was found to be symptomatically better.


  Discussion Top


Situs ambiguous or Heterotaxy syndrome is characterized by a spectrum of abnormalities. Its incidence has been reported to vary widely between 1:4,000 to 1:20,000. [4] There are two major subcategories 1) Situs ambiguous with polysplenia (SAP) which is characterized by midline or ambiguous location of the majority of the abdominal organs and multiple spleens. 2) Situs ambiguous with asplenia. SAP has a lower prevalence of congenital heart disease (50%-90%) [5] than patients of situs ambiguous with asplenia (99%-100%). [5] In our case dextrocardia was the only abnormality noted. Situs ambiguous with polysplenia is associated with multiple discrete spleens in the majority of patients but some studies report patients having a single spleen. [6],[7],[8] The consistent relationship between the spleens and the stomach has been noted and is explained by the fact that splenic tissue develops in the dorsal mesogastrium. [9] In majority of patients, the liver and gallbladder were midline. [6] Biliary abnormalities including biliary atresia have been reported in paediatric patients. [7] In most patients with SAP, the pancreas was truncated. Only the pancreatic head was present in most patients and was located to the right of midline. [10] In our case the head and tail of the pancreas were absent.

In conclusion, laparoscopic cholecystectomy for calculus cholecystitis in a patient with situs ambiguous is feasible and safe, although technically more demanding. It should be done by an experienced surgeon who should be able to recognize anatomical relationships and coexistence of other anomalies during surgery.


  Acknowledgements Top


We all are working in Government Medical College, Patiala, Punjab and we don't have any support in the form of grants, equipment, and/or pharmaceutical items.

 
  References Top

1.
Afzelius B, Mossberg B. The metabolic and molecular basis of inherited disease. In: Scriver C, Beaudet A, Sly W, Valle D, editors. Immotile-Cilia Syndrome (Primary Ciliary Dyskinesia), Including Kartagener Syndrome. Vol. 3. New York: McGraw-Hill; 1995. p. 3943-54.  Back to cited text no. 1
    
2.
Ito K, Matsunaga N, Mitchell DG, Fujita T, Honjo K, Awaya H, et al. Imaging of congenital abnormalities of the portal venous system. AJR Am J Roentgenol 1997;168:233-7.  Back to cited text no. 2
    
3.
Winer-Muram HT, Tonkin IL. The spectrum of heterotaxicsyndromes. Radiol Clin North Am 1989; 27: 1147-70.  Back to cited text no. 3
    
4.
Nugent EW, Plauth WH Jr, Edwards JE,Williams WH. The pathology, abnormal physiology, recognition and treatment of congenital heart disease. In: Schlant RC, Alexander RW, C' Rourke RA, Roberts R, Sonnenblick EH, editors. The Heart, Arteries and Veins. 8 th ed. New York: McGraw Hill Inc; 1994. p. 1761-828.  Back to cited text no. 4
    
5.
Tonkin IL. The definition of cardiac malpositions with echocardiography and computed tomography. In: Friedman WF, Higgins CB, editors. Pediatric Cardiac Imaging. Philadelphia: Saunders, 1984;157-87.  Back to cited text no. 5
    
6.
Applegate KE, Goske MJ, Pierce G, Murphy D. Situs revisited: Imaging of the heterotaxy syndrome. Radiographics 1999;19:837-52.  Back to cited text no. 6
    
7.
Chandra RS. Biliary atresia and other structural anomalies in the congenital polysplenia syndrome. J Pediatr 1974;85: 649-55.  Back to cited text no. 7
[PUBMED]    
8.
Chen SC, Monteleone PL. Familial splenic anomaly syndrome. J Pediatr1977;91:160-1.  Back to cited text no. 8
[PUBMED]    
9.
Van Mierop LH, Gessner IH, Schiebler GL. Asplenia and polysplenia syndromes. Birth Def 1972;8:74-82.  Back to cited text no. 9
    
10.
Fulcher AS, Turner MA. Abdominal manifestations of situs anomalies in adults. Radiographics 2002;22:1439-56.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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Introduction
Case Report
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Acknowledgements
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