JOURNAL OF LAPAROENDOSCOPIC SURGERY Volume 2, Number 4, 1992 Mary Ann Liebert, Inc., Publishers

Brief Clinical

Report

Laparoscopic Cholecystectomy in Situs Inversus Totalis H. THOMAS TAKEI, THOMAS V. CLANCY,

GARY MAXWELL, M.D.,1'2 3 M.D.,2 32 J. 3 M.D.,1 and ELLIS A. TINSLEY, M.D.1 2

ABSTRACT A 51-year-old woman with known dextrocardia presented with left-sided abdominal pain and symptoms consistent with biliary colic and cholelithiasis. Abdominal ultrasound confirmed the diagnosis of gallstones, as well as situs inversus with the liver and gallbladder on the left side and the spleen on the right. Laparoscopic cholecystectomy was performed without incident. The procedure was uncomplicated except for being the mirror image of that done with the gallbladder in the normal location. Cholelithiasis occurring with situs inversus is rare and may present a diagnostic problem. The extrahepatic anatomy of the biliary and venous system is the mirror image of the right sided liver. Historic and genetic aspects of situs inversus, as well as current theories regarding its etiology are presented. Situs inversus totalis does not appear to be a contraindication to laparoscopic treatment of cholelithiasis.

INTRODUCTION

Although

situs inversus does not predispose individuals to any specific disease, the diagnosis of disease processes may be confusing and more difficult in these patients. Appendicitis is the most commonly reported surgical disease described in conjunction with situs inversus, while reports of situs inversus and biliary disease are few. Only 32 cases of cholelithiasis in patients with situs inversus have been reported in the English literature.I_7 Laparoscopic cholecystectomy is now widely accepted as the preferred therapeutic modality for symptomatic cholelithiasis and in some instances, for surgical therapy of acute common

cholecystitis.

From the Departments of Surgery, University of North Carolina School of Medicine, Chapel Hill, North Carolina1 and Hanover Regional Medical Center2 and the Area Health Education Center,3 Wilmington, North Carolina.

new

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TAKEI ET AL.

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FIG. 1.

Pre-op chest x-ray demonstrating dextrocardia. CASE REPORT

A 51 -year-old Caucasian woman presented with right-sided chest and arm pain in 1984. Chest x-ray (Fig. 1 ) and EKG showed dextrocardia; no evidence of cardiac disease was found. In July of 1990, she presented with a 5 year history of recurrent mid-epigastric and left upper quadrant pain associated with nausea and vomiting. She denied jaundice. She had hypertension requiring medication and gave a history of rheumatic fever. Her mother had gallstones. Examination showed a healthy appearing woman with normal temperature, a heart of 72, and blood pressure of 160/90. Scierai icterus was absent. Heart tones were normal but heard on the right side. There was no abdominal tenderness and no masses were noted. Chest x-ray again revealed dextrocardia. Abdominal ultrasound demonstrated situs inversus totalis with the liver and gallbladder on the left side and the spleen on the right. There were multiple stones in the gallbladder. Abdominal CT scan confirmed these findings (Fig. 2). On August 13, 1990, laparoscopic cholecystectomy was performed. Videoscopic examination confirmed situs inversus totalis with the appendix in the left lower quadrant, spleen in the right upper quadrant, and liver and gallbladder in the left upper quadrant. Mirror image anatomy of the gallbladder, common bile duct, and cystic duct was noted. Blunt dissection in the area of Calot's triangle revealed the cystic artery to also be in the mirror image position. Intraoperative cholangiogram showed a normal biliary tree (Fig. 3). Cholecystectomy was performed without difficulty. The procedure was the mirror image of that carried out on the right. The postoperative course was uneventful and the patient was discharged the following day. She continues to do well and has no complaints.

DISCUSSION Since Aristotle first noted the reversal of the position of the spleen and liver in animals, situs inversus has both intrigued and confused physicians.8 The first reported case of mirror image transposition in man was by 172

LAP CHOLE IN SITUS INVERSUS TOTALIS

showing mirror image positioning of the abdominal viscera.

FIG. 2.

CT

FIG. 3.

lntra-op cholangiogram showing normal biliary tree on the

scan

173

left side.

TAKEI ET AL. Fabricius in 1600.9 The first radiologie documentation of dextrocardia was made by Vehemeyer in 1897, and later, Rahman (1917) and Cleveland (1926) described complete visceral transposition in cadavers.I0 Estimates on the incidence of situs inversus vary from 1 in 5000 to 1 in 20,000 and probably reflect the difference in the method of diagnosing this rare condition.910 Situs inversus may be complete or partial (situs inversus totalis or partialis) and in the latter may be confined to the thoracic cavity or the abdominal viscera. Situs indeterminus is the third form of abdominal situs, in which the relationship of the atria and viscera is inconsistent; asymmetrical structures may be symmetrical and symmetrical structures may be asymmetrical (e.g. having a spleen on both the right and left). Associated anomalies are known to be quite frequent and Kartagener's syndrome (situs inversus, bronchiectasis, and sinusitis) is believed to be present in 25% of the cases.8 All patients with situs inversus totalis have dextrocardia, but associated cardiac anomalies are not as ' ' common as in isolated dextrocardia, where congenital heart disease is especially frequent. Situs inversus with levocardia is extremely rare and, like situs inversus indeterminus, is almost always associated with cardiac malformations. ' ' Anomalies of abdominal viscera such as esophageal atresia, duodenal and other intestinal atresia, common mesentery, asplenia, and polysplenia have also been described.3-812 The incidence of abdominal anomalies parallel those of the cardiac system and appear to be most frequent in patients with situs inversus indeterminus.8" The cause of the transposition is unknown. Cockayne believed that a single autosomal recessive gene was responsible and that gender distribution was nearly equal. After reviewing Cockayne's hypothesis and additional data, however, Campbell concluded that there was insufficient data to support this conclusion.I0 Others believe that mechanical forces present during embryonic development are responsible. They have postulated an altered relationship of the embryo to the chorion such that the right side of the embryo, rather than the left, is closer to the blood supply. The importance of temperature, growth, and nutrition as the cause of this condition have also been emphasized.910 Von Baer suggested an altered relationship between the embryo and umbilical vessels, while Virchow and Cleveland emphasized the significance of the reversed spiral twist in the umbilical cord.913 In the evaluation of patients with situs inversus, the location of pain can be variable and confusing. DePol (1933) first reported the discrepancy between localization of signs and symptoms and the site of the actual disease process. '4 Embryologically, the gallbladder is derived from the embryonic foregut, and thus visceral pain is referred to the midline or right side. There have been several reports of pain in the right upper quadrant in patients with cholecystitis and situs inversus. Visceral pain may be perceived as referred or direct. In order to explain the discrepancy of disease location and pain, King postulated that the peripheral nerves may or may not be transposed independently of the viscera.7"91415 Thus, if the nerves were not transposed then the visceral pain may be falsely projected to the opposite side. Rao noted that 60% of patients with left-sided cholelithiasis had left sided pain, 30% with mid-epigastric pain and about 10% with right-sided pain.2 Once the parietal peritoneum becomes involved, direct somatic perception should occur. Unfortunately, there are reports of patients with maximal tenderness on the opposite side of the disease in both appendicitis and cholecystitis. In 37-50% of the patients with appendicitis and situs inversus, pain was described in the right lower quadrant even though the appendix was located in the left lower quadrant.9 The explanation for this phenomenon is not clear. Correct anatomical diagnosis is important in any surgical problem, but it is of particular importance in the patient with situs inversus because the surgeon must be alert for and delineate any accompanying anomalies which are frequently associated with the condition. Wike has reported a case of non-rotation with cholecystitis while Fonkalsrud, Winer-Muram, and Warkany have described other intra-abdominal anomalies associated with situs inversus.38"12 It is also important to note that the finding of a left-sided gallbladder does not necessarily indicate transposition. Left-sided gallbladders and normal situs have been reported.16 This appears to be a congenital anomaly caused by the gallbladder being drawn to the left of the falciform ligament, creating a long tortuous cystic duct entering the common bile duct on the normal right side; or a gallbladder bud appearing on the opposite side of the hepatic diverticulum leading to the entrance of the cystic duct on the left side of the common bile duct.16 Physical examination can reveal findings which may arouse suspicion of situs inversus. The cardiac apical beat may be located in the right fifth intercostal space with heart tones best heard on the right, liver dullness

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LAP CHOLE IN SITUS INVERSUS TOTALIS

in the left upper quadrant, and gastric tympany on the right. In addition, the right testicle hanging lower than the left has been suggested as a helpful diagnostic sign, as has the direction of hair curls.I4 Chest x-ray can reveal dextrocardia while abdominal films may reveal the liver shadow on the left and the stomach bubble on the right. Further confirmation of transposition can be made with upper gastrointestinal study, barium enema, or CT scan. Abdominal ultrasound can detect cholelithiasis and also the mirror image positioning of viscera and vessels. Oral cholecystography and radionuclide scans have also demonstrated The diagnosis of abdominal situs inversus can be made with arteriography, however, other less invasive diagnostic studies are usually adequate. ' ' Finally, endoscopie retrograde cholangiopancreatography (ERCP) has been successfully performed in these patients. Situs inversus does not seem to be associated with an increased risk of biliary tract or Vaterian segment anomalies and is amenable to both contrast imaging In the patient with situs inversus, ERCP can be technically more difficlt, and a and right-sided dependent positioning is recommended.17 Although the intrahepatic anatomy of the biliary and venous system seem to correspond to the mirror image of the normal liver, the arterial irrigation does not follow the segmental biliary branching and appears to be quite different.I8 The extrahepatic biliary and arterial and venous anatomy appears to correspond to the mirror image of the normal. The other reports of cholecystectomy in transposition also support this observation. ',2,5_7 The only exception was a report by Chandraraj where the common hepatic artery originated from the superior mesenteric artery,10 a variant known to occur in about 17% of individuals with normal organ

transposition.3-4'7

sphincterotomy.8'17

placement.

Since first performed by Mouret in France in 1987, laparoscopic cholecystectomy has rapidly evolved as the standard therapeutic modality for gallstone disease.I9 Patients recognize that it is associated with less pain, reduced length of stay, and early return to work. In this patient with cholelithiasis and situs inversus, laparoscopic treatment was successfully performed without difficulty. Precision in diagnosis is essential whenever a laparoscopic approach is considered, but it becomes even more critical in transposition due to the higher likelihood of associated anomalies. The diagnosis may be more difficult and finding associated anomalies may alter the surgical approach. In situs inversus, as in the patient with normal anatomy, when the anatomic details are unclear, the threshold to convert the procedure to an open cholecystectomy or perform intraoperative cholangiogram should be lowered.

REFERENCES 1. Southam JA: Left sided

gallbladder: Calculous cholecystitis with situs inversus.

2. Rao PG,

Ann

Surg 1975;182:135-137. cholecystitis and mucinous

Katariya RN, Sood S, Rao PLNG: Situs inversus totalis with calculous cystadenomas of ovaries. J Postgrad Med 1977;23:89-90.

3. Wike CC, Caldwell BF, Parrish RA: A 4.

case

of abdominal situs inversus with non-rotation and cholelithiasis. Am

Surg 1970;36:346-348. Moreno AJ, Toney MA, Henry CD, Rodriguez AA, Turnbull GL: Acute cholecystitis in a patient with situs inversus.

Clin Nucí Med 1990;15:350-351.

5. McFarland SB: Situs inversus with cholelithiasis: A

6. Heimann T, Sicular A: Acute

cholecystitis

case

report. J Tenn Med Assoc 1989;82:69-70.

with situs inversus. NY State J Med 1979;79:253-254.

7. Haas GE, Harris JL, Segal LB: Situs inversus totalis with calculi in Osteopath Assoc 1984;83:718-720.

a

left sided

gallbladder: Report of a case. J Am

8. Fonkalsrud EW, Thompkins RC, Clatworthy HW: Abdominal manifestations of situs inversus in infants and children. Arch Surg 1966;92:791-795. 9. 10.

11.

Biegen HM: Surgery in situs inversus. Ann Surg 1949;129:244-259. Chandraraj S: Observations on some additional abnormalities in situs inversus viscerum. J Anat 1976;122:377-388. Winer-Muram HT, Tonkin ILD: The spectrum of heterotaxic syndromes. Radiol Clin North Am 1989;27:1147— 1170.

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17.

Warkany J: Congenital Malformations (lllus). Chicago, Year Book Medical Publishers, 1971:183-186. Cleveland M: Situs inversus viscerum: An anatomic study. Arch Surg 1926;13:342-368. Cholst MR: Discrepancies in pain and symptom distribution. Position of the testicle as a diagnostic sign in situs inversus totalis. Am J Surg 1947;73:104-107. Carmichael KA, Gayle WE: Situs inversus and appendicitis. South Med J 1979;72:1147-1150. KinoshitaH, SakaiK, KuboS, Ohno K: Two cases of left sided gallbladder associated with cholelithiasis: Review of the literature in Japan. Osaka City Med J 1988;34:67-75. Venu RP, Geenen JE, Hogan WJ, Johnson GK, Taylor AJ: ERCP and endoscopie sphincterotomy in patients with situs inversus. Gastrointest Endose 1985;31:338-340.

18. Ion A, Tiberiu CG: Anatomical features of the liver in situs inversus. Acta Anat 1982; 112:353-364.

19.

Phillips EH, Daykhovsky L, Carroll B, Gershman A, Grundfest WS: Laparoscopic cholecystectomy: technique. J Laparoendosc Surg 1990;1:3-15.

Instrumenta-

tion and

Address reprint requests to: J. Gary Maxwell, M.D. Area Health Education Center, 2131 South 17th Street, Wilmington, North Carolina, 28402

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Laparoscopic cholecystectomy in situs inversus totalis.

A 51-year-old woman with known dextrocardia presented with left-sided abdominal pain and symptoms consistent with biliary colic and cholelithiasis. Ab...
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