Abstract Congenital anomalies of the gall bladderinclude its complete absence, hypoplasia and atresia, duplication, excessive mobility and ectopias, but they are very rare.Suprahepaticanteriorly placed gall bladder with chronic budd chiari syndrome is an extremely rare congenital anomaly. We report incidental radiological findings associated with a 66 yr old male, with no past history of related symptoms,who was admitted for evaluation after a history of assault. Imaging by computed tomographydemonstrated suprahepatic anteriorly placed gall bladder and portal vein with chronic budd chiari syndrome.

Key words: gall bladder, anomalies, Budd ChiariSyndrome

Introduction

Congenital anomalies gall bladders are not common. These can be associated with other systemic malformations and additional alterations in bile ducts and vascular tree. Preoperative diagnosis of malformation is obtained only in exceptional cases.

Case report

A previously healthy 66 yr old patient was admitted in our emergency with alleged history of assault. On physical examination, he did not present any signs and symptoms related to hepatobiliary abnormality. All the routine laboratory investigations were within normal limits. Ultrasound revealed ectopically suprahepatic anteriorly placed gall bladder and portal vein.

CT scan was performed which revealed gall bladder lying ectopically anterior to the liver below the anterior abdominal wall. In addition porta hepatis is located in relation to antero-lateral surface of the liver. Suprahepatic portion of the inferior vena cava was absent. The infrahepatic portion showed focal narrowing at the level of hepatic venous confluence. The infra hepatic inferior venacava showed marked dilatation with foci of calcification in its wall. Venous collaterals were seen in bilateral paravertebral region,retroperitoneum,greater omentum and anterior abdominal wall. Azygous and hemiazygous,superior and inferior epigastric, lumbar vein showed marked dilatation. Findings were suggestive of chronic budd chiari syndrome. The liver was shrunken with heterogeneous mosaic pattern enhancement. There was no clear demarcation line between the left and the right lobe. Spleenomegalyis seen with well distended spleenic vein and minimal portal vein dilatation.

 Discussion

Normally gall bladder is situated adjacent to the inferior surface of liver; in the plane of interlobar fissure with gall bladder neck maintaining constant relationship to portahepatis. Gall bladder is generally found in the right upper quadrant, but may be seen in other parts of the abdomen most commonly under the left hepatic lobe, Intra hepatic, Transverse or may be Retro placed (retro hepatic and retroperitoneal). The less common locations are Supra diaphragmatic and Sub hepatic. Gall bladder is intra hepatic during embryonic period and becomes extra hepatic later1.

Till date supra hepatic anteriorly placed gall bladder with chronic buddchiari syndrome has not been reported.

One of the case reportsthe normal insertion of gall bladder with 180°rotation upwards to an intrathoracic position after eventration of diaphragm. Abnormally mobile gall bladder trapped between chest wall and upper border of liver was found in other two studies2. Fauintuchet al3 reported three cases of supra hepatic gall with hypoplasia of right hepatic lobe and upward migration of gall bladder. Gansbeke4 reported a case of supra hepatic gall bladder with hepatomegaly. Kabaroudis5 reported a case of floating gall bladder with hypoplasia of right lobe of liver and Maeda6 reported a case with left hepatic lobe hypoplasia. Hibbs H7 reported a case of inverted liver with supra hepatic anteriorly displaced gall bladder.

 

 

 

Summary

So the congenital anomalies of gall bladder are exceptionally rare but it is important to emphasize that it is difficult to obtain the diagnosis and these malformation process preoperatively8. They may provide a real surprise during laparoscopic surgery.

Bibliography

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  2. Gross RE: Congenital anomalies of gallbladder: A report of 148 cases with report of a double gallbladder. Arch Surg; 1936(1); 32: 131-162.
  3. Fauintach J;Machado ML,Raicier AA. Suprahepatic GB with hypoplasia of right lobe of liver. Arch Surg 1980; 115:658-9.
  4.  Van Gansbeke D, de Toeuf,Cremer M,Engelholm L, Struyven J. Suprahepatic GB;A rare congenitalanomaly. GastrointestRadiol. 1984;9:341-3.
  5. Kabarounders A,Papazioges B, Atmatzides K, Argiriadur E, PanskevasA,Galanis I, et al. Hypoplasia of the right hepatic lobe combined with floating gall bladder. ActaChir Belg. 2003; 103: 425 – 7.
  6. Maeda N, Horie Y,Shiota G, Suou T, Andachi H,Hypoplasia of the left lobe associated with floating GB. 1998: 45; 1100-3.
  7. Hibbs H, Ahmed U, Inverted liver with suprahepatic,anteriorly displaced gall bladder. Hepaticgastroenterology. 1998; 45 (22): 1100-3.
  8. Idu M, Jakimowicz, Iuppa A, Cuschieri A. Hepatobilliary anatomy in patients with transposition of gall bladder: implications for safe laproscopic cholecystectomy. Br J Surg. 1996; 83(10): 1442-43.

 

LEGENDS

  1.  Fig1: CECT abdomen showing ectopically suprahepatic anteriorly placed   gall bladder and portal vein with dilated infrahepatic inferior vene cava. Liver is lobulated in outline. Spleen is enlarged and spleenic vein is prominent.
  2.  Fig 2: CECT abdomen showing ectopically placed gall bladder anrerior to liver with abnormal location of portal vein with porta placed anteriorly. Right branch of portal vein is visualised. Multiple venous channels are seen anterolateral to liver. Infrahepatic IVC is dilated. VC- Venous Channels, RBPV- Right Branch of Portal Vein.
  3. Fig.3: CECT abdomen coronal section showing dilated infrahepatic inferior vene cava with marked narrowing of intrahepatic (arrow head) and suprahepatic inferior vene cava.

 

 

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