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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 53  |  Issue : 3  |  Page : 109-112

Bile duct lavage and cholecystectomy: A single-stage operation for inspissated bile syndrome


1 Department of Paediatric Surgery, SMS Medical College, Jaipur, Rajasthan, India
2 Department of Radiodiagnosis, SMS Medical College, Jaipur, Rajasthan, India
3 Department of Paediatrics, SMS Medical College, Jaipur, Rajasthan, India

Date of Submission23-Dec-2019
Date of Decision04-Feb-2020
Date of Acceptance12-Mar-2020
Date of Web Publication30-May-2020

Correspondence Address:
Dr. Rahul Gupta
Department of Paediatric Surgery, SMS Medical College, Jaipur, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/fjs.fjs_110_19

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  Abstract 


A 90-day-old infant with inspissated bile syndrome unresponsive to medical management is presented. The infant was born premature, low birth weight (1600 g) at 28 weeks, became jaundice few days after birth. He received neonatal intensive care therapy for 4 weeks. Investigations on admission revealed conjugated hyperbilirubinemia (total bilirubin – 8.26 mg/dl). Abdominal ultrasound and magnetic resonance cholangiopancreatography were suggestive of biliary atresia with nonvisualization of the gallbladder and common bile duct (CBD). Intraoperatively, cholecystotomy confirmed the presence of bile; placement of infant feeding tube in the infundibulum with saline irrigation confirmed CBD obstruction. After multiple gentle attempts with saline lavage, distal clearance was achieved, suggested by duodenal filling up with saline and by nasogastric aspiration. The patient passed colored (bile containing) stools with bile plugs on the 5th postoperative day. Bilirubin values returned to normal, and the outcome was favorable.

Keywords: Bile duct lavage, common bile duct obstruction, cholecystectomy, cholecystotomy, conjugated hyperbilirubinemia, inspissated bile syndrome


How to cite this article:
Gupta R, Gupta AK, Tanger R, Bhandari A, Gupta RK, Gupta AK. Bile duct lavage and cholecystectomy: A single-stage operation for inspissated bile syndrome. Formos J Surg 2020;53:109-12

How to cite this URL:
Gupta R, Gupta AK, Tanger R, Bhandari A, Gupta RK, Gupta AK. Bile duct lavage and cholecystectomy: A single-stage operation for inspissated bile syndrome. Formos J Surg [serial online] 2020 [cited 2020 Sep 24];53:109-12. Available from: http://www.e-fjs.org/text.asp?2020/53/3/109/285398




  Introduction Top


Inspissated bile syndrome is the presence of inspissated bile resulting in extrahepatic bile duct obstruction.[1] It is a rare but correctable cause of conjugated hyperbilirubinemia in neonates and infants.[1],[2] We present herein a 90-day-old infant with inspissated bile syndrome, in whom peroperative cholecystotomy and bile duct lavage were performed which were both diagnostic and therapeutic; a mini review of literature is presented.


  Case Report Top


A 90-day-old male infant (weight – 3200 g) presented with clay (pale)-colored stools, yellow discoloration of the skin, and passage of yellowish (discolored) urine from few days after birth. There was a presence of gradually increasing abdominal distention for the past 1 month; and there was no history of fever or episodes of vomiting. The infant was first in birth order, born premature, low birth weight (1600 g) at 28 weeks and received neonatal intensive care therapy (parenteral nutrition, exchange transfusion, phototherapy, and incubator support) for 4 weeks. Antenatal ultrasounds were not done. On examination, the neonate was icteric and hemodynamically stable, pulse rate was 112/min and respiratory rate was 32/min. The abdomen was soft, mildly distended with palpable liver (one finger breadth below the subcostal margin).

Laboratory investigations revealed anemia (hemoglobin – 10.8 g/dL), conjugated hyperbilirubinemia (total bilirubin – 8.26 mg/dl and direct bilirubin – 5.06 mg/dl), and raised liver enzymes (ASAT-260 IU/L, ALAT-159 IU/L and gamma-glutamyl transpeptidase [GGT]-352 IU/L). Renal functions, serum electrolytes, and coagulation profile were normal; qualitative C-reactive protein was normal, and viral markers were negative. Abdominal ultrasound showed nonvisualization of the gallbladder and common bile duct (CBD) along with hepatomegaly suggestive of biliary atresia. Magnetic resonance cholangiopancreatography (MRCP) revealed few nondilated intrahepatic biliary radicals, nonvisualization of CBD [Figure 1], along with a small gallbladder-like structure in favor of biliary atresia [Figure 1]. Tru-cut liver biopsy was inconclusive.
Figure 1: T2W magnetic resonance cholangiopancreatography images (transverse sections, left side) showing few nondilated intrahepatic biliary radicals; magnetic resonance cholangiopancreatography images (coronal sections, center, and right side) showing the absence of dilated common bile duct or dilated gallbladder in patient with cholestasis

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Preoperative optimization was performed with blood transfusion, phenobarbitone, ursodeoxycholic acid (15 mg/kg), medium-chain triglyceride-rich formula, and nutritional hyperalimentation 2 weeks before the surgery. Intraoperatively, the liver was enlarged with altered color and texture; a nondilated relatively small gallbladder was present. Cholecystotomy was performed, which confirmed the presence of bile; placement of infant feeding tube (IFT) no. 6 in the infundibulum with saline irrigation confirmed CBD obstruction. Intraoperative cholangiography could not be performed due to resource limitations. After multiple gentle attempts with saline lavage, distal clearance was achieved, suggested by duodenal filling up with saline (containing methylene blue) and by nasogastric aspiration. After bile duct lavage with saline, cholecystectomy was performed before completing the operation. The patient passed colored (bile containing) stools with bile plugs on the 5th postoperative day. There were no postoperative complications; ultrasound examination was done after the stool became colored. The infant continued to be on phenobarbitone (5 mg/kg/day). Serum bilirubin and liver enzymes values gradually started to fall and returned to normal after 6 weeks. Postoperative ultrasounds did not reveal any abnormality in the biliary tree. The outcome was favorable, and the patient is doing well, gaining weight at 5 months follow-up.


  Discussion Top


Neonatal hyperbilirubinemia associated with extrahepatic bile duct obstruction (obstructive jaundice) may be caused by inspissated bile. William E. Ladd used the term “inspissated bile syndrome” for the first time in 1935.[3] Inspissated bile syndrome is defined as partial or complete obstruction of the extrahepatic biliary system by the impaction of thick bile or sludge in the distal CBD during the neonatal period.[2]

Incidence of inspissated bile syndrome is 1/175,000 live births in England and accounts for about 8% of all surgical jaundice during infancy.[4] The most common components of inspissated bile are cholesterol crystals and calcium salts. The predisposing causes of inspissation are sludge in the gallbladder, for example., secondary to prolonged parenteral nutrition, prior major abdominal surgery, sepsis, prematurity, congenital heart disease, hemolytic disease of newborn, erythroblastosis fetalis, hemolysis, diuretic therapy, and idiopathic.[5] The other causes of inspissated bile are metabolic disease, cystic fibrosis, or bile duct malformations.[6] In our case, bile inspissation was due to the conglomeration of factors, for example, prematurity, hemolysis requiring exchange transfusion, and parenteral nutrition in the neonatal period. According to the large series (16 infants) on inspissated bile syndrome from China, females predominated (9:7), and their age ranged from 40 days to 3 months.[2]

In contrast to biliary atresia, where jaundice gradually but steadily deepens and the outcome is invariably fatal unless surgical correction can be achieved, the inspissated bile syndrome is characterized by early onset of jaundice and a generally favorable outcome.[3] Its diagnosis is established on the clinical presentation and typical ultrasound findings which show a dilated extrahepatic bile duct, biliary sludge/plugs, and a distended gallbladder [2] although, equivocal findings may be present, as seen in our case.[7] In our case, the gallbladder was nondistended; it is proposed to be due to its nonusage and limited bile production. This was in contrast to distended gallbladder, as seen in 100% cases of inspissated bile syndrome.[2]

Spectrum of inspissated bile syndrome may range from transient (with spontaneous resolution) to resolution after medical treatment with ursodeoxycholic acid and to other extreme progressing relentlessly and requiring surgical intervention.[1],[2],[5] In some cases, removal of the precipitating cause can lead to spontaneous resolution. As per the recent study, conservative treatment with ursodeoxycholic acid failed and required surgical intervention in all cases in the series.[2]

Neonatal cholestasis remains a major diagnostic and therapeutic challenge till date. Laboratory values reveal elevated conjugated bilirubin and elevated total alkaline phosphatase. Aspartate aminotransferase (AST) or alanine aminotransferase may be elevated. GGT is high, but inconsistent levels can be found in patients with sepsis and metabolic disorders of bile acid synthesis. Cholestasis screen includes viral markers for hepatitis A, hepatitis B, CMV, and HIV, TORCH screen (toxoplasmosis, other [HIV, hepatitis viruses, varicella, parvovirus], rubella [German measles], cytomegalovirus, herpes simplex, syphilis) and alpha1 antitrypsin levels. MRCP and liver biopsy are performed to rule out biliary tract malformations. Intraoperative cholangiography using meglumine and diatrizoate is highly informative to evaluate the level of obstruction and delineate the biliary tract anatomy.[2]

Bile duct dilatation (more than 3 mm), persisting conjugated hyperbilirubinemia, and increasing liver enzymes are regarded as indication for surgical intervention; the latter two were present in our patient.[5],[8] In our case, the patient was planned for surgery with the preoperative diagnosis of biliary atresia; only when the cholecystostomy revealed bile and inspissated bile in CBD, diagnosis of inspissated bile syndrome was made.

Techniques used for clearance of obstructed bile ducts are: (a) laparoscopic or (b) open cholecystostomy with the placement of a catheter (or IFT) into the gallbladder for repeated biliary duct lavage (irrigation) with saline or ursodeoxycholic acid,[5] (c) endoscopic retrograde cholangiopancreatographic sphincterotomy, (d) percutaneous transhepatic gallbladder puncture and balloon dilatation of CBD.[9]

In cases where clearance of obstructed bile ducts is not established peroperatively, cholecystostomy is performed and daily (or alternate day) lavage of the gallbladder with boluses of 5 ml saline and 3 ml of ursodeoxycholic acid (50 mg/ml) till complete clearance of CBD has been recommended by one study.[2],[5] Other agents used are the hydrostatic infusion of a mucolytic agent, 2% N-acetyl cysteine, and pancreatin.[5],[6] Adjunctive treatment with intravenous synthetic cholecystokinin and phenobarbitone has been used.[6] In our case, cholecystostomy was not feasible as the gallbladder was small, and it was not possible to bring the fundus to the anterior abdominal wall.

Among all the techniques for inspissated bile syndrome not responding to medical therapy, laparoscopic aided cholecystostomy and bile duct lavage, performed for 2–4 weeks, have been most commonly performed.[1],[2],[6] In our case, we believe that resolution could be achieved in single stage due to prior preparation, preoptimization and also due to continuous, gentle, and sustained attempts with saline lavage.[1] Furthermore, multiple stage operation is required when cholecystostomy is performed to either detach (separate) the gallbladder from the abdominal wall or to perform cholecystectomy.[5]

In the present case, the gallbladder was small, nondilated (collapsed), and cholecystotomy was performed after applying stay sutures on the gallbladder. After bile plugs were completely washed out, and clear bile was seen coming out from cholecystotomy, and also duodenal filling up with saline, cholecystectomy was performed instead of primary closure of the cholecystotomy site due to multitude of factors: (i) closure of the cholecystotomy could result in small gallbladder remnant, (ii) risk of bile leakage from the stump and biliary peritonitis, (iii) nidus for gallbladder calculi in the following years, and (iv) it is also recommended by other authors.[10]

The serum levels of bilirubin, direct bilirubin, AST, and GGT decreased postoperatively in our case. This was similar to other series where resolution is expected over 4–8 weeks period.[2],[6] Medical therapy must continue to prevent inspissation postoperatively till there is complete resolution.


  Conclusion Top


A single-stage comprising bile duct lavage with normal saline for clearance of obstructed bile ducts followed by cholecystectomy in inspissated bile syndrome with preoptimization is feasible and safe in some carefully selected patients.

Declaration of patient consent

In the form the patient's guardian has given the patient's consent for the images and other clinical information to be reported in the journal. The patient's guardian understands that the patient's name and initials will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest



 
  References Top

1.
Gubernick JA, Rosenberg HK, Ilaslan H, Kessler A. US approach to jaundice in infants and children. Radiographics 2000;20:173-95.  Back to cited text no. 1
    
2.
Gao ZG, Shao M, Xiong QX, Tou JF, Liu WG. Laparoscopic cholecystostomy and bile duct lavage for treatment of inspissated bile syndrome: A single-center experience. World J Pediatr 2011;7:269-71.  Back to cited text no. 2
    
3.
Kibel MA. Hereditary spherocytosis complicated by the inspissated bile syndrome. S Afr Med J 1961;35:50-3.  Back to cited text no. 3
    
4.
Gunnarsdóttir A, Holmqvist P, Arnbjörnsson E, Kullendorff CM. Laparoscopic aided cholecystostomy as a treatment of inspissated bile syndrome. J Pediatr Surg 2008;43:e33-5.  Back to cited text no. 4
    
5.
Berger S, Schibli S, Stranzinger E, Cholewa D. One-stage laparoscopic surgery for inspissated bile syndrome: Case report and review of surgical techniques. Springerplus 2013;2:648.  Back to cited text no. 5
    
6.
Evans JS, George DE, Mollit D. Biliary infusion therapy in the inspissated bile syndrome of cystic fibrosis. J Pediatr Gastroenterol Nutr 1991;12:131-5.  Back to cited text no. 6
    
7.
Jun WY, Cho MJ, Han HS, Bae SH. Use of omega-3 polyunsaturated fatty acids To Treat inspissated bile syndrome: A case report. Pediatr Gastroenterol Hepatol Nutr 2016;19:286-90.  Back to cited text no. 7
    
8.
Fitzpatrick E, Jardine R, Farrant P, Karani J, Davenport M, Mieli-Vergani G, et al. Predictive value of bile duct dimensions measured by ultrasound in neonates presenting with cholestasis. J Pediatr Gastroenterol Nutr 2010;51:55-60.  Back to cited text no. 8
    
9.
Duman L, Büyükyavuz BI, Akcam M, Koroglu M, Tepeli H. Percutaneous management of bile-plug syndrome: A case report. J Pediatr Surg 2011;46:e37-41.  Back to cited text no. 9
    
10.
Brownschidle S, Sullivan J, Sartorelli K, Potenta S, Zenali M. Neonatal cholestasis due to biliary sludge-review of literature and report of a case associated with use of diflucan. Ann Clin Pathol 2014;2:1018.  Back to cited text no. 10
    


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