Saudi Journal of Gastroenterology
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Year : 1998  |  Volume : 4  |  Issue : 1  |  Page : 8-12
Hepatobiliary scintigraphy in the distinction between biliary hypoplasia and biliary atresia


1 Department of Nuclear Medicine, King Khalid University Hospital, Riyadh, Saudi Arabia
2 Department of Pediatric Surgery, King Khalid University Hospital, Riyadh, Saudi Arabia
3 Department of Pediatrics, King Khalid University Hospital, Riyadh, Saudi Arabia

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Date of Submission03-Nov-1996
Date of Acceptance21-Oct-1997
 

   Abstract 

The aim of this work is to see whether distinction between biliary atresia and biliary hypoplasia is possible or not and to present the value and usefulness of hepatobiliary scintigraphy in the investigation of infants with persistent hyperbilirubinemia. Seventy-seven patients of the age between five days and six months (average 62 days), 43 females and 34 males of which 65 Saudi, and 12 non-Saudis were investigated. Laboratory tests, abdominal ultrasound, hepatobiliary scintigraphy, liver biopsy, explorative laparatomy and intraoperative cholangiography were performed whenever indicated. The findings on hepatobiliary scintigraphy of nonvisualization of the gallbladder and no activity in the bowel upto 24 hours post injection were considered consistent with the diagnosis of biliary atresia. Minimal bowel activity, or visualization of the gallbladder where consistent with biliary hypoplasia. In addition, the liver in the two entities appeared in a different shape and can be used as an additional distinctive feature. Thirty-four infants were diagnosed by hepatobiliary scintigraphy as having biliary atresia. Only 3 of the 34 were false positives as compared to cholangiogram and liver biopsy results. Five cases were biliary hypoplasia. Hepatobiliary scintigraphy in infants with persistent hyperbilirubinemia can be distinguished between BA and BH in a simple and noninvasive approach.

How to cite this article:
El-Desouki M, Mohamadiyah M, Al Rabeeah A, Othman S, Al Jurayyan N. Hepatobiliary scintigraphy in the distinction between biliary hypoplasia and biliary atresia. Saudi J Gastroenterol 1998;4:8-12

How to cite this URL:
El-Desouki M, Mohamadiyah M, Al Rabeeah A, Othman S, Al Jurayyan N. Hepatobiliary scintigraphy in the distinction between biliary hypoplasia and biliary atresia. Saudi J Gastroenterol [serial online] 1998 [cited 2021 Dec 8];4:8-12. Available from: https://www.saudijgastro.com/text.asp?1998/4/1/8/33900


Disorders of the biliary tree are an important cause of neonatal Hyperbilirubinemia (HB). The most frequent diagnoses are biliary atresia (BA) and neonatal hepatitis (NH). In BA the bile flow is obstructed due to obliteration of the extrahepatic ducts [1] . Biliary hypoplasia (BH) is a known entity in pediatric surgery [2],[3] . Nevertheless, BH has been debated to be as such on hepatobiliary scintigraphy (HBS). Using 99m Tc-imino diacetic acids (IDA), HBS has shown to be both useful and valuable in the investigation of infants with persistent neonatal HB In this work we present our experience at the King Khalid University Hospital, Riyadh investigating persistent HB in infants in order to distinguish between biliary atresia and biliary hypoplasia.


   Patients and Methods Top


Seventy-seven patients of the age between five days and six months (average 62 days), 43 females and 34 males, 65 Saudis and 12 non-Saudis, were investigated.

Hepatobiliary scintigraphy

After five days of administration of 5mg/kg/day of phenobarbitol, 1-2 mCi of 99m 'Tc-Dis-isopropyl (Disofenin) were administered intravenously to infants after 3-4 hours of fasting. Imaging of the abdomen starts immediately after injection with flow (16 frame/2 sec each) on 64 matrix. Followed by sequential static images of 3-5 minutes each for 60 minutes in anterior projection until visualization of gallbladder (GB) and/or bile ducts was observed, then other views in the right anterior oblique, right lateral, and posterior projections were taken. If activity in the bowel was not identified, delayed images of the abdomen were obtained at 2-3, 4-8, and 20-24 hours post injection.

Diagnostic criteria for BH were prompt and good liver uptake, visualization of the GB at any time during the study, and activity in the bowel at any time during the study. The first criterion is considered common between BH and BA and distinctive for BH and BA against the diagnosis of hepatitis. The other two criteria were considered distinctive for BH against the diagnosis of BA.

The scintigraphic images were reinterpreted by three nuclear medicine physicians (MD, MM, and SO) without the knowledge of the final diagnosis. The degree and pattern of radioactive tracer uptake (RATU) by the liver, GB visualization, and time of appearance of activity in the bowel, if occurred, were recorded. Nonvisualization of the GB and bowel was considered a positive indicator of BA. Poor RATU and/or inhomogenous uptake pattern by the liver was considered suggestive of NH. Conclusions were reached by consensus.

Diagnostic laboratory tests

Serial liver function tests were done for total bilirubin and the percent direct fraction in addition to the investigation of infectious microorganisms (i.e. TORCH).

Ultrasound

All patients underwent abdominal ultrasounds. Images were obtained using a variety of realtime ultrasound machines with 5.0-7.5 MHz sector transducers. All studies were performed after adequate time of fasting in relation to expected time of feeding). Ultrasound evaluation included the gallbladder; size, shape, and wall thickness and the intra- and extra-hepatic biliary trees; dilatation, filling defect, and presence of associated congenital malfounation (i.e. choledocal cyst).

Liver biopsy

Percutaneous liver biopsy was performed to confirm clinical suspicions. The findings of fibrosis, bile duct proliferation and fibrosis, and canalicular bile stasis were looked for in each patient.

Explorative laparotomy and operative cholangiogram

Exploration of the porta hepatis region was performed under general anesthesia and endotracheal intubation in each case in which biliary atresia is still highly considered. Liver gross appearance; size, color, surface and consistency was evaluated. The gallbladder and the extrahepatic ducts were also evaluated for size, fibrosis and connections. Intraoperative cholangiogram was performed through the fundus of the GB by inserting a purse string and then a cholangio catheter to depict the bile ducts and to evaluate the passage of the dye through them.


   Results Top


Cholangiogram was performed in 58 patients. The diagnosis of BH was concluded when the bile ducts appeared small and fail to demonstrate complete patency and when sections of wedge biopsy showed disturbed hepatic architecture, bile ducts proliferation and fibrosis. Five of the seven pateints with BH were classified as the syndromic type whereas the other two were arteriohepatic dysplasia type.


   Hepatobiliary scintigraphy Top


All 34 patients with BA showed prompt and homogenous hepatic uptake and no bowel activity throughout the study which lasted between 20 and 24 hours [Figure - 1]. Only three patients resulted with different diagnoses; two hepatits and one total parenteral nutrition. Distinctive scintigraphic features in the cases of biliary hypoplasia were visualization of the gallbladder (in 5/7 patients) between 6 and 12 hours after the injection, minimal activity in the bowel (in 5/7 patients), in addition to prompt and good hepatic uptake [Figure - 2]. In typical cases, the shape of the liver was different between the two entities.

The sensitivity rate of HBS for BH resulted 72% (5/7). For more informatin about the diagnostic accuracy of HBS in the investigation of neonatal hyperbilirubinemia, interested reader may be referred to EI-Desouki et al [7] .


   Ultrasonography Top


In patients with BA, the most common findings on ultrasound (32 of 34) were non-dilated bile ducts and visualization of a tiny, contracted gallbladder (29 of 34). A gallbladder was visualized in 38 of 43 patients with no BA.


   Discussion Top


Biliary hypoplasia (BH) is a lesion characterized by small intraheptic bile ducts (IBD) and patent extraheptic bile ducts (EBD) as demonstrated by explorative cholangiography [8],[9] . Diagnosis of BH on hepatobiliary scintigraphy (HBS) has admittedly not become popular for many reasons. First, HBS can not evaluate IBD accurately especially those of moderate to mild size or degree of hypoplasia. Second, the investigation of BH is the same as that of biliary atresia (BA). Explorative cholangiogram and percutaneous liver biopsy are part of the diagnostic work of these cases. Third, in the long run most cases of BH are complicated by liver cirrhosis. However, distinction between BH and BA is helpful to both the surgeon and the patient since the management for the two entities can be different.

Surgery may not improve BH or may even result in a rapidly deteriorating clinical course [10],[11] .

Conservative management has been recommended to a variant of BH known as arteriohepatic dysplasia characterized by paucity of the interlobular bile ducts and extrahepatic BH [12],[13] . Characterized by prompt radioactive tracer uptake by the liver, some degree of activity in the bowel and visualization of the gallbladder, BH can be distinguished by these scintigraphic findings from BA. In addition, the shape of the liver, where the left lobe appears enlarged in typical cases of BA, can be used as an adjunct feature in making the distinction [Figure - 1],[Figure - 2]. Visualization of the gallbladder, regardless of its size, is very useful to distinguish between BA and BH [Figure - 2]. These findings were consistently seen in five cases with BH. Our findings may lend support to the case report by Cynamon et al [13] .

When the remnants of the gallbladder and common bile duct were not patent, the HBS showed no activity in the bowel. By the age of one year and when the EBC developed further showing patent lumen, the HBS demonstrated prompt excretion of the tracer into the duodenum. In that report the findings on HBS reflected exactly what the real status of the liver architecture was as shown by the histopathology and the explorative cholangiography.


   Conclusion Top


Biliary hypoplasia can be recognized by hepatobiliary scintigraphy and be distinguished from biliary atresia. Being a noninvasive and quite simple diagnositc test, hepatobiliary scintigraphy can be used as part of the work up plan to investigate this disease entity and to follow it up as the biliary system continue to evolve.

 
   References Top

1.Houwen RH, Bax NM. Anatomic anomalies in neonatal cholestatic jaundice. Tijdschr-Kindergeneeskd 1993;61:151-7.  Back to cited text no. 1  [PUBMED]  
2.Silverman A, Roy C. Biliary hypoplasia. ln:Pediatric clinical gastroenterology. 3rd Eds. St. Louis:Mosby 1983;526-38.  Back to cited text no. 2    
3.Lilly JR, Hall RJ, Vasquez-Estevez-J, et al. The surgery of "correctable" biliary atresia. J Pediatr Surg 1987;22:522-5.  Back to cited text no. 3    
4.Kirks DR, Coleman RE, Filston HC, et al. An imaging approach to persistent neonatal jaundice. AJR 1984;142:461-5.  Back to cited text no. 4  [PUBMED]  
5.Maid M, Reba RC, Altman RP. Hepatobiliary scintigraphy with 99mTc-PIPIDA in the evaluation of neonatal jaundice. Pediatrics 1981;67:140-5.  Back to cited text no. 5    
6.Hitch DC, Leonard JC, Pysher TJ, et al. Differentiation of cholestatic jaundice in infants:Utility of diethyl-IDA. Am J Surg 1981;42:671-7.  Back to cited text no. 6    
7.El-Desouki M, Mohamadiyeh M, Al-Rabeah A, et al. Hepatobiliary scintigraphy in persistent direct hyperbilirubinemia in the neonate. Saudi Medical Journal 1997;18:305-8.  Back to cited text no. 7    
8.Garel LA, Belli D, Grignon A, Roy CC. Percutaneous cholecystography in children. Radiology 1987;165:639-41.  Back to cited text no. 8  [PUBMED]  
9.Schwartz MZ. An alternate method for intraoperative cholangiography in infants with severe obstructive jaundice. J Pediatr Surg 1985;20:440-2.  Back to cited text no. 9  [PUBMED]  [FULLTEXT]
10.Markowitz J, Daum F, Kahn El, et al. Arteriohepatic dysplasia. I. Pitfalls in diagnosis and management. Hepatology 1983;3:74-6.  Back to cited text no. 10    
11.Kocoshis SA, Cottrill CM, O'Connor WN, et al. Congenital heart disease, butterfly vertebra and extrahepatic bilairy atresia; a variant of arteriohepatic dysplasia ? J Pediatr 1981;99:436-9.  Back to cited text no. 11  [PUBMED]  
12.Alagille D, Odievre M, Gautier M, et al. Hepatic ductular hypoplasia associated with characteristic facies. vertebra malformation, retarded physical, mental and sexual development, and cardiac murmur. J Pediatr 1975;86:63-71.  Back to cited text no. 12    
13.Cynamon HA, Powell GK, Isenberg JN, Lobe TE. Support for a conservative approach to mixed intrahepatic and extrahepatic biliary hypoplasia. J Pediatr Surg 1987;22:1031-2.  Back to cited text no. 13  [PUBMED]  [FULLTEXT]

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Correspondence Address:
Mahmoud El-Desouki
Department of Medicine, College of Medicine and King Khalid University Hospital, King Saud University, P.O. Box 7805 (46), Riyadh 11742
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


PMID: 19864779

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