Saudi Journal of Gastroenterology
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CASE REPORT Table of Contents   
Year : 1996  |  Volume : 2  |  Issue : 3  |  Page : 156-159
Extrahepatic biliary atresia with hyaline cartilage and multiple congenital anomalies: A case report


1 Department of Pediatric Surgery, Medical Complex, Riyadh, Saudi Arabia
2 Department of Central Laboratory and Blood Bank, Medical Complex, Riyadh, Saudi Arabia

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Date of Submission20-Nov-1995
Date of Acceptance09-Mar-1996
 

How to cite this article:
Hassab MH, Baez-Giangreco A, Afzal M, Shadi SM, Al Olayet YF. Extrahepatic biliary atresia with hyaline cartilage and multiple congenital anomalies: A case report. Saudi J Gastroenterol 1996;2:156-9

How to cite this URL:
Hassab MH, Baez-Giangreco A, Afzal M, Shadi SM, Al Olayet YF. Extrahepatic biliary atresia with hyaline cartilage and multiple congenital anomalies: A case report. Saudi J Gastroenterol [serial online] 1996 [cited 2019 Aug 22];2:156-9. Available from: http://www.saudijgastro.com/text.asp?1996/2/3/156/34022


Congenital extrahepatic biliary atresia (EHBA) constitutes the most common hepatic surgical disorder of infancy. In the USA, it occurs with a frequency of 1/8000 to 1/20,000 live births and is higher in Orientals and females [1] . Controversy continues over the origin of this disorder, whether it is the end result of different causal and pathogenic mechanisms during the gestation or postnatal period [2] or if it is due to gene alteration since it has been reported in a few twins and in families [3] . A range of extrahepatic developmental anomalies are present in approximately 20% of the affected infants with EHBA [4] . Carmi et al [5] distinguished two and possibly three different patterns of anomalies suggestive of heterogeneity of EHBA with probable early prenatal onset. We report a case of biliary atresia associated with abdominal situs inversus, intestinal malrotation, unilateral agenesis of the left kidney and presence of ring of fragmented hyaline cartilage in the atretic biliary duct.


   Case Report Top


A five-month-old female infant was admitted to Maternity and Children's Hospital in Riyadh because of jaundice and hepatosplenomegaly. The baby was born to a 30-year-old Saudi mother. She was a sixth baby to the nonconsanguineous couple with no previous family history of malformations. The second male baby died on the third day of life with no detectable cause. The pregnancy was normal and delivery was at 39 weeks of gestation. Birth weight was 3.5 kg (50th percentile) and length was 51 cm (50th percentile) The head circumference was 35.5 cm (50th percentile). She was breast fed. Jaundice appeared in the third day of life. Clinical examination showed jaundice with mild abdominal distention and hepatosplenomegaly and no ascites. The lungs and the heart were essentially normal. Stool was typically clay colored. Laboratory studies revealed total serum bilirubin 561 umol/L. (Direct 340 umol/L and indirect 221 umol/L). SGOT 37 u/L, SGPT 97 u/L, Alkaline phosphatase 1028 u/L. Bile salts and bile pigments were detected in urine. Infection screen and hepatitis markers were negative. Ultrasound examination as well as radio­isotope study of liver suggested biliary atresia.

True-cut needle biopsy showed bile stasis with mild ductular proliferation. Laparotomy was planned for operative cholangiogram and corrective surgical procedure. On laparotomy large symmetrical bilobed brown firm liver was noted. What was suspected as a spleen, was found to be the left lobe of liver. Biliary tree was atrophic, hence cholangiography was not done. A picture of situs inverses was seen with the spleen located to the right side and of normal size and no multiplicity. Stomach was lying on right side and nonrotated, and the atretic gallbladder was located to the left side of the falciform ligament [Figure - 1],[Figure - 2]. Pancreas was lying between stomach and duodenum with a picture of nonrotation of midgut. The small intestine is grouping on the left side and colon was lying on the right side. No lymph nodes were noted in the porta hepatis. Hepatic artery was anterior and to the right side of the portal vein. The left kidney was absent while the right one appeared normal. Ovaries and  Fallopian tube More Detailss looked normal. Excision of gallbladder and the extrahepatic atretic biliary tissue was done and was associated with detectable whole bile flow from the porta hepatis. Hepatoportoenterostomy (Kasai one stage procedure) was done. Postoperatively improvement was observed in the form of decreasing jaundice and appearance of green-colored stool. Radioisotope study showed improved uptake in the liver, but still the main excretion was through the kidney.


   Pathologic Study Top


A firm cord of soft tissue measuring 1.8 x 1.0 x 0.8 cm was received. Cross sections showed no bile material. Microscopic examination of the small fragment of tissue revealed a hypoplastic gallbladder with no bile in the lumen and lined by columnar epithelium. The wall was made up by connective tissue mixed with smooth muscle [Figure - 3]. Sections from the fibrotic cord in the external biliary tract showed several clusters of narrowed ductular formation lined partially by cuboidal epithelium surrounded by hypoplastic glandular-like structures. In some areas, the lumen showed some acute inflammatory cell infiltration as well as chronic inflammation in the surrounding soft tissue. A few hyaline cartilaginous plates were observed around the lumens [Figure - 4]. No muscular coats were present. Some nerve structures, blood vessels, and fibrous tissues surrounded this structure. Special stain with Alcian Blue pH 2.5 showed no Alcian Blue positive material in the accessory glands.


   Discussion Top


Extrahepatic biliary atresia (EHBA) is characterized by complete or incomplete discontinuity of the major hepatic and or common bile ducts with the duodenum, resulting in persistent conjugated hyperbilirubinemia with subsequent fatal cirrhosis in childhood [6] . Etiologically there are probably at least two separate groups of EHBA, one with no associated anomalies presumed to be caused by an inflammatory process affecting at a late intrauterine or early neonatal period [7] , and a second group with associated nonhepatobiliary anomalies, for whom an early embryological insult is hypothesized [5] . Stowens [8] observed that a wide spectrum of simultaneous malformations (25% of his autopsy series of 515 cases) were present with EHBA. He also first pointed out the prolonged period of fetal growth and differentiation of the hepatobilairy system, therefore potentially vulnerable to teratogenic offense throughout the gestational period.

The biliary tract, gallbladder, and liver arise as a bud of cells, the hepatic diverticulum from the ventral wall of the foregut during the fourth week of human gestation. The parts cranialis differentiate into the liver, the caudal part of the hepatic diverticulum develops into the gallbladder and the common bile duct at the fifth week of gestation [9] . The patterns of these relative rare malformations such as situs inversus, cardiovascular malformation, polysplenia [10],[11] , intestinal malrotation associated with biliary atresia, make several authors look for a common cause for all of them, or for one malformation that may explain at least partially the reason of the other. Therefore, it was postulated that the insult, the nature of which is undetermined, may occur before the development of the biliary tract or before the fifth week of fetal development.

Carmi et al., [5] after analyzing segregation patterns of these anomalies suggested the existence of two major groups affecting the early onset of EHBA. One group represented 29.4% of the cases with combinations of anomalies with a recognizable pattern similar to those caused by loss of development control in early embryogenesis of symmetry determination or lateralization [12] such as situs inversus, cardiovascular malformation, intestinal malrotation, and splenic malformation and a second largest group, 58.8% of the cases with a nonrecognizable pattern of malformations with one or two anomalies mainly involving the cardiac, gastrointestinal, and urinary systems. The abnormalities observed in our case are more related with group one of malformation of Carmi. The associated malformations observed were abdominal situs inversus with symmetrical liver, right spleen, left atrophic gallbladder, malrotation of the intestine and agenesis of the left kidney.

An interesting finding in our patient was the presence of anomalous cartilaginous tissue around the atretic bile ducts. Since the gallbladder was markedly hypoplastic and the malformation in the bile duct showed microductular structures with some accessory glands beside the hyaline cartilaginous tissue, we wondered if this case represented a variant of bronchopulmonary foregut malformation associated with atresia of the bile duct and gallbladder [13] . However, no communication of the structure with the gastrointestinal tract was seen. Also the lesion was present in the portal area and not near the posterior abdominal wall. Furthermore, no ciliated epithelium, muscular coats or serious type of accessory gland as observed in the respiratory tract were seen with Alcian Blue pH 2.5 stain [14] .

The possibility of teratoma was excluded since no other tissue was seen. The absence of the left kidney, at the same side of the EHBA, brings up the possibility that this malformation could represent a dysplastic kidney remnant [15] . However, no other renal parenchymal element or smooth muscle was noted around the structure. The possibility that the cartilaginous tissue is the result of metaplastic change due to an inflammatory process is unlikely since the hyaline cartilages appear to be forming plates and encircling some ductular structure.

 
   References Top

1.Hendren WH, Vacanti JP. Pediatric liver disease. In: Mcdermott. Jr. WV (ed). Surgery of the liver. Blackwell Scientific Publications, Cambridge, MA 1989;525-31.  Back to cited text no. 1    
2.Chandra RS, Altman RP. Ductal remnants in extrahepatic biliary atresia. A histopathologic study with clinical correlation. J Pediatr 1978;93:196-200.  Back to cited text no. 2    
3.Sommer A, Moody PE, Riener CB. Familial extrahepatic biliary atresia. Clin Pediatr 1976;15:627-9.  Back to cited text no. 3    
4.Devenport M, Savage M, Mowat AP, Howard ER. Biliary atresia splenic malformation syndrome: An etiology and prognostic subgroup. Surgery 1993; 113:662-8.  Back to cited text no. 4    
5.Carmi R, Magee CA, Neill CA, Karrer FM. Extrahepatic biliary atresia and associated annomalies. Etiologic heterogeneity suggested by distinctive patterns of association. Am J Med Genet 1993;45:683-93.  Back to cited text no. 5    
6.Alagille D. Extrahepatic biliary atresia. Hepatology 1984;4(1 Suppl.):7S-l OS.  Back to cited text no. 6    
7.Blanc WA. In Brent RL. Persistent jaundice in infancy. J Pediatr 1962;61:111-5.  Back to cited text no. 7    
8.Stowens D. Congenital biliary atresia. Ann NY Acad. Sc 1963;111:337-51.  Back to cited text no. 8    
9.Desmet VJ. Embryology of the liver and intrahepatic biliary tract, and an overview of malformations of the bile duct. In: McIntyre N, Benhamon JP, Bircher J, Rizzeto M, Rodes J., ed. The Oxford textbook of clinical hepatology. Vol 1. Oxford: Oxford University Press 1991:497-519.  Back to cited text no. 9    
10.Chandra RS. Biliary atresia and their structural anomalies in the congenital polysplenia syndrome. J Pediatr 1993:196-200.  Back to cited text no. 10    
11.Silveira TS, Salzano FM, Howard ER, Mowat AP. Congenital structural abnormalities in biliary atresia: Evidence for etiopathogenic heterogeneity and therapeutic implications. Acta Pediatr Scand 1991;80:1 192-9.  Back to cited text no. 11    
12.Toriello HV, Kokx N, Higgins JV, Hofman R, Waterman DF. Sibs with the polyasplenia developmental field. Am J Med Genet 1986;Supp.2:31-6.  Back to cited text no. 12    
13.Gerle RD, Jaretzki A, Ashley CA, Berne AS. Congenital bronchopulmonary-foregut malformations: Pulomonary sequestration communicating with the gastrointestinal tract. N Engl J Med 1968;278:1413-9.  Back to cited text no. 13    
14.Keohane ME, Schwart 1, Freed J, Dische R. Subdiaphragmatic ronchogenic cyst with communication to the stomach: A Case Report. Hum Pathol 1988;19:868-71.  Back to cited text no. 14    
15.Hitchcock R, Burge DM. Renal agenesis: An acquired condition ? J Pediatr Surg 1994;29:454-6.  Back to cited text no. 15  [PUBMED]  [FULLTEXT]

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Correspondence Address:
Atilio Baez-Giangreco
P.O. Box 60179, Riyadh 11545
Saudi Arabia
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PMID: 19864825

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