Conduit decompression in biliary atresia

Stellin, G; Uceda, Jorge E.; Lilly, John R.

doi:10.1016/s0022-3468(83)80023-2

“…These include stomas or catheters in the proximal limb of the Roux loop,[5455] use of an omental wrap applied to the porta hepatis,[56] jejunal loop "valves",[57] creation of an intussuscepted ileocecal conduit[58] and use of an appendiceal conduit based on its vascular pedicle. [44] Roux loop stomas are associated with problems such as prolapse, retraction, fluid and electrolyte loss, bleeding and malabsorption and have been largely abandoned.…”

Section: Complicationsmentioning

confidence: 99%

Biliary atresia

Sinha

¹

,

Davenport

²

2008

J Indian Assoc Pediatr Surg

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Biliary atresia (BA) is a cholangiodestructive disease affecting biliary tract, which ultimately leads to cirrhosis, liver failure and death if not treated. The incidence is higher in Asian countries than in Europe. Up to 10% of cases have other congenital anomalies, such as polysplenia, asplenia, situs inversus, absence of inferior vena cava and pre-duodenal portal vein, for which we have coined the term Biliary Atresia Splenic Malformation (BASM) syndrome. For these infants the aetiology lies within the first trimester of gestation. For others affected with BA, aetiology is more obscure and perinatal destruction of fully-formed ducts perhaps by the action of hepatotropic viruses has been suggested. Whatever the cause, the lumen of the extrahepatic duct is obliterated at a variable level and this forms the basis for the commonest classification (Types I, II, III). All patients with BA present with varying degree of conjugated jaundice, pale non-pigmented stools and dark urine. Key diagnostic tests include ultrasonography, biochemical liver function tests, viral serology, and (in our centre) a percutaneous liver biopsy. In some centres, duodenal intubation and measurement of intralumenal bile is the norm. Currently BA is being managed in two stages. The first stage involves the Kasai operation, which essentially excises all extrahepatic biliary remnants leaving a transected portal plate, followed by biliary reconstruction using a Roux loop onto that plate as a portoenterostomy. If bile flow is not restored by Kasai procedure or life-threatening complications of cirrhosis ensue then consideration should be given to liver transplantation as a second stage. The outcome following the Kasai operation can be assessed in two ways: clearance of jaundice to normal values and the proportion who survive with their native liver. Clearance of jaundice (<2 mg/dL or <34 µmol/L) after Kasai has been reported to be around 60%, whereas five years survival with native liver ranges from 40% to 65%.

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“…Several patients with absence of the common hepatic duct have been described. [2355][2356][2357] They presented with obstructive jaundice in very early infancy and at operation were found to have no proximal biliary tree. There were no ductal structures in the hepatic hilum.…”

Section: Agenesis Of Common Hepatic Ductmentioning

confidence: 99%

Developmental and Inherited Liver Disease

Quaglia¹,

Roberts²,

Torbenson³

2018

Macsween's Pathology of the Liver

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Diagnostic approach to histology 113 Neonatal and early infantile liver disease 113 Postneonatal, childhood and adulthood presentation 118 Neonatal hepatitis 119 Histopathological features 120 Biliary atresia 121 Classification and aetiopathogenesis 121 Pathological features at surgical intervention 124 Pathology of intrahepatic changes 126 Paucity of intrahepatic bile ducts 129 Alagille syndrome (arteriohepatic dysplasia) 129 Nonsyndromic duct paucity disorders 131 Bile duct anomalies, congenital dilations and ductal plate malformation disorders 131 Choledochal cyst 132 Hereditary fibropolycystic disease (ductal plate malformation) 133 Cystic fibrosis 141 Hereditary disorders of bile acid synthesis and bilirubin metabolism 143 Primary disorders of bile acid synthesis 143 Disorders of bile canalicular transporters 146 Other diseases causing intrahepatic cholestasis 150 Hereditary defects of bilirubin metabolism 151 Disorders of porphyrin metabolism 153 Porphyria cutanea tarda 154 Erythropoietic protoporphyria 155 Disorders of carbohydrate metabolism and related conditions 156 Glycogen storage diseases (glycogenoses) 156 Myoclonus epilepsy, Lafora type (Lafora disease) 161 Galactosaemia Hereditary fructose intolerance Disorders of glycoprotein and glycolipid metabolism Mucopolysaccharidoses Aspartylglucosaminuria α-Mannosidosis Fucosidosis Mucolipidoses Congenital disorders of glycosylation (carbohydrate-deficient glycoprotein syndrome) Endoplasmic reticulum storage diseases α1-Antitrypsin deficiency α1-Antichymotrypsin deficiency Afibrinogenaemia and hypofibrinogenaemia Antithrombin III deficiency Disorders of amino acid metabolism Hereditary tyrosinaemia type 1 Congenital hyperammonaemia syndromes and urea cycle disorders Cystinosis Homocystinuria (cystathionine β-synthase deficiency) Disorders of lipoprotein and lipid metabolism Abetalipoproteinaemia Familial hypobetalipoproteinaemia Familial high-density lipoprotein deficiency (Tangier disease) Familial hypercholesterolaemia Wolman disease and cholesteryl ester storage disease Gangliosidoses α-Galactosidase A deficiency (Fabry disease) Sulphatide lipidosis (metachromatic leucodystrophy) Chapter 3 Developmental and Inherited Liver Disease

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“…Historically, various measures have been tried with to reduce cholangitis although none have stood the test of time. These include stomas or catheters in the proximal limb of the Roux loop, [54,55] use of an omental wrap applied to the porta hepatis, [56] jejunal loop "valves", [57] creation of an intussuscepted ileocecal conduit [58] and use of an appendiceal conduit based on its vascular pedicle. [44] Roux loop stomas are associated with problems such as prolapse, retraction, fluid and electrolyte loss, bleeding and malabsorption and have been largely abandoned.…”

Section: Cholangitismentioning

confidence: 99%

Biliary atresia

Beattie¹,

Dhawan²,

Puntis³

2011

Oxford Medicine Online

0

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Biliary atresia (BA) is a cholangiodestructive disease affecting biliary tract, which ultimately leads to cirrhosis, liver failure and death if not treated. The incidence is higher in Asian countries than in Europe. Up to 10% of cases have other congenital anomalies, such as polysplenia, asplenia, situs inversus, absence of inferior vena cava and pre-duodenal portal vein, for which we have coined the term Biliary Atresia Splenic Malformation (BASM) syndrome. For these infants the aetiology lies within the first trimester of gestation. For others affected with BA, aetiology is more obscure and perinatal destruction of fully-formed ducts perhaps by the action of hepatotropic viruses has been suggested. Whatever the cause, the lumen of the extrahepatic duct is obliterated at a variable level and this forms the basis for the commonest classification (Types I, II, III). All patients with BA present with varying degree of conjugated jaundice, pale non-pigmented stools and dark urine. Key diagnostic tests include ultrasonography, biochemical liver function tests, viral serology, and (in our centre) a percutaneous liver biopsy. In some centres, duodenal intubation and measurement of intralumenal bile is the norm. Currently BA is being managed in two stages. The first stage involves the Kasai operation, which essentially excises all extrahepatic biliary remnants leaving a transected portal plate, followed by biliary reconstruction using a Roux loop onto that plate as a portoenterostomy. If bile flow is not restored by Kasai procedure or life-threatening complications of cirrhosis ensue then consideration should be given to liver transplantation as a second stage. The outcome following the Kasai operation can be assessed in two ways: clearance of jaundice to normal values and the proportion who survive with their native liver. Clearance of jaundice (<2 mg/dL or <34 µmol/L) after Kasai has been reported to be around 60%, whereas five years survival with native liver ranges from 40% to 65%. KEY WORDS: Biliary atresia, surgical jaundice Review ArticleFigure 2: Clearance of jaundice (<20 µmol/L) by age cohort and cumulatively for Isolated. Biliary atresia (n = 177) Figure 3: Two-year native liver survival by age cohort and cumulatively for Isolated. Biliary atresia (n = 177) Sinha and Davenport: Biliary atresia

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Conduit decompression in biliary atresia

Cited by 13 publications

References 1 publication

Biliary atresia

Biliary atresia

Developmental and Inherited Liver Disease

Biliary atresia

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