Portal Plate with Hyaline Cartilage in Biliary Atresia

Vij, Mukul; Bachina, Prashant; Safwan, Mohamed

doi:10.1007/s12098-015-1805-4

Cited by 3 publications

(2 citation statements)

References 4 publications

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“…However, another study of the extrahepatic biliary remnants of 205 cases of BA determined that the patterns of bile duct obliteration are not indicative of prognosis [69]. Occasionally, nodules of cartilage have been noted at the porta [73] and squamous metaplasia has been described.…”

Section: Morphological Findings In the Resected Extrahepatic Biliary Tree Remnantsmentioning

confidence: 99%

Biliary Atresia: pathology, Etiology and Pathogenesis

Vij¹,

Rela

2020

Future Sci. OA

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Biliary atresia is a progressive fibrosing obstructive cholangiopathy of the intrahepatic and extrahepatic biliary system, resulting in obstruction of bile flow and neonatal jaundice. Histopathological findings in liver biopsies include the expansion of the portal tracts, with edematous fibroplasia and bile ductular proliferation, with bile plugs in duct lumen. Lobular morphological features may include variable multinucleate giant cells, bilirubinostasis and hemopoiesis. The etiopathogenesis of biliary atresia is multifactorial and multiple pathomechanisms have been proposed. Experimental and clinical studies have suggested that viral infection initiates biliary epithelium destruction and release of antigens that trigger a Th1 immune response, which leads to further injury of the bile duct, resulting in inflammation and obstructive scarring of the biliary tree. It has also been postulated that biliary atresia is caused by a defect in the normal remodelling process. Genetic predisposition has also been proposed as a factor for the development of biliary atresia.

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Section: Morphological Findings In the Resected Extrahepatic Biliary Tree Remnantsmentioning

confidence: 99%

Biliary Atresia: pathology, Etiology and Pathogenesis

Vij¹,

Rela

2020

Future Sci. OA

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“…Altamirano and Drut [8] proposed that cartilage at the porta hepatis was due to alteration resulting from metaplasia of local connective tissue rather than true heterotopia as seen in their case of a 3-month-old infant (gender not specified). Vij et al [9] described a similar case in a 2-monthold infant (gender not specified) and agreed that the hyaline cartilage at porta hepatis was likely to represent defective morphogenesis. Gupta et al [10] reported hyaline cartilage and squamous epithelium within biliary ductules at the porta hepatis in a 65-day-old male child with no associated congenital anomalies or evidence of viral infection and hypothesised that these unusual findings could have occurred due to metaplastic response.…”

Section: Discussionmentioning

confidence: 98%

Hyaline cartilage at porta hepatis in extrahepatic biliary atresia: metaplasia or choristoma

Elhence

Sinha

2020

Sudan J Paed

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We report a case of a 5-month-old child who presented with the features of obstructive jaundice, anaemia and had no associated congenital anomalies. The child was suspected to have biliary atresia on hepatobiliary iminodiacetic acid (HIDA) scan and underwent Kasai portoenterostomy. Microscopic examination of the atretic gallbladder revealed islands of mature hyaline cartilage, lymphoid aggregates and fibrosis. There was an abrupt transition between the cartilage and the adjacent stroma. Only rare case reports have documented this finding with hypothesis ranging from metaplasia occurring secondary to the associated chronic inflammation or whether it is a choristoma. These cases need to be documented to elucidate the pathways of occurrence and may lead to have a better and detailed understanding of the disease process.

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