A Study of Histopathological Profile of Liver in Paediatric Choledochal Cysts
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Jebmh.com Original Research Article A Study of Histopathological Profile of Liver in Paediatric Choledochal Cysts G.J. Vani Padmaja1, Othuluru Radhika Krishna2, Seereen Siddiqa3, Mandakini Kotaiah Srinivas Reddy4, Kota Ramesh Reddy5 1Department of Pathology, Niloufer Institute of Paediatrics, Red Hills, Hyderabad, Telangana, India. 2, 3 Department of Pathology, Osmania Medical College, Koti, Hyderabad, Telangana, India. 4, 5 Department of Paediatric Surgery, Niloufer Institute of Paediatrics, Red Hills, Hyderabad, Telangana, India. ABSTRACT BACKGROUND Choledochal cysts are rare congenital dilatations of the biliary tract with an Corresponding Author: Dr. Othuluru Radhika Krishna, incidence of approximately 1 in 1000 live births. Most cases present with Osmania Medical College, jaundice or other obstructive symptoms and are treated by surgical resection of Koti, Hyderabad, Telangana, the cyst and re-anastomosis. A liver biopsy at the time of surgery is mandatory Hyderabad, India. to study the histological changes including fibrosis and to grade these changes to E-mail: [email protected] determine prognosis. We wanted to study the histology of liver biopsies received along with choledochal cysts, grade the hepatocellular damage and fibrosis, and DOI: 10.18410/jebmh/2020/623 correlate these findings with clinical presentation of the patients. How to Cite This Article: Padmaja GJV, Krishna OR, Siddiqa S, et METHODS al. A study of histopathological profile of 40 liver biopsies received along with choledochal cysts in paediatric patients liver in paediatric choledochal cysts. J were assessed for histological changes using 7 parameters including liver fibrosis Evid Based Med Healthc 2020; 7(50), and scored semi-quantitatively according to established scoring systems. These 3055-3060. DOI: scores were correlated with the clinical data, age at surgery, sex of the patient, 10.18410/jebmh/2020/623 type of choledochal cyst, clinical features, and the results were analysed for their role in predicting patient prognosis. Submission 02-09-2020, Peer Review 11-09-2020, Acceptance 31-10-2020, RESULTS Published 14-12-2020. We found that all the cases showed histological variations in the liver biopsy. Age of children showed an inverse relationship with histological scoring. Infants Copyright © 2020 G.J. Vani Padmaja showed higher scores in comparison to older children. Fibrosis of the liver was et al. This is an open access article distributed under Creative Commons also higher in Infants. Most of the cases presented with clinical jaundice. Patients Attribution License [Attribution 4.0 with jaundice, pain abdomen, fever and acholic stools showed higher scores. We International (CC BY 4.0)] found that the sex of the patient and type of choledochal cysts were independent of the scores. CONCLUSIONS Younger age and more severe clinical presentation warrant an urgent surgical intervention in paediatric choledochal cyst. Histological assessment of the liver is mandatory to grade the hepatocellular changes including fibrosis and suggest prognosis and follow up of patients. KEYWORDS Liver Histology, Grading, Paediatric Choledochal Cyst J Evid Based Med Healthc, pISSN -2349-2562, eISSN - 2349-2570 / Vol. 7 / Issue 50 / Dec. 14, 2020 Page 3055 Jebmh.com Original Research Article BACKGROUND Haematoxylin and Eosin and the tissue sections were examined microscopically. Detailed histopathological Choledochal cyst is a cystic or fusiform dilatation of the examination of choledochal cysts and liver biopsies was biliary tree, intrahepatic or extrahepatic or both1. performed. Choledochal Cysts (CCs) are rare medical conditions with The gross findings of the choledochal cysts were an incidence of 1 in 100,000 to 1 in 150,000 individuals in documented along with a histopathological examination of Western countries to 1 in 13,000 individuals in Japan. High the H & E slides. All choledochal cysts were evaluated for prevalence of choledochal cysts is seen in in East Asia. First the following histological findings, simple columnar case was reported by Douglas in 1852.2,3 In 1959, Alonso- epithelium, hyperplasia, metaplasia, dysplastic or neoplastic Lej et al reported 94 cases of choledochal cyst disease and changes in the epithelial lining, mural ulceration, mural suggested the initial three classes. This classification system fibrosis, acute and chronic inflammation. has been modified by Todani et al to include two additional Liver biopsies were graded for specific histological groups of patients, those with combined intrahepatic and changes and fibrosis in a semi-quantitative manner and extrahepatic disease, and those with isolated intrahepatic cases were segregated based on the score. Each disease, Caroli’s disease.4 The aetiology of congenital parameter was separately scored according to the changes 7 dilatation of Common Bile Duct (CBD) in choledochal cysts observed. Fibrosis was assessed separately according to 8 is not well defined. Some studies indicate congenital causes Okhuma, classification. The six histological parameters while others acquired. Most widely accepted explanation is considered were, Cholestasis (CHS), Hepato-Cellular that it may be related to chronic reflux of the digestive and Damage (HCD), Parenchymal Inflammation (PI), Bile Duct caustic pancreatic juice into the bile duct secondary to an Proliferation (BDP), Bile Duct Inflammation (BDI), and abnormal connection between pancreatic duct (Wirsung’s Central Venous Distention (CVD), were graded as grades 0 duct) and the CBD.5 Congenital biliary dilatation is to 3, lowest score being 0, indicating absence of the associated with pancreaticobiliary ductal malunion, distal feature and highest score being 3 [Table 1] CBD stenosis, intrahepatic duct dilatation and abnormal Ohkuma’s classification was used to evaluate the 7 hepatic histology.2,6 Histological changes in the liver in a fibrotic changes in the liver. Fibrosis was graded as 0 to 4, case of choledochal cyst are seldom reported and a liver the lowest score being 0 indicating no fibrosis and the biopsy not performed. Liver changes and hepatic fibrosis highest score being 4 indicating cirrhosis of the liver. We associated with choledochal cyst have considerable chose Ohkuma classification for this study, due its wide use association with patient characteristics and also determine in all the research related to choledochal cyst associated prognosis of patients. liver histology. The score obtained for the histological In this study we have used microscopic parameters to parameters was correlated with clinical data which included assess and grade the histological changes of the liver age, sex, type of the choledochal cyst and symptoms at including fibrosis in a semi quantitative manner and admission. Due to the small number of cases in each group correlated the scoring with clinical characteristics, type of no statistical correlation was done. choledochal cyst, the age of patients and clinical presentation. RESULTS METHODS The total number of cases included in this study was 40 and age range of patients was 21 days to 12 years. 12 cases (30 %) belonged to the infantile group (age 1 year A cross sectional study of 5 years duration was taken up at or less than a year), 28 cases (70 %) belonged to classic a tertiary paediatric referral hospital, from September 2014 paediatric group (more than 1 year).13 cases (32.5 %) to August 2019 after obtaining ethical clearance from the were seen in age group of 1 - 5 yr. most common age Institutional Ethics Committee. All the histological group was more than 5 years (37.5 %). 15 cases (37.5 %) specimens of choledochal cyst along with simultaneous were male patients, and 25 cases (62.5 %) were female, liver biopsy received at the Department of Pathology were Male to female ratio being 1:1.6. included in the study. Detailed recording of clinical history The commonest clinical presentation of choledochal and radiological findings were done in every case. All cases cyst was jaundice, seen in 29 cases (72.5 %). Vomiting underwent preoperative abdominal ultrasound. Contrast- was seen in 25 cases (62.5.5 %), fever in 18 cases (45 %), Enhanced Computed Tomography (CECT) of abdomen was pain abdomen in 17 cases (42.5 %), abdominal lump in 12 done to confirm the diagnosis and to classify the cyst into cases (30 %), and only 8 [20 %] in the study patients one of the subtypes according to Todanis classification. All presented with pale stools (20 %) The classical triad of cases later underwent cystectomy with Roux-en-Y pain, lump and jaundice was seen in only 6 cases (15 %). hepaticojejunostomy or hepaticoduodenostomy. Most of the infants presented with jaundice and Gross examination of the specimens was done and vomiting while older children presented most commonly representative sections (2 - 3 per case) were submitted. with pain abdomen. On CT (Computed Tomography)-Scan Specimens were fixed in 10 % buffered formalin and abdomen, all cases showed dilated cystic lesion processed by automatic tissue processor. Routine paraffin communicating with the bile duct and separate from the embedding was done and 4µ sections were stained with gall bladder. CT scan showed normal liver in 26 cases (65 J Evid Based Med Healthc, pISSN -2349-2562, eISSN - 2349-2570 / Vol. 7 / Issue 50 / Dec. 14, 2020 Page 3056 Jebmh.com Original Research Article %), altered echotexture in 7 (17.5 %) cases and Correlation of Histological Findings with hepatomegaly in 7 cases (17.5 %). Clinical Data In 23 cases (57 %), Roux en Y hepaticojejunostomy We correlated liver histology with the age of the patient at was performed and 17 cases (43 %) were managed by the time of surgery. hepaticoduodenostomy. 31 cases had type I choledochal cyst and 9 cases had type IV. Most of the cases belonged Age CHS HCD PI BDP BDI CVD PF 1 2 3 1 2 3 1 2 3 1 2 3 1 2 3 1 2 3 1 2 3 4 to type I. The specimen received in all the cases was an Infants 3 5 4 0 7 6 2 6 4 3 6 3 4 5 3 4 6 2 1 2 5 4 already cut open specimen of the choledochal cyst with gall (10) 1 – S yrs.