Kulyk, Kurylo, Nykyforuk, and Hrytsak: Our Experience in Diagnosis and Treatment of Children with Biliary Atresia



Problem statement and analysis of the recent research

Atresia of bile ducts or biliary atresia is a congenital disease occurring with lesions of the bile ducts leading to the development of cholestasis and manifests in the neonatal period [5, 6]. This type of atresia occurs with a frequency of 1: 10,000 - 13,000 of live newborns and is the most common cause of liver transplantation in children [1, 3]. Biliary atresia leads to fatal result during the first year of life in case of late diagnosis and untimely surgical treatment [2]. This disease is necessary to be diagnosed and surgery to be conducted if possible (the Kasai operation – hepato-porto-enterostomy) under two months of age [4] allowing bile outflow from the liver to the intestine in 30-80% of cases.

The objectiveof the researchwas to analyze the results of examinations and treatment of 21 children with biliary atresia who were treated at the surgical department of conformational abnormalities in children in Lviv City Children’s Clinical Hospital since 2008 to 2015.

Materials and methods

Methods of the research included follow-up, laboratory ones, duodenal intubation, ultrasound, scintigraphy, MRI, diagnostic laparoscopy, liver paracentesis, determination of hepatitis B and C markers, DNA of CMV virus.

Results and Discussion

Follow-up studies detected that all children with biliary atresia were born full-term with normal anthropometric measurements corresponding to physiological norm. Jaundice manifested on 2nd-3rd day of life (similar to physiologic jaundice). “Light period” that is reduction of jaundice intensity till about the tenth day followed by gradual increase till the end of first month of life was noted in 14 children. After meconium discharge, acholic stool was observed being the earliest clinical criterion of the disease. Urine was intensely colored.

Sizes of the liver after birth were normal. Hepatomegaly and increase in its density developed during two months after birth in all children.

Weight defficiency was observed the severity of which depended on the type of feeding, spleen significantly increased, portal hypertension with ascites occurred worsening the general condition till the second month of life in all children.

Increase in bilirubin by conjugated bilirubin was the earliest sign of the disease during laboratory examinations. Total bilirubin constituted 170 - 450 mmol /l on average, conjugated bilirubin amounted 90 - 290 mmol / l. Increase in biochemical markers of cholestasis such as gamma-glutamyltransferase (GGT) (up to 885), alkaline phosphatase (ALP) (650), cholesterol (to 7.0), bile acids (FA), ß - lipoproteins, etc. was noted since second-third weeks. The highest concentration increased till 2nd month of life.

Hemorrhagic syndrome was observed in 12 children at the age of 1.5 months of life. It was associated with changes of coagulation parameters of blood system as a result of vitamin K malabsorption.

Cytolysis enzymes (ALT, AST) increased mainly after 1 month of life as a result of hepatocytes cytolysis. Indicators showing synthetic function (albumin, fibrinogen, etc.) changed primarily at the later stages of the disease.

Ultrasound detected absent or significantly reduced (in a triangular band) gallbladder in all children. It did not change its sizes during eating. We put nasoduodenal tube in distal part of duodenum using ultrasound for 24 hours with further researching of contents – bile was absent.

Absence of radioisotope substance income into intestine was noted during hepatobiliary scintigraphy on the background of its satisfactory accumulation in liver.

During MRI study visualization of the bile ducts was abscent, gallbladder had small sizes.

Changes of liver color and gallbladder sizes, an absence of bile ducts were noted during diagnostic laparoscopy.

Liver puncture with the following histomorphological study detected signs of cholestasis, bile pigment accumulation in the cytoplasm of hepatocytes, signs of progressive periportal fibrosis. Signs of cirrhosis developed individually since 1 to 6 months of life.

Markers of hepatitis B, C, DNA of CMV virus were studied in order to identify causes of neonatal cholestasis in all patients. DNA of CMV virus was found in 16 children (80%) in liver biopsy and in 5 children (25%) in blood.

Biliary atresia was diagnosed in 18 patients less than 2 months of age among all the examined children. All children were operated timely. 6 patients needed liver transplantation. Diagnosis was made in 3 children under the age of 3 months. The Kasai operation was conducted in 2 children. Liver transplantation was performed in three children (relatives were the donors of the recipient) in IP CZD (Warsaw) and Saint-Lus (Brussels) hospitals. These children with satisfactory quality of life are on permanent immunosuppressive therapy. They have signs of biliary cirrhosis and periodically undergo inpatient treatment of an ascending cholangitis.

Maintenance of normal nutritional (food) status, biliary tract patency and prevention of cholangitis and infections are the primary task in the course of postoperative treatment (The Kasai procedure). All somatic diseases of children should be treated under the strict control of biochemical indicators for their timely correction under the supervision of the surgeon and gastroenterologist.

Conclusions

Early diagnosis of biliary atresia and timely conducted surgical treatment (under 2 months of age) makes it possible to improve the prognosis, neurological status, quality of life and to prevent the necessity of liver transplantation at an early age.

References

1 

AV Dehtyareva, NV Kulikova, AY Razumkovskyy, et al. Age-specific time course of changes in the clinical and laboratory manifestations of biliary atresia in children. Rossiyskiy vestnik perinatologiyi i pediatriyi. 2013;58 (2):17-23.

2 

VA Savvina, AR Varfolomeev, VN Nikolaev, et al. Biliary therapy as the cause of neonatal cholestasis. Detskaya khirurgiya. 2013;4:25-28.

3 

A Giannattassio, F Cirillo, D Liccardo, et al. Diagnostic Role for biliary atresia. Radiology. 2008;247 (3):912-913. doi:10.1148/radiol.2473071715

4 

M Kasai. Treatment of biliary atresia with special reference to hepatic portoenterostomy and its modifications. Progr Pediatr Surg. 1974;6:5-52.

5 

Li Shi-Xing, Zhandg Yao, Sun Mei, et al. Ultrasonic diagnosis of biliary atresia: a retrospective analysis of 20 patients. World J Gastroenterol. 2008;14 (22):3579-3582. doi:10.3748/wjg.14.3579

6 

TM Humphrey, MD Stringer. Biliary atresia: US diagnosis. Radiology. 2007;244:845 – 851. doi:10.1148/radiol.2443061051



Copyright (c) 2017 O. Kulyk, H. Kurylo, O. Nykyforuk, D. Hrytsak

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