Biliary atresia

Biliary Atresia.

1. SURGICAL CASE PRESENTATION ON BILIARY ATRESIA

2. Biliary atresia is characterized by obliteration or discontinuity of the extra hepatic biliary system, resulting in obstruction to bile flow. OR B iliary atresia is a condition in which the normal extrahepatic biliary system is disrupted. Progressive damage of extrahepatic & intrahepatic bile ducts secondary to inflammation may occur, leading to fibrosis, biliary cirrhosis & eventual liver failure. DEFINITION

3. EMBRYOLOGY The biliary system originates from the hepatic diverticulum of the foregut at 4the week of gestation. Beginning in the fourth week of gestation, the liver bud arises from the distal extent of the foregut. As the liver parenchyma develops, the cells between it and the foregut proliferate, forming the precursor to the bile duct. Between the fourth and fifth weeks of gestation, the gallbladder primordium buds off the caudal extent of the bile duct giving rise to the gallbladder and cystic duct. This bud lies in close proximity to the ventral pancreatic bud. The shared stalk rotates posteriorly and medially to join the dorsal pancreatic bud .

4. The ventral pancreatic bud gives rise to the uncinate process; its duct, the duct of Wirsung , typically joins with the common bile duct (CBD) The extrahepatic biliary tree develops in close concert with the hepatic artery. Further details of the development of the extrahepatic biliary tract remain nebulous. It was initially thought that the biliary tract lumen passed through a phase in which the lumen was obliterated by proliferating endothelial cells, and failure to recanalize resulted in biliary atresia in neonates.

5. 1:10,000 live births. International : highest in Asian populations, and it may be more common in Chinese infants compared with Japanese infants . Sex: more common in females than in males . Age: Biliary atresia is a disorder unique to the neonatal period. The fetal /perinatal form is evident within the first 2 weeks of life; the postnatal type presents in infants aged 2-8 weeks. E P I D E M I O L G Y

6. Unknown. Reovirus 3 infection or rotavirus infection. Some  chromosomal  abnormality trisomy 17, 18 and 21; as well as the presence of anomalous organs in 30% of cases of biliary atresia. congenital malformation ( believed to be caused by an inflammatory process that obliterates the bile duct system) may begin utero or postnataly . CMV infection. Autoimmunity. ETIOLOGY

7. CLINICAL FORMS FETAL EMBRYONIC OR SYNDROMIC PERINATAL / ACQUIRED

8. KASAI CLASSIFICATION SYSTEM Is widely used & divides cases of biliary atresia according to their location & degree of pathology.

9. Is seen 10 to 20% of biliary atresia cases. CARDIAC MALFORMATION POLYSPLENIA SITUS INVERSUS MALROTATION ABSENT OF VENACAVA BILOBED LUNGS PREDUODENAL PORTAL VEIN INTESTINAL ATRESIAS ASSOCIATED ANOMALIES

10. PREDUODENAL PORTAL VEIN POLYSPLENIA

11. MALROTATION INTESTINAL OBSTRUCTION

12. CLINICAL PRESENTATION THE SYMPTOMS ARE SEEN USUALLY BETWEEN 1 TO 6 WEEKS OF LIFE. BOOK PICTURE SEEN IN PATIENT Conjugated jaundice which is prolonged. Present Dark Urine because of bilirubin in urine. Present Alcoholic /Clay- coloured stools because of absence of stercobilin . Present Vit k deficiency/ coagulopathy Cirrhosis (ascites & hepatospleenomegaly ) Present In advance cases, hepatic failure, bleeding disorders, esophageal varices , geberalized cachexia due to nutritional failure

13. CHILD WITH BILIARY ATRESIA

14. Intraoperative Cholangiogram ( before 8 week of age): Injection of the bile ducts with contrast in the operating room to watch for drainage into intestines Biliary USG. LFT Liver biopsy. Triangular cord sign: presence of fibrous cone of bile duct remnant at porta hepatis . Hepatobiliary scintigraphy (HIDA Scan) : Checks for excretion of bile from liver into the intestines. INVESTIGATION

15. COMPLETE BLOOD COUNT TEST OBSERVED VALUE NORMAL RANGE Hemoglobin 9.3mg/dl 11-13gm/dl R.B.C. Count 3.67 4-5.2mili/ cumm PCV 26.2 35-45% MCV 71.39 80.0-99.0fL MCH 25.34 27.0-33.0pg Total WBC count 15100 4500-11000/ cmm Neutrophils 58 40-75% Lymphocytes 37 20-45% Eosinophils 02 0-6% Monocytes 03 0-10% Platelet count 326 150,000 to 450,000 / ml REMARK: Microcytic Hypochromic Anaemia cons. With Iron deficiency. Leucocytosis.

16. TEST 28/1 30/1 31/1 1/2 Hemoglobin 8.4 7.2 9.4 9.6 R.B.C. Count 2.80 PCV 28.1 MCV 80.7 81.6 MCH Total WBC count 16000 8300 Neutrophils Lymphocytes 56 32 34 Eosinophils 02 Polymorphs 42 68 65 Platelet count 2.8 lac/mm3 2.6 lac/mm3

17. USG OF ABDOMEN & PELVIS : 31/12 Mild hepatomegaly Mild spenomegaly Umbilllical hernia is seen with bowel loops as content. Gall bladder not visualized. 22/1 IMPRESSION: Biliary atresia with liver cirrhosis with heterogenous liver echotexture with splenomegaly s/o portal hypertension

18. BIOCHEMISTRY LAB REPORT (27/12) PARAMETER RESULTS NORMAL RANGE PARAMETER RESULTS NORMAL RANGE Calcium 7.5 9.0-11.0 mg% Glucose (F/R) phosphorus 3.6 2.5-7.0 mg% Glucose (P/P) Alk . phosphotase 361.0 50-370 U/L T.Billirubin 13.91 Upto 1.0mg% Urea nitrogen 14.1 10-20 mg/dl D. BILLIRUBIN 8.26 Upto 0.5 mg% creatinine 0.20 0.2-1.0 mg/dl SGOT (AST) 129.0 Upto 40.0U/L Total proteine 6.4 6.0- 7 .5 gm % SGPT (ALT) 101.0 Upto 40.0U/L albumin 2.9 3.0 – 5.0 gm % Sodium 126.0 132 – 144 mEq /L Uric acid 3.0 2.5 -7.0 mg% Potassium 4.7 3.6 - 4.8 mEq /L chloride 110.0 96-105mEq/L cholesterol 43.0 Less than 170mg/dl

19. PARAMETER 28/1 PARAMETER Calcium 10.6 Glucose (F/R) phosphorus 3.3 Glucose (P/P) Alk . phosphotase 494.0 T.Billirubin 11.09 (high) Urea nitrogen 15.2 D. BILLIRUBIN 6.55 (high) creatinine 0.24 SGOT (AST) 229.0 (high) Total proteine 6.3 SGPT (ALT) 131.0 (high) albumin 3.4 Sodium 130.0 Uric acid 2.1 Potassium 4.1 chloride 109.0 cholesterol 185.0

20. ABG ANALYSIS PARAMETER RESULTS pH 7.339 pCO2 38.5mmHg pO2 39.7mmhg Na+ 150.1 mmoL K+ 2.75 mmol /L Cl 111.3 mmoL /l Ca + 0.77 mmoL /l Glu Lac 6.4 mmol / L HCT 22.3 HCO3 20.2

21. MANAGEMENT

22. No primary medical treatment is relevant in the management of extrahepatic biliary atresia. The pediatrician's objective is to confirm the diagnosis . Symptomatic treatment with Vit k, Vit D, Antihistaminics , antibiotics, diuretics. Once biliary atresia is suspected, surgical intervention is the only mechanism available for a definitive diagnosis (intraoperative cholangiogram ) and therapy (Kasai portoenterostomy ).

23. SURGICAL MANAGEMENT Kasai Hepatic P ortoenterostomy Blocked bile ducts are removed and a piece of small intestine is connected to the liver, to allow direct flow of bile from the liver . Best outcome if performed before 45 days of life. If performed after 3 months of age, only 25% of infants will have successful drainage of Bile.

24. LIVER TRANSPLANTATION: Up to 80% of patients ultimately require a liver transplant due to progression and complications of end stage liver disease, such as GI bleeding, ascites (increased abdominal fluid), worsening jaundice, and poor growth. Only 25% of patients with a Kasai procedure will survive into their 20s without requiring a liver transplant. Long-term survival after liver transplantation is over 90%, similar to children receiving a liver transplant for other reasons.

25. PATIENT PROCEDURE: EXPLORATORY LAPAROTOMY WITH INTRAOPERATIVE CHOLENGIOGRAM LIVER BIOPSY WITH KASAI PARTOENTEROSTOMY WITH UMBILLICAL HERNIA REPAIR.

26. PATIENT ORDERS : NBM RTA 2hrly Replace RL QID TPR/IO CHARTING AG CHARTING 4HRLY CPT with Neb NS+Asthalin QID Neb NS+Ipravent QID Neb NS+Mucomix QID Inj. Piperacillin tozabactum 700mg-TDS Inj. Amikacin 35mg TDS Inj.Pan 7mg OD INJ. Tramadol 7mg +NS TDS IV Albumin 14ml BD IV FLUIDS: 310ML DNS+3 ml KCL +1ml MVI 8am-8pm 310ML DNS+3 ml KCL +1ml MVI 8pm-8am

27. FAILURE TO THRIVE. Without treatment, infants with biliary atresia would develop cirrhosis within 6 months and liver failure within 1 year. MALNUTRITION PORTAL HYPERTENSION LIVER FAILURE COMPLICATIONS

28. Survival rates Overall survival with a native liver (not transplanted) ranges from 30-55 percent at 5 years of age; 30-40 percent at 10 years of age. It is thought that approximately 80 percent of patients with  biliary atresia  will require liver transplantation by the age of 20.

29. Nursing Diagnosis for Biliary  Atresia Hyperthermia  related to inflammatory damage due to progressive extrahepatic biliary duct . Acute pain related to postoperative procedure. Ineffective breathing pattern related to an increase in abdominal  distension. Imbalanced Nutrition: Less Than Body Requirements related to anorexia and impaired absorption of fat. characterized by weight loss and conjunctival pallor .  Impaired skin integrity related to accumulation of bile salts in the network. characterized by pruritis .  Deficient fluid volume related to nausea and vomiting. Anxiety  related to lack of information about the disease due to lack of knowledge .

30. Biliary Atresia – Clinical Series Bárbara Netoa ;Mariana Borges-Dias (2017) Biliary atresia is the main cause of death by hepatic failure and the main indication for liver transplant in children. This study aims to analyze the population with this diagnosis, treated between 2000 and 2015 at Hospital de São João . Material and Methods: Descriptive, observational, and retrospective study, including the patients with biliary atresia, diagnosed and treated between January 1, 2000 and December 31, 2015. We analyzed epidemiologic, clinical, biochemical, and image data, as well as registered complications and present status. Results: Eighteen patients were evaluated. The median age at time of Kasai portoenterostomy was 63 days of life, with better prognosis for those patients who had surgery before 72 days. The procedure was successful in 2/3 of cases. There was a significant association between recurrent cholangitis and survival. Five cases of transplant and 2 deaths, one of them after transplant, were registered. Survival with native liver was 77.8%, 72.2%, and 64.2% at 1, 5, and 10 years of follow-up, respectively.

31. Seyed Mohsen Dehghani conducted study on Long-term Outcome of Kasai Operation on Children with Biliary Atresia between 2001-2010 in Namazi Hospital, Shiraz, Iran. : A database of medical records of 61 patients (F=41, M=20) with biliary atresia, aged between 30 to 100 days at the time of operation, who had undergone the Kasai operation in Shiraz Namazi Hospital from 2001 to 2010 was examined in a retrospective review. Risk factors of failure (death and liver transplantation) were analyzed by Kaplan-Meier test. Results: Age at the operation time, type of atresia, and dilatation of the bile ducts were identified as the risk factors; 29 patients had signs of hepatomegaly, 6 patients had liver transplantation. Among them, 3 survived . Conclusion : Of the patients, 61% with biliary atresia survived for more than 5 years with their native liver while the overall survival rate among 52 patients was 85.30±5.96 months. Postoperative care and regular checkups should be considered for all survivors as a lifelong procedure to prevent any possibilities of future hepatic deterioration