الفهرس 
INTRODUCTIONOnly 14 pages are availabe for public view
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Abstract
Biliary atresia is a uniquely pediatric liver disease characterized by an idiopathic, progressive, fibrosclerosing obliteration of large bile ducts. Delay in its diagnosis, with resultant late intervention and treatment, results in high morbidity and mortality.
While well-timed diagnosis is the most important prognostic factor for BA, early detection has been hampered by the lack of an effective screening strategy.
Multi detector computed tomography of the abdomen has an essential role in patients with BA; identifying associated congenital anomalies or cirrhosis-related complications. Moreover, MDCT can identify the cirrhotic liver hepatic masses, portal vein thrombosis, extra-hepaticporto systemic shunts, and other radiological signs of portal hypertension.
In our study we tried to evaluate the utility of MDCT in patients with BA. Forty nine patients with proven BA admitted or attending the outpatient clinic of Pediatric Hepatology, Gastroenterology and Nutrition Department, National Liver Institute, Menoufia University were enrolled.
The age of studied patients ranged from (50-2093) days and its mean was (375) days. The studied patients were divided into three groups; group 1(early BA) including 16 patients, group 2 (neglected BA) this group included 12 patients aged more than 3 months who did not undergo KPE and group 3 (post Kasai) includes 21 patients who underwent KPE.
Inclusion criteria were:
1- Patients fulfilling the criteria of BA according to the scoring system for BA. For those patients in whom liver biopsy was contraindicated; diagnosis was relied on exclusion of other possible etiologies of NC.
2- Patients with confirmed BA after intraoperative cholangiogram.
Exclusion criteria were:
1- Patients with severe decompensated liver disease (Child C 13-15).
2- Patients with systemic infection.
The entire study groups were subjected to the following: -
1- Full history taking and thorough clinical examination.
2- Liver function tests (liver enzymes, TB/DB, serum albumin, PT, PTT, and INR).
3- Complete blood count.
4- Calculation of APRI, PELD and CTP score.
5- Abdominal US.
6- Liver biopsy.
7- Upper GIT endoscopy.
8- MDCT abdomen.
Data were analyzed using the SPSS package for Windows, version 22.0 (SPSS Inc., Chicago, Illinois, USA) and the results were presented in tables and graphs.
Results showed that:
1. Females represented 53.1% of the studied patients.
2. Studied children had undergone KPE at a mean age of 68±18 days.
3. There was a statistical difference between the studied groups regarding their body weight.
4. Development was significantly affected in patients with neglected and post Kasai.
5. Neglected BA and post Kasai patients had higher CTP and PELD score (P = 0.013, 0.003).
6. There is intermediate correlation between CTP and PELD score; each increase in PELD by one unite the risk of patients to be at a higher CTP score is doubled (OR=2.21).
7. In non-operated patients with BA; clay stool was present in 96% meanwhile; jaundice was present in all of them.
8. Clinically, there was no statistical significance regarding hepatomegaly or ascites among studied groups but regarding splenomegaly there was statistically significant difference between studied groups.
9. Laboratory data revealed that, there was a noted statistically significant difference between the three groups concerning liver transaminases, total and direct bilirubin, coagulation profile and platelet count.
10. Ultrasound examination of patients at the initial time of diagnosis disclosed the following, sub capsular flow, TC sign and Rt hepatic artery hypertrophy were present in 57%, 53%, and 40% of patients with BA respectively. Splenomegaly was present in 65% of cases, 96% of patients with BA had
abnormal gall bladder; lastly cystic lesions in the liver were detected in four
patients (8%).
11. Cirrhotic changes were more evident in patients with advanced liver disease (neglected and post Kasai patients) (p= 0.009).
12. The most common histopathological findings in patients with BA were intracellular cholestasis, ductular proliferation, hepatocyte swelling and feathery degeneration, bile plugs and cholestatic rosette formation.
13. Liver fibrosis increases with increased disease severity.
14. APRI correlates with severity of fibrosis.
15. Upper GIT endoscopy revealed that, bile was not detected in all patients who were not operated.
16. By MDCT, gall bladder was normal in 3.5% of patients of BA.
17. There was significant statistical difference regarding the presence of ascites between studied patients, none of patients within group 1 had ascites but it was present in 30% of neglected and post-Kasai.
18. Associated congenital anomalies were detected in 26.5% of patients; the most common associated anomalies are hernia (either umbilical or inguinal) and splenic cyst.
19. Varices were present in 22% of BA patients; all of them were of the advanced liver disease groups (neglected and post Kasai). Hepatic nodules and biliary cysts are more associated with neglected and post-Kasai patients.
20. High specificity and NPV of MDCT in detection of gasroeosphageal varices (97% and 87%) respectively, it has a sensitivity of 50% and its PPV and accuracy were (83.3%- 86.7%) respectively.
21. There is no pre-eminence of MDCT to US in detecting congenital anomalies, splenomegaly, ascites, hepatic nodules, PVT, choledocal cyst or assessment of liver cirrhotic changes but MDCT can more accurately detect IHBDs dilation and Lymphadenopathy.
22. APRI has sensitivity of 60% and a specificity of 51.5% in detection of varices.
from the previous data, we concluded that, MDCT had no predominance over US. Furthermore, it has a low sensitivity (50%) in detection of esophageal varices. Performing MDCT should not be recommended as a diagnostic tool for BA. In addition, larger researches should be carried out to evaluate the efficacy of MDCT to detect associated vascular anomalies especially before LDLT.