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BILIARY ATRESIA: EXPERIENCE IN A MILITARY HOSPITAL.

Byline: Shams-ud-din, Ghulam Rasool Tariq, Ch. Aqeel Safdar and Rehan-e-Kibria

Abstract

The purpose of this case series is to determine the types of biliary atresias presenting to our department, options available to confirm the diagnosis with appropriate management and their results. A total of 12 patients with suspected biliary atresia presented to the Department of Paediatric Surgery at Military Hospital Rawalpindi. Out of them 11 patients underwent surgical intervention. Age of the patients ranged from 6 weeks to 16 weeks (mean age 68.80 days). One patient was beyond the scope of biliary drainage. Out of 11 patients operated, operative cholangiography revealed patent extrahepatic biliary tree in 6 patients (55%), thus excluding biliary atresia. One patient (9%) had patency of distal biliary tree with proximal obliteration (Type B) and 4 patients (36%) had complete obliteration of extrahepatic ducts (Type A). These 5 patients of biliary atresia (45%) underwent traditional Kasai portoenterostomy.

Two patients (40%) were stable postoperatively, 2 patients (40%) had gradual deterioration after initial improvement, and 1 patient (20%) died in perioperative period.

INTRODUCTION

Biliary atresia is a disorder unique to neonatal period. It is a rare but potentially devastating disease. The incidence of biliary atresia ranges from 1 in 10,000 to 1 in 18,000 live births1-3. It seems to affect girls slightly more often than boys. Asian and African-Americans are affected more frequently than the Caucasians4. Biliary atresia is the most common cause of end stage liver disease in the infant and is the leading paediatric indication for liver transplantation. Earlier diagnosis is associated with improved outcomes following the Kasai portoenterostomy (after the Japanese Surgeon who developed the surgery, Dr Morio Kasai) and larger survival with native liver. Late referral remains problematic; policies to ensure timely diagnosis and referral are required5,6.

The jaundiced newborn presents a complicated and extensive differential diagnosis7. Infants with biliary atresia typically appear normal at birth, becoming clinically jaundiced at 2 to 6 weeks of age. If unrecognised, the condition leads to liver failure but not kernicterus8. Pathologic jaundice is suspected when the conjugated fraction of bilirubin accounts for more than 20% of the total serum bilirubin. Because no individual test is absolutely reliable, a combination of serologic evaluation, ultrasonography (USG), hepatobiliary scintigraphy (HIDA scan) and percutaneous liver biopsy is included in an expeditious evaluation of an infant with suspected biliary atresia. Definitive exclusion of biliary atresia in the infant with cholestatic jaundice usually requires operative cholangiography (OCG)/ percutaneous cholecysto-cholangiography (PCC) and open liver biopsy/ percutaneous liver biopsy9.

The purpose of this series is to share the experience of diagnosis of biliary atresia in infants and the early results of traditional Kasai portoenterostomy.Text Box: Correspondence: Lt Col Rehan-e-Kibria, classified Paediatric Surgeon, Military Hospital Rawalpindi Received: 20 April 2009; Accepted: 03 Nov 2009

CASE SERIES

These cases presented to the Paediatric Surgery Department of Military Hospital, from January 2008 to March 2009. During this period 12 patients of suspected biliary atresia were referred to us. They were initially diagnosed by Paediatricians based on history, blood biochemistry, USG and HIDA scan and referred to us for subsequent management. Out of them 1 patient had age more than 5 months (too late for biliary drainage) and had features of liver failure. The remaining 11 patients were prepared for elective surgery. Operative cholangiography, being the final diagnostic manoeuvre, was performed as a preliminary step, prior to proceeding to Kasai portoenterostomy. Exploration was performed through a small right upper quadrant incision, planned to allow a subsequent Kasai procedure if necessary. In 6 patients, cholangiogram showed patency of the extrahepatic biliary tree and free flow of contrast (Diatrozoate) in the duodenum, thus excluding biliary atresia.

For them liver biopsy was taken and laparotomy was closed. Subsequently they were referred back to Paediatricians for further work up and management. One patient had patency of distal biliary tree with proximal obliteration (Type B). In 4 patients; there was no lumen in gall bladder (Type A), as such cholangiogram could not be performed. For them traditional Kasai procedure was performed.

Steroid (prednisolone 2mg/kg/day) was given postoperatively for 1 month, and then tapered. Ursodeoxycholic acid was not used in any patient. Vitamin supplements were given to all. Average postoperative hospital stay was 10 days. They were discharged with an advice for follow up after 2 weeks, then monthly for 3 months.

During the study period a total of 8035 patients reported to Paediatric Surgery OPD, out of them 12 (0.15%) were cases of suspected biliary atresia. A total number of 1155 operations were done under general anaesthesia during this period. Out of them 11 (0.95%) underwent procedures for suspected biliary atresia. On exploration, 6 patients (55%) had gall bladder with a lumen for canulation, subsequently proved by cholangiogram to have patent extrahepatic biliary tree, thus excluding biliary atresia. One patient (9%) showed free flow of dye in the duodenum, but no flow of dye in hepatic ducts indicating proximal obliteration (Type B). Four patients (36%) had complete obliteration of extrahepatic biliary tree as such cholangiogram was not possible (Type A). Traditional Kasai procedure was performed in all the 5 patients (45%) with biliary atresia. Demographic distribution with sex, age, type of atresia and outcome are given in Tables.

Table: Show demographic data of the patients

Sex###No of patients###Percentage

Females###3###60

Males###2###40

Age

Less than 8 weeks###2###40

More than 8 weeks###3###60

Type

Postnatal###4###80

Prenatal###1###20

Outcome

Stable###2###40

Deterionting###2###40

Death###1###20

DISCUSSION

Biliary atresia is a disorder that is characterized by complete obliteration of bile flow as a result of destruction or absence of all or part of extrahepatic bile ducts4,10. The destruction of extrahepatic bile ducts is accompanied by variable degree of intrahepatic damage, which eventually leads to cirrhosis of liver. Early diagnosis is imperative for better outcome. However, establishing the diagnosis is problematic because of its rarity, the much more common indirect hyperbilirubinemia that occurs in the newborn period and no single test being absolutely diagnostic1,5. Direct hyperbilirubinemia is always an abnormal finding. If another diagnosis can not be firmly established quickly, infants with cholestatic jaundice should be considered for biliary atresia.

Identification of a normal gall bladder in USG is highly predictive of the absence of biliary atresia. Percutaneous biopsy is usually performed but most of the times a definite diagnosis cannot be reached, ultimately leading to operative cholangiogram, and in the process causing undue delay4,9. Most of the experience in our country is from postoperative liver biopsies11. Special attention is required to detect Type B biliary atresia, here dye will outline the distal biliary tree with free flow to the duodenum, but will fail to delineate the proximal hepatic ducts. The pathogenesis of biliary atresia remains obscure despite numerous aetiologic theories. It appears to involve immune-mediated fibro-obliteration of the extrahepatic and intrahepatic biliary tree in most patients and defective morphogenesis in the remainder1,5.

Traditional Kasai portoenterostomy, gall bladder Kasai, and porto-appendiceal duodenostomy have been utilised for biliary atresia. Laparoscopic Kasai has also been done in this era of laparoscopic surgery, but seems to be associated with more postoperative complications and worse early outcome12,13. Till today traditional Kasai portoenterostomy appears to achieve better biliary drainage. Surgical correction of biliary atresia offers long-term survival for about one quarter of patients, provides palliation until liver transplantation becomes necessary, and if surgical correction is not feasible, biliary atresia is uniformly fatal14. The determinants of the outcome of portoenterostomy include age at surgery (preferably before 8 weeks), the centre's experience, the presence of associated anomalies, and the postoperative occurrence of cholangitis.

The outcome of children with biliary atresia is related to the caseload of the surgical centre where they have their primary surgery. Children with biliary atresia should be managed in surgical centres with a caseload of more than five cases annually5,15.

In this study, 12 patients with suspected biliary atresia were referred to Paediatric Surgery OPD by Paediatricians during the study period. Out of them 11 patients underwent surgical exploration for OCG/ Kasai procedure. Six (55%) out of these 11 patients had patent extrahepatic biliary tree excluding biliary atresia. Their possible diagnosis could be neonatal hepatitis, paucity of bile duct syndrome, Alagille's syndrome or a-1 antitrypsin deficiency. This is higher than that reported from other centres (10-28%), possibly due to less experience in diagnostic workup and less confidence in interpreting Percutaneous liver biopsies1. Four patients (80%) had Type A and 1 Patient (20%) had Type B biliary atresia, similar to that reported in the literature1. All the 5 patients with biliary atresia underwent traditional Kasai procedure. Among them 3 patients (60%) were female and 2 (40%) were male.

It is established that biliary atresia occurs more commonly in female than male 4, 10. Four patients (80%) had postnatal form of biliary atresia and only I patient (20%) had perinatal form with polysplenia. Schwarz described postnatal form in 65-90% of the cases and perinatal form in 10-35% of the cases. Perinatal form of biliary atresia presents earlier4. The only patient with perinatal form of biliary atresia in this study presented earlier, at 6th week, as reported by Schwarz4.

Cholangitis is the most frequent complication after Kasai procedure, with an incidence ranging from 33% to 60% and is most common in first 2 years1,10. One of our patients (20%) required readmission in next 3 months following surgery due to cholangitis. With timely referral for surgical reconstruction biliary drainage typically occurs in more than two thirds of the patients (range 25% to 86%). The half of those with initial bile drainage; progressive liver insufficiency will insidiously develop. Mortality rate within 30 days is extremely low1,8. In the study, 2 patients (40%) were stable after Kasai procedure, 2 patients (40%) were deteriorating after initial improvement, and 1 patient (20%) died in perioperative period. Re-exploration was not planned for any. Biliary atresia, being a progressive disease, long term follow up is indicated to detect possible complications early for timely intervention.

CONCLUSION

A high index of suspicion is the key to making a timely diagnosis of biliary atresia. Special attention is needed during cholangiography to detect Type B biliary atresia. Early surgical intervention is imperative for favourable result. Traditional Kasai portoenterostomy continues to be the initial procedure in the treatment of infants with biliary atresia. Long term follow up is a must as the disease is a progressive disease.

Reference

1. Altman RP, Buchmiller TL, The Jaundiced Infant: Biliary Atresia. In: Grosfeld JL, O'Neill JA, Coran AG, Fonkalsrud EW. Pediatric Surgery. 6th ed. Mosby, Inc. Philadelphia 2006; 1603-1619.

2. Petersen C, Harder D, Abola Z, Alberti D, Becker T, Chardot C, et al. European biliary atresia registries: summary of a symposium. Eur J Pediatr Surg. 2008; 18(2): 111-116.

3. Wildhaber BE, Majno P, Mayr J, Zachariou Z, Hohlfeld J, Schwoebel M, et al. Biliary atresia: Swiss national study, 1994-2004. J Pediatr Gastroenterol Nutr. 2008 Mar; 46(3): 299-307.

4. Schwarz SM. [Online] Biliary Atresia. Available at : emedicine.medscape.com//927029-overview. Accessed 2009:22.

5. Sokol RJ, Shepherd RW, Superina R, Bezerra JA, Robuck P, Hoofnagle JH. Screening and outcomes in biliary atresia: summary of a National Institutes of Health Workshop. Hepatology. 2007: 46(2): 566-581.

6. Schreiber RA, Barker CC, Roberts EA, Martin SR, Alvarez F, Smith L, et al. Biliary atresia: the Canadian experience. J Pediatr. 2007: 151(6): 659-665.

7. Azmy AAF, Raine PAM. Neonatal surgical emergencies. In: Raine PAM, Azmy AAF. Surgical Emergencies in Children. 1st ed. Butterworth-Heinemann Ltd. UK 1994; 40-77.

8. Karrer FM, Pence JC. Biliary Atresia and Choledochal Cyst. In: Ziegler MM, Azizkhan RG, Weber TR. Operative Pediatric Surgery. 1st ed. McGraw-Hill Professional. United States 2003; 775-788.

9. Nwomeh BC, Caniano DA, Hogan M. Definitive exclusion of biliary atresia in infants with cholestatic jaundice: the role of percutaneous cholecysto-cholangiography. Pediatr Surg Int. 2007: 23(9): 845-849.

10. Kulshrestha R. Biliary Atresia. In: Kulshrestha R. Common Problems in Pediatric Surgery. 2nd ed. CBS Publishers and Distributors. New Delhi 2006; 249-254.

11. Qureshi MA, Shaukat M. Biliary Atresia: Liver histology and its relation to the age of the patient. Pak Paed J. 2006; 30: 193-6.

12. Wong KK, Chung PH, Chan KL, Fan ST, Tam PK. Should open Kasai portoenterostomy be performed for biliary atresia in the era of laparoscopy? Pediatr Surg Int. 2008: 24(8): 931-933.

13. Tsao K, Rosenthal P, Dhawan K, Danzer E, Sydorak R, Hirose S, et al. Comparison of drainage techniques for biliary atresia. J Pediatr Surg. 2003: 38(7): 1005-1007.

14. Karrer FM, Price MR, Bensard DD, Sokol RJ, Narkewicz MR, Smith DJ, et al. Long-term results with Kasai operation for biliary atreia. Arch Surg. 1996: 131(5): 493-496.

15. McKiernan PJ, Baker AJ, Kelly DA. The frequency and outcome of biliary atresia in UK and Ireland. Lancet 2000: 1; 355(9197): 25-29.
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Author:Tariq, Ghulam Rasool; Safdar, Aqeel; Shams-ud-din; Rehan-e-Kibria
Publication:Pakistan Armed Forces Medical Journal
Article Type:Disease/Disorder overview
Geographic Code:9PAKI
Date:Dec 31, 2010
Words:2145
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