Conjugated Hyperbilirubinemia in Infants: Is There Still a Role for ERCP?

Over a twenty-year period, we performed 255 ERCP procedures in infants aged up to 1 year. ERCP was indicated in cholestatic infants with suspicion of biliary obstruction. The most common diagnosis was biliary atresia (48%), choledochal cysts (13%), and choledocholithiasis (4%). The procedure complication rate was 13.7%. Hyperamylasemia occurred in 12.9%. More severe complications were rare‐0.8% of ERCP procedure. There were no cases of postprocedural pancreatitis or death. Our study has proved that ERCP is a safe and reliable method in this age group. Its high specificity and negative predictive value for extrahepatic biliary atresia can prevent unnecessary surgeries in patients with normal bile ducts or endoscopically treatable pathologies.

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Biliary Atresia

Pediatrics in Review ◽

10.1542/pir.11.2.57 ◽

1989 ◽

Vol 11 (2) ◽

pp. 57-62

Author(s):

Elizabeth A. Wanek ◽

Frederick M. Karrer ◽

Carlos T. Brandt ◽

John R. Lilly

Keyword(s):

Biliary Atresia ◽

Biliary Obstruction ◽

Bile Flow ◽

Bile Ducts ◽

Current Treatment ◽

Histopathologic Examination ◽

The Past ◽

The World ◽

Hepatic Portoenterostomy ◽

Extrahepatic Bile Ducts

Biliary atresia is a pathologic entity in which there is obliteration of some portion of the extrahepatic bile ducts. In the past, occlusion of the proximal ducts (at the liver hilus) was referred to as "noncorrectable" (Fig 1). If only the distal duct is occluded (and the proximal duct is patent), the lesion was referred to as "correctable." The distinction is academic because current treatment and prognosis are identical. The disease is panductular, ie, both extrahepatic and intrahepatic ducts are involved. Early in the disease, however, occlusion is complete only in the extrahepatic system. Without intervention, intrahepatic biliary obstruction and, subsequently, cirrhosis supervene. In the past, except for a few cases of the correctable variant, surgical procedures were unsuccessful until Morio Kasai performed a hepatic portoenterostomy, which was first reported in English in 1968. Bile flow was effectively reestablished in both correctable and noncorrectable forms of biliary atresia. The operation was only successful when done before the patient was 4 months of age. Subsequent confirmation of Kasai's results were reported throughout the world. ETIOLOGY/PATHOLOGY Biliary atresia was originally thought to be a congenital malformation. Careful histopathologic examination of excised surgical specimens indicate that this is not the case; instead, the disease is a dynamic, progressive panductular sclerotic process that may continue in the intrahepatic ducts even after surgical relief of biliary obstruction.

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BILE-PLUG SYNDROME: A CORRECTABLE CAUSE OF OBSTRUCTIVE JAUNDICE IN INFANTS

PEDIATRICS ◽

10.1542/peds.43.2.273 ◽

1969 ◽

Vol 43 (2) ◽

pp. 273-276

Author(s):

Jay Bernstein ◽

Raul Braylan ◽

A. Joseph Brough

Keyword(s):

Liver Biopsy ◽

Obstructive Jaundice ◽

Biliary Atresia ◽

Choledochal Cysts ◽

Newborn Period ◽

Surgical Exploration ◽

Obstructive Lesion ◽

Hepatocellular Damage ◽

Conjugated Hyperbilirubinemia ◽

Histopathological Effects

Conjugated hyperbilirubinemia in the newborn period and early infancy can result from choledochal obstruction or hepatocellular damage. Examples of the former include biliary atresia, choledochal cyst, and choledochal bile plugs. The recognition of these conditions and their differentiation are of importance in determining the best and most effective course of therapy. Choledochal cysts and bile plugs can be corrected surgically, and the presence of these two conditions is best determined by surgical exploration and transcholecystic cholangiography. Previous liver biopsy is helpful in establishing the presence of an obstructive lesion; but, because of the similarity of histopathological effects on the liver, it will be of little help in differentiating the causes of obstruction.

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Pathogenesis of Biliary Atresia

PEDIATRICS ◽

10.1542/peds.78.1.183 ◽

1986 ◽

Vol 78 (1) ◽

pp. 183-183

Author(s):

THOMAS C. MOORE

Keyword(s):

Biliary Atresia ◽

Bile Ducts ◽

Early Operation ◽

Spontaneous Perforation ◽

Round Cell ◽

Choledochal Cysts ◽

Viral Etiology ◽

Round Cell Infiltration ◽

Extrahepatic Bile Ducts ◽

Inflammatory Component

In Reply.— The letter of Esterly is an important one with some significant observations regarding a potential acquired inflammatory and viral etiology of biliary atresia. A marked and progressive inflammatory component of biliary atresia with extensive early round cell infiltration and progressive fibrosis was reported by one of the authors in 1953.1 This observation led to strong recommendations for early operation in biliary atresia.2,3 It has been suggested that biliary atresia, neonatal spontaneous perforation of the extrahepatic bile ducts, and choledochal cysts may be variants of an acquired infalmmatory process, possibly viral, such as cytomegalovirus, and possibly oncogenic.4

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Issues of geriatric otolaryngologic emergency have not been widely applied despite increase in geriatric population. (Preprint)

10.2196/preprints.13383 ◽

2019 ◽

Author(s):

Waheed Atilade Adegbiji ◽

Shuaib Kayode Aremu ◽

AbdulAkeem Adebayo Aluko

Keyword(s):

Foreign Body ◽

Head And Neck ◽

Clinical Features ◽

University Teaching ◽

Age Group ◽

Geriatric Population ◽

Female Ratio ◽

Common Diagnosis ◽

Foreign Body Impaction ◽

Nose And Throat

BACKGROUND Issues of geriatric otolaryngologic emergency have not been widely applied despite increase in geriatric population. OBJECTIVE This study aimed at determining prevalence, sociodemographic features, aetiology, clinical features, Complications and sources ofreferral of geriatric otorhinolaryngological, head and neck emergency in our center. METHODS This was a prospective hospital based study of geriatric otorhinolaryngology emergency in the Ear, Nose and Throat Department of Ekiti State University Teaching Hospital. The study was carried out between October 2016 and September 2018. Data were obtained by using pretested interviewers questionnaire.All data were collated and analyzed using SPSS version 18.0. The data were expressed by frequency table, percentage, bar charts and pie charts. RESULTS Geriatric otorhinolaryngology, head and neck emergency accounted for 5.3%. Major prevalence age group was 43.9% in the age group (60-64). There were 38.6% males with male to female ratio of 1:1.5. The main aetiology of geriatric otorhinolaryngology emergency was 29.5% trauma/road traffic accident/foreign body impaction and 25.8% tumour. Main anatomical distribution of geriatric otorhinolaryngology emergency were 38.6% throat diseases and 31.1% ear diseases. The most frequent clinical features were pain in 27.3%, hearing loss in 21.2%, tinnitus in 15.9%, bleeding in 14.4%, difficulty breathing in 12.9% and discharge in 11.4%. Common diagnosis in this study were 15.9% sinonasal tumour, 14.4% upper aerodigestive foreign body impaction, 10.6% earwax impaction and 19.8% otitis externa. Acute presentation (<13 weeks) occurred in 1 week in 74.2% and 2-13 weeks In 19.7%. Commonest time of presentation was daytime in 65.9%. Major sources of referral were 43.2% general practitioner and 31.1% casualty officers. Presentation of geriatric otorhinolaryngology emergency were mainly ear, nose and throat clinic in 59.8% with accident and emergency in 28.8%. Commonest associated comorbid illnesses among the geriatric patients were 18.2% hypertension, 14.4% arthritis and 9.8% diabetes mellitus. CONCLUSIONS Geriatric otorhinolaryngological emergency are common pathology with associated with comorbid illnesses. Detailed clinical assessment are mandatory for effective management outcome.

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Anomalous choledocho-pancretic ductal junction in a choledochal cyst: A case report

Srpski arhiv za celokupno lekarstvo ◽

10.2298/sarh0406179j ◽

2004 ◽

Vol 132 (5-6) ◽

pp. 179-181

Author(s):

Miodrag Jovanovic ◽

Dragoljub Bilanovic ◽

Radoje Colovic ◽

Nikica Grubor ◽

Milenko Ugljesic

Keyword(s):

Bile Duct ◽

Choledochal Cyst ◽

Bile Ducts ◽

Postoperative Recovery ◽

Biliary Colic ◽

Duodenal Wall ◽

Choledochal Cysts ◽

Bile Duct Wall ◽

Intrahepatic Bile Ducts ◽

Inflammatory Changes

Choledochal cysts are rare congenital anomalies, mostly detected in adults. Pathogenesis of these cysts seems to be in anomalous junction between pancreatic and common bile duct, above the papillary sphincterand outside of the duodenal wall. The absence of the sphincter above the junction is followed by reflux of the pancreatic juice into the bile duct leading to dilatation and fibrous changes of bile duct wall. A 38-year-old female is presented in whom a choledochal cyst was found 11 years earlier, during the operation performed for obstructive jaundice, when cystojejunostomy with Roux-en Y jejunal limb was carried out. In February 1990, she was admitted to our Institution for jaundice and biliary colic. The patient was reoperated. Operative cholangiography showed an anomalous pancreatobiliary junction, choledochal cyst, dilated cystic duct and moderate dilatation of intrahepatic bile ducts. Cholecystectomy, desanastomosis with partial excision of choledochal cyst, and retrocolic choledochojejunostomy with the same Roux-en-Y jejunal limb were performed. Total excision of choledochal cyst was too risky due to chronic inflammatory changes in the hepatoduodenal ligament. Postoperative recovery was uneventful and the patient remained symptom-free so far.

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Cystic form of biliary atresia. Treatment experience

Russian Journal of Pediatric Surgery, Anesthesia and Intensive Care ◽

10.17816/psaic603 ◽

2020 ◽

Vol 10 (1) ◽

pp. 17-24

Author(s):

Irina I. Borisova ◽

Anatoliy V. Kagan ◽

Svetlana A. Karavaeva ◽

Aleksey N. Kotin

Keyword(s):

Surgical Treatment ◽

Bile Duct ◽

Common Bile Duct ◽

Biliary Atresia ◽

Bile Ducts ◽

Surgical Intervention ◽

Ultrasound Examination ◽

Duct Cyst ◽

Cystic Form ◽

The Common

Background. The cystic form of biliary atresia is a rare form of atresia of the biliary tract, which is a relatively favorable variant of the defect and can be diagnosed antenatally. In practice, it is important not only to suspect this diagnosis, but also to differentiate this variant of impaired development of the external bile duct from the cyst of the common bile duct. This is due to the difference in approaches and methods of surgical treatment of choledochal cysts and biliary atresia. Obliteration (atresia) of the bile ducts in the absence of timely surgical intervention quickly leads to the progression of cirrhosis and the development of liver failure. The method of choice in the treatment of AD is Kasai surgery, often palliative in nature, but allowing to delay the time until liver transplantation. The cyst of the common bile duct rarely requires early surgical treatment, and the risk of cirrhosis is significantly lower. Surgical intervention is aimed at removing the cyst and restoring the flow of bile by anastomosing the external bile ducts with the intestines, which is a radical method of treatment and leads to the recovery of the child. External similarity in ultrasound examination of the fetus and newborn baby of the cystic form of biliary atresia of the bile ducts with a cyst of the common bile duct does not always allow differentiation of one defect from another, which can lead to untimely correction of the defect and an unfavorable outcome. Aim. Demonstrate a rare type of biliary atresia. Materials and methods. Between 2001 and 2019, 33 patients with biliary atresia were treated in the Childrens City Multidisciplinary Clinical Specialized Center for High Medical Technologies in St. Petersburg, only two patients had a cystic form. Both children were initially treated as patients with bile duct cyst. Children were operated on at the age of 2 and 3.5 months. The first patient underwent surgery Kasai, the second hepaticoyunoanastomosis. Results. During the observation period (9 years and 4 years), the synthetic function of the liver is normal, and there are currently no indications for transplantation. Conclusion. If a fetus or a newborn with neonatal jaundice is detected during ultrasound examination of a cystic formation in the gates of the liver, it is very important to correctly and quickly make a differential diagnosis between the cystic form of biliary atresia of the biliary tract and the common bile duct cyst.

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