560 Section III / Subspecialty Surgery |
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What if the Kasai fails? |
Revise or liver transplantation |
What are the possible |
Cholangitis (manifested as decreased |
postoperative complications? |
bile secretion, fever, leukocytosis, |
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and recurrence of jaundice), |
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progressive cirrhosis (manifested as |
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portal hypertension with bleeding |
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varices, ascites, hypoalbuminemia, |
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hypothrombinemia, and fat-soluble |
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vitamin K, A, D, E deficiencies) |
What are the associated |
Between 25% and 30% have other |
abnormalities? |
anomalies, including annular pancreas, |
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duodenal atresia, malrotation, polysplenic |
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syndrome, situs inversus, and preduodenal |
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portal vein; 15% have congenital heart |
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defects |
CHOLEDOCHAL CYST |
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What is it? |
Cystic enlargement of bile ducts; most |
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commonly arises in extrahepatic ducts, but |
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can also arise in intrahepatic ducts |
What is the usual |
50% present with intermittent jaundice, |
presentation? |
RUQ mass, and abdominal pain; may also |
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present with pancreatitis |
What are the possible |
Cholelithiasis, cirrhosis, carcinoma, and |
complications? |
portal HTN |
What are the anatomic |
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variants: |
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I? |
Dilation of common hepatic and common |
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bile duct, with cystic duct entering the |
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cyst; most common type (90%) |
Chapter 67 / Pediatric Surgery 561
II? |
Lateral saccular cystic dilation |
III? |
Choledochocele represented by an |
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intraduodenal cyst |
IV? |
Multiple extrahepatic cysts, intrahepatic |
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cysts, or both |
V? |
Single or multiple intrahepatic cysts |
562 Section III / Subspecialty Surgery |
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How is the diagnosis made? |
U/S |
What is the treatment? |
Operative cholangiogram to clarify |
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pathologic process and delineate the |
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pancreatic duct, followed by complete |
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resection of the cyst and a Roux-en-Y |
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hepatojejunostomy |
What condition are these |
Cholangiocarcinoma often arises in |
patients at increased risk |
the cyst; therefore, treat by complete |
of developing? |
prophylactic resection of the cyst |
CHOLELITHIASIS |
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What is it? |
Formation of gallstones |
What are the common |
Etiology differs somewhat from that |
causes in children? |
of adults; the most common cause is |
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cholesterol stones, but there is an |
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increased percentage of pigmented |
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stones from hemolytic disorders |
What is the differential |
Hereditary spherocytosis, thalassemia, |
diagnosis? |
pyruvate kinase deficiency, sickle-cell |
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disease, cystic fibrosis, long-term |
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parenteral nutrition, idiopathic |
What are the associated |
Use of oral contraceptives, teenage, |
risks? |
positive family history |
What is the treatment? |
Cholecystectomy is recommended for all |
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children with gallstones |
ANNULAR PANCREAS |
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What is an annular |
Congenital pancreatic abnormality with |
pancreas? |
complete encirclement of the duodenum |
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by the pancreas |
What are the symptoms? |
Duodenal obstruction |
What is the treatment? |
Duodenoduodenostomy bypass of |
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obstruction (do not resect the pancreas!) |