أسئلة التاسعة جراحة رابعة دمياط (أطفال)

Jaundice

• Jaundice in the first 2 weeks of infancy is usually due to indirect (unconjugated) hyperbilirubinemia.
• Causes include physiological jaundice due to immature hepatic function (associated with breast feeding), Rh ABO and rare blood group incompatibilities that produce hemolysis, and infections.
• Jaundice persisting beyond the first 2 weeks involves elevated levels of both indirect and direct bilirubin.
• Potential surgical disorders include biliary atresia (60%), various forms of hepatitis (35%), and choledochal cyst (5%).
• Mild indirect hyperbilirubinemia occurs with pyloric stenosis and disappears after pyloromyotomy.
• I.O. can intensify jaundice by increasing the enterohepatic circulation of bilirubin.
• Jaundice can be an early and important sign of septicemia in newborns

Biliary Atresia

• Biliary atresia involves the absence of patent bile ducts draining the liver.
• The incidence is 1 per 10,000 live births.
• Etiology is unknown, it may be due to inflammatory process occlusion of variable lengths of biliary tree or congenital onset.
• Familial cases and frequent association with the polysplenia syndrome.
• Cholestasis with plugging.
• Histologically, atretic ducts consist of solid fibrous cords with occasional islands of biliary epithelium.
• Liver biopsy shows proliferation of bile canaliculi containing inspissated bile, leading to failure to excrete bile, progressive periportal fibrosis, and obstruction of intrahepatic portal veins, resulting in biliary cirrhosis.

Types of Biliary Atresia

• Type 1 is obstruction at the level of the common bile duct (CBD) and is correctable.
• Type 2 is obstruction at the level of the common hepatic duct (CHD).
• Type 3 occurs in 90% of cases, where the extrahepatic portion of the biliary tract is occluded and appears as a cord-like structure, making it non-correctable

Clinical Picture of Biliary Atresia

• Jaundice is noted at 2-3 weeks of age.
• Urine may be dark.
• Stools may be normal or clay-colored and contain an increased quantity of fat but are of normal consistency and not frothy.
• The liver may initially be of normal size but later becomes enlarged, progressing to cirrhosis and hardening.
• Splenomegaly commonly develops.
• Clinical Triad: Conjugated jaundice, pale acholic stool and dark urine

Investigations for Biliary Artesia

• Liver Function Tests: Direct bilirubin >3 mg/dL, Alkaline phosphatase elevated to 500-1000 units/L, Glutamyltranspeptidase >300 units.
• Radiological study, Ultrasonography: Gallbladder ghost triad, triangular cord sign.
• HIDA Scan: Technetium Tc 99m-labeled iminodiacetate used to observe the intensity of uptake within the liver and evidence of secretion into the bowel, usually preceded by a 2- to 3-day course of phenobarbital to promote tracer uptake.
• Liver biopsy: Inflammation, intrahepatic bile duct proliferation and cholestasis with plugging.

Differential Diagnosis of Biliary Artesia

• Differential includes: Choledochal cyst, inspissated bile syndrome, hepatitis, genetic metabolic diseases and neonatal physiologic jaundice.
• Choledochal cyst is identified by the presence of a palpable mass located in the right upper quadrant and ultrasonographic confirmation.
• Inspissated bile syndrome is due to a hemolytic process and TPN, and is recognized by abdominal US.
• Hepatitis cause can be unknown, and due to a variety of maternal infections like Toxoplasmosis, Rubella syndrome Cytomegalovirus, Herpes simplex and Coxsackievirus and Varicella.
• Genetic metabolic diseases include antitrypsin deficiency, galactosemia & cystic fibrosis with Alagille's syndrome.
• Neonatal physiologic jaundice is self-limited and responds to phototherapy.

Treatment for Biliary Artesia

• Timing: As early as possible in infancy to avoid liver cirrhosis.
• In rare correctable types, a hepaticojejunostomy is performed.
• In confirmed biliary atresia, hepatic portoenterostomy (Kasai procedure) is utilized.

Kasai Procedure Technique

• Scarred bile ducts and gallbladder are removed.
• A Roux-en-Y limb of the jejunum is sutured to an area of the hilum bounded laterally by the hepatic artery branches.
• If this fails or the child develops significant complications of chronic liver disease, a liver transplantation will be required.

Choledochal Cyst

• Dilation or diverticulum of all or a portion of the common bile duct.
• More common in females at 3:1 ratio.
• More common in Asians specifically Japan.
• Pathogenesis is multifactorial.
• In many patients, an anomalous junction between the common bile duct and the pancreatic duct can be demonstrated.
• This occurs when the pancreatic duct empties into the common bile duct >1 cm proximal to the ampulla.

Todani Classification of Choledochal Cysts

• Type I: A fusiform dilation of the extrahepatic bile duct.
• Type II: A saccular outpouching of the common bile duct.
• Type III: Referred to as a Choledochocele and is a wide-mouth dilation of the CBD at its confluence with the duodenum.
• Type IV: Cystic dilation of both the intra-and extrahepatic bile ducts.
• Type V: Consists of lakes of multiple intrahepatic cysts with no extrahepatic component; when associated with hepatic fibrosis, it is termed Caroli's disease.

Clinical Picture of Choledochal Cysts

• The majority of cases are in childhood before 10 years of age.
• Symptoms & signs include recurrent abdominal pain, jaundice, and a right upper quadrant mass.
• As children age, the cyst may become infected, leading to painful and recurrent attacks of pyrexia.
• Perforation of the cyst can lead to biliary peritonitis
• Complications if left untreated: Cholangitis and cholangiocarcinoma

Investigations for Choledochal Cysts

• Ultrasonography detects choledochal cysts in the fetus.
• Technetium Tc 99m-labeled HIDA scan.
• CT & MRI scans.
• ERCP
• PTC (Percutaneous transhepatic cholangiography)
• Operative cholangiography.

Treatment for Choledochal Cysts

• The cyst should be excised and hepaticojejunostomy is performed.

Anomalies of the Gallbladder

• Absence of the gallbladder: Rare (0.03% of cases).
• The phrygian cap is present in 2-6% of the population. -It is a kinking of the fundus of the gall bladder with no clinical significance.
• Double gall bladder is present in 1 in 4000 individuals. -It presents with two separate cavities and cystic ducts.
• Floating gall bladder hangs on a mesentery and is liable to undergo torsion.
• Left sided gall bladder: Rare
• Intrahepatic gall bladder: increased incidence of gall stones

Cystic Duct Anomalies

• Absence of the cystic duct leading to injury of the CBD during cholecystectomy.
• In low insertion of cystic duct, the cystic duct may open into the common duct near the ampulla leading to ligation of CBD during cholecystectomy.
• The cystic duct may enter the right hepatic duct, the right hepatic duct may be mistaken for the cystic duct and ligated.

Anomalies of the Hepatic & Cystic Arteries

• Accessory cystic artery (the most common) may be torn if it is not identified.
• A large accessory left hepatic artery (in about 5% of people) may arise from the left gastric artery.
• The right hepatic artery may arise from the SMA artery and be mistaken for the cystic artery, leading to ligation.