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Paediatric Surgical approach to

Surgery
Jaundiced infant
BY:
ISSA AHMED HASSAN
FACILITATOR:
DR. NDEGWA
Introduction
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SURGERY
• Jaundice is the clinical description for the yellowish tinge to
the skin, mucous membranes and sclera caused by raised
levels of bilirubin in the blood.
• The word itself derives from Old French (Jaundice =
yellow), while medical term, icteric, derives from the Greek
Jaundiced
word (Ikteros) for a variety of yellow bird. It was believed that
infant
a sufferer from jaundice would be cured by gazing at such a
bird!
• The normal range of total and conjugated bilirubin
are < 1 mg/dL (< 17 μmol/L) and < 0.25 mg/dL
(< 5 μmol/L) respectively,
Cont..
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•Jaundice can only be appreciated when total bilirubin is
greater than
4 mg/dL (~50 μmol/L).

Jaundiced •Physiological jaundice usually presents from the second


infant to third day of life and resolves by 5-7 day of life
•Jaundice persisting more than two weeks is considered
pathologic and can be due to the following causes
•Billiary obstruction
•Increased red cell destruction
•Liver dysfunction
Obstructive Jaundice
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•Obstructive disorders which are of


surgical importance include:
Jaundiced •Billiary atresia
infant
•Choledochal cyst
•Inspissated bile syndrome
•Others- cystic fibrosis, intrahepatic
hypoplasia, neonatal sclerosing
cholangitis
Biliary Atrasia
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•Rare condition characterized by
fibroproliferation of the biliary tree
that can progress to hepatic fibrosis
Jaundiced
infant
and cirrhosis and finally end stage
hepatic failure.
•Cause is multifactorial and has been
described previously and arrest of
biliary development during the solid
stage of bile duct development
Etiology
Etiology andand pathogenesis
pathogenesis
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•The exact aetiology of biliary atresia
remains obsecure, however there
has been an association with the
Jaundiced
infant following:
•Other congenital anomalies
•Infections
•Immunological mechanisms
•Genetic predisposition
Pathogenesis
pathogenesis
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• The gross appearance of the extrahepatic biliary tract varies
from an inflamed, hypertrophic occluded biliary tract to an
atrophic remnant
• The lumen of the extrahepatic duct is obliterated at a variable
level and this forms the basis for the commonest
Jaundiced
classification in clinical use as suggested by the Japanese
infant
Society of Pediatric Surgeons (JSPS)
Classification
Classification
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•Type I Atresia of the common bile
duct
Jaundiced
•Type II Atresia of the common
infant hepatic duct
•Type III Atresia of the whole
extrahepatic biliary tree up to the
portahepatis
Clinical presentation
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•Jaundice at birth or shortly
thereafter
Jaundiced
•Acholic pale gray appearing stool
infant
•Later failure to thrive, stigmata of
portal hypertension life
splenomegaly and esophageal
varices
Diagnosis
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•Liver function tests
•Ultrasound scan
Jaundiced
•TORCH infection titres analysis
infant •Nuclear medicine scan
•Surgical exploration and
cholangiography
•Liver biopsy
•Duodenal drainage test
Treatment
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•Treatment is via surgery through Kasai
procedure or liver transplant
•Kasai procedure is most effective when
Jaundiced
infant
done before 60 days
•First phase: In Kasai procedure, the
surgeon creates a
hepatoportoenterostomy allowing bile
to flow straight to the small intestine
•Second phase- Liver transplantation
Complications
Complications
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•Cholangitis
•Portal hypertension
Jaundiced •Biliary Lakes
infant
•Malignancies
Choledochal Cyst
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•Refers to a rare congenital
dilatation of the hepatic or bile
duct.
Jaundiced
infant •First reported by Douglas in 1852, it
was not until early this century that
precise clinical recognition and
surgical treatment were developed
Classification
Classification
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•Todani classification
• Type I- cystic dilatation of common
bile duct
Jaundiced
infant
• IA- cystic
• IB- fusiform
• IC- saccular
Cont..
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•Type II- simple diverticulum of the
extrahepatic biliary tree
•Type III- cystic dilatation of the
Jaundiced
infant
intraduodenal portion of the common
bile duct
•Type IV- multiple cyst of intra and
extrahepatic biliary tree
•Type V-isolated intrahepatic biliary
cystic disease
Clinical presentation
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•Classical triad
• Pain, jaundice and abdominal mass
• Conjugated bilirubin
Jaundiced
infant • Failure to thrive
•Intermittent jaundice and recurrent
cholangitis
•pancreatitis
Investigations
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• CBC
• LFTs
• Abdominal ultrasonography
• Abdominal CT and MRI
Jaundiced • PCT and ERCP
infant
Treatment
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•Supportive treatment for
pancreatitis or cholangitis
Jaundiced
•Surgery- radical excision of the cyst
infant with reconstruction of the biliary
tract using a Roux-en-Y loop of
jejunum
Choice ofof
Choice surgery
surgery
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• Cyst excision with Roux-en-Y
hepatoenterostomy is the
definitive treatment of
choice in all patients with
Choledochal cyst, regardless
Jaundiced of age or symptomatology
infant
Mucosectomy
Mucosectomy
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• In a patient in whom complete excision of the distal portion
of the cyst is difficult.
• mucosectomy of the distal portion of the cyst is
recommended.
Jaundiced • To avoid not only damage to the pancreatic duct, hepatic
infant artery and portal vein, but also to avoid cancer rising from the
remaining epithelium of the distal portion of the cyst.
Staged procedure
Staged Procedure
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• If primary excision is considered to be difficult, for example, in
the case of perforated bile peritonitis, severe cholangitis or
severe general condition, or in neonates, external biliary
drainage is recommended
• Subsequently, delayed primary excision may be carried out
Jaundiced
one or two months later
infant
Biliary Reconstruction
Biliary Reconstruction
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• Most surgeons use a Roux-en-Y hepaticojejunostomy,
however some surgeons recommend a wide anastomosis at
the level of the hepatic hilum to allow free drainage of bile to
prevent postoperative anastomotic stricture and stone
formation
Jaundiced • After this is done a biliary reconstruction is then done
infant
Inspissated Bile syndrome
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•This condition is described when
there is normal biliary system in
patients with persistent obstructive
Jaundiced
infant jaundice
•Increased viscosity of bile and
obstruction of the canaliculi are the
implicated causes
Laparascopic
Laparoscopic Cyst cyst excision
Excision
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• Recent advances in laparoscopy have enabled pediatric
surgeons to perform minimally invasive surgery for
Choledochal cyst.
• Although technically demanding and long-term follow-up
results remain unknown, experienced laparoscopic surgeons
Jaundiced
can obtain results as good as those for open surgery.
infant
• The disadvantages are mainly the longer operating time and
the higher costs, which, however, may be offset by a shorter
hospital stay.
Associated
ASSOCIATED conditions
CONDITIONS
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• Intrahepatic Bile Duct Dilatation


• Disorders of Pancreatic Duct and Common Channel

Jaundiced
infant
References
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• Schwartz’s Principle of Surgery.
• Baileys and Love Short practice of surgery.
• Sabiston Texbook of Surgery.

Jaundiced
infant
THANK YOU

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