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Penatalaksanaan Bedah Anak

Pada Kelainan GEH

Dr. Mahyuddin Rasyid SpB


Bakordik RSUD Andi Makkasau Parepare
BILIARY ATRESIA
• Biliary atresia is characterized by obliteration or discontinuity
of the extrahepatic biliary system, resulting in obstruction to
bile flow.
Symptom
• Typical symptoms include variable degrees of jaundice, dark
urine, and light stools.
• In the case of biliary atresia, most infants are full-term,
although a higher incidence of low birthweight may be
observed.
• In most cases, acholic stools are not noted at birth but
develop over the first few weeks of life. Appetite, growth, and
weight gain may be normal
• Direct hyperbilirubinemia is always an abnormal finding and
may be present from birth in the fetal/embryonic form.
WORKUP
• Serum bilirubin (total and direct): Conjugated
hyperbilirubinemia, defined as any level exceeding either 2
mg/dL or 20% of total bilirubin, is always abnormal
• Ultrasonography
In biliary atresia, ultrasonography may demonstrate
absence of the gallbladder and no dilatation of the biliary
tree.
. CT Scanning
Endoscopic retrograde
cholangiopancreatography (ERCP)
ERCP may become part of the management algorithm in
assessing neonatal direct hyperbilirubinemia
TREATMENT
• Once biliary atresia is suspected, surgical intervention is the
only mechanism available for a definitive diagnosis
(intraoperative cholangiogram) and therapy (Kasai
portoenterostomy).
KASAI PROCEDURE
NEOPLASMA HEPAR
• Tumors of the liver may be either malignant or benign :
 Benign :
hemangiomas, hamartomas, and focal nodular hyperplasia
(FNH)
Malignant
Hepatoblastoma (HB) and hepatocellular carcinoma (HCC) are the
most common and account for two thirds of all hepatic
neoplasms
• Benign Tumor
Hemangiomas
Hemangiomas are the most common benign liver tumors in
children and commonly occur within the first 6 months of life
Most hemangiomas are incidentally discovered on imaging studies
• Laboratory abnormalities associated with this tumor include
anemia, elevated aspartate transaminase levels,
hyperbilirubinemia, and occasionally an elevated α-
fetoprotein (AFP) level
• Ultrasonography, CT scanning, or MRI is used to characterize
the size and location
TREATMENT
• The natural history for hemangiomas is spontaneous
regression in the first 2 years of life
• Initially, high-dose corticosteroids (3-5 mg/kg/d) are
administered for 3-5 weeks
• Daily subcutaneous administration of interferon-alfa (3 million
U/m2/kg) may lead to involution of hemangiomas located
throughout the body
• Focal lesions are treated with complete surgical excision or
with selective hepatic artery embolization
• Maligna
Hepatoblastoma
the most common form of liver cancer in children, although it is
a comparatively uncommon pediatric solid tumor. The
disease usually affects children younger than 3 years
• Hepatoblastomas originate from immature liver precursor
cells and present morphologic features that mimic normal
liver development
• Loss of heterozygosity (LOH) of chromosome arm 11p markers
occurs commonly in hepatoblastoma identified in association
with Beckwith-Wiedemann syndrome (BWS)
Symptom
 Hepatoblastoma is usually diagnosed as an asymptomatic
abdominal mass.
 Approximately 10% of patients have incidental findings of
hemihypertrophy
 Severe osteopenia is present in most patients and regresses
with resection of the tumor.
LABORATORY STUDIES

• Normochromic normocytic anemia is often present.


Thrombocytosis may be present
• AFP is found in high concentrations in fetal serum and in
children with hepatoblastoma, hepatocellular carcinoma.
Imaging
• Ultrasonography Abdominal ultrasonography allows
assessment of tumor size and anatomy, which helps in surgical
planning
• CT scanning reveals involvement of nearby structures.
Regional lymph nodes are almost never involved
Treatmet
Chemoterapy:
 The most important advance in the care of children with
hepatoblastoma has been the discovery of effective
chemotherapy
Initial reports showed the efficacy of vincristine (VCR),
cyclophosphamide (CPM), and doxorubicin with 5-fluorouracil
(5-FU)
Radiotherapy
Doses used for treatment of hepatoblastoma are usually 1200-
2000 centigray (cGy). These dose limits are based on the
liver's limited ability to regenerate after radiation.
Adjuvant radiotherapy may have a role in the treatment of
chemoresistant pulmonary metastases.
Surgical Care
The hepatoblastoma can be completely resected at diagnosis
in approximately one third of patients
Initial resection of operable primary tumors by lobectomy is
the standard of care. Occasionally, pulmonary lesions are
resected
• The following cases warrant early referral to a transplant
surgeon:
- Multifocal or large solitary lesions
- Tumors involving all 4 sectors of the liver
- Unifocal, centrally located tumors that involve the main
hilar structures or main hepatic veins
Cholelithiasis/Choledocolithiasi
s
• The distribution of gallstone types in children differs from the
adult population, with cholesterol stones being the most
common type of stone in adults and black pigment stones
being the most common type in children
• Black pigment stones make up 48% of gallstones in children.
They are formed when bile becomes supersaturated with
calcium bilirubinate, the calcium salt of unconjugated
bilirubin. Black pigment stones are commonly formed in
hemolytic disorders
• The complications of cholelithiasis in children are similar to
those in adults. Cholelithiasis primarily affects the gallbladder
and may cause irritation of the gallbladder mucosa, resulting
in chronic calculous cholecystitis and symptoms of biliary colic.
Etiology

• Cholelithiasis in children has various causes related to


predisposing factors. Hemolytic disease, hepatobiliary disease,
obesity, [7] prolonged parenteral nutrition
SYMPTOMATIC
• In symptomatic patients, pain, primarily in the right upper
quadrant, is the most common presenting symptom and may
be accompanied by nausea and vomiting
Laboratory tests
• Should include a complete blood count, gamma-
glutamyltransferase (GGT), amylase, urinalysis, direct and
indirect bilirubin, alkaline phosphatase, and transaminase
levels.
Imaging in Gallstones

USG
Ultrasonography (US) is the procedure of choice for identifying
gallstones. Current high-resolution, real-time US can identify
gallstones as small as 2 mm, with a sensitivity greater than
95%
CT Scan
Only 74-79% of gallstones are identified in patients with
computed tomography (CT) scanning. CT is not a screening
tool for uncomplicated cholelithiasis.
X-Ray
Only 15-20% of stones are visible on plain radiographs
Treatment
Asyptomatic Gallstone
• Ursodeoxycholic acid can be useful in the medical management of
cholelithiasis
• The primary disadvantage with ursodeoxycholic acid therapy is the
high incidence of gallstone recurrence
• Therefore, this treatment is not recommended in patients with
symptomatic cholelithiasis and is indicated only for patients either
unfit or unwilling to undergo surgical intervention.
Symptomatic Gallstone
• Laparoscopic cholecystectomy is currently the criterion
standard in the treatment of symptomatic cholelithiasis
• Removal of the gallbladder in asymptomatic children with
cholelithiasis is not standard practice
TERIMA KASIH