IKTERUS · 2019. 3. 23. · SISTEM BILIER Agenesis Double gall bladder Floating gall bladder...
Transcript of IKTERUS · 2019. 3. 23. · SISTEM BILIER Agenesis Double gall bladder Floating gall bladder...
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IKTERUS
Fisiologis Patologis
Pada 60% aterm, dan
80% preterm
1.Obstruksi atresia biliaris
2. Kelainan darah (ABO, Rh)
3. Metabolik
4. Infeksi (rubella, syphillis)
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KELAINAN KONGENITAL
SISTEM BILIER
Agenesis
Double gall bladder
Floating gall bladder
Atresia biliaris
Kista duct choledochus
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Insidens
• Jarang
• 1 diantara 14 000 – 20 000 kelahiran
hidup
• Laki-laki dan wanita sama
• Asia Timur lebih banyak dibanding
tempat lain
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Atresia bilier
Ekstra hepatik
Intrahepatik Embrional
Perinatal
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Patogenesis
OBLITERASI SALURAN EMPEDU
1. Terganggunya perkembangan saluran
empedu pada waktu “solid stage” (?)
2. Gangguan sistem imun
3. Infeksi virus
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Gambaran Klinis
1.Ikterus sejak lahir
2.Ikterus akhir mggu I progresif
3.Feses acholik, urin gelap
Steatorrhoe osteomalacia
Gangguan pertumbuhan
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Tipe I : atresia terbatas pada ductus choledochus
Tipe II : atresia dustus hepaticus communis
Tipe III : atresia ductus hepaticus kanan dan kiri
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Diagnosis
• Pemeriksaan fisik
• Pipa lambung ke duodenum aspirasi
• USG
• Skintigrafi radio-isotop
• Biopsi hati perkutan
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Penanganan
Klinis / pem fisik
USG / skintigrafi
EKSPLORASI
LAPAROTOMI
SEBELUM
UMUR 8 MGGU
1. Kerusakan sel hepar / sirosis
2. Infeksi sekunder
3. Hipertensi portal
4. Splenomegali
5. Varices esophagus
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Penanganan
Tipe I
Tipe II
Tipe III
ANASTOMOSIS
PORTOENTEROSTOMI
(Kasai)
TRANSPLANTASI
HEPAR ???
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• Cholangitis : 40% fibrosis sel
hepar
• Portal hipertensi varices
perdarahan
Komplikasi
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• Prognosis jelek. Tipe I lebih baik
• Kemungkinan hidup 5 tahun hanya
40%
• Kematian karena kegagalan hepar
Prognosis
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Insidens
• Jarang
• Asia Timur dan Asia Tenggara relatif
lebih banyak ditemukan
• Jarang ditemukan di negara Barat
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Patogenesis
GANGGUAN PENYALIRAN EMPEDU
dan PANKREAS
1. Infeksi gangguan sphincter Oddi
2. Pelebaran duktus KISTA
Obstruksi
Cholangitis
Abses hati
Ruptur
Keganasan
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KLASIFIKASI KISTA DUKTUS KHOLEDOKHUS
1.Meliputi seluruh duktus kholedokus, duktus
hepatikus komunis
2. Divertikulum yang terpisah dari kandung
empedu dan saluran ekstrahepatik
3. Dilatasi kistik sal empedu di dinding
duodenum
4. Campuran
5. Dilatasi sal intrahepatik dan fibrosis hati
bawaan (peny Caroli)
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Gambaran Klinis
Kelompok Infantil (umur 3 bulan)
obstruksi seperti atresia biliaris
Kelompok lambat ( 9 tahun) nyeri,
teraba massa perut kanan atas,
ikterus
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Diagnosis
• Nyeri, massa intraabdomen, ikterus
• Bilirubin, alkali fosfatase dan amilase
meningkat
• USG
• Biopsi hati perkutan : kholangitis
• ERCP
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Penanganan
• Reseksi kista
• Menyalirkan empedu : sistoenteros-
tomi
• Pemasangan pipa empedu
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SUMMARY
• Waspadai bayi ikterik, fisiologis atau
patologis?
• Diagnosis dan tindakan cepat (sebelum 8
minggu) diperlukan pada atresia biliaris
• Prognosis atresia biliaris tidak baik
• Bayi ikterik lama juga dipikirkan kelainan
kongenital lain seperti kista kholedokhus