kelainan kongenital urethra
DESCRIPTION
menjelaskan kelainan bawaan pada uretjhraTRANSCRIPT
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Kelainan Kongenital Urethra
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Posterior Urethral Valve
Penyebab utama BOO Morgagni 1717 Hough Hampton Young 1919
• Mukosa memanjang dari dasar veromontanum ke distal sepanjang urethra pars membranacea dan prostatika
Tipe I
• Valvula meluas ke proksimal dari veromontanum sampai ke leher buli-buli
Tipe II
• Valvula mengelilingi urethra dengan lubang sentral di distal (IIIa) atau proksimal (IIIb) dari veromontanum
Tipe III
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Posterior Urethral Valve
Insiden laki-laki Insidens 1:5000-8000Type I > 95 %Type III < 5 %Prognosis lebih buruk pada type IIIVesicoureteral refluks 50 %
50 % unilateral, 50 % bilateral
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Posterior Urethral Valve
Gambaran Klinik Tergantung derajat obstruksi
Antenatal Hidronefrosis bilateral Distensi kandung kemih serta dinding yang menipis Dilatasi uretra pars prostatika Oligohidramnion
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Posterior Urethral Valve
Newborn Teraba massa intra abdominal
o Distensi kandung kemih, Hidronefrosiso 40 % akibat obstruksi uropathy
Ascites Riwayat oligohidramnion Gangguan napas akibat hipoplasia pulmoner
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Posterior Urethral Valve
Early infant Pancaran kencing lemah / terminal dribbling Urosepsis Dehidrasi Gangguan elektrolit Uremia Gangguan ginjal
Toodler Fungsi Ginjal lebih baik Demam (UTI) Inkontinensia
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Posterior Urethral Valve
Initial Management Drainage urine
Feeding tube 5 / 8 fr Kateter foley tidak dianjurkan Antibiotik spektrum luas Kimia Darah
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Posterior Urethral Valve
Pemeriksaan Radiologi Saluran Kemih Bawah
VCUG Mandatory Dilatasi uretra pars prostatika
USG IVU
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Posterior Urethral Valve
Tata laksana Trans-Urethral valve ablastion
4, 8, & 12 Urethral Spinkter VCUG setelah 2 bulan USG
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Anterior Urethral Valve
Insiden jarang Obstruksi saluran kemih bawah Urethra pars bulbosa (40 %) Terletak di distal divertikulum Kegagalan penyatuan uretra proksimal dan distal
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Anterior Urethral Valve
Gambaran Klinik Tergantung usia dan derajat obstruksi Antenatal (USG)
Oligohidramnion Hidronefrosis bilateral Dilatasi kandung kemih
Anak Pancaran urin yang lemah Menetes ISK berulang
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Anterior Urethral Valve
Pemeriksaan Radiologi USG VCUG / MCU
Tata Laksana Ablasi Transurethra Open surgical
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Striktur Urethra Kongenital
Jarang>> fossa naviculare dan uretra pars
membranacea Derajat Penyempitan :
• Oklusi < 1/3 lumen
Ringan
• Oklusi 1/3 – ½ lumen
Sedang
• Oklusi > ½ lumen
Berat
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Striktur Urethra Kongenital
Gejala dan Tanda Klinis Gejala iritasi dan obstruksi Udem penis Abses / fistel
Pemeriksaan Penunjang Laboratorium Uroflowmetri Uretrografi Uretroskopi Sistoskopi
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Striktur Urethra Kongenital
Tata Laksana Non-operatif
bouginasi Operatif
Meatotomi Internal uretrotomi uretroplasti
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Megalouretra
Nesbitt 1954 Dilatasi uretra tanpa obstruksi di distal 90 kasus Perkembangan abnormal korpus spongiosum dan kavernosum Umumnya pada Prune-Belly Syndrome Dua tipe
Skapoid Fusiform
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Megalouretra
Etiologi Kegagalan rekanalisasi uretra Obstruksi temporer awal perkembangan tipe fusiform Kegagalan perkembangan jaringan erektil tipe skapoid Abnormalitas perkembangan mesodermal
Pemeriksaan Penunjang USG VCUG
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Megalouretra
Penanganan Prenatal : Shunt vesico – Amnion Neonatus : Vesikostomi / uretrostomi Dewasa : Prostesis pada tipe fusiformis
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Atresia Urethra
Jarang lahir hidup Ditemukan saat prenatal dengan USG
Gangguan perkembangan paru Oligohidramnion Ginjal yang membesar Abdomen yang melebar Dilatasi organ-organ traktus urinarius Kematian fetus
Penatalaksanaan : shunt vesico-amniotik prenatal
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Duplicatio Urethra
Cenderung pada laki-laki Klasifikasi Effmann :
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Duplicatio Urethra
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Duplicatio Urethra
Penanganan