abe dani boby hisprung
DESCRIPTION
hirschprung diseaseTRANSCRIPT
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HIRSCHSPRUNG’S DIS-EASE
Syahmardani/sumrahadi/bobby
PEMBIMBING :dR. Muntadhar Sp.BA
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Patofisiologi
Proses embriologi usia 5 – 12 mgg kehami-lan => migrasi sel dr neural crest yg ter-hambat teori migrasi craniocaudal
75% :Segmen pendek => aganglionik rek-tum sampai colon sigmoid Hirschsprung klasik
15 % :Segmen panjang => melebihi colon sigmoid
3 % : total colon => kolon aganglionik total2 % : total colon + Ileum terminal =>
aganglionosis universalSegmen ultrashort
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Epidemiologi :
• Insidensi :1 tiap 5000 kelahiran hidup
• Tidak tergantung umur ibu a/ faktr kelahiran
• Herediter (familiar) dng resesif autosomal dan sex linked ; Down syndrome.
• Pria : wanita = 4 : 1
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Manifestasi klinis :
Pada neonatus : • Obstruksi intestinal totalis => muntah
bilier, obstipasi, distensi abdomen• Pasase mekonium yang terlambat ( N =
24 jam pertama) , biasanya disertai muntah bilier dan distensi abdomen => trias klasik
• Enterokolitis => diare mengandung darah dan lendir (bau dan warna khas ok pertumbuhan bakteri yg berlebihan pd colon yg iskemi) disertai muntah dan distensi abd
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Manifestasi klinis :
Pada bayi dan anak• Konstipasi kronis • Defekasi dng interval tdk teratur
dan susah• Konsistensi feces keras atau lem-
bek spt pasta & sangat bau
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Rectal toucher :
• Ujung jari terasa terjepit lumen rektum
• Penarikan jari => feces encer menyemprot (pd bayi)
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Diagnosa
• Anamnese perjalanan penyakit yg khas
• Pemeriksaan klinis • Pemeriksaan penunjang : a. Radiologi b. Biopsi c. Manometri
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• Barium enema (kriteria Bordon dan Baker) => zona transisional spt corong ( bagian
yng menyempit ke daerah proksimal yng
dilatasi) => kontour iregular sbg filling-defect spt
gigi gergaji (kontraksi iregular)
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Biopsi :
• Blind suction biopsi atau Punch biopsi, bila ragu biopsi operasi
• Suction biopsi jarak 2, 3 dan 5 cm dari anus (pd bayi jarak 2 dan 3 cm) => krite-ria D/ :
- sel-sel ganglion (-) pd submukosa - Truncus saraf yg besar (+) pd submu-
cosa - peningkatan aktifitas asetil-kho-
linesterasi pd serat saraf parasimpatis di muscularis mucosa & lamina propria (pewarnaan / histokimia)
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Diagnosa Banding :
• Atresia ileum• Sumbatan makanan• Atresia Rektal• Enterokolitis nekrotikan neonatal• Peritonitis intrauterin• Neonatus dengan sepsis• Sindroma kolon kiri kecil• Obstipasi Psikogenik
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Pengobatan• Prinsip : mengatasi obstruksi, mencegah
enterokolitis (sementara) , reseksi segmen aganglionik, mengembalikan kontinuitas usus (definitif)
• Penanganan darurat obstruksi• A. Sementara : => Wash out ( irigasi NaCl hangat 15- 20
cc melalui kateter 16 atau rektal tube) vol masuk = keluar
bahaya : perforasi => Colostomy : Indikasi : wash out gagal, enterocolitis, distensi >>, malnutrisi
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Pengobatan
• B. Definitif => BB sudah cukup > 9 kg (6 bln)
1. Swenson 2. Duhamel & Soave 3. Rehbein, Lynn
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TERIMA KASIH