hirschprung's disease
TRANSCRIPT
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dr. SEVINA MARISYA, MKED(ped), SpAdr. SEVINA MARISYA, MKED(ped), SpA
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LATAR BELAKANG Kelainan kongenital,Ruysch 1691, Harold Hirschprung 1886 Gagal kembang pleksus parasimpatis Auerbach,Meisner Rectum, kolon. Insidens 1 : 5000 kelahiran hidup Laki-laki : Wanita 4 : 1
1994,Gen RET , EDNRB ,EDN-3 Gangguan neurocristopathy, gangguan migrasi
LOKASI: 80% recto-sigmoid Dapat seluruh kolon jarang Gangguan peristaltik konstipasi,obstruksi usus Problem Nasional: 1200 bayi HD lahir di Indonesia.
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Normal
Sel neuroenterik bermigrasi dari krista neuralis ke GITdari oral ke anal,-esofagus (mgg ke V),
-midgut (mgg ke VII)
-kolon distal (mgg ke XII)
HD
migrasi sel neuroblas terhenti sebelum sampai rektum-segmen aganglionik.
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Penyebab kelainan ini diperkirakan akibat :
- neuroblast gagal untuk migrasi
- Neuroblast gagal untuk menjadi matur
- Neuroblast mengalami degradasi atau destruksi
1994 : Gen yang berpengaruh: RET, EDNRB, EDN-3, Glial cell line-derived neurotrophic(GDNF),
NEURTURIN, ENDOTHELIN CONVERTING ENZYM 1 (ECE 1), SOX 10
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Normalterdapat 2 pleksus yaitu : Auerbach : intramural
Meissner : submukosa
Hirschsprung DiseaseSerabut saraf hipertropi,Gangguan peristaltikKolon proksimal menjadi hipertropiObstruksi fungsionalEnterokolitisHipoproteinemia
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ULTRA SHORT – SEGMENT :
< 3 cm.
SHORT –SEGMENT
RECTO-SIGMOID : 75%-80 %
LONG – SEGMENT : 20 %
TOTAL COLONIC –
AGANGLIONIC : LESS 5 %
POPULASI TARGET:
TIPE SHORT SEGMEN
Gambar
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AnamnesisKeterlambatan mekoneum ( 24 -48 jam)Kembung,muntah,konstipasi,malnutrisi
Pemeriksaan FisikDistensi abdomenColok dubur: mencengkeram,dilepas nyemprot.
.Rontgent: zone spastik,zone transisi,zone dilatasi.
.PA:Auerbach,Meissner :absen
.Manometri
.Imunohistokimia: -enolase spesifik neuron,protein S 100
.Histokimia :-pewarnaan asetilkolinesterase
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Komplikasi Mortalitas bila tidak ditangani: 80% Mortalitas yang ditangani : 30% karena
enterokolitis Obstruksi Intestinal Konstipasi Enterokolitis Malnutrisi
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Prinsip: PEMBEDAHAN
Dua tahap:
-kolostomi
-Pull through
Transabdomen
Satu tahap:
-Tanpa kolostomi
-Transintergluteal
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Penanggulangan Pertama:
1. Dekompresi :pipa rektum,pipa lambung
2. Tiga Stabilitas : cairan ,elektrolit, asam
basa dan temperatur
3. Nutrisi
4. Kolostomi
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Prosedur Swenson: 1948, 1964. Duhamel: 1956.
Modifikasi:Grob,Talbert,Ravitch,Ikeda,Adang
Rehbein Soave; 1960 Transanal
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Duhamel
SOAVE- Bolley
Rehbein
Swenson 1
Swenson 2
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Kebocoran Anastosmosis = leakage 5% Cuff abses 5% Infeksi jaringan 10% Striktur 10% Enterokolitis Inkontinensia Ileus 5%(Lee,S., 2003) MORTALITAS: Swenson:2,5%,Duhamel:6,2%
Soave :4,5%(Swenson,O.2005)
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Tergantung tipe HD Skill Operator Kondisi Pasien Umur saat dilakukan operasi Prosedur Persiapan Operasi, Antibiotika Perawatan Pasca Operasi
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