bronkogenik karsinoma
DESCRIPTION
radiologiTRANSCRIPT
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Pembimbing :Dr. Lisa Irawati Sp.Rad
Fakultas Kedokteran Universitas Tarumanagara Kepaniteraan Klinik Ilmu Radiologi
Rumah Sakit Husada JakartaPeriode 17 Februari 2014 – 15 Maret 2014
Oleh :Cindy Christine
406118003
Referat
Hirschsprung’s Disease
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Anatomi
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Hirschsprung’s disease
Kelainan kongenital di colon
Tanda : Sel ganglion parasimpatis di: • pleksus submukosus Meissneri (-) • pleksus Myenterikus Auerbachi.
(-)
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• Terjadi dalam 1 : 5000 kelahiran,
• Diturunkan : ibu aganglionosis > ayah
• Ratio laki-laki 3-5 : perempuan 1.
• Resiko tertinggi :
• Riwayat keluarga (+)
• Penderita Down Syndrome.
• Bagian yang paling sering terkena:
• Recto-sigmoid + 75 kasus,
• Colon transversum atau flexura lienalis + 17 kasus.
Epidemiologi
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• Ketiadaan sel ganglion
• Mutasi pada RET Proto-oncogene
• Kelainan pada lingkungan
• Matriks Protein Ekstraseluller
Etiologi
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Sindrom Down Sindrom Neurocristopathy Sindrom Waardenburg-Shah Sindrom buta-tuli Yemenite Piebaldism Sindrom Goldberg-Shprintzen Neoplasia endokrin multiple tipe II Sindroma hipoventilasi kongenital terpusat Cartilage-hair hypoplasia Sindrom hipoventilasi entral primer (Ondine’s curse) Penyakit Chagas, pada penyakit ini Trypanosoma menginvasi
langsung dinding usus dan menghancurkan pleksus.
Beberapa kelainan kongenital lainnya yng sering disertai Hirschsprung disease , yaitu :
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• Hipoganglionosis
• Imaturitas dari sel ganglion
• Kerusakan sel ganglion
Patofisiologi
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Tipe Penyakit Hirschsprung Recto-sigmoid aganglionosis
Short or ultra short aganglionosis
Long segment Total colonic aganglionosis
Aganglionosis sampai ke bagian usus kecil dengan varian PH
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Diagnosa
•pengeluaran mekonium > 24 jam setelah lahir, distensi abdomen, gangguan pasase usus, poor feeding, vomiting, kegagalan pertumbuhan, enterokolitis, faktor genetik.
Anamnesa
•Periode Neonatal. Trias : pengeluaran mekonium yang terlambat, muntah hijau dan distensi abdomen. Gejala berupa diarrhea, distensi abdomen, feces berbau busuk dan disertai demam.
•(ii). Anak. konstipasi kronis dan gizi buruk, terlihat gerakan peristaltik usus di dinding abdomen. Colok dubur: feces keluar menyemprot.
Gambaran Klinis
•Radiologi :Foto polos abdomen (BNO), Barium enema
•Pemeriksaan patologi anatomi
•Manometri anorektal
Pemeriksaan
Penunjang
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Gambaran Klinis seorang bayi dan anak yang menderita Penyakit Hisprung :
Distensi abdomenGizi Buruk
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Diagnosis
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Hir
sch
sp
run
g's
dis
ease
( sh
ort
seg
men
t )
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TCA
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Clinical, Histopathologic, and CT Findings of Adult HD and Adult HG
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Contrast-enhanced transverse CT scans (a obtained at lower level than b) obtained in 31-year old woman with HG of entire colon show markedly dilated feces-filled ascending colon (AC) and transverse colon (arrowheads) compared with a nondilated descending colon (DC) with a transition zone of the proximal descending colon (_).
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Double-contrast barium enema radiographs (a, anteroposterior; b, oblique anteroposterior views) obtained in 64-year-old woman who hasHDwith aganglionic segment of rectosigmoid junction show markedly dilated distal sigmoid colon and rectosigmoid junction (arrowheads) and a narrowed rectum (arrows).
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Gambaran Histopatologi pada Penyakit Hisprung
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Manometri anorektal
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Diagnosa Banding
Obstruksi mekanik
• Meconium ileus• Simple• Complited (with
meconium cyst or peritonitis)
• Meconium plug syndrome
• Neonatal small left colon syndrome
• Malrotation with volvulus
• Incarcerated hernia• Jejunoileal atresia• Colonic atresia• Intestinal duplication• Intussusception• NEC
Obstruksi fungsional
• Sepsis• Intracranial hemorrhage• Hypothyroidism• Maternal drug ingestion
or addiction• Adrenal hemorrhage• Hypermagnesemia• Hypokalemia
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Meconium ileus
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Meconium plug syndrome
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Incarcerated Hernia
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Neonatal small left colon
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Malformation with volvulus
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Jeju
nal A
tresia
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Jeju
nal A
tresia
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NEC
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Tatalaksana
Pre-operatif
Tangani distensi abdomen :
Pemasangan pipa anus atau
pemasangan pipa lambung
Irigasi rektum. Antibiotik (pencegahan infeksi
terutama untuk enterokolitis dan
sepsis)Infus (menjaga kondisi
nutrisi serta keseimbangan
elektrolit dan asam basa tubuh)
Enterokolitis resusitasi cairan agresif, antibiotic
spekrum luas, dekompresi usus.
Bedah
Kolonostomi
Bedah definitive
Prosedur Swenson
Prosedur Duhamel
Prosedur Endorectal Pull Through (Soave)
Prosedur Boley
Prosedur Rehbein
Prosedur miomektomi anorektal
Prosedur Transanal Endorectal Pull-
Through
Posterior Sagital Neurektomi Repair for Hirschsprung disease
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Komplikasi • Perdarahan, • Infeksi,• Perlukaan pada organ sekitar serta risiko
anaestesi.
Komplikasi bedah pasca
operasi• Retraksi stoma• Striktur• Prolaps dan ekskoriasis kulit. Komplikasi
kolostomi :
• Obstruksi• Inkontinensi • Enterokolitis (pada 50% kasus )
• Insidens : 27,6% tipe segmen pendek; 37,8% pada segmen panjang.
Komplikasi lainnya :
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Perubahan pada komponen musin dan sel neuroendokrin, kenaikan aktivitas prostaglandin E1, infeksi Clostridium difficile atau rotavirus
Iskemia mukosa dengan invasi bakteri dan translokasi
Enterokolitis : demam, muntah hijau, diare hebat, distensi abdomen, dehidrasi dan syok
Ulserasi da nekrosis
Sepsis, pnematosis dan perforasi usus
Enterokolitis
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En
tero
coliti
s w
ith
H
irsch
pru
ng
dis
ease
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Baik
Gejala obstruksi diatasi
Prognosis
Baik
Dapat diatasi
Penyulit pascabedah :
kebocoran anastomosis, atau striktur
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• PH : penyakit anomali kongenital yang bila ditegakkan secara dini dan ditangani secara tepat dapat menghasilkan prognosis yang baik.
• Etiologi : absennya sel ganglion pada pleksus mienterik (Auerbach) dan pleksus submukosa (Meissner)
• Diagnosis : anamnesis, pemeriksaan fisik, gejala klinis dan pemeriksaan penunjang.
• Gejala klinis khas : mekonium keluar > 24 jam, distensi abdomen, obtipasi kronik.
• Anak : kesulitan makan, distensi abdomen yang kronis dan riwayat konstipasi berulang serta gagal tumbuh kembang.
• Beberapa kasus : diare tanda enterokolitis kematian
Kesimpulan
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• Tanda enterokolitis : demam, muntah berisi empedu, diare yang menyemprot serta berbau busuk, distensi abdominal, dehidrasi dan syok.
• Pemeriksaan penunjang : radiologi, anorektal manometri, dan pemeriksaan histopatologi.
• Pemeriksaan radiologi : foto polos abdomen (melihat adanya tanda obstruksi usus letak rendah (setinggi ileum terminal atau lebih rendah lagi)), foto barium enema ( gambaran segmen sempit, segmen transisi dan segmen dilatasi).
• Anorektal manometri : hiperaktivitas segmen yang dilatasi, tidak didapati kontraksi peristaltik yang terkoordinasi pada segmen usus aganglionik, dan tidak dijumpai relaksasi sfingter interna setelah distensi rektum akibat desakan feses.
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Terima Kasih