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  • 1.berbuih; 2.sianosis; 3.batuk dan sesak nafas; 4.pneumonia; 5.perut kembung; 6.oliguri; 7.kelainan jantung bawaan; 8.atresia rectum atau anus)

    Diagnosis

    Diagnosis dibuat sebelum bayi diberi minum, NGT akan terhenti pada jarak 10-12 cm foto polos thorax esophagus berdilatasi berisi udaraudara dalam lambung /usus fistula trakea - esophagus bagian distal.Endoscopy bronchoscopy

  • Diagnosis

    Penatalaksanaan

    Telungkup / sikap setengah duduk, kepala ekstensiAnak tanpa fistel posisi Trendelenburg Isap NGT/OGTObs suhu, respirasi, status cairan tubuh dan elektrolitAntibiotik.Operasi satu tahap Bertahap pada : premature bayi dengan anomaly berat gap yang cukup panjang

    Penatalaksanaan

  • Penatalaksanaan

    Komplikasi

    Disfagia Striktur anastomosisChondromalacia Refluks esofagitis pneumonitis aspirasi dan esofagitis rekuren.

    PrognosisBayi dgn anomaly yang berat, angka bertahan hidup dapat mencapai 95%.Tanpa komplikasi bertahan hidup 100%Kematian terjadi akibat komplikasi paru-paru, adanya anomaly berat lainnya, prematuritas dan sepsis

    KESIMPULAN

    AE kelainan kongenital penyebabnya belum diketahuiKelainan ini dapat berdiri sendiri / disertai kelainan lain, yang sering ditemukan VACTERL syndrome.Kesulitan yg terjadi adalah bahwa dugaan terhadap kelainan tersebut baru terpikirkan setelah selesai pemberian minum pada bayi, sehingga bayi sudah mengalami aspirasi. diagnosis dini prognosis dan mengurangi komplikasi Penatalaksanaan operasi satu tahap/ lebih

    Atresia usus

    PENDAHULUANAtresiaDefek kongenital sal. cernaStenosis

    Etiologi gangguan aliran darah

    USG prenatal Frekuensi >>>>

    G/ bergantung: penyebab, letak, waktu penanganan

    Atresia PilorusAutosomal

    G/ nonbilous vomiting, distensi abdomen

    R abdomen single gas bubble

    Th/ Eksisi atresia membranosa atau side-to-side gastroduodenostomy

    Atresia Duodenum 1/2500 kelahiran hidup

    Insidensi pada: - Sindroma Down - Perkawinan sedarah

    Standar OP duodenoduodenostomy tapering duodenoplasty

    Atresia Duodeni

    Lanjutan Atresia DuodeniEmbriologi & Etiologi Mgg 3 pembentukan sistem hepatobilier & pankreas, serta pemadatan duodenum Mgg 8-10 Rekanalisasi

    Gangguan rekanalisasi

    Atresia, Stenosis, & Web

    Klasifikasi & Patologi Stenosis diafragma / membran dgn lumen kecil Atresia obstruksi yang lengkap

    50% berhubungan dgn anomali lainKlasifikasi (berdasarkan lokasi obstruksi):Preampullaris Postampullaris

    Lanjutan Atresia Duodeni

    Diagnosis Polihidramnion

    USG antenatal

    G/ pada neonatus:Muntah proyektilDistensi abdomen bag. Atas, peristaltik lambung (+)50% mekonium dikeluarkanAspirasi NGT 20 cc

    R double bubble sign

    Lanjutan Atresia Duodeni

    Gambaran Radiologis Atresia Duodenum

    Foto Kontras Atresia duodenal

    Terapi NGT Dekompresi lambungTermoregulasi & resusitasi cairan

    OP :Duodenoduodenostomy >>>Duodenotomy + eksisi membran Pendekatan sisi transversa di supraumbilikus

    Post OP: Fungsi usus (+) R/ ASILanjutan Atresia Duodeni

    Atresia Usus

    Atresia Usus Halus(Jejunum & Ileum)E/ Iskemia Intrauterin

    Insidensi 1/1000 kelahiran hidup

    1/3 kasus obstruksi pd neonatus E/ Atresia usus

    EtiologiGangguan pembuluh darah terlokalisir (intra uterin)

    iskemia & nekrosis usus

    reabsorpsi segmen yang terkena Lanjutan Atresia Usus Halus

    Klasifikasi Atresia tipe I (membran) Web (+) dr lap. Mukosa & submukosaBag. Prox dilatasiBag. Distal kolapsPanjang usus normal Lanjutan Atresia Usus Halus

    Atresia tipe III

    - Paling sering ditemukan- Pemendekan usus (+)- Bag. Distal mengecil & terdekompresi

    Lanjutan Atresia Usus Halus

    Atresia tipe IIIa - Kedua ujung yang buntu terpisah - Defek mesenterium berbentuk V - Usus memendek- Kantung prox dilatasi, Viabilitas ???Lanjutan Atresia Usus Halus

    Atresia tipe IIIb (defomitas Apple-peel) - = tipe IIIA defek mesenterium >>- Bag. Distal kolaps, mengelilingi p.d.- Sering disertai Atresia tipe I & IILanjutan Atresia Usus Halus

    Atresia tipe IV (Multiple Atresias) - Kombinasi atresia tipe I, II dan III di beberapa tempat- E/ inflamasi saat intrauterin- Autosomal resesifLanjutan Atresia Usus Halus

    Patofisiologi

    Prox : - Dilatasi & Hipertrofi- vili normal, peristaltik < efektif- Def. Enzim mukosa & ATP otot Iskemia Lokal

    Bag. Atresia:- Ganglion atrofi & hiposeluler-

    Lanjutan Atresia Usus Halus

    Gejala Klinis Kehamilan dgn Polihidramnion

    Prematur & BBLR

    Tanda klasik bilious emesis, distensi abdomen, ikterus & gagal mengeluarkan mekonium dlm 24j pertama

    Dehidrasi

    Iritabel, letargi, komaLanjutan Atresia Usus Halus

    Diagnosis *G/ Distensi abdomen, bilious emesis, mekonium (-)*RO BNO polos

    Lanjutan Atresia Usus Halus

    Atresia Jejuni

    Atresia Jejuni

    Atresia IleumTampak mikrokolon

    Lanjutan Atresia Usus Halus (Penatalaksanaan) Kondisi metabolik & hemodinamik harus stabil

    Pendekatan bedah : incisi transversa di supraumbilikal

    Lanjutan Atresia Usus Halus

    PROGNOSIS

    Bergantung jumlah fungsi usus yg tersisa

    Lanjutan Atresia Kolon Pengobatan

    OP bergantung : lokasi & luasnya atresia, klinis

    OP Reseksi bag. Atresia dibuat kolostomi & fistula mukosa

    Lanjutan Atresia Kolon Diagnosis

    USG prenatal ukuran kolon lebih >

    G/ : distensi abdomen, dilatasi bag. Proksimal Foto polos abdomen:* Obstruksi bagian distal* Dilatasi bag. Proksimal

    KESIMPULAN Atresia usus Kel. Kongenital

    Polihidramnion USG prenatal

    D/ gejala klinis & pemeriksaan radiologi

    D/ dini perlu dilakukan

    MAR occur in approximately 1 : 4000 to 1 : 5000 newbornsMost common defect in males imperforate anus with retro urethral fistula, retro perineal fistula (low defects)10% retro bladder neck fistula (highest defect) in male

    A. Cutaneus Perineal Fistula :Lowest & simplest defect of the spectrum center of the sphincterManifestation perineal defect :Subepithelial midline tractOpening somewhere along midline perineal raphescrotum or base penisProminent midline skintag Buchet HanelleAnal membraneThe sphincter & the sacrum usually very good (continens)

    B. Recto urethral Fistula :Most common fistula opens into posterior urethra (retro urethral bulbar fistula)Sacrum & sphincter usually goodLess commonly sacrum opens into upper position posterior urethra retro urethral prostatic fistula; the sphincter is less developed & sacrum shows abnormalities & dysmorphism

    C. Recto Bladder neck fistula (Rectovesical fistula)Rectum opens into bladder neck true supralevator malformationThe sphincter mechanism poorly developedThe sacrum shows sign of severe hypoplasia and/or dysmorphismPerineum often flat & entire pelvis seems to be underdevelopedIncidence of associated defects extremely high

    D. Imperforate Anus without fistula - rectum ends completely blind approxi mately 2 cm above perineal skin - > 90% patient with Down Syndrome - this patient have : = good sphincters = good sacrum = good clinically prog nosis

    E. Rectal Atresia - born with normal canal - perineum looks normal - recognized during attempt to pass thermometer in the rectum - Anal canal separated by thin membrane / dense portion of fibrous tissue - occurs approximately 1 % - prognosis excellent

  • A. Cutaneous defects- rectum opens externally through an abnormal, usually narrow fistulous tract, located somewhere between female genital & center of the sphincter- prognosis & frequency of a associated defect = male

    B. Vestibular fistula - rectum opens within the female genitalia - located outside the hymen - the precise diagnosis clinical & inspec- tion of newborn genitalia - good prognosis

  • C. Imperforate Anus without fistula PERSITENT CLOACARepresent the most complex malformationDefined defect in which rectum, vagina & urinary tract meet and fuse into a single Common ChannelThe length common channel from 1 10 cm correlates with : + complexity of malformation + the final functional prognosis

    Short common channels represent defectsrelatively easy to repair good functional prognosisLong common channels very high defects difficult technical challenge less than optimal functional results

    The turning point around 3,5 cm in length

    Frequently suffer from atresia cervix or atresia fallopian tubesShort Common Channel : - have good sphincter mechanism - normal sacrum - low frequency of associated defects - good functional prognosis

    Long Common Channels : - flat perineum - poor sphincter development - very hypoplastic sacrum - high incidence of associated defects (mainly urologic) - poor prognosis for bowel & urinary control

    Simple but good inspection of the perineum is mandatory provides the most valuable informationHow many orificiums ?Observe 18 24 hours. If no fistula Knee Chest Position photo : < 1 cm low type > 1 cm high type

    .Low type Anoplasty (local anesthesia) High type Colostomy 4 6 months PSARP