Text Book Reading Dr. Iwan S.

MECONIUM ILEUS
Pediatric Surgery Jay L. Grosfeld

MECONIUM ILEUS

Landsteiner (1905): observasi obstruksi usus halus penderita dg kelainan pankreas ada hubungan dengan def enzim

cystic fibrosis 20,8% penderita di AS

Etiologi unknown genetic

PATHOGENESIS

Mulai terjadi di intrauterin sebagai hasil akumulasi mekonium viskositas tinggi dan padat: Defisiensi enzim pankreas dan sekresi mukus yang kental oleh kelenjar usus abnormal akumulasi dan obstruksi mekonium intraluminal komplikasi

GAMBARAN KLINIK

Uncomplicated meconium ileus Segera setelah lahir, ditandai dgn distensi abdomen, mekonium yang kental menyumbat usus, muntah hijau, tidak bisa BAB.

Complicated mec ileus, Intrauterin atau setelah lahir perforasi dan atau nekrosis usus: gambaran kalsifikasi berbentuk bulan sabit atau butiran-butiran intra abd, peritonitis, eritem atau edema ddg abd dan atau nyeri tekan abd. Simple mec ileus

Bayi atau dewasa muda mec ileus equivalent or distal ileal obstruction syndr

Riwayat CF 10%-33%, amniosintesis accurate diagnosis Serial USG intrauterin intestinal obstr of mec ileus (about 20% of the CF population) with or without a complicating meconium cyst.

Polihidramnion menggambarkan adanya mekonium ileus intrauterin, yang berasal dari obstruksi usus berat. simple atau uncomplicated: peritonitis (-)

Pemeriksaan fisik Distensi abdomen pada saat bayi lahir Peristaltik usus terlihat dan teraba, kadang terlihat gambaran usus PUTTY SIGN; palpated mass RT: Tidak ada mekonium yang keluar Teraba massa, perubahan warna ddg abd, dan tanda-tanda peritonitis Hypovolemia NGT kehijauan > 20 ml

RADIOLOGI

USG: Echogenic bowel wall in the third trimester Plain abdomen, supine and erect films: a. Perbedaan ukuran lekukan usus b. No or few air-fluid levels c. Gambaran butiran, soap bubble or groundglass sering terlihat pada abd kanan. Contrast enema (barium, gastrografin, or any water-soluble)microcolon or unused colon

Laboratorium

Analisa tripsin dan chymotrypsin feses Trypsin < 80 mg/g CF immunoreactive trypsinogen darah

DIFF DIAG Neonatal intestinal obstruction: Ileal atresia Hirschsprungs disease Neonatal small left colon Meconium plug syndrome

MANAGEMENT Non operative

Tergantung dari inspissated intraluminal meconium dan patensi dari ileokolon. Noblett described : 1. contrast enema distal intestinal obst 2. comp of volvulus,atresia,perforation,peritonitis excluded 3. enema with careful fluroscopic control 4. Intravenous antibiotic 5. Pediatric surgeon 6. full fluid resuscitation 7. Should be prepared for imminent operation

Gastrografin enema meglumine ditriazone(tween 80),40% sodium diatrizoat

solubilizing enema treatment guidelines Noblett fluoroskopic guidance dan pemberian larutan gastrografin 50% dalam air abdominal radiograph diulang dalam 8-12 jam obstruksi (-) The success of nonoperative treatment is variable

Operative management Simple Meconium Ileus

a.

b. c. d. e.

Tujuan menghilangkan obstruksi: evakuasi mekonium atau reseksi bagian yang mengandung inspissated material Enterotomies with irrigation coupled with a limited resection The Miculicz resection and enterestomy The Bishop-koop resection and enterostomy The Santulli enterostomy Tube enterestomy

Kriteria Bishop-koop prosedur 1. Limit intaoperative bowel trauma 2. Resect disparately ileal loop 3. Create appropriately sized end to side anastomosis 4. Inserting catheter into distal bowel 5. Enterostomy closure by ligation

chimney stoma

Santulli a proximal chimney enterostomy, an operation that in essence is the reverse of the resection coupled with a distal chimney enterostomy

Complicated Mec Ileus

Hampir selalu dgn operasi Indikasi : persisting intestinal obst, enlarging abdominal mass, sign of peritonitis, dan clinical and lab sign of ongoing sepsis

 Meconium peritonitis meconium accumulating in peritonial cavity A calcified pseudocyst fibrous wall and spared bowel loop

At operation mandatory asses residual intestinal length

Postoperative management Support of the infants general physiologi

Komplikasi
Gasrointestinal
a. Intussuception b. Rectal prolapse c. Colonic stricture

Pulmonary
a. Bacterial sepsis b. Bronchopnemonia

Inguinoscrotal Disease
a. Hernia and hydrocele b. Cryptorchidism

TERIMA KASIH