Congenital Anomaly of Digestive SystemPresenter:1. Novan Ardiansyah2. Ismail Harun ZihaParticipan:1. Kurnia Tejawati2. Marizca Saras Chitra3. Nunie Ismi AmriPreceptor:dr. Liza, Sp.B

Esophageal Atresia and Transesophageal FistulaType:Isolated EA: 8-10 %EA with TEF between proximal segment of esophagus and trachea: 1%EA with TEF between distal esophagus and trachea: 75-85%EA with TEF between both proximal and distal end of esophagus and trachea: 1-2%TEF without EA (H-type fistula):5-8%

Etiology:- The esophagus and trachea share a common embryologic origin fail to separate:Absent of sonic-hedhog signaling pathwayThyroid transcription factor one (TTF-1)Fobroblast growth factor (FGF-10)

Clinical presentation:Excessive droolingChocking or coughing after feedingAbdominal distention: caused by air pass into stomachDifficult to breathe: caesed by distentionAtelectasis pulmonary disfunctionC and D type: regurgitated gastric juice passes through fistula chemical pneumonitis

Diagnosis:Percussion of abdomen: airInability to pass orogastric tubeRadiography: dilated upper pouch, air in abdomen.Coexisting anomalies: cardiac defect, skeletal defect, neurological defect, anorectal decet, other.

Management:Initial: place in an infant warmer, head elevated 30, sump catheter, iv antibiotic, warmed electrolyte solution, search other defect: cardiac.Esophagoesophagostomy

Hypertrophic Pyloric StenosisOccurs in 1 in 300 live births, between 3 and 6 weeks of age.Etiology: has not been determined. Study have shown: familial link, Erythromycin in early infancy.

Clinical presentation:Prijectile nonbilious vomitingIntolerant to feed even clear liquidSevere dehydrationJaundice may occurLess flatusHypochloremic, hypokalemic, metabolic alkalosis.

Diagnosis:PE: inspection: visible gastric waves. Palpation: olive in RUQ, if cannot palpated use USGUSG: channel length of over 16 mm and pyloric thickness over 4 mm.

Management:Fluid resuscitationFredet-Ramstedt pyloromyotomy

Incidence: 1 in 2000 to 1 in 5000. male = femaleClinical presentation:Jejunal and ileal atresia: bilious vomiting and progressive abdominal distention.More distal more abdominal distention.

Diagnosis:Plain abdominal film: 1.Obstructed loop. 2.Duodenal atresia: double bubble. 3.Air-fluid level.Barium enema: DD: microcolon, meconium plug, small left colon syndrome, Hirschsprungs disease, or meconium ileus.Management: 1. decompression. 2. laparotomy

Malrotation and Midgut VolvulusDuring 6th week the mudgut grow rapid and prolapses into umbilical cord, and 10th 12th return to abdominal cavity undergoing 270 degree counterclockwise. If the rotation is incomplete, the caecum remains in the epigastrium, but the bands fixing the duodenum to the retroperitonium and caecum continue to form.

This result in (Ladds) bands extending from the caecum to the lateral abdominal wall and crossing the duodenum, which creates the potential for obstruction. Volvulus may occur around the mesentery resulting obstruction of proximal jejunum and infarction of the midgut.

Clinical presentation:Bilious vomitingBloody stoolCirculatory collapse.Erythema and edema of abdominal wall due to ischemia which progresses to shock and death

Diagnosis: abdominal film: air-fluid level, volvulus: corkscrew shape.

Management:Fluid resuscitationExploratory laparotomy.

Intestinal DuplicationMost common in ileumMay long and tubular, but usually are cystic masses.Clinical presentation:Recurrent abdominal painEmesis: obstructionHematochezia: ulceration or ectopic gastric mucose

PE: palpable massDiagnosis: CT-scan, USG, barium enemaManagement: resection: laparotomy

Meckels DiverticulumRemnant of portion of the embryonic omphalomesenteric (vitelline) duct.It is located on antimesenteric border of ileum, usually within 60 cm of the ileocaecal valve.It can contain heterotopic mucose: gastric mucose. Also pancreatic acini. Other: brunners gland, pancreatic islet, colonic mucose, endometriosis, hepatobiliary tissue

Clinical presentation:1. GI bleeding, maroon-colored stool.Management:Surgical: ileostomy

Mesenteric CystDuplication within the mesentery, but do not contain mucosa or muscular wall.Can cause intestinal obstruction and may present as an abdominal mass.Diagnosis: USG or CT-scanManagement: surgical excision

Hirschsprungs DiseaseCaused by a malformation in pelvic parasympathetic system which result in the absence of ganglion cells in Auerbachs plexus of a segment of distal colon.

Clinical presentation: functional distal intestinal obstructionAbdominal distentionFailure to pass meconium within 48 hoursBilious emesisConstipationComplication: enterocolitis: abdominal distention and tenderness, systemic toxicity: fever, failure to thrive, lethargy. Dehydrate, leukocytosis.

Rectal examination: forceful propulsion of foul-smelling liquid fecesDiagnosis: rectal biopsy: sample 1,2,3 cm from the dentate line. Barium enema: dilated colon.

Management:decompression: NGT, IVFD.AntibioticsRectal irrigationSurgery: pull-through

Anorectal Malformation