Diagnosis

Intestinal atresia

Etiology

Cause [bl6][wua]

1. The intestinal lumen of the embryo is reestablished by the gathering of vacuoles, and recanalization occurs

2. Insults during the period of development --> failure of recanalization --> atresia, stenosis, and webs

   • Intrauterine thromboembolic occlusion or kinking of mesenteric vessels

   • Ischemic necrosis of the fetal bowel

   • Blind proximal and distal bowel with a mesentery defect between the ends

Pathophysiology [wua]

1. Defective peristalsis between proximal and distal blind ends

2. Risk of mucosal necrosis of the blind ends

3. GI obstruction, abdominal distension, emesis

Associated conditions

• Down syndrome, Esophageal atresia, Imperforate anus, Small for gestational age, Polyhydramnios, Cystic fibrosis, congenital heart disease, bowel rotational abnormality, annular pancreas, esophageal atresia, anorectal atresia and genitourinary conditions.

Risk factors [wua]

• Pregnant mothers exposed to vasoconstrictive drugs and cigarette smoking

Complications [wua]

• Emesis

• Electrolyte imbalance

• Aspiration pneumonia

• Sepsis

• Anatomic leak

• Adhesive bowel obstruction

• Short bowel syndrome

Epidemiology

Incidence per 100.000 [stc][pvn]

Approach

• Prenatal diagnositic: prenatal ultrasound or fetal MRI (suspected if polyhydramnios) [pvn]

• Blood tests: RBC, WBC, hemoglobin, hematocrit, electrolytes, creatinine, bilirubin, blood culture

• Arterial blood gas

• Abdominal ultrasound

• Kidney ultrasound

• Abdominal x-ray with contrast

• Fetal echocardiogram

• Rectal biopsy

Treatment

1. Stabilization after delivery: oxygenation [pvn] [bl6]

2. Nasogastric tube for decompression

3. Intravenous lines in the umbilical artery and vein for fluid/nutrition

4. Further diagnostics of needed: malrotation, cardiac, and/or respiratory abnormalities

5. Surgical repair: [wua]

   • Resection of proximal dilated and atretic bowel with primary end-to-end anastomosis between proximal and distal bowel

   • Temporary ileostomy if there is questionable bowel viability

Differential diagnoses

Hirschsprung's Disease, Imperforate anus, Intestinal malrotation, Intussusception, Meconium ileus, Volvulus