CONGENITAL ANOMALIES OF THE GASTROINTESTINAL TRACT
Written by Rhiana Devadasan
Oesophageal atresia
Definition = absence or abnormal narrowing of the oesophagus' lumen.
Presentation:
- Upper oesophagus ends in a blind proximal pouch and does not connect with the lower oesophagus and stomach.
- Normally also consists of a distal tracheo-oesophageal fistula (connection between oesophagus and trachea)
Signs & symptoms:
- Present with frothy saliva and cyanotic episodes, exacerbated by any attempt to feed
Associated anomalies
- Associated anomalies are common including cardiac, renal and skeletal defects
Surgical treatment:
- Right-sided extra-pleural thoracotomy (cutting of/incision into thorax) within a day or 2 of birth.
- Fistula (small connection between esophagus & trachea) is divided and tracheal side is sewn
- Oesophageal ends then anastomosed
- Usually good prognosis
Congenital diaphragmatic hernia
Definition = a defect in the diaphragm in which the diaphragm failed to close during prenatal development, resulting in the contents of the abdomen (stomach, intestines, liver, etc.) migrate into the chest through this hole.
Presentation:
- Typically, there is a left-sided posterolateral diaphragmatic defect associated with herniation of abdominal viscera into the chest and pulmonary hypoplasia (underdevelopment of lungs)
Surgical treatment:
- Prognosis is dependent on severity of pulmonary hypoplasia
- Despite intensive respiratory support, up to 30% of babies born with this condition die from neonatal respiratory failure
- If the infant can be adequately oxygenated and stabilised, the diaphragmatic defect can be repaired.
- Attempts to salvage severely affected infants by foetal surgery have not yet improved overall survival rates.
Intestinal atresia
Definition = complete blockage or obstruction anywhere in the intestine.
Clinical Presentation:
- Prenatal ultrasound scan findings show a ‘double bubble’ in the fetal abdomen together with maternal polyhydramnios (excess of amniotic fluid in amniotic sac)
- It is associated with down’s syndrome
Surgical treatment:
- Duodenoduodenostomy – anastomosis to bypass the obstructed segment of the duodenum.
- Proximal bowel is usually extremely dilated and must be tapered (decreased) prior to anastomosis.
Intestinal malrotation
Definition = an abnormality that can occur early in pregnancy where a baby's intestines don't form into a coil in the abdomen, but rather twisting, which causes obstruction.
Normally:
- During 12th week of gestation the midgut begins rotating counterclockwise around the superior mesenteric artery axis
Congenital anomaly: