GI Malformation - Mordechai Flashcards
Congenital Diaphragmatic Hernia
Etiology, properties
Left side of coelum closes after the right so hernia mroe common on this side. Bilateral is very rare.
Lungs compressed by bowel and can’t develop.
The survival of infants with diaphragmatic hernias dependent in large part on the size and degree of maturity of the lungs and reversibility of pulmonary hypertension. Pulmonary hypertension causes vasospasm which increases pressure, eventually greater than systemic pressure so blood doesn’t enter the heart or lungs and creases a R->L shunt causing hypoxia and acidosis.
Treatment of Congenital Diaphragmatic Hernia
Avoidance of further enlargement and pressure of the viscera within the chest
NASOGASTRIC TUBE
The provision of endotracheal ventilation
The avoidance of barotrauma
High frequency ventilation
Control of the blood gases
Alkalosis (prevent vasospasm caused by acidosis)
pCO2 ~ 25-30 Torr - this will tend to reduce the degree of pulmonary vasospasm
Large volumes of IV fluids are contraindicated
Circulation must be maintained to provide an aortic pressure which will prevent Rt-to-Lt shunting
Delayed operation until stable
Controlled pulmonary hypertension
Direction of shunt
Optimal oxygenation
Nitric Oxide
ECMO (give oxygen through heart)
Esophageal atresia
Epidemiology, types, features
Incidence - 1 : 3000 live birth
Aetiology is unknown
50% of patients have associated congenital anomalies
Often associated with tracheoesophageal fistula
a and b) Can’t swallow air, airless abdomen
c) Most common: distal fistula, air through fistula goes to the GI tract
d) double fistula: proximal and distal
e) fistula without atresia (H-type)
Polyhydramnios prenatally
Stomach is empty on U/S
Drooling or unable to swallow postnatally
Cyanosis during feeding
Aspiration pneumonia (if diagnosed after feeding)
A nasogastric tube can not be passed
Management of Esophageal Atresia
Withholding of feeding
Suction from upper esophageal pouch
Head is raised (avoid reflux)
Antibiotic covering
Search for associated anomalies
Operation
Surgery: Don’t enter pleural cavity, push pleura aside. Enastamose the pouches. If not: graft with a piece of colon. Pull stomach into posterior mediastinum.
Treatment of omphalocele
Stabilization & AB
Primary closure
Secondary closure after plastic mesh/silastic chimney placement-to prevent compartment syndrome by squeezing too much bowel back in.
Pyloric Stenosis
Properties, incidence, diagnosis
Thickening of pyloris, closing the lumen, causing pressured vomiting.
More common in boys.
Not sick-want to eat after vomiting.
Distention of stomach.
Gastric waves try to overcome obstruction but no peristalsis seen.
Palaptation of the rubbery, oval hypertrophied pyloric muscle “OLIVE” – 75-80% of cases. Way to detect: insert an NG tube to remove fluid, have baby drink bottle and if he is quiet and not crying the abdomen will be soft and can palpate “olive” pyloris. Diagnostic.
Other Diagnosis:
Abdominal plain film.
Gastric dilatation.
Paucity of distal air.
Hypokalemic, hypochloremic alkalosis (paradoxical because of compensation by urine from conservation of K in kidney-acidodic urine). Hyponatremic.
Dehydration – Hb , BUN
Weight loss.
Paradoxic aciduria.
Treatment of Pyloric Stenosis
Nasogastric tube – decompression.
IV fluids.
TPN (depends on duration of symptoms).
Surgery – Pyloromyotomy-cut mucosa and submucosa
NEVER URGENT.
