GI Malformations Flashcards
Presentation of Hirschprung’s disease?
Failure or delay to pass meconium Constipation Abdominal distention Faeces may be felt per abdomen PR may reveal tight anal sphincter and explosive discharge of stool
What are complications of Hirschprungs?
GI perforation Bleeding Ulcers Enterocolitis Short gut syndrome
How is Hirschprungs diagnosed? Mx?
Recta suction biopsy of aganglionic section staining for acetylcholisterase positive nerve excess
Excision of aganglionic segment
What are prenatal signs of oesophageal atresia + tracheo-oesophageal fistula
Poly hydramnios
Small stomach
What are postnatal signs of oesophageal atresia and tracheo-oesophageal fistula?
Cough Airway obstruction Increased secretions Blowing bubbles Distended abdomen Cyanosis Aspiration
How is oesophageal atresia/TOF diagnosed:?
Inability to pass catheter into stomach, x-ray shows it coiled in oesopagus
Mx of oesophageal atresia?
Stop feeding
Suck out oesophageal pouch
Primary surgical repair
What is congenital diaphragmatic hernia?
Developmental defect in the diaphragm allowing herniation of abdominal contents into the chest
Leads to impaired lung development: pulmonary hypoplasia and pulmonary hypertension
How is congenital diaphragmatic hernia diagnosed?
Prenatal US
Postnatal CXR
What are signs of congenital diaphragmatic hernia?
Difficult resuscitation at both Respiratory distress Bowel sounds in one hemithroax - usually the left pH < 7.3 Cyanosis
What is associated with congenital diaphragmatic hernia?
Trisomy 18
Pierre Robin
Other malformation - NTDs
What is prenatal and post natal treatment for congenital diaphragmatic hernia?
Prenatal:
Consideration of fetal surgery - tracheal obstruction by balloon
Postnatal:
Inset large bore NG tube when diagnosis suspected
Aim is to keep all air out of the gut
Facemask ventilation is contraindicated so immediately intubate, ventilate and paralyse
Surgery
What causes inguinal hernias? How do they present?
Patent processus vaginalis (passage which ushers the descending testicle into the scrotum)
Present as a lateral bulge to the pubic tubercle e..g during crying
Management for inguinal hernia?
Surgical repair
baby < 6w - operate within 2 days
<6 months operate within 2 weeks
< 6 years operate within 2 months
What is hydrocele?
Processus vaginalis patent at birth allowing only fluid from the peritoneal cavity to pass down it
Generally closes during the first year of life so no action is usually needed
If it persists until 2 years, surgical correction may be needed
What should you do for billions vomiting in neonates?
Surgical referral
Pass NGT
May be a midgut malrotation
What does acute gastric volvulus cause?
Non-bilious vomtiing
Epigastric distention
signs of pain
What is gastroschisis?
Paraumbilical (lateral to umbilicus) defect with evisceration of the abdominal contents
Most diagnosed antenatally on US
What is management for gastroschisis?
At delivery cover exposed bowel in clingfilm
Keep baby warm and hydrated
Surgical correction ASAP
May involve a staged procedure because the abdomen at birth is too small to accommodate gut
What is exomphalos?
Omphalocele
Ventral defects of the umbilical ring with herniation of abdominal viscera - covered in peritoneum and amnion
Often associated with other malformations
May contain bowel, stomach, liver, bladder
What is management of exomphalos?
Protect herniated viscera Maintain fluids and electrolytes Prevent hypothermia Gastric decompression Prevent sepsis
Primary or staged closure may be used to repair
