Surgery Flashcards
What is the pyloric sphincter?
It is a ring of smooth muscle that forms the canal between the stomach and the duodenum.
What is pyloric stenosis?
Hypertrophy (thickening) of the pylorus which prevents food traveling from the stomach to the duodenum as normal.
After feeding, there is increased peristalsis in the stomach as it tries to push food into the duodenum. Eventually this becomes so powerful that it ejects the food into the oesophagus and out of the mouth as projectile vomiting.
What are the features of pyloric stenosis?
- presents in first few weeks of life
- hungry baby
- thinness
- pallor
- failing to thrive
What may be seen on abdominal examination of pyloric stenosis?
- firm, round mass felt in the upper abdomen (‘feels like a large olive’) caused by hypertrophic muscle of the pylorus
What can be seen on a blood gas for pyloric stenosis?
Hypochloric metabolic alkalosis
- due to baby vomiting HCl from the stomach
How is pyloric stenosis diagnosed?
Abdominal USS - visualise thickened pylorus
How is pyloric stenosis managed?
Laparoscopic pyloromyotomy (Ramstedt’s)
- incision in SM of pylorus to widen the canal to allow food to pass from the stomach to the duodenum
- excellent prognosis
What is Hirschsprung’s disease?
A congenital condition where nerve cells of the myenteric plexus (which forms the enteric nervous system) are absent in the distal bowel and rectum.
What is the myenteric nerve plexus responsible for?
It runs all the way along the bowel in the bowel wall and is a complex web of neurones, ganglion cells, receptors, synpases and neurotransmitters.
It stimulated peristalsis of the large bowel; without it, the bowel loses its motility and is unable to pass food along its length.
What is the pathophysiology in Hirschsprung’s disease?
During foetal development, parasympathetic ganglion cells start higher in the GI tract and gradually migrate to the distal colon and rectum.
In Hischsprung’s, these parasympathetic ganglion cells do not travel all the way down the colon so a section of colon at the end is left without them.
The aganglionic section does not relax so becomes constricted leading to a loss of movement of feaces and obstruction in the bowel.
Proximal to the aganglionic part becomes distended and full.
What risk factors exist for Hirschsprung’s disease?
- genes on various chromosomes have a modifying effect to increase risk
- family history
What other syndromes is Hirschsprung’s disease associated with?
- Down’s syndrome
- Neurofibromatosis
- Waardenburg syndrome
- multiple endocrine neoplasia type II
How does Hirschsprung’s disease present?
- acute intestinal obstruction shortly after birth
- delay in passing meconium (>24 hours)
- chronic constipation since birth
- abdominal pain and distension
- vomiting
- poor weight gain
- failure to thrive
What is inflammation and obstruction of the intestine in those with Hirschprung’s disease called?
Hirschsprung-associated enterocolitis (HAEC)
Occurs in 20% neonates
How is Hirschsprung’s disease managed?
- abdominal x-ray (intestinal obstruction, features of HAEC)
- rectal biopsy (absence of ganglionic cells)
- fluid resuscitation and management of intestinal obstruction (in unwell children/enterocolitis)
- IV abx (HAEC)
- NG tube
Definitive management:
- surgical removal of aganglionic bowel (may have long term disturbance in bowel function, some incontinence)