BIOMED
Pathophysiology
Everything be alphabetical here (except for Homeostasis at the bottom) 😘
(& Mental Health, Lmao)
There are a lot of neonatal conditions covered, seen in high level NICU/NCCU Units.
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Table of Contents:
1 | Ageing
2 | Canc...
Hirschsprung's disease refers to a congenital condition affecting all or part of the large intestine, and thus, passing stool This is a result of a lack of ganglion nerve cells responsible for peristalsis in an affected segment of the colon (and sometimes in the small intestine also) causing intestinal malfunction, and obstruction from back up of. contents. These patients also have an abnormal involuntary anal sphincter that does not relax normally during delectation.
This disease occurs most commonly in the rectum or sigmoid colon, however regardless of the affected area, surgical intervention is necessary to remove the non-functional intestinal segment. With successful surgery, most of these these children will go on to recover normal bowel control and function.
Incidence & Risk Factors 1 in 5'000 live briths, more common in males, may be associated with inherited conditions including Down Syndrome.
Note that having had a baby with Hirschsprung's disease before increases the risk of a future biological sibling being born with the disease, and the condition is associated with other inherited conditions, including congenital heart disease.
Children who have Hirschsprung's disease are prone to enterocolitis, that can be life-threatening and requires immediate treatment.
Signs & Symptoms Children with Hirschsprung disease will not usually have a bowel movement in the first days post birth, leading to an abdominal x-ray to be undertaken to investigate for intestinal obstructions. Patients may have associated obstructive symptoms including:
☞ Abdominal distinction ☞ Bilious or fecal vomiting ☞ Constipation or gas (babies may lift their legs to their chest) ☞ Diarrhoea ☞ Delayed passing of meconium ☞ Failure to thrive/fatigue (in older children)
Diagnosis Abdominal X-ray is used to assess for intestinal obstruction and a bulge in the bowel, and diagnosis can be confirmed via rectal biopsy to investigate whether the ganglion nerve cells responsible for peristalsis are missing.
Treatment Babies may require a nasogastric tube (NGT) to aspirate fluid and air collection in the stomach, and may need intravenous nutrition administration and regular bowel washouts. Antibiotics will also be given if they have enterocolitis.
Surgically, most patients will undergo a 'pull-through' operation, where the affected segment of the bowel is removed and the healthy ends are joined back together. This can be performed in stages if necessary, where in stage 1, there will be colostomy (stoma) formation, and then in stage 2, the affected bowel segment will be removed.
These can be either laparoscopic or open surgical procedures.
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