Surgery Flashcards
(115 cards)
acute apendicitis
…
gastroschisis definition
full thickness abdominal wall defect in which foetal organs protrude outside the abdomen with no protective membrane covering it. Direct intestinal exposure to amniotic fluid in utero leads to chemical reactions, creating a thick inflammatory film or peel over the bowel
prevalence gastroschisis
1-6 per 10000 live births
gastroschisis patho
unknown
possible..compromised vascular supply to anterior abdo wall, defect in primordial umbilical ring, or abdnormal involution of R umbilical vein so weakened point at risk of rupture
risk factors gastroschisis
Maternal smoking (possibly due to placental insufficiency and abnormal development of the vascular system)
Maternal age <20 years old
Environmental exposures e.g. Nitrosamines
Maternal cyclooxygenase inhibitors use e.g. aspirin and ibuprofen
clinical features gastroschisis
can be present visible at birth or detected on prenatal USS at 20 weeks
often found to R of umbilicalcord
organs involed - S and L intestines, liver, stomach
swollen/thickened intestines
abdo cavity may appear smaller
intestinal malrotation sometimes - intestinal atresia
gastroschisis v omphalocoele
omphalocoele - no membrane covering abdo contents
extra intestinal or structural abnormalities
gastroschisis investigations
mainly clinical diagnosis
alpha fetoprotein - elevated
routine USS in 2nd trimester - detected
Ultrasonography - echogenic and dilated loops of bowel freely floating in amniotic fluid
coloured doppler - localise herniation
immediate management gastroschisis
fluid resus
maintain temp
sterile, clear covering over herniated contents
definitive management gastroschisis
surgery - protrude and close abdo wall
may need to be staged if large - nvolve placing the bowel in a clear sac called a silo, which is tightened until there is enough space to reduce the bowel completely
then NG tube and parenteral feeding
complications gastroschisis
Abdominal Compartmental Syndrome
Persistent bowel dysfunction
Wound infection
Necrotizing Enterocolitis
Short gut syndrome
Abdominal Compartmental Syndrome
prognosis gastroschisis
87-100% survival rates
intestinal atresia - poor prognostic factor
hirschsprung’s disease definition
also known as congenital aganglionic megacolon disease, is a congenital disease in which ganglionic cells fail to develop in the large intestine. This commonly presents as delayed or failed passage of meconium around birth.
prevalence hirschsprung’s disease
1 case per 1500-1700
median age 2 days
males 4 times more likley
genes hirschprung’s disease
those that encode the proteins for the RET signaling pathway and endothelin type B receptor pathway. The strongest association with Hirschsprung’s, is the Receptor tyrosine kinase (RET) gene, a proto-oncogene on chromosome 10q11. HD is strongly associated with chromosomal abnormalities, with 10-15% HD cases associated with trisomy 21 (Down Syndrome)
hirschsprung’s disease subtypes
short-segment - most common
long segment - aganglionosis extends past rectosignoid portion of colon to splenic flexture
total colonic aganglionosis disease - entire colon affected
hirschsprung’s disease pathophysiology
ganglionic cells of the myenteric and submucosal plexuses in the bowel aren’t present proximally from the anus to a variable length along the large intestine.
The most common accepted aetiology of this disease is due to the arrest of the neuroblast, derived from neural crest cell migration in fetal development between week 8 to 12.
hirschsprung’s enterocolitis
Increased intraluminal pressure can lead to decreased blood flow and deterioration in the mucosal layer. This stasis can lead to bacterial proliferation and the subsequent complication of Hirschsprung’s enterocolitis, which has a mortality rate of 25-30%. If not recognised early this can lead to sepsis and death
risk factors hirschsprung’s disease
males
chromsomal abnormalities - down’s
family hx - mutations
25% have classic triad in hirscsprung’s
failure to pass (within 48 hrs) meconium
abdo distention
billous vomiting
examination hirschsprung’s diease
faecal mass palpated in left lower abdomen
tympanic abdomen
empty rectal vault…forceful discharge of gas and faecal material
ddx hirschprung’s disease
meconium plug syndrome
meconium ileus
intestinal atresia
hirschsprung’s disease investigations
gold standard: rectal suction biopsy to confirm aganglionosis using acetylcholinesterase
contrast enema - short transition between proximal end of colon and narrow distal end of colon plus rectal diameter equal to sigmoid colon
laparatomy - if perfortation present as enema contraindicatedr
rectal suction biopsy guidelines
clinical features indicated, otherwise avoided:
Delayed passage of meconium (more than 48 hours after birth in term babies)
Constipation since first few weeks of life
Chronic abdominal distension plus vomiting
Family history of Hirschsprung’s disease
Faltering growth in addition to any of the previous features