Omphalocele and Gastroschesis

total_recall_stomach_creature2
A case of unrepaired Omphalocele

 

From Lange: Handbook of Pediatric Anesthesia and Lange: The Anesthesia Guide

Omphalocele: viscera herniate through umbilicus, covered in peritoneum
etiology is failure of gut migration from yolk sac into abdomen
– 1:6-10k
– 2/3 have congenitial abnormalities: CV(significantly increasese mortality) , GU(bladder extrophy), GI (meckel’s, malrotation), Cranio, trisomy 13, Bckwith-Widemann (visceromegaly, macroglossia, microcephaly, hypoglycemia)
-survival rate: 70-95%
Gastroschisis: no covering, defect lateral to umbilicus (right)
etiology is occlusion of omphalomesenteric artery with ischemia to the right of periumbilical area
– 1:30k
– low incidence of associated abnormalities, but if so similiar to omphalocele; GI intestinal atresia
-premature labor and devliery common, more issues with bowel function
-survival rate: >90%

Both dx prenatal u/s
Both  M>F
Both a/w Trisomy 21, congential diaphragmatic hernia
Pre-Op

NG tube to decompress stomach
Broad-spectrum A/B
Expedited surgery to minimze heat loss, infection, trauma
Fluid resuc. 150-300mg/kg/d with balanced salt soln., maintain 1-2ml/kg/h UOP
urgent surgery, but w/u associated abnormalities: echo, renal u/s
Anesthetic Management

-suction stomach and pre-oxygenate
-awake or RSI with ETT
-fluid resucitation
-prevent hypothermia
-opioid + muscle relaxant (nondep)
-pulse ox on lower extremity can detect congestion d/t obstructed venous return
+/- a line (esp. if cardiac)
– increased intra-abdominal pressure warnings: vent compromise (peak airway pressure higher, decreased TV), bowel edema, anuria, hypotension, decreased organ perfusion)
*if insp pressure >25-30 or intragastric >20, don’t do primary closure

P/O
-ventilation for 24-48 hrs, monitor airway pressure, fluid resuc., a/b
-watch out: abdominal compartment syndrome (gastric pressure<20, vesical <20,  peak vent pressure<30cm/h20)

Do’s and Don’ts
-Do: pre-op echo for omphalocele
-Do: aggressive fluid resuc.
-Do: communicate to surgeon about tight closure
-Do: adequated nondepolarizer for closure
-DON’T: Mask ventilate!
Surgical concerns
-small defect: primary closure
-large defect: staged closure with silastic silo which is secured at edge and reduced over about a week, when pt goes to OR for closure

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