4.6 Flashcards
Hirschsprung disease is also called
congenital ganglionic megacolon
megacolon in Hirschsprung disease is due to
absence of ganglion cells in the mucosal and muscular layers of the colon –> functional obstruction (failure of colonic muscles to relax)
most common Hirschsprung disease
rectum and sigmoid colon involvement
risk factors for Hirschsprung disease
most common in males (4:1)
pathophys of Hirschsprung disease
failure of neural crest cells to migrate completely –> absence of enteric ganglion cells
what plexuses are affected in Hirschsprung disease
Auerbach and meissner
clinical manifestations of Hirschsprung disease
large bowel obstruction –> failure of meconium passage in first 48 H
Bilious vomiting
Progressive abdominal distention
Poor weight gain
FTT
Hirschsprung disease may also present with
enterocolitis –> sepsis like symptoms
PE in Hirschsprung disease
no stool in rectal vault : tight anal sphincter and the anal canal and rectum are devoid of fecal material
abdominal distention
what disease is associated with squirt/blast sign
Hirschsprung disease
what is the squirt/blast sign
explosive gush of flatus and liquid stool as the finger is withdrawn during rectal exam –> may relieve obstruction temporarily
what is the best initial test for Hirschsprung disease
contrast enema –> funnel shaped transition zone between normal and aganglionic bowel
when should you avoid contrast enema for Hirschsprung disease
if enterocolitis suspected
definitive diagnosis of Hirschsprung disease
rectal suction biopsy –> absence of ganglion cells in mucosal and submucosal layers of involved bowel
management for Hirschsprung disease
surgical resection of affected bowel
rectus abdominis muscles separate
diastasis recti
complete absence of closure of portion of the esophagus
esophageal atresia
esophageal atresia most commonly presents with
tracheoesophageal fistula
polyhydramnios
