Pediatrics 3 Flashcards
What are the types of neonatal obstruction?
DYNAMIC
- meconium ileus
- meconium plug
- congenital atresia
- Hirschsprung’s disease
- imperforate anus
- volvulus neonatorum
- intussusception
ADYNAMIC
- necrotizing enterocolitis
How does neonatal intestinal obstruction present?
- recurrent vomiting & dehydration
- absolute constipation
- abdominal distention
- crying and flexion of hip (colicky abdominal pain)
- sepsis, & shock
What are the signs of neonatal intestinal obstruction?
- dehydration, sepsis, & shock
- distention, visible peristalsis, hernia, imperforate anus
- tenderness, rigidity, palpable abdominal mass
- hyper resonance
- hyper audible intestinal sounds or dead silent abdomen
- empty rectum, bleeding, grips on examining finger
What investigations are done to diagnose intestinal obstruction?
PLAIN X-RAY -> more than 3 fluid levels
pelvi-abdominal US & CT
exclude VACTERL
What is meconium ileus associated with?
cystic fibrosis
- characterized by viscous secretions affecting more than just the intestine
- manifests occur within -> 24-48hrs after birth
How can u diagnose meconium ileus?
X-ray -> dilated bowel with absent air fluid levels
Gastrografin enema -> most diagnostic & therapeutic
How should meconium ileus be treated?
1) contrast enema to dissolve & wash out meconium
2) enterostomy with irrigation with N-acetyl cysteine
3) exploration if perforated
What is the cause of Meconium plug syndrome?
- occurs in relation to Hirschsprung’s disease
- contrast enema is diagnostic & therapeutic
What is the cause of duodenal atresia?
failure of canalization of duodenum
- presented with: recurrent vomiting (bilious or non-bilious)
What is the diagnostic sign in duodenal atresia?
DOUBLE BUBBLE SIGN
How should duodenal atresia be treated?
duodenoduodenostomy after pre-op prep
What is the commonest type of GI atresia?
Jejunal atresia
- presents by bilious vomiting & upper abdominal distention
- treated by resection & anastomoses
How is colonic atresia treated?
- confirmed by contrast enema
- treated by diverting colostomy -> 2nd set for reconstruction
Which type of dynamic intestinal obstruction requires urgent exploration?
VOLVULUS NEONATORUM
resect gangrenous gut & untwist viable gut
What are the types of abnormal gut rotation?
- GUT NON ROTATION -> volvulus
- GUT INCOMPLETE ROTATION -> volvulus neonatorum
- INCOMPLETE FIXATION -> left mesocolic internal hernia
- INCOMPLETE REDUCTION -> congenital umbilical hernia
- FAILURE OF DESCENT -> subhepatic caecum
What is the cause of volvulus neonatorum?
- incomplete rotation of the gut -> twist gut along the axis of SMA -> strangulation of midgut
How does a neonate present with volvulus neonatorum?
- intestinal obstruction with peritonitis due to massive strangulation
- rapidly progressive associated with septic & hypovolemic shock
when does necrotizing enterocolitis occur?
2nd - 3rd week of life in premature infants
- characterized by variable damage to intestinal tract
What are the manifestations of necrotizing enterocolitis?
Intestinal Obstruction bleeding/rectum peritonitis septic or hypovolemic shock in pneumoperitoneum -> surgery
absence of ganglionic cells in the distal colon & rectum is known as?
HIRSCHSPRUNG’S DISEASE
- failure of migration of ganglionic cells along neural crest to developing gut
- failure of relaxation of lower rectum during defecation
What is the most effected site in Hirschsprung’s disease?
anorectal junction up to rectosigmoid junction
what is the macroscopic picture of Hirschsprung’s disease?
- distal spastic segment -> aganglionic
- middle conical zone -> transitional zone
- proximal dilated zone -> normal segment
What is the microscopic picture of Hirschprung’s disease?
- absence of Aurebach’s & Meissner’s plexus
- hypertrophic nerve fibers
What is the clinical picture of Hirschsprung’s disease?
- delayed passage of meconium >24hrs
- chronic constipation -> defecation after several days & only after insertion of mother’s finger
- abdominal distention & visible peristalsis
- doughy mass in left iliac fossa
- hyperresonant
- early -> hyperperistaltic late -> diminished intestinal sounds
What are the complications of Hirschsprung’s disease?
- growth retardation
- acute on top of chronic obstruction
- fatal obstructive toxic enterocolitis
What is the most diagnostic method of investigation in case of Hirschsprung’s disease?
RECTAL BIOPSY -> absent ganglionic cells + hypertrophied nerve fibers
Gastrografin enema -> megacolon with very characteristic funneling
Anorectal manometry -> failure of relaxation in response to rectal distention
How should Hirschsprung’s disease be treated?
if there is intestinal obstruction
- conservative -> no response -> colostomy
- > responding -> definitive operation
If the is no intestinal obstruction - pre-op prep - elective definitive operation Swenson's operation Soave's operation Duhmel's operation
What are the high anorectal anomalies?
HAVE DEFECTIVE ANAL SPHINCTER
- anorectal agenesis -> rectum forms blind pouch above pelvic floor with fistulous connection in vagina or urethra
- rectal atresia -> rectum forms blind pouch above pelvic floor -> anal canal forms blind pouch below pelvic floor
What are the low anorectal anomalies?
INTACT ANAL SPHINCTER
- covered anus -> anal canal covered by skin -> ectopic anus
- membranous anus -> membrane at level of dentate line
What is the clinical picture of an imperforate anus?
- neonatal intestinal obstruction
- meconium at urethral opening
- anal dimple
- ectopic anus
What investigations could be used to diagnose imperforate anus?
INVERTOGRAM
- differentiates between high & low anomalies
URINE ANALYSIS
- detection of meconium in urine
What is the treatment of high anomalies?
3 stage operation
- temporary colostomy
- anorectal pull through
- closure of colostomy
continence may be affected
What is the treatment of low anorectal anomalies?
- anal stenosis -> regular anal dilatation
- imperforate without fistula -> PSARP
- anteriorly displaced with long gap -> transposition anoplasty
- anteriorly displaced with short gap -> cutback anoplasty
