GI, Misc + CDB Flashcards
MC type of TEF
Type A: Proximal EA with distal TEF
Best to do lumbar tap at
L4-5
MC and life-threatening complication of esophageal atresia with TEF
Aspiration pneumonia (gastric contents, chemical pneumonitis)
-h/o of polyhydramnios -vomiting w/ 1st feeding -choking/coughing & cyanosis -recurrent aspiration pneumonia
EA with TEF
Inability to pass an NGT or OGT in the NB
EA with TEF
___% of EA have TEF
90
Sequence associated with EA
VACTERL V-vertebral anomalies A-anal atresia C-cardio anomalies T-TEF E-esopageal atresia R-renal anomalies L-limb anomalies
In EA, the esophagus ends blindly approximately ___ from the nares
10 to 12 cm
T/F All TEFs are congenital
F, may be acquired
T/F EA is a surgical emergency
T
Types of TEF
___ is the least common but the most likely to be seen in ED
H-type tracheoesophageal fistula (Type C)
New born infant with frothing and bubbling at the mouth with episodes of coughing and cyanosis noted to be exacerbated on feeding
EA
MC esophageal disorder in children of all ages is
GERD
Loss of normal peristalsis and failure of LES to relax in response to swallowing
Achalasia
Pathophy of achalasia
Decreased ganglion cells surrounded by inflammatory cells
Bird’s beak on barium swallow
Achalasia
Achalasia is confirmed by the most sensitive test for it which is
Manometry
Medical management for achalasia when surgery cannot be done
Nifedipine Botulinum toxin injection
Surgical management for achalasia
Heller myotomy or pneumatic dilatation
Syndrome of familial achalasia, alacrima, corticotropin insensitivity
Allgrove syndrome
What is the primary mechanism of GER
Transient LES relaxation
Main stimulus for transient LES relaxation is ___
Gastric distention
Neck contortions seen in GER is called what syndrome
Sandifer syndrome
What position worsens the infant GER
Seated position
Recommended position in infants with GER
If the patient is asleep – supine ( for risk of SIDS); if the patient is awake, prone position or upright carried position
What are the most important predictor of successful surgical outcome of GER
Preoperative accuracy of diagnosis; skill of the surgeon
It is seen in males and blood type O and B presenting as non billous vomiting after feeding at 3rd wk of life , jaundice, with olive size non movable mass above and right of the umbilicus
Hypertrophic pyloric stenosis
Shoulder sign (bulge of pyloric muscle into the antrum), double tract sign (parallel streaks of barium in the narrow channel; excess mucosa), or string sign (from elongated pyloric channel) on barium studies (upper GI series)
Hypertrophic pyloric stenosis
Management for hypertrophic pyloric stenosis
Surgery: Ramstedt pylorotomy
MC cause of pyloric stenosis
Idiopathic
T/F Hypertrophic pyloric stenosis is usually present at birth
F
Drug associated with pyloric stenosis if given to infants within the first 2 weeks of life
Erythromycin
Congenital anomalies associated with hypertrophic pyloric stenosis
Turner’s, Trisomy 18
Systemic conditions assoc with hypertrophic pyloric stenosis
Eosinophilic GE, epidermolysis bullosa
Hypertrophic pyloric stenosis may appear as late as
5 months
Most sensitive test to detect hypertrophic pyloric stenosis; initial test
Ultrasound Elongated pyloric channel (> 14 mm) Thickened pyloric wall (> 4 mm)
__ is the most common clinical association of pyloric stenosis
Unconjugated hyperbilirubenemia ( icteropyloric syndrome)
Acid-base abnormality in hypertrophic pyloric stenosis
Hypochloremic, hypokalemic metabolic alkalosis: acid loss in vomit
Mushroom sign in upper GI series
Hypertrophic pyloric stenosis; Hypertrophic pylorus against duodenum
MC cause of non bilous vomiting in children
Hypertrophic pyloric stenosis
Best test for hypertrophic pyloric stenosis
Barium swallow
Triad: S I R ( sudden onset of severe epigastric pain, inability to pass tube into stomachl, retching with emesis)
Gastric volvulus
Management for gastric volvulus
Emergency surgery
First month of life, bilious vomiting, crampy/colicky abd pain, blood/mucus in stool
Intestinal volvulus (congenital malrotation of midgut)
MC location for volvulus (twisted loop) in children
Ileum
XR: air fluid levels upper GI series: “bird beak” at rotation sight
Intestinal volvulus
Initial management for intestinal volvulus
Endoscopic decompression, fluids
Inverted U sign of volvulus represents
Distended sigmoid loop
Midline crease in the coffee bean sign of volvulus represents
Mesenteric root in a greatly distended sigmoid
T/F Ligament of treitz is abnormally located in intestinal volvulus
T
Duodenal atresia is due to failure to recanalize (lack/absence of normal duodenal apoptosis) the lumen after the ___ phase of intestinal development during the ___ weeks AOG
solid, 4th-5th
Duodenal atresia is associated with what chromosomal abnormality
Down syndrome
What is the hallmark of duodenal obstruction
Billous vomiting without abdominal distention
Double bubble sign
Duodenal atresia
What GI anomaly is commonly assoc w/ down syndrome
Duodenal atresia
Bile stained vomitus w/in 12 hrs after birth, dehydration; Annular pancreas: wraps around duodenum
Duodenal atresia
Management of duodenal atresia
Decompress w/ NG tube, correct electrolytes, treat life-threatening anomalies, surgery (duodenoduodenostomy)
Presents in infancy w/ coughing, vomiting, gagging after feeding; Resp distress and frothy bubbles in oral cavity
TEF
Distended abdomen w/ dilated loops of bowel
Malrotation
Triple bubble sign
Jejunal atresia
“Ground glass” appearance in the RLQ with trapped “soap bubbles”; egg-shell pattern
Meconium ileus
Meconium ileus is diagnostic for what other disease
Cystic fibrosis
What is meconium ileus
Obstruction of the small intestine (terminal ileum) in the newborn caused by impaction of thick, dry, tenacious meconium
Failure to pass meconium w/in first 24 hours of life; bilious vomiting, h/o polyhydramnios, family history of CF
Meconium ileus
Intestinal perforation–> pneumoperitoneum (after birth) or intrabdominal calification (before birth)
Meconium ileus (complications)
Similar presentation to meconium ileus (bilious vomit, failure to pass meconium w/in first 24 h) BUT non-cystic fibrosis babies; no complication with intestinal perforation
Meconium plug syndrome
___ % of meconium ileus are due to CF
99
The most common presentation of cystic fibrosis in the neonatal period
Meconium ileus
MC congenital GI anomaly
Meckel diverticulum
What is the cause of meckels diverticulum
Failure of omphalomesenteric duct/vitelline duct to obliterate
Omphalomesenteric duct/vitelline duct is a remnant of
Yolk sac
Significant sudden intermittent painless rectal bleeding with brick-colored or currant jelly stool
Meckel diverticulum
MCC of LGIB in children
Meckel diverticulum
Diagnostic test for Meckel
Technetium 99M scan aka Meckel scan (scintigraphy)
Management for Meckel
Diverticular resection with transverse closure of the enterotomy
The only true congenital diverticulum
Meckel diverticulum
MC tissue in Meckel
Gastric
When is a diverticulum considered “true”
If involves all layers of bowel
What is the meckel’s diverticulum rule of 2’s
-2% of population -2 feet from ileocecal valve -2 types of ectopic tissue (gastric/pancreatic) -2x more affects males than females -less than 2 years of age
MC complication of Meckel
LGIB
Omphalomesenteric (vitelline) duct should disappear by
7th week AOG
Meckel’s diverticulum may mimic ___ and also act as lead point for ___
Acute appendicitis; intussusception
MC cause for emergent surgery in childhood
Appendicitis
Perforation rates are greatest in youngest children because ___
They can’t localize symptoms
Peak age of appendicitis
10-12
Appendicitis is rare in children younger than
5
Stages of appencidicits
1) Luminal obstruction 2) Bacterial invasion 3) Necrosis of wall
Appendiceal lumen is most commonly obstructed by
Fecalith (other: lymphoid hyperplasia from viral infection, appendices carcinoid)
Appendiceal rupture usually occurs within ___ hours of onset of symptoms
48
It is the single most reliable finding in the diagnosis of acute appendicitis
Localized abdominal tenderness
Congenital lack of distal bowel innervation by auerbach plexus -> constant contracture of muscle tone
Hirschprung disease (Congenital aganglionic megacolon)
What syndrome is hirschsprung disease freq assoc w/
Down syndrome (less frequently, MEN type 2 and Waardenburg, Laurence-Moon-Bardet-Biedl)
Hirschprung disease, boys vs girls
Boys
Do not pass meconium in 1st 48 hrs or at all (normal = 1st 24 hrs); bilious vomiting
Hirschprung disease
Treatment of choice for Hirschprung
Single stage laparospcopic endorectal pullthrough is the tx of choice