McGuffin- Peds GI- Melissa Flashcards
How does a pediatric diaphragmatic hernia develop?
What is the most severe complication?
Which type is most common?
How does it present–auscultation? CXR, prenatal US, belly exam?
Trauma to diaphragm or failure of pleuroperitoneal folds to close –> pulmonary hypoplasia
- Most common in left posteriolateral quadrant (left pleuroperitoneal membrane)
Presentation: (Varies in severity, most severe below)
- Severe Respiratory distress
- Hear bowel sounds, not air in affected chest
- Scaphoid (scooped out) abdomen
- CXR = bowel in chest, NG tube in chest
- Prenatal US = bowel in chest
What is Duodenal or Esophageal atresia; how does this happen?
When and how does it present?
With what genetic disorder is this typically associated?
Esophageal atresia- esophageal obstruction, possible TE fistula–> polyhydraminos
Duodenal Atresia- Failure of apoptosis–> no recanalization of duodenal lumen
Trisomy 21 assc
Presentation:
- Polyhydraminos (^ uterus size wks 26-30)
- BILIOUS VOMITING with FIRST FEED
- Recurrent aspiration pneumonia (choke, cough, cyanosis)
- XRAY = DOUBLE BUBBLE SIGN (air in stomach + just past pylors); coiled NG tube
Three diseases that present with bilious vomiting and how to differentiate them:
Occurs in obstructions Distal to Ampulla of Vater
Duodenal atresia–> presents with FIRST FEED
Malrotation of midgut volvulus–> presents LATER (not at first feed)
Hirschsprung Disease–> look for failure to pass myonium or chronic constipation
What is Gastroschisis?
How is it detected and how does it present?
How do we manage this?
What are some special concerns?
Defect in ventral abdominal wall b/w rectus mm’s lateral to umbilicus–> NO SAC; insides on the outside; usually on RT
- Detected with neonatal US
- SILO for staged repair after birth
- Attn. Hydration, nutrition, infection
- Feed with G tube for several mos
Whats Omphalocele?
How is it detected and how does it present?
How is it managed and what are some special concerns?
With what genetic disease is this defect associated?
Incomplete closure of umbilical ring:
Normal herniation of midgut @ wk 6–> FAILURE TO RETRACT in wk 10–> VAC-COVERED herniation through umbilicus
- Detected on neonatal US
- ^ AFP in amniotic fluid + mom’s blood
- Asstd. with Trisomy 18 (Edward’s Syndrome)
Describe the PE findings for Edward’s syndrome:
Facial, MSK, cardio findings
- *Triangular face w low-set, malformed EARS
- clenched hands with index over middle, 5th over 4th
- rocker bottom feet
- PDA, VSD
**SO MANY OF THE TRISOMIES SOUND ALIKE. HERE’S A WAY TO REMEMBER IF YOU DIDNT ALREADY KNOW:
(E)DWARDS= 18, YEAR YOU CAN (E)LECT PRESIDENT & has low set (E)ARS!!! I have gotten questions right on uwolrd just by knowing Edwards= Ears.
What is the Penology of Cantrell and with which disease is it associated?
Associated with Ompohalocele:
- omphalocele
- diaphragmatic hernia
- sternal cleft
- ectopic cordis
- intracardiac anomaly
What causes Hirschsprung disease?
How does it present (Xray, PE, etc.)
With what genetic anomaly is it associated?
How do we treat it?
Neural crest cell migration failure–> aganglionosis of bowel wall submucosa–> SEVERE CONSTIPATION –> Bowel rupture + sepsis possible!
Presentation:
- Baby fails to pass meconium in first 48 OR older kid never poops without assistance (enema, etc.)
- Distended abdomen, BILIOUS VOMITING, can’t fart
- XRAY: DILATED bowel loops, No air in rectum
- Trisomy 21
- Tx by resecting affected portion of bowel
What is an Imperforate Anus?
How does it present and how is it treated?
What are some common associated malformations (2)?
With what genetic anomaly is it associated?
Improper descent–> Rectum ends in blind pouch–> DIMPLE ANUS (no hole)–> CAN’T POOP –> SURGERY
- Associated with urogenital or urovaginal fistulas
- Associated with Trisomy 21
VACTERL: What is this?
Common anomalies associated with Imperforate Anus:
Vertebral defects Anal atresia Cardiac anomalies Traheoesophageal fistulas Esophageal atresia Renal anomalies Limb anomales
Neonatal jaundice: What are the two types?
When is this pathological?
What is benign?
DIRECT: conjugated hyperbilirubinimea; RARE; GREEN baby; evaluate for LIVER DISEASE
INDIRECT: undonjugated hyperbilirubinimea; COMMON; YELLOW baby
- *Pathological jaundice happens within first 24hrs
- *Benign jaundice is yellowing of skin and sclera that peaks bw days 3-5
What are some potential complications of severe neonatal jaundice?
What should one consider as causes (4)?
Lethargy, poor feeding, dehydration (remember capillary refill will be longer than 3 seconds)
- *Consider in Pathologic Jaundice:
- ^ RBC breakdown (hemolytic disease)
- G6PD Deficiency in severe cases
- Maternal sulfa drug use
- Sepsis
How do we manage Hyperbilirubinimia?
- Biliblanket for babies w bilirubin OVER 15mg/mL
- OVER 20mg/mL bive biliblanket and phototherapy
This will change the conformation of bilirubin so that it can be excreted
What is Kernicterus and how does it happen?
What are 3 risk factors and 4 complications?
- Hyperbilirubinimia OVER 20 mg/mL
- Bilirubin deposited in basal ganglia
Risks: Hypoalbuminimia, Metabolic acidosis, Sepsis
Complicaitons:
Choreoathetosis, Hypotonia, Hearing loss, Seizures
Describe the pathogenesis of Malrotation of Midgut with Volvulus?
How does it present in babies and older kids?
What do you see on imaging + labs?
Failure of midgut to rotate counterclockwise 270 degrees as it returns to the abdominal cavity during WEEK 10 development –>
VOLVULUS (twists into itself) –> ISCHEMIC INJURY
- Presents w vomiting, abdominal pain + distention
- XRAY = DOUBLE BUBBLE sign, BIRDS BEAK at site of rotation
- Metabolic acidosis (ischemic tissue–> ^ lactate)
