GI Disorders

Omphalocele + Gastroschisis both need staged reduction (لا تتحمس
Amniotic Fluid
Oligohydroamnios
Polyhydroamnios
?
Baby can’t pee
Baby cant swallow
DDx
Weak abd muscles (tx w/ serial foley)
Renal agenesis
Wardeng-hoffman
Intestinal atresia
 Congenital Umbilical Hernia
Path
Incomplete closure of abdominal muscles
Clx
·       Soft, nontender bulge at umbilicus
·       Protrudes with increased abdominal pressure
·       Typically reducible
·       Non tender
·       Dx CLINICALLY (no need for dxic tests)
Tx
·       Observe (spontaneous closure)
·       Elective surgery around age 5
·       Do US if it’s ACQUIRED umbilical hernia.
Coanal Atresia
CHARGE syndrome is a set of congenital defects which are seen in conjunctions.
C: coloboma of the eye, central nervous system anomalies
H: heart defects
A: atresia of the choanae
R: retardation of growth and/or development
G: genital and/or urinary defects (hypogonadism)
E: ear anomalies and/or deafness
S/S
·       Baby turns blue while feeding & pink while crying
Dx
Clx → CT
Tx
Surgery
Hirshsprung dz
?
·       Associated w/ Down
·       Failure of neurons to migrate
·       ø aurbach / myenteric
S/S
Can presents in 2 ways:
·       FTPM (on DRE → diarrhea)
·       Toddler w/ overflow incontinence
Dx
·       1˚ X-Ray
·       2˚ Barium
·         Manometry (⤴️ Pressure)
·       BEST: Bx
Tx
3-stage Surgery
Doudenal Atresia
?
Associated w/ Down,
Annular pancreas
S/S
First 12 hours: Billous Vomiting
Dx
X-ray: double-bouble
Tx
NGT
Surgery
 Biliary atresia
Path
·       Idiopathic
·       Progressive obliteration of extrahepatic bile ducts
Clx
·       Asymptomatic at birth
·       Age 1-8 weeks:
Jaundice
Acholic stools &/or dark urine
Hepatomegaly
Dx
·       Conjugated hyperbilirubinemia
·       Liver biopsy: bile duct plugs & proliferation, portal tract edema, fibrosis
·       Intraoperative cholangiogram (gold standard): biliary obstruction
Tx
·       Hepatoportoenterostomy (Kasai procedure)
·       Liver transplant
Intususseption
?
Associated with previously used Rotavirus vaccine and HSP.
S/S
colicky abdominal pain, bilious vomiting, and currant jelly stool.
On PEx: right quadrant sausage-shaped mass
Dx
US
Contrast enema “Target sign”
Tx
1˚: IVF + Electrolytes
2˚ NGT
Barium Enema (both dx & Tx)
·       contraindicated if the child has signs of peritonitis, shock, or perforation.
Intussusception
RF
·       Recent viral illness or rotavirus vaccination
·       Pathological lead point
Congenital malformation of the intestines (eg, Meckel
diverticulum)
Henoch-Schönlein purpura
Celiac disease
Clx
·       Sudden, intermittent abdominal pain
·       “Currant jelly” stools
·       Sausage-shaped abdominal mass
Dx
·       “Target sign” on ultrasound
Tx
·       Air or saline enema
·       Surgery
VACTERL syndrome
·       V: vertebral anomalies
·       A: anal atresia
·       C: cardiovascular anomalies
·       T: tracheoesophageal fistula
·       E: esophageal atresia
·       R: renal anomalies
·       L: limb anomalies
 Diarrhea (Gastroenteritis)
?
Can be Infectious / non-infectious
S/S
Diarrhea → Dehydration
Dx
Look for an organism
Tx
Fluid Resuscitation
Antidiarrheals such as loperamide are always the wrong answer.
 Necrotizing enterocolitis (NEC)
RF
Prematurity (but not only to premature!)
Very low birth weight (<1.5 kg [3.3 1b])
Enteral feeding (formula > breast milk)
Pt w/ CHD -> ⤵️ BF to intestines
Clx
Feeding intolerance / Lethargy
Unstable VS (⤵️ T°)
Bilious emesis, bloody stools, abdominal distention
X-Ray
Pneumatosis intestinalis
Portal venous gas
Pneumoperitoneum
Tx
Bowel rest; parenteral nutrition
Broad-spectrum IV Abx
± Surgery
Wilms
Neuroblastoma
?
·       Semi-hypertrophy of 1 kidney
·       (Wilms tumor, aniridia, GU malformations, and mental retardation is referred to as WAGR syndrome. The a deletion on chromosome 11)
·       metanephros
adrenal medulla tumor
(neural crest)
S/S
Abdominal mass (does not cross midline)
Anirdia (no iris)
Constipation
N/V
Abdominal mass
Hypsarrythmia (dancing eyes) and opsoclonus (dancing feet) are hallmarks.
Dx
·       1˚: US
·       Best˚: Contrast CT
·       ⤴️ URINE VMA and metanephrines
·       Bx: small round blue/purple cells
·       N-myc gene amplification
·       131I-MIBG body scan to detect metastasis
·       MRI
Tx
Total nephrectomy + chemo + radiation
Surgical resection
Neuroblastoma
Path
·       Neural crest origin
·       Involves adrenal medulla, sympathetic chain
Clx
·       Median age <2
·       Abdominal mass
·       Periorbital ecchymoses (orbital metastases)
·       Spinal cord compression from epidural invasion (“dumbbell tumor”)
·       Opsoclonus-myoclonus syndrome
Dx
·       Elevated catecholamine metabolites
·       Small, round blue cells on histology
·       N-myc gene amplification
Meckel diverticulum
#
Rule of 2s:
·       2% prevalence
·       Age 2
·       2:1 male/female ratio
·       Location within 2 feet of ileocecal valve
Clx
·       May be asx, incidental finding
·       Painless lower gastrointestinal bleeding
·       ± Anemia
Comp
·       Intussusception
·       Volvulus
·       Intestinal obstruction
Dx
·       Technetium-99m pertechnetate scan
Evaluation of neonatal hydration
Signs
Decreased wet diapers
Absence of tears
Sunken fontanelle
Dry mucous membranes
Decreased skin turgor
Delayed capillary refill
Tx
<7%
·       Continue exclusive breastfeeding
·       Follow-up at age 10-14 days to check that infant has regained birth weight
7%
·       Assess for oromotor dysfunction
·       Assess for lactation failure
·       Dally weights
·       Consider formula supplementation
Differential diagnosis of regurgitation & vomiting in infants
Dx
Clx
Tx
GERD
Physiologic
·       Asymptomatic
·       Happy spittter
Pathologic
·       FTT
·       Significant irritability
·       Sandifer syndrome
·       Reassurance
·       Positioning therapy
·       Thickened feeds
·       Antacid therapy
·       If severe, esophageal pH probe monitoring & upper endoscopy
Milk protein allergy
·       Regurgitation/vomiting
·       Eczema
·       Bloody stools
·       Elimination of dairy & soy protein from diet
Pyloric stenosis
·       Projectile nonbilious vomiting
·       Olive-shaped abdominal mass
·       Dehydration, weight loss
·       Abdominal U/S
·       Pyloromyotomy 
HUS
Path
Shiga toxin (infection w/ E.coli or Shigella)
Clx
·       Hx of bloody diarrhea
·       Fatigue, pallor
·       Bruising, petechiae
·       Oliguria, edema
Dx
·       Hemolytic anemia (schistocytes, ⤴️bilirubin)
·       ⤵️PLT
·       Acute kidney injury (t BUN, T creatinine)
Tx
·       Fluid & electrolyte management
·       Blood transfusions
·       If severe → Dialysis
 PED constipation
RF
·       Initiation of solid food & cows milk
·       Toilet training
·       School entry
Clx
·       Painful/hard bowel movements
·       Stool withholding
·       Encopresis (feces in underwear >4ys)
Complications
·       Anal fissures
·       Hemorrhoids
·       Enuresis/urinary tract infections
Tx
·       ⤴️Dietary fiber & water intake
·       Limit cow’s milk intake to <24 oz
·       Laxatives
·       ± Suppositories, enema
 Food protein—induced allergic proctocolitis
RF
Family history of allergies, eczema, or asthma
Clx
·       Young infant
·       Painless, bloody stools
·       ± Spit-up
Tx
·       Elimination of milk & soy from maternal diet in breastfed infants
·       Hydrolyzed formula in formula-fed infants
Prognosis
resolution by 1 year

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