Gastrointestinal and hepatobiliary disorders

GASTROINTESTINAL AND
HEPATOBILIARY DISORDERS
See also GI chapter on this topic.
Umbilical hernia
Omphalocele
Gastroschisis
Failure of the umbilical ring closure, weakness of abdominal muscles
Most are small and resolve in 1-2 years without any treatment
Surgery if getting larger after 1-2 years, symptoms (strangulation,
incarceration), and/or persistent after age 4
Failure of intestines to return to abdominal cavity with gut through umbilicus
Covered in a sac (protection)
Associated with other major malformations and possible genetic disorders
(trisomy)
Large defects need a staged reduction (use of a surgical Silo), otherwise
respiratory failure and ischemia
Defect in abdominal wall lateral to umbilicus (vascular accident)
Any part of the GI tract may protrude
Not covered by a sac
Major problem with the intestines: atresia, stenosis, ischemia, short gut
Necrotizing enterocolitis (NEC)
Imperforate anus
Jaundice
A 2-day-old infant is noticed to be jaundiced. He is nursing and stooling
well. Indirect bilirubin is 11.2 mg/dL; direct is 0.4 mg/dL. Physical exam is
unremarkable except for visible jaundice.
Surgery based on condition of gut; if no ischemia, large lesions need a staged
reduction as with omphalocele
Transmural intestinal necrosis
Greatest risk factor is prematurity; rare in term infants
Symptoms usually related to introduction of feeds: bloody stools, apnea,
lethargy, and abdominal distention once perforation has occurred
Pneumatosis intestinalis on plain abdominal film is pathognomonic (air in
bowel wall)
Treatment: cessation of feeds, gut decompression, systemic antibiotics, and
supportive care; surgical resection of necrotic bowel may be necessary
Failure to pass stool after birth
No anal opening visible
Treatment is surgical correction.
May be part of VACTERL association.
Pathophysiology
Increased production of bilirubin from breakdown of fetal red blood
Table 1-6. Physiologic Jaundice Versus Pathologic Jaundice
Physiologic Jaundice Pathologic Jaundice
Appears on second to third day of life (term) May appear in first 24 hours of life
Disappears by fifth day of life (term)—7th Variable
Peaks at second to third day of life Variable
Peak bilirubin <13 mg/dL (term) Unlimited
Rate of bilirubin rise <5 mg/dL/d Usually >5 mg/dL/d
cells plus immaturity of hepatic conjugation of bilirubin and elimination in
first week of life
Rapidly increasing unconjugated (indirect reacting) bilirubin can cross
the blood-brain barrier and lead to kernicterus (unconjugated bilirubin
in the basal ganglia and brain stem nuclei). Hypotonia, seizures,
opisthotonos, delayed motor skills, choreoathetosis, and sensorineural
hearing loss are features of kernicterus