Children digestive disorders

pathology

Syndromic vs non-syndromic CL&CP

Can be caused by genetic or environmental issues: genetics, B vitain/folate deficiency, alcohol, tobacco use, statins, steroids

Syndromic: part of chromosomal, medelian, or teratogenic syndromes

Non-Syndromic: Cleft palate occurring alone

Cleft Lip

Caused by incomplete fusion of the nasomedial or intermaxillary process during the 4th week of embryonic development

Commonly occurs under one nostril, but can be bilateral and symmetric or asymmetric

Cleft Palate

Commonly associated with cleft lip but can occur without it

May affect only the uvula and soft palate or may extend forward to the nostril and involve the hard palate and the maxillary alveolar ridge

CL & CP clinical manifestations

Feeding difficulties

Cannot generate negative pressure for normal sucking

CL & CP treatment and evaluation

Ultrasound and postnatal imaging

surgical correction

speech training

prosthodontist and orthodontist follow up

Esophageal Atresia

The esophagus ends in a blind pouch usually accompanied with a fistula

Increases with polyhydramnios (too much amniotic fluid)

Signs: drooling, inability to swallow, respiratory distress

Place tube with suction, HOB elevated and no oral feedings

Infantile hypertrophic pyloric stenosis

Narrowing and distal obstruction of the pylorus (stomach connecting to small intestine)

Forceful nonbilious vomiting immediately after feeding starting 2-3 weeks after birth. Causes weight loss, electrolyte imbalances, dehydration, irritability

Palpable in RUQ (olive sized mound)

Treatment is pyloromyotomy

Intestinal Malrotation

Ileum does not rotate to RLQ. Small intestine lacks posterior attachment. Intestine twists up on itself (volvulus)

Most cases develop during first year of life

Signs: dehydration, electrolyte imbalance,bile stained vomiting, fever pain, scanty stools, bloody stools, diarrhea

Treatment: xrays, laparoscopic or open surgery

Meckel Diverticulum

Pocketing of the layers of the small intestinal wall.

Most common symptom is painless rectal bleeding

2% of popularion

2 feet from the ileocecal valve

length of 2 inches

Asymptomatic before age 2

2 types of ectopic mucosa (gastric and pancreatic)

2:1 M:F ratio

Meconium Syndromes

Substance that fills the intestine before birth. Usually passed 12-48 hrs after birth

abdominal distention after birth followed by vomiting

Diagnosed with radiographic examination

treated with intestinal lavage and laxatives

Meconium Ileus

Meconium caused intestinal obstruction in a newborn. Many complex cases occur with cystic fibrosis

Meconium plug syndrome

Delayed passage of meconium >24-48 hrs and intestinal dialation

Distal intestinal obstruction syndrome

complete or incomplete intestinal obstruction of viscid fecal accumulation in the terminal ileum

Hirschsprung Disease

Obstruction of the colon

Absence of parasympathetic nervous system intrinsic ganglion cells

abnormally innervated colon impairs fecal movements. Caused obstruction and distention.

Mild to severe constipation, diarrhea, sepsis, death

Treatment: surgery

GERD

(Note some gastric reflux is normal!)

S/S: Vomiting, food refusal, crying, choking, gagging

Treatment: feeding volume adjusted, thickened foods, no caffeine, chocolate or spicy foods

Causes: transient lower esophageal sphincter relaxations

Inadequate adaptation of sphincter tone to changes in abdominal pressure

Intussusception

Intestine obstruction

most common is ileum telescopes into the cecum and part of the ascending colon

causes bleeding, necrosis, CURRANT JELLY STOOLS, knees to chest

Treatment:

Ultrasonography, reduction with enema, surgical reduction

Celiac disease

Autoimmune disease that damages small intestinal villous epithelium when gluten is ingested (wheat, rye, barley, malt)

Celiac crisis: severe diarrhea, dehydration, hypoproteinemia,

Treatment: gluten free diet for life

Diagnosis confirmed with serologic autoantibody measurement

Malnutrition

Imbalance between nutrient requirements and intake

-impaired absorption

-increased nutrient losses

-increased nutrient requirements

Causes:

moderate to severe illnesses

lack of access to nutrients

behavioral factors

Protein energy malnutrition (PEM)

Associated with long-term starvation

Kwashiorkor: deficiency in dietary protein

Marasmus: all forms of inadequate nutrient intake

Manifestations: muscle wasting, diarrhea, dermatosis, low hemoglobin leverl, infection, generalized edema, loss of subQ fat, delays in development

Faltering Growth (FTT)

Slower weight gain than expected, low BMI, Low head circumference ratio

Necrotizing Enterocolitis

Intestine wall lining dies

Most common neonatal GI emergency. Primarily affects smallest and premature infants

S/S: Feeding intolerance, bloody stools, abdominal distention, septicemia and elevated WBC and low platelets

Treatment: antibiotics, surgery, gastric suction

Primary Lactose intolerance

Inadequate production of lactase.

causes: flatulence, bloating, abdominal pain, diarrhea

Wilson’s disease

Defect of copper metabolism

-Toxic levels of copper in the liver, brain, kidneys & corneas

Galactosemia

Toxic levels of galactose in body tissues, liver and brain

Fructosemia

Toxic levels of fructose build up in the body

Biliary atresia

Absence or obstruction of extrahepatic bile ducts.

S/S: jaundice, fat absorption may be impaired, liver transplant is needed

Pathologic jaundice

Bilirubin >20 mg/dl associated with severe illness

Risks: blood type incompatibility, premature birth, exclusive breast feeding, maternal age > 25 years, male sex, brith trauma, delayed meconium passing

Physiologic jaundice

Bilirubin concetration greater than 2 mg/dL

Common in healthy newborns, caused by lack of maturity of bilirubin uptake and conjugation. May also be due to poor intake or dehydration

associated with hemolytic disease, liver disorders, infections