Pathology High-Yield Topics – CONGENITAL

Prepared by Sara Chakel (schakel@umich.edu)

1. Maternal complications of birth (e.g., Sheehan’s syndrome, puerperal infection)

Sheehan’s syndrome (a.k.a. postpartum pituitary necrosis)

• Due to ischemic necrosis of the pituitary gland, characteristically associated with hemorrhage and shock during childbirth
• Clinical manifestations: First due to loss of gonadotropins, then due to subsequent loss of TSH and ACTH
• Clinical complaints: Patient with complicated birth and uneventful recovery seen six months later with complaints of amenorrhea, loss of weight, loss of muscle strength. Physical exam would reveal vaginal atrophy and loss of axillary and pubic hair. Labs would show decreased serum T4 and decreased serum cortisol.
• Source: BRS Pathology

Puerperal infection

• Puerperal infection: Refers to reproductive organ infection during first few weeks post-partum (as near as I can figure)
• Differential diagnosis: Endometritis, urinary tract infection, lower genital tract infection, wound infections, pulmonary infections, thrombophlebitis, and mastitis
• Most common cause of postpartum fever: Endometritis (occurs after 2% vaginal deliveries, 10-15% caesarian deliveries)
• Endometritis: Ascending infection from pathogens in the lower genital tract, including a variety of aerobic and anaerobic organism.
• 1-2 days after delivery: Think Group A Strep
• 3-4 days after delivery: Think E. coli or anaerobic organisms
• Late onset (> 7 days): Think Chlamydia trachomatis
• Predisposing factors: Prolonged rupture of membranes, long labors, multiple vaginal examinations during labor, cesarean delivery especially following a long labor, and anemia
• Symptoms: Fever, chills, lower abdominal pain, anorexia, malaise, and malodorous vaginal discharge
• Signs: Elevated temperature, abdominal tenderness, mucopurulent vaginal discharge, and uterine and parametrial tenderness on bimanual pelvic examination; may have lower abdominal rebound tenderness (associated pelvic cellulitis and peritonitis)
• Source: Gabbe: Obstetrics - Normal and Problem Pregnancies, Third Edition (from MDConsult)

2. Failure to thrive: Common causes

FTT: Usually refers to growth below the 3rd or 5th percentile or a change in growth that has

crossed two major growth percentiles (i.e., from above the 75th percentile to below the 25th) in a short time.

• Nonorganic or psychosocial FTT: Child usually younger than age 5 yr with no known medical condition that cause poor growth
• Causes: 5-10% of low-birthweight children, children living in poverty, family discord, poor parent-child interactions, neonatal problems other than low birthweight, maternal depression, child abuse
• More common in U.S. than organic FTT


• Organic FTT: Underlying medical condition present

System	Cause
Gastrointestinal	Gastroesophageal reflux, celiac disease, pyloric stenosis, cleft palate/cleft lip, lactose intolerance, Hirschsprung's disease, milk protein intolerance, hepatitis, cirrhosis, pancreatic insufficiency, biliary disease, inflammatory bowel disease, malabsorption
Renal	Urinary tract infection, renal tubular acidosis, diabetes insipidus, chronic renal insufficiency
Cardiopulmonary	Cardiac diseases leading to congestive heart failure, asthma, bronchopulmonary dysplasia, cystic fibrosis, anatomic abnormalities of the upper airway, obstructive sleep apnea
Endocrine	Hypothyroidism, diabetes mellitus, adrenal insufficiency or excess, parathyroid disorders, pituitary disorders, growth hormone deficiency
Neurologic	Mental retardation, cerebral hemorrhages, degenerative disorders
Infectious	Parasitic or bacterial infections of the gastrointestinal tract, tuberculosis, human immunodeficiency virus disease
Metabolic	Inborn errors of metabolism
Congenital	Chromosomal abnormalities, congenital syndromes (fetal alcohol syndrome), perinatal infections
Miscellaneous	Lead poisoning, malignancy, collagen vascular disease, recurrently infected adenoids and tonsils

Source: Behrman: Nelson Textbook of Pediatrics, Sixteenth Edition

3. Causes of kernicterus (hemolytic disease of the newborn)

Kernicterus (a.k.a. bilirubin encephalopathy, nuclear jaundice): Yellow staining of basal ganglia and other CNS structures by unconjugated bilirubin with resultant neurologic damage

• Most significant long-term consequence of hemolytic disease of the newborn
• Causes: Rh or ABO erythroblastosis, G6PD deficiency, neonatal sepsis, Crigler-Najjar syndrome
• Source: BRS Pathology, Stedman’s Concise Medical Dictionary