Hydrops

Question 1

Define hydrops fetalis.

Answer 1

Abnormal fluid in 2+ different fetal compartments (subcutaneous edema, pericardial effusion, pleural effusion, ascites)
Often associated with polyhydramnios & placental thickening

Question 2

What are the three primary mechanisms of hydrops?

Answer 2

Intrauterine anemia
Intrauterine heart failure
Hypoproteinemia

Question 3

By what mechanism is the fetal oncotic pressure reduced in immune hydrops?

Answer 3

Impaired hepatic protein synthesis secondary to portal hypertension from hematopoiesis in the liver

Question 4

List five etiologic categories for non-immune hydrops, and give one specific example per category.

Answer 4

Cardiovascular (structural, cardiomyopathies, arrhythmias)
Chromosomal (Turners, trisomies)
Hematologic (hemoglobinopathies, FMH)
Infections (parvo, CMV)
Thoracic abnormalities (CCAM, diaphragmatic hernia, mediastinal tumours)
Lymphatic abnormalities (cystic hygroma)
Placental, twin, cord anomalies (TTTS, TRAP)
Kidney & urinary tract anomalies (bladder outlet obstruction)
Syndromic (Noonan)
Other rare disorders (inborn errors of metabolism, tumours)
Idiopathic

Question 5

Describe the history you would take from a woman with a hydropic fetus.

Answer 5

Current pregnancy: Accurate gestational age, symptoms of HDP (rule out mirror syndrome)
Previous pregnancies: Previous fetal, neonatal, infant deaths
Past medical history: Viral exposure or illness, travel, bleeding, medication use
Family history: Ethnic background, consanguinity
Genetic history: 3 generation pedigree (fetal or neonatal losses, developmental delays, congenital anomalies, genetic syndromes, chronic infantile illnesses)

Question 6

What are the three worst prognostic markers in cases of non-immune hydrops?

Answer 6

Chromosomal abnormalities
Gestational age < 24 weeks
Structural anomalies (excluding chylothorax)

Question 7

List seven urgent investigations for workup of hydrops.

Answer 7

Imaging:

• Detailed morphological US
• Dopplers
• Echo

Labs:

• CBC
• Nierhaus
• Group & screen
• Indirect Coombs
• VDRL
• Serology: parvo, toxo, CMV, rubella
• Liver enzymes, coags, uric acid (?mirror syndrome)
• Anti-Ro, anti-La
• Hgb electrophoresis, G6PD testing (depending on ethnicity