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1414 PART IV Obstetric and Fetal Sonography

TABLE 41.1 Nonimmune Hydrops: Common Causes and Associations a

Cause

(Percentage of

Nonimmune Hydrops)

Mechanism

Examples

Cardiovascular (20%) Increased central venous pressure Structural heart disease (hypoplastic left or right

heart syndrome, atrioventricular canal)

Myocardial or pericardial tumors (rhabdomyoma in

tuberous sclerosis)

Tachyarrhythmia

Bradyarrhythmia (congenital heart block [anti-Rho]

or other maternal autoimmune disease)

Monochorionic twins

(4%)

Lymphatic dysplasia

(15%)

Neck/chest (2%)

Gastrointestinal (1%)

Urinary tract (1%)

Aneuploidy (13%)

Hematologic (9% b )

Infection (7%)

Inborn errors of

metabolism (1%)

Other syndromes

(5.5%)

Extrathoracic tumors

(1%)

Maternal

Placental/cord

Idiopathic 20%

Anemia and/or high-output cardiac failure

Abnormal lymphatic drainage

Vena caval obstruction or increased

intrathoracic pressure with impaired

venous return

Obstruction of venous return,

gastrointestinal obstruction and infarction

with protein loss and decreased colloid

osmotic pressure

Urinary ascites; nephrotic syndrome with

hypoproteinemia

Cardiac anomalies, lymphatic dysplasia,

anemia, abnormal myelopoiesis

Anemia, high-output cardiac failure; hypoxia

(α-thalassemia)

Anemia, anoxia, endothelial cell damage,

increased capillary permeability,

myocarditis

Anemia

Hemolysis

Visceromegaly, obstruction of venous

return, decreased erythropoiesis,

anemia, hypoproteinemia

Anemia, high-output cardiac failure,

hypoproteinemia

a Adapted from references 4, 23, 36, 43, 226, and 230-237.

b Much higher percentage of hydrops in regions with α-thalassemia, such as Southeast Asia.

Twin-to-twin transfusion syndrome (usually the

recipient but can occur in the donor), twin

anemia polycythemia sequence

Acardiac twin (donor)

Congenital lymphatic dysplasia

Cystic hygroma

Congenital high airway obstruction

Pleural effusion (chylothorax)

Chest mass (congenital pulmonary airway

malformation, sequestration, congenital

diaphragmatic hernia)

Portal vein thrombosis

Volvulus

Obstruction

Meconium peritonitis

Finnish nephrosis, urinary tract obstruction

45,XO, trisomy 21, trisomy 18, triploidy

α-Thalassemia (homozygous)

Fetomaternal transfusion

Parvovirus, cytomegalovirus, adenovirus, syphilis,

toxoplasmosis

Lysosomal storage disease,

mucopolysaccharidoses,

Gaucher disease, Niemann-Pick disease

G6PD deiciency

Noonan syndrome

Skeletal dysplasias such as achondroplasia,

osteogenesis imperfecta, thanatophoric

dysplasia arthrogryposis, congenital myotonic

dystrophy

Vascular/lymphatic tumors

Teratoma, neuroblastoma, arteriovenous

malformation

Severe diabetes mellitus, severe anemia, severe

hypoproteinemia, thyrotoxicosis

Indomethacin use (premature closure of ductus

arteriosus)

Placental or umbilical vein thrombosis, cord knot,

chorioangioma

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