Pathology
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Fetopathology and developmental pathology of the embryo and fetus
Marta Ježová, Josef Feit et al.
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Chronic twin transfusion syndrome
Etiology

There are anastomoses between the circulations of the two fetuses in the monochorionic placenta.

Classification

Types of anastomoses:

  • arterio — arterial (a-a) superficial
  • veno — venous (v-v) superficial
  • arterio — venous (a-v) deep
Clinical signs
  • with balanced blood flow in both directions, the fetuses are not in danger
  • if the blood flow is unbalanced, predominantly one way — because of majority of large a-v anastomoses, where the pressure gradient makes the blood flow from the artery into the vein, the effect is that one twin, the donor, has decreased blood volume (hypovolemia), while the other twin, the recipient, has increased blood volume (hypervolemia)
  • neither fetus has optimal conditions for further intrauterine growth, one or both can die
  • this condition usually begins around 20th w.g., 15 – 30% monochorionic diamniotic twins present this syndrome

Consequences:

  • intrauterine death of one or both twins
  • serious intrauterine problems result into premature delivery, high perinatal mortality and morbidity
  • possible intervention:
    • amnioreduction
    • in some cases it is possible to occlude the anastomoses (laser ablation)
Macroscopic appearance
  • Donor:
    • intrauterine growth retardation
    • anemic
    • oligohydramnion (with insufficient renal perfusion the fetus has decreased urinary output)
    • compressed in the uterus by overpressure of the other twin's polyhydramnion, stuck twin
    • deformites of lower limbs
    • often it has a velamentous umbilical cord insertion
  • Recipient:
    • larger fetus, thrives on the expenses of the donor twin
    • plethoric
    • polyhydramnion
    • cardiac hypertrophy as a result volume overload, congestive heart failure
    • hepatosplenomegaly
    • ascites, hydropericardium and hydrops — severe generalized edema
    • usually central insertion of the umbilical cord

Chronic twin transfusion syndrome (72409)

Pictures

Chronic twin transfusion syndrome: Twins, fetofetal transfusion, Macro, autopsy (72409)

Another case of chronic twin transfusion syndrome: Twins, feto-fetal transfusion, Macro, autopsy (73975)

Case study
Fetofetal transfusion syndrome
Marta Ježová
History

Spontanous twin gestation, ultrasound examination in 18th w.g shows two female fetuses, severe fetofetal transfusion, donor — stuck twin, no congenital malformations; recipient with hepatosplenomegaly and also no congenital malformations.

Macroscopic appearance
  • Twin A — donor:
    • female
    • body weight 152 g
    • body length 21.5 g
    • weight of brain 35.7 g
    • weight of liver 5.5 g
    • weight of heart 0.67 g
    • intrauterine growth retardation
    • bladder and ureter hypoplasia
    • deformites of lower limbs
    • agenesis of the left umbilical artery
  • Twin B — recipient:
    • female
    • body weight 210 g
    • body length 16.5 cm
    • weight of brain 39.5 g
    • weight of liver 16.53 g!
    • weight of heart 2.21 g!
    • urinary bladder dilatation, coiled ureters
    • has both umbilical arteries
  • Placenta:
    • monochorionic diamnionic
    • insertion of the twin septum is distictly asymmetric (oligohydramnion versus polyhydramnion)
    • Twin A (donor): marginal (paraseptal) insertion of the umbilical cord
    • Twin B (recipient): central insertion of the umbilical cord
    • A-V anastomoses were found