Perinatal Pathology Case Studies


CASE 4: PROM with Intrauterine Infection and HMD


History:

A 21-year-old primigravida has noted leakage of amniotic fluid for a couple of days and presents to the emergency room with a fever of 38.8 C. On physical examination, there is a foul-smelling vaginal discharge. A culture is taken. The cervix is dilated to 5 cm. An ultrasound reveals a fetus of 28 week gestational age with evidence for cardiac activity. She is in premature labor. Several hours later, a 900 gm male fetus is delivered. The Apgar scores are 5 at 1 minute and 6 at 5 minutes. The baby receives artificial surfactant via endotracheal tube following intubation. The baby is ventilated with PEEP. The following day, the baby has a generalized seizure and cranial ultrasound reveals a massive intraventricular hemorrhage. The baby expires later that day.

Image 4.1:

The lungs are shown here? What do you see?
There are numerous pink hyaline membranes lining poorly aerated alveoli. There is also evidence for pulmonary interstitial emphysema from dissection of air from bronchioles or alveolar ducts out to the pleura. This may rupture out and produce a pneumothorax.

Image 4.2:

The fetal membranes and cord are shown here. What is wrong?
There are numerous neutrophils in the fetal membranes (acute chorioamnionitis) and umbilical cord (acute funisitis).

Image 4.3:

This is a sagittal section of the brain. What is the lesion?
There is a bilateral intraventricular hemorrhage.

Questions:

  1. Is this baby small, appropriate, or large for gestational age?
  2. The baby is appropriate for gestational age.

  3. What is the pathophysiology of premature rupture of membranes? What did the culture probably grow?
  4. The premature rupture of membranes (PROM) leads to leakage of amniotic fluid. Since the amniotic cavity is no longer an enclosed environment, infectious agents can reach the fetus. The risk for infection is very high 24 hours after PROM. The inflammation leads to prostaglandin production and this leads to premature labor. The culture in this case grew group B streptococcus.

  5. What is the cause for the pulmonary disease?
  6. The baby has hyaline membrane disease (HMD) of the newborn. This is due to the prematurity with lung immaturity such that there is not enough surfactant. Use of artifical surfactant has helped to reduce the problems of HMD, but it has not entirely eliminated the problem.

  7. What is the cause for the intraventricular hemorrhage?
  8. From about 22 to 30 weeks gestation, the fetal brain is very susceptible to develop intraventricular hemorrhage during birth.

  9. Name the major causes for congenital infections.
    • T = toxoplasmosis
    • O = other: syphilis, bacteria (group B strep, listeria)
    • R = rubella
    • C = cytomegalovirus
    • H = herpes simplex, HIV

  10. What social and ethical issues are raised with long-term ventilatory support?

  11. The question becomes whether the baby can eventually be weaned from the ventilator, and what to consider if the baby cannot. Doctors and nurses may indicate to parents that the worst cases 'decide for themselves' in effect choosing words that make it seem like the patient had made a decision, when in truth medical realities beyond everyone's control made the decision irrelevant. This may be a way to try and make parents of children (or families of older patients) feel better--a sort of predestination or 'fate' as if it was meant to be thus.

    However, there will probably be cases where nature doesn't make the decision because we can apply technology for continued life support, but at great financial and emotional costs to society, the patient, and the family. Continued application of the most advanced technology tends to have more bad outcomes than good outcomes, and the technology is probably best used only when there is reasonable probability of a reversible condition of a day's to a week's duration. The costs are very high, and the number of people with sufficient skill very few, so transport to a major neonatal ICU within a specialized pediatric hospital is usually necessary. To this you add to the total costs to the family, including financial and emotional, when all this happens away from home and away from the rest of the family, their church, their neighbors, and other support systems.