Causes of Death in Newborn with Double Nuchal Cord and Bilateral Adrenal Necrosis
The immediate cause of death is acute adrenal insufficiency (Addisonian crisis) leading to cardiovascular collapse; the antecedent cause is perinatal hypoxic-ischemic injury with resultant bilateral adrenal hemorrhagic necrosis and acute tubular necrosis; and the underlying cause is nuchal cord compression causing intrapartum asphyxia.
Pathophysiologic Sequence
Underlying Cause: Nuchal Cord Compression
- Double nuchal cord coil represents the initiating mechanical obstruction that compromised umbilical blood flow during labor, leading to fetal hypoxia-ischemia 1, 2
- Umbilical cord compression causes transient or permanent restriction of blood supply, with severity depending on intensity and duration of the hypoxic-ischemic insult 1
- The clinical presentation would typically include signs of perinatal asphyxia at birth, such as inability to cry or breathe spontaneously, requirement for assisted ventilation, and low Apgar scores (<5 at 5 and 10 minutes) 3
Antecedent Cause: Hypoxic-Ischemic Injury with Multi-Organ Failure
- Perinatal asphyxia from nuchal cord resulted in systemic hypoxic-ischemic injury affecting multiple organs, particularly those with high metabolic demands and vulnerable vascular beds 1, 2
- The bilateral adrenal glands are particularly susceptible to hemorrhagic necrosis during hypoxic-ischemic events due to their rich vascular supply and high metabolic activity 4
- Acute tubular necrosis of both kidneys developed as a consequence of ischemic injury, representing acute renal failure from hypoxic insult 4
- The relatively normal histology of other organs suggests the adrenals and kidneys bore the brunt of the ischemic injury, likely due to redistribution of blood flow during the hypoxic event 1
Immediate Cause: Acute Adrenal Insufficiency
- Extensive coagulative necrosis of bilateral adrenals resulted in complete loss of adrenal function, causing acute adrenal crisis (Addisonian crisis)
- Without functional adrenal tissue, the newborn could not produce cortisol or aldosterone, leading to:
- Profound hypotension and cardiovascular collapse from loss of mineralocorticoid and glucocorticoid support
- Severe electrolyte disturbances (hyponatremia, hyperkalemia)
- Hypoglycemia from cortisol deficiency
- Inability to mount stress response
- The acute tubular necrosis compounded the metabolic derangements through inability to regulate fluid and electrolyte balance 4
Clinical Context and Timing
- The biphasic pattern of hypoxic-ischemic encephalopathy may have been present, with initial resuscitation followed by secondary deterioration 36-96 hours after birth as adrenal insufficiency became clinically apparent 5
- Metabolic acidosis and cardiovascular instability would be expected findings in this clinical scenario 5
- The combination of bilateral adrenal necrosis and acute tubular necrosis indicates severe, prolonged hypoxic-ischemic insult rather than brief or mild asphyxia 1, 3
Key Diagnostic Considerations
- Laboratory findings would likely show:
- The absence of other organ pathology excludes alternative diagnoses such as sepsis, primary cardiac disease, or metabolic disorders 5
Critical Pitfall
Do not attribute death solely to "birth asphyxia" without recognizing the specific organ failure mechanisms. The nuchal cord caused the initial hypoxic event, but death resulted from the specific consequence of bilateral adrenal destruction, which is a recognized but uncommon complication of severe perinatal asphyxia 1, 4. The adrenal necrosis represents the critical link between the initial hypoxic insult and ultimate cardiovascular collapse.