Placental Dysfunction Syndrome (Postmaturity Syndrome, Dysmaturity)

1. Introduction & Definition

• Definition: A clinical syndrome characterized by distinctive physical changes in the newborn resulting from progressive placental senescence and insufficiency.
• Synonyms: Postmaturity Syndrome, Dysmaturity, Clifford’s Syndrome.
• Incidence: Occurs in ~20–43% of post-term pregnancies (>42 weeks); can occasionally occur in term infants with placental insufficiency.
• Historical Context: First described by Clifford in 1954, classifying the physical stigmata of prolonged gestation and placental failure.

2. Etiology & Pathophysiology

The primary mechanism is Chronic Placental Insufficiency due to placental aging.

• Placental Senescence:
  • Decreased villous vascularity and intervillous space.
  • Increased fibrin deposition and calcification (Grade III placenta).
  • Reduced surface area for gas and nutrient exchange.
• Fetal Consequences:
  • Nutritional Deprivation: Loss of subcutaneous fat and muscle mass (wasting) due to utilization of glycogen and fat stores.
  • Chronic Hypoxia: Leads to polycythemia (increased erythropoietin) and potential neurological injury.
  • Oligohydramnios: Reduced fetal urine output due to renal hypoperfusion; increases risk of cord compression.
  • Meconium Passage: Hypoxia stimulates gut peristalsis and sphincter relaxation; oligohydramnios makes meconium thick/particulate (aspiration risk).

3. Clinical Features (Clifford’s Staging)

Infants are typically SGA (Small for Gestational Age) or have signs of wasting despite normal length/head circumference (asymmetrical growth restriction).

General Appearance

• "Old Man" Look: Wrinkled, patchy skin due to loss of subcutaneous fat.
• Alertness: Baby appears wide-eyed, alert, and hungry ("worried" expression).
• Body: Long and thin; skin may hang loosely around thighs/buttocks.
• Skin: Dry, cracked, peeling (desquamation), parchment-like; absence of vernix caseosa and lanugo.
• Nails: Long and stained.

Clifford’s Classification of Postmaturity

4. Complications

A. Intrapartum

• Fetal Distress: Late decelerations due to uteroplacental insufficiency.
• Cord Compression: Due to oligohydramnios.
• Meconium Aspiration Syndrome (MAS): High risk; meconium is often thick.
• Trauma: If macrosomia is present (dysmaturity can coexist with macrosomia in diabetic pregnancies, but typically these infants are wasted).

B. Neonatal (Metabolic & Systemic)

• Hypoglycemia: Depleted hepatic glycogen stores and reduced gluconeogenesis.
• Hypothermia: Loss of subcutaneous fat (insulation) and poor metabolic reserve.
• Polycythemia: Hematocrit >65% due to chronic hypoxia.
• Perinatal Asphyxia: Low Apgar scores; risk of Hypoxic-Ischemic Encephalopathy (HIE).
• PPHN (Persistent Pulmonary Hypertension): Secondary to chronic hypoxia and pulmonary remodeling.

5. Diagnosis & Evaluation

Antenatal (Surveillance)

• Dating: Accurate confirmation of gestational age (LMP, 1st-trimester USG).
• Doppler Velocimetry: Umbilical artery (S/D ratio, absent/reversed diastolic flow) and MCA Doppler (brain sparing effect).
• Biophysical Profile (BPP): Specifically looking for oligohydramnios (AFI <5 cm).

Postnatal

• Clinical Assessment: Assessment of gestational age (Ballard Score) vs. physical appearance.
  • Discrepancy: Baby scores "post-term" on neuromuscular maturity but looks malnourished.
• Ponderal Index (PI): Calculation: $Weight(g)\times 100/Length(cm{)}^3$. PI is typically low (<2.2), indicating wasting.
• Labs:
  • Blood Glucose (monitoring for hypoglycemia).
  • Hematocrit (screen for polycythemia).
  • Arterial Blood Gas (if respiratory distress/asphyxia).
  • Calcium (risk of hypocalcemia).

6. Management

A. Obstetric Management

• Induction of Labor: generally recommended at 41+ weeks to prevent the syndrome.
• Intrapartum: Continuous fetal heart rate monitoring. Amnioinfusion (controversial) for severe variable decelerations/oligohydramnios.

B. Neonatal Management

1. Resuscitation:
   • Be prepared for Meconium Aspiration.
   • Current NRP Guidelines: If meconium-stained and vigorous $\to$ Routine care. If non-vigorous $\to$ Initiate PPV if not breathing (routine endotracheal suctioning is no longer recommended).
2. Thermoregulation:
   • Aggressive prevention of heat loss (Kangaroo Mother Care, radiant warmer).
3. Metabolic Support:
   • Hypoglycemia: Early breastfeeding (within 1 hour). Screen glucose at 2 hours. IV Dextrose (Start at 6-8 mg/kg/min) if symptomatic or persistent hypoglycemia.
4. Respiratory Support:
   • Manage MAS (Oxygen, CPAP, Surfactant, iNO, or ECMO for severe PPHN).
5. Polycythemia:
   • Hydration. Partial exchange transfusion if symptomatic (Hct >65-70% with symptoms).

7. Prognosis

• Mortality: Perinatal mortality is increased 2–3 fold compared to term infants (primarily due to asphyxia and MAS).
• Morbidity:
  • Short-term: Respiratory failure, seizures (HIE).
  • Long-term: Increased risk of cerebral palsy and neurodevelopmental delay if significant intrapartum asphyxia occurred.
  • Barker Hypothesis: Intrauterine growth restriction/dysmaturity is linked to adult-onset metabolic syndrome (hypertension, diabetes, CAD).