Epidemiology


Etiology

  • Chronic placental insufficiency
  • ↓ Renal output (e.g., due to bilateral renal agenesis, ARPKD, obstruction of posterior urethral valves)
  • Chronic amniotic fluid leakage

Pathophysiology

oligohydramnios → intrauterine compression and decreased amniotic fluid ingestions → ↓ space for fetal development → internal and external deformations


Clinical features

  • Pulmonary hypoplasia (cause of death due to severe neonatal respiratory insufficiency)
  • Craniofacial abnormalities (e.g., prominent epicanthal and infraorbital folds, flattened nose, receding chin, low set ears)
  • Wrinkling of the skin
  • Limb anomalies (e.g., bowed legs, clubbed feet)

Mnemonic

Potter babies cannot Pee.

Mnemonic

POTTER sequence associated with: Pulmonary hypoplasia Oligohydramnios (trigger) Twisted face Twisted skin Extremity defects Renal failure (in utero)


Diagnostics


Treatment