Rationale: Late-onset transient adrenal insufficiency with circulatory collapse is a rare condition that occurs in preterm infants. Although the incidence of late-onset transient adrenal insufficiency in preterm infants has been reported in Japan, reports from Western countries are lacking. In addition, no study has investigated the effect of twin-to-twin transfusion syndrome (TTTS) in monozygotic twins. Patient concerns: A pair of extremely low birth weight twins presented with TTTS. Diagnoses: Both twins developed late-onset adrenal insufficiency with oliguria, hypotension, hyponatremia, and pulmonary edema at a postnatal age of 24 days and 51 days, respectively. Intervention: Temporary administration of intravenous hydrocortisone was initiated. Outcomes: Their symptoms improved dramatically and they survived the event without any neurologic sequelae after 3 years of follow-up. Lessons: Late-onset circulatory collapse may occur, especially in extremely preterm infants, even at 2 months after birth. Hydrocortisone therapy is an effective treatment to rescue circulatory collapse caused by adrenal insufficiency in preterm infants and may not affect long-term neuromotor and cognitive outcomes.
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