Late-onset transient adrenal insufficiency in preterm twins with twin-to-twin transfusion syndrome

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.