Late-onset transient adrenal insufficiency in preterm twins with twin-to-twin transfusion syndrome: A case report

Abstract

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.