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Long-Term Somatic Follow-Up of Prenatally Treated Children with Congenital Adrenal Hyperplasia¹

Department of Woman and Child Health, Pediatric Endocrinology Unit (S.L., E.M.R., M.H.), and the Department of Molecular Medicine, Clinical Genetics Unit (S.L., A.W., T.-H.B.), Karolinska Hospital, S-171 76 Stockholm, Sweden

Address all correspondence and requests for reprints to: Svetlana Lajic, M.D., Ph.D., Pediatric Endocrinology Unit (Q2:08), Karolinska Hospital, S-171 76 Stockholm, Sweden. E-mail: svetlana.lajic{at}kbh.ki.se

Prenatal virilization of female fetuses is a serious symptom associated with severe congenital adrenal hyperplasia. In attempt to avoid sexual ambiguity, prenatal treatment of 21-hydroxylase deficiency was initiated in 1984, with the first Scandinavian case treated in 1985. Here we have studied the outcome of prenatal diagnosis and therapy of 44 at-risk pregnancies monitored during the years 1985–1995 in Scandinavia. Treated mothers and children were compared with matched controls.

Compared to their elder affected sisters, all 5 girls with severe congenital adrenal hyperplasia who were treated until term showed little virilization. Only 1 required surgery for labial fusion. The majority of the 44 dexamethasone-treated fetuses demonstrated normal pre- and postnatal growth compared to matched controls. However, several adverse events such as failure to thrive and delayed psychomotor development, were reported among the treated infants. In addition, treated mothers reported more side-effects during pregnancy than did controls. A significant increase in weight gain was observed during early pregnancy when treatment was initiated, but this initial rapid weight gain declined during late pregnancy or when treatment was terminated.

Thus, experience to date suggests that prenatal treatment of affected female fetuses is generally efficient in minimizing virilization of external genitalia. However, there is still a need to collect more data concerning possible rare unfavorable effects of this therapy on mother and child.

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