TY - JOUR
T1 - Prevalence of Nelson’s syndrome after bilateral adrenalectomy in patients with cushing’s disease
T2 - a systematic review and meta-analysis
AU - Papakokkinou, Eleni
AU - Piasecka, Marta
AU - Carlsen, Hanne Krage
AU - Chantzichristos, Dimitrios
AU - Olsson, Daniel S.
AU - Dahlqvist, Per
AU - Petersson, Maria
AU - Berinder, Katarina
AU - Bensing, Sophie
AU - Höybye, Charlotte
AU - Engström, Britt Edén
AU - Burman, Pia
AU - Follin, Cecilia
AU - Petranek, David
AU - Erfurth, Eva Marie
AU - Wahlberg, Jeanette
AU - Ekman, Bertil
AU - Åkerman, Anna Karin
AU - Schwarcz, Erik
AU - Johannsson, Gudmundur
AU - Falhammar, Henrik
AU - Ragnarsson, Oskar
PY - 2021
Y1 - 2021
N2 - Purpose: Bilateral adrenalectomy (BA) still plays an important role in the management of Cushing's disease (CD). Nelson’s syndrome (NS) is a severe complication of BA, but conflicting data on its prevalence and predicting factors have been reported. The aim of this study was to determine the prevalence of NS, and identify factors associated with its development. Data sources: Systematic literature search in four databases. Study Selection: Observational studies reporting the prevalence of NS after BA in adult patients with CD. Data extraction: Data extraction and risk of bias assessment were performed by three independent investigators. Data synthesis: Thirty-six studies, with a total of 1316 CD patients treated with BA, were included for the primary outcome. Pooled prevalence of NS was 26% (95% CI 22–31%), with moderate to high heterogeneity (I2 67%, P < 0.01). The time from BA to NS varied from 2 months to 39 years. The prevalence of NS in the most recently published studies, where magnet resonance imaging was used, was 38% (95% CI 27–50%). The prevalence of treatment for NS was 21% (95% CI 18–26%). Relative risk for NS was not significantly affected by prior pituitary radiotherapy [0.9 (95% CI 0.5–1.6)] or pituitary surgery [0.6 (95% CI 0.4–1.0)]. Conclusions: Every fourth patient with CD treated with BA develops NS, and every fifth patient requires pituitary-specific treatment. The risk of NS may persist for up to four decades after BA. Life-long follow-up is essential for early detection and adequate treatment of NS.
AB - Purpose: Bilateral adrenalectomy (BA) still plays an important role in the management of Cushing's disease (CD). Nelson’s syndrome (NS) is a severe complication of BA, but conflicting data on its prevalence and predicting factors have been reported. The aim of this study was to determine the prevalence of NS, and identify factors associated with its development. Data sources: Systematic literature search in four databases. Study Selection: Observational studies reporting the prevalence of NS after BA in adult patients with CD. Data extraction: Data extraction and risk of bias assessment were performed by three independent investigators. Data synthesis: Thirty-six studies, with a total of 1316 CD patients treated with BA, were included for the primary outcome. Pooled prevalence of NS was 26% (95% CI 22–31%), with moderate to high heterogeneity (I2 67%, P < 0.01). The time from BA to NS varied from 2 months to 39 years. The prevalence of NS in the most recently published studies, where magnet resonance imaging was used, was 38% (95% CI 27–50%). The prevalence of treatment for NS was 21% (95% CI 18–26%). Relative risk for NS was not significantly affected by prior pituitary radiotherapy [0.9 (95% CI 0.5–1.6)] or pituitary surgery [0.6 (95% CI 0.4–1.0)]. Conclusions: Every fourth patient with CD treated with BA develops NS, and every fifth patient requires pituitary-specific treatment. The risk of NS may persist for up to four decades after BA. Life-long follow-up is essential for early detection and adequate treatment of NS.
KW - Bilateral adrenalectomy
KW - Corticotroph adenoma
KW - Cushing’s disease
KW - Nelson’s syndrome
U2 - 10.1007/s11102-021-01158-z
DO - 10.1007/s11102-021-01158-z
M3 - Article
C2 - 34036460
AN - SCOPUS:85106532324
SN - 1386-341X
VL - 24
SP - 797
EP - 809
JO - Pituitary
JF - Pituitary
IS - 5
ER -