TRANSNASAL MICROSURGERY IN CHILDREN AND ADOLESCENTS WITH CUSHINGS-DISEASE

OBJECTIVE: Transnasal adenomectomy is the treatment of choice for Gush ing's disease. We review the diagnostic peculiarities, specific surgic al problems, and outcome of children and adolescents with Gushing's di sease. METHODS: We report on our series of 55 consecutive children and adolescents (range, 4.2-18.9 yr [mean age, 14.4 yr]; female:male = 1. 1:1.0; mean follow-up, 54.5 +/- 38.6 mo [standard deviation]) with Gus hing's disease on whom we performed surgery since 1980. The indication for transsphenoidal surgery is based on endocrinological parameters a nd not on neuroradiological findings. RESULTS: Detection rate of the t umor site was 22% using computed tomography and 33% using magnetic res onance imaging. Only 7 of 13 interpetrosal adrenocorticotropic hormone gradients obtained during inferior petrosal sinus sampling correctly lateralized the tumor site preoperatively (53.8%). In cases of incompl ete sphenoid pneumatization, adequate exposure is achieved by drilling . The tumor finding rate is 98%. The remission rate is 100% when two e arly subsequent operations are included. The recurrence rate for 45 pr imary operations with follow-up of at least 1 year is 15.5%. Seven of nine subsequent operations for recurrent hypercortisolism were success ful. One patient needed three more operations until hypercortisolism s ubsided; one patient achieved remission after additional pituitary irr adiation. The surgical morbidity was low in this series, which consist ed of two cerebrospinal fluid fistulas. The incidence of hypopituitari sm after primary operations (10.3%) is significantly lower than after subsequent operations (45.5%). CONCLUSION: Direct transnasal submucosa l surgery for Gushing's disease is successful, and pituitary function can be preserved in most of these young patients.