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Role of bilateral adrenalectomy in Cushing's disease

  • International Association of Endocrine Surgeons—Manuscripts Presented at the 35th World Congress of the International Society of Surgery, Hong Kong, August 1993
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Abstract

Of 206 patients with Cushing's syndrome observed from 1975 through 1991, 144 (69.9%) had pituitary-dependent Cushing's disease. Of the 110 patients who underwent pituitary surgery, 31 (28%) developed an early recurrence and 23 patients (20%) had a late relapse 1 to 5 years after primary pituitary exploration. We performed a one- or two-step total bilateral adrenalectomy in 43 patients—9 men and 34 (79.4%) women, with an average age of 47.5 years (range 13–58 years). Thirty-three of these patients had already been treated by previous transsphenoidal surgery or alternatively by external pituitary irradiation over a period of 1 to 10 years prior to adrenal surgery. Thirty-one patients underwent adrenalectomy by a double lumbar access or left lumbar and right subcostal incisions. In our series of 55 operations, perioperative complications included two splenectomies and two hemorrhages (7.3%). The early mortality rate was 3.6% (two patients). Minor complications consisted of wound infection (13.5%), bronchopneumonia or pneumothorax (four cases) with a 7- to 12-day longer hospital stay. Nelson syndrome occurred in 6 of 41 patients (14.6%). We therefore believe that bilateral adrenalectomy does play a major role in the treatment of patients with pituitary-dependent Cushing's disease unsuccessfully managed by trans-sphenoidal surgery. It represents the definitive therapy for those patients in whom hypophysectomy was not able to provide satisfactory control of the disease.

Résumé

Parmi 206 patients ayant un syndrome de Cushing, observés entre 1975 et 1991, 144 (69.9%) avaient une maladie de Cushing hypophysaire. Trente et un (28%) parmi 110 patients qui ont été opérés de l'hypophyse ont eu une récidive précoce alors que 23 (20%) ont eu une récidive tardive, un à cinq ans après l'exploration initiale. Une surrénalectomie totale bilatérale a été réalisée chez 43 patients (9 hommes et 34 (79.4%) femmes, d'âge moyen de 47.5 ans (extrêmes 13–58)). Trente-trois de ces patients avaient déjà été traités par une chirurgie trans-sphénoïdienne ou, alternativement, par la radiothérapie externe hypophysaire, un à 10 ans avant la surrénalectomie. Trente et un patients ont eu une surrénalectomie bilatérale par une lombotomie bilatérale ou une lombotomie gauche associée à une à une incision sous-costale droite. Les complications périopératoires observées ont été deux splénectomies et deux hémorragies (7.3%). La mortalité précoce a été de 3.6% (2 patients). Les complications mineures ont été un abcès de paroi (13.5%), une infection pulmonaire ou pneumothorax (4 cas) rallongeant le séjour hospitalier de 7 à 12 jours. Un syndrome de Nelson a été observé chez 6 des 41 patients (14.6%). Nous pensons que la surrénalectomie bilatérale est une alternative importante dans le traitement de la maladie de Cushing hypophysaire ne réagissant pas à la chirurgie trans-sphénoïdienne, puisqu'elle représente une thérapeutique définitive pour les patients qui peuvent être traités efficacement par l'hypophysectomie.

Resumen

De 206 pacientes con síndrome de Cushing estudiados entre 1975 y el final de 1991, 144 (69.9%) presentaron enfermedad de Cushing dependiente de la pituitaria. Treinta y uno (28%) de 110 pacientes sometidos a cirugía hipofisiaria desarrollaron una recidiva precoz y 23 pacientes (20%) una recidiva tardía entre 1 y 5 años luego de la exploración de la pituitaria. Hemos practicado adrenalectomía total bilateral en una o dos etapas en 43 pacientes, 9 del sexo masculino y 34 (79.4%) del femenino, con una edad promedio de 47.5 años (rango 13–58). Treinta y tres de estos pacientes ya habían sido tratados mediante cirugía transesfenoidal previa o, alternativamente, mediante irradiaciòn externa de la pituitaria en un período de 1 a 10 años previos a la cirugía suprarenal. Treinta y un pacientes fueron sometidos a adrenalectomía por un doble acceso lumbar o por incisión lumbar izquierda y subcostal derecha. En nuestra serie de 55 operaciones las complicaciones perioperatorias incluyeron 2 esplenectomías y 2 hemorragias (7.3%). La tasa de mortalidad precoz fue 3.6% (2 pacientes). Las complicaciones menores consistieron en infección de la herida (13.5%), bronconeumonía o neumotórax (4 casos), con una prolongación de la hospital-ización de 7–12 días. El síndrome de Nelson se presentó en 6 de 41 pacientes (14.6%). Nuestra conclusión es que la adrenalectomía bilateral juega un papel de importancia en el tratamiento de la enfermedad de Cushing dependiente de la pituitaria cuando ha fallado la cirugía transesfenoidal, puesto que representa la modalidad terapéutica definitiva para estos pacientes en quienes la hipofisectomía no resultó en un control satisfactorio de la enfermedad.

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Authors and Affiliations

  1. Cattedra di Endocrinochirurgia, Istituto di Clinica Chirurgica I, Via Giustiniani 2, 35128, Padua, Italy

    Gennaro Favia M.D., Marco Boscaro M.D., Franco Lumachi M.D. & Davide F. D'Amico M.D.

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  1. Gennaro Favia M.D.
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  2. Marco Boscaro M.D.
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  3. Franco Lumachi M.D.
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  4. Davide F. D'Amico M.D.
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Favia, G., Boscaro, M., Lumachi, F. et al. Role of bilateral adrenalectomy in Cushing's disease. World J. Surg. 18, 462–466 (1994). https://doi.org/10.1007/BF00353737

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