Robert M. Starke, John A. Jane Jr., Ashok R. Asthagiri and John A. Jane Sr.
A historical perspective
Adam S. Kanter, Aaron S. Dumont, Ashok R. Asthagiri, Rod J. Oskouian, John A. Jane Jr. and Edward R. Laws Jr.
Over the last century, the transsphenoidal approach has evolved into the first-line method of treatment for sellar as well as select groups of parasellar and suprasellar lesions. The journey to its current popularity has been marked by controversy and near abandonment in the late 1920s, followed by its renaissance in the late 1960s. Despite the profound skepticism with which this procedure was viewed, several visionary neurosurgeons persevered through its nadir in popularity, preserving this surgical corridor to the skull base. Advances in medical and surgical techniques, paralleling an improved understanding of pituitary pathophysiology, contributed to its resurgence. The transsphenoidal procedures now performed stem from an array of modifications and refinements accumulated through nearly 100 years of medical and surgical evolution. This era's critical innovations and neurosurgical personalities are the topic of this historical overview.
Adam S. Kanter, Alfa O. Diallo, John A. Jane Jr., Jason P. Sheehan, Ashok R. Asthagiri, Rod J. Oskouian, David O. Okonkwo, Charles A. Sansur, Mary Lee Vance, Alan D. Rogol and EdwardR. Laws Jr
Despite ongoing advances in surgical and radiotherapeutic techniques, pediatric Cushing's disease remains a diagnostic and therapeutic challenge. The authors report on the results of a single-center retrospective review of 33 pediatric patients with Cushing's disease, providing details with respect to clinical presentation, diagnostic evaluation, therapeutic course, complications, and outcomes.
There were 17 female and 16 male patients whose mean age was 13 years (range 5–19 years) in whom a diagnosis of Cushing's disease was based on clinical and biochemical criteria. Typical symptoms included weight gain (91%), prepubertal growth delay (83%), round facies (61%), hirsutism (58%), headache (45%), abdominal striae (42%), acne (33%), amenorrhea (24%), and hypertension (24%). In 67% of the cases, preoperative magnetic resonance images revealed a pituitary lesion and in 82% of the cases the imaging studies effectively predicted lateralization. Inferior petrosal sinus sampling was performed in seven patients (21%), and in all of these cases lateralization was 100% reliable. Fifty-five percent underwent selective adenomectomies and 45% underwent subtotal hypophysectomies.
Complications included one case of diabetes insipidus, one of persistent hypocortisolemia necessitating prolonged glucocorticoid replacement therapy, and one minor vascular injury that did not necessitate postoperative management modification or cause sequelae. There were no surgery-related deaths and no cases of postoperative cerebrospinal fluid leakage or meningitis. During a mean follow-up period of 44 months, clinical remission was ultimately achieved in 91% of patients: 76% after transsphenoidal surgery alone and an additional 15% after adjuvant radiosurgery and/or adrenalectomy following surgical failure. Three patients (12%) experienced disease recurrence and underwent a second surgical procedure at 18, 81, and 92 months, respectively; based on clinical and biochemical criteria a second remission was achieved in all. Three patients (9%) remain with persistent disease.
Pediatric Cushing's disease is a rare condition, often requiring a multidisciplinary diagnostic and a multimodal therapeutic approach for successful long-term remission.