Hermann Schloffer and the origin of transsphenoidal pituitary surgery

A little over a century ago, in 1907, at the University of Innsbruck, Hermann Schloffer performed the first transsphenoidal surgery on a living patient harboring a pituitary adenoma. Schloffer used a superior nasal route via a transfacial lateral rhinotomy incision. This was perhaps his greatest academic contribution to neurosurgery. Despite the technological limitations of that time, Schloffer's operation was groundbreaking in that it laid the foundation for future development and refinement of transsphenoidal pituitary surgery, influencing prominent surgeons such as Oskar Hirsch and Harvey Cushing. Even after undergoing multiple modifications and a brief fall into obscurity, the transsphenoidal approach has endured through generations of surgeons and remains the preferred approach for lesions of the sella turcica to this day. Although Schloffer performed primarily abdominal surgery in his practice, his contributions to the transsphenoidal approach have had a lasting impact in the field of pituitary and skull base surgery. The authors review the life and career of Hermann Schloffer, the surgical details of his transsphenoidal operation, and the legacy that it has left on the field of pituitary surgery.

Modified orbitozygomatic craniotomy for craniopharyngioma resection in children

Object In this study, the authors evaluated the efficacy and safety of modified orbitozygomatic craniotomy for resection of craniopharyngioma in children. Methods A prospective, institutional review board–approved database was retrospectively reviewed for pediatric patients undergoing craniopharyngioma resection performed by a single surgeon. Results Ten patients underwent craniopharyngioma resection surgery between July 2000 and January 2006 (4 girls and 6 boys, ages 1.5–17 years). Nine patients presented to the authors' institution, and 1 patient was referred after surgery and radiation therapy were administered elsewhere. Nine patients presented with visual field deficits (2 with unilateral or bilateral light perception only) and 5 with endocrine dysfunction. Eight patients had large tumors that significantly displaced the optic chiasm and hypothalamus. All patients underwent a modified frontotemporal orbitozygomatic osteotomy in a single piece. The lamina terminalis was opened in 4 patients with third ventricular extension. One patient required a staged transsphenoidal operation to remove residual tumor in the sella turcica, and 1 patient underwent a contralateral subtemporal approach to resect a daughter lesion in the prepontine cistern. Complete radiographic resection was achieved in all patients. Follow-up averaged 55 months (range 12–95 months). Vision was improved in 8 patients and remained stable in 2. All patients had postoperative endocrine dysfunction. One patient experienced transient cranial nerve IV palsy and 1 suffered a small caudate stroke 5 months after surgery without sequelae. Two patients experienced polyphagia and weight gain without other symptoms of hypothalamic dysfunction. There were no other new neurological deficits. Conclusions Modified orbitozygomatic craniotomy provides excellent exposure of the suprasellar region with minimal brain retraction, allowing complete resection of craniopharyngiomas with good visual and neurological results.

Results of Transsphenoidal Extirpation of Craniopharyngiomas and Rathke's Cysts

Fourteen patients undergoing transsphenoidal extirpation of craniopharyngiomas or Rathke's cysts underwent endocrinological evaluation before and after surgery. One patient died during the procedure because of uncontrollable arterial bleeding. The other patients were followed for up to 16 years (average, 8.5 years). One patient had recurrence of tumor 12 years after surgery. Another patient, the only one operated on because of a recurrent craniopharyngioma after previous radiation therapy, died 2 years after the transsphenoidal operation. No patient recovered pituitary functions that had been lost before surgery. One patient, who had an isolated growth hormone deficiency, and another, who had panhypopituitarism with sustained antidiuretic hormone secretion, had no change postoperatively from their preoperative endocrine status. The other 11 patients lost pituitary function and required pharmacological replacement of one to four pituitary hormones.

Observations on the Pathophysiology of Nelson's Syndrome: A Report of Three Cases

Nelson's syndrome is generally regarded as an unusual sequela of primary bilateral adrenalectomy when performed for Cushing's disease. It is classically defined by cutaneous hyperpigmentation, considerably elevated adrenocorticotropic hormone (ACTH) levels, and an enlarged sella turcica. In this report, we present three cases initially treated by transsphenoidal sellar exploration for Cushing's disease. In two of these cases, remission of hypercortisolism did not occur after the initial pituitary exploration. A microadenomectomy was performed in one case and, in the other, no microadenoma was found. In both, Nelson's syndrome occurred after adrenalectomy. A second transsphenoidal operation and radiotherapy were required to control tumor growth. In another case, transsphenoidal adenomectomy of an ACTH-secreting tumor initially led to a remission of hypercortisolism for 4 years, but recurrent Cushing's disease necessitated adrenalectomy, and again Nelson's syndrome occurred. The documentation of a pre-existing ACTH-secreting basophilic pituitary microadenoma before adrenalectomy, as seen in two of our cases, has not been previously reported, and these observations of “non-classical” courses have major implications for the pathophysiology of Nelson's syndrome.

Spontaneous Postoperative Normalization of Growth Hormone Levels in Two Patients with Acromegaly Not Cured by Transsphenoidal Surgery

Postoperative persistence of elevated growth hormone (GH) levels in patients with acromegaly usually suggests residual adenoma tissue secreting GH and is an indication for further treatment. In rare cases, spontaneous normalization of serum GH levels in patients with acromegaly has been reported to occur as a consequence of intratumoral hemorrhage (pituitary apoplexy). We report two patients in whom persisting acromegaly was documented 1 and 2 weeks, respectively, after transsphenoidal operation and who experienced spontaneous normalization of GH levels. In one patient, this favorable outcome was found 1.5 years after the operation and followed a pregnancy. The other patient, whose GH values normalized 3 months after operation, had received preoperative treatment with a new, long-acting somatostatin analog.

Response of luteinizing hormone secreting pituitary adenoma to a long-acting somatostatin analogue

A large pituitary tumour was discovered in a 20 year old man who came to medical attention bacause of grand-mal seizures. The tumour produced biologically active LH as demonstrated by supranormal plasma LH and plasma testosterone values. Free alpha-subunit values were also elevated. In contrast, plasma FSH was in the lower normal range. Transsphenoidal operation failed to remove all tumour tissue. Detailed studies were carried out in the postoperative period. TRH and GnRH administration were associated with a rise of plasma LH and alpha-subunit, whereas plasma FSH was low and unresponsive. Bromocriptine treatment was ineffective. In contrast, both during and after treatment with SMS 201-995 for 6 weeks, a decrease of basal plasma LH values was observed. Furthermore, the administration of a single dose of SMS 201-995 reproducibly induced a decrease of plasma LH lasting for a period of about 6 h. The study suggests that SMS 201-995 may be useful in the treatment of patients with gonadotrope cell adenomas.