Tension pneumocranium, a rare complication of transsphenoidal pituitary surgery: Mayo Clinic experience 1976-1998.

We describe four cases of symptomatic pneumocranium, a rare, potentially life-threatening complication of transsphenoidal pituitary surgery. Symptomatic pneumocranium manifested as impaired mental status, headaches, and grand mal seizures, early in the postoperative course after transsphenoidal pituitary surgery. Furthermore, a Cushing response, including systemic hypertension and bradycardia (secondary to intracranial hypertension) was seen, which has not been previously described in association with symptomatic pneumocranium. We describe a previously unreported risk factor for tension pneumocranium, untreated obstructive sleep apnea. Other factors predisposing to tension pneumocranium in our patients included: cerebrospinal fluid leaks, postoperative positive-pressure mask ventilation, large pituitary tumors, and intraoperative lumbar drainage catheters. Surgical drainage of the pneumocranium and repair of any coexistent cerebrospinal fluid leak markedly improved neurologic status. Symptomatic pneumocranium occurring early in the postoperative course after transsphenoidal pituitary surgery is rare, but prompt recognition and treatment of this condition can be life-saving.

Benign paragangliomas: clinical presentation and treatment outcomes in 236 patients.

Paragangliomas are rare tumors that arise from extraadrenal chromaffin cells. We examined the clinical characteristics, location, treatment, and outcome of 236 patients (141 females, 60%) with 297 benign paragangliomas evaluated at the Mayo Clinic during 1978-1998. The mean age (+/-SD) at diagnosis was 47 +/- 16 yr. Of the 297 paragangliomas, 205 were in the head and neck region, and 92 were below the neck. Paragangliomas were discovered and diagnosed incidentally on imaging studies in 9% of patients. Biochemical screening was performed in 128 patients; 40 patients (17% of the total and 31% of those screened) had hyperfunctional tumors. Of the 40 patients with tumoral catecholamine excess, 38 had documented hypertension. In patients identified with catecholamine-secreting paragangliomas, the sensitivities achieved by measurements in the 24-h urine collection were 74% for total metanephrines, 84% for norepinephrine, 18% for dopamine, and 14% for epinephrine. Multiple imaging modalities were used for tumor localization. The false negative rates were 0% for magnetic resonance imaging, 5.8% for computed tomography, 3.4% for angiography, 10.7% for ultrasonography, and 39% for radioactive iodine-labeled metaiodobenzylguanidine scintigraphy. Of 192 patients (81.4%) with follow-up data (mean, 43.9 months; range, 0.5-240), operative cure was achieved in 133 (69%). Of the 59 patients without cure, 23 had persistent disease, 5 had recurrent disease, 16 had multiple persistent synchronous tumors, and 15 subsequently developed metachronous tumors. In conclusion, most paragangliomas are nonhypersecretory and located in the head and neck region. Magnetic resonance imaging was associated with the lowest false negative rate, and metaiodobenzylguanidine was the least sensitive imaging study. A significant proportion of patients (31%) has persistent or recurrent disease, and long-term follow-up is important.

Congenital gigantism due to growth hormone-releasing hormone excess and pituitary hyperplasia with adenomatous transformation.

The cause of gigantism in most patients is a GH-secreting pituitary tumor. In this report, a case of congenital gigantism due to probable central hypersection of GH-releasing hormone (GHRH) is described. Normal at birth (4.4 kg; 53 cm), our 7-yr-old male patient grew progressively thereafter to attain a height of 182 cm and a weight of 99.4 kg at the time of our evaluation. The markedly increased baseline plasma levels of GH (730 micrograms/L) did not suppress during a standard 3-h oral glucose tolerance test, but did increase 54% after iv infusion of GHRH. Baseline plasma levels of insulin-like growth factor-I, PRL, and immunoreactive GHRH were also markedly increased. Computed imaging of the head showed a large, partially cystic sellar and suprasellar mass. Extensive imaging studies did not localize a potential source of GHRH. Preoperative treatment with octreotide and bromocriptine for 4 months resulted in a 25% reduction of suprasellar tissue mass. The pituitary tissue removed at transsphenoidal and transfrontal operations showed massive somatotroph, lactotroph, and mammosomatotroph hyperplasia. Areas of GH- and PRL-secreting cell adenomatous transformation were also evident. No histological or immunohistochemical evidence of a pituitary source of GHRH was found. The peripheral plasma immunoreactive GHRH concentration remained unaffected by pharmacological and surgical interventions. We suspect that a congenital hypothalamic regulatory defect may be responsible for the GHRH excess in this case.

Effect of BAEP monitoring on hearing preservation during acoustic neuroma resection.

We measured the effect of brainstem auditory evoked potential (BAEP) monitoring on hearing preservation in acoustic neuroma resection in 90 consecutive patients with monitoring compared with 90 historical controls matched for tumor size and preoperative hearing status. In small tumors (less than 2 cm), BAEP monitoring was associated with a higher rate of hearing preservation and a greater chance that the hearing preserved was clinically useful. Changes in the BAEP intraoperatively showed a good correlation with postoperative hearing status.

Esthesioneuroblastoma: the role of adjuvant radiation therapy.

PURPOSE: We describe our experience with adjuvant radiation therapy in patients who underwent operation for esthesioneuroblastoma. METHODS AND MATERIALS: Between January 1951 and December 1990, 49 patients with esthesioneuroblastoma received their initial treatment at the Mayo Clinic. There were 27 male and 22 female patients; their ages ranged from 3 to 79 years (median, 54 years). The tumors were Kadish Stage A in 4 patients, Stage B in 13, Stage C in 29, and modified Kadish Stage D in three (cervical nodal or distant metastasis). The tumors were graded according to Hyams' classification. Treatment included gross total resection alone in 22 patients and gross total resection and postoperative adjuvant radiation therapy in 16. The patients treated with adjuvant radiation had a greater proportion of advanced-stage and high-grade tumors. RESULTS: The 5-year actuarial overall survival, disease-free survival, and local control rates were 69.1% + 7.0%, 54.8% + 7.6%, and 65.3% + 7.4%, respectively. The only significant predictor for overall survival, disease-free survival, and local control was Hyams' grade. Local control was improved in patients who received postoperative adjuvant radiation even though this group of patients had more advanced and higher-grade tumors (5-year rate of local control was 85.9% + 9.3%, compared with 72.7% + 9.5% for those who had operation alone, p = 0.26). CONCLUSION: Adjuvant radiation therapy for esthesioneuroblastoma improves local tumor control, particularly for high-grade and high-stage tumors. We recommend additional treatment with radiation (55.5 Gy) after complete resection of esthesioneuroblastoma.

Summated auditory evoked potentials in the cerebellum and inferior colliculus in the young rat.

The summated auditory evoked responses recorded at the superior surface and depth of the cerebellum of the young rat had a similar latency and configuration as did summated auditory evoked potentials in the inferior colliculus. Electric stimulation of the inferior colliculus did not evoke a response (other than the shock-artifact response) at the cerebellum. Stimulation of the cerebellum did not modify the evoked responses to click in the inferior colliculus (unless current levels were large enough to cause current spread to the brain stem). There is insufficient evidence to conclude that the cerebellum has an auditory receiving area in the young rat.

Measurements of autonomic function before, during, and after transcutaneous stimulation in patients with chronic pain and in control subjects.

Transcutaneous stimulation (TCS) has been reported to modify peripheral blood flow, skin temperature, blood pressure, and heart rate, all of which are under the influence of the autonomic nervous system (ANS). In 20 patients with intractable pain and in 10 control subjects, TCS was not found to alter significantly any of the observed properties of ANS function of control subjects or patients, except to decrease skin impedance in the patient group after the stimulation. Patients reporting pain relief during TCS were found to have significantly higher systolic blood pressure under all test conditions than patients reporting no pain relief (p less than 0.05). No other significant differences were found between these two groups. Finally, in the patients, no significant localized autonomic changes were demonstrated in the painful area by comparison with the homologous body part.

Improved preservation of facial nerve function with use of electrical monitoring during removal of acoustic neuromas.

Continuous spontaneous electromyographic activity and responses to electrical stimulation of the facial nerve in the surgical field were monitored in 48 patients undergoing primary removal of an acoustic neuroma. The operative and postoperative results in these patients were compared with the results in 48 patients who were matched for age and size of tumor and who underwent the same surgical procedure without intraoperative monitoring. Eighty-three percent of the patients had preoperative evidence of facial neuropathy, which was more severe with larger tumors. Postoperative facial nerve function was most accurately predicted on the basis of the extent of facial neuropathy on preoperative electrophysiologic testing. Anatomic preservation of the facial nerve in patients with large tumors was substantially improved in the monitored patients (67%) in comparison with those without monitoring (33%). No difference was noted in facial nerve function in the two groups of patients immediately postoperatively. By 3 months, the degree of improvement in the monitored group exceeded that in those who were not monitored, particularly in patients with medium-sized and large tumors.

Sarcoid masquerading as optic nerve sheath meningioma.

Sarcoid manifesting as an optic nerve tumor without evidence of systemic disease is uncommon. Throughout a 2-year period, a 22-year-old white woman had progressive monocular loss of vision to the level of no light perception. Optic atrophy but no uveitis was noted in the affected eye. Magnetic resonance imaging revealed thickening and enhancement of the apical optic nerve, with "tram-tracking." The presumptive diagnosis was optic nerve sheath meningioma; however, a biopsy specimen from the optic nerve revealed sarcoid. Extensive postoperative investigations revealed no systemic sarcoidosis. To our knowledge, 17 cases similar to ours, with the diagnosis proved by optic nerve biopsy, have been previously reported in the English-language literature. Most of these were mistaken preoperatively for optic nerve sheath meningioma. None of the patients had evidence of systemic sarcoidosis on initial postoperative testing. Neuroimaging, serum level of angiotensin-converting enzyme, and clinical characteristics such as age, race, sex, and optochoroidal collaterals do not distinguish optic nerve sheath meningioma from sarcoid of the optic nerve. In the absence of uveitis or systemic involvement, optic nerve sarcoid manifesting as an orbital tumor is virtually impossible to diagnose without results of biopsy.

Cervical fractures and spinal cord injury: outcome of surgical and nonsurgical management.

Data were collected retrospectively for 102 consecutive patients with a cervical spinal cord injury admitted to a spinal cord injury center between 1976 and 1986. Frankel's classification and level of spinal cord injury stayed the same or improved in all patients. The complications that occurred compared favorably with outcomes reported in the literature. Approximately 60% of patients achieved a catheter-free voiding status before dismissal from primary rehabilitation. Patients treated with early surgical stabilization of the cervical column were hospitalized a mean of 21 fewer days than their nonsurgical counterparts. In addition, patients treated with early surgical stabilization achieved their first therapeutic leave of absence from primary rehabilitation approximately 40 days sooner than patients stabilized nonsurgically. At final follow-up, however, no appreciable differences in achievement in activities of daily living and mobility were noted between patients treated with surgical stabilization of the cervical spinal column and those treated nonsurgically.

Transfrontal orbital decompression after failure of transantral decompression in optic neuropathy of Graves' disease.

Transantral and transfrontal orbital decompression procedures are effective for treating optic neuropathy of Graves' disease. We studied 10 patients with Graves' disease to clarify whether transfrontal decompression is effective after prior failure of transantral orbital decompression. All patients had persistent or recurrent optic neuropathy after transantral decompression and had failed to respond to systemic corticosteroid therapy. After transfrontal decompression, visual acuity improved in 70% of the eyes, and visual field scotomas decreased in 80%. No major intraoperative or postoperative complications occurred. We conclude that in optic neuropathy of Graves' disease, transfrontal orbital decompression after failure of transantral decompression is an acceptable and beneficial salvage procedure.

Transsphenoidal microsurgical treatment of prolactin-producing pituitary adenomas. Results in 100 patients.

One hundred patients, 84 females and 16 males, with presumed prolactin-producing pituitary adenomas were treated with transsphenoidal microsurgery. Criteria for success of the operation were return of serum prolactin values to normal and reversal of the endocrine effects of hyperprolactinemia, eradication of the adenoma, and preservation of anterior pituitary function. Of the 84 females, 54 had a microadenoma (a noninvasive adenoma 10 mm or less in diameter), and of the 32 with preoperative values of serum prolactin of less than 100 ng/ml, 28 (88%) had a successful operative result, whereas 11 of 22 (50%) with preoperative values of 100 ng/ml or more had a successful result. Five of 10 patients (50%) with a diffuse adenoma (a noninvasive adenoma more than 10 mm in diameter) and 5 of 20 (25%) with an invasive adenoma had a successful result. Of the 16 males, 3 of 7 (43%) with diffuse adenoma and 3 of 16 (19%) with invasive adenoma had a successful result. There were no males with microadenoma. The largest success rate (88%) was among female patients with microadenoma and preoperative serum prolactin values of less than 100 ng/ml.

Pituitary adenomas associated with hyperprolactinemia: a clinical and immunohistochemical study of 97 patients operated on transsphenoidally.

Immunohistochemical studies were performed on the tumors of 97 of 100 patients who had undergone an operation for a presumed prolactin-secreting pituitary adenoma; no tissue was available for study in the other 3 patients. Appropriate immunohistochemical studies were done to identify the presence of growth hormone, prolactin, adrenocorticotropic hormone, luteinizing hormone, follicle-stimulating hormone, and thyroid-stimulating hormone within the adenoma cells. The presence of a prolactin-producing pituitary adenoma was confirmed in 91 patients, 3 of whom had an adenoma that consisted of cells that contained prolactin and growth hormone. One other patient, not counted among the 91 with prolactinoma, had lactotrope and thyrotrope hyperplasia. Among the five patients whose adenoma did not contain prolactin cells, four had a null cell adenoma and one had a tumor consisting of follicle-stimulating hormone and luteinizing hormone immunoreactive cells. On the basis of preoperative serum prolactin values in these patients, we concluded that moderately increased values (30 to 200 ng/ml) of serum prolactin are not a reliable guide for determining whether a prolactin-producing pituitary adenoma is present, whereas levels exceeding 200 ng/ml are usually associated with a prolactin-secreting tumor.

Intracranial fibromatosis.

Fibromatoses are uncommon infiltrative lesions affecting musculoaponeurotic structures, most often of the limbs and trunk. Lesions involving the cranial cavity are rare and require the same aggressive surgical management as elsewhere in the body. This case illustrates their clinical and neuroradiological features and underscores the necessity for aggressive resection to avoid recurrence. The literature is reviewed.

Impact of cranioplasty on headache after acoustic neuroma removal.

We reported previously the incidence of headache after the retrosigmoid removal of an acoustic neuroma as 23% at 3 months, declining to 9% at 2 years after surgery. In an attempt to reduce the incidence and the severity of these headaches, we made one change in our surgical procedure, which was to perform a cranioplasty with methyl methacrylate. Twenty-four patients underwent the cranioplasty and were followed for at least 3 months postoperatively. These patients were matched to 24 patients who did not undergo a cranioplasty. We found a 4% incidence of headache in the cranioplasty group and a 17% incidence in the matched group. No complications were related to this change in our procedure.

Resolution of traumatic hypertrophic periodontoid cicatrix after posterior cervical fusion: case report.

The case of a 38-year-old man with delayed myelopathy 19 years after a nontreated odontoid type II fracture is reported. Magnetic resonance imaging of the craniocervical region revealed a periodontoid cicatrix. The clinical syndrome improved, and complete resolution of the retro-odontoid mass was achieved 9 months after posterior cervical fixation. The implications of this unique case for the management of myelopathy associated with nonunion of odontoid fractures are discussed.

Hemangioblastoma of the optic nerve: case report.

An optic nerve hemangioblastoma arising in the optic nerve of a patient with von Hippel-Lindau syndrome is reported. This represents the 10th published example of a hemangioblastoma arising at this site, the second description of the magnetic resonance imaging features of a hemangioblastoma at this location, and the second patient from whom an optic nerve hemangioblastoma has been removed with the goal of preserving the optic nerve as well as vision. A majority of these optic nerve hemangioblastomas have arisen in patients with von Hippel-Lindau syndrome. All patients experienced progressive loss of vision either to blindness or surgical intervention, although a vast majority of the lesions were sharply demarcated from the adjacent nerve and, thus, potentially resectable. Optic nerve hemangioblastomas are a rare cause of blindness but potentially preventable when treated with a conservative surgical approach aided by neuroimaging guidance. The recognition of discordance between the degree of vision loss and the extent or progression of a retinal hemangioblastoma may be an important clue to diagnosis in the patient with von Hippel-Lindau syndrome.

Coexisting corticotroph and lactotroph adenomas: case report with reference to the relationship of corticotropin and prolactin excess.

A 57-year-old obese woman with hypertension, diabetes mellitus, osteoporosis, and a 40-year history of secondary amenorrhea was diagnosed with corticotropin-dependent Cushing's syndrome. Dynamic endocrine testing and radiological evaluation did not reveal definitively the source of the excess corticotropin. Bilateral adrenalectomy was performed with resolution of the signs and symptoms of hypercortisolism. Four years later, the patient was noted to have rising serum corticotropin levels and an enlarging pituitary mass; hyperprolactinemia also was documented. A diagnosis of Nelson-Salassa syndrome was made, and she underwent a transsphenoidal adenomectomy. A histological examination of the specimen revealed two distinct, albeit contiguous, adenomas: a corticotroph adenoma and a lactotroph adenoma. Postoperatively, the serum prolactin and corticotropin levels decreased significantly. Although the stalk section effect resulting from compression by a pituitary adenoma can raise serum prolactin levels, a concurrent lactotroph adenoma should be considered in patients with nonfunctional or functional pituitary adenomas of other types associated with significantly elevated prolactin levels. The mechanisms underlying simultaneous adrenocorticotropic hormone and prolactin excess are discussed.

Haemophilus paraphrophilus frontal lobe abscess: case report.

We report a case of cerebral abscess due to Haemophilus paraphrophilus in a 51-year-old man who was otherwise healthy and had no cardiac abnormality. A source for the infection was not definitively identified; however, the patient had had dental manipulations 2 weeks before the onset of symptoms, and an oral source is probable. The patient was treated successfully with antibiotics after removal of the abscess. Bacteriological studies are discussed.

Symptomatic intradural adrenal adenoma of the spinal nerve root: report of two cases.

The second and third cases of an adrenal adenoma involving the intraspinal space, a rare location for a lesion that occurs rather commonly at a variety of extraneural sites, are reported. The first patient, a 16-year-old girl with a 3-month history of radicular pain, was found to have an intradural, extramedullary mass arising on a spinal nerve root. Its resection resulted in cure. The second patient, a 63-year-old woman with a 10-month history of low back pain with radiation to both lower extremities, had a similar mass arising from one of the nerve roots of the cauda equina. In both cases, ultrastructural examination of these demarcated, oncocytic, lipid-rich lesions demonstrated abundant smooth endoplasmic reticulum and mitochondria with tubular cristae. The presence of steroidogenic enzymes was noted on immunohistochemical examination. The literature is reviewed, as is the differential diagnosis of these rare lesions.