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.

Post-traumatic cerebrospinal fluid leakage.

Posttraumatic cerebrospinal fluid (CSF) leakage frequently complicates skull base fractures. While most CSF leaks will cease without treatment, patients with persistent CSF leaks may be at increased risk for meningitis, and many will require surgical intervention. We reviewed the medical records of 51 patients treated between 1984 and 1998, with CSF leaks that persisted for 24 hours or longer after head trauma. Twenty-eight patients (53%) had spontaneous resolution of the leakage at an average of 5 days. Twenty-three patients (47%) required surgery. Eight patients (16%) had occult leaks presenting with recurrent meningitis at an average of 6.5 years posttrauma. Forty-three (84%) patients with CSF leaks had an associated skull fracture, most commonly involving the frontal sinus, while only 18 patients (35%) had parenchymal brain injury or extra-axial hematoma. Eight patients (16%) had delayed leaks at an average of 13 days posttrauma. Among patients with clinically evident CSF leakage the frequency of meningitis was 10% with antibiotic prophylaxis, and 21% without antibiotic prophylaxis. Thus, prophylactic antibiotic administration halved risk of meningitis. A variety of surgical approaches was used, with minimal morbidity. Three of 23 surgically treated patients (13%) required additional surgery for continued leakage. Patients with CSF leaks that persist greater than 24 hours are at risk for meningitis, and many will require surgical intervention. Prophylactic antibiotics may be effective and should be considered in this group of patients. Patients with skull fractures involving the skull base or frontal sinus should be followed for delayed leakage. Surgical outcome is excellent.

Symptomatic C1-2 fusion failure due to a fracture of the lateral C-1 posterior arch in a patient with rheumatoid arthritis. Case report and review of the literature.

The authors report a case in which the lateral C-1 arch fractured in a patient with rheumatoid arthritis and an intact fusion mass; this patient had previously undergone a C1-2 Brooks-type fusion. This unique complication occurred secondary to continued resorption of the C1-2 rheumatoid pannus. Two years after occipitocervical fusion the patient has made a complete neurological recovery.

Persistent posttraumatic cerebrospinal fluid leakage.

OBJECT: Persistent posttraumatic cerebrospinal fluid (CSF) leakage frequently complicates skull base fractures. Although many CSF leaks will cease without treatment, patients with CSF leaks that persist greater than 24 hours may be at increased risk for meningitis, and many will require surgical intervention. The authors reviewed their 15-year experience with posttraumatic CSF leaks that persisted longer than 24 hours. METHODS: The authors reviewed the medical records of 51 patients treated between 1984 and 1998 with CSF leaks that persisted for 24 hours or longer after traumatic head injury. In 27 patients (55%) spontaneous resolution of CSF leakage occurred at an average of 5 days posttrauma. In 23 patients (45%) surgery was required to resolve the leakage. Eight patients (16%) with occult CSF leaks presented with recurrent meningitis at an average of 6.5 years posttrauma. Forty-three (84%) patients with CSF leaks sustained a skull fracture, most commonly involving the frontal sinus, whereas parenchymal brain injury or extraaxial hematoma was demonstrated in only 18 patients (35%). Delayed CSF leaks, with an average onset of 13 days posttrauma, were observed in eight patients (16%). Among patients with clinically evident CSF leakage, the frequency of meningitis was 10% with antibiotic prophylaxis, and 21% without antibiotic prophylaxis. Thus, prophylactic antibiotic administration halved the risk of meningitis. A variety of surgical approaches was used, and no significant neurological morbidity occurred. Three (13%) of 23 surgically treated patients required additional surgery to treat continued CSF leakage. CONCLUSIONS: A significant proportion of patients with CSF leaks that persist greater than 24 hours will require surgical intervention. Prophylactic antibiotic therapy may be effective in this group of patients. Patients with skull base or frontal sinus fractures should be followed to detect the occurrence of delayed leakage. Surgery-related outcome is excellent.

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.

Headache after resection of acoustic neuroma.

Long-lasting severe headaches are reported to occur in up to 83% of patients who have undergone resection of acoustic neuroma, especially through a suboccipital approach. These headaches, however, are not well defined. The objective of this study was to assess the frequency and character of new-onset headaches after resection of acoustic neuroma by a suboccipital approach with cranioplasty. Review of the medical record was followed by a telephone interview with 48 patients (67% female; mean age, 52 years) who had undergone resection of an acoustic neuroma through a suboccipital craniotomy during the 2 years before the study. Of the 48 patients, 58% had post-operative head pain that lasted more than 7 days and could be categorized into two types. A moderate to severe, short-term head pain with gradual resolution occurred in 35% of the patients, and a mild, unremitting pain was reported by 23%. Both types of pain had a dull ache or pressure quality and were adjacent to or confined to the incisional area. Overall, 77% of the patients were pain-free within 4 months after operation. Age, sex, tumor size, or preoperative history of headache did not influence development of the postoperative pain. We found that new-onset headache after resection of acoustic neuroma by a suboccipital approach with cranioplasty is much less common than previously reported and is best described as mild incisional pain rather than a severe headache. The literature regarding headaches after different surgical approaches for acoustic neuroma resection is reviewed, and possible explanations for development of the pain are discussed.

Venous air embolism in sitting and supine patients undergoing vestibular schwannoma resection.

OBJECTIVE: This study retrospectively compares the incidence of venous air embolism (VAE) detection and morbidity in the sitting and supine positions. All patients underwent vestibular schwannoma resection via the retrosigmoid approach by a single surgical team. METHODS: A total of 432 consecutive operations were reviewed, 222 of which were performed with the patients in the sitting position and 210 of which were performed with the patients in the supine position. Charts were reviewed for evidence of intraoperative VAE, intraoperative hypotension secondary to VAE, postoperative morbidity related to VAE, and other variables to compare the groups. RESULTS: This study demonstrated a 28% incidence of VAE detection when patients were in the sitting position compared to a 5% incidence of VAE detection when patients were in the supine position (P < 0.0001). Intraoperative hypotension secondary to VAE was noted in 1.8% of the sitting patients and 1.4% of the supine patients (P=0.72, no significant difference). Postoperative morbidity caused by VAE was noted in one sitting patient (0.5%) (pulmonary edema) and in no supine patients (P=0.48, no significant difference). Blood loss was slightly greater in the supine group, and operative times were similar in both groups, despite that the average tumor size of patients operated on in the sitting position was 2.8 cm versus 2.2 cm in the supine group (P < 0.0001). CONCLUSION: Our results indicate that although there is a higher incidence of VAE detection in sitting patients, the morbidity is not statistically greater. We conclude that because morbidity from VAE is similar in either position, patient positioning should be based on surgical team preference.

Long-term outcome of lumbar discectomy in children and adolescents sixteen years of age or younger.

We retrospectively reviewed the cases of seventy-two consecutive patients who had a lumbar discectomy, between 1950 and 1983, when they were sixteen years of age or younger. There were forty boys and thirty-two girls. At the time of the lumbar discectomy, twelve patients (17 per cent) also had a spinal arthrodesis. The mean duration of follow-up was 27.8 years (range, twelve to forty-five years). Twenty patients (28 per cent) had one reoperation or more, with the first reoperation performed at a mean of 9.7 years after the initial discectomy. Fourteen patients had one reoperation, four had two reoperations, one had three, and one had five. Fifty-two patients (72 per cent) did not need a reoperation. At the time of the latest follow-up, forty-eight (92 per cent) of the fifty-two patients either had no pain or had occasional pain related to strenuous activity and fifty-one (98 per cent) could participate in daily activities with no or mild limitations. Survivorship analysis showed that the overall probability that a patient would not need a reoperation was 80 per cent at ten years and 74 per cent at twenty years after the initial operation. With the numbers available for study, we could not show that age, gender, or an arthrodesis performed at the time of the initial operation were risk factors for a reoperation. We could not detect a difference, with respect to pain or the level of activity, between the patients who had had an arthrodesis at the initial operation and those who had not or between those who had a coexisting structural abnormality of the lumbar spine and those who did not.

Surgical treatment of spontaneous spinal cerebrospinal fluid leaks.

OBJECT: Spontaneous spinal cerebrospinal fluid (CSF) leaks are an increasingly recognized cause of intracranial hypotension and may require neurosurgical intervention. In the present report the authors review their experience with the surgical management of spontaneous spinal CSF leaks. METHODS: Between 1992 and 1997, 10 patients with spontaneous spinal CSF leaks and intracranial hypotension were treated surgically. The mean age of the seven women and three men was 42.3 years (range 22-61 years). Preoperative imaging showed a single meningeal diverticulum in two patients, a complex of diverticula in one patient, and a focal CSF leak alone in seven patients. Surgical exploration in these seven patients demonstrated meningeal diverticula in one patient; no clear source of CSF leakage could be identified in the remaining six patients. Treatment consisted of ligation of the diverticula or packing of the epidural space with muscle or Gelfoam. Multiple simultaneous spinal CSF leaks were identified in three patients. CONCLUSIONS: All patients experienced complete relief of their headaches postoperatively. There has been no recurrence of symptoms in any of the patients during a mean follow-up period of 19 months (range 3-58 months; 16 person-years of cumulative follow up). Complications consisted of transient intracranial hypertension in one patient and leg numbness in another patient. Although the disease is often self-limiting, surgical treatment has an important role in the management of spontaneous spinal CSF leaks. Surgery is effective in eliminating the headaches and the morbidity is generally low. Surgical exploration for a focal CSF leak, as demonstrated on radiographic studies, usually does not reveal a clear source of the leak. Some patients may have multiple simultaneous CSF leaks.

Cerebellar astrocytoma: experience with 54 cases surgically treated at the Mayo Clinic, Rochester, Minnesota, from 1978 to 1990.

A comprehensive review of the literature has shown that the treatment of choice for cerebellar astrocytomas has primarily been gross-total resection of the mass and gross-total resection of the enhancing portion of pilocytic astrocytomas. Most large scale studies of postresection survival rates of patients with cerebellar astrocytomas were conducted when computerized tomography (CT) and magnetic resonance (MR) imaging were not readily available. It has been shown that postoperative CT scans or MR images are more reliable than the surgeon's estimate of the degree of tumor resection at the time of surgery. It is not possible, therefore, to make an accurate determination regarding a postresection prognosis based on the degree of suspected tumor resection without the availability of appropriate radiographic imaging. In this study, the authors retrospectively evaluated the treatment of 54 patients with cerebellar astrocytoma who underwent surgery at the Mayo Clinic in Rochester, Minnesota, from 1978 through 1990. Preoperative and postoperative CT scans or MR images were available in all 54 patients.

Spinal column deformity and instability after lumbar or thoracolumbar laminectomy for intraspinal tumors in children and young adults.

STUDY DESIGN: A retrospective study about the occurrence of spinal column deformity or instability after multilevel lumbar or thoracolumbar total laminectomy for removal of benign intraspinal tumors in children and young adults. OBJECTIVES: To analyze the long-term clinical and radiographic outcome of these patients, and to specify factors that affect the occurrence of postlaminectomy spinal column deformity and instability. SUMMARY OF BACKGROUND DATA: Spinal column deformity is not uncommon after multilevel cervical or thoracic laminectomies for removal of intraspinal tumors in children. Its incidence in the lumbar and thoracolumbar spine reportedly is low. METHODS: Thirty-six consecutive patients (23 male, 13 female) underwent multilevel lumbar or thoracolumbar total laminectomy for removal of benign intraspinal tumors from 1966 to 1989. Twelve patients were aged 17 years or younger ("children and adolescents"; mean age, 11 years), and 24 were aged 18-30 years ("young adults"; mean age, 24 years). All patients had preoperative, immediate postoperative, and follow-up clinical and radiographic examinations. RESULTS: At a mean follow-up period of 14 years (range, 4-28 years), six patients (16.6%) had spinal deformity (lordosis or thoracolumbar kyphosis associated with scoliosis), and four (11%) had spondylolisthesis. Spinal column deformity occurred in 33% of children and adolescents and in 8% of young adults. Spondylolisthesis occurred in 16.6% of children and adolescents and in 8% of young adults. Three patients had fusion for spinal column deformity. Pain was present in eight patients, and other neurologic signs and symptoms were found in 18. There was an increased incidence of postoperative spinal deformity in patients who had more than two laminae removed (P < 0.01) or a facetectomy performed at the time of the initial operation (P < 0.05). There was no association between the occurrence of the deformity and sex, neurologic condition after laminectomy, or length of follow-up period. CONCLUSIONS: Spinal deformity or instability after multilevel lumbar or thoracolumbar total laminectomy is not uncommon in children and adolescents. Limiting laminae removal and facet destruction may decrease this incidence. Fusion may be required to correct post-laminectomy deformity and to stabilize the spine.

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.

Conservative management of acoustic neuroma: an outcome study.

OBJECTIVE: This study analyzed selection criteria, clinical outcome, and tumor growth rates in patients with acoustic neuromas in whom the initial management strategy was observation. METHODS: A retrospective review of patients with conservatively managed unilateral acoustic neuromas was conducted. Minimum follow-up was 6 months. Patients with neurofibromatosis Type II were excluded. Differences in tumor growth rates were analyzed by use of the Wilcoxon rank sum test. RESULTS: Sixty-eight patients (31 men and 37 women) with a mean age of 67.1 years were followed for an average of 3.4 years after diagnosis. The reasons for a trial of observation included advanced age (55%), patient preference (21%), minimal symptoms (9%), poor general medical condition (7%), asymptomatic tumor (4%), and tumor in the only hearing ear (4%). Fifty-eight patients (85%) were successfully managed with observation alone. Ten patients (15%) ultimately required treatment (nine received microsurgical treatment and one patient underwent radiosurgical intervention) at a mean time interval of 4.0 years after diagnosis. Forty-eight tumors (71%) showed no growth and 20 (29%) enlarged during the study period. The mean tumor growth rate at the 1-year follow-up was significantly higher in the group requiring treatment (3.0 mm) than in the group not requiring treatment (0.36 mm) (P < 0.0001). Thus, the tumor growth rate at the 1-year follow-up was a strong predictor of the eventual need for treatment. CONCLUSION: Observation is a reasonable management strategy in carefully selected patients with acoustic neuromas. Diligent follow-up with serial magnetic resonance imaging is recommended, because some tumors will enlarge to the point at which active treatment is required.

Results of treatment of pituitary disease in multiple endocrine neoplasia, type I.

OBJECTIVE: The aim of the present study was to examine the clinical and pathological features of pituitary disease in patients with multiple endocrine neoplasia, Type I (MEN I) and to assess the prognosis. METHODS: Fifty-two patients with pituitary disease and MEN I were studied retrospectively. Medical records were reviewed, and all of the patients known to be alive were sent a questionnaire to ascertain current disease status. RESULTS: In 12 patients, pituitary disease was the initial manifestation of MEN I. The most common lesion was prolactinoma, followed, in frequency, by acromegaly and nonsecretory adenoma. Thirty-four of the patients had surgical treatment at the Mayo Clinic, Rochester, MN, as primary treatment, 3 had radiotherapy, and 12 received no specific therapy. Twelve patients had adjunctive radiotherapy postoperatively. Of the 34 patients receiving surgical treatment, 33 had adenoma and 1 had adenoma and pituitary hyperplasia. Immunocytochemical examination demonstrated that many tumors showed reactivity for more than one pituitary hormone. On survival analysis, no excess pituitary-related mortality was found, either in the surgically treated group or in the group as a whole. CONCLUSION: On the basis of this study, we conclude that pituitary disease is frequently the initial manifestation of MEN I; that adenomas, particularly prolactinomas, are the rule and hyperplasia is rare; that a significant proportion of tumors are plurihormonal; and that excess pituitary-related mortality is not a factor in patients with MEN I.

Far-field auditory brainstem response in neurotologic surgery.

This is a review of our experience using far-field auditory brainstem monitoring during acoustic neuroma removal. The observations are based on 144 consecutive cases beginning in 1986. The factors of importance are tumor size, preoperative auditory function, and the preoperative presence of a wave V on the auditory brainstem response. Our experience suggests that preservation of hearing in tumors > 2.5 cm is rare. It was observed that preserving wave V does not guarantee preservation of hearing. Conversely, loss of wave V does not preclude preservation of hearing. It has also been noted that the presence of only wave I preoperatively does offer some hope that hearing can be preserved postoperatively. Finally, postoperative hearing function is usually equal to or worse than the preoperative function. Only rarely does the postoperative function improve.

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.

Acoustic schwannoma and pregnancy: a DNA flow cytometric, steroid hormone receptor, and proliferation marker study.

For a long time, it has been speculated that pregnancy stimulates the growth of acoustic schwannomas. To test this hypothesis, immunohistochemical stains for estrogen receptor, progesterone receptor, and proliferating cell nuclear antigen (PCNA) were performed. Flow cytometric studies for DNA ploidy and S-phase fraction determinations were also performed. The study subjects included 6 female patients with unilateral acoustic tumors; at the time of tumor removal, 1 woman was pregnant and the other 5 women were 2 to 10 months postpartum. The age-sex-matched control group consisted of 6 men and 12 nonpregnant women, all with acoustic schwannomas similar in size to those of the study group. The study found no statistically significant association between the presence or quantity of estrogen or progesterone receptors and pregnancy, DNA ploidy, proliferation indices, or clinical data. Based on PCNA indices, large tumors tended to be less "biologically active" than small lesions (P < .01). The authors concluded that pregnancy does not significantly stimulate the cellular growth of acoustic schwannomas.