Quality of hearing preservation in acoustic neuroma surgery

OBJECTIVE: This study aimed to investigate the factors affecting the quality of postoperative hearing in acoustic neuroma. STUDY DESIGN: The study was designed as a retrospective case review. SETTING: The study was performed at the Department of Otolaryngology, School of Medicine, Keio University, Tokyo, Japan. PATIENTS: The subjects were 94 patients with unilateral acoustic neuroma. INTERVENTION: Hearing preservation surgery was performed in the subjects via the extended cranial fossa approach or the middle cranial fossa approach. MAIN OUTCOME MEASURES: The outcome measures included patient's age and gender, hearing level, speech discrimination score, tumor size, and surgical approach. The relationship between the qualities of preoperative and postoperative hearing and the long-term prognosis of preserved hearing also was investigated. RESULTS: In 94 subjects, there were 47 patients whose hearing was preserved (HP group) and 47 patients whose hearing was not preserved (non-HP group). Overall, hearing preservation rate was 50%. There were no significant differences in age, gender, and tumor size between the two groups. The hearing preservation rate was significantly higher in patients with an intracanalicular tumor than that with a larger tumor. The better the preoperative quality of hearing was, the higher the postoperative one. Although the preserved hearing deteriorated after surgery in 4 patients, no significant hearing deterioration was observed in the other 43 patients. CONCLUSIONS: The results of this study indicated that the diagnosis for acoustic neuroma in the early stage with serviceable hearing is the most important to improve the quality of postoperative hearing.     

Vagal paraganglioma: a review of 46 patients treated during a 20-year period

BACKGROUND: Vagal paragangliomas (VPs) arise from paraganglia associated with the vagus nerve. Approximately 200 cases have been reported in the medical literature. Because of their rarity, most information regarding these tumors has arisen from case reports and small clinical series. OBJECTIVE: To detail the clinicopathologic features of 46 patients with VP with an emphasis on the role of a multidisciplinary skull base team in both the successful extirpation and rehabilitation. DESIGN: Retrospective review of 46 patients with VP managed by a single skull base team. SETTING: An academic tertiary medical center. RESULTS: Forty-six patients were treated over a 20-year period (1978-1998). Ten (22%) demonstrated intracranial extension. There was a history of familial paragangliomas in 9 (20%) of the patients. The incidence of multicentric paragangliomas was 78% in patients with familial paragangliomas vs 23% in patients with nonfamilial paragangliomas. Management of this group of 46 patients consisted of surgery (n = 40), radiation therapy (n = 4), and observation (n = 2). The operative approach consisted of a transcervical excision often combined with a transtemporal or lateral skull base approach as dictated by the tumor extent. Postoperative cranial nerve deficits were common, and, as such, aggressive rehabilitation was a vital component in the management of these tumors. CONCLUSIONS: The management of VP and its associated cranial nerve deficits remains a difficult clinical problem. Options for treatment include surgical resection, radiation therapy, and, in selected cases, observation. Surgical extirpation requires a multidisciplinary skull base team to achieve complete tumor resection. Radiation therapy is reserved for elderly patients and patients at risk for bilateral cranial nerve deficits. Rehabilitation of cranial nerve deficits is an integral part of the management of VP.     

Predictors of a medical-offset effect among patients receiving antidepressant therapy

OBJECTIVE: The study was conducted to describe the diagnosis and management of pneumocephalus after acoustic tumor resection. STUDY DESIGN: The study design was a retrospective chart review. SETTING: The study was conducted as a tertiary otologic referral to the senior authors' practices (PAF, MDA). PARTICIPANTS: Three patients operated on for acoustic tumor in St. Vincent's General Hospital and the Scottish Hospital, Sydney, Australia, were studied. INTERVENTION: Observation in both cases and surgery in a case in which tension pneumocephalus was progressive were performed. MAIN OUTCOME MEASURE: Recovery will follow either spontaneously or after intervention. RESULTS: All patients had an uneventful recovery. CONCLUSION: In most cases, spontaneous resolution occurs. However, if surgical re-exploration is indicated, it is best performed via the blind sac subtotal petrosectomy of Fisch.     

Expression of aquaporin-4 in fast-twitch fibers of mammalian skeletal muscle

OBJECT: Ependymomas in children continue to generate controversy regarding their histological diagnosis and grading. optimal management, and possible prognostic factors. To increase our knowledge of these tumors the authors addressed these issues in a cohort of children with prospectively staged ependymomas treated with radiotherapy and chemotherapy. METHODS: Children between the ages of 2 and 17.3 years harboring an intracranial ependymoma confirmed by a central review of the tumor's pathological characteristics were treated according to Children's Cancer Group Protocol 921 from 1986 to 1992. Treatment following surgery and postoperative tumor staging (including brain computerized tomography or magnetic resonance [MR] imaging, spinal MR imaging or myelography, and cerebrospinal fluid cytological investigation) included craniospinal irradiation with a local boost to the primary tumor and patient randomization to receive adjuvant chemotherapy with either 1) CCNU, vincristine, and prednisone, or 2) the eight-drugs-in-1-day regimen. Centralized review of the tumor pathological characteristics revealed 20 ependymomas and 12 anaplastic ependymomas in the 32 children included in the study. Diagnoses made at the individual institutions included anaplastic (malignant) ependymoma (15 patients), ependymoma (four patients), ependymoblastoma (nine patients), ependymoastrocytoma (one patient), and primitive neuroectodermal tumor (three patients), which were discordant with the centralized review diagnosis in 22 of 32 cases. Only three of the 32 patients had metastatic disease (two with M and one with M3 stages). At surgery, 47% of tumors were estimated to be totally resected. Among the 14 of 17 patients who suffered a relapse and were evaluated for site of relapse, 10 (71%) had an isolated local relapse, three (21%) had concurrent local and metastatic relapse, and only one (7%) had an isolated metastatic relapse. Kaplan-Meier estimates of 5-year progression-free survival (PFS) and overall survival rates were 50 +/- 10% and 64 +/- 9%, respectively. CONCLUSIONS: Predictors of PFS duration included an estimate of the extent of resection made at surgery (total compared with less than total, p = 0.0001) and the amount of residual tumor on postoperative imaging as verified by centralized radiological review (< or = 1.5 cm2 compared with > 1.5 cm2, p < 0.0001). No other factors, including centrally reviewed tumor histopathological type, location, metastasis and tumor (M and T) stages, patient age, race, gender, or chemotherapy treatment regimen significantly correlated with PFS duration. The pattern of predominantly local relapse and the important influence of residual tumor or the extent of resection on PFS duration confirms a prevailing impression that local disease control is the major factor in the prediction of outcome of ependymoma. Survival rates were comparable with those reported by other investigators who have treated patients with similar doses of radiation and no chemotherapy.     

Treatment of intracranial ependymomas of children: review of a 15-year experience

PURPOSE: There are still major controversies in the optimal management of children with intracranial ependymomas. To assess the impact of tumor site, histology, and treatment, the outcome of children treated at the Institut Gustave Roussy was reviewed retrospectively. METHODS AND MATERIALS: Between 1975 and 1989, 80 children aged 4 months to 15.8 years were seen at the Institut Gustave Roussy for postoperative management of an intracranial ependymoma. Location of tumor was infratentorial in 63 cases and supratentorial in 17. Surgical treatment consisted of complete resection in 38, incomplete resection in 38 and biopsy only in 4. Postoperative irradiation was done in 65 patients and chemotherapy in 33. Surviving patients have been followed from 12-197 months with a median of 54 months. RESULTS: The 5-year actuarial survival and event-free survival are 56% and 38%, respectively. Thirty-four patients relapsed from 3-72 months after diagnosis (median 25 months). In 20 patients, the only site of failure was the original tumor site. Three patients failed locally and at distance, while 10 others failed only at distance. Survival at 5 years was significantly better for patients who had complete resection of the tumor (75% vs. 41%, p = 0.001) and for those who received radiation therapy (63% vs. 23%, p = 0.003). Event-free survival at 5 years was superior in patients with complete resection of the tumor (51% vs. 26%, p = 0.002) and in patients who received radiation therapy (45% vs. 0%, p < 0.001). Sex and tumor site had no impact on survival or event-free survival. There was no difference in survival, event-free survival, or pattern of failure between patients treated with local field, whole brain or craniospinal irradiation, while severe longterm sequelae were noted predominantly in the latter two groups. CONCLUSION: Considering that failures were predominantly local and that there was no apparent benefit from prophylactic irradiation, we recommend local field irradiation with doses above 50.0 Gy for all children with intracranial ependymomas, without meningeal dissemination at diagnosis. Special considerations are necessary for children < 3 years of age.     

Traumatic neuromas of the head and neck

An interesting case of a traumatic neuroma of the greater auricular nerve provides the impetus for a discussion of head and neck neuromas. Traumatic neuromas of the head and neck are relatively rare. Division of the greater auricular nerve during parotidectomy occasionally results in a traumatic neuroma. We report a case of a 73-year-old woman who presented with a traumatic neuroma nine years after undergoing superficial parotidectomy with dissection of the facial nerve for a mixed tumor. The patient had a 1.5 cm x 1.0 cm mass located below the old surgical site over the anteromedial border of the sternocleidomastoid muscle. The patient's past history was significant for Frey's syndrome, which is the result of abnormal neurologic growth. On first impression, the tumor was thought to be a recurrence of neoplastic disease; however, because of the evaluation, traumatic neuroma was suspected. An attempt at fine-needle aspiration of the mass was too painful to be carried out. At surgery, a whitish tumor was excised which, on final pathologic examination, revealed traumatic neuroma. The surgical literature is reviewed and the subject of head and neck neuromas, including their evaluation and management, is thoroughly discussed. Knowledge of this possible diagnosis may spare the patient and the surgeon needless worry, as well as unnecessary procedures, once tumor recurrence has been ruled out.     

Reoperative surgery for organic hyperinsulinism: indications and operative strategy

Organic hyperinsulinism has a good chance of cure by operation, although patients with diffuse or multiple disease run a high risk of recurrence or persistence of disease. Surgical management and outcome in these patients are presented and discussed. Between 1986 and April 1997 a total of 62 patients were operated on for organic hyperinsulinism [solitary 48, multiple 3, multiple endocrine neoplasia type I (MEN-I) 2, diffuse 4, malignant 5]. Persistence or recurrence occurred in 10 patients (16%). Among the six that persisted, four were malignant and two benign. All four of those that recurred were benign. Patients with benign disease presented with multiple tumors (n = 3), MEN-I syndrome (n = 1), and diffuse/nodular hyperplasia (n = 2). The duration between diagnosis and reintervention ranged from 1 to 10 years. Preoperative diagnosis was able to localize tumors in three patients (computed tomography 1, angiography 2, calcium stimulation 1). Operative procedures were multiple enucleations in two patients with sporadic disease, subtotal resection plus enucleation in the case of MEN-I syndrome, subtotal resection for diffuse hyperplasia, left resection for adenomatosis, and tumor extirpation after multiple previous operations. Long-term clinical and biochemical cure was achieved in five of six patients (mean follow-up 5 years). Octreotide therapy shows good symptomatic control in the patient with operative failure. Reintervention for organic hyperinsulinism is successful (80% cure) and requires preoperative imaging and individual surgical management.     

Full functional recovery after surgical repair of transected abducens nerve: case report

OBJECTIVE AND IMPORTANCE: Results of surgical repair of the injured abducens nerve are rarely reported in the literature. A full functional recovery of a completely resected abducens root may be exceptional. We describe a patient who obtained normal ocular alignment and binocular vision after surgical reconstruction of a transected abducens nerve. CLINICAL PRESENTATION: A 56-year-old woman with a petroclival meningioma was presented. She underwent total removal of the tumor through a combined supra/infratentorial transpetrosal approach. The abducens nerve was tightly attenuated by the tumor and thickened dura. During dissection, the nerve was completely transected just behind the entrance to Dorello's canal. INTERVENTION: The abducens nerve was the single root type and inevitably required surgical repair. To obtain a sufficient length of the distal stump for trimming, part of the petrosphenoidal ligament was cut and the superior border of the petrous bone was exposed. The proximal stump of the nerve was also trimmed to obtain healthy tissue, and reconstruction was performed with five 10-0 nylon sutures. Five months later, esodeviation began to improve. Nine months after the surgery, the patient did not complain of diplopia and an objective assessment reported normal ocular alignment and estimated binocular function as "excellent" according to Biglan's system. Overcorrection of abduction did not occur. CONCLUSION: The result in our patient confirms the possibility of full functional recovery after surgical repair of a totally transected abducens nerve.     

Medication control in hospitals: a practical approach to the problem of drug-drug interactions

We present 19 patients with tuberous sclerosis complex and subependymal giant cell astrocytoma. The mean age at the time of tumor diagnosis was 9.4 years (range, 1.5 to 21 years). Computed cranial tomography (CT) or cranial magnetic resonance imaging (MRI) identified the lesion which was resected in all cases. Seven patients had hydrocephalus and there was an interval increase in the tumor size or a large tumor without hydrocephalus in 12 patients. Surgical criteria included: (1) presence of hydrocephalus; (2) interval increase in tumor size; (3) new focal neurologic deficit attributable to the tumor; and/or (4) symptoms of increased intracranial pressure. Eight patients were identified through a surveillance program involving annual computed cranial tomography. All of these eight patients had their tumor removed prior to the development of symptoms, none had neurologic deficits which persisted after surgery, and none has so far developed recurrent subependymal giant cell astrocytoma. In contrast, of the 11 patients from the non-surveillance group 7 were symptomatic at tumor diagnosis, 1 had a complicated postoperative course, 2 developed recurrent giant cell astrocytoma, and 1 had an extensive lesion that could not be completely excised. Periodic cranial imaging may help to identify subependymal giant cell astrocytomas in tuberous sclerosis patients before they become symptomatic. Earlier diagnosis and treatment could reduce surgical morbidity and the risk of tumor recurrence.     

Food bolus intestinal obstruction in a Chinese population

BACKGROUND: The aim of the present study was to review the authors' experience of the diagnosis and management of food bolus obstruction in a Chinese population. METHODS: During the period 1990-96, 31 patients were treated for food bolus obstruction. The records of these patients were retrospectively reviewed. RESULTS: Eighteen patients (58%) had a history of previous gastric surgery and 13 patients (42%) had an intact gastrointestinal tract. The most common site of impaction was the terminal ileum, and a synchronous site of food bolus obstruction was present in 16% of cases. A total of 91% of the food bolus retrieved was phytobezoar. In 15 cases, the food bolus was successfully milked into the caecum. Enterotomy for removal was necessary in 12 patients, and two underwent small-bowel resection for necrosis. CONCLUSION: Food bolus obstruction affects not only those who have had previous gastric surgery, but also a significant proportion of those with an intact gastrointestinal tract.     

Incorporation of nuclear matrix attachment regions into the herpes simplex virus type 1 genome does not induce long-term expression of a foreign gene during latency

We report two cases of skull base metastasis from renal cell carcinoma. Case 1: A 55-year-old female presented with a skull base tumor located on the clivus. Partial removal of the tumor was performed via the transsphenoidal approach. Case 2: A 44-year-old male presented with a skull base tumor occupying the orbital, nasal, and paranasal cavities. The first operation, partial removal of the tumor, was performed via the transsphenoidal approach. Thereafter, the tumor regrowth and the patient's symptoms were progressive, so a second operation was performed via Le-Forte I craniotomy to prevent cranial nerve dysfunction and air way obstruction. The initial symptom of these two cases was abducens nerve paresis, which is caused by the tumor extending from the clivus to the cavernous sinus. The neuroradiological features were the destruction of skull base bone and angiographic tumor stain. Histopathological examination of the surgically resected specimens revealed clear cell carcinoma, and thereafter they were diagnosed as skull base metastasis from renal cell carcinoma. We emphasize the necessity of a thorough medical workup to enable speedy diagnosis of renal cell carcinoma in such cases.     

Brain tumors with symptomatic onset in the first two years of life

Eighty children who in the first 2 years of life had signs and symptoms relating to a cerebral neoplasm were studied over an 18-year period (1970-1987), the mean follow-up being 8.2 years. In each case age at onset, clinical presentation, tumor location and pathological diagnosis, extent of surgical resection, postoperative mortality, adjuvant therapy length of survival and quality of life were assessed. Supratentorial tumors (59%) were more common than infratentorial. The most frequent clinical presenting feature (70%) was increased intracranial pressure. Sixty-three patients (79%) were operated on and in all of these cases a histological diagnosis was obtained. Astrocytomas (41%) and medulloblastomas (20%) were the most common oncotypes. Surgical mortality was 17.4% and the 5-year survival rate was 54%. Quality of life was assessed for all long-term surviving patients using a specifically designed protocol. Normal physical and intellectual performances were found in 46% of cases, and all together 75% of the patients had sufficient autonomy in daily life. The prognosis is more closely related to tumor location and type of treatment than to histological diagnosis or age at onset.     

Complications of craniofacial surgery for tumors involving the anterior cranial base

OBJECTIVE: To evaluate the risk factors for postoperative complications among patients undergoing craniofacial resection for the treatment of anterior cranial base tumors, a retrospective analysis of patients treated in University of Tokyo Hospital between September 1987 and November 1996 was conducted. METHODS: Twenty-nine patients underwent 33 craniofacial resections for tumors involving the anterior cranial base. Twenty-three of the 29 patients had malignant tumors and 6 patients had benign tumors. Anterior craniofacial resection was performed using a combination of intracranial and extracranial approaches. Radiotherapy and neoadjuvant chemotherapy were administrated to some patients. RESULTS: Severe intracranial infections were more common among patients who underwent partial frontal lobectomies (P < 0.03). These infections occurred only in patients who had been treated previously with a craniotomy (P < 0.02) and a total radiation dose of > or =60 Gy (P = 0.06). Neither management of the extracranial structures nor methods of reconstruction of the cranial base showed significant correlation with major postoperative complications. CONCLUSION: Compared with previous reports, craniofacial resection has become a relatively safe and effective procedure for the treatment of tumors involving the anterior cranial base. However, additional care should be taken with patients who have experienced a previous craniotomy, frontal lobe involvement, or radiotherapy with a total dose of > or =60 Gy.     

Surgical management of periocular basal cell carcinoma using frozen section control and immediate plastic reconstruction--indications and methods in 106 patients

PURPOSE: To review our first experiences with the surgical removal of basal cell carcinoma (BCC) utilizing frozen section control and immediate plastic reconstruction. PATIENTS AND METHODS: We analyzed the accuracy of clinical diagnosis, methods of surgical excision, plastic repair and recurrence rates in 162 consecutive patients who underwent surgical excision utilizing either frozen section (n = 106) or biomicroscopic control (n = 56) between January 1991 and June 1996. Specimens used for intraoperative frozen-section monitoring were also fixed, processed, and sectioned for permanent sections and served as postoperative controls for the frozen sections (n = 43). RESULTS: The accuracy of clinical diagnosis was 86% (n = 189). In 106 patients with BCC excised with frozen section control, there were no recurrences reported after a mean follow-up of 2.9 years. The mean surgical defect measured 55% of total eyelid length (range 10%-100%). The incidence of morphea pattern was 34%. Intraoperative re-excisions due to frozen sections positive for tumor were necessary in 31% of cases. Permanent postoperative sections of the tissue that had been used for intraoperative frozen sections confirmed in 97.5% of patients (n = 43) the preliminary findings made with frozen sections. In 56 patients with BCC excised with biomicroscopic control, three tumors recurred (5%) after a mean follow-up of 4.4 years. The mean surgical defect measured 42% of total eyelid length (range 16%-100%). The incidence of morphea type was 23%. CONCLUSION: Surgical removal utilizing frozen section control and immediate plastic repair appears to represent a reliable and effective option in the management of selected patients with periocular BCC.     

Breast implants

We describe the case - to our knowledge unique - of an 8-year-old boy who presented with acute onset of lower cranial nerve palsy and tetraparesis caused by a hematoma in a dorsal exophytic pilocytic astrocytoma of the medulla oblongata. The boy showed near-complete recovery after neurosurgical management in two stages: first, emergency evacuation of the hematoma with tumor biopsy, and second, complete tumor removal 5 months after the initial event. Intraoperative electrophysiological monitoring techniques for the lower cranial nerves are of value in preserving their functional integrity. Ultrasonography is helpful in assessing the extent of tumor removal. Although the pathological diagnosis of a pilocytic astrocytoma would not justify radiotherapy, local field radiotherapy was added mainly because of the unexpectedly rapid tumor progression during the interval between the two surgical procedures. The literature on brainstem and tumor hematoma in children is reviewed.     

Inhibitory effects of saiboku-to and compornent herbs on the production of peptide leukotrienes (LTs) and LTB4]

We report our early experience using stereotactic radiotherapy (radiosurgery) in the treatment of cranial neuroma, by the linear accelerator method. We report the first 13 neuromas treated in 12 patients (follow-up 6-60 months). Radiologically, seven of 10 patients demonstrated signs of central tumour necrosis on follow-up scanning and four of these also demonstrated shrinkage. Of seven assessable acoustic neuroma patients treated, hearing was stabilized in three and improved in two. An abducent neuroma patient treated by this method is reported and demonstrated good response. Our preliminary studies endorse the opinion that stereotactic radiosurgery is a suitable and safe alternative to microsurgical procedures in the management of many cranial neuromas. Indications for stereotactic radiosurgery are discussed.     

Cost-effectiveness in the management of patients with oesophageal cancer

BACKGROUND: The aim of this study was to assess the relationship between clinical outcome, quality of life and cost for treatment modalities commonly employed in the management of oesophageal carcinoma. METHODS: A series of 51 patients diagnosed with oesophageal carcinoma in a 6-month period was used to derive a cost analysis profile for their treatment. All patients underwent quality of life assessment. Patients diagnosed in 1993 and managed in Newcastle upon Tyne were identified from the Northern Cancer Registry and Hospital Episode Statistics. Intervention profiles were documented for a 3-year follow-up period and cost analysis was conducted. A further 51 patients were recruited prospectively for quality-of-life studies. RESULTS: Some 139 individuals were identified retrospectively. Median survival was significantly better in patients treated by resection (n=31; median 20 months) than in those receiving palliative treatments (n=108; median 6 months) (P < 00001). Median cost was significantly greater in individuals who underwent resection (8070 pound sterling) than for patients subjected to a palliative strategy (radiotherapy 4720 pound sterling, brachytherapy 1790 pound sterling, laser 3540 pound sterling, intubation 2450 pound sterling, no treatment 1390 pound sterling) (P < 0.01). When considering the median cost per month of life (after treatment) resection (457 pound sterling) compared favourably with the palliative options (range 342-1125 pound sterling). CONCLUSION: Surgical resection for oesophageal carcinoma confers greatest benefit in terms of survival. Costs are inherently greater in individuals undergoing resection but, allowing for time, resection is at least as cost-effective as other treatment modalities.     

How to take a weapons history: interviewing children at risk for violence at school

BACKGROUND: Malignant melanoma is the most common metastatic tumor of the gastrointestinal tract and can present with abdominal pain, small bowel obstruction, or occult gastrointestinal bleeding. Diagnosing abdominal pain due to metastatic melanoma is best accomplished by intraluminal studies and computed tomography. Surgical resection of the tumor burden limited to the gastrointestinal tract has been shown to extend periods of palliation in this poor long-term survival group. OBJECTIVES: We will review a case of abdominal pain in a melanoma patient and review the literature.     

Changes in epidemic patterns of Kawasaki disease in Japan

Cholangiocarcinoma is the second most common primary tumor of the liver after hepatocellular carcinoma and accounts for 5 to 25% of primary hepatic malignancies. Patients with intrahepatic or peripheral cholangiocarcinoma (ICC) most often present at an advanced stage leading to a poor prognosis. A review of the literature has produced only 10 patients who have survived over five years. We review the case of a young woman with a large cholangiocarcinoma, who has been disease free for eight years. The patient was treated with a right hepatic lobectomy, and received 4 cycles of 5-fluorouracil and levamisole postoperatively. Known factors associated with longer survival in patients with ICC include lack of evidence of local invasion (i.e. capsular, lymphatic, or vascular), negative margins, mucoblia, and well differentiation of the tumor, as well as the absence of lymph node metastases. Our patient had negative margins and lymph nodes, and showed no local invasion. However, no mucobilia was noted, and the tumor was only moderately differentiated. Young age has never been associated with increased survival. ICC remains a relatively uncommon tumor with an insidious onset and late presentation contributing to poor survival. Surgical resection remains the only therapeutic option. Since few patients are potentially resectable at the time of presentation, efforts at early diagnosis and options for adjuvant therapy are imperative.