Snoring associated with Ondine's curse in a patient with brainstem glioma.

UNLABELLED: Ondine's curse is an uncommon type of sleep apnea syndrome characterized by failure of automatic respiration. We present an adult patient with brainstem glioma who presented with snoring and Ondine's curse as the only symptoms. CASE REPORT: A 52-year-old female was brought to the hospital by a fellow resident due to exceptionally loud snoring. During the hospitalization, Ondine's curse was diagnosed after monitoring using Apnomonitor 5 (Chest Co., Tokyo), a cheap, non-invasive respiratory monitoring procedure. MRI and MR spectroscopy revealed a brainstem glioma. After radiation therapy, clinical response was documented using repeat apnomonitoring. CONCLUSION: Exceptionally loud snoring in non-obese adult patients with sleep apnea may be an early feature of a brainstem space-occupying lesion. Overnight sleep respiratory evaluation and neuroimaging should be considered in such instances.

Ultrasonographic screening of carotid artery in patients with vascular retinopathies.

BACKGROUND AND PURPOSE: Retinal vasculopathy is commonly found with complaint of burred vision by ophthalmologist and closely related to asymptomatic cerebrovascular disease. The purpose of this study was to evaluate patients with microvascular retinopathy by ultrasound sonography of neck carotid artery and to find high risk group for stroke at outpatient of ophthalmology with associated retinopathy. METHODS: Between 1999 and 2001, a total of 480 consecutive patients visited at outpatient of ophthalmology with a complaint of burred vision were evaluated by ultrasound sonography of neck carotid artery. The patients were divided to with retinopathy (n=253) and without retinopathy (n=227). The former was subgrouped as follows; central retinal vein occlusion (CRVO; n=11), branch of retinal vein occlusion (BRVO; n=71), retinal artery occlusion (RAO; n=8), hypertensive retinopathy group-I (according to Keith, Wagener and Barker's criteria) (HTN-R I; n=43) and group-II (HTN-R II; n=42), and diabetic retinopathy (DM-R; n=78). The latter group was with various ophthalmological diseases, mainly inflammation of cornea, with healthy retinal vessels and constituted as the control group. RESULTS: There was no statistically significant difference between patients with retinopathy and the control group with respect to sex distribution, nor among the retinopathy groups. Patients with retinopathy had a mean age of 68 years, while those with HTN-R I and II were older with a mean age of 71 years. By ultrasonography, RAO showed pathology in intima-media thickness (IMT; 1.13 +/- 0.55 vs. 0.81 +/- 0.26 mm in control), average number of plaques (1.88 +/- 0.99), degree of stenosis of internal carotid artery (42.4 +/- 28.5%) and common carotid artery (27.4 +/- 21.3%). Those with DM-R had the second thickest IMT (0.96 +/- 0.42 mm) and the second highest quantity of plaque (1.23 +/-1.23), and the second most stenotic common carotid artery (13.1 +/- 17.28%). CONCLUSION: Moderate sclerotic changes are found in ophthalmology patients with RAO and DM-R, although the degree pathology do not indicate medical nor surgical prophylactic treatment. Retinal vein occlusion, that has been discussed with diminished retinal blood circulation, does not show any differences in comparison with control group and ultra sonography has less value for screening of asymptomatic carotid atherosclerosis.

Surgical experience with skull base approaches for foramen magnum meningioma.

The surgical treatment of patients with foramen magnum meningioma remains challenging. This study evaluated the outcome of this tumor according to the evolution of surgical approaches during the last 29 years. A retrospective analysis of medical records, operative notes, and neuroimages of 492 meningioma cases from 1972 to 2001 identified seven cases of foramen magnum meningioma (1.4%). All patients showed various neurological symptoms corresponding with foramen magnum syndrome. The tumor locations were anterior in five cases and posterior in two. Surgical removal was performed through a transoral approach in one patient, the suboccipital approach in three, and the transcondylar approach in two. Total removal was achieved in all patients, except for one who refused any surgical treatment. The major complications were tetraparesis and lower cranial nerve paresis for tumors in anterior locations, and minor complications for posterior locations. One patient died of atelectasis and pneumonia after a long hospitalization. The transcondylar approach is recommended for anterior locations, and the standard suboccipital approach for posterior locations.

Evaluation of the management of arachnoid cyst of the posterior fossa in pediatric population: experience over 27 years.

INTRODUCTION: The necessity of surgical treatment of the arachnoid cyst in general and posterior fossa arachnoid cyst (PFAC) in particular is sometimes controversial. Surgery is warranted in symptomatic patients. In this study, we evaluated our experience throughout 27 years in the management of patients with PFAC. MATERIALS AND METHODS: The study was designed with special emphasis on clinical features, surgery, and outcome. We investigated a total of 112 arachnoid cysts. Of them, 23 patients were symptomatic for PFAC. We assessed clinical characteristic and outcome for all patients. We obtained information from medical records and outpatient charts on age, sex, presenting symptoms, associated abnormalities, psychomotor status, modality of treatment, complications and follow-up in 23 patients (9 boys, 14 girls, ages 1 day-6 years) who had been admitted for evaluation and treatment of PFAC at Kobe Children's Hospital between 1978 and 2004. RESULTS: The mean follow-up period was 93 months (range 5-313 months). More than half of the patients had a history of increased head size and signs of intracranial pressure as presenting symptoms. One-third of the patients had associated abnormalities and six patients (26%) presented hydrocephalus. Computed tomography cisternography displayed delayed filling of the cyst in 11 patients (48%). All patients were treated surgically; the total number of surgical procedures was 55 with a surgical rate of 2.4 per patients. The marsupialization and marsupialization with cyst-peritoneal (C-P) shunt were the most common open surgical procedure in 14 and 5 patients, respectively. Endoscopic cysternostomy was performed in three patients. In our series, 25 (45%) of 55 surgical procedures included shunt malfunction and removal. The marsupialization of the cyst wall was successful in 9 of 14 operated patients (64%); the other five patients needed additional C-P or ventriculoperitoneal (V-P) shunt. Marsupialization with cyst-peritoneal shunt was successful in only one of five patients (20%), and for the other four patients, additional C-P or V-P shunt was necessary. No mortality is reported. Eight patients presented minor surgical morbidities that were resolved conservatively. DISCUSSION: The relatively good results using marsupialization or endoscopic cisternostomy as surgical procedure and the high incidence of shunt malfunction buttresses our use of both operations as a first-line surgery at present. Surgical procedure that does not include shunting decreases the ratio of recurrent operation because this kind of complication develops over time and should be consider as a second-line procedure.

Presacral meningocele associated with hereditary sacral agenesis and treated surgically: evaluation in three members of the same family.

OBJECTIVE AND IMPORTANCE: Presacral meningocele in hereditary sacral agenesis is a complex and unusual spinal dysgenetic syndrome. Recognition of the syndromic triad, its natural history, and familial presentation has important practical applications for the management of this disease as well as its complications. CLINICAL PRESENTATION: This report concerns three patients in one family with Currarino syndrome. We detail its clinical presentation, operative management, and outcome and suggest management procedures based on reports in the literature and the results of our surgical techniques, which focus on cases with an "incomplete" triad. INTERVENTION: Three members of the same family, one adult and two children, underwent surgery through the posterior sacral approach tying off the communication between dural sac and anterior meningocele. The adult underwent a second surgical procedure in which a custom-designed surgical technique was used to resolve postoperative cerebrospinal fluid leakage. One of the children underwent an additional posterior sagittal anorectoplasty to remove a presacral teratoma. CONCLUSION: We report a rare occurrence of three familial cases of sacral agenesis accompanied by a presacral mass with various degrees of phenotypic expression and with male dominant transmission. Because of its rarity, the best surgical technique and timing remain an open question especially in cases with incomplete triad syndrome.