Microvascular decompression is a very effective and relatively safe surgical modality in the treatment of hemifacial spasm. But rare debilitating complications have been reported such as cranial nerve dysfunctions. We have experienced a very rare case of unilateral soft palate palsy without the involvement of vocal cord following microvascular decompression. A 33-year-old female presented to our out-patient clinic with a history of left hemifacial spasm for 5 years. On postoperative 5th day, patient started to exhibit hoarsness with swallowing difficulty. Symptoms persisted despite rehabilitation. Various laboratory work up with magnetic resonance image showed no abnormal lesions. Two years after surgery patient showed complete recovery of unitaleral soft palate palsy. Various etiologies of unilateral soft palate palsy are reviewed as the treatment and prognosis differs greatly on the cause. Although rare, it is important to keep in mind that such complication could occur after microvascular decompression.
Microvascular decompression; Hemifacial spasm; Lower cranial nerve; Soft palate palsy; Vocal cord
The purpose of this study was to evaluate the characteristics and surgical outcomes of familial hemifacial spasm (HFS) and to discuss the role of genetic susceptibility.
Between 2001 and 2011, 20 familial HFS patients with ten different pedigrees visited our hospital. The data from comprehensive evaluation of these patients, including clinical, radiological and electrophysiological data and surgical outcomes were reviewed to characterize familial HFS and to compare the characteristics between familial HFS and sporadic HFS.
According to the family tree, the inheritance pattern was difficult to define clearly using these data. Radiologic findings suggested that the vertebral artery (VA) was a more frequent offender in familial HFS than in sporadic cases (35.0% vs. 10.0%, p<0.001). Chi-square test showed that there were no correlation between VA tortuosity and underlying morbidity such as diabetes or hypertension (p=0.391). Eighteen out of 19 patients who underwent microvascular decompression showed no residual spasm. Other features of familial HFS overlap with sporadic cases. These findings suggest that certain genetic susceptibilities rather than hypertension or diabetes may influence vascular tortuosity and HFS development.
In this study, familial HFS seems not so different from sporadic cases. Authors thought familial HFS could have heterogeneous etiology. Further study of familial HFS including clinical, anatomic, genetic, and molecular information may help identify a gene or trait that can provide insight into the mechanisms of sporadic and familial HFS.
Hemifacial spasm; Familial; Microvascular decompression; Neurovascular conflict
Microvascular decompression (MVD) for hemifacial spasm (HFS) is a safe and effective treatment with favorable outcomes. The purpose of this study was to evaluate the incidence of delayed cranirve ( VI, VII, and VIII ) palsy following MVD and its clinical courses.
Between January 1998 and December 2009, 1354 patients underwent MVD for HFS at our institution. Of them, 100 patients (7.4%) experienced delayed facial palsy (DFP), one developed sixth nerve palsy, and one patient had delayed hearing loss.
DFP occurred between postoperative day number 2 and 23 (average 11 days). Ninety-two patients (92%) completely recovered; however, House-Brackmann grade II facial weakness remained in eight other patients (8%). The time to recovery averaged 64 days (range, 16 days to 9 months). Delayed isolated sixth nerve palsy recovered spontaneously without any medical or surgical treatment after 8 weeks, while delayed hearing loss did not improve.
Delayed cranial nerve (VI, VII, and VIII) palsies can occur following uncomplicated MVD for HFS. DFP is not an unusual complication after MVD, and prognosis is fairly good. Delayed sixth nerve palsy and delayed hearing loss are extremely rare complications after MVD for HFS. We should consider the possibility of development of these complications during the follow up for MVD.
Delayed facial palsy; Microvascular decompression; Delayed cranial palsy; Delayed hearing loss; Delayed abducens palsy
The aim of this study was to assess the safety and efficacy of radiation-sterilized allografts of iliac bone and fascia lata from cadaver specimens to repair skull base defects after transsphenoidal surgery.
Between May 2009 and January 2010, 31 consecutive patients underwent endonasal transsphenoidal surgery and all patients received sellar reconstruction using allografts following tumor removal. The allografts were obtained from the local tissue bank and harvested from cadaver donors. The specimens used in our approach were tensor fascia lata and the flat area of iliac bone. For preparation, allografts were treated with gamma irradiation after routine screening by culture, and then stored at -70℃.
The mean follow-up period after surgery was 12.6 months (range, 7.4-16 months). Overall, postoperative cerebrospinal fluid (CSF) leaks occurred in three patients (9.7%) and postoperative meningitis in one patient (3.2%). There was no definitive evidence of wound infection at the routine postoperative follow-up examination or during re-do surgery in three patients. Postoperative meningitis in one patient was improved with the use of antibiotics and prolonged CSF diversion.
We suggest that allograft materials can be a feasible alternative to autologous tissue grafts for sellar reconstruction following transsphenoidal surgery under selected circumstances such as no or little intraoperative CSF leaks.
Endoscopic endonasal approach; Transsphenoidal approach; Allograft; Sellar reconstruction; CSF leak
Intrathecal methotrexate (MTX) therapy combined with whole brain radiotherapy (WBRT) is one of the major treatment modalities for leukemia and lymphoma involving the central nervous system (CNS). The purpose of this study was to retrospectively determine the incidences of leukoencephalopathy and disseminated necrotizing leukoencephalopathy (DNL) following intrathecal MTX therapy for CNS lymphoma or leukemia and to assess the potential risk factors.
Between January 2000 and August 2009, 143 patients with CNS lymphoma or leukemia received intrathecal MTX therapy alone or in combination with WBRT at a single institution. Patients were followed up clinically and radiologically at regular two- or three-month intervals. Medical records were reviewed to obtain information regarding the patients' demographics, medical histories, radiologic characteristics, treatments, and clinical courses.
On follow-up MR images, leukoencephalopathy was found in 95 of 143 patients (66.4%). The median time to develop leukoencephalopathy was 6.6 months. Among those with leukoencephalopathy, four patients showed seven extensive white-matter changes with strongly enhancing lesions demonstrating DNL. Histological confirmation was done in six lesions of three patients and radiological diagnosis alone in one patient. Four lesions spontaneously disappeared on MR images without any treatment, with a mean duration of 14 months before disappearance of DNL.
Leukoencephalopathy is a common phenomenon that occurs following intrathecal MTX therapy; however, DNL occurs at a very low incidence. For newly developed enhancing lesions, consideration for the occurrence of DNL should be taken to avoid unnecessary invasive procedures or therapies.
Leukoencephalopathy; Methotrexate; Intrathecal; Lymphoma; Leukemia
The purpose of this study was to examine the efficacy and safety of microvascular decompression (MVD) for hemifacial spasm (HFS) in elderly patients.
Between 1997 and June 2008, 1,174 patients had undergone MVD for HFS at our institute. Among these, 53 patients were older than 65 years. We retrospectively reviewed and compared the complication and the cure rates of these patients with those of younger patients.
There were 38 females and 15 males. The mean duration of symptoms of HFS of these patients was 94.6 months (range, 12-360 months), compared with 67.2 months (range, 3-360 months) in the younger group. The overall cure rate in elderly patients who underwent MVD for HFS during this period was 96.2%. Permanent cranial nerve dysfunctions, such as hearing loss and facial palsy, were seen in 2 patients (3.8%, 2/53) in the elderly group and 19 patients (1.7%, 19/1121) in the younger group. The difference in permanent cranial nerve dysfunction between the two groups was not statistically significant. There was no operative mortality in either group.
Microvascular decompression is the most effective surgical modality available for the treatment of HFS. Results of this study indicate that such technique can be performed in the elderly without higher rates of morbidity or mortality. Any patient with HFS, whose general health is acceptable for undergoing general anesthesia, should be considered as a candidate for MVD.
Microvascular decompression; Hemifacial spasm; Elderly patients
A case of delayed progressive extradural pneumatocele after microvascular decompression (MVD) is presented. A 60-year-old male underwent MVD for hemifacial spasm; the mastoid air cell was opened and sealed with bone wax during surgery. One month after surgery, the patient complained of tinnitus, and progressive extradural pneumatoceles without cerebrospinal fluid (CSF) leakage was observed. Revision surgery was performed and the opened mastoid air cell was completely sealed with muscle patch and glue. The patient's symptoms were resolved, with no recurrence of pneumatoceles at 6 month follow up. Progressive extradural pneumatocele without CSF leakage after posterior fossa surgery is a very rare complication. Previous reports and surgical management of this rare complication are discussed.
Pneumatocele; Posterior fossa surgery; Hemifaical spasm; Mastoid air cell
Combined hyperative dysfunction syndrome (HDS) defined as the combination of HDSs such as trigeminal neuralgia (TN), hemifacial spasm (HFS) and glossopharyngeal neuralgia (GPN), which may or may not occur simultaneously on one or both sides. We reviewed patients with combined HDS and demonstrated their demographic characteristics by comparing them with those of patients with a single HDS.
Between October 1994 and February 2006, we retrospectively studied a series of 1,720 patients who suffered from HDS and found 51 patients with combined HDSs. We analyzed several independent variables in order to evaluate the prevalence and etiologic factors of combined HDS.
The combined HDS group accounted for 51 of 1,720 (2.97%) patients with HDS; 27 cases of bilateral HFS, 10 cases of bilateral TN and 14 cases of HFS with TN. Their mean age was 52.1 years (range, 26-79 years). There were 5 men and 46 women. Seven patients had synchronous and 44 patients metachronous onset of HDSs. By comparison of combined and single HDS groups, we found that age and hypertension were closely associated with the prevalence of combined HDS (p < 0.05).
This study revealed that combined HDS was very rare. Hypertension and age might be the most important causative factors to evoke combined HDS.
Combined hyperactive dysfunction syndrome; Prevalence; Hemifacial spasm; Trigeminal neuralgia; Etiology
The factors that influence the prognosis of patients with hemifacial spasm (HFS) treated by microvascular decompression (MVD) have not been definitely established. We report a prospective study evaluating the prognostic factors in patients undergoing MVD for HFS.
From January 2004 to September 2006, the authors prospectively studied a series of 293 patients who underwent MVD for HFS. We prospectively analyzed a number of variables in order to evaluate the predictive value of independent variables for the prognosis of patients undergoing MVD. The patients were followed-up at regular intervals and divided into as cured and unsatisfactory groups based on symptom relief. Uni- and multivariate analyses were performed using logistic regression models.
A total 273 of 293 (94.2%) patients achieved symptom relief within one year after the operation. Intraoperatively, the indentation of the root exit zone was observed in 259 (88.5%) patients. Uni- and multivariate analyses revealed that the symptoms at postoperative 3 months (p<0.001) and indentation of the root exit zone (p=0.036) were associated with good outcomes.
The intraoperative finding of root exit zone indentation will help physicians determine the prognosis in patients with HFS. To predict the prognosis of HFS, a regular follow-up period of at least 3 months following MVD should be required.
Hemifacial spasm; Microvascular decompression; Prognosis; Chronology
This study was performed to assess the efficacy of GKS in patients with ten or more brain metastases.
From Aug 2002 to Dec 2007, twenty-six patients (13 men and 13 women) with ten or more cerebral metastatic lesions underwent GKS. The mean age was 55 years (32-80). All patients had Karnofsky performance status (KPS) score of 70 or better. According to recursive partitioning analysis (RPA) classification, 3 patients belonged to class I and 23 to class II. The location of primary tumor was lung (21), breast (3) and unknown (2). The mean number of the lesions per patient was 16.6 (10-37). The mean cumulated volume was 10.9 cc (1.0-42.2). The median marginal dose was 15 Gy (9-23). Overall survival and the prognostic factors for the survival were retrospectively analyzed by using Kaplan Meier method and univariate analysis.
Overall median survival from GKS was 34 weeks (8-199). Local control was possible for 79.5% of the lesions and control of all the lesions was possible in at least 14 patients (53.8%) until 6 months after GKS. New lesions appeared in 7 (26.9%) patients during the same period. At the last follow-up, 18 patients died; 6 (33.3%) from systemic causes, 10 (55.6%) from neurological causes, and 2 (11.1%) from unknown causes. Synchronous onset in non-small cell lung cancer (p=0.007), high KPS score (≥80, p=0.029), and controlled primary disease (p=0.020) were favorable prognostic factors in univariate analysis.
In carefully selected patients, GKS may be a treatment option for ten or more brain metastases.
Multiple; Brain metastases; Gamma knife radiosurgery; Prognostic factor
Radiosurgery may be contraindicated for lesions adjacent to the optic pathways because of the substantial risk of visual complication. Multisession radiosurgery has been tried as a compromise between single session radiosurgery and fractionated radiotherapy. The purpose of this study is to evaluate the outcomes of multisession gamma knife radiosurgery (GKRS) in 22 patients with perioptic lesions of benign pathology.
In all 22 cases, the lesions were within 1 mm of the optic apparatus and were therefore not considered suitable for single session radiosurgery. Radiation was delivered in 3 to 4 fractions with a median cumulated marginal dose of 20 Gy (range, 15-20 Gy).
During a mean follow-up of 29 months (range, 14-44 months), tumor control was achieved in 21 patients. Visual function improved in 7 patients, remained unchanged in 14 patients, and deteriorated in 1 patient with tumor progression. No other complication was observed.
This preliminary result supports the idea that multisession GKRS may be an effective and safe alternative for treatment in perioptic lesions that are unsuitable for single session radiosurgery.
Multisession radiosurgery; Gamma knife; Visual complication
Hemifacial spasm (HFS) is characterized by tonic clonic contractions of the muscles innervated by the ipsilateral facial nerve. Compression of the facial nerve by an ectatic vessel is widely recognized as the most common underlying etiology. HFS needs to be differentiated from other causes of facial spasms, such as facial tic, ocular myokymia, and blepharospasm. To understand the overall craniofacial abnormalities and to perform the optimal surgical procedures for HFS, we are to review the prevalence, pathophysiology, differential diagnosis, details of each treatment modality, usefulness of brainstem auditory evoked potentials monitoring, debates on the facial EMG, clinical course, and complications from the literature published from 1995 to the present time.
Hemifacial spasm; Microvascular decompression; Craniofacial abnormalities