Spontaneous intracranial hypotension (SIH) is a syndrome with serious neurological sequelae. As demonstrated by the following report, recurrent episodes of SIH can be difficult to diagnose when associated with other neurosurgical procedures, such as craniectomies. In this paper, the authors demonstrate SIH presenting as a subdural hematoma with recurrence of CSF leaks. Spontaneous intracranial hypotension was further complicated by paradoxical herniation following a craniectomy. Treatment of SIH necessitated multiple epidural blood patches for CSF leaks at different spinal levels and at different times. The efficacy of each epidural blood patch was confirmed with radionuclide imaging. Confirmation of effective blood patch placement may be useful for identifying patients at risk for a failed epidural blood patch or for patients whose neurological examination results have not fully improved.
Timothy W. Vogel, Brian J. Dlouhy and Matthew A. Howard III
Timothy W. Vogel, Brian J. Dlouhy and Matthew A. Howard III
The object of this study was to evaluate the causes of plunging events associated with automatic-releasing cranial perforators at the authors' institution.
The authors analyzed a consecutive series of 1652 cranial procedures involving one type of automaticreleasing cranial perforator over a 2-year period. Plunging occurrences were recorded for 2 drill speeds: 1000 rpm in the 1st year and 800 rpm during the 2nd year. Intraoperative observations, neuroimaging studies, and clinical data were evaluated for each plunging event.
The authors identified 9 plunging events for an overall incidence of 0.54%. In the 1st year, they identified 2 plunging events at a speed of 1000 rpm for an incidence of 0.19%. In an effort to reduce this occurrence, the speed of the drill was lowered to 800 rpm. There were 7 additional events, for a significantly increased incidence of 1.16% (p = 0.014, Fisher exact test) after the change was implemented. These cases spanned a number of procedures in adults and pediatric patients, including ventriculostomy placement, craniotomies for tumor resection, tumor biopsy, and endoscopic third ventriculostomy. Despite plunging, no immediate postoperative complications were noted on clinical examination.
While technology continues to improve cranial perforator performance, the use of such a device is still associated with a risk of complications causing dural lacerations and injury to the underlying cortex. Decreasing the drill speed may not decrease the incidence of plunging.
Sunil Manjila, Timothy W. Vogel, Yunwei Chen, Mark S. Rodgers and Alan R. Cohen
Hypothalamic hamartomas (HHs) are rare developmental lesions arising from the inferior hypothalamus that may cause gelastic seizures and central precocious puberty. Cystic changes in HHs are rare, usually occurring in giant lesions. The authors describe an unusual case of cystic HH masquerading as a suprasellar arachnoid cyst in an 18-month-old girl presenting with precocious puberty. Microsurgical removal of the lesion led to complete resolution of the precocious puberty on long-term follow-up. This case is the first reported HH with pathological demonstration of corticotropin-releasing hormone immunostaining in the solid tumor and glial cells in the cyst wall of the lesion. The clinical and radiological characteristics of HHs are reviewed, along with the unique surgical strategies used to manage cystic lesions in the suprasellar region.
Gregory W. Albert, Nader S. Dahdaleh, Chandan Reddy, Daniel R. Hansen, Timothy W. Vogel, Hiroto Kawasaki and Matthew A. Howard III
In this study the authors sought to determine whether any correlations existed between postimplantation head CT findings and the need to perform decompression surgery in patients with grid electrodes.
The authors identified 74 patients who underwent intracranial electrode monitoring for medically refractory epilepsy from January 2000 through June 2008. Only the 46 patients who had head CT scans available for review were included in the study. The authors were able to determine the number and types of electrodes placed as well as complications experienced. They reviewed the CT scans for abnormal findings including extraaxial fluid collections, intracranial hemorrhages, and signs of mass effect.
All patients developed some degree of extraaxial fluid collection following the placement of intracranial electrodes. The maximum width of the extraaxial fluid collection and the degree of midline shift were not predictive of the need for decompressive surgery. The presence, but not degree, of midline shift was associated with the need for decompressive surgery. Likewise, the presence of ventricular asymmetry was correlated with the need for removal of the electrodes and bone flap. Patients without midline shift or ventricular asymmetry on CT did not require decompressive surgery.
After undergoing placement of intracranial electrodes all patients develop extraaxial fluid collections. In addition, many patients develop signs of mass effect including midline shift and ventricular asymmetry. When these findings are absent it is highly unlikely that surgical decompression is required.
Brian J. Dlouhy, Steven V. Viljoen, David K. Kung, Timothy W. Vogel, Mark A. Granner, Matthew A. Howard III and Hiroto Kawasaki
Vagus nerve stimulation (VNS) has demonstrated benefit in patients with medically intractable partial epilepsy. As in other therapies with mechanical devices, hardware failure occurs, most notably within the VNS lead, requiring replacement. However, the spiral-designed lead electrodes wrapped around the vagus nerve are often encased in dense scar tissue hampering dissection and removal. The objective in this study was to characterize VNS lead failure and lead revision surgery and to examine VNS efficacy after placement of a new electrode on the previously used segment of vagus nerve.
The authors reviewed all VNS lead revisions performed between October 2001 and August 2011 at the University of Iowa Hospitals and Clinics. Twenty-four patients underwent 25 lead revisions. In all cases, the helical electrodes were removed, and a new lead was placed on the previously used segment of vagus nerve. All inpatient and outpatient records of the 25 lead revisions were retrospectively reviewed.
Four cases were second lead revisions, and 21 cases were first lead revisions. The average time to any revision was 5 years (range 1.8–11.1 years), with essentially no difference between a first and second lead revision. The most common reason for a revision was intrinsic lead failure resulting in high impedance (64%), and the most common symptom was increased seizure frequency (72%). The average duration of surgery for the initial implantation in the 15 patients whose VNS system was initially implanted at the authors' institution was much shorter (94 minutes) than the average duration of lead revision surgery (173 minutes). However, there was a significant trend toward shorter surgical times as more revision surgeries were performed. Sixteen of the 25 cases of lead revision were followed up for more than 3 months. In 15 of these 16 cases, the revision was as effective as the previous VNS lead. In most of these cases, both the severity and frequency of seizures were decreased to levels similar to those following the previous implantation procedure. Only 1 complication occurred, and there were no postoperative infections.
Lead revision surgery involving the placement of a new electrode at the previously used segment of vagus nerve is effective at decreasing the seizure burden to an extent similar to that obtained following the initial VNS implantation. Even with multiple lead revisions, patients can obtain VNS efficacy similar to that following the initial lead implantation. There is a learning curve with revision surgery, and overall the duration of surgery is longer than for the initial implantation. Note, however, that complications and infection are rare.
Libby Kosnik-Infinger, Craig Gendron, Christopher B. Gordon, Brian S. Pan, John A. van Aalst and Timothy W. Vogel
Hypophosphatasia (HPP) is a rare inherited disorder of bone metabolism that results in the loss of function of the gene coding for tissue-nonspecific alkaline phosphatase (TNSALP). Patients with HPP have defective bone mineralization as well as craniosynostosis that can be seen in the infantile and childhood forms of this disease. Traditionally, HPP has had a poor prognosis, with few children surviving to exhibit the phenotype of clinical craniosynostosis that requires surgical intervention. Here, the authors report on new advancements in enzyme replacement therapy (ERT) for children affected by HPP, allowing these patients to survive and undergo surgery to address complex craniosynostosis. The authors discuss their case series of 4 HPP patients treated at their institution with ERT who have undergone successful surgical intervention for craniosynostosis. These children had no complications related to their surgeries and exhibited decreased neurological symptoms following cranial vault remodeling. This study reveals that ERT administered either pre- or post- operatively paired with cranial vault remodeling strategies can yield improved neurological outcomes in children affected by HPP.
Timothy W. A. Vogel, Alexander O. Vortmeyer, Irina A. Lubensky, Youn-Soo Lee, Makoto Furuta, Barbara Ikejiri, H. Jeffrey Kim, Russell R. Lonser, Edward H. Oldfield and Zhengping Zhuang
Object. Von Hippel—Lindau (VHL) disease is characterized by multiple tumors in specific organs. The cell of origin and the reason for the particular organ distribution of the tumors remains unknown. Endolymphatic sac tumor (ELST) is one of the lesions associated with VHL disease. Data from previous studies of VHL disease—associated hemangioblastomas (HBs) and renal cell carcinomas (RCCs) have indicated that VHL gene deficiency causes coexpression of erythropoietin (Epo) and its receptor (Epo-R), which facilitates tumor growth.
Methods. The authors studied ELSTs from five patients with VHL germline mutations. Analysis of the five ELST samples revealed loss of the wild-type allele, consistent with Knudson's two-hit hypothesis for tumorigenesis. All five ELST specimens were characterized microscopically and by immunohistochemical analysis. Coexpression of Epo and Epo-R was found in all five tumors on immunohistochemical studies and confirmed through reverse transcription—polymerase chain reaction and Western blot analysis.
Conclusions. Expression of Epo appears to be a result of VHL gene deficiency, whereas the simultaneous coexpression of Epo-R may reflect a developmental mechanism of tumorigenesis. Coexpression of Epo and Epo-R in ELSTs together with the morphological and genetic similarities of these lesions with other VHL disease—associated tumors indicates that VHL disease—associated tumors in different organs share common pathogenetic pathways.
Timothy W. Vogel, Albert S. Woo, Alex A. Kane, Kamlesh B. Patel, Sybill D. Naidoo and Matthew D. Smyth
The surgical management of infants with sagittal synostosis has traditionally relied on open cranial vault remodeling (CVR) techniques; however, minimally invasive technologies, including endoscope-assisted craniectomy (EAC) repair followed by helmet therapy (HT, EAC+HT), is increasingly used to treat various forms of craniosynostosis during the 1st year of life. In this study the authors determined the costs associated with EAC+HT in comparison with those for CVR.
The authors performed a retrospective case-control analysis of 21 children who had undergone CVR and 21 who had undergone EAC+HT. Eligibility criteria included an age less than 1 year and at least 1 year of clinical follow-up data. Financial and clinical records were reviewed for data related to length of hospital stay and transfusion rates as well as costs associated with physician, hospital, and outpatient clinic visits.
The average age of patients who underwent CVR was 6.8 months compared with 3.1 months for those who underwent EAC+HT. Patients who underwent EAC+HT most often required the use of 2 helmets (76.5%), infrequently required a third helmet (13.3%), and averaged 1.8 clinic visits in the first 90 days after surgery. Endoscope-assisted craniectomy plus HT was associated with shorter hospital stays (mean 1.10 vs 4.67 days for CVR, p < 0.0001), a decreased rate of blood transfusions (9.5% vs 100% for CVR, p < 0.0001), and a decreased operative time (81.1 vs 165.8 minutes for CVR, p < 0.0001). The overall cost of EAC+HT, accounting for hospital charges, professional and helmet fees, and clinic visits, was also lower than that of CVR ($37,255.99 vs $56,990.46, respectively, p < 0.0001).
Endoscope-assisted craniectomy plus HT is a less costly surgical option for patients than CVR. In addition, EAC+HT was associated with a lower utilization of perioperative resources. Theses findings suggest that EAC+HT for infants with sagittal synostosis may be a cost-effective first-line surgical option.