Training and teaching of vagus nerve stimulation surgery: Worldwide survey and future perspectives.

OBJECTIVE: Vagus nerve stimulation (VNS) therapy has been used for more than two decades to treat drug resistant epilepsy and depression and most recently received FDA approval for stroke rehabilitation. Expanding indications will renew the interest in the technique and increase the number of surgeons to be trained. The aim of this study was to survey surgeons with substantial expertise on optimal teaching and training approaches. METHODS: Anonymous forms comprising 16 questions were sent by e-mail to surgeons with substantial expertise. Statistical analyses were used to compare the answers of the most experienced surgeons (>5 years) with the less experienced ones (<5 years). RESULTS: Fully-completed forms were collected from 57 experts from 20 countries. The placement of the helical coils was deemed to be the most difficult step by 36 (63.2%) experts, and the use of optical magnification during this step was deemed necessary by 39 (68.4%) experts. Vocal cord palsy should be largely avoidable with proper surgical technique according to 44 (77.2%) experts. The teaching tool considered the most useful was mentoring (38, 66.7%). The future of VNS surgery teaching was deemed to be in anatomical workshops (29, 50.9%) and surgical simulation (26, 45.6%). Overall, answers did not vary significantly according to experience. CONCLUSIONS: VNS surgery should be mastered by actively participating in dedicated practical training courses and by individual mentoring during actual surgery, which is still the best way to learn. This study highlights the need for a formal training course and possible specific accreditation.

Thoracoscopic Microdiscectomy with Preservation of Rib and Costovertebral Joint.

INTRODUCTION: Thoracic disc herniations (TDH) may cause major morbidity. While thoracoscopic microdiscectomy (TMD) is an excellent technique, postoperative band-like pain is an important drawback. MATERIAL AND METHODS: We performed 181 consecutive TMDs (including 39 high-risk cases) with preservation of rib and costovertebral joint (CVJ). We shave a few mm of the rib, drill straight to target, and avoid opening the canal before the TDH is completely free and (in case of giant TDHs) internally debulked, creating initial decompression and limiting epidural venous oozing. Subsequently, we gently mobilize and remove the residual TDH while avoiding leverage. RESULTS: Skin-to-skin time was <90' in 64, 90-120' in 48, >120' in 20, unknown in 10, and 162' mean in 39 high-risk procedures. Blood loss was <100 mL in 76, <250 mL in 48, and 537 mL mean in 39 high-risk procedures. The technique was successfully applied in all (including nine dural repairs) without a single conversion. We observed an increased neurological deficit in two (1.1%) and inadequate decompression in merely one (wrong level). Complications (mainly pulmonary) were few and managed conservatively, except for a segmental artery pseudoaneurysm treated endovascularly. We observed a substantial decrease in acute and chronic postoperative pain. DISCUSSION: The technique is fast, straightforward, minimizes bone resection and blood loss, improves orientation, safely and effectively deals with any TDH, and prevents postoperative band-like pain as the CVJ is preserved. CONCLUSION: We hope this technique will find broader acceptance among a new generation of spine surgeons to benefit patients suffering TDH-related myelopathy or merely intractable pain.

Treatment of a Large Skull Defect and Brain Herniation in a Newborn With Adams-Oliver Syndrome.

Adams-Oliver syndrome (AOS) is a rare congenital disorder characterised by a wide variety of clinical expression ranging from the occurrence of aplasia cutis congenita (ACC), transverse limb defects, and cutis marmorata telangiectica to extensive lethal anomalies. In this article, we present the conservative and surgical management of a male newborn infant diagnosed with AOS. Surgical treatment included wound management, the removal of protruding brain, and treatment of cerebrospinal fluid (CSF) leakage. After spontaneous reepithelization of the wounds, conservative treatment was chosen instead of reconstruction with an occipital flap; this was continued until the total healing of the dermal defect after eight months, during which the patient was continuously treated with antibiotics. At 17 months, the child was in good physical condition with a three-month development delay in comparison with infants of his age and no evidence of neurological deficit.

Unexpected death after occipital condylar fracture.

We present a rare fatal complication of an occipital condylar fracture. The patient was initially neurologically intact, but showed secondary clinical deterioration. Imaging revealed extensive extra-axial hemorrhage at the craniocervical junction and an acute obstructive hydrocephalus. MR imaging demonstrated a T2 hyperintens signal in both the lower brainstem and upper cervical spinal cord, likely caused by the extra-axial hemorrhage. As prognosis was estimated infaust, supportive treatment was discontinued and the patient died soon thereafter. This case report illustrates a rare, delayed complication and unexpected death in a patient having sustained an occipital condylar fracture.

Childhood angular kyphosis: a plea for involvement of the pediatric neurosurgeon.

INTRODUCTION: Childhood angular kyphosis is rare, as most children are affected by a mixed kyphotic and scoliotic deformity. Published series involving a mix of kyphosis and kyphoscoliosis, pediatric and adult, congenital and acquired cases are almost exclusively authored by orthopedic surgeons, suggesting that (pediatric) neurosurgeons are not involved. CASE SERIES: We present five cases that illustrate the spectrum of angular kyphosis, and these were treated by a multidisciplinary team including child neurologist, orthopedic surgeon, and pediatric neurosurgeon as complementary partners. DISCUSSION: Angular kyphosis is a cosmetic problem but above all a serious threat to the spinal cord and as such to the child's ambulatory, sphincter, and genito-urinary functions. Spinal cord stretch over the internal kyphosis may cause pain and/or neurological deficit, often accompanied by myelomalacia or even segmental cord atrophy. Spinal cord function may be additionally affected by associated disorders such as syringomyelia or tethered cord, an orthopedic surgeon may be less familiar with. The decision when and how to proceed surgically should be made by a multidisciplinary team, including a pediatric neurosurgeon who actively participates in the operation and helps to safely achieve adequate spinal cord decompression and stabilization. CONCLUSION: Childhood angular kyphosis is a complex, heterogeneous disorder that should be managed by a multidisciplinary team in specialized pediatric spine centers. While every case is truly unique, the spinal cord is always at risk, especially during decompression, stabilization, and eventual correction of deformity. Pediatric neurosurgeons have an important role to play in preoperative work-up, actual operation, and follow-up.

Fever after intraventricular neuroendoscopic procedures in children.

PURPOSE: The purpose of this paper was to study the incidence and clinical significance of fever after intraventricular neuroendoscopic procedures in children. METHODS: We retrospectively assessed all children subjected to an intraventricular neuroendoscopic procedure between 2004 and 2015. Body temperature 6 days postoperatively, symptoms and signs, and eventual cerebrospinal fluid analysis were evaluated. Fever was defined as temperature above 38 °C. RESULTS: Fifty-five children (mean age 4.8 years) had 67 procedures. Forty-three children (47 procedures, 70 %) developed fever, mostly the day of surgery (n = 17; 25 %) or the next day (n = 33; 49 %). All children who were clinically ill (n = 9, including 7 with fever) suffered serious illness, as opposed to none of the children with fever without being clinically ill (n = 36). Fever was unrelated to gender, indication for, and type of procedure and did not influence ETV success rate at 3 months. Children under 1 year less frequently developed fever (p = 0.032). CONCLUSIONS: Fever frequently develops after intraventricular neuroendoscopic procedures in children and follows a rather predictable course, peaking the day of surgery and/or the next day, and rapidly subsiding thereafter. Fever is not a cardinal symptom except when combined with other symptoms in children who are clinically ill (which most of them are not). Close observation avoiding invasive diagnostic tests may suffice for those who are not clinically ill, while extra attention should be paid to those whose temperature rises after day 2 especially when clinically ill, as they likely suffer serious illness. We recommend to closely observe children after any intraventricular neuroendoscopic procedure for at least 5 days.

Sprengel's deformity and spinal dysraphism: connecting the shoulder and the spine.

BACKGROUND AND PURPOSE: Sprengel's deformity, a rare congenital malformation of the scapula, may be observed in combination with spinal dysraphism. The co-occurrence of these malformations suggests an unknown shared etiology. Therefore, we reviewed the medical records of eight children presenting with both malformations and performed a review of the literature. PATIENTS AND METHODS: Databases from four university medical centers were searched for children presenting between 1992 and 2012 with spinal dysraphism and a Sprengel's deformity. CONCLUSION: The combination of spinal dysraphism and Sprengel's deformity is rare, and is associated with segmentation defects of the spine and ribs. Although the etiology of both spinal dysraphism and Sprengel's deformity remains unclear, all deformities of the spine, ribs, and shoulder might result from a common genetic defect affecting somitogenesis.

Implementation of a mobile 0.15-T intraoperative MR system in pediatric neuro-oncological surgery: feasibility and correlation with early postoperative high-field strength MRI.

INTRODUCTION: We analyze our preliminary experience using the PoleStar N20 mobile intraoperative MR (iMR) system as an adjunct for pediatric brain tumor resection. METHODS: We analyzed 11 resections in nine children between 1 month and 17 years old. After resection, we acquired iMR scans to detect residual tumor and update neuronavigation. We compared final iMR interpretation by the neurosurgeon with early postoperative MR interpretation by a neuroradiologist. RESULTS: Patient positioning was straightforward, and image quality (T1 7-min 4-mm sequences) sufficient in all cases. In five cases, contrast enhancement suspect for residual tumor was noted on initial postresection iMR images. In one case, a slight discrepancy with postoperative imaging after 3 months was no longer visible after 1 year. No serious perioperative adverse events related to the PoleStar N20 were encountered, except for transient shoulder pain in two. CONCLUSIONS: Using the PoleStar N20 iMR system is technically feasible and safe for both supra- and infratentorial tumor resections in children of all ages. Their small head and shoulders favor positioning in the magnet bore and allow the field of view to cover more than the area of primary interest, e.g., the ventricles in an infratentorial case. Standard surgical equipment may be used without significant limitations. In this series, the use of iMR leads to an increased extent of tumor resection in 45 % of cases. Correlation between iMR and early postoperative MR is excellent, provided image quality is optimal and interpretation is carefully done by someone sufficiently familiar with the system.

Sacral nerve modulation for defaecation and micturition disorders in patients with spina bifida.

AIM: Sacral nerve modulation is a well accepted method for the treatment of defaecation disorders and voiding dysfunction. Results of sacral nerve modulation in patients with spinal cord lesions are not well assessed, but preliminary results look poor. Therefore, the purpose of this study was to assess the effectiveness of sacral nerve modulation for defaecation disorders and voiding dysfunction in patients with spina bifida. METHOD: Consecutive patients with spina bifida suffering from a myelomeningocele and combined faecal and urinary functional disorders that were eligible for peripheral nerve evaluation (PNE) were studied. A permanent sacral nerve modulation implantation was performed after successful PNE. RESULTS: Ten patients (four female) were included in this study with a median age of 26.4 (range 11.1-41.0) years. In two the PNE was not possible. The median faecal incontinence days (6.0 vs 3.5) and episodes (8.5 vs 3.5) per 21 days decreased significantly during the 3-week period of PNE (P = 0.033). Only 3/10 (30%) patients had a more than 50% improvement and proceeded to a permanent sacral nerve modulation implantation. In one patient it was not possible to perform the permanent implant. CONCLUSION: Preliminary results of sacral nerve modulation in a subgroup of spina bifida patients with combined faecal and urinary functional disorders look promising, but long-term results in larger patient groups need to be studied.

An evidence-based mobile decision support system for subaxial cervical spine injury treatment.

Bringing evidence to practice is a key issue in modern medicine. The key barrier to information searching is time. Clinical decision support systems (CDSS) can improve guideline adherence. Mounting evidence exists that mobile CDSS on handheld computers support physicians in delivering appropriate care to their patients. Subaxial cervical spine injuries account for almost half of spine injuries, and a majority of spinal cord injuries. A valid and reliable classification exists, including evidence-based treatment algorithms. A mobile CDSS on this topic was not yet available. We developed and tested an iPhone application based on the Subaxial Injury Classification (SLIC) and 5 evidence-based treatment algorithms for the surgical approach to subaxial cervical spine injuries. The application can be downloaded for free. Users are cordially invited to provide feedback in order to direct further development and evaluation of CDSS for traumatic lesions of the spinal column.

Transsphenoidal treatment of secondary empty sella syndrome using low field strength intraoperative MRI: case report.

BACKGROUND: The purpose of this study is to demonstrate the added value of intraoperative MRI in treating secondary empty sella syndrome. CASE REPORT: We describe the case of a 66-year-old woman who was diagnosed with a prolactinoma stage IIIb. During treatment with cabergoline she presented with a secondary empty sella syndrome resulting in visual symptoms. We performed intraoperative MRI-guided packing of the secondary empty sella. We explain why this is useful in surgical treatment of secondary empty sella syndrome. CONCLUSION: Intraoperative MRI helps to achieve adequate sellar packing while avoiding insufficient packing as well as overpacking.

Intraspinal dermoid and epidermoid tumors: report of 18 cases and reappraisal of the literature.

Intraspinal dermoid and epidermoid tumors are two histopathological subtypes of cutaneous inclusion tumors of the spine. This classification is based on obsolete embryological knowledge. In fact, according to current embryology, both tumor types consist of ectodermal derivatives. Therefore, we hypothesized that dermoid and epidermoid tumors do not differ in clinical practice. To explore this hypothesis, we studied the clinical, radiological and intraoperative findings of 18 patients, and related these findings to the histopathological characteristics of the tumor. No differences were found between dermoid and epidermoid tumors regarding clinical presentation, radiological examination and outcome, while intraoperative diagnosis by the surgeon correlated with the histopathological diagnosis in only 8 of 18 cases. Therefore, the histopathological difference between intraspinal dermoid and epidermoid tumors is not important in clinical practice and should be avoided. A new nomenclature is proposed in which both tumor types are referred to as 'spinal cutaneous inclusion tumors'.

The spinal dermal-sinus-like stalk.

OBJECTS: In this study, a disjunction anomaly mimicking the spinal congenital dermal sinus (DS) is described. This anomaly is referred to as the dermal-sinus-like stalk. Dissimilarities between a true dermal sinus and a dermal-sinus-like stalk are discussed. CLINICAL MATERIAL: Three cases in which a spinal congenital dermal sinus was suspected are presented. A similar anatomical configuration, different from that of a dermal sinus, was found. All cases presented with a skin-covered dimple from which a solid tract was seen continuing intramedullary in two cases and intraspinally in one case. None of the patients presented with signs of infection or an associated dermoid-epidermoid tumor. Clinical, radiological, and surgical findings are discussed. A hypothesis is made on the pathological genesis of this malformation. CONCLUSION: A dermal-sinus-like stalk is a malformation similar to a spinal congenital dermal sinus but is not associated with DS-related complications. Despite important clinical, radiological, surgical, and histopathological differences, it is difficult to distinguish this malformation from a true DS based on clinical and radiological examination alone. Therefore, surgical intervention, at the time of diagnosis, is recommended in all cases.

Hardware failure in vagus nerve stimulation therapy.

A 20 year old male patient who had been successfully treated for epilepsy with vagus nerve stimulation (VNS) for 7 years (50% seizure frequency reduction), had experienced multiple episodes of severe hoarseness, throat pain and impaired breathing during physical exercise. As malfunctioning of the pulse generator was suspected, it was decided to replace the device. During surgery, the pulse generator was found to have broken in two, due to an unstable connection between the battery subunit and the connector subunit. With a new pulse generator seizure frequency reduction was restored. No side effects occurred.

Analysis of the success and failure of endoscopic third ventriculostomy in infants less than 1 year of age.

OBJECTIVES: In infants less than 1 year of age, the value of endoscopic third ventriculostomy (ETV) is controversial. It is believed to cause more morbidity and to have higher failure rates. We analyzed our data enlarging the reported pool of ETV outcome in infants less than 1 year of age. MATERIALS AND METHODS: We performed 12 ETVs in ten patients younger than 1 year of age. All patients had predominant supratentorial hydrocephalus. We defined ETV success as a shunt-free follow-up of at least 12 months, however, allowing re-ETV. CONCLUSION: ETV should be considered as initial treatment and carries low morbidity in these infants. As the immune system rapidly matures, postponing shunt implantation for several months or even weeks would make an ETV procedure worthwhile. On the other hand, as success probability rapidly increases 4 months after birth, re-ETV should always be considered first.

Anatomy and surgery of the infected dermal sinus of the lower spine.

OBJECT: Cases of infected dermal sinus are scarce and detailed surgical anatomical descriptions are hardly found in literature. The clinical, radiological, and surgical findings in four cases of an infected dermal sinus located at the lower spine are presented to elucidate the pathological anatomical configuration. CLINICAL MATERIAL: The first case showed two dermal sinuses with a parallel course extra- and intradurally, ending in a confluence of cavities connected to the conus. In this case, as well as in the fourth case, the signs and symptoms were those of meningitis. The second case presented with meningitis and a subdural empyema, while the third case presented with an intradermoid-intramedullary abscess at the junction between the DS and the conus. This child probably showed signs and symptoms of conus involvement as early as during pregnancy. CONCLUSION: The anatomy of the nervous elements in this congenital anomaly is heavily disturbed, more particularly in case of infection, due to extensive arachnoidal scarring. The latter renders dissection laborious and recognition of anatomical details difficult, resulting in complete excision of a dermal sinus in less than half of the cases. Despite their variability in presentation, most cases of an infected dermal sinus show similar characteristic features.

The intermediate type split cord malformation: hypothesis and case report.

METHODS: A patient is described in which a complete osteofibrotic dorsally implanted septum was found in combination with a split cord malformation in a single dural tube. This case cannot be explained using the widely used theory as proposed by Pang et al. [Pang D, Dias MS, Ahab-Barmada M (1992) Split cord malformation, part I: A unified theory of embryogenesis for double spinal cord malformations. Neurosurgery 31:451-480] but must be regarded as a combination of type I and II split cord malformation. RESULTS: The authors state that all types of split cord malformation can be reduced to a single derailment during development, with various degrees of severity. CONCLUSIONS: The configuration of the malformation is determined by the way the median parts of the mesoderm come to development. Type I and II split cord malformation are not distinct entities.

The Arnold-Chiari type II malformation at midgestation.

The Arnold-Chiari malformation type II (ACMII) is reported to be reversible after closure of a myelomeningocele at midgestation. To elucidate the developmental state of the ACMII malformation at the approximate time fetal surgery is performed, the ACMII of a 20-week human fetus was investigated in vitro using high-field magnetic resonance microscopy at 9.4 T and compared with the hindbrain of a neurologically intact fetus of the same gestational age. Up to 20 weeks of gestation, the developmental failures caused by the early embryonic herniation of the posterior fossa contents are the dominant feature of fetal ACMII, but after 20 weeks, the accelerated and disproportionate growth of the cerebellum dominates. As midgestational surgery stops the leakage of cerebrospinal fluid, the posterior fossa will expand in time to allow further normal growth of both the cerebellum and brain stem. Some early developmental anomalies already present in the primitive rhombencephalon due to early embryonic hindbrain herniation as well as some intra-axial anomalies are probably not reversible.