Showing papers in "Pediatric Neurosurgery in 2015"

National Trends, Complications, and Hospital Charges in Pediatric Patients with Chiari Malformation Type I Treated with Posterior Fossa Decompression with and without Duraplasty

Abstract: Background: The treatment of type 1 Chiari malformation (CM-1) with posterior fossa decompression without (PFD) or with duraplasty (PFDD) is controversial. The au

Measures of ventricles and evans' index: from neonate to adolescent.

Abstract: Ventricle sizes are important for the early diagnosis of hydrocephalus or for follow-up after ventriculostomy. Diameters of ventricles may change, especially in childhood. This study aims to provide normative data about ventricle diameters. Among 14,854 cranial MRI performed between 2011 and 2013, 2,755 images of Turkish children aged 0-18 years were obtained. After exclusions, 517 images were left. Four radiologists were trained by a pediatric radiologist. Twenty images were assessed by all radiologists for a pilot study to see that there was no interobserver variation. There were 10-22 children in each age group. The maximum width of the third ventricle was 5.54 ± 1.29 mm in males in age group 1 and 4.98 ± 1.08 mm in females in age group 2. The Evans' index was 1 and <2 in all age groups and both gender groups. Our study showed the ventricle size data of children in various age groups from newborn to adolescent. The ventricle volume/cerebral parenchyma ratio seems to decrease with age. We think that these data can be applied in clinical practice, especially for the early diagnosis of hydrocephalus.

Intracranial aneurysms in the youngest patients: characteristics and treatment challenges.

Abstract: Background: Characteristics and treatment challenges of aneurysms in young children differ from those in older pediatric patients. Methods: Records of children undergoing cerebral or spinal angiography by the senior neurointerventionalist (D.B.O.) from October 2006 to January 2014 were reviewed to identify patients with digital subtraction angiography-confirmed intracranial aneurysms. Demographics, presentation, subtype of aneurysm, treatment strategy, and outcome were evaluated. Results: Of 763 pediatric cases, 33 were of children harboring cerebral aneurysms. Overall, 48% were male, 24% presented with hemorrhage and 67% harbored fusiform/dissecting aneurysms. Patients aged 0-10 years were significantly more likely to harbor nonsaccular, dissecting/fusiform aneurysms (84 vs. 43%, p = 0.02). A total of 13/22 pediatric patients harboring dissecting/fusiform aneurysms were male (59%) compared to 3/11 with saccular aneurysms (27%, p = 0.14). Overall, 15 patients underwent endovascular treatment (45%) and 8 underwent microsurgical treatment (24%). There was no significant difference in treatment approach based on patient age or aneurysm morphology. Overall, 19/23 aneurysms were occluded after initial treatment (83%); there were 2 procedure-related complications with neurological sequelae (9%). Conclusion: We demonstrate that the youngest patients (10 years and under) have aneurysms even more distinct and less similar to adult aneurysms than those in the larger pediatric cohort.

Successful endovascular reconstruction of a recurrent giant middle cerebral artery aneurysm with multiple telescoping flow diverters in a pediatric patient.

Abstract: Intracranial aneurysms of the pediatric population are rare, but giant fusiform aneurysms (GFAs) of the middle cerebral artery (MCA) are common within this cohort of patients. These aneurysms are difficult to treat and often require advanced microsurgical skills, as they are usually not amenable to direct clipping. Here, we report the successful treatment of a recurrent GFA of the MCA with three telescoping Pipeline Embolization Devices 6 months after attempted clip reconstruction in a pediatric patient.

Treatment Progress of Paroxysmal Sympathetic Hyperactivity after Acquired Brain Injury

Abstract: Paroxysmal sympathetic hyperactivity (PSH) is a common complication of various acquired brain injuries such as traumatic brain injury, subarachnoid hemorrhage, anoxic brain injury, intracerebral hemorrhage, and others. It is manifested by tachycardia, hypertension, tachypnea, diaphoresis, and dystonic posturing. The development of PSH can prolong hospitalization and lead to secondary brain injury and even death. Despite the awareness of the serious clinical impact, there is no consensus on diagnostic criteria. Thus, misdiagnosis and delayed recognition is very common. Most of the current treatment programs come from case reports and small case series; there are very few large-scale randomized controlled trials. Generally accepted medications are opioids, β-blockers and gabapentin (usually used in combination). However, the efficacy of these drugs has not been systematically assessed. The purpose of this review is to determine the treatment strategies and drugs commonly used for PSH at the overall level.

Transcortical Transventricular Endoscopic Approach and Ommaya Reservoir Placement for Cystic Craniopharyngioma.

Abstract: The treatment of craniopharyngioma is varied. It ranges from radical excision to direct radiotherapy. As the morbidity of excision is high, more conservative approaches are used. Placement of a catheter and reservoir is one such option for cystic craniopharyngiomas. The positioning of catheters has been performed by various means. A method of endoscopic reservoir catheter placement is described. Three children with ages ranging from 5 to 12 years presented with clinical features of raised intracranial pressure. They did not have vision impairment. Imaging showed a predominantly cystic craniopharyngioma extending into the third ventricle with hydrocephalus. All underwent precoronal burr hole, transcortical transventricular endoscopic biopsy of craniopharyngioma, and Ommaya reservoir placement. There were no complications. All children did well after surgery and did not require further cyst aspiration.

Traumatic Pediatric Posterior Fossa Extradural Hematomas: A Tertiary-Care Trauma Center Experience from India.

Abstract: Introduction: Traumatic posterior fossa extradural hematomas (PFEDH) are rare lesions constituting

The Role of Fast Cell Cycle Analysis in Pediatric Brain Tumors

Abstract: Cell cycle analysis by flow cytometry has not been adequately studied in pediatric brain tumors. We investigated the value of a modified rapid (within 6 min) cell cycle analysis protocol for the characterization of malignancy of pediatric brain tumors and for the differentiation of neoplastic from nonneoplastic tissue for possible intraoperative application. We retrospectively studied brain tumor specimens from patients treated at our institute over a 5-year period. All tumor samples were histopathologically verified before flow-cytometric analysis. The histopathological examination of permanent tissue sections was the gold standard. There were 68 brain tumor cases. All tumors had significantly lower G0/G1 and significantly higher S phase and mitosis fractions than normal brain tissue. Furthermore low-grade tumors could be differentiated from high-grade tumors. DNA aneuploidy was detected in 35 tumors. A correlation between S phase fraction and Ki-67 index was found in medulloblastomas and anaplastic ependymomas. Rapid cell cycle analysis by flow cytometry is a promising method for the identification of neoplastic tissue intraoperatively. Low-grade tumors could be differentiated from high-grade tumors. Thus, cell cycle analysis can be a valuable adjunct to the histopathological evaluation of pediatric brain tumors, whereas its intraoperative application warrants further investigation.

Kyphectomy and Pedicular Screw Fixation with Posterior-Only Approach in Pediatric Patients with Myelomeningocele

Abstract: Purpose: Defective posterior spinal arch and paraspinal musculature lead to progressive kyphosis in patients with myelomeningocele. Kyphosis decreases the patients' functional status and quality of life. To correct or prevent further deterioration, different surgical techniques have been introduced. Our aim is to present our clinical experience in kyphectomy and pedicle screw fixation with a posterior-only approach in pediatric patients with myelomeningocele and to discuss the technique with a review of the literature. Materials and Methods: Four patients with lumbar and 2 patients with thoracolumbar kyphosis (female:male ratio = 1:5) secondary to myelomeningocele were operated between January 2009 and October 2012. The median age was 5.5 years (range = 3-10 years). The criteria of the patient selection for the procedure were progression of kyphosis angle, impaired truncal balance and cosmetic deformity. In this retrospective study, we performed chart reviews for demographic and clinical data. We measured the pre- and postoperative kyphosis angles by using the Cobb method on lateral x-rays. Results: The mean preoperative kyphosis angle was 114.3° (range = 91-136°). The mean operative time was 171.7 min (range = 110-220 min). The mean intraoperative blood loss was 450 cc (range = 300-700 cc). The postoperative mean kyphosis angle was 28.2° (range = 13-33°). Five patients had skin breakdown. After osteofusion was established, those 5 patients' instrumentations were explanted. No acute or immediate postoperative complications occurred. Other complications were pneumonia and urinary tract infection. In the long term, 2 patients died due to pneumonia and slit-ventricle syndrome, respectively. Conclusions: Kyphectomy and pedicle screw instrumentation with the posterior-only approach dramatically reduces the kyphosis angle that develops in patients with myelomeningocele. The method itself is less time-consuming and leads to less intraoperative blood loss compared to other methods used for this patient population. Skin breakdown is the most common short-term complication.

Intracranial Injuries from Dog Bites in Children.

Abstract: Background/Aims: Infants are especially at risk for intracranial injuries from dog bites due to their small stature and thin skull. Only 21 case reports have been

Endoscopic Third Ventriculostomy in Children with Myelomeningocele: A Case Series

Abstract: Hydrocephalus develops in up to 80-90% of children with myelomeningocele (MM) after closure of the defect. Traditionally, ventriculoperitoneal shunts have been used to manage hydrocephalus in these patients. A role for endoscopic third ventriculostomy (ETV) in MM has provoked much debate, principally due to anatomical variants described, which may complicate the procedure. We present 7 cases of children with MM and hydrocephalus undergoing a total of 10 ETV procedures. All patients demonstrated clinical improvement (in acute/subacute cases) or stabilization (in chronic cases). Three patients requiring a second ETV have shown clinical stability and renewed radiological evidence of functioning ventriculostomies in follow-up since reintervention. ETV can be used, albeit cautiously, in selected cases of hydrocephalus associated with MM. However, the frequency with which anatomical variation is encountered and the difficulty of the assessment of success make the procedure more challenging than usual.

Symptom Experience and Quality of Life in Children after Sport-Related Head Injuries: A Cross-Sectional Study.

Abstract: Background: Sports are a major cause of concussions, and little is known about the symptom experience and health-related quality of life (HRQL) in children who remain symptomatic for over 3 months following such head injuries. Methods: A cross-sectional study of children aged 10-18 years was performed who were referred to the Neurosurgery Clinic at our centre following a head injury. Symptom experience was measured using the modified Concussion Symptom Scale, and HRQL was measured using the Pediatric Quality of Life Inventory (PedsQL). The Immediate Postconcussion Assessment and Cognitive Test (ImPACT) was administered to assess neurocognitive and neurobehavioural sequelae. Results: Symptoms with the highest mean symptom scores on a Likert scale of 0-6 in 35 children at the time of assessment included headaches (3.1), poor concentration (2.7), memory problems (2.1), fatigue (2.1) and sensitivity to noise (2.0). Compared with normative data, children in this study had ImPACT summary scores between the 28th and 38th percentiles and a comparably low Cognitive Efficiency Index score. Mean scores for females were consistently statistically significantly lower (p Conclusions: Children continue to experience many symptoms at least 3 months following sport-related head injuries that significantly impact their HRQL and neurocognitive abilities.

Ruptured Giant Left Distal Anterior Cerebral Artery Aneurysm in a Two-Month-Old Baby

Abstract: Background: The incidence of intracranial aneurysm in childhood is rare, especially in infancy. In the literature, most of childhood intracranial aneurysms occur in the posterior and middle cerebral circulation. We report a case of a ruptured giant left distal anterior cerebral artery aneurysm in a 2-month-old baby. This report will discuss the rarity of this case as well as the diagnostic and surgical challenges in treating this condition. Case Report: A 2-month-old baby girl presented to our centre with a 1-day history of multiple afebrile seizures. A CT scan of her brain showed a large frontal interhemispheric acute bleed with intraventricular extension and acute hydrocephalus. An external ventricular drain was inserted and she was nursed in the Paediatric Intensive Care Unit. Subsequent CT angiogram and 4-vessel angiogram showed a giant aneurysm originating from the A3 segment of the left anterior cerebral artery. She underwent craniotomy and clipping and excision of the giant aneurysm. Discussion: Giant distal anterior artery aneurysms are very rare in infants. They are more commonly associated with the posterior and middle cerebral arteries. The aneurysms in infants tend to be larger compared to adults and they commonly present with subarachnoid haemorrhage and seizures. CT angiogram and 4-vessel cerebral angiogram are important diagnostic tools in this case. The angiogram demonstrated no distal runoff from the aneurysm and this assisted in our planning for surgical treatment of the aneurysm. A bifrontal craniotomy was done and the giant aneurysm was approached interhemispherically. The proximal and distal parent vessel was clipped and the aneurysm wall was excised. The baby recovered from surgery and did not require any cerebrospinal fluid diversion. Conclusion: Giant distal anterior cerebral artery aneurysms are rare in infants. Detailed angiographic investigation is important. Surgery is the treatment of choice, although there may be a role for endovascular intervention.

Transnasal Transsphenoidal Surgical Method in Pediatric Pituitary Adenomas

Abstract: Aim: To evaluate the clinical outcome in a 13-year consecutive series of children operated for pituitary adenomas with transnasal transsphenoidal surgery.

Rare Pathologies in the Posterior Third Ventricular Region in Children: Case Series and Review

Abstract: Tumors of the pineal region in children often belong to 2 categories, namely germ cell tumors and pineal parenchymal tumors. Very rare pathologies have previously been reported in this region. Most of these tumors may be similar radiologically, while their management differs. The present series reports 2 children with pineal region tumors, each one being a rare pathological entity by itself, namely an embryonal tumor with abundant neuropil and true rosettes (ETANTR) and a rosette-forming glioneuronal tumor (RGNT). Very few such cases in each pathology have been reported in the literature for the pediatric age group up to now. Our series consists of 2 children, both presenting with a raised intracranial pressure of short duration. Imaging revealed lesions in the pineal region with similar radiological features. Both ETANTR and RGNT demonstrated mild enhancement. The 2 patients underwent surgical decompression either by Poppen's approach (n = 1) or a supracerebellar infratentorial approach (n = 1). The patient with ETANTR was advised radiotherapy, while the child with RGNT was advised a regular follow-up. This series presents some rare pathologies which can occur in the posterior third ventricular region with similar radiological features. Management differs based on the histology of the case.

Upward Translation of Cerebellar Tonsils following Surgical Expansion of Supratentorial Cranial Vault: A Unified Biomechanical Explanation of Chiari Type I.

Abstract: Cerebellar tonsils moved significantly upward in 3 patients with Chiari type I who underwent supratentorial cranial vault expansion to alleviate intracranial pressure related to multisutural craniosynostosis. The Chiari type I deformities in these patients were the biomechanical consequence of posterior fossa-cerebellar disproportion caused by supratentorial craniocerebral disproportion secondary to multisutural craniosynostosis. The authors postulate that all cases of Chiari type I deformity share the sine qua non feature of posterior fossa-cerebellar disproportion.

Cervical spine MRI findings in patients with Mucopolysaccharidosis type II.

Abstract: Mucopolysaccharidosis type II (MPS II; Hunter syndrome) is an X-linked, recessive, lysosomal storage disorder caused by deficiency of iduronate-2-sulfatase (EC 3.1.6.13). The purpose of this report is to describe cervical spine magnetic resonance (MRI) findings in MPS II patients and to correlate them with clinical phenotype. Seven cervical spine MRI examinations from Polish MPS II patients (mean age 11.4 years, median age 8 years, range 5-30) were evaluated. Six patients were classified as neurological (85.7%) and 1 as attenuated (14.3%). Five patients were treated with idursulfase (range 110-260 weeks, mean 195, median 200), while 2 patients never received the treatment. The following features were assessed: periodontoid thickening, spinal stenosis, dens hypoplasia, myelopathy, and vertebral and intervertebral disc abnormalities. Mean age at evaluation was 11 years (range 5-30, median 8). Cervical spine MRI was abnormal in all the patients and the most frequent abnormalities found were dens hypoplasia (100%), periodontoid thickening (100%), disc abnormalities (100%) and spinal stenosis (43%). There was no clear correlation between MRI findings and patients' phenotypes.

Interhemispheric Vertical Hemispherotomy: A Single Center Experience

Abstract: Purpose: Hemispheric epileptogenic lesions such as hemimegalencephaly often manifest as intractable epilepsy in early infancy. Hemispherotomy is the treatment of choice for controlling intractable hemispheric epilepsy. Less invasive procedures are desirable for surgery on infants with low body weight. This study compared our experience with interhemispheric vertical hemispherotomy (IVH) and peri-insular lateral hemispherotomy (PIH). Methods: Thirteen consecutive patients underwent hemispherotomy for treatment of intractable epilepsy in our institution between 2001 and 2012. The etiology of epilepsy included hemimegalencephaly in 7 patients and cortical dysplasia in 3. PIH was performed on the first 5 patients and IVH on the last 8 patients. In the latter procedure, complete section of the corpus callosum was first performed via the interhemispheric approach. After removing part of the cingulate gyrus, section of the descending fibers was performed anterolaterally to the thalamus. Clinical characteristics, duration of operation and amount of blood transfusion were compared between the PIH and IVH groups. Results: There was no difference in age at surgery, body weight and age of epilepsy onset between the two groups. No surgery-related death was observed. No patients required shunt operation. One patient who underwent IVH required reoperation for incomplete disconnection. The amount of intraoperative blood transfusion was smaller and the total duration of operation was shorter in the IVH group than in the PIH group. Conclusion: The interhemispheric approach minimizes cortical resection and may be less invasive than PIH. IVH is advantageous for treating infants with low body weight.

Ventriculobiliary Shunts, Another Option.

Abstract: The basic management of hydrocephalus includes shunts to the peritoneum and atrium. However, there are particularly complex patients in whom it is necessary to look for atypical places for implanting the distal catheter. Since 2000, 1,325 shunts have been implanted in pediatric patients. Only 3 patients required a ventriculobiliary shunt. We report 3 cases: a 7-year-old boy with a surgically treated complex heart disease, a 16-month-old girl with hydrocephalus secondary to a brain tumor and multiple bacteremias secondary to an infection of the central venous reservoir, and a 4-year-old girl with nonreabsorptive hydrocephalus caused by intraventricular bleeding due to premature birth, necrotizing enterocolitis and shunt infections with abdominal pseudocysts, which caused multiple abdominal septations and, finally, a nonreabsorptive peritoneum. At present, cases 1 [45 months after ventriculobiliary shunt (VBS)] and 3 (27 months after VBS) are symptom free, while case 2 (14 months after VBS) died of infectious respiratory complications. The gold standard for the treatment of nonreabsorptive hydrocephalus is a ventriculoperitoneal shunt, the second option is a ventriculoatrial shunt, and the third option is uncertain. In our short experience, a ventriculo-gallbladder shunt is a good option when there is no abdominal hypertension.

Dermal Sinus Tract of the Thoracic Spine Presenting with Intramedullary Abscess and Cranial Nerve Deficits

Abstract: Congenital dermal sinus tract of the spine is an unusual developmental defect which represents a failure of the surface ectoderm and dermal elements to separate from the neuroectoderm. A 15-month-old female presented with high fever, severe right hemiparesis, difficulty breathing and cranial nerve deficits. Magnetic resonance imaging (MRI) of the spine revealed a congenital dermal sinus tract at the Th6 level, an intramedullary collection extending up to the brainstem and a probable intramedullary cystic lesion. The child was operated acutely with ligation of the sinus tract, drainage of the abscess and partial removal of the intramedullary lesion. Due to abscess recurrence, she was reoperated with complete excision of the dermal sinus tract, abscess redrainage and subtotal excision of the dermoid cyst (retaining a part of its capsule). Pus culture isolated Corynebacterium species and Peptococcus species and histology of the lesion showed a dermoid cyst. Postoperatively, after an initial neurologic deterioration, she progressively improved. An MRI scan at 15 months neither showed recurrence of the collection nor regrowth of the lesion. Spinal dermal sinus tracts that remain unnoticed or untreated can result in serious complications and should be operated as soon as possible to prevent undesirable sequelae.

Infected Subgaleal Hematoma Following Blunt Head Trauma in a Child: Case Report and Review of the Literature

Abstract: Subgaleal hematoma (SGH), an uncommon but potentially dangerous complication, has been reported to occur with delivery in newborns, as well as in young patients following head trauma. Infection of a SGH is extremely rare, especially in cases where no disruption of the skin barrier occurs. We report a case of an infected SGH in an 8-month-old following closed skull fracture. The patient presented with scalp swelling 1 day after falling 3 feet. Initial evaluation found a nondisplaced skull fracture on computed tomography. She was discharged following an uneventful 23-hour observation. Three days later, she developed symptoms concerning for a viral upper respiratory tract infection and received symptomatic treatment. Nine days after injury, she returned with continued fevers, irritability, and significant increase in scalp swelling. Magnetic resonance imaging showed a subgaleal abscess with osteomyelitis. Needle aspiration revealed an infected hematoma with cultures positive for Streptococcus pneumoniae, treated with intravenous ampicillin. Purulent drainage from an enlarging necrotic needle aspiration site required subsequent surgical debridement of the subgaleal abscess with drain placement. She recovered well following surgery and intravenous antibiotics. Physicians should be aware that SGH carries a risk of serious morbidity and mortality. SGH can serve as a nidus for infection, typically from skin barrier breakdown or, as in this case, hematogenous spread. Early recognition, appropriate antibiotic therapy, and surgical debridement are critical in treating infected SGH.

Complete response and long-term survival (>20 years) of a child with tectal glioma: a case report.

Abstract: Tectal glioma is a midbrain tumor. The patient generally presents with symptoms related to increased intracranial pressure and requires treatment for hydrocephalus. No effective pharmacological treatments have yet been introduced. This report discusses a case of a 13-year-old male diagnosed with tectal glioma who obtained a complete response and long-term survival after the treatment with antineoplastons (ANP) in phase II trial. Prior treatment consisted of placement of a ventriculoperitoneal shunt. After 6 years of stabilization there had been an increase in tumor size with signs of malignant transformation. The patient received treatment with ANP A10 and AS2-1 infusions for 20 months, obtained a complete response, and was switched to maintenance with ANP capsules. All treatments were discontinued in December 2003. Adverse events according to CTCAE v3.0 included: hypernatremia (two events of grade 3, one event of grade 2, four events of grade 1), one case of fatigue (grade 2), and one allergic reaction (grade 1). Currently, over 20 years from his diagnosis and over 13 years from treatment start he is symptom-free and leads a normal life. This report indicates that it is possible to obtain long-term survival of a child with tectal glioma with currently available investigational treatment.

Evolution of a Pediatric Primary Cerebral ALK-1-Positive Anaplastic Large Cell Lymphoma on Serial MRI.

Abstract: Background: Primary central nervous system lymphoma (PCNSL) is a rare central nervous system tumor, especially in the pediatric population. There are fewer than 20 described cases of pediatric primary central nervous system anaplastic large cell lymphoma. The child described in our case report demonstrated a dramatic evolution of this tumor in the first 4 weeks on serial imaging. Methods: Serial MRI imaging was performed followed by biopsy and chemotherapy. Results: Initial imaging revealed a T2 hyperintense lesion in the frontal lobe with abnormally enhancing sulci and minimal surrounding edema and diffusion restriction. Serial imaging revealed progressive increase in the degree of gadolinium enhancement, and the hyperintense T2 edema progressed markedly to exert mass effect. The lesion itself grew marginally. Biopsy revealed an anaplastic large cell lymphoma, only described in 14 previous pediatric patient case reports. The patient was successfully treated with chemotherapy and autologous stem cell transplant. Conclusions: Our case demonstrates the rapidity with which a PCNSL lesion can develop, and the evolution of the imaging characteristics prior to definitive diagnosis and treatment. Serial imaging by MRI may help differentiate the behavior of a PCNSL from other imitating lesions.

The Filum Terminale Revisited: A Histological Study in Human Fetuses.

Abstract: Previous studies have suggested that secondary neurulation provides no functional neurons but only the filum terminale. However, no studies have confirmed that the coccygeal and lower sacral nerves do not originate from the secondary neural tube but only from the primary tube. To obtain a better understanding of these relationships, we examined sagittal or frontal sections from 20 embryonic and fetal specimens ranging from 6 to 14 weeks of gestation. During the growth of the vertebral column as well as the subsequent upward migration of the caudal end of the dural sac, the secondary neural tube was stretched to maintain the original attachment to the coccyx or the lower sacral vertebra. The filum-like structure showed much individual variability but in all cases appeared to be derived from the stretched neural tube. Intermediate age morphology revealed that the secondary neural tube itself provided an initial filum terminale before the ascent of the dural sac. Given that the coccygeal and lower sacral nerves are likely to originate from the secondary neural tube, these parts of the tube persisted and differentiated into spinal neurons to form the anococcygeal nerves. Likewise, the filum terminale was also most likely to contain some neurons that persisted postnatally. Depending on the timing and site of degeneration of the secondary neural tube, individual variations could occur in proportion to the amount of sensory and motor elements in the anococcygeal nerve supply.

The Chiari 3 Malformation and a Systemic Review of the Literature.

Abstract: Introduction: Chiari type 3 is a rare hindbrain malformation that has been reported in the literature primarily as case reports and case series. Radiological, pathophysiological and surgical definitions of the malformation are inconsistent in the literature and subsequently can be confusing, and outcomes have also been uniformly poor. The definition of this rare malformation will be clarified through a case presentation. Methods: A retrospective review of prior publications in the PubMed and MEDLINE databases was performed looking for reports of ‘Chiari 3 +/- malformation' and ‘occipital encephalocele'. Relevant papers were reviewed and compiled into table format with associated descriptions of a Chiari type 3 malformation. A case illustration is presented with radiological and intraoperative imaging to reinforce and clarify the definition. Results: Upon review of the prior publications in the detail of the descriptions and imaging associated with each article, there is a wide range of variability in the description of what is considered a Chiari 3 malformation. Occipital, occipitocervical and high cervical defects have all been described as Chiari 3 malformation. Our case illustration presents a patient with an occipitocervical encephalocele with neural elements, which is the classic and accepted definition of the Chiari 3 malformation. Conclusion: Chiari type 3 is a rare congenital malformation, and prior publications describing this developmental disorder have not demonstrated a consensus in its definition. In addition, outcomes have traditionally been reported as poor. This case illustration of a Chiari type 3 enforces the definition of an occipitocervical encephalocele with hindbrain herniation, and with proper management not all Chiari 3 malformation patients have bad outcomes.

A Novel Bilateral Approach for Suprasellar Arachnoid Cysts: A Case Report.

Abstract: The endoscopic method is used to treat suprasellar arachnoid cysts (SACs) but it is sometimes difficult to make sufficiently sized fenestrations. Creating a larger fenestration on the cyst wall is preferable to prevent closure of the stoma. In this paper, we report a novel endoscopic approach for SAC treatment in which we use bilateral burr holes to achieve a more extensive cyst fenestration. A 7-year-old girl was referred to our hospital because of incidentally detected hydrocephalus by computed tomography scans. Physical examination did not show any signs of intracranial hypertension, but a digital impression of her skull on X-ray implied chronic intracranial hypertension. Magnetic resonance imaging (MRI) revealed enlargement of both lateral ventricles and a cystic mass occupying the third ventricle. We performed cyst wall fenestration using a bilateral approach in which we created two burr holes to introduce a flexible endoscope and a rigid endoscope. The cyst wall was held by forceps with the flexible endoscope, and resection of the cyst wall was achieved by using a pair of scissors with the rigid endoscope. There were no postoperative complications, and MRI performed 1 year after treatment showed disappearance of the superior part of the cyst wall.

An Acute Complication of Ventriculoperitoneal Shunt with Bladder Perforation and Extrusion through the Urethra in a Newborn: Case Report and Review of the Literature

Abstract: Ventriculoperitoneal shunt (VPS) surgery is a standard procedure for the management of hydrocephalus. Bladder perforation is an extremely rare complication of VPS surgery. Herein, we present for the first time an acute complication of VPS with bladder perforation and extrusion through the urethra in a newborn. We also reviewed the complication of VPS with bladder perforation as reported in the literature.

Neural Tube Defects in Jarcho-Levin Syndrome: Study of Twenty-Eight Cases

Abstract: Jarcho-Levin syndrome (JLS) is a congenital disorder consisting of various vertebral and costal anomalies. Congenital heart defects, abdominal wall malformations, urogenital and anal abnormalities, multiple skeletal anomalies, upper limb anomalies, spina bifida, and inguinal, umbilical and diaphragmatic hernias can be seen as components of JLS. Spina bifida appears to be a common finding in reported JLS cases. We retrospectively reviewed the medical records, plain X-rays and MRIs of patients with spina bifida between 2010 and 2014 and discussed the results.

Clinical Significance of Prenatal and Postnatal Heavily T2-Weighted Magnetic Resonance Images in Patients with Myelomeningocele

Abstract: Purpose: To compare the utility and limitations of prenatal magnetic resonance (MR) imaging using half-Fourier acquisition single-shot turbo spin-echo (HASTE) with postnatal heavily T2-weighted imaging (hT2WI) for the evaluation of central nervous system abnormalities associated with myelomeningocele (MMC). Methods: Sixteen patients with MMC who had undergone pre- and postnatal MR imaging were included in this study. MR imaging, including HASTE, was undertaken in the 3rd trimester, and hT2WI was performed immediately after delivery. The precision with which each could distinguish MMC, hindbrain herniation and ventriculomegaly was compared retrospectively. Results: The skin defects and MMC sacs were clearly visible on prenatal HASTE images, although it was difficult to identify precisely the level of MMC compared with postnatal hT2WI, in which the detailed anatomical relationships of the spinal cord, neural placode and ventral nerve roots were evident in every case. Hindbrain herniation could be visualized on prenatal HASTE images, although its severity was difficult to evaluate because of the small size of the structures and neck flexion; again, the resolution was superior on postnatal hT2WI. For hydrocephalus, there were no significant differences in the diagnostic precision and ability to grade the severity between pre- and postnatal imaging. Conclusion: Prenatal HASTE imaging permits the diagnosis and understanding of the gross anatomy of MMC and associated hindbrain herniation and ventriculomegaly, but postnatal hT2WI is superior for evaluating detailed anatomy.

Spontaneous Extrusion of Ventriculoperitoneal Shunt Catheter through the Right Lumbar Region: A Case Report and Review of the Literature.

Abstract: Ventriculoperitoneal shunt surgery is the most frequently performed procedure in the management of hydrocephalus. Many varied complications related to this procedure have been reported. Distal migration of the peritoneal catheter and extrusion from the intact skin in an area unrelated to the surgical incision constitute a rare complication. We report a 1-year-old patient with the extrusion of the peritoneal catheter from the intact skin in the right lumbar region and present a literature review.