Currarino syndrome: report of five consecutive patients.

INTRODUCTION: The Currarino syndrome is regarded as a developmental disorder based on its recognized etiological heterogeneity. This syndrome is thought to result from abnormal separation of the neuroectoderm from the endoderm. Our aim was to report the neurosurgical management of Currarino syndrome in children and adults and to describe what clinician could do if the Currarino triad was suspected. CASE REPORTS: We present five cases of Currarino triad who underwent surgical intervention. All patients had sacral bony deformity, anorectal malformations, and anterior sacral meningocele. A 40-year-old-male had chronic constipation. He was incidentally diagnosed with Currarino syndrome. A 19-year-old-female suffered from a slight weakness in lower extremities and urinary incontinence. Her past medical history was remarkable for anal atresia. The other three cases were children. CONCLUSION: When an anterior sacral meningocele is encountered, Currarino syndrome should be taken into consideration. Although it is rarity, the Currarino syndrome might be one of the causes of chronic constipation. Endoscopic or endoscope-assisted surgery via a posterior sacral route can be feasible for treatment of some of the patients with anterior sacral meningocele. Anterior meningocele pouch associated with Currarino syndrome will regresses over time following transdural ligation of its neck.

Endoscope-assisted repair of metopic synostosis.

PURPOSE: Endoscopic treatment of nonsyndromic craniosynostosis has been used in recent decades. The aim of this study is to present the results of endoscope-assisted surgery of the patients with metopic synostosis. METHODS: Nineteen patients with metopic synostosis underwent endoscope-assisted surgery between 2005 and 2012. The frontal angle was measured on CT scans pre- and postoperatively. A midline strip craniectomy was made, making sure that the removal extended inferiorly to the nasofrontal suture. Postoperatively, all patients wear a custom molding helmet. The helmet was then worn for 23 h daily and worn for the next 10-12 months. RESULTS: There were 15 boys and 4 girls, ranging in age from 4 to 24 weeks. Mean operative time and transfused blood volume were 43.42 min and 76.31 ml, respectively. Mean follow-up was 59.3 weeks. Good esthetic results, judged by radiological examination and photograph comparison, were obtained in all patients. CONCLUSIONS: Endoscope-assisted repair of metopic synostosis is safe and offers promising results in infants before the age of 6 months. Molding helmet therapy following surgery is very important in obtaining the good results. Early diagnosis and referral for surgery are crucial.

Split cord malformation types I and II: a personal series of 131 patients.

PURPOSE: A personal series of 131 patients with split cord malformation (SCM) operated on is presented. METHODS: Age, gender, symptoms and signs, radiological and operative findings, complications, associated anomalies, outcome, and pathological specimens were analyzed. RESULTS: There were 88 girls (73 %) and 43 boys (27 %). The female predominance was slightly more remarkable in type I SCMs than in type II SCMs. The presenting symptoms can be summarized as skin lesions, spina bifida aperta, scoliosis or kyphoscoliosis, sphincter disturbance, foot deformities and weakness, and/or atrophy in the lower extremities. The ages of patients with neurological deficits and orthopedic deformities were significantly older than those without deficits (P = 0.030). The duration of symptoms was longer in the patients with neurological deficits and orthopedic deformities than that in those without deficits (P = 0.00095). In six patients, composite SCMs were present. Only one patient with a type II SCM did not have an associated spinal cord lesion. A type I SCM was more frequently encountered in patients with spina bifida (P < 0.0005). Transient postoperative complications were seen in 29 patients (22 %). There was no permanent complication. Retethered cord syndrome developed in five patients with a type I SCM. CONCLUSIONS: The risk of neurological and orthopedic deficits increases with the age of the patient. The risk of permanent deficit after surgery is very low. The whole spine must be examined for additional lesions. All patients should be surgically treated when diagnosed, especially before the development of orthopedic and neurological manifestations, and all associated lesions should also be treated at the same session.

Safety and diagnostic accuracy of neuroendoscopic biopsies: an international multicenter study.

OBJECT: Analysis of the safety and morbidity of neuroendoscopic biopsies (NEBs), as well as the reliability in obtaining an accurate diagnosis, has until now been based on studies with relatively small sample sizes. Through the cooperative efforts of several international medical centers, authors of the present study collected data on a large number of patients to obtain better insight into this issue. When possible, they compared pathology obtained through an NEB with the "gold-standard" pathology obtained in open surgery. METHODS: Thirteen randomly chosen medical centers in 9 countries collected data for patients who had undergone NEB, which were then analyzed for universal complications, bleeding, navigation technique, pathology, mismatch between biopsy results and final diagnosis, and a number of other potentially influential factors. RESULTS: Data for 293 patients were analyzed. Sixty percent of the patients were male, and patient ages ranged from 0.1 to 78.7 years (median age 20.4 years). The most common tumor locations were pineal (33.1%), thalamic (16.7%), tectal (13%), and hypothalamic (4.4%). Fifty percent of the tumors were larger than 20 mm, 36% were between 10 and 20 mm, and 14% were smaller than 10 mm. Intraoperative bleeding was seen in 275 patients (94%). The amount of blood was noted as mild in 75%, moderate in 13%, and severe in 6%. Infection occurred in 8 patients (3%). Death occurred in 1 patient (0.3%), which was caused by severe intraoperative bleeding. Biopsies were informative in 265 patients (90.4%). Seventy-eight patients (26.6%) had open surgery following the NEB. For these patients, the pathology results from the NEB were compared with those from the open surgery that followed. In 14 cases (17.9%) there was disagreement on the pathology. Of these cases, a meaningful mismatch, in which the erroneous NEB pathology could have led to an inappropriate management decision, occurred in 9 cases (11.5%). Most of these meaningful mismatches were lesions diagnosed as low-grade or pilocytic astrocytoma on the NEB and later proved to be high-grade astrocytoma (4 cases) and 1 case each of meningioma, cavernoma, primitive neuroectodermal tumor, neurocysticercosis, and pineocytoma. CONCLUSIONS: In experienced hands, NEBs can be performed with low morbidity and mortality, providing meaningful pathological data for the majority of patients with a wide range of tumor types, locations, and presentations. These biopsies also offer other advantages, such as the ability to perform concomitant endoscopic third ventriculostomy and septum pellucidotomy. However, due caution must be maintained, since pathology obtained from an NEB, as with stereotactic biopsies, may be subject to sampling errors, especially when the results seem to indicate a low-grade glial tumor.

Neurotoxic effect of povidone-iodine on the rat spine using a laminectomy-durotomy model.

OBJECTIVE: The efficacy and safety of povidone-iodine in wound dressing and irrigation of some operative cavities were established by many in vitro and in vivo experimental reports and clinical series. However, its use in defective tissue in neural structures has not been confirmed yet. The aim of the present study was to histopathologically investigate its effect on neural tissues when applied on the upper side of defective dura. METHODS: Wistar rats were randomly divided into two experimental groups: control and povidone-iodine groups. In the control group, durotomy was performed following laminectomy, and the spinal cord was covered with a dry sponge. In the study group, the same procedure was performed, but open duras were covered with a sponge that had been wetted with 0.1 % povidone-iodine solution. Three weeks after surgery, all experimental animals were sacrificed, and histopathological evaluations were conducted. RESULTS: Myelin changes were absent or minimal in all cases of the control group but were present as markedly increased myelin degeneration in nearly all cases in the study group. Axonal degeneration and hypoxic neuronal damage were absent in the control group, whereas they were marked in half of the study group. No statistically significant differences were established in Schwann cell proliferation, venous congestion, and lymphocytic proliferation between the two groups. CONCLUSIONS: Based on the present study, 0.1 % povidone-iodine solution cannot be recommended for wound dressing for neural structures such as myelomeningocele cases because of possible damage to underlying neural tissues.

Fenestration methods for Sylvian arachnoid cysts--endoscopy or microsurgery.

INTRODUCTION: Two different approaches to fenestration, endoscopic and microsurgical, are in use for Sylvian arachnoid cysts (SACs), the most frequent among intracranial arachnoid cysts. We presented the clinical data and compared our results, with regard to technique and clinical success, with either microsurgical or neuroendoscopic fenestration of SACs. MATERIALS AND METHODS: Twenty-nine patients who subjected to cysto-cisternostomy by the same team, using either of the two methods, were studied retrospectively. RESULTS: Thirteen patients underwent microsurgery, and 16 had endoscopic cysto-cisternostomy. No reoperation was needed in either of these groups, i.e., full clinical and radiological success was achieved in both. The complication rate was 23% in the microsurgery patients and 47% in the endoscopic surgery group, with no statistically significant difference. CONCLUSIONS: No relevant differences between the two methods are seen either in the published data or in our results. Adding our results to the published data, both techniques were reviewed in a discussion of the one that allows a better controlled or safer fenestration. We conclude that endoscopic fenestration of SACs is not superior to microsurgical cyst fenestration, and the latter seems safer.

Neuroendoscopic surgery in empty ventricular system under continuous gas infusion experimental study of pressure changes and complications.

INTRODUCTION: The most important limitations to endoscopic procedures in the ventricular system of the brain are due to the constraint of working inside a fluid. The evacuation of cerebrospinal fluid (CSF) from the ventricles is performed often in microsurgical interventions using a surgical microscope. This study aimed at studying the evacuation of CSF during neuroendoscopic surgery in animals while infusing gas to avoid ventricular collapse. MATERIALS AND METHODS: Hydrocephalus was provoked in five adult New Zealand rabbits by intracisternal injection of kaolin. Endoscopic intervention was performed later; fluid was given as a continuous infusion at constant speed into the CSF for 3 min. In the next stage, CSF was evacuated from the ventricles, which were infused with gas at a stable rate for the same amount of time. The intracranial pressure (ICP) of the rabbits was recorded during both operations. The animals were sacrificed and the brain subjected to pathology examination at the end of the experiment. RESULTS: Mean ICP value in the rabbit ventricle was 19.1 while working in CSF and 17.6 when working in air. The difference by a paired test was statistically significant for each individual rabbit except one. The ICP measurement, however, was never lower than the ambient pressure, even while working in continuous gas infusion. No epidural or subdural hematomas were found at autopsy. CONCLUSIONS: Endoscopic surgery is feasible in a ventricular system that has been insufflated with gas after CSF has been evacuated. During the experiment, however, steadily diminishing ICP values were measured. As a result, new devices, such as small-flow insufflators able to perform sensitive pressure adjustments are needed.

Indirect bypass procedures for moyamoya disease in pediatric patients.

AIM: Many direct and indirect surgical intervention methods have been defined for the treatment of moyamoya disease. Indirect surgical procedures have been increasingly used. In this study, indirect surgical intervention methods especially used in combination with pial synangiosis were assessed together with other indirect methods. MATERIAL AND METHODS: 11 patients who were treated with an indirect bypass procedures at our clinic and followed for at least three years were retrospectively examined. 19 surgical interventions were performed for 18 patient hemispheres pertaining to these patients. 4 indirect surgical revascularization methods were used for three patient hemispheres. On the other hand, indirect procedures combined with pial synangiosis were applied in 11 operations. In addition, the "multiple burr-hole surgery" method was used in 4 four operations performed for two patients. RESULTS: The clinical success rate was 66.6% for patients where the techniques were applied with pial synangiosis. No new ischemic or hemorrhagic attack was observed during the follow-up period in any of these patients in this series. CONCLUSION: Pial synangiosis is a modification that Increases the success rate of indirect surgical methods. In addition, high success rates have been reported in recent publications related to multiple burr-hole surgery. This method is a candidate choice of surgical intervention for selected patients.

Cerebro-spinal fluid shunt revisions, importance of the symptoms and shunt structure.

AIM: CSF shunt failure is still a frequent problem in children. This prospective study was designed for focusing symptoms and reasons of shunt failure. We also especially focused on the mechanical reasons of shunt failure. MATERIAL AND METHODS: We focused on the causes of shunt failures, and the symptoms and signs in patients who were operated for shunt malfunction between January 1, 2001 and December 31, 2005 in the neurosurgery department. All examination and operative data were collected prospectively. Evaluation of these data was with the chi-square and Fisher exact tests. RESULTS: After the evaluation of data on 111 patients and 153 revision procedures, the major symptoms in this group were vomiting (62.16%), somnolence (59.45%) and headache (48.64%). In the majority of the shunt revisions (115 operations, 75.2% of the all 153 procedures), one or more mechanical problems of the shunt systems were identified in surgery. CONCLUSION: Shunt failures in children sometimes appear with very unusual symptoms. Also, probable structural problems of the shunt systems seem very important for shunt failure according to patient characteristics and etiology of the hydrocephalus. A systematic approach including CT, shunt series and abdominal ultrasound is needed to rule out shunt malfunction.

Near-infrared camera for intraventricular neuroendoscopic procedures: in vitro comparison of the efficiency of near-infrared camera and visual light camera during bleeding.

PURPOSE: Bleeding is the most important problem in neuroendoscopic procedures. The visibility of the bleeding point for a long period of time is very crucial for the surgeon to stop the bleeding. In this study, the performances of a near-infrared camera (NIRC) and a visible light camera (VLC), which is widely used today, were compared in terms of the visibility duration of the bleeding point. METHODS: A bleeding point was generated in vitro, and it was monitored with VLC and NIRC (sensitive to 850-nm infrared light), which were connected to two identical telescope systems. This trial was repeated for 40 times using different telescope systems (Clarus neurochannel endoscopy and Storz Hopkins). The images were merged and analyzed digitally. RESULTS: Statistically, sharper brightness difference levels between bleeding point and background are achieved by NIRC than VLC. Analyses revealed that the bleeding point could be observed for a longer time with NIRC, when compared to VLC. CONCLUSION: NIRCs may provide very significant advantages against bleedings encountered during intraventricular operations.

Intraventricular migration of the bone dust. Is a second operation for removal necessary? Case report and review of the literature.

PURPOSE: As the number of endoscopic third ventriculostomy (E3V) operations increase, new rare complications are encountered. In this article, a complication caused by bone particles that migrated into the third ventricle will be described. Additionally, the methods of avoidance as well as the necessity of a new approach will be discussed. METHODS: After the video images of the first and second operations of a patient who was subjected to E3V twice were compared, it was discovered that one of the bone particles within the ventricle had occluded the ostium after the second operation. Most of the bones were removed and their pathological investigations were performed. RESULTS: Video images of the patient, surgical observations of the second operation, emergence of the time of dysfunction, and other similar cases in the literature were assessed, and it was concluded that the bones that localized intraventricularly were living tissues. DISCUSSION: Abandoning usage of bone dust for sealing burr holes is a solution to avoid this complication. In addition, it should be kept in mind that intraventricular bone particles might grow and lead to obstructions. If such particles are detected, removal of the bones in certain locations before formation of neovascularization can be an option.

Can using a peel-away sheath in shunt implantation prevent ventricular catheter obstruction?

PURPOSE: Shunt obstruction is the most common shunt complication. In 2003, Kehler et al. used peel-away sheath while implanting the ventricular catheter in 20 patients. They found less revision rate in the peel-away sheath group. We aimed to test the efficacy of this technique in cadavers. METHODS: We used 100 fresh brains obtained from medicolegal autopsies. Posterior parietal and frontal approaches were used to puncture the lateral ventricle in each cerebral hemisphere. The ventricle is punctured with a peel-away sheath system. After the ventricle is reached, the mandarin is retracted and the ventricular catheter is introduced through the opening. The ventricular catheter was removed from the ventricle, the opening at the tip of the ventricular catheter was checked out for obstruction, and the number of patent and plugged openings was recorded. This procedure was repeated four times for each location with and without using peel-away sheath. The control group consisted of the procedures done without using peel-away sheath. RESULTS: The number of the plugged openings in the peel-away sheath group was significantly smaller than the control group. There was no significant difference between the two groups in terms of gender and left and right cerebral hemispheres. The obstruction rate was significantly lower in the posterior parietal approach. Pearson's correlation showed that increasing age was associated with less obstruction rate. CONCLUSION: Peel-away sheath decreases the number of plugged openings of the ventricular catheter. A clinical cooperative study is needed to prove that a peel-away sheath should be included in the ventricular shunt systems in the market.

Establishment of interdisciplinary child protection teams in Turkey 2002-2006: identifying the strongest link can make a difference!

OBJECTIVES: The University of Iowa Child Protection Program collaborated with Turkish professionals to develop a training program on child abuse and neglect during 2002-2006 with the goals of increasing professional awareness and number of multidisciplinary teams (MDT), regional collaborations, and assessed cases. This paper summarizes the 5-year outcome. METHODS: A team of instructors evaluated needs and held training activities in Turkey annually, and provided consultation when needed. Descriptive analysis was done via Excel and SPSS software. RESULTS: Eighteen training activities were held with 3,570 attendees. Over the study period, the number of MDTs increased from 4 to 14. The MDTs got involved in organizing training activities in their institutions and communities. The number of medical curriculum lectures taught by MDTs to medical students/residents, conferences organized by the MDTs, and lectures to non-medical professional audiences increased significantly (R(2)=91.4%, 83.8%, and 69.2%, respectively). The number of abuse cases assessed by the MDTs increased by five times compared to pre-training period. CONCLUSIONS: A culturally competent training program had a positive impact on professional attitudes and behaviors toward recognition and management of child abuse and neglect in Turkey. The need to partner with policy makers to revise current law in favor of a greater human services orientation became clear. PRACTICE IMPLICATIONS: Pioneers in developing countries may benefit from collaborating with culturally competent instructors from countries with more developed child protection systems to develop training programs so that professional development can improve recognition and management of child abuse and neglect.

Atypical teratoid/rhabdoid tumor of the central nervous system: clinicopathologic and immunohistochemical features of four cases.

BACKGROUND: Atypical teratoid/rhabdoid tumor (AT/RT) is a rare aggressive infantile neoplasm of uncertain origin. This study was performed to assess the clinicopathologic and immunohistochemical features of four AT/RT cases. CASE REPORTS: Two cases were male and two were female, and their ages ranged from 8 to 103 months. Tumors were located in the cerebellum (two cases), frontoparietal lobe (one case), and third ventricle (one case). Histopathologically, the tumors were composed of rhabdoid cells and undifferentiated small cells mixed with epithelial or mesenchymal components. However, one of the tumors was composed predominantly of a mesenchymal component mimicking a sarcoma. Immunohistochemically, vimentin (4/4), epithelial membrane antigen (4/4), cytokeratin (3/4), smooth muscle actin (4/4), glial fibrillary acidic protein (4/4), S-100 (4/4), and synaptophysin (1/4) were positive in varying proportions, while desmin and INI-1 were negative in all the cases. All of the patients died within a mean of 14 months due to tumor progression despite the chemotherapy. Only one of our patients lived for 40 months after the diagnosis. In conclusion, AT/RTs are aggressive tumors. They can occur in a variety of locations, such as the third ventricle. Morphologically, a large spectrum can be seen, like predominantly sarcoma in appearance, but immunohistochemistry is helpful in the correct diagnosis.

Medulloblastoma: clinicopathologic evaluation of 42 pediatric cases.

PURPOSE: The aim of this study was to assess the prognostic value of MIB-1 and p53 in the pediatric medulloblastoma group. MATERIALS AND METHOD: Forty-two pediatric medulloblastoma cases diagnosed in a single institution during the past 10 years were evaluated. Follow-up data were available for 35 patients. RESULTS: The immunoreactivity of MIB-1 ranged from 10% to 95%; p53 immunoreactivity was found in five cases. Of the 35 patients with follow-up, 34 patients received a combination of chemotherapy and radiotherapy, while one received chemotherapy alone. The follow-up period ranged from 5 to 64 months. Of the 35 patients, 21 were alive without any evidence of recurrent disease, three were alive with evidence of recurrent disease and 11 died of disease during follow-up. The mean survival for these 11 patients was 21.9+/-10.4 months. Of the 35 cases, 16 had MIB-1 value of 25% or lower and 19 had a value of 26% or more. Of the 16 cases with low MIB-1 value, six died of disease; of the 19 cases with high MIB-1 value, five died of disease. The statistical difference between MIB-1 and prognosis was not significant. Three of the 35 (8.5%) cases were found to be positive with p53 protein; no correlation was observed between p53 immunoreactivity and prognosis. CONCLUSION: It appears that the MIB-1 value and p53 immunoreactivity have no relation with prognosis in pediatric medulloblastomas. However, it is convenient to support these findings with large series.

Endoscopic management of quadrigeminal arachnoid cysts.

OBJECTIVE: Quadrigeminal cistern arachnoid cysts are rare lesions, accounting for 5% to 10% of all intracranial arachnoid cysts and 9% of all supratentorial localizations. We reviewed the patients with quadrigeminal arachnoid cyst (QAC) who were treated with neuroendoscopic intervention. MATERIALS AND METHODS: Seventeen patients with QAC had been operated on between 2000 and 2007 in our institution. Four patients had undergone shunting prior to neuroendoscopic surgery. There were nine girls and seven boys with age ranging from 7 days to 17 years (mean, 40 months). All patients had hydrocephalus. A wide ventriculocystostomy (VC) and endoscopic third ventriculostomy were performed by using rigid neuroendoscopes. An aqueductal stent was also placed in two of the patients. Psychometric evaluation was administered postoperatively when possible. Follow-up of the patients ranged from 6 to 96 months (mean, 51.8 months). RESULTS: Of the 17 patients, 12 underwent endoscopic procedure as the primary surgery. Five patients had been previously shunted. Macrocrania and psychomotor retardation were the main symptom and sign in all infants with QAC. Older children presented with the symptoms and signs of intracranial hypertension. Of the eight patients who were 6 months old or younger, only one did not need a ventriculo-peritoneal (VP) shunt. Endoscopic procedures were successful in all patients older than 6 months of age (P=0.005). CONCLUSION: The patients presenting in their infancy had a psychomotor retardation, and all patients except for one, younger than 6 months of age, needed a VP shunt. Neuroendoscopic procedure is effective particularly in the patients with QAC older than 6 months of age.

Melanotic progonoma of the skull in infancy.

INTRODUCTION: Melanotic progonoma or melanotic neuroectodermal tumor is a rare tumor in infancy. This lesion has to be considered in the differential diagnosis of benign or malignant lesions of calvarium. CASE REPORT: The authors present a case of a 4-month-old infant with left retroauricular mass. The patient had a subcutaneous mass that is fixed to the underlying skull. CT and MRI scans showed left occipitotemporal expansile mass. The tumor was removed by surgery. A tumor, brownish-black in color, was diagnosed as melanotic progonoma. The patient remained symptom-free for the last 2 years after complete surgery. DISCUSSION: Extracranial subcutaneous masses involving the skull are uncommon in infants. Benign or malignant lesions may occur as lumps on calvarium. Physical examination and some laboratory findings are helpful in the assessment of patient. Benign or malignant lesions can be differentiated by craniography, CT, or MRI scans, but exact diagnosis of melanotic progonoma is made by histopathology and immunostaining, as was in the presented case. Cranial vault progonomas have a better outcome by complete surgery. The tumors usually do not recur in long-term period.