Farideh Nejat

Initial Management of Childhood Brain Tumors: Neurosurgical Considerations

Intracranial tumors are the most common solid tumors in children. The infratentorial compartment will be the primary site for 60% to 70% of these tumors, including astrocytomas, medulloblastomas, and ependymomas. Several technological advancements have increased our knowledge of the cell biology of pediatric brain tumors, facilitated earlier diagnosis, and improved neurosurgical resections while minimizing neurological deficits. These in turn have not only improved the survival of children with brain tumors but also their quality of life. Current management strategies in most cases rely on surgery coupled with adjuvant therapies, including radiation therapy and chemotherapy. The vulnerability of the immature brain to adjuvant therapies creates many challenges for the treating physician. We review current diagnostic and therapeutic approaches and outcome for children harboring the most common pediatric brain tumors: astrocytomas (low-grade and high-grade glioma), ependymoma, medulloblastoma, and craniopharyngioma. The emphasis will be on the neurosurgical management of children with these tumors.

Nonneural congenital abnormalities concurring with myelomeningocele: report of 17 cases and review of current theories.

Objective: Meningomyelocele (MMC) is a common central nervous system birth defect. Various congenital and acquired abnormalities have been reported with MMC, some of which are secondary to the pathophysiology and some are morbidities of the underlying disease. The aim of this study was to discuss current possible theories explaining diverse anomalies/abnormalities seen in a series of 390 patients with MMC. Methods: A retrospective study was performed using the records of 390 patients with MMC at Children’s Hospital Medical Center in Tehran, Iran, from January 2001 to January 2007. A series of 17 cases of MMC with attributed organ anomalies, not explained by a causal effect of the underlying disorder, were compiled. There were 3 cardiac anomalies including ventricular septal defect, pulmonary artery atresia and tetralogy of Fallot, 4 musculoskeletal malformations, consisting of missing rib, polydactylia and complex distal limb anomaly, 4 urological anomalies such as bladder exstrophy, horseshoe kidney and dysplastic kidneys, 2 occipital encephaloceles, 2 congenital adrenal hyperplasia patients with ambiguous genitalia, 1 omphalocele, 1 albinism and 1 Klippel-Feil syndrome. A review of the literature and discussion explaining each of these observations, have been performed and some possible theories have been proposed.Conclusions: Although various organ anomalies with different embryological origin had been observed and reported with MMC, it is difficult to explain their development using one of the current theories of MMC formation. It could be attributed to a possible genetic defect or merely an incidental finding. A teratological insult during the embryogenic phase would be an alternative assumption.

Recurrent meningitis caused by cervico-medullary abscess, a rare presentation.

BackgroundRecurrent bacterial meningitis is usually a complication of cranial anatomical defect or the result of impaired humoral immunity, notably, defects of the complement system and agammaglobulinemia. It could present a diverse range of symptoms.DiscussionMeningitis as a presentation of intramedullary abscess is not common. Here we present a 7-year-old boy with recurrent meningitis as the first manifestation of an underlying upper cervical cord abscess.

Cystic choroid plexus papilloma with multiple mural nodules in an infant

BackgroundA case of a 13-month-old male infant with progressive increase in head circumference, neurodevelopmental delay, and episodic generalized seizures is reported. He had a history of increase in intracranial pressure because of an arachnoid cyst at the age of 2xa0months which was managed by cystoperitoneal shunting, but the symptoms were still progressive.DiscussionMagnetic resonance imaging revealed an intraventricular cystic tumor with intensely enhancing mural nodules. He underwent surgery and the cyst with the nodules was totally excised. Histopathological findings confirmed the diagnosis of cystic choroid plexus papilloma.ConclusionWe recommend precise evaluation of intracranial cystic lesions to prevent wrong diagnosis and operation. Total resection of all enhancing nodules in cystic choroid plexus papilloma is proposed to warrant total cure of tumor.

Parasitic rachipagus twins; report of two cases

BackgroundParasitic fetus is a kind of conjoined twin, in which one of the twins is resorbed and some parts of the body can remain attached to the other twin. In the spinal form of parasitic twin—the so-called parasitic rachipagus—an ectopic or accessory limb is attached to the back of a normal baby or only a mass of viable tissue may remain.MethodHere, we report two cases of rachipagus that one had a lower extremity on her back and the other had some tissues similar to genitalia and formed bony structures on his back.

Predicting ventriculoperitoneal shunt infection in children with hydrocephalus using artificial neural network

ObjectivesThe relationships between shunt infection and predictive factors have not been previously investigated using Artificial Neural Network (ANN) model. The aim of this study was to develop an ANN model to predict shunt infection in a group of children with shunted hydrocephalus.Materials and methodsAmong more than 800 ventriculoperitoneal shunt procedures which had been performed between April 2000 and April 2011, 68 patients with shunt infection and 80 controls that fulfilled a set of meticulous inclusion/exclusion criteria were consecutively enrolled. Univariate analysis was performed for a long list of risk factors, and those with p valuexa0<xa00.2 were used to create ANN and logistic regression (LR) models.ResultsFive variables including birth weight, age at the first shunting, shunt revision, prematurity, and myelomeningocele were significantly associated with shunt infection via univariate analysis, and two other variables (intraventricular hemorrhage and coincided infections) had a p value of less than 0.2. Using these seven input variables, ANN and LR models predicted shunt infection with an accuracy of 83.1xa0% (AUC; 91.98xa0%, 95xa0% CI) and 55.7xa0% (AUC; 76.5, 95xa0% CI), respectively. The contribution of the factors in the predictive performance of ANN in descending order was history of shunt revision, low birth weight (under 2000xa0g), history of prematurity, the age at the first shunt procedure, history of intraventricular hemorrhage, history of myelomeningocele, and coinfection.ConclusionThe findings show that artificial neural networks can predict shunt infection with a high level of accuracy in children with shunted hydrocephalus. Also, the contribution of different risk factors in the prediction of shunt infection can be determined using the trained network.

Myelomeningocele defect closure

Dear Editor: We read with great interest the recent article by Kobraei et al. (A comparison of techniques for myelomeningocele defect closure in the neonatal period. Childs nervous system 30: 9, 1535–1541, September 2014), concerning the techniques that they have used in myelomeningocele (MMC) surgery. The authors compared the outcome of repair in 32 consecutive patients who underwent MMC defect closure with primary closure, myocutaneous, and fasciocutaneous flaps. According to their experience, there was no significant statistical difference between the three kinds of wound closure, but they finally suggested that myocutaneous flaps as a confident method of repair to be considered for smaller MMCs in addition to larger defects [1]. Surgical closure is essential to cover the exposed neural tissue and prevent infection and cerebrospinal fluid leakage. Most skin defects in MMC patients are small enough that can be closed by tension-free approximation in the midline. In 25 % of the instances, there is a defect that is too large to be closed by this simple technique (a width more than half of the width of the infant’s back). In addition to the size of defect, the associated kyphosis influences the appropriate method of repair and the success rate of skin reconstruction [2]. In spite of folate supplementation and relatively regular prenatal ultrasound screening, MMC repair is a common spinal surgery in pediatric neurosurgery service in our center, and the whole procedure from cord untethering to skin closure is done by the neurosurgery team. Regardless of different techniques used for MMC repair, the closure of large MMC defects remains a challenging issue. Most methods described for the closure of largeMMCs prolong the time of surgery and anesthesia which complicate the surgery of young infants. Therefore, we recommend our routine method of primary closure for most defects with diameter of less than half of the back (considered as “not large defect”). This method is fast with acceptable cosmesis and less complicated wound closure. For large lesions that make the primary closure impossible and are associated with risk of wound necrosis, we use fasciocutaneous flap that covers the large defect without the risk of dehiscence and necrosis. This method makes the procedure longer than usual and can complicate the surgery of neonates with MMC due to prolonged operation time, but warrants a successful reconstruction.

Double dermal sinuses: a case study

IntroductionDermal sinus tracts are rare congenital lesions located in the midline characterized by a cutaneous pit or dimple. They occur all along the midline neuroaxis, from the nasion and occipital area down to the lumbar and sacral regions, most frequently in the lumbar and lumbosacral region.Case presentationHere we report a 5-year-old girl who presented with occasional headache. There were two dimples, one on the dorsal aspect of her head and another on her neck.ConclusionDermal sinuses are almost always singular and the co-existence of double dermal sinuses has not been reported previously.

Spontaneous regression and complete disappearance of the vein of Galen aneurysmal malformation.

IntroductionThrombosis is frequently observed in Galen malformation, but propagation of thrombosis resulting in the disappearance of the aneurysmal malformation is a very rare clinical condition.Case reportA rare case of spontaneous regression and disappearance of the vein of Galen aneurysmal malformation (VoGAM) in a pediatric patient with repeated generalized seizure, increased head circumference, and congestive heart failure is recorded. The course of regression from infancy to 8xa0years of age has been depicted. Radiological studies initially demonstrated VoGAM complicated by an intra-cerebral hemorrhage and hydrocephalus, which later underwent spontaneous regression. Long-term clinical and radiological follow-up is presented.DiscussionDifferent conditions including hemodynamic alteration, compression of adjacent hematoma, and narrowing of related vascular structures have been described to cause thrombosis of VoGAM. The relevant literature to address possible mechanism is reviewed.

Complete absence of the roof of the third ventricle in a case of hydrocephalus: an endoscopic view

Dear Editor: We would like to present an extremely rare case of hydrocephalus, corpus callosum agenesis, and complete absence of the third ventricle’s roof and septum pellucidum. Although several cases of septum pellucidum agenesis, either isolated or combined with other anomalies, have been described in the literature [1], the association with complete disturbance of the third ventricular roof—including tela choroida and velum interpositum—is hardly ever recorded. A 2-month-old infant was hospitalized with enlarged head and full fontanel. Magnetic resonance image revealed hydrocephalus, aqueductal stenosis, corpus callosum agenesis, and the absence of septum pellucidum (Fig. 1a, b). The interhemispheric fissure, however, was in normal contour, and the floor of the third ventricle and prepontine cistern was appropriate for third ventriculostomy. The patient was scheduled for endoscopic third ventriculostomy (ETV). Once rigid endoscope entered the right lateral ventricle, as anticipated, the septum pellucidum was not observed. Moreover, the roof of the third ventricle was defective, so that fornixes were hanged freely between the body of the lateral ventricles upstairs and the third ventricle downstairs (Fig. 1c). In such situation, by entering the body of lateral ventricles, the anteroposterior perspective of the floor of the third ventricle could be seen uncovered; For instance, the posterior commissure at the most posterior part of the third ventricle was easily observed (Fig. 1c). The right and left thalami were separated from each other, staying in the floor of both lateral ventricles in an inappropriately abnormal distance. In the absence of both septum pellucidum and tela choroida, the unsupported fornix was caudally displaced leading to distortion of the outline of the foramina of Monro toward Bteardrop shape^ instead of normal Brounded shape^ (Fig. 1c). By cautiously directing endoscope through the foramen of Monro, EVT was performed in a routine way. The endoscope was never directed under the fornix, as internal cerebral veins were coursing just below the fornixes (in the virtual place of velum interpositum). The postoperative course was uneventful, and the patient had normal head growth in a follow-up period of 6 months. The developmental milestones were compatible to age, and the optic disc had normal appearance on fundoscopy. The absence of the septum pellucidum may be due to developmental anomalies like septo-optic dysplasia and holoprosencephaly, or to secondary disruptions as can be seen in aqueductal stenosis and porencephaly [4]. The association between hydrocephalus and septum pellucidum anomalies has been previously described [2]. As a theory, since the inferior border of septum pellucidum is attached to the upper face of the fornix, the abnormally low positioned fornix—in the absence of the tethering effect of septum pellucidum—may lodge in the foramina of Monro causing intermittent obstruction and mild ventriculomegaly [1]. The clinical significance of this mild ventriculomegaly is uncertain; it may represent an obstructive hydrocephalus or just a diagnostic feature of the absence of the septum pellucidum [1]. Moreover, potential links have been proposed between hydrocephalus and anomalies in the roof of the third ventricle [5]. The velum interpositum is a triangular space between the two layers of the tela choroidea in the roof of the third * Zohreh Habibi z-habibi@sina.tums.ac.ir