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Background: Bleeding Worldwide, approximately 300,000 infants are born annually with neural tube defects (NTDs), which carry a high risk of morbidity and mortality., Objective: The aim of the study was to describe the experience with NTD patients born at a tertiary academic center., Methods: A retrospective record review of all neonates with NTD admitted to the neonatal intensive care unit over six years., Results: Out of the 39 patients identified, 32 (82.1%) were diagnosed antenatally. Most NTD cases were of the myelomeningocele 26 (66.7%) type. The most common site of the myelomeningocele was lumbar, and the thoracolumbar site had the worst prognosis., Conclusion: Early detection of the disease allows better planning of delivery and treatment decisions. Nevertheless, understanding the magnitude of the problem necessitates adopting public health prevention strategies for better outcomes., Competing Interests: There are no conflicts of interest., (© 2023 Heidi Al-Wassia, Hanan Bamanie, Haneen Rahbini, Nada Alghamdi, Raghad Alotaibi, Wed Alnagrani.)

Introduction: Uterine incision based on the placental location in open maternal-fetal surgery (OMFS) has never been evaluated in regard to maternal or fetal outcomes., Objective: The aim of this study was to investigate whether an anterior placenta was associated with increased rates of intraoperative, perioperative, antepartum, obstetric, or neonatal complications in mothers and babies who underwent OMFS for fetal myelomeningocele (fMMC) closure., Methods: Data from the international multicenter prospective registry of patients who underwent OMFS for fMMC closure (fMMC Consortium Registry, December 15, 2010-June 31, 2019) was used to compare fetal and maternal outcomes between anterior and posterior placental locations., Results: The placental location for 623 patients was evenly distributed between anterior (51%) and posterior (49%) locations. Intraoperative fetal bradycardia (8.3% vs. 3.0%, p = 0.005) and performance of fetal resuscitation (3.6% vs. 1.0%, p = 0.034) occurred more frequently in cases with an anterior placenta when compared to those with a posterior placenta. Obstetric outcomes including membrane separation, placental abruption, and spontaneous rupture of membranes were not different among the 2 groups. However, thinning of the hysterotomy site (27.7% vs. 17.7%, p = 0.008) occurred more frequently in cases of an anterior placenta. Gestational age (GA) at delivery (p = 0.583) and length of stay in the neonatal intensive care unit (p = 0.655) were similar between the 2 groups. Fetal incision dehiscence and wound revision were not significantly different between groups. Critical clinical outcomes including fetal demise, perinatal death, and neonatal death were all infrequent occurrences and not associated with the placental location., Conclusions: An anterior placental location is associated with increased risk of intraoperative fetal resuscitation and increased thinning at the hysterotomy closure site. Individual institutional experiences may have varied, but the aggregate data from the fMMC Consortium did not show a significant impact on the GA at delivery or maternal or fetal clinical outcomes., (© 2021 S. Karger AG, Basel.)

The aim was to analyze systemic and oral aspects of patients with myelomeningocele (MMC), relevant for their dental treatment. Dental treatment of these individuals requires the dentist's extensive knowledge and careful information to determine the dental treatment plan. Thirty participants aged 3 to 16 years were divided into 2 groups: study group (15 MMC individuals) and control group (15 healthy subjects). All patients were evaluated for: systemic conditions, use of medications, sequela of the underlying pathology, oral hygiene, diseases, convulsion, cerebrospinal fluid (CSF), allergy, behavior, learning, and mouth physical examination to assess the oral conditions. The study group presented: higher percentage using drugs (P < 0.05), 40% had convulsions (P < 0.05), 86.67% using CSF shunt, 53.33% presented latex allergies (P < 0.05), 53.33% were classified as anxious and with cognitive deficit (P < 0.05), showed more gingivitis (P < 0.05). The MMC individuals, in the majority, have shown cognitive impairment and anxious behavior, use of controlled drugs, convulsion, CSF shunt use, and latex allergy. The presence of periodontal tissue damage was higher in patients with MMC.

This paper describes some of the more common patterns in neurobehavioral deficits and their underlying neuroanatomical basis in myelomeningocele (MMC). Patients with MMC can face a lifetime of specific organ system dysfunction, chief among them spinal cord malformations, orthopedic issues, hydrocephalus, and urological disabilities. In addition, patients can experience specific patterns of neurobehavioral difficulties due to the changes in neuroanatomy associated with the open spinal defect. Although there is variability in these patterns, some trends have been described among MMC patients. It is thought that early recognition of these potential neurobehavioral deficits by treating neurosurgeons and other members of the treatment team could lead to earlier intervention and positively impact the overall outcome for patients. Neurodevelopmental and neurobehavioral follow-up assessments are recommended to help guide planning for relevant treatments or accommodations.

Objectives: Fluency is a major problem for individuals with neurodevelopmental disorders, including fluency deficits for academic skills. The aim of this study was to determine neurocognitive predictors of academic fluency within and across domains of reading, writing, and math, in children and adults, with and without spina bifida. In addition to group differences, we expected some neurocognitive predictors (reaction time, inattention) to have similar effects for each academic fluency outcome, and others (dexterity, vocabulary, nonverbal reasoning) to have differential effects across outcomes., Methods: Neurocognitive predictors were reaction time, inattention, dexterity, vocabulary, and nonverbal reasoning; other factors included group (individuals with spina bifida, n=180; and without, n=81), age, and demographic and untimed academic content skill covariates. Univariate and multivariate regressions evaluated hypotheses., Results: Univariate regressions were significant and robust (R 2 =.78, .70, .73, for reading, writing, and math fluency, respectively), with consistent effects of covariates, age, reaction time, and vocabulary; group and group moderation showed small effect sizes (&lt;2%). Multivariate contrasts showed differential prediction across academic fluency outcomes for reaction time and vocabulary., Conclusions: The novelty of the present work is determining neurocognitive predictors for an important outcome (academic fluency), within and across fluency domains, across population (spina bifida versus typical), over a large developmental span, in the context of well-known covariates. Results offer insight into similarities and differences regarding prediction of different domains of academic fluency, with implications for addressing academic weakness in spina bifida, and for evaluating similar questions in other neurodevelopmental disorders. (JINS, 2019, 25, 249-265).

Background: Split cord malformation (SCM) is a rare congenital anomaly of the spinal cord. Rarely, SCM coexists with a variety of dysraphic pathologies that occur at the same or different spinal level in a patient. Exceptionally rare is the occurrence of SCM type 1 and lipomeningomyelocele of each hemicord., Case Description: A 15-month-old girl presented with gradually progressive, painless swelling in the lower back since birth. Spinal imaging showed the presence of type I SCM associated with lipomeningomyelocele of each hemicord. Surgical exploration and detethering was done., Conclusions: Management of such complex cases of spinal dysraphism is challenging. Delineating their embryologic basis, detailed radiologic assessment, and meticulous microneurosurgical techniques are the cornerstone for successful management., (Copyright © 2018. Published by Elsevier Inc.)

Introduction: The surgically induced fetal lamb model is the most commonly used large animal model of myelomeningocele (MMC) but is subject to variation due to surgical technique during defect creation., Material and Methods: Thirty-one fetal lambs underwent creation of the MMC defect, followed by defect repair with either an extracellular matrix (ECM) patch (n = 10) or ECM seeded with placental mesenchymal stromal cells (n = 21). Postnatal hindlimb function was assessed using the Sheep Locomotor Rating (SLR) scale. Postmortem magnetic resonance imaging of the lumbar spine was used to measure the level and degree of spinal angulation, as well as cross-sectional area of remaining vertebral bone., Results: Median level of angulation was between the 2nd and 3rd lumbar vertebrae, with a median angle of 24.3 degrees (interquartile range 16.2-35.3). There was a negative correlation between angulation degree and SLR (r = -0.44, p = 0.013). Degree of angulation also negatively correlated with the normalized cross-sectional area of remaining vertebral bone (r = -0.75, p < 0.0001)., Discussion: Surgical creation of fetal MMC leads to varying severity of spinal angulation in the ovine model, which affects postnatal functional outcomes. Postnatal assessment of spinal angulation aids in standardization of the surgical model of fetal MMC repair., (© 2019 S. Karger AG, Basel.)

A 51-year-old man who had undergone right orbital decompression 5 months earlier developed a meningoencephalocele extending in the right sphenoid sinus through a skull base defect of the right ethmoid, sphenoid, and frontal bones. The authors report the third case to their knowledge of meningoencephalocele with cerebrospinal fluid leak after orbital decompression and discuss its management and measures that can be taken to prevent this rare but serious complication.

Background: Neural tube defects are debilitating birth defects that occur when the developing neural plate fails to close in early gestation. Arsenic induces neural tube defects in animal models, but whether environmental arsenic exposure increases risk of neural tube defects in humans is unknown., Methods: We describe a new case-control study in Bangladesh, a country currently experiencing an epidemic of arsenic poisoning through contaminated drinking water. We plan to understand how arsenic influences risk of neural tube defects in humans through mechanisms that include disruption of maternal glucose and folate metabolism, as well as epigenetic effects. We also investigate whether sweat chloride concentration, a potential new biomarker for arsenic toxicity, can be used to identify women at higher risk for having a child affected by neural tube defect. We will collect dural tissue from cases, obtained at the time of surgical closure of the defect, and believe investigation of these samples will provide insight into the epigenetic mechanisms by which prenatal arsenic exposure affects the developing nervous system., Conclusion: These studies explore mechanisms by which arsenic may increase risk of neural tube defects in humans and use a unique population with high arsenic exposure to test hypotheses. If successful, these studies may assist countries with high arsenic exposure such as Bangladesh to identify populations at high risk of neural tube defects, as well as direct development of novel screening strategies for maternal risk.Birth Defects Research 109:92-98, 2017.© 2016 The Authors Birth Defects Research Published by Wiley Periodicals, Inc., (© 2016 The Authors Birth Defects Research Published by Wiley Periodicals, Inc.)

Jarcho-Levin syndrome (JLS) is a rare axial skeletal growth disorder characterized by the presence of multi-level costovertebral malformations, a short neck, a short trunk, and kyphoscoliosis at birth. Neural tube defects appear to be common findings in patients with JLS, and chest hypoplasia frequently leads to respiratory insufficiency and death in early childhood. There have been no previous studies in the literature on the anesthetic management of JLS with neural tube defects and hydrocephalus. Therefore, we have reported a case of JLS in a female full-term newborn, who underwent surgery for myelomeningocele repair and ventriculoperitoneal shunting, with the associated anesthetic challenges and their management.

Triple spinal dysraphism is extremely rare. There are published reports of multiple discrete neural tube defects with intervening normal segments that are explained by the multisite closure theory of primary neurulation, having an association with Chiari malformation Type II consistent with the unified theory of McLone. The authors report on a 1-year-old child with contiguous myelomeningocele and lipomyelomeningocele centered on Type I split cord malformation with Chiari malformation Type II and hydrocephalus. This composite anomaly is probably due to select abnormalities of the neurenteric canal during gastrulation, with a contiguous cascading impact on both dysjunction of the neural tube and closure of the neuropore, resulting in a small posterior fossa, probably bringing the unified theory of McLone closer to the unified theory of Pang.

Object: Treatment for hydrocephalus has not advanced appreciably since the advent of CSF shunts more than 50 years ago. The outcome for pediatric patients with hydrocephalus has been the object for several studies; however, much uncertainty remains regarding the very long term outcome for these patients. Shunting became the standard treatment for hydrocephalus in Norway during the 1960s, and the first cohorts from this era have now reached middle age. Therefore, the objective of this study was to review surgical outcome, mortality, social outcome, and health-related quality of life in middle-aged patients treated for hydrocephalus during childhood., Methods: Data were collected in all patients, age 14 years or less, who required a CSF shunt during the years 1967-1970. Descriptive statistics were assessed regarding patient characteristics, surgical features, social functioning, and work participation. The time and cause of death, if applicable, were also determined. Kaplan-Meier survival estimates were used to determine the overall survival of patients. Information regarding self-perceived health and functional status was assessed using the 36-Item Short Form Health Survey (SF-36) and the Barthel Index score., Results: A total of 128 patients were included in the study, with no patient lost to follow-up. Of the 128 patients in the study, 61 (47.6%) patients died during the 42-45 years of observation. The patients who died belonged to the tumor group (22 patients) and the myelomeningocele group (13 patients). The mortality rate was lowered to 39% if the patients with tumors were excluded. The overall mortality rates at 1, 2, 10, 20, and 40 years from time of initial shunt insertion were 16%, 24%, 31%, 40%, and 48% respectively. The incidence of shunt-related mortality was 8%. The majority of children graduated from a normal school (67%) or from a school specializing in education for physically handicapped children (20%). Self-perceived health was significantly poorer in 6 out of 8 domains assessed by SF-36 as compared with the background population. Functional status among the survivors varied greatly during the follow-up period, but the majority of patients were self-dependent. A total of 56% of the patients were socially independent, and 42% of the patients were employed., Conclusions: Approximately half of the patients are still alive. During the 42-45 year follow-up period, the mortality rate was 48%. Two deaths were due to acute shunt failure, and at least 8% of the deaths were shunt related (probable or late onset). The morbidity in middle-aged individuals treated for pediatric hydrocephalus is considerable. The late mortality rate was low, but not negligible. Twelve patients died during the last 2 decades, 1 of whom died because of acute shunt failure. Although the shunt revision rate was decreasing during the study period, many patients required shunt surgery during adulthood. Forty-one revisions in 21 patients were performed during the last decade. Thus, there is an obvious need for life-long follow-up in these patients.

The present study examined prospective and episodic memory in relation to age, functional independence, and hippocampal volume in younger to middle-aged adults with spina bifida myelomeningocele (SBM) and typically developing (TD) adults. Prospective and episodic memory, as well as hippocampal volume, was reduced in adults with SBM relative to TD adults. Neither memory performance nor hippocampal volume showed greater decrements in older adults. Lower hippocampal volume was associated with reduced prospective memory in adults with SBM, and this relation was specific to the hippocampus and not to a contrast structure, the amygdala. Prospective memory mediated the relation between hippocampal volume and functional independence in adults with SBM. The results add to emerging evidence for reduced memory function in adults with SBM and provide quantitative evidence for compromised hippocampal macrostructure as a neural correlate of reduced memory in this population., (PsycINFO Database Record (c) 2015 APA, all rights reserved.)

Objective: Bariatric surgery results in decreased food intake and a variable degree of malabsorption. Without adequate supplementation, the most common complications of this surgery are nutritional disorders. Pregnancy following surgery for obesity is a particular condition requiring strict monitoring of nutrient intake necessary for fetal development and a favourable neonatal prognosis., Patients: Malnutrition in pregnancy and congenital neural malformations are reported in three women who had previously undergone bariatric surgery (1, 5 and 18 years before pregnancy, respectively). Two patients underwent the Roux en Y bypass and one bilio-pancreatic diversion with gastroplasty. None of the three received pre-conceptional nutritional counselling. Patients 1 and 2 did not undergo postoperative nutritional surveillance; nutrient supplementation was started at 22 and 20 weeks gestation, respectively. In patient 3, supplementation was stopped at six weeks gestation., Results: Newborns 1 and 2 presented with dorsal myelomeningocele and ventricular dilation. Both underwent surgery and a ventriculo-peritoneal shunt was inserted in the first month of life. Newborn 3 had microcephaly, bilateral microphthalmia and sensorineural deafness., Conclusions: Diet and nutritional status, before and during pregnancy, play an important role in the early processes of fetal development and neonatal outcome. Women of childbearing age who have had bariatric surgery, should be encouraged to follow a well-balanced diet as part of a weight management strategy. They should be advised to take recommended maternal supplements.

Background: The prevention of neural tube defects (NTDs) is now an issue of major public health concern. Myelomeningocele, the most common NTD, often results in severe disabilities and may be life threatening. In Africa, there is little awareness of the NTDs, and most African countries lag behind in the global efforts at preventing them., Purpose: Following a review of global literature, we discuss the burden of myelomeningocele on the society and the current state of its prevention with an emphasis on Africa within the global context. The realization that folate supplementation reduces the occurrence of NTDs by as much as 85 % offers an opportunity to promote global reduction in the incidence of NTDs through (1) effective folate supplementation strategies combined with (2) secondary prevention using prenatal detection and termination of NTD pregnancies within respective national legal frameworks. We call attention to the poor state of understanding of NTDs in Africa and the alarming rarity of policies to prevent the condition in the continent., Conclusions: The understanding of the contributions of folate deficiency to the causation of NTDs has enabled appropriate, though still inadequate, preventive measures to be taken in several countries. We call on African governments and the international community to rapidly promote policies aimed at making fortification of wheat (and or other substitute staple foods) with folic acid universally available.

Introduction: Iodine deficiency is the most devastating event in developing brain in the fetus and neonate. Iodine is absolutely necessary on the myelination, neuronal differentiation, and formation of neural processes, synaptogenesis, and neuronal migration by thyroidal hormones throughout pregnancy and shortly after birth. Neural tube defects (NTD) form after third and fourth gestational weeks and their etiologies are multifactorial., Case Report: We herein present a male newborn with iodine deficiency and thoracic neuroenteric cyst bound to a myelomeningocele via a pedinculi. We hypothesize that iodine deficiency may be a cause of NTD, and iodine supplementation in preconception and pregnancy may prevent NTD.

Myelomeningocele is characterized by the extrusion of the spinal cord into a sac filled with cerebrospinal fluid. One part of the postnatal disabilities could be related to the spinal damage and to the cerebral repercussion of the leak of cerebrospinal fluid from the defect. Several experimental studies in animals have demonstrated that a surgical repair of the lesion at middle gestation reduced the postnatal disabilities. These results were confirmed in humans by the Management of Myelomeningocele (MOM) Trial. However, the prenatal surgical repair is associated with maternal and fetal morbidity., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Background: In general, it seems that both genetic and environmental factors play important roles in the induction of neural tube defects. Lipomyelomeningocele (LipoMMC) is a rather common type of closed neural tube defect, but only limited studies have investigated the potential risk factors of this anomaly. Therefore, the purpose of this case-control study was to investigate the risk factors involved in LipoMMC formation., Material and Method: Various risk factors were evaluated in 35 children between 1 month and 10 years of age with LipoMMC in a hospital-based case-control study. The 2 control arms consisted of 35 children with myelomeningocele (MMC group) and 35 children with congenital anomalies other than central nervous system problems (control group). All groups were matched for age and visited the same hospital. A structured questionnaire was used for the collection of all data, including the mothers' weight and height during pregnancy, education, reproductive history, previous abortions, and socioeconomic status, as well as the parents' consanguinity and family history of the same anomalies., Results: Univariate analysis of the children with LipoMMC compared to the control group showed that the use of periconceptional folic acid supplementation was significantly lower in the MMC and LipoMMC groups compared to the control group. In addition, comparison of the MMC and control groups revealed statistically significant differences regarding the use of folic acid and maternal obesity. In multivariate analysis, use of folic acid in the periconceptional period and during the first trimester was an independent risk factor for LipoMMC and MMC. Furthermore, maternal obesity was a significantly positive risk factor for MMC., Conclusion: The probable risk factors for LipoMMC were investigated in this case-control study. Consumption of folic acid in the periconceptional period and during the first trimester is an independent protective factor against LipoMMC. It seems that larger studies are needed to examine other possible risk factors., (© 2014 S. Karger AG, Basel.)

Background: Somatosensory evoked potentials (SSEP) have been described as excellent indicators of the degree of medullar injury in degenerative and metabolic diseases of the central nervous system (CNS). The prevalence of neural tube defects (NTD) is 6 cases/10,000 live newborns worldwide. It is thought that genetic as well as environmental factors contribute to the etiology of NTD. The objective of this study was to analyze and compare the latencies obtained by means of SSEP in a clinically healthy monkey vs. rhesus monkeys with intrauterine surgery in order to simulate surgically the neural tube defect (myelomengocele) by performing an intrauterine laminectomy and in which later the defect was corrected., Methods: This study was performed using three non-human primates of the Macaca mulatta species. There were practice intrauterine surgeries in two monkeys to simulate the neurological defect produced by myelomeningocele, using the third monkey as control. For statistical methodology four monkeys were used. They were born by natural birth without any surgical manipulation. With the cesarean-obtained products, stimulation was performed of the tibial and median nerve., Results: We observed that the hind limbs were the most affected, in particular, the left afferent of the monkey. The spinal cord was exposed to amniotic fluid, and there were no significant differences in the forelimbs., Conclusions: The use of SSEP provides valuable information regarding preservation of sensorial functions in a variety of experimental neurological abnormalities.

Surgical procedures for spinal lipoma or tethered spinal cord after myelomeningocele (MMC) repair are often difficult and complicated, because the anatomical structures can be deformed in complex and unpredictable ways. Imaging helps the surgeon understand the patient's spinal anatomy. Whereas two-dimensional images provide only limited information for surgical planning, three-dimensional (3D) reconstructed computed tomography (CT)-magnetic resonance (MR) fusion images produce clearer representations of the spinal regions. Here we describe simple and quick methods for obtaining 3D reconstructed CT-MR fusion images for preoperative planning of surgical procedures using the iPlan(®) cranial (BrainLAB AG, Feldkirchen, Germany) neuronavigation software. 3D CT images of the vertebral bone were combined with heavily T(2)-weighted MR images of the spinal cord, lipoma, cerebrospinal fluid (CSF) space, and nerve root through a process of fusion, segmentation, and reconstruction of the 3D images. We also used our procedure called "Image Overlay" to directly project the 3D reconstructed image onto the body surface using an LED projector. The final reconstructed 3D images took 10-30 minutes to obtain, and provided the surgeon with a representation of the individual pathological structures, so enabled the design of effective surgical plans, even in patients with bony deformity such as scoliosis. None of the 19 patients treated based on our 3D reconstruction method has had neurological complications, except for CSF leakage. This 3D reconstructed imaging method, combined with Image Overlay, improves the visual understanding of complicated surgical situations, and should improve surgical efficiency and outcome.

A 43-year-old man with no cardiac history presented with chest pain followed by cardiac arrest. He was successfully defibrillated and underwent primary percutaneous coronary angioplasty to a culprit coronary artery lesion. He later re-presented with a diffuse urticarial rash and lip swelling, reporting that these symptoms had been present for 4 weeks before his cardiac arrest and voicing concern that a further cardiac arrest may be imminent. A diagnosis of post-viral or idiopathic autoimmune urticaria and angioedema was made. Given the absence of cardiac symptoms before the development of the rash, it was hypothesised that coronary artery spasm precipitated by histamine release due to his dermatological condition contributed to his myocardial infarction and cardiac arrest. The final diagnosis was therefore cardiac arrest secondary to type II Kounis syndrome, resulting from idiopathic autoimmune or post-viral urticaria and angioedema.

Purpose: Evaluate the anesthetic management in intrauterine surgery to induce myelomeningocele in non human primates Macaca mulatta., Methods: A total of nine fetuses had intrauterine surgery; laminectomy was performed on them in L5 and L6. The studied variables were: maternal death, fetus death, cardiac frequency, respiratory frequency, arterial pressure, temperature, and oxygen saturation., Results: No maternal or fetal deaths occurred; the only variable that was reported below the normal ranges was temperature., Conclusion: No maternal or fetal deaths occurred; the only variable that was reported below the normal ranges was temperature.

An infant presented with a rare cervical (non-terminal) myelocystocele as a congenital skin-covered mass located in the midline of the posterior aspect of her neck. Magnetic resonance (MR) imaging and computed tomography showed a cystic mass filled with cerebrospinal fluid in the midline of the posterior aspect of the neck, with a fibrous streak extending from the bottom of the sac to the dorsal surface of the cervical cord. Brain MR imaging also showed a dilated ventricular system and Chiari type II malformation. The patient underwent plastic repair of the lesion, which was diagnosed as myelocystocele. After the surgery, the patient experienced respiratory distress. Ultrasound tomography from the anterior fontanel revealed deterioration of hydrocephalus, so a ventriculoperitoneal shunt was inserted, and the respiratory distress improved. The present case illustrates the possibility of rapidly worsening of hydrocephalus and Chari type II malformation after surgical repair of cervical (non-terminal) myelocystocele.

Recognition of cutaneous markers of spinal dysraphism is important to prevent the morbidity associated with underlying spinal anomalies. To investigate the frequency and type of cutaneous stigmata in different forms of spinal dysraphism and to assess the role of ultrasonography and/or magnetic resonance imaging in diagnosing spinal dysraphism at two pediatric dermatology tertiary care centers. Over a 4-year period, all pediatric patients presenting to the dermatology clinic with dorsal midline cutaneous stigmata were evaluated clinically and with imaging studies (radiography, ultrasonographic examination and magnetic resonance imaging/Doppler). Surgical interventions were planned in conjunction with neurosurgery and orthopedic specialists. On examination, 245 (4.2%) had 285 cutaneous stigmata. Of the 180 patients evaluated with radiography, ultrasonographic examination and magnetic resonance imaging, 50 patients (28%) had spinal dysraphism (with 64 cutaneous stigmata). The most common stigmata associated with occult spinal dysraphism were lipoma (10) and dimples (12) and in open spinal dysraphism lipomeningomyelocoele (10) and meningomyelocoele (10). Statistically, lipomeningomyelocoele/myelomeningocoele, atypical dimples and port-wine stains were most associated with spinal dysraphism (p < 0.001). In 80 children less than 6 months of age, radiography with ultrasonographic examination revealed an SD in 16, while magnetic resonance imaging was diagnostic in four cases. Ultrasonographic examination performed fairly well in children less than 6 months and in cases of flat cutaneous stigmata it missed only 5% of cases, but in cases with bulky overlying masses (lipoma, hemangioma) it missed 15% of cases.

Background: A variety of complications after FMD with or without duraplasty for Chiari malformation have been described. Although cerebellar ptosis through the dural opening and pseudomeningocele are well-known rare complications of this procedure, spinal cord herniation manifesting as pseudomyelomeningocele formation has not previously been cited in the literature. In this report, we present a case of delayed pseudomyleomeningocele after FMD., Case Description: A 22-year-old man presented with progressive spinal cord compression symptoms 7 years after undergoing FMD for Chiari malformation. Craniocervical MRI of the patient revealed pseudomyelomeningocele at C1 level together with cord distortion. The patient underwent untethering surgery for spinal cord adhesions; neural tissue was freed microsurgically from the adjacent structures and duraplasty was performed with synthetic graft. In the early postoperative period, his symptoms resolved moderately., Conclusion: Neural tissue shift including the spinal cord through the dural opening may occur after a large posterior fossa decompression without duraplasty for Chiari malformation. Postoperative inadequate CSF circulation due to arachnoid scarring around the decompression area may facilitate this type condition. Early diagnosis of such a complication is of vital importance to prevent serious neurologic deficits, and release surgery with proper duraplasty and also restoration of CSF circulation is the choice of treatment.

Object: The authors retrospectively investigated whether midgestational fetal myelomeningocele (fMMC) repair alters intrauterine meconium exposure., Methods: Prior to the National Institutes of Health Management of Myelomeningocele Study, 54 fetuses underwent fMMC repair at the authors' institution. Forty-six fMMC sacs were available for pathological examination and 53 MMC sacs from postnatally repaired MMCs (pMMCs) were available for comparison. The presence and distribution of meconium were blindly evaluated using a grading system defined as follows: absent (no meconium present), mild (<10 meconium-positive histiocytes [MPHs]/hpf), moderate (10-25 MPHs/hpf), and severe (>25 MPHs/hpf). Hall's bile stain was used to confirm meconium and Prussian blue and Fontana Masson stains to exclude hemosiderin and melanin, respectively., Results: Compared to pMMCs (79%), meconium histiocytosis was less prevalent in fMMC sacs (57%; p=0.017). Meconium staining was completely absent in 43% of the fMMC sacs. Mild meconium histiocytosis was found in 35% fMMC and 61% pMMC sacs (p=0.035). There was no statistical difference between groups with moderate and severe meconium histiocytosis., Conclusions: Meconium passage in MMCs can occur early in fetal life. Fetal MMC repair may reduce the duration of meconium exposure, thereby potentially limiting the toxic injury to the vulnerable neural elements.

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., ((c) 2008 S. Karger AG, Basel.)

Damage of neural elements (spinal cord and encephalus) in myelomeningocele (MMC) seems to be progressive during gestation because of amniotic fluid chemical contact and continuous leakage of CSF. We studied the effect of preterm delivery and steroid treatment in a model of MMC in the rabbit foetus. Twelve New Zealand White rabbits underwent laparotomy and hysterotomy at 23 days of gestation. Fifty-nine out of 107 foetuses underwent lumbar laminectomy (three to four levels). Dura was opened to expose the neural elements to the amniotic fluid. Six rabbits underwent caesarean section on gestational day 31 for fetal harvest; three of them had no treatment (group T) and three received corticosteroid treatment (group TC). The other six rabbits underwent caesarean section on gestational day 29 for fetal harvest (preterm delivery); three of them had no treatment (group P) and three received corticosteroid treatment (group PC). Alive newborns were clinically, neurophysiologically and histologically analysed. None of mothers died during the procedure. After birth, animals in group preterm showed statistically significant less deformity than animals in group at term. Lower kyphosis was observed in group PC (preterm and steroids). Pain related and spontaneous mobility of lower extremities was higher in groups treated with corticosteroids (TC and PC). Only newborns at term (T and TC groups) showed response to evoked potentials (CMEPs). The response was earlier and higher in group treated with steroids (TC). Histologically, we observed progressive lesion of the spinal cord. Groups treated with steroids (TC and PC) show less inflammatory response. Arnold-Chiari malformation was present in all groups. Animals in group preterm with steroids show statistically significant less herniation than those group at term. Preterm delivery and prenatal steroid therapy seem to be an effective treatment to get less neural injury (spinal cord and encephalus) in myelomeningocele foetuses.

A 12-year-old girl with neurofibromatosis type 1 underwent subtotal anterior exenteration of a blind, unsightly, buphthalmic eye and orbital/eyelid plexiform neurofibroma. Simultaneously, a channeled Medpor orbital implant was positioned to reduce a meningoencephalocele resulting from severe sphenoid wing dysgenesis. Two-stage osseointegration was subsequently performed to permit secure fitting of an orbital prosthesis. This patient's cosmesis and social interaction were improved markedly with this surgical approach for disfiguring ocular, eyelid, and orbital disease due to neurofibromatosis type 1.

Object: Idiopathic spinal cord herniation (ISCH) is an uncommon clinical entity typically presenting with lower-extremity myelopathy. Despite the existence of 85 ISCH cases in the literature, misdiagnosis and delayed diagnosis remain a major concern., Methods: The authors conducted a retrospective review of patients who underwent surgery for ISCH at their institutions between 1993 and 2004. Seven patients were treated for ISCH, five in New York and two in Buenos Aires. The patients' ages ranged from 32 to 72 years. There were three men and four women. The interval between the onset of symptoms and surgery ranged from 12 to 84 months (mean 42.1 months). Preoperatively, spinal cord function in four patients was categorized as American Spinal Injury Association (ASIA) Grade D, and that in the other three patients was ASIA Grade C. In all patients a diagnosis of posterior intradural arachnoid cyst had been rendered at other institutions, and three had undergone surgery for the treatment of this entity. In all cases, the herniation was reduced and the defect repaired with a dural patch. The follow-up period ranged from 10 to 147 months (mean 49.2 months). Clinical recovery following surgery varied; however, there was no functional deterioration compared with baseline status. Syringomyelia, accompanied by neurological deterioration, developed post-operatively in two patients at 2 and 10 years, respectively., Conclusions: Patients presenting with a diagnosis of posterior intradural arachnoid cyst should be evaluated carefully for the presence of an anterior spinal cord herniation. Based on the authors' literature review and their own experience, they recommend offering surgery to patients even when neurological compromise is advanced.

Spondylocostal dysostosis is a rare congenital segmental costovertebral malformation. Neural tube defects associated with it have been reported several times, and a genetic cause has been proposed. The authors report on the first patient with both spondylocostal dysostosis and an intrathoracic myelomeningocele in whom surgical treatment was successful.

Introduction: Children with spina bifida (SB) have a high degree of exposure to latex products as a consequence of repeated surgical procedures, implantation of latex-containing materials and catheterisation. The consequence is a higher incidence of latex allergic reactions., Objective: The aim of this study is to evaluate the prevalence of latex sensitisation and allergy in a population of children with myelomeningocele (MMC) and to assess the role of associated risk factors., Results: Forty-eight percent of the patients (29 out of 60) showed a latex sensitisation with specific IgE >0.7 kU/l while 15% (9 out of 60) were allergic to latex (specific IgE >0.7 kU/l and clinical manifestations). The principal factor correlated with allergy to latex was specific serum IgE to latex (radioallergosorbent test [RAST]) values (p<0.01). Other factors were total serum IgE (paper radioimmunosorbent test [PRIST]) values, number of surgical procedures and familiarity with allergy., Conclusion: These results underline the importance of prophylactic measures to avoid the exposure, not only in the sanitary environment, through the institution of latex-safe routes, but also in daily life, to prevent potentially serious allergic reactions.

Objective: The purpose of this study was to determine how closely the surgically induced sheep myelomeningocele (MMC) model resembles the central nervous system derangements seen in human disease, and identify which aspects of MMC are the result of the early neuronal developmental defect, and which are secondary to the cerebrospinal fluid (CSF) drainage., Study Design: An MMC-like lesion was created surgically in 16 fetal sheep at 75 days' gestation: 5 died in utero, 7 underwent no fetal repair, 4 were repaired (2-layer closure or biological glue) at 100 days' gestation. MMC sheep were delivered at term and allowed to survive up to 17 days for analysis of behavioral status and feeding behavior. Animals not repaired in utero were repaired at birth. All lambs were sacrificed and analyzed for hindbrain herniation, hydrocephalus, and other CNS derangements., Results: Hindbrain herniation was observed in 43% of animals not repaired in utero, and in 1 lamb repaired with Bioglue. No animal developed hydrocephalus or other CNS derangements., Conclusion: Although this sheep MMC-like model reproduces the CSF leak, but not the developmental defect seen in humans, it suggests CSF leak contributes to hindbrain herniation seen in humans. This model may be useful to develop new minimally invasive techniques to halt CSF leak in utero.

Object: As patients with myelomeningocele age, tethered cord syndrome (TCS) in adults with postrepair myelomeningocele has become more common. The authors have structured an evidence-based review of the literature for the purpose of addressing the following questions. (1) How is the diagnosis made? (2) What is the natural history that occurs in adults with postrepair myelomeningocele and TCS? (3) What are the criteria for operative intervention? (4) What is the functional outcome for patients with postrepair myelomeningocele?, Methods: A computerized search of the National Library of Medicine of the English-language literature published from 1966 to 2003 was performed. Articles pertaining to the clinical aspects and management of postrepair myelomeningocele in adults, TCS in adults resulting from closed defects, and pediatric patients with postrepair myelomeningocele were reviewed. No Level 1 or 2 data were located; however, the search yielded Level 3 and 4 evidence in the literature in which the clinical syndrome, underlying pathology, and the imaging and electrophysiological evaluation for TCS in adults are discussed., Conclusions: Analysis of the available data indicates the following. (1) A lower lesion level predisposes patients to symptomatic tethering; moreover, orthopedic and urological deterioration will occur in the majority of these patients. (2) Tethered cord release should be considered for adult patients with postrepair myelomeningocele when clinical symptoms, imaging studies, urodynamics, and somatosensory evoked potentials are consistent with TCS. (3) Prompt, aggressive untethering surgery within 5 years of symptom onset, along with long-term follow up to check for delayed retethering, is recommended. The overall outcome for patients with postrepair myelomeningocele may not be as good as the outcome for adults with closed dysraphism.

In Type I split cord malformation (SCM) lesions, the osteocartilaginous spur and its dural sleeve are almost always located in the caudal extent of the median cleft. The authors present a case of lumbar myelomeningocele associated with a thoracic Type I SCM in which an osseous septum is uniquely situated in the proximal extent of the median cleft. Split cord malformations are cord-tethering lesions, which may be associated with a myelomeningocele that causes additional tethering, even in the opposite direction; therefore, both lesions should be treated. In view of this unique case, however, the surgical approach used for tethering lesions in such cases of complex spina bifida should perhaps be tailored. Whenever feasible, meticulous preoperative examination of a patient with spina bifida, including entire craniospinal magnetic resonance imaging followed with perioperative dynamic evaluation, is important for the effectiveness and safety of the surgery.

We analyzed 31 children with myelomeningocele born between July 1990 and July 2000. Follow-up median was 24 months (6-68 months). Only 2 mothers had a known etiologic factor (diabetes mellitus). Twelve had the correct prenatal diagnosis. All children were born at term; 23 by cesarean; 13 had rupture of the membrane. Surgical correction had a 4 days median (1 to 44 days). Lumbosacral lesions were the most frequent (46%). Thirty patients were hydrocephalic, shunt was placed in 27. Meningitis was 4 times more frequent in shunted patients. Seven became epileptic (19.4%). Denver II test showed significant delay in gross motor development. Neurogenic bladder was diagnosed in 12 patients. Congenital clubfoot was the main orthopedic malformation (53%). Six infants died. Nowadays, 17 patients are being followed. A multidisciplinary approach probably helps for a better quality of life.

There are different interpretations of tethered cord syndrome (TCS) partly due to difficulty in understanding the concept of this syndrome as a functional disorder not merely based on gross anatomy of congenital anomalies. The essential mechanical factor of cord tethering is that any of the inelastic structures fastening the caudal end of the spinal cord produces traction effects on the lumbosacral cord. The production of such traction is the key to understanding this disorder. In a significant number of patients who present with the typical clinical signs and symptoms of TCS, the diameter of the filum terminale is found within normal limits and the caudal end of the spinal cord is located in the normal position. Therefore, the definition of TCS requires the demonstration that there is a posterior displacement of the conus and filum by MRI, lack of viscoelasticity by the stretch test of the filum during surgery, and fibrous displacement of glial tissue within the filum by histological studies. This is because there is inconsistency from such studies as ultrasonography, MRI and CT myelography, which attempt to establish the presence of a tight filum terminale. A goal of this article is to provide basic understanding of TCS so that clinicians can use the concept of stretch-induced spinal cord dysfunction for proper diagnosis and treatment of this disorder.

Object: The aims of this study were to describe the embryological changes that drive the formation of a split spinal cord malformation, analyse the forms of presentation, evaluate the diagnostic procedures and discuss the indications for the different forms of therapy for each case., Methods: Clinical and radiological features of 22 cases of split spinal cord malformations (SSCM) are reported. Three groups of patients are considered: group a, patients with signs of tethered cord or scoliosis; group b, patients with midline cutaneous stigmata and group c, patients with a meningocele or meningomyelocele., Conclusion: While CT scan is particularly useful for the evaluation of vertebral bodies and posterior arch abnormalities and spur characteristics in SSCM, MRI gives complementary information on the anatomy of spinal cord, dural sac, conus and filum terminale and permits the exclusion of associated lesions such as hydro-syringomyelia, dermal sinus or dermoid and epidermoid cysts. Surgery should be considered indicated in all cases of SSCM, even the asymptomatic ones, except in very badly handicapped meningomyelocele patients with nonprogressive disability and type II SSCM.

Objective: To determine the prevalence of upper and lower neural tube defects and identify its association with the exposure to illnesses and drugs during pregnancy., Material and Methods: This is a case-control study of 107 newborns with upper neural tube defects, 59 with lower neural tube defects, and 166 newborns without malformations, in 56,926 consecutive births between 1989 and 1997. The exposure was documented by a direct interview to the mother of those subject of study. The association was measured by the odds ratios, with confidence interval of 95%., Results: The prevalence of upper neural tube defects was of 1.9 for 1,000 newborn (alive or dead) and of lower neural tube defects of 1.0 for 1,000. The exposure to illnesses of less than a month of duration was associated with upper neural tube defects (OR = 3.11; IC = 1.34-7.39) the most important was flu; also the exposure to drugs (OR = 5.85; IC = 2.97-11.62), the most prominent was acetaminophen. These factors of risk were not associated with lower neural tube defects. The mother's occupation, illness of more than a month of duration and X-ray exposure were not associated with of upper and lower neural tube defects., Conclusions: More studies are needed in the association among illnesses of less than a month of duration and drugs with upper neural tube defects. The different exposure frequencies between upper and lower neural tube defects suggest heterogeneity.

Introduction: The association of teratoma and neural tube defects is very rare., Case Report and Discussion: A case of teratoma resembling bowel within a meningomyelocele is presented and the literature reviewed., Conclusion: The hypotheses of a possible common aetiology for this association include a single dysembryogenic process and neoplastic transformation of heterotopic primordial elements incorporated in the defect.

Though folic acid supplementation has reduced the incidence of Neural Tube Defects (NTD), NTD still constitutes one of the important congenital malformations having wide medical, social and ethical implications. Zinc deficiency has been reported to produce NTD in animals. This study was designed to evaluate zinc status of the newborn babies with NTD and their mothers. Eighty newborn babies with NTD and their mothers served as cases. Eighty apparently normal newborn babies and their mothers served as controls. Serum and scalp hair zinc levels were analyzed by atomic absorption spectrophotometry. The mean (+/- SD) serum and hair levels in normal mothers were 74.1 +/- 4.1 micrograms/dl and 142.3 +/- 8.0 micrograms/g respectively. The mean (+/- SD) serum and hair levels of the mothers who delivered NTD babies were 75.7 +/- 5.6 micrograms/dl and 129.9 +/- 5.3 micrograms/g respectively. The mean (+/- SD) serum and hair levels in normal newborn babies were 77.8 +/- 5.3 micrograms/dl and 188.8 +/- 6.2 micrograms/g respectively. The mean (+/- SD) serum and hair levels in NTD babies were 80.1 +/- 12.9 micrograms/dl and 174.2 +/- 10.7 micrograms/g respectively. The hair zinc levels of the affected babies and their mothers were significantly lower (P < 0.001) than the controls. This study has found association between NTD and decreased hair zinc levels and large population based studies are recommended to confirm the association between zinc and NTD and to investigate whether zinc supplementation would reduce the overall incidence of NTD.

Aim: To assess the diagnostic value of spinal ultrasound in cloacal exstrophy, a caudal malformation which is associated with spinal dysraphism, and to assess the prevalence of spinal dysraphism in cloacal exstrophy., Materials and Methods: Ten infants under 1 year old with cloacal exstrophy underwent spinal ultrasound at presentation. Three patients also had a magnetic resonance imaging (MRI) examination. Ultrasound and MRI images were reviewed and correlated., Results: Nine of 10 patients had no external signs of spinal dysraphism. One patient had a clinically apparent myelomeningocele. Five of 10 patients (50%) had spinal dysraphism on ultrasound: there were two patients with a low cord, two with tethered cords and a lipoma, and one patient with tethering and a myelomeningocele. Thus, in four of these five patients spinal dysraphism was occult. In a small number of patients (n = 3) MRI was also performed-in these cases the MRI and ultrasound appearances correlated, however MRI was not performed in those patients in whom spinal ultrasound was normal., Conclusion: In three cases where spinal ultrasound detected occult dysraphism and MRI was performed, spinal ultrasound and MRI correlated. Advantages of spinal ultrasound include ease of examination, production of high quality multi-planar images and the facility for portable imaging at the bedside. Spinal ultrasound should be the first investigation in all babies with cloacal exstrophy to diagnose occult and non-occult spinal dysraphism.

Objective: Myelomeningocele is a neural tube defect resulting in an exposed spinal cord, which leads to irreversible neurologic damage at birth. We proposed development of a fetal rabbit model of myelomeningocele to study in utero spinal cord injury and repair strategies., Methods: New Zealand white rabbits (n = 10) at 22 days of gestation (term = 31 days) underwent laparotomy to expose the gravid uterus; a hysterotomy exposed the fetal hindlimbs and back. A three to four level lumbar laminectomy was performed, and the dura over the posterior spinal cord was removed. At 30 days of gestation, the does underwent C-section for fetal harvest, and total fetal number, length, weight, and the presence or absence of a spinal defect were recorded for all viable fetuses., Results: All injured fetuses were smaller and weighed less than the nonoperated littermate controls, and histologic examination confirmed a spina bifida-like lesion of their spinal cords., Conclusions: We successfully created an exposed spinal cord defect in the fetal rabbit model similar to the lesion found in humans. Advantageous because of low animal cost, relatively large fetal size, multiple fetuses per pregnancy, and short total gestation, this model will allow us to study the mechanism of injury to the exposed spinal cord, and perhaps develop strategies to repair human myelomeningoceles., (Copyright 2000 S. Karger AG, Basel.)

In 2 cases of infants with the Chiari II malformation, a prepontine cyst presented in association with the hindbrain abnormalities characteristic of the Chiari II syndrome. Both infants presented with bulbar dysfunction and had poor outcome despite decompression of the cerebellar tonsillar herniation and drainage of the prepontine cysts. No such association between prepontine lesions and Chiari II has been described previously., (Copyright 2000 S. Karger AG, Basel)