Spinal Defects Research Articles

Investigating the impact of the phosphorylation status of tyrosine residues within the TACC domain of TACC3 on microtubule behavior during axon growth and guidance.

Axon guidance is a critical process in forming the connections between a neuron and its target. The growth cone steers the growing axon toward the appropriate direction by integrating extracellular guidance cues and initiating intracellular signal transduction pathways downstream of these cues. The growth cone generates these responses by remodeling its cytoskeletal components. Regulation of microtubule dynamics within the growth cone is important for making guidance decisions. TACC3, as a microtubule plus-end binding (EB) protein, modulates microtubule dynamics during axon outgrowth and guidance. We have previously shown that Xenopus laevis embryos depleted of TACC3 displayed spinal cord axon guidance defects, while TACC3-overexpressing spinal neurons showed increased resistance to Slit2-induced growth cone collapse. Tyrosine kinases play an important role in relaying guidance signals to downstream targets during pathfinding events via inducing tyrosine phosphorylation. Here, in order to investigate the mechanism behind TACC3-mediated axon guidance, we examined whether tyrosine residues that are present in TACC3 have any role in regulating TACC3's interaction with microtubules or during axon outgrowth and guidance behaviors. We find that the phosphorylatable tyrosines within the TACC domain are important for the microtubule plus-end tracking behavior of TACC3. Moreover, TACC domain phosphorylation impacts axon outgrowth dynamics such as growth length and growth persistency. Together, our results suggest that tyrosine phosphorylation of TACC3 affects TACC3's microtubule plus-end tracking behavior as well as its ability to mediate axon growth dynamics in cultured embryonic neural tube explants.

Anterior column reconstruction of the thoracolumbar spine with a new modular PEEK vertebral body replacement device: retrospective clinical and radiologic cohort analysis of 48 cases with 1.7-years follow-up.

To evaluate whether a new PEEK vertebral body replacement can maintain the sagittal alignment as an anterior column reconstruction device in thoracic and lumbar spinal defects due to trauma or tumor. Retrospective review of 48 patients who underwent a corpectomy between T5 and L5 due to trauma or tumor and were stabilized with the new PEEK vertebral body replacement, between 2013 and 2017. We excluded patients who underwent a corpectomy for infection or degenerative disease and patients without complete follow-up in our institution. The primary outcome was the bi-segmental kyphotic angle (BKA). Secondary outcomes were the assessment of pedicle screw loosening, cage height, and subsidence or tilting of the cage. The clinical outcomes were assessed through the COMI-Score, EuroQol-5D, and Karnofsky indexes. Bony fusion and complications were registered. After the surgery BKA decreased by 12.1° (p < 0.001). At the end of the follow-up, we observed a mean loss of reduction of 1.6° (p = 0.002). This was accompanied by an increase in subsidence of 2.1mm (p < 0.001) and mean tilting of the cage of 1.4° (p = 0.003). The height of the cage and other parameters did not experience any changes. Clinically, the COMI-Score (p = 0.02) and the EuroQol-5D Index (p = 0.012) showed significant improvement, same as Karnofsky-Index (p = 0.015) at final follow-up. The fusion rate according to Bridwell was 92.1%. The 2% late complications were related to implant malpositioning. The new PEEK expandable vertebral body replacement is effective and safe in thoracic and lumbar anterior column reconstruction in tumor and trauma diseases.

Impact of Transanal Irrigation Device in the Management of Children With Fecal Incontinence and Constipation.

Children with fecal incontinence and constipation can be classified into 3 groups: neurogenic bowel dysfunction (NBD) related to spinal cord defects (NBD), refractory constipation (RC), or anorectal malformations (ARMs). The transanal irrigation (TAI) device (Peristeen) was approved in 2012 by the Food and Drug Administration. This system uses a pump rather than gravity to instill water as a colonic irrigant and uses balloon occlusion of the rectum. Our aim was to evaluate the effectiveness of TAI (Peristeen) in children who failed to respond to conservative measures for stool incontinence and constipation. Retrospective study of 147 patients prescribed TAI between January 2014 and January 2020. Data collected included demographics, prior bowel regimen, symptoms before and after, patient satisfaction scores, and NBD scores. Of the 147 patients initiated, 114 remain active users (13 lost to follow-up and 20 discontinued use). Multiple bowel regimens including laxatives (n = 139), cone enema (n = 40), and cecostomy (n = 7) were tried previously. The majority of our patients (n = 85) have NBD, primarily spina bifida, followed by RC (n = 43), and ARM (n = 19). For all patient groups, there was significant improvement in symptoms of fecal incontinence and constipation (P ≤ 0.001). Abdominal pain was improved in the NBD and RC group, but not significantly in the ARM group. We provide a single-center review of a large pediatric cohort using TAI (Peristeen) for management of fecal incontinence and constipation. Peristeen offered significant improvement in patients with NBD, RC, and ARM.

Abnormality of “butterfly” sign of choroid plexus: new ultrasound marker of fetal open spinal defects in the first trimester

Results of retrospective assessment of the choroid plexus pattern obtained from 17 fetuses with open spinal defects during ultrasound screening between 11 and 13 weeks of gestation are presented. To obtain the picture of the “butterfly” sign conducted a study of the axial plane of the fetal brain at the level of roof third ventricle with a correction of verticality. The absence of a typical “butterfly” picture is noted in all fetuses with spina bifida at 11–14 weeks of gestation. Prospective studies are needed to assess the effectiveness of the choroid plexus picture change for screening in the first trimester.

Effect of posture correction and moderate-intensity exercises on respiratory system in teenagers

Objective: Teenage is the time of shaping health behavior and preventing postural defects and improving respiratory fitness. The teenagers possess insufficient knowledge of their respiratory system. Postural defects are commonly seen among teenagers due to lack of physical activity and poor postural habits which can lead to non-harmonious development of organs and affect respiratory system as well. For efficient respiratory function, moderate-intensity exercises with posture correction would be useful. There is a paucity of literature showing the effect of posture correction and moderate-intensity exercises on respiratory system in teenagers, hence, this study would be helpful to analyze the same. The objectives of the study were to find out the effect of posture correction exercise on respiratory system in teenagers and to find out the effect of moderate-intensity exercises on respiratory system in teenagers. Materials and Methods: The study was carried out in Karad area. The subjects were selected according to the inclusion and exclusion criteria. A total of 35 subjects were participated in this study. Prior consent and assent form was taken. The aim and procedure were explained to the subjects in their preferred language before data collection. Pre- and post-assessment was done by 6 min walk test for respiratory function and flexicurve was measured using flexible ruler to recognize spinal postural defect. Peak flow meter was used to measure lung function. Results: In a conducted study, 6 min walk test, peak flow meter, and flexicurve showed statistically significant difference between pre- and post-treatment values. Conclusion: On the basis of the result of the study, it can be concluded that posture correction and moderate- intensity exercises help in improving respiratory function in teenagers.

In Utero Restoration of Hindbrain Herniation in Fetal Myelomeningocele as Part of Prenatal Regenerative Therapy Program at Mayo Clinic

ObjectiveTo assess our initial experience with prenatal restoration of hindbrain herniation following in utero repair of myelomeningocele (MMC). Patients and MethodsThree consecutive patients with prenatally diagnosed MMC (between January 1, 2018 and September 30, 2018) were managed with open in utero surgery. As per institutional review board approval and following a protocol designed at the Mayo Clinic Maternal & Fetal Center, fetal intervention was offered between 19 0/7 and 25 6/7 weeks of gestation. Prenatal improvement of hindbrain herniation was the declared restorative end point. Obstetrical and perinatal outcomes were also assessed. ResultsDiagnosis of MMC was confirmed upon referral between 20 and 21 weeks’ gestation by using fetal ultrasound and magnetic resonance imaging. In all cases reported here, the spinal defect was lumbosacral with evidence of hindbrain herniation. Open in utero MMC repair was performed between 24 and 25 weeks’ gestation with no notable perioperative complications. Postprocedure fetal magnetic resonance imaging performed 6 weeks after in utero repair documented improvement of hindbrain herniation. Deliveries were at 37 weeks by cesarean section without complications. Most recent postnatal follow-ups were unremarkable at both 11 months (baby 1) and 3 months of age (baby 2), with mild ventriculomegaly. Antenatal and postnatal follow-up of baby 3 at 1 month of age was also unremarkable. ConclusionOur study highlights the prenatal restoration of hindbrain herniation following in utero MMC repair in all cases presented here as an example of a prenatal regenerative therapy program in our institution.

Fetal profile in fetuses with open spina bifida

ObjectiveTo determine whether the frontomaxillary facial (FMF) angle and the prefrontal space ratio (PFSR) are helpful in screening for open spinal defects by ultrasound in the second and third trimesters of pregnancy.MethodsThe FMF angle and the PFSR were measured in fetuses with spina bifida according to standardized protocols. The normal range of the PFSR was previously published by our group. To determine the normal values for the FMF angle in the second and third trimesters of pregnancy, we used the same stored images from the above-mentioned study.Results71 affected and 279 normal fetuses were included in this study. Median gestational ages in the two groups were 21.1 weeks and 21.6 weeks, respectively. In fetuses with spina bifida, the FMF angle was significantly smaller than in the normal population (72.9° versus 79.6°). However, the measurement was below the fifth centile in only 22.5% of the affected fetuses. The PFSR was similar in both groups.ConclusionsThe FMF angle is smaller in second and third trimester fetuses with open spina bifida. However, the difference is not large enough to implement this marker in current screening programs.

Complex wound closure by plastic surgery following resection of spinal neoplasms minimizes postoperative wound complications in high-risk patients.

Wound breakdown and infection are common postoperative complications following resection of spinal neoplasms. Accordingly, it has become common practice at some centers for plastic surgeons to assist with closure of large posterior defects after spine tumor resection. In this study, the authors tested the hypothesis that plastic surgery closure of complex spinal defects improves wound outcomes following resection of spinal neoplastic disease. Electronic medical records of consecutive patients who underwent resection of a spinal neoplasm between June 2015 and January 2019 were retrospectively reviewed. Patients were separated into two subpopulations based on whether the surgical wound was closed by plastic surgery or neurosurgery. Patient demographics, preoperative risk factors, surgical details, and postoperative outcomes were collected in a central database and summarized using descriptive statistics. Outcomes of interest included rates of wound complication, reoperation, and mortality. Known preoperative risk factors for wound complication in spinal oncology were identified based on literature review and grouped categorically. The presence of each category of risk factors was then compared between groups. Univariate and multivariate linear regressions were applied to define associations between individual risk factors and wound complications. One hundred six patients met inclusion criteria, including 60 wounds primarily closed by plastic surgery and 46 by neurosurgery. The plastic surgery population included more patients with systemic metastases (58% vs 37%, p = 0.029), prior radiation (53% vs 17%, p < 0.001), prior chemotherapy (37% vs 15%, p = 0.014), and sacral region tumors (25% vs 7%, p = 0.012), and more patients who underwent procedures requiring larger incisions (7.2 ± 3.6 vs 4.5 ± 2.6 levels, p < 0.001), prolonged operative time (413 ± 161 vs 301 ± 181 minutes, p = 0.001), and greater blood loss (906 ± 1106 vs 283 ± 373 ml, p < 0.001). The average number of risk factor categories present was significantly greater in the plastic surgery group (2.57 vs 1.74, p < 0.001). Despite the higher relative risk, the plastic surgery group did not experience a significantly higher rate of wound complication (28% vs 17%, p = 0.145), reoperation (17% vs 9%, p = 0.234), or all-cause mortality (30% vs 13%, p = 0.076). One patient died from wound-related complications in each group (p = 0.851). Regression analyses identified diabetes, multilevel instrumentation, and BMI as the factors associated with the greatest wound complications. Involving plastic surgery in the closure of spinal wounds after resection of neoplasms may ameliorate expected increases in wound complications among higher-risk patients.

Arp2/3 inactivation causes intervertebral disc and cartilage degeneration with dysregulated TonEBP-mediated osmoadaptation

Extracellular matrix and osmolarity influence the development and homeostasis of skeletal tissues through Rho GTPase-mediated alteration of the actin cytoskeleton. This study investigated whether the actin-branching Arp2/3 complex, a downstream effector of the Rho GTPases Cdc42 and Rac1, plays a critical role in maintaining the health of matrix-rich and osmotically loaded intervertebral discs and cartilage. Mice with constitutive intervertebral disc- and cartilage-specific deletion of the critical Arp2/3 subunit Arpc2 (Col2-Cre; Arpc2fl/fl) developed chondrodysplasia and spinal defects. Since these mice did not survive to adulthood, we generated mice with inducible Arpc2 deletion in disc and cartilage (Acan-CreERT2; Arpc2fl/fl). Inactivation of Arp2/3 at skeletal maturity resulted in growth plate closure, loss of proteoglycan content in articular cartilage, and degenerative changes in the intervertebral disc at 1 year of age. Chondrocytes with Arpc2 deletion showed compromised cell spreading on both collagen and fibronectin. Pharmacological inhibition of Cdc42 and Arp2/3 prevented the osmoadaptive transcription factor TonEBP/NFAT5 from recruiting cofactors in response to a hyperosmolarity challenge. Together, these findings suggest that Arp2/3 plays a critical role in cartilaginous tissues through the regulation of cell-extracellular matrix interactions and modulation of TonEBP-mediated osmoadaptation.

Spinal dysraphism as a new entity in V.A.C.TE.R.L syndrome, resulting in a novel acronym V.A.C.TE.R.L.S.

Anorectal malformation (ARM) is the most common symptom in VACTERL syndrome (vertebral, anal, cardiac, tracheo-esophageal fistula, renal, and limb anomalies). The association of ARM and spinal dysraphisms (DYS) is well documented. We aim to better evaluate children with VACTERL association and ARM, considering the presence or not of DYS. Between 2000 and 2015, 279 children with VACTERL associations were identified in Necker Children's Hospital, Paris. We identified 61 VACTERL children (22%) with ARM. A total of 52 VACTERL children with ARM were included. DYS were identified in 36/52 of cases (69.2%). A total of 33 (63.5%) VACTERL children presented with sphincterial dysfunction. We constated that 28/33 (84.8%) of them had DYS + (p < 0.0001). More children in ARM (DYS +) subgroup are presenting with initial urinary sphincter dysfunction (58 vs 19%, p < 0.009) than ARM (DYS-). We identified 29 lipoma filum in our series, which were not statistically associated with urinary disorders (p = 0.143).Conclusion: We propose to refine the definition of VACTERL association, by adding S as Spinal defect to include it as an integral part of this syndrome, resulting in a novel acronym V.A.C.TE.R.L.S.What is Known:• The VACTERL association: congenital anomalies of the bony vertebral column (V), anorectal malformation (A), congenital cardiopathy (C), tracheo-esophageal defects (TE), renal and urinary tract anomalies (R), and limb malformations (L).• VACTERL children needs a complete appraisal, as early as possible, to adopt the most appropriate therapeutic management.What is New:• Include spine dysraphism (DYS) as a part of this syndrome, resulting in a novel acronym V.A.C.TE.R.L.S.• The significant correlation between VACTERL/DYS and urinary dysfunction requires to investigate the spine cord prenatally.

Evaluation of the spinal effects of phthalates in a zebrafish embryo assay

Phthalates (phthalate esters, PAEs) are commonly used as plasticizers and are emerging concerns worldwide for their potential influence on the environment and general public health. Thus, identification of the negative effects and involved mechanisms of PAEs is necessary. Herein, we found that embryonic exposure of zebrafish to di-(2-ethylhexyl) phthalate (DEHP) and di-butyl phthalate (DBP) significantly induced spinal defects, such as inhibited spontaneous movement at 24 h post-fertilization (hpf), spine curvature and body length decrease at 96 hpf. The transcriptional level of the genes that are related to the development of the notochord (col8a1a and ngs), muscle (stac3, klhl41a and smyd2b) and skeleton (bmp2, spp1) were significantly altered by DEHP and DBP at 50 and 250 μg/L, which might be associated with the observed morphological changes. Notably, DBP and DEHP altered the locomotor activity of zebrafish larvae at 144 hpf, which might be due to the abnormal development of the spine and skeletal system. In conclusion, phthalates caused spinal birth defects in zebrafish embryos, induced transcriptional alterations of the spinal developmental genes, and led to abnormal behavior.

Impact of Spinal Defects on Urinary and Sexual Outcome in Adults With Anorectal Malformations—A Cross-sectional Study

To examine the impact of spinal defects on urinary function, sexual outcome and quality of life in adult patients born with anorectal malformations. A prospective cohort study at Odense University Hospital in Denmark was conducted. From 1985 to 2000, 93 patients were found eligible for participation. Twenty-seven (29%) consented to participate. One patient refrained from clinical examinations. Patients were examined with magnetic resonance imaging, uroflowmetry, and validated questionnaires on urinary function, sexual function, and quality of life. There were 14 were females and 13 were males, median age of 25 (range 19-31) years and 23 (18-32) years, respectively. The type of anorectal malformations in females were vestibular fistula (n=6), anocutaneous fistula (n=4), anal stenosis (n=3), and cloaca (n=1). In males the type of malformations were anocutaneous fistula (n=4), bulbar fistula (n=4), rectovesical fistula (n=2), anal stenosis (n=1), rectal atresia (n=1), and anal atresia with no fistula (n=1). Patients with spinal defects had a lower average voiding rate compared to patients with normal spinal anatomy (P .03), a lower voiding-related quality of life (P .02), and a tendency was observed toward a worse total urinary incontinence-related quality of life score (P .06). Moreover in patients with spinal defect a tendency was seen toward a worse general quality of life (P .09). Spinal defects detected by magnetic resonance imaging in adults with anorectal malformations were found to be associated with urinary voiding function.

The epilepsy and intellectual disability-associated protein TBC1D24 regulates the maintenance of excitatory synapses and animal behaviors.

Perturbation of synapse development underlies many inherited neurodevelopmental disorders including intellectual disability (ID). Diverse mutations on the human TBC1D24 gene are strongly associated with epilepsy and ID. However, the physiological function of TBC1D24 in the brain is not well understood, and there is a lack of genetic mouse model that mimics TBC1D24 loss-of-function for the study of animal behaviors. Here we report that TBC1D24 is present at the postsynaptic sites of excitatory synapses, where it is required for the maintenance of dendritic spines through inhibition of the small GTPase ARF6. Mice subjected to viral-mediated knockdown of TBC1D24 in the adult hippocampus display dendritic spine loss, deficits in contextual fear memory, as well as abnormal behaviors including hyperactivity and increased anxiety. Interestingly, we show that the protein stability of TBC1D24 is diminished by the disease-associated missense mutation that leads to F251L amino acid substitution. We further generate the F251L knock-in mice, and the homozygous mutants show increased neuronal excitability, spontaneous seizure and pre-mature death. Moreover, the heterozygous F251L knock-in mice survive into adulthood but display dendritic spine defects and impaired memory. Our findings therefore uncover a previously uncharacterized postsynaptic function of TBC1D24, and suggest that impaired dendritic spine maintenance contributes to the pathophysiology of individuals harboring TBC1D24 gene mutations. The F251L knock-in mice represent a useful animal model for investigation of the mechanistic link between TBC1D24 loss-of-function and neurodevelopmental disorders.

Toxicity Assessment of 4 Azo Dyes in Zebrafish Embryos.

Azo dyes are used widely as color additives in food, drugs, and cosmetics; hence, there is an increasing concern about their safety and possible health hazards. In the present study, we chose 4 azo dyes tartrazine, Sunset Yellow, amaranth, and Allura red and evaluated their developmental toxicity on zebrafish embryos. At concentration levels of 5 to 50 mM, we found that azo dyes can induce hatching difficulty and developmental abnormalities such as cardiac edema, decreased heart rate, yolk sac edema, and spinal defects including spinal curvature and tail distortion. Exposure to 100 mM of each azo dye was completely embryolethal. The median lethal concentration (LC50), median effective concentration (EC50), and teratogenic index (TI) were calculated for each azo dye at 72 hours postfertilization. For tartrazine, the LC50 was 47.10 mM and EC50 value was at 42.66 mM with TI ratio of 1.10. For Sunset Yellow, the LC50 was 38.93 mM and EC50 value was at 29.81 mM with TI ratio of 1.31. For amaranth, the LC50 was 39.86 mM and EC50 value was at 31.94 mM with TI ratio of 1.25. For Allura red, the LC50 was 47.42 mM and EC50 value was 40.05 mM with TI ratio of 1.18. This study reports the developmental toxicity of azo dyes in zebrafish embryos at concentrations higher than the expected human exposures from consuming food and drugs containing azo dyes.

Neonatal Genital Prolapse

Neonatal genital prolapse is a rare condition, usually associated with congenital spinal defects and is often resistant to simple reduction. A case of complete uterine prolapse which was noted shortly after birth in a female baby with a meningocele at the lumbar region with rectal prolapse is reported. The vagina and uterus was restored to their normal position but came out immediately after reduction.

TECHNOLOGIA INFORMACYJNA W PRACY PŁOCKICH PRZEDSZKOLI NA PRZYKŁADZIE PUBLICZNEGO PRZEDSZKOLA NR 33 IM. JEAN’A VANIER I AKADEMII PRZEDSZKOLAKA MAX W PŁOCKU

In the proposed article, the authors present the results of research related to the use of information technology resources, which were conducted in two preschool institutions in Plock. The article emphasizes the importance of introducing the various means of modern information technology in the lessons. In preschool education, as in other fields of education, the using of information technology resources is very useful and has a positive attitude of the children, which increases the effectiveness of didactic and educational interactions in the educational process. The use of modern technologies is an attempt to achieve educational reform. It is also an opportunity to present interesting and innovative activities that accelerate and promote the acquisition of various skills. The author also emphasizes that the constant use of a computer can lead to addiction, which in turn will lead to aggression, fatigue, frustration, insomnia, and loss of health, such as impaired vision, severe spinal defects, caused by lack of movement. Such results are presented by numerous developments. The aim of the article is to find «the golden mean» — the appropriate ways, methods, forms of introducing information technology into the didactic process from the beginning of education, from an early age. The article presents an attempt to outline the problems associated with the introduction of ICT use and to inform about them. The data obtained as a result of the study highlight another issue: the arrangement of public kindergartens doesn’t improve the quality of teachers’ work. Many educators still avoid modern technologies (due to their age, lack of skills in operating modern equipment, beliefs, etc.). Young teachers who want to work on the basis of modern technology, they are much more open to such work. Therefore, it is important for the city to consider the possibility of introducing teacher training courses for the development of all innovations.

Use of an Omental Flow-Through Flap for Recipient Vessels in the Reconstruction of a Lumbar Spine Defect

Spine reconstruction after en bloc spondylectomy is challenging and may require multidisciplinary intervention. En bloc spine tumor resection with embolization of local recipient vessels for tumor control limits reconstructive options. Herein, we describe a case where combined efforts from orthopaedic, general, and plastic surgery teams permitted the successful reconstruction of a multilevel lumbar vertebral defect. A fibula-free flap within a titanium cage construct anastomosed to the left gastroepiploic vessels via a pedicled omental flow-through flap is a viable and novel method for reconstruction of a multilevel vertebral defect.

First trimester detection of fetal open spina bifida using BS/BSOB ratio.

Despite the well-known second trimester ultrasound signs, current possibilities of in utero surgical repair of open spina bifida require a timely detection of the spine defect. To evaluate the diagnostic accuracy of the ratio between brain stem (BS) diameter and its distance to the occipital bone (BSOB) (BS/BSOB ratio) in the detection of fetuses with open spina bifida at first trimester ultrasound. A systematic review and meta-analysis of diagnostic accuracy was performed by searching seven electronic databases from their inception to February 2019 for all studies assessing the association between BS/BSOB ratio and diagnosis of spine bifida. Diagnostic accuracy of BS/BSOB ratio in prenatal diagnosis of spine bifida was assessed as sensitivity, specificity, positive and negative likelihood ratios (LR + and LR-), and area under the curve (AUC) on SROC curves. Four studies, including 17,598 fetuses with 23 cases of open spina bifida, were included in the meta-analysis. BS/BSOB ratio showed pooled sensitivity of 0.70 (95% CI 0.47-0.87; I2 = 78.3%), specificity of 1.00 (95% CI 0.99-1.0; I2 = 99.2%), LR + and LR- of 51.44 (95% CI 9.53-277.64; I2 = 85.5%) and 0.23 (95% CI 0.04-1.17; I2 = 64.8%), respectively, and an AUC of 0.9649. First trimester BS/BSOB ratio has a high diagnostic accuracy in detecting fetuses with open spina bifida.

BIRTH DEFECTS OF HUMAN SPINAL COLUMN

Various anomalies of spinal column development have common clinical manifestations. However, each defect has certain features that affect the nature of the deformity, the rate of its progression, the function of the spinal cord and the possibility of developing neurological disorders. An increase in deformation in the anomaly zone can lead to segmental instability at this level, which further cause disturbance of the biomechanics of the spine in general. The cervical section is the most mobile section of the spine, which is provided by the structure of the Atlantic-axial complex and functional mobility of the subaxial section. The detected anomaly should be evaluated in terms of the likelihood of developing instability. Mechanical instability is manifested by the displacement of vertebrae beyond the physiological range of movements. Neurological instability is mostly manifested in the subaxial section of the spine, as the reserve space of the spinal canal is extremely small. Congenital abnormalities of the thoracic spine that lead to deformity are the most common defects against the ground of impaired formation of vertebral bodies. Children with congenital kyphosis are motionless, and dyspnea occurs while walking fast or jogging. Surgical treatment of kyphotic deformities of the thoracic and lumbar spine is one of the most difficult problems in modern orthopedics, the relevance of which is stipulated by a relatively high frequency of unsatisfactory results of treatment. Lumbarisation is a congenital malformation of the spine, accompanied by the formation of an additional lumbar vertebra, which is formed from the superior sacral vertebra that has not fused into a single bone with the other sacral vertebrae. The frequency of occurrence is unknown, because lumbarisation in some cases is asymptomatic and undiagnosed. Depending on the nature of anatomical changes and the peculiarities of the influence on the dynamic and static functions of the spine, unilateral and bilateral lumbarisation are distinguished. Both unilateral and bilateral forms can be bone, cartilage and joint. The development of pain is characteristic only of the joint form of lumbarisation. Caudal regression syndrome is a rare severe congenital malformation of the distal spine and spinal cord. Clinically the disease is accompanied by hypoplasia of the lower half of the trunk and extremities due to a gross defect in the development of the caudal spine and spinal cord. Depending on the level and severity of the lesion of the latter there is a different stage of neurological deficit. In most observations, caudal regression syndrome is combined with defects in other organs and systems. Depending on the level of damage to the spine, coccyx, sacral, lumbar and even lower thoracic vertebrae may be absent, which determines the variant of the defect. An extremely severe form of caudal regression is called sirenomelia, or "mermaid syndrome". A sign of this anomaly is the fusion of the lower extremities. The fusion can be osseous or within soft tissue. In most cases with sirenomelia renal agenesis is observed, the colon ends blindly, the external and internal genitalia are absent, a single umbilical artery and anus atresia are detected. The quality of life prognosis for patients with caudal regression syndrome depends on the severity of spinal and spinal cord defects, the severity of neurological disorders, and urological complications. Children need constant medical rehabilitation.

Anesthesia During Positive-pressure Myelogram: A New Role for Cerebral Oximetry.

Positive-pressure myelogram (PPM) is an emerging radiologic study used to localize spinal dural defects. During PPM, cerebrospinal fluid pressure (CSFp) is increased by injecting saline with contrast into the cerebrospinal fluid. This has the potential to increase intracranial pressure and compromise cerebral perfusion. We performed a retrospective chart review and analysis of 11 patients. The aim was to describe the periprocedural anesthetic management of patients undergoing PPM. All patients underwent PPM with general anesthesia and intra-arterial blood pressure and near-infrared spectroscopy monitoring of regional cerebral tissue oxygen saturation. Mean±SD maximum lumbar CSFp was 58±12 mm Hg. Upon intrathecal injection, mean systolic blood pressure increased from 115±21 to 142±32 mm Hg (P<0.001), diastolic blood pressure from 68±12 to 80±20 mm Hg (P≤0.001), and mean blood pressure from 87±10 to 98±14 mm Hg (P=0.02). Ten of 11 patients received blood pressure augmentation with phenylephrine to minimize the risk of reduced cerebral perfusion secondary to increased CSFp after intrathecal injection. The mean heart rate before and following injection was similar (68±15 vs. 70±15 bpm, respectively; P=0.16). There was a decrease in regional cerebral oxygen saturation after positioning from supine to prone position (79±10% to 74±9%, P=0.02) and a further decrease upon intrathecal injection (75±10% to 69±9%, P≤0.01). Systemic blood pressure increased following intrathecal injection during PPM, possibly due to a physiologic response to intracranial hypertension/reduced cerebral perfusion or administration of phenylephrine. Regional cerebral oxygen saturation decreased with the change to prone position and further decreased upon intrathecal injection. Cerebral near-infrared spectroscopy has a potential role to monitor the adequacy of cerebral perfusion and guide adjustment of systemic blood pressure during PPM.