Hidrocefalia infantil en el África subsahariana: impacto de los cuidados perioperatorios en el archipiélago de Zanzíbar / Infant hydrocephalus in sub-Saharan Africa: Impact of perioperative care in the Zanzibar archipelago

INTRODUCCIÓN: La hidrocefalia infantil en países de bajo y mediano ingreso, representa uno de los problemas éticos y sanitarios más conflictivos con los que se enfrenta el desarrollo de la salud a nivel internacional. Las estimaciones más optimistas señalan que 200.000 nacidos anualmente desarrollarán una hidrocefalia o nacerán con un defecto del tubo neural en África oriental, central y sur de África (ECSA). Se calcula que menos del 10% de estos niños serán operados mediante derivaciones ventriculoperitoneales y, en general, en condiciones de mala calidad o con un índice de complicaciones muy elevado. OBJETIVO: Describir las características generales, la epidemiología y los datos demográficos de la hidrocefalia infantil de los pacientes atendidos en el Instituto NED en el archipiélago de Zanzíbar y valorar los detalles clínicos y los resultados a medio plazo del impacto de los cuidados de enfermería implantados. MATERIAL Y MÉTODOS: Se trata de un estudio observacional descriptivo y analítico de carácter retrospectivo, en pacientes diagnosticados y tratados de hidrocefalia infantil, en el período comprendido entre septiembre del 2016 y septiembre del 2018. Con la implantación de una serie de protocolos perioperatorios de enfermería en dichos pacientes, se describieron y analizaron los resultados obtenidos. RESULTADOS: Un total de 96 pacientes fueron atendidos de hidrocefalia infantil. Un 51% (n = 49) de estos pacientes eran varones, con una media de edad de 9,25 meses. Todas las madres de los pacientes fueron controladas durante el embarazo, pero solamente un 8% de ellas fueron tratadas con ácido fólico durante su gestación. El 81% de los niños nacieron mediante parto vaginal o parto espontáneo no complicado. Con respecto a la etiología, el 27,1% de la hidrocefalia tratada estaba asociada a una causa infecciosa y un 35,4% a una causa desconocida. Se realizaron 67 cirugías de derivación ventriculoperitoneal y 15 ventriculostomías endoscópicas. La tasa de complicaciones fue del 23,17%. CONCLUSIONES: Los resultados de esta investigación indican que la hidrocefalia infantil en Zanzíbar tiene una etiología, una evolución y unas complicaciones similares o menores que las descritas hasta la fecha en el África oriental. El hecho de implantar una serie de protocolos perioperatorios y cuidados estandarizados de enfermería influyen positivamente en los resultados obtenidos. En la actualidad, el Mnazi Mmoja Surgical NED Institute es uno de los escasos centros en África del Este con un registro exhaustivo de la actividad asistencial y el primer centro sanitario que oferta una formación continuada al personal de enfermería

INTRODUCTION: Child hydrocephalus in low- and middle-income countries represents one of the most sensitive ethical and health problems facing international health development. The most optimistic estimates indicate that 200,000 newborns annually will develop hydrocephalus or be born with a neural tube defect in East, Central and South Africa (ECSA). It is estimated that less than 10% of these children will be operated by ventriculoperitoneal shunts, and in general in poor quality conditions or with a very high complication rate. OBJECTIVE: To describe the general characteristics, epidemiology and demographic data of childhood hydrocephalus of patients treated at the NED Institute in the Zanzibar archipelago, and assess the clinical details and medium-term results of the impact of the set-up nursing care. MATERIAL AND METHODS: This is a descriptive and analytical observational study of a retrospective nature, in patients diagnosed and treated with childhood hydrocephalus, in the period from September 2016 to September 2018. With the implementation of a series of perioperative nursing protocols in these patients, the results obtained were described and analyzed. RESULTS: A total of 96 patients were treated for childhood hydrocephalus. 51% (n = 49) of these patients were male, with a mean age of 9.25 months. All the mothers of the patients were monitored during pregnancy, but only 8% were treated with folic acid during pregnancy. 81% of children were born through vaginal delivery or uncomplicated spontaneous delivery. Regarding the etiology, 27.1% of treated hydrocephalus was associated with an infectious cause and 35.4% with an unknown cause. 67 ventriculoperitoneal shunt surgery and 15 endoscopic ventriculostomies were performed. The complication rate was 23.17%. CONCLUSIONS: The results of this research indicate that childhood hydrocephalus in Zanzibar has etiology, evolution and complications that are similar to or less than those described to date in East Africa. Implementing a series of perioperative protocols and standardized nursing care positively influences the results obtained. Currently, the Mnazi Mmoja Surgical NED Institute is one of the few centers in East Africa with an exhaustive record of healthcare activity and is the first health center that offers further training to nurses

Single-injection ex ovo transplantation method for broad spinal cord engraftment of human pluripotent stem cell-derived motor neurons.

BACKGROUND: Transplantation of human pluripotent stem cell (hPSC)-derived neurons into chick embryos is an established preliminary assay to evaluate engraftment potential. Yet, with recent advances in deriving diverse human neuronal subtypes, optimizing and standardizing such transplantation methodology for specific subtypes at their correlated anatomical sites is still required. NEW METHOD: We determined the optimal stage of hPSC-derived motor neuron (hMN) differentiation for ex ovo transplantation, and developed a single injection protocol that implants hMNs throughout the spinal cord enabling broad regional engraftment possibilities. RESULTS: A single injection into the neural tube lumen yielded a 100% chick embryo survival and successful transplantation rate with MN engraftment observed from the rostral cervical through caudal lumbar spinal cord. Transplantation of HB9+/ChAT- hMN precursors yielded the greatest amount of engraftment compared to Pax6+/Nkx6.1+/Olig2+ progenitors or mature HB9+/ChAT+ hMNs. COMPARISON WITH EXISTING METHOD(S): Our single injection hMN transplant method is the first to standardize the optimal hMN phenotype for chick embryo transplantation, provide a rubric for engraftment quantification, and enable broad engraftment throughout the spinal cord with a single surgical intervention. CONCLUSION: Transplantation of HB9+/ChAT- hMN precursors into chick embryos of Hamburger Hamilton (HH) stages 15-18 using a single luminal injection confers a high probability of embryo survival and cell engraftment in diverse regions throughout the spinal cord.

Tratamento cirúrgico de úlcera por pressão na unidade de Pediatria de hospital de reabilitação / Pressure ulcer treatment in the Pediatric Unit of a Rehabilitation Hospital

Introdução: A úlcera por pressão (UPP) é uma das complicações mais comuns em portadores de lesão medular, dos mais graves e frequentes problemas destes pacientes. A incidência e evolução depende dos grupos estudados, tendo as crianças e adolescentes características específicas durante o crescimento. A maior parte das descrições na literatura referem-se a adultos que desenvolveram UPP durante internação. O objetivo é descrever os resultados de pacientes operados para fechamento de úlcera por pressão na unidade de Pediatria do Hospital Sarah Brasília. Métodos: Estudo descritivo, retrospectivo de pacientes operados de 2005 a 2010 devido à lesão por pressão e análise estatística. Resultados: 116 pacientes foram admitidos para tratamento cirúrgico de UPP no Hospital Sarah, sendo 20 (17,2%) internados na Pediatria; 15 (75%) homens e 5 (25%) mulheres, todos provenientes de ambiente comunitário; 60% localizadas na região isquiática; com seguimento de 15 anos; 70% eram portadores de malformação do tubo neural. Os procedimentos cirúrgicos foram indicados para úlcera por pressão grau III e IV. Utilizou-se retalho do músculo glúteo (3), em ilha (8) ou península (3), fechamento primário e coccigectomia; retalho do músculo tensor da fáscia lata. Três casos apresentaram complicações, 65% evoluíram com recidiva e 15% com surgimento de novas lesões na região pélvica. Conclusão: A principal causa de lesão por pressão em Pediatria foi devido à malformação do tubo neural (70%), adquiridas na comunidade, localizadas na região isquiática. Embora estivessem em atendimento no programa de reabilitação, ainda foi observado alto índice de recidivas (65%).

Introduction: Pressure ulcers (PUs), or pressure sores, are among the most common serious complications in patients with spinal cord injury. The incidence and evolution in children and adolescents have specific characteristics. Most prior reports evaluated adults who developed PUs in the hospital. The objective is to describe the outcomes of surgical PU closure in children and adolescents in the Hospital Sarah Brasília pediatric unit. Methods: This was a retrospective statistical analysis of patients who underwent surgery for PUs. Statistical analysis of the incidence of PUs was performed using Epi Info version 3.2.2. Results: Of 116 patients who underwent surgery for PUs at the Hospital Sarah between 2005 and 2010, 20 (17.2%) were admitted to the Pediatric Unit. All 15 (75%) male and 5 (25%) female patients were admitted from a community setting. PUs were located in the ischial region in 60% of patients. The followup period was 15 years. Neural tube malformations were present in 70% of patients. We performed surgery for grade III and IV PUs, using gluteal flaps, primary closure and coccygectomy, and tensor fascia lata flaps. Three cases had complications, 65% developed recurrences, and 15% developed new ulcers in the pelvic region. Conclusion: The main cause of PUs in pediatric cases admitted to the rehabilitation hospital was a neural tube malformation (70%) in the ischial region. Despite admission to the rehabilitation program, a high recurrence rate (65%) was observed.

Unjoined primary and secondary neural tubes: junctional neural tube defect, a new form of spinal dysraphism caused by disturbance of junctional neurulation.

INTRODUCTION: Primary and secondary neurulation are the two known processes that form the central neuraxis of vertebrates. Human phenotypes of neural tube defects (NTDs) mostly fall into two corresponding categories consistent with the two types of developmental sequence: primary NTD features an open skin defect, an exposed, unclosed neural plate (hence an open neural tube defect, or ONTD), and an unformed or poorly formed secondary neural tube, and secondary NTD with no skin abnormality (hence a closed NTD) and a malformed conus caudal to a well-developed primary neural tube. METHODS AND RESULTS: We encountered three cases of a previously unrecorded form of spinal dysraphism in which the primary and secondary neural tubes are individually formed but are physically separated far apart and functionally disconnected from each other. One patient was operated on, in whom both the lumbosacral spinal cord from primary neurulation and the conus from secondary neurulation are each anatomically complete and endowed with functioning segmental motor roots tested by intraoperative triggered electromyography and direct spinal cord stimulation. The remarkable feature is that the two neural tubes are unjoined except by a functionally inert, probably non-neural band. CONCLUSION: The developmental error of this peculiar malformation probably occurs during the critical transition between the end of primary and the beginning of secondary neurulation, in a stage aptly called junctional neurulation. We describe the current knowledge concerning junctional neurulation and speculate on the embryogenesis of this new class of spinal dysraphism, which we call junctional neural tube defect.

Tratamiento prenatal del mielomeningocele / Prenatal treatment of myelomeningocele

El mielomeningocele es la malformación congénita, dentro de los defectos del tubo neural, más grave compatible con la vida. El diagnóstico prenatal suele realizarse en la ecografía morfológica aunque recientemente se han descrito marcadores precoces de primer trimestre. En 2011 se publicó el estudio Management of Mielomeningocele Study (MOMS), estudio aleatorizado comparando los fetos operados prenatalmente con los operados postnatalmente. Los resultados mostraron la reducción de la necesidad de derivaciones ventrículo-peritoneales y una mejoría de la función motora con la intervención prenatal sin reportar una importante morbilidad materna. Desde hace años, en el Hospital Universitari Vall d’Hebron se está trabajando en experimentación animal, inicialmente mediante la creación de un modelo animal de mielomeningocele y posteriormente de diferentes técnicas de reparación. Esta investigación traslacional ha sido aplicada a la práctica clínica. Desde el año 2010 se ofrece un programa multidisciplinar de cirugía prenatal del mielomeningocele(AU)

Myelomeningocele is the most severe congenital malformation among neural tube defects that are compatible with life. Although prenatal diagnosis is usually performed with the 20-22nd week scan, early first trimester markers have been recently described. Management of Myelomeningocele Study (MOMS), a randomized study that compares the prenatally operated fetuses with those that were operated on post-natally, was published in 2011.The results showed a reduction in the need for peritoneal shunts and improved motor function with the prenatal intervention without reporting any significant maternal morbidity. The Hospital Universitari Vall d’Hebron has been working on animal experimentation for many years. Initially, they created an animal model of myelomeningocele, and later on developed several repair techniques. This translational research has been applied to clinical practice. Since 2010, we have offered a multidisciplinary program of prenatal myelomeningocele surgery(AU)

Optimisation of in ovo electroporation of the chick neural tube.

Chick in ovo neural tube electroporation has become a widely used method for assaying gene function during embryonic development. Since its first description, many variants of this technique have been described, with varying values for specific parameters such as electrode type and spacing, voltage, pulse duration and plasmid DNA concentration. Here we examine the influence of some of these variables and derive a detailed and optimal protocol for electroporating the caudal neural tube during the third day of embryonic development. Our findings highlight the importance of electrode placement and DNA dilution buffer for optimal expression and absence of electroporation artifacts.

[Microanatomic and three-dimensional reconstruction study of lateral wall and related structures of optic canal].

OBJECTIVE: To investigate the key microanatomic and radiological structures of optic canal comprehensively so as to provide anatomic parameters and procedural flows for the decompression of optic canal. METHODS: Gross observations and microscopic measurements were applied on 10 (20 sides) formalin-treated cadaveric specimens and 15 (30 sides) adult skulls. Using multislice helical CT (computed tomography)-aided three-dimensional reconstruction in combination with direct anatomic measurement, the investigators dissected, photographed, measured and analyzed the shape of optic canal and analyze its anatomic relationship with the adjoining structures. RESULTS: Optic canal was formed by the superior, inferior, medial and external walls and distal proximal opening. The lateral wall of optic canal was formed by anterior clinoid process with a length of (9.87 ± 1.34) mm, a width of (11.66 ± 2.35) mm, a base thickness of (5.35 ± 1.07) mm and a middle thickness of (4.50 ± 1.06) mm. Optic strut separating the optic canal from the superior orbital fissure was located inferiorly. And the distance between the apex of anterior clinoid process and the middle of ICA (internal carotid artery) groove was (4.25 ± 2.30) mm. The CSF (cerebrospinal fluid) leakage and secondary injury of optic nerve and injury of ICA, ophthalmic artery might occur during the surgical procedures due to the variation of anterior clinoid process. The microanatomic figures and radiological measurements had a mean difference very close to each other at (0.08 - 0.48) mm. No statistical difference was found (P > 0.05). CONCLUSION: Optic nerve, ophthalmic artery and ICA may be exposed by a high-speed drilling of the lateral wall of optic canal. The drilling dissection of lateral wall plays a vital role during a successful optic canal decompression. Radiological measurement and three-dimensional reconstruction of skull base may be of great clinical significance in lesion visualization. And it helps to make a better choice of surgical approaches. The measurements provide valuable references for surgeons and researchers.

Xenotransplantation of human stem cells into the chicken embryo.

The chicken embryo is a classical animal model for studying normal embryonic and fetal development and for xenotransplantation experiments to study the behavior of cells in a standardized in vivo environment. The main advantages of the chicken embryo include low cost, high accessibility, ease of surgical manipulation and lack of a fully developed immune system. Xenotransplantation into chicken embryos can provide valuable information about cell proliferation, differentiation and behavior, the responses of cells to signals in defined embryonic tissue niches, and tumorigenic potential. Transplanting cells into chicken embryos can also be a step towards transplantation experiments in other animal models. Recently the chicken embryo has been used to evaluate the neurogenic potential of human stem and progenitor cells following implantation into neural anlage. In this video we document the entire procedure for transplanting human stem cells into the developing central nervous system of the chicken embryo. The procedure starts with incubation of fertilized eggs until embryos of the desired age have developed. The eggshell is then opened, and the embryo contrasted by injecting dye between the embryo and the yolk. Small lesions are made in the neural tube using microsurgery, creating a regenerative site for cell deposition that promotes subsequent integration into the host tissue. We demonstrate injections of human stem cells into such lesions made in the part of the neural tube that forms the hindbrain and the spinal cord, and into the lumen of the part of the neural tube that forms the brain. Systemic injections into extraembryonic veins and arteries are also demonstrated as an alternative way to deliver cells to vascularized tissues including the central nervous system. Finally we show how to remove the embryo from the egg after several days of further development and how to dissect the spinal cord free for subsequent physiological, histological or biochemical analyses.

Encefalocele occipital: reporte de un caso / Occipital encephalocele: a case report

Presentamos el caso de un recién nacido femenino, con encefalocele occipital, diagnosticado en el período perinatal a las 29 semanas de gestación; atendido en los Servicios de Obstetricia y Neurocirugía del Hospital Universitario de Caracas. Se realizó una revisión la literatura dada la poca frecuencia de esta patología

We presente a case of female newborn child with occipital encephalocele diagnosed at 29 weeks of pregnancy; being attended in the Service of Obstetrics and Neurosurgery of the Hospital Universitario de Caracas. A literature review was done by infrecuent pathology