Figure - available from: Child's Nervous System
This content is subject to copyright. Terms and conditions apply.
Illustrative case of a fetus that presented occipital encephalocele and trending to microcephaly that underwent to fetal correction at the 26th week of gestation. Left pictures illustrate the preoperative ultrasound and MRI evidencing the occipital encephalocele sac. Right pictures illustrate the postoperative MRI showing the correction of the Chiari type III
Source publication
The advance in the imaging tools during the pregnancy (ultrasound and magnetic resonance) allowed the early diagnose of many fetal diseases, including the neurological conditions. This progress brought the neurosurgeons the possibility to propose treatments even before birth. Myelomeningocele is the most recognized disease that can be treated durin...
Similar publications
p> Introduction: Neural tube defects are among the most common congenital malformations and a major cause of health problems in surviving children, especially in developing countries. Although the incidence of spinal dysraphism has significantly decreased over the last few decades, all over the world; however, the incidence is much higher in develo...
Introduction
In 2003, pediatric neurosurgeons were surveyed under the auspices of the education committee of the International Society for Pediatric Neurosurgery (ISPN) to determine prevailing opinions regarding the management of Chiari I malformation (C1M) with and without associated syringomyelia. In the ensuing years, there has been further info...
The use of endoscopic third ventriculostomy (ETV) for treatment of pediatric hydrocephalus has higher failure rates in younger patients. Here we investigate the impact of select perioperative variables, specifically gestational age, chronological age, birth weight, and surgical weight, on ETV failure rates. A retrospective review was performed on p...
Objective:
The goal of this study was to analyze the factors that have an impact on morbidity and mortality in patients with myelomeningocele (MMC).
Methods:
A retrospective cohort study was conducted to analyze factors associated with MMC that influence the morbidity and mortality of the disease. Data were collected from medical records of chil...
Among fetal surgical procedures, neurosurgery stands out due to the number of cases and the possibility of developing new procedures that can be performed in the fetal period. To perform fetal neurosurgical procedures, there is a need for specialized centers that have experts in the diagnosis of fetal pathologies and a highly complex obstetrics ser...
Citations
... [6] High-resolution images and ultrarapid sequences of one pondered burst in T2 or of one turbo burst allow the execution of the study without the need for fetal sedation and are sufficiently precise to carry out an analysis of the fetal anatomy. [4] e most important thing in the treatment of this pathology is the adequate prenatal diagnosis utilizing all the ancillary laboratory tests available, such as blood makers, obstetric ultrasound, magnetic resonance, and fetal karyotype. [3,4,8] Surgery remains the only treatment for encephalocele. ...
... [4] e most important thing in the treatment of this pathology is the adequate prenatal diagnosis utilizing all the ancillary laboratory tests available, such as blood makers, obstetric ultrasound, magnetic resonance, and fetal karyotype. [3,4,8] Surgery remains the only treatment for encephalocele. [2][3][4]6,7] The main objectives of surgery are the following: To reintegrate neural tissue, meninges, or CSF into the cranial cavity without causing neurologic deficit, repair the dural defect, and complete a cranioplasty if possible. ...
... [3,4,8] Surgery remains the only treatment for encephalocele. [2][3][4]6,7] The main objectives of surgery are the following: To reintegrate neural tissue, meninges, or CSF into the cranial cavity without causing neurologic deficit, repair the dural defect, and complete a cranioplasty if possible. [11] The majority of surgical techniques recommend the repair at the time of birth or within the first three postnatal months. ...
Background
Occipital encephalocele is a congenital defect of the neural tube at the level of the cranial midline, which results in herniation of meninges and brain tissue. The results of the management of myelomeningocele study determine the maternal and fetal risks for an open fetal surgery and have motivated the constant review of the concepts and strategies which the pediatric neurosurgeon can employ for the treatment of neural tube defects in the prenatal period.
Case Description
We present a case of a female patient in utero of 26 gestational weeks with the diagnosis of an occipital encephalocele treated by open fetal surgery. During week 20 of gestation, the diagnosis of occipital encephalocele was made by ultrasound, which was corroborated by fetal magnetic resonance that showed cranial protrusion of neural and meningeal content in the occipital region, measuring 1.6 × 2.8 × 3.3 cm with an approximate volume of 7.7 cc through a bone defect of 6 mm. The closure of the defect was performed by the postnatal surgical technique adapted to the open fetal surgery. Later, the patient was born transabdominal with a 2.8 cm occipital wound, with suture points and approximated borders, normocephalic, without clinical signs of sepsis, hydrocephalus, or overt neurologic compromise.
Conclusion
Open fetal surgery is a therapeutic option in the face of an isolated occipital encephalocele. This case report demonstrates the viability of the surgical procedure by the adaptation of a postnatal surgical technique to a prenatal surgery. Further studies are needed to evaluate the long-term functional results, comparing them with those seen in patients who undergo a postnatal procedure.
... The prevalence of encephalocele is estimated to be 0.8-5.0 per 10,000 live births. The most frequent encephalocele is the occipital encephalocele [1]. It is a congenital neural tube defect characterized by the protrusion or herniation of intracranial contents (meninges, brain, and a portion of the ventricles) through a cranial defect. ...
Background
The prevalence of encephalocele is estimated to be 0.8-5.0 per 10,000 live births. The most frequent encephalocele is the occipital encephalocele. It is a congenital neural tube defect characterized by the protrusion or herniation of intracranial contents through a cranial defect. The term "giant/massive/large encephalocele" is used to describe an encephalocele that is significantly larger than the size of the head.
Case description
A 2-month-old male infant presented in the neurosurgery outpatient department with one of the largest head masses over the posterior aspect since birth. The swelling was gradually progressive and developed ulceration over the swelling with intermittent cerebrospinal fluid (CSF) discharge but no associated weakness in limbs. Magnetic Resonance Imaging (MRI) brain showed a large occipital meningoencephalocele containing predominantly cyst with part of the cerebellar and occipital lobe. The surgery was planned. The sac contained CSF with the gliotic occipital lobe. The sac and gliotic brain tissue was excised. He had an uneventful postoperative course.
Conclusion
Surgery serves several functions, including reducing the torque and weight of the head to allow for more normal motor development, removal of the thin, leaking scalp and dural closure to prevent CSF leak and subsequent infection, and improving the cosmetic and social issues that the child and family may have to endure.
... Regardless of the timing of the surgical intervention, the release of the tethered cord is a vital stage of a MMC repair [26]. The ligament which connects the placode to the dura mater is observed in 90% of the fetuses undergoing surgery [26]. ...
... Regardless of the timing of the surgical intervention, the release of the tethered cord is a vital stage of a MMC repair [26]. The ligament which connects the placode to the dura mater is observed in 90% of the fetuses undergoing surgery [26]. In case of fetoscopic surgeries, where OSB is merely covered with skin graft or biological material, the cord is not released, which constitutes a significant limitation of the surgical intervention. ...
Objectives:
The aim of the study was to determine the effectiveness of an alternative method of open fetal surgery to prevent severe unfavorable prenatal events, both for the mother and the fetus.
Material and methods:
In this study, the previously published results for a cohort of 46 patients, who had undergone intrauterine myelomeningocele repair (IUMR) at our Center by 2014, constituted the retrospective control group (CG). The MOMS protocol had been applied for hysterotomy, with an automatic uterine stapling device. The study group (SG) n = 57 was assembled during a prospective observation. IUMR was performed using an alternative method of hysterotomy, with the typical opening and closure of the uterus, without automatic stapling device, as described by Moron et al. Additionally, our single-center results were compared with the post-MOMS findings of other centers: CHOP (Children' S HOSPITAL OF PHILADELPHIA: ) and VUMC (Vanderbilt University Medical CENTER: ).
Results:
No cases of delivery before 30 weeks of gestation (0%, 0/55) were observed in the study group, which is a statistically significant difference (p < 0.05) as compared to controls (15/44). Statistically significantly lower incidence of chorioamniotic separation (5.4% (3/55) vs CHOP 22.9% (22/96), p < 0.001) and contractile activity resulting in preterm labor (16.3% (9/55) vs CHOP 37.5% (36/96), p < 0.05) was found in the study group. Premature rupture of the membranes was statistically significantly less common in the study group as compared to controls, CHOP and VUMC (SG 12.7% (7/55) vs CG 52.2% (24/46), p < 0.001; vs CHOP 32.3% (31/96), p < 0.001; vs VUMC 22% (9/43), p < 0.01, respectively).
Conclusions:
The presented IUMR method is associated with improved perinatal outcomes, i.e., lower rates of preterm delivery at < 30 weeks of gestation, preterm premature rupture of membranes, and uterine contractility resulting in preterm delivery. That, in turn, results in lower prematurity rates and, consequently, more favorable neonatal outcomes.
... Esto puede ser un desafío en la columna sacra, porque la osificación de L5 es a las 16 semanas, los arcos S1 y S2 no se osifican hasta las 19 y 22 semanas 21 . Este es el momento en que se evalúa a los pacientes para cirugía fetal y se entiende porque los pacientes de 26 semanas de gestación o más ya no se consideran candidatos para cirugía fetal 21,22 . ...
... Los hallazgos ecográficos de la MC-II son "signo del limón" y "signo del plátano". El signo del limón es la descripción que se da a la concavidad bifrontal del cráneo, mientras que el signo del plátano está en relación con la envoltura del cerebelo, alrededor del tronco encefálico, con borramiento asociado del cuarto ventrículo y de la cisterna magna, la dolicocefalia es otro hallazgo común marcado por un índice cefálico menor al percentil setenta 21,22 . ...
... El ultrasonido de las extremidades inferiores es importante desde una perspectiva anatómica y funcional, la presencia o ausencia de deformidad del pie equinovaro, es un factor de predicción importante de la futura deambulación independiente y se observa en aproximadamente un tercio de los fetos con DTN abierto 21,22 . ...
Introducción: El mielomeningocele fetal, es la extrusión de la médula espinal que ocurre por un cierre incompleto del neuróporo caudal, lo cual expone la placa neural al trauma mecánico y químico de la pared uterina y líquido amniótico respectivamente, con graves secuelas. La detección prenatal innovó el diagnóstico y con ello la reparación intra útero ha mejorado estrategias del tratamiento, generando opciones de atención en aquellas pacientes que cumplen con los criterios de selección para cirugía prenatal. Objetivo: Presentar una revisión bibliográfica sobre diagnóstico, manejo y tratamiento del mielomeningocele fetal y ofrecer a la comunidad científica una herramienta de consulta para mejorar los conocimientos del tema y alternativas de tratamiento oportuno para los fetos con esta malformación. Metodología: Se trata de un estudio de revisión sistemática sin metaanálisis, realizado en base a las recomendaciones PRISMA. La búsqueda de información se estructuró bajo el sistema PICO. Las búsquedas se realizaron en Pubmed, Tripdatabase y Pubmed Central; seleccionando artículos publicados durante los últimos diez años en inglés o español, sobre diagnóstico, manejo y tratamiento del mielomeningocele fetal. Resultados: 120 artículos cumplieron con criterios de búsqueda, de los cuáles fueron elegibles 42, con información relevante para determinar el diagnóstico, manejo y tratamiento actual del mielomeningocele fetal a través de procedimientos innovadores. Discusión: Los defectos del tubo neural aparecen como consecuencia de una alteración del proceso de neurulación entre el día 21-28 después de la concepción. El mielomeningocele fetal es considerado el defecto congénito no letal más común del SNC. Se caracteriza por la protrusión de las meninges y la médula espinal con daño neurológico permanente. Por ello el diagnóstico y manejo oportuno de esta patología, han permitido que la cirugía fetal intra útero se considere el método óptimo, mejorando la hernia del rombencéfalo, reduciendo la necesidad de una derivación ventricular y manteniendo la motricidad inferior, así como la función neuronal, vesical y gastrointestinal, mejorando la calidad de vida del paciente afecto por esta patología.
... The rates of perinatal mortality vary from 1,8 to 6,0% depending on the experience in different centers. Paulista Medical School performed a trial with 220 cases showing perinatal mortality rate of 1,8%; MOMS trial − 2,5% and the Children's Hospital of Philadelphia (CHOP) − 6% [4,12,14]. ...
We present the rst case of meningomyelocele (MMC) in utero repair with the bovine pericardial patch performed in Belarus. We followed the maternal and fetal inclusion and exclusion criteria used by the MOMS trial. Prenatal fetal magnetic resonance imaging (MRI) shows the anomaly of cerebellar tonsils, brain stem herniation and MMC at the level of L5-S1 vertebra. During the standard surgical hysterectomy, we performed exposure of the fetus and applied microsurgical repair of the MMC defect with the use of bovine pericardial patch. In our case, we demonstrated the use of bovine pericardial patch for MMC defect repair as optimal and safe material which resulted into skin epithelialization by the rst month of life.
... 6,7 Ultrasonography is the gold standard for diagnosing congenital hydrocephalus. 8 Depending on the extent of ventriculomegaly and presence of other congenital anomalies, hydrocephalus is classified as mild, moderate, or severe. 8,9 Literature about the clinical outcome of congenital hydrocephalus according to its severity is sparse. ...
... 8 Depending on the extent of ventriculomegaly and presence of other congenital anomalies, hydrocephalus is classified as mild, moderate, or severe. 8,9 Literature about the clinical outcome of congenital hydrocephalus according to its severity is sparse. 9,10 Considering all the above-mentioned facts, the present study was aimed to determine the incidence, risk factors, severity, and outcome of congenital hydrocephalus in our institute, which is a tertiary healthcare center located in northern India. ...
... Extrinsic causes of hydrocephalus include hemorrhage, neoplasm, infection (bacterial meningitis, CMV infection, toxoplasmosis.), and drug/alcohol intake. 3 Developmental hydrocephalus is often associated with several pediatric syndromes comprising of anomalies such as myelomeningocele, aqueductal stenosis, posterior fossa crowding, etc. 8 Numerous genes such as L1CAM, AP1S2, POMT, MPDZ, FUZ, VANGL1, CELSR1, OFD1, GPSM2, FGFR, and NF1 have also been implicated in its pathogenesis. 3,5 Once the diagnosis is confirmed via ultrasonography, the parent's apprehension needs to be tackled with great care. ...
Background: Hydrocephalus is a heterogeneous disease marked by abnormal dilatation of the cerebral ventricles secondary to varying etiologies. This study was aimed to determine the incidence, risk factors, severity, and outcome of hydrocephalic fetuses presenting to a tertiary healthcare hospital located in northern India.Methods: In this prospective observational study, pregnant women visiting the obstetrics outpatient department of our hospital from 01 July 2017 to 31 June 2018 were screened for hydrocephalic fetuses via ultrasonography along with a detailed history, and a comprehensive battery of diagnostic investigations. They were followed up for a minimum period of two months after delivery/termination of pregnancy.Results: A total of 3627 pregnant women were screened, of which 10 had hydrocephalic fetuses in the observed time period. The incidence of hydrocephalus was determined to be 2.75 per 1000 live births. Low socio-economic status was identified as a major risk factor. 50% of the hydrocephalic fetuses were severely afflicted and were discontinued. The remaining 50% were successfully delivered and were managed via a ventriculo-peritoneal shunt or are under close observation in the postnatal period without any adverse outcome.Conclusions: The burden of hydrocephalus is considerably high in India, as compared to western countries. In rural settings, low socioeconomic status and lack of folic acid supplementation have a major influence in the etiopathogenesis of hydrocephalus. Management of hydrocephalus requires a multidisciplinary approach and is tailored according to the severity of the presentation. Severe cases of hydrocephalus and cases with associated anomalies have a poor prognosis.
... The advance in the prenatal diagnostics, particularly in imaging tools during the pregnancy (ultrasound and magnetic resonance) allowed the early diagnose of many fetal diseases, including the neurological conditions. This progress brought the neurosurgeons the possibility to propose treatments even before birth (1) . ...
... Amniocentesis, the first available prenatal chromosomal diagnostic testing option, was first described in the 1950s (1) . Amniocentesis became increasingly safe and is now used for several purposes, including genetic screening and infectious evaluations. ...
Background: The advance in the prenatal diagnostics, particularly in imaging tools during the pregnancy (ultrasound and magnetic resonance) allowed the early diagnose of many fetal diseases, including the neurological conditions. This progress brought the neurosurgeons the possibility to propose treatments even before birth. Objective: The aim is to study the role and effect of prenatal diagnostics for neurosurgical pathologies as regard to early detection and management. Conclusion: Further progress is necessary to enable fetal neurosurgery in becoming the main technique used in treating fetal neurosurgical diseases. However, we believe that correct prenatal diagnosis and adequate selection of fetuses with myelomeningocele, hydrocephalus, and occipital encephalocele may contribute to the benefits provided by neurosurgical procedures during the fetal period. Keywords: Prenatal Diagnostics, Neurosurgical Pathologies
... The commonest neurosurgical anomaly treated during antenatal period with a high success rate is myelomeningocele. [9] The limited number of fetal repair of encephaloceles has also been reported in the literature. [9] Surgical procedures for giant occipital encephalocele are a challenging task, not only for neurosurgeons, but also for the anesthesiologists. ...
... [9] The limited number of fetal repair of encephaloceles has also been reported in the literature. [9] Surgical procedures for giant occipital encephalocele are a challenging task, not only for neurosurgeons, but also for the anesthesiologists. These challenges are due to its site, large size, contents, associated intracranial anomalies, intraoperative blood loss, intraoperative hypothermia, and prolonged anesthesia. ...
... [7] Encephalocele has an estimated prevalence of 0.8-5.0 per 10,000 live births. [8,9] Occipital encephalocele is the most common of all encephaloceles. [1,2] Depending on the anatomical location of encephalocele, it can be named as occipital, anterior, parietal, temporal, and vertex encephalocele. ...
... Although entry and closure techniques of the hysterotomy were not specifically enquired about, four centres reported using a different uterine entry technique to the auto-stapling device (US Surgical CS-57, Covidien, USA) described in MOMS, in order to perform a 6 to 8-cm hysterotomy. 18,19 Two centres reported using a "mini" (less than 4 cm) rather than a wide hysterotomy for uterine access. One centre reported a modified hysterotomy closure technique, using three rather than two layers. ...
... Encephalocele is a cranial bone defect, which is characterized by protrusion of the meninges and/or neural tissue through the defect. 1 This sac-like protrusion is covered by skin. The incidence of this abnormality is 1-5/10 000 live births. ...
... Half of the patients who live beyond the first day have various degrees of neurodevelopmental delays. 1 Occipital encephaloceles may be accompanied by other congenital abnormalities including of the central nervous system. These associated abnormalities are important for prognosis. ...
Posterior encephalocele is a neural tube defect, which is a sac‐like protrusion of the neural tissue and cerebrospinal fluid through a defect in the occipital bone. This embryonic anomaly may coexist with cortical dysplasia, agenesis of the corpus callosum, hydrocephalus, microcephaly, craniofacial abnormalities, ventricular and atrial septal defect. We report a case of a large posterior encephalocele in a fetus accompanied by unexpected major abnormalities including transposition of the great arteries, severe ventriculomegaly and cerebellar atrophy. Postnatal surgical corrections of the posterior encephalocele and then of the transposition of the great arteries were performed but the neonate died 2 months after delivery.
