Prenatal and postnatal evaluation for syringomyelia in patients with spinal dysraphism (original) (raw)

Prenatal ultrasound diagnosis of neural tube defects in the era of intrauterine repair e Eleven years' experiences

Taiwan Journal of Obstetrics and Gynecology, 2021

Objective: To modify the current neural tube defect (NTD) classification for fetal medicine specialists, and to investigate the impact of prenatal ultrasound conus medullaris position screening on the detection rate of closed spinal dysraphism and pregnancy outcomes. Materials and methods: The clinical data of 112 patients prenatally diagnosed with neural tube defects in Taiji clinic from 2008 to 2018 were retrospectively analyzed. All cases were classified following the modified classification. We compared the detection rate before and after introducing the conus medullaris screening and pregnancy outcomes for NTD types. Results: Closed spinal dysraphism type prevailed in our sample (43.8%). The median gestational age at the time of detection for cranial dysraphism was 13.3 weeks, open spinal dysraphism was 22.0 weeks, and closed spinal dysraphism was 22.6 weeks. All cranial dysraphism (n ¼ 43) and open spinal dysra-phism cases (n ¼ 20) had pregnancies terminated. For closed spinal dysraphism Class 1, the live-birth rate was 100.0% in the cases without other anomalies and 33.3% in the cases with other anomalies, respectively (X 2 ¼ 17.25, p < 0.001). Similarly, for Class 2, pregnancy continuation rate was 50.0% in cases without other anomalies and 20.0% in cases with other anomalies, yet it failed to reach statistical significance (X 2 ¼ 0.9, p ¼ 0.524). Conclusion: Our case series may help to improve early screening and prenatal diagnosis of NTDs. Modified classification is adjusted for use in ultrasound fetal care facilities, which could be used for predicting pregnancy outcome. We suggest promoting first-trimester anatomical screening in order to make an earlier diagnosis and therefore provide better prenatal care for open spinal dysraphism cases in the era of intrauterine repair. Our findings imply that the use of fetal conus medullaris position as a marker for closed spinal dysraphism improves the detection rate and would unlikely lead to a higher termination rate.

Magnetic resonance imaging in the evaluation of the fetal spinal canal contents

Brain and Development, 2011

Ultrasonography (US) remains the first method in the evaluation of fetal central nervous system (CNS) abnormalities but in case of the spinal canal and cord it is often insufficient since the bony structures may obscure these structures. Prenatal magnetic resonance imaging (MRI) is therefore the final noninvasive tool for the assessment of these malformations allowing for correction of sonographic findings, revealing the full extent of complex lesions and choosing the candidates for in utero treatment. The authors present the most frequent anomalies of spinal canal and spinal cord in the consecutive phases of pregnancy, illustrated with their own MR images, with reference to the literature and own experience. In 58 out of 252 fetuses examined due to suspicion of CNS anomalies (23.0%) the spinal canal and spinal cord abnormalities were found on MRI. The cases of diastematomyelia, myelomeningocele, tethered cord, caudal regression syndrome, anterior meningocele, cystic sacrococcygeal teratoma and syringohydromyelia are demonstrated.

Clinical Significance of Terminal Syringomyelia and Accompanying Congenital Anomalies of Neurosurgical Interest in Adult and Pediatric Patients with Tethered Cord Syndrome

Journal of child science, 2022

Magnetic resonance imaging (MRI) can be used to examine tethered cord syndrome (TCS) and terminal syringomyelia (TS). Additionally, there is increasing evidence of an association between congenital anomalies and TCS. We aimed to identify the clinical and radiological characteristics of syringomyelia and other anomalies in pediatric and adult patients with TCS. This study included 54 TCS patients (mean age, 17.37 AE 15.83 years; 31 females) admitted to our department between 2010 and 2019. The patients were divided into two age groups: pediatric (<18 years; 63%) and adult (>18 years). Clinical findings, direct vertebrae radiographs, lower extremity radiographs, and spinal/cranial MRI findings were used to evaluate all patients. Computed tomography (CT) was performed to reveal the structure of the septum in patients with Diastematomyelia. Cranial ultrasonography or CT was performed if the fontanel was open or closed, respectively, in pediatric hydrocephalus cases. Pelvic ultrasonography and urodynamic tests were performed to evaluate other comorbid anomalies and urinary system pathologies. A thick filum terminale (73.3%) and diastematomyelia (44.4%) were found to cause spinal tension. The most common accompanying pathology was syringomyelia (78%). The common symptoms were urinary incontinence and bowel problems (71%), scoliosis (68%), and progressive lower extremity weakness (64.4%). It is difficult to distinguish the exact cause of symptoms in patients with TCS and TS. Due to the greater occurrence of other congenital spinal anomalies accompanying TCS, both preoperative symptoms and clinical findings are more severe in the pediatric group than in the adult group, and postoperative results may be more negative.

Spinal Imaging Findings of Open Spinal Dysraphisms on Fetal and Postnatal MRI

American Journal of Neuroradiology, 2018

Fetal MRI has become a valuable tool in the evaluation of open spinal dysraphisms making studies comparing prenatal and postnatal MRI findings increasingly important. Our aim was to determine the accuracy of predicting the level of the spinal dysraphic defect of open spinal dysraphisms on fetal MR imaging and to report additional findings observed when comparing fetal and postnatal MR imaging of the spine in this population. A single-center retrospective analysis was performed of fetal MRIs with open spinal dysraphisms from 2004 through 2016 with available diagnostic postnatal spine MR imaging. Images were reviewed by 2 board-certified fellowship-trained pediatric neuroradiologists. Corresponding clinical/operative reports were reviewed. One hundred nineteen fetal MRIs of open spinal dysraphisms were included. The level of the osseous defect between fetal and postnatal MR imaging was concordant in 42.9% (51/119) of cases and was 1 level different in 39% (47/119) of cases. On postnatal MR imaging, type II split cord malformation was seen in 8.4% (10/119) of cases, with only 50% (5/10) of these cases identified prospectively on fetal MR imaging. Syrinx was noted in 3% (4/119) of prenatal studies, all cervical, all confirmed on postnatal MR imaging. Fetal MR imaging is accurate in detecting the level of the spinal dysraphic defect, which has an impact on prenatal counseling, neurologic outcomes, and eligibility for fetal surgery. In addition, fetal MR imaging is limited in its ability to detect split cord malformations in patients with open spinal dysraphisms. Although rare, fetal MR imaging has a high specificity for detection of cervical spinal cord syrinx.

The Fetal Study on Cranial and Spinal Dysraphism of Neural Tube

Neural tube defects (NTD) are more common congenital birth defect affecting the babies all over the world. .It is one of the causes of fetal mortality and morbidity .Defective neurulation process during embryonic period leads to this condition. Based on the level of neural tube involvement the defects are divided as cranial dysraphism and spinal dysraphism.Cranial dysraphism include lethal anencephaly ,encephalocele and iniencephaly .In spinal dysraphism ,failure in the fusion of caudal end of neural tube leads to meningocele and myelomeningocele.These conditions are associated with mesodermal involvement leading to bony defects .The present study was done on 50 human fetuses that were collected for development of museum in Anatomy.In this processmore number of fetuses with neural tube defects were identified. The fetuses were separated based on the level of neural tube involvement. In anencephaly,encephalocele and rare iniencephaly in which the cranial end of neural tube is involved and in meningocele and myelocele the caudal end neural tube is involved. All these fetuses were studied further. Manifestation of NTD is multifactorial that have genetic or environmental basis with high recurrence rate. Neural tube defects are usually diagnosed in early weeks of intrauterine life by ultrasound examination .In the various forms of neural tube defects,anencephaly is incompatible with life and depending on the level of exposure of neural tissue ,open spinal defects will survive with permanent disability.Folic acid is given as a supplement to prevent the neural tube defects and the role of folic acid in NTD's was studied by authors and they expressed that folic acid is important in nucleic acid formation and in the metabolic activity of enzymes. The deficiency of folic acid blocks all this process and leads to formation of neural tube defect. So pregnant woman with previous history of neural tube defect are advised to take folic acid before planning the pregnancy and continue for three months after conception to prevent the recurrence of neural tube defects..

Syringomyelia and pregnancy-case report

European Journal of Obstetrics & Gynecology and Reproductive Biology, 2001

The course of a pregnancy in a woman with syringomyelia is presented. She was ®rst admitted at 28 weeks' gestation suffering neurologic symptoms associated with a spinal cord injury, which had happened in the past. The disease was diagnosed with a magnetic resonance imaging (MRI). Delivery was accomplished by elective caesarean section under general anaesthesia at 37 weeks, in order to avoid straining during the second stage of an imminent labour.

MRI of fetal spinal malformations

Fetal magnetic resonance imaging is well accepted as secondary image tool for the evaluation of pathologies affecting the fetal brain as detected on prenatal ultrasonography. Significantly, fewer articles have focused on the fetal spinal canal and its contents. Many malformations and pathologies involving the spinal canal and cord may however have a significant impact on the quality of life. In addition, anomalies of the spinal cord may affect the development of the fetal brain or may be part of a more extensive malformation that may also affect the major thoracic and abdominal organs. A thorough knowledge of the normal and abnormal development of the spinal column and its contents is necessary to diagnose and understand the encountered findings. In the current review the value of fetal magnetic resonance imaging of the spinal column and cord is discussed. The most frequently encountered malformations will be presented and discussed in the context of the most relevant embryological processes.

Neural Tube Defects: Distribution and Associated Anomalies Diagnosed by Prenatal Ultrasonography in Iranian Fetuses

Journal of Obstetrics, Gynecology and Cancer Research

Objectives: The current study aimed at providing detailed information about the distribution, associated anomalies, and syndromes in Iranian fetuses with neural tube defects (NTDs). Methods: The current study was conducted in Yas Females' referral and teaching hospital in 18 months from 2014 to 2016. All fetuses with a prenatally detected neural tube defect were included in the study. Neural tube defect characterization, gestational age, maternal reproductive factors, maternal risk factors, and associated anomalies were recorded. Results: Neural tube abnormalities were identified prenatally in 80 fetuses: 40 cases of ex/anencephaly, 22 cases of spina bifida, 13 cases of cephalocele and 5 cases of anencephaly /craniorachischisis. All the cases were detected before 21st week of gestation and 92.5% of the ex/anencephaly cases were diagnosed in the 1st trimester. Moreover, 40% of the open spina bifida cases in the current study were myelocele, while 75% of them referred only due to abnormal cranial findings. Incomplete consumption of folate was the most common associated risk factor (45%). Associated anomalies were recorded in 53 (66%) fetuses, with more prevalence in the fetuses with spina bifida (90%). Among the associated anomalies, central nervous system (CNS) anomalies were the most common type (26.26%). Chiari II was found in all the cases of open spina bifida and the ventriculomegaly rate was 30% in this group. Extremities anomalies and spine deformities were the 2nd and 3rd common associated findings, respectively. Limb-body-wall complex/amniotic band syndrome was the most common identified associated syndrome (6%). Conclusions: Results of the current study confirmed the high prevalence of associated anomalies in neural tube defect cases and revealed the capability of detailed sonography to detect and define such abnormalities.

Fetal MRI in the evaluation of fetuses referred for sonographically suspected neural tube defects (NTDs): impact on diagnosis and management decision

Neuroradiology, 2009

Introduction We hypothesized that magnetic resonance imaging (MRI) can assess fetuses with sonographically (ultrasonography (US))-suspected neural tube defects (NTD) that might influence their diagnoses and management decision. Methods Institutional review board approval and informed consents were obtained to perform MRI for 19 fetuses referred with US-suspected NTD. Prenatal imaging findings were correlated with management decision, postnatal clinical, postnatal imaging, and pathology. Results Prenatal MRI correctly ruled out US diagnosis of cephalocele in a fetus. In the other 18 fetuses, MRI detected detailed topography and contents of NTD sacs in five, added central nervous system (CNS) abnormalities that were not apparent on US in three, and confirmed non-CNS findings in three fetuses. MRI changed diagnosis of 3/19 fetuses (15.8%), caused minor change in diagnosis of 5/19 (26.3%), and did not influence US diagnosis of 11/19 fetuses (57.9%). MRI findings changed/modified management decision in 21% of the fetuses. Conclusion Fetal MRI is an important adjunct to US in assessing NTD. It can identify topography and contents of sacs, add CNS and non-CNS findings, and influence management decision.