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Ultrasound in Obstetrics & Gynecology :... Jun 2022To determine the prevalence of brain anomalies at the time of preoperative magnetic resonance imaging (MRI) assessment in fetuses eligible for prenatal open spina bifida...
OBJECTIVES
To determine the prevalence of brain anomalies at the time of preoperative magnetic resonance imaging (MRI) assessment in fetuses eligible for prenatal open spina bifida (OSB) repair, and to explore the relationship between brain abnormalities and features of the spinal defect.
METHODS
This was a retrospective cross-sectional study, conducted in three fetal medicine centers, of fetuses eligible for OSB fetal surgery repair between January 2009 and December 2019. MRI images obtained as part of the presurgical assessment were re-evaluated by two independent observers, blinded to perinatal results, to assess: (1) the type and area of the defect and its anatomical level; (2) the presence of any structural central nervous system (CNS) anomaly and abnormal ventricular wall; and (3) fetal head and brain biometry. Binary regression analyses were performed and data were adjusted for type of defect, upper level of the lesion (ULL), gestational age (GA) at MRI and fetal medicine center. Multiple logistic regression analysis was performed in order to identify lesion characteristics and brain anomalies associated with a higher risk of presence of abnormal corpus callosum (CC) and/or heterotopia.
RESULTS
Of 115 fetuses included, 91 had myelomeningocele and 24 had myeloschisis. Anatomical level of the lesion was thoracic in seven fetuses, L1-L2 in 13, L3-L5 in 68 and sacral in 27. Median GA at MRI was 24.7 (interquartile range, 23.0-25.7) weeks. Overall, 52.7% of cases had at least one additional brain anomaly. Specifically, abnormal CC was observed in 50.4% of cases and abnormality of the ventricular wall in 19.1%, of which 4.3% had nodular heterotopia. Factors associated independently with higher risk of abnormal CC and/or heterotopia were non-sacral ULL (odds ratio (OR), 0.51 (95% CI, 0.26-0.97); P = 0.043), larger ventricular width (per mm) (OR, 1.23 (95% CI, 1.07-1.43); P = 0.005) and presence of abnormal cavum septi pellucidi (OR, 3.76 (95% CI, 1.13-12.48); P = 0.031).
CONCLUSIONS
Half of the fetuses assessed for OSB repair had an abnormal CC and/or an abnormal ventricular wall prior to prenatal repair. The likelihood of brain abnormalities was increased in cases with a non-sacral lesion and wider lateral ventricles. These findings highlight the importance of a detailed preoperative CNS evaluation of fetuses with OSB. © 2021 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.
Topics: Central Nervous System Diseases; Cross-Sectional Studies; Female; Fetus; Gestational Age; Humans; Magnetic Resonance Imaging; Meningomyelocele; Nervous System Malformations; Pregnancy; Prevalence; Retrospective Studies; Spina Bifida Cystica; Ultrasonography, Prenatal
PubMed: 34396624
DOI: 10.1002/uog.23761 -
Acta Ortopedica Mexicana 2021The aim of the study was to evaluate the outcomes and complications in patients with myelomeningocele who have undergone spinal fusion for neuromuscular scoliosis.
OBJECTIVE
The aim of the study was to evaluate the outcomes and complications in patients with myelomeningocele who have undergone spinal fusion for neuromuscular scoliosis.
MATERIAL AND METHODS
Retrospective study of 40 consecutive patients with myelomeningocele with neuromuscular scoliosis who underwent spinal arthrodesis, treated at our center between July 1991 and July 2028.
RESULTS
Mean follow up was 10 years. There were 19 male and 21 females. The average age at operation was 13 years. The mean preoperative scoliosis curve was 90o. At last follow up, the mean scoliosis curve was 43o. Mean correction of 52%. The average of pelvic obliquity was 19o. At last follow up de obliquity was 9o. Mean correction of 53%. The mean preoperative coronal balance was 28.4 mm. At the last follow up it was 17 mm. Mean correction of 40%. The mean preoperative kyphosis was 50o. At the last follow up it was 41o. Mean correction of 18%. The mean preoperative sagittal balance was 63.3 mm. At the last follow up it was 38.3 mm. Mean correction of 40%. There were 13 complications (32.5%), with infection being the most frequently observed complication.
CONCLUSIONS
The goals of the spinal surgery in patients with MMC are to obtain a stable, balanced and painless spinal fusion. Although the surgical treatment of these patients remains difficult, it is associated with high complication rate.
Topics: Female; Humans; Male; Meningomyelocele; Neuromuscular Diseases; Retrospective Studies; Scoliosis; Spinal Fusion; Treatment Outcome
PubMed: 35451247
DOI: No ID Found -
Seminars in Pediatric Neurology Jul 2022In this article we review the last 40 years of progress in fetal neurosurgery with special attention to current controversies and upcoming challenges in the field. We... (Review)
Review
In this article we review the last 40 years of progress in fetal neurosurgery with special attention to current controversies and upcoming challenges in the field. We surveyed the published literature describing prenatal interventions for spinal malformations, cerebral malformations, and hydrocephalus. Even the most mature treatment paradigm, intrauterine repair of myelomeningocele, stands to benefit from advances in imaging and therapeutic modalities to improve patient selection, refine surgical techniques, validate novel biologic therapies, and streamline postoperative patient care. Other conditions under evaluation include congenital cerebral malformations, such as encephalocele, cerebrovascular malformations, and hydrocephalus. We describe cross-cutting needs for advances in fetal neuroimaging, basic disease models and new therapeutic devices to support further progress across various neurosurgical conditions affecting patients during the fetal period.
Topics: Female; Humans; Hydrocephalus; Meningomyelocele; Nervous System Malformations; Neuroimaging; Neurosurgical Procedures; Pregnancy
PubMed: 35868731
DOI: 10.1016/j.spen.2022.100964 -
Journal of Pediatric Urology Feb 2020Over the last 40 years, vast changes have occurred in the care of children with open neural tube defects. Not only has newborn survival dramatically improved but... (Review)
Review
Over the last 40 years, vast changes have occurred in the care of children with open neural tube defects. Not only has newborn survival dramatically improved but survival into adulthood has improved as well. Now, the ability to accurately identify and repair myelomeningocele (MMC) lesions before birth has become a reality. Pioneering efforts at several institutions in the United States paved the way for such advancements in care. Substantial data now exist to support the positive benefits of fetal MMC repair from a neurosurgical standpoint, chiefly the significant reduction in hindbrain herniation, decrease in shunt-dependent hydrocephalus, and improvement in lower-extremity motor function. However, until only recently, the urological impact of fetal repair has not been nearly as positive overall. Multiple retrospective reports of newborn bladder function from the United States suggest that prenatal repair has provided neither short-term nor long-term improvements in bladder function. Yet, the retrospective nature of these data and their focus upon urodynamic studies (UDS) parameters have hampered the ability to draw conclusions. Recently, published data from the landmark Management of Myelomeningocele Study indicate that fetal repair may improve certain aspects of bladder function when compared with conventional repair. This review provides an overview of the history and timeline of fetal repair in the United States and brings the reader quickly up to date on the current impact of repair on both neurosurgical and urological outcomes.
Topics: Fetus; Humans; Meningomyelocele; Treatment Outcome; Urinary Bladder; Urology
PubMed: 31902678
DOI: 10.1016/j.jpurol.2019.11.019 -
Child's Nervous System : ChNS :... Dec 2021A growing body of literature demonstrates the clinical promise of dehydrated human amnion/chorion membrane (dHACM), a cryopreserved tissue product derived from placental... (Review)
Review
A growing body of literature demonstrates the clinical promise of dehydrated human amnion/chorion membrane (dHACM), a cryopreserved tissue product derived from placental amniotic membrane, to enhance post-operative wound healing. The purpose of this study is to review the potential of dHACM to facilitate post-surgical and myelomeningocele wound repair. A comprehensive literature search of PubMed was conducted to identify studies investigating dHACM use in pediatric and surgical wound care published from inception to October 2020. For each study, patient characteristics, wound characteristics, and outcomes following dHACM application were documented and assessed. Of the 190 articles reviewed, 15 publications were included in the final analysis. Results demonstrated that the average wound healing time varied across clinical indications (e.g., 14 days for trauma reconstruction to 116 days for Moh's surgery repair). Across indications, pediatric patients had shorter healing periods compared to adults (P < 0.01). Chronic wounds (> four weeks old) were documented in both adult (n = 3) and pediatric (n = 2) wound repair publications; all chronic surgical wounds demonstrated complete wound closure with dHACM. No complications from dHACM use were reported. Advantages of dHACM included increased patient satisfaction, cost-savings, and faster wound healing. We then present two cases of myelomeningocele wound repair facilitated successfully by dHACM. Overall, dHACM proves to successfully expedite wound repair in pediatric patients with chronic or complicated wounds such as those from myelomeningocele repair. It is important for surgeons to consider wound duration, size, and patient age to better predict graft success in enhancing wound repair.
Topics: Adult; Allografts; Amnion; Child; Chorion; Female; Humans; Meningomyelocele; Placenta; Pregnancy; Wound Healing
PubMed: 34609611
DOI: 10.1007/s00381-021-05352-z -
Neurosurgical Focus Oct 2019This paper describes some of the more common patterns in neurobehavioral deficits and their underlying neuroanatomical basis in myelomeningocele (MMC). 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.
Topics: Arnold-Chiari Malformation; Brain; Female; Humans; Hydrocephalus; Male; Meningomyelocele; Pregnancy; Spinal Cord
PubMed: 31574480
DOI: 10.3171/2019.7.FOCUS19445 -
Child's Nervous System : ChNS :... Nov 2020The cystic spinal dysraphism of the cervical and upper thoracic region (CDCT) accounts for only 3.9 to 8% of spina bifida cystica lesions. The presence of external... (Review)
Review
BACKGROUND
The cystic spinal dysraphism of the cervical and upper thoracic region (CDCT) accounts for only 3.9 to 8% of spina bifida cystica lesions. The presence of external cerebrospinal fluid (CSF) leakage is infrequent and very few authors have reported about surgical complications.
CASE REPORT
We present the case of a new born diagnosed of CDCT C1-C3 with a stalk of fibrovascular tissue, CSF leakage, hydrocephalus, and type Chiari II malformation, discuss about the chosen surgical technique and the associated complications, and make a review of the literature focusing on the main aspects of CDCT.
CONCLUSIONS
The CDCT with a stalk of neuroglial and/or fibrovascular tissue originates from the dorsal surface of the spinal cord and penetrates into a cervical cystic sac. The presence of CSF leakage, hydrocephalus, and/or type Chiari II malformation can influence to decide the most appropriate surgical technique.
Topics: Arnold-Chiari Malformation; Humans; Hydrocephalus; Infant, Newborn; Meningomyelocele; Spina Bifida Cystica; Spinal Dysraphism
PubMed: 32621007
DOI: 10.1007/s00381-020-04743-y -
Journal of Pediatric Rehabilitation... 2021To assess hand dexterity in children with myelomeningocele (MMC) and to explore factors related to hand dexterity in these children.
PURPOSE
To assess hand dexterity in children with myelomeningocele (MMC) and to explore factors related to hand dexterity in these children.
METHODS
Ninety-four children with myelomeningocele, aged 4 to 18 years, were assessed. Demographic characteristics, disease factors, visual perception (Beery test of Visual Motor Integration), cognition (WeeFunctional Independence Measure), and self-care (Pediatric Evaluation of Disability Inventory) were assessed in relation to the Nine-Hole Peg Test (9HPT) for hand dexterity using Spearmen correlations and linear regressions.
RESULTS
The children's performance on the 9HPT in both hands was significantly slower than the norms for their age groups. Children without a shunt showed significantly better function in both hands (p = .005) than those with a shunt. Factors most related to hand dexterity were neurological spinal level of MMC, presence of shunt, age, cognitive ability, and years of mother's education.
CONCLUSION
Children with MMC appear to have poorer hand skills than typically developed children, which was related to pathology as well as functional and environmental factors. When addressing hand dexterity in children with MMC, it is important that rehabilitation professionals continue to work with these children as they get older, and put greater emphasis on parent education using materials that are adapted to varying educational levels.
Topics: Adolescent; Child; Child, Preschool; Cognition; Hand; Humans; Meningomyelocele; Motor Skills; Self Care; Upper Extremity
PubMed: 33935118
DOI: 10.3233/PRM-200713 -
International Braz J Urol : Official... 2022Defective closure of the neural tube affects different systems and generates sequelae, such as neurogenic bladder (NB). Myelomeningocele (MMC) represents the most... (Review)
Review
INTRODUCTION
Defective closure of the neural tube affects different systems and generates sequelae, such as neurogenic bladder (NB). Myelomeningocele (MMC) represents the most frequent and most severe cause of NB in children. Damage of the renal parenchyma in children with NB acquired in postnatal stages is preventable given adequate evaluation, follow-up and proactive management. The aim of this document is to update issues on medical management of neurogenic bladder in children.
MATERIALS AND METHODS
Five Pediatric Urologists joined a group of experts and reviewed all important issues on "Spina Bifida, Neurogenic Bladder in Children" and elaborated a draft of the document. All the members of the group focused on the same system of classification of the levels of evidence (GRADE system) in order to assess the literature and the recommendations. During the year 2020 the panel of experts has met virtually to review, discuss and write a consensus document.
RESULTS AND DISCUSSION
The panel addressed recommendations on up to date choice of diagnosis evaluation and therapies. Clean intermittent catheterization (CIC) should be implemented during the first days of life, and antimuscarinic drugs should be indicated upon results of urodynamic studies. When the patient becomes refractory to first-line therapy, receptor-selective pharmacotherapy is available nowadays, which leads to a reduction in reconstructive procedures, such as augmentation cystoplasty.
Topics: Child; Humans; Intermittent Urethral Catheterization; Meningomyelocele; Spinal Dysraphism; Urinary Bladder, Neurogenic; Urodynamics
PubMed: 33861059
DOI: 10.1590/S1677-5538.IBJU.2020.0989 -
Developmental Medicine and Child... Apr 2020Fetal myelomeningocele (fMMC) closure (spina bifida aperta) has become a care option for patients that meet inclusion criteria, but it is clear that fetal intervention,... (Review)
Review
Fetal myelomeningocele (fMMC) closure (spina bifida aperta) has become a care option for patients that meet inclusion criteria, but it is clear that fetal intervention, while improving outcomes, is not a cure. This review will: (1) focus on the rationale for fMMC surgery based on preclinical studies and observations that laid the foundation for human pilot studies and a randomized controlled trial; (2) summarize important clinical outcomes; (3) discuss the feasibility, efficacy, and safety of recent developments in fetal surgical techniques and approaches; and (4) highlight future research directions. Given the increased risk of maternal and fetal morbidity associated with prenatal intervention, accompanied by the increasing number of centres performing interventions worldwide, teams involved in the care of these patients need to proceed with caution to maintain technical expertise, competency, and patient safety. Ongoing assessment of durability of the benefits of fMMC surgery, as well as additional refinement of patient selection criteria and counselling, is needed to further improve outcomes and reduce the risks to the mother and fetus. WHAT THIS PAPER ADDS: High-quality prospective studies are needed to broaden the indication for fetal surgery in the general myelomeningocele population. Innovative minimally invasive approaches have had promising results, yet lack comprehensive and robust experimental or clinical evaluation. Important information to help families make informed decisions regarding fetal surgery for myelomeningocele is provided.
Topics: Female; Fetoscopy; Fetus; Humans; Meningomyelocele; Pregnancy
PubMed: 31840814
DOI: 10.1111/dmcn.14429