High-fidelity, low-cost synthetic training model for fetoscopic spina bifida repair.

BACKGROUND: Fetoscopic spina bifida repair is increasingly being practiced, but limited skill acquisition poses a barrier to widespread adoption. Extensive training in relevant models, including both ex vivo and in vivo models may help. To address this, a synthetic training model that is affordable, realistic, and that allows skill analysis would be useful. OBJECTIVE: This study aimed to create a high-fidelity model for training in the essential neurosurgical steps of fetoscopic spina bifida repair using synthetic materials. In addition, we aimed to obtain a cheap and easily reproducible model. STUDY DESIGN: We developed a 3-layered, silicon-based model that resemble the anatomic layers of a typical myelomeningocele lesion. It allows for filling of the cyst with fluid and conducting a water tightness test after repair. A compliant silicon ball mimics the uterine cavity and is fixed to a solid 3-dimensional printed base. The fetal back with the lesion (single-use) is placed inside the uterine ball, which is reusable and repairable to allow for practicing port insertion and fixation multiple times. Following cannula insertion, the uterus is insufflated and a clinical fetoscopic or robotic or prototype instruments can be used. Three skilled endoscopic surgeons each did 6 simulated fetoscopic repairs using the surgical steps of an open repair. The primary outcome was surgical success, which was determined by water tightness of the repair, operation time <180 minutes and an Objective Structured Assessment of Technical Skills score of ≥18 of 25. Skill retention was measured using a competence cumulative sum analysis of a composite binary outcome of surgical success. Secondary outcomes were cost and fabrication time of the model. RESULTS: We made a model that can be used to simulate the neurosurgical steps of spina bifida repair, including anatomic details, port insertion, placode release and descent, undermining of skin and muscular layer, and endoscopic suturing. The model was made using reusable 3-dimensional printed molds and easily accessible materials. The 1-time startup cost was €211, and each single-use, simulated myelomeningocele lesion cost €9.5 in materials and 50 minutes of working time. Two skilled endoscopic surgeons performed 6 simulated, 3-port fetoscopic repairs, whereas a third used a Da Vinci surgical robot. Operation times decreased by more than 30% from the first to the last trial. Six experiments per surgeon did not show an obvious Objective Structured Assessment of Technical Skills score improvement. Competence cumulative sum analysis confirmed competency for each surgeon. CONCLUSION: This high-fidelity, low-cost spina bifida model allows simulated dissection and closure of a myelomeningocele lesion. VIDEO ABSTRACT.

Stratification of neurogenic bladder risk in spina bifida using the urinary peptidome.

Neurogenic bladder poses a major morbidity in children with spina bifida (SB), and videourodynamic studies (VUDS) are used to stratify this risk. This small-scale pilot study utilized current mass-spectrometry-based proteomic approaches to identify peptides or proteins in urine that may differentiate children at high risk of developing renal complications from a neurogenic bladder. Twenty-two urine samples of which nine had high bladder pressure storage that put the upper urinary tract at risk, while 13 with a lower risk for renal compromise were analyzed. More than 1,900 peptides across all 22 samples were quantified, and 115 peptides differed significantly (P < 0.05) between the two groups. Using machine learning approaches five peptides that showed the greatest differences between these two clinical categories were used to build a classifier. We tested this classifier by blind analysis of an additional six urine samples and showed that it correctly assigned the unknown samples in their proper risk category. These promising results indicate that a urinary screening test based on peptides could be performed on a regular basis to stratify the neurogenic bladder into low or high-risk categories. Expanding this work to larger cohorts as well as across a broad spectrum of urodynamics outcomes may provide a useful diagnostic test for neurogenic bladder.NEW & NOTEWORTHY This approach could help risk stratify the neurogenic bladder in patients with spina bifida and could allow us to safely defer on up to 1/3 of urodynamic studies. These pilot data justify a larger trial before this approach becomes a clinical tool.

Prenatal imaging of the normal and abnormal spinal cord: recommendations from the Fetal Task Force of the European Society of Paediatric Radiology (ESPR) and the European Society of Neuroradiology (ESNR) Pediatric Neuroradiology Committee.

Spinal dysraphisms are amenable to diagnosis in utero. The prognosis and the neonatal management of these conditions differ significantly depending on their types, mainly on the distinction between open and closed defects. A detailed evaluation not only of the fetal spine, but also of the brain, skull, and lower limbs is essential in allowing for the right diagnosis. In this article, recommendations from the Fetal Task Force of the European Society of Paediatric Radiology (ESPR) and the European Society of Neuroradiology (ESNR) Pediatric Neuroradiology Committee will be presented. The aim of this paper is to review the imaging features of the normal and abnormal fetal spinal cord, to clarify the prenatal classification of congenital spinal cord anomalies and to provide guidance in their reporting.

Systematic review and meta-analysis to study the outcomes of proactive versus delayed management in children with a congenital neurogenic bladder.

PURPOSE: To review the urological outcomes of proactive versus delayed management of children with a neurogenic bladder (NB). METHODS: We performed a literature search on EMBASE, MEDLINE, Scopus, Web of Science, and Cochrane Central Register of Controlled Trials between January 1, 2000 to August 21, 2023 for studies investigating the management of spina bifida-associated NB in pediatric patients (0-18 years of age). Proactive management was defined as use of clean intermittent catheterization, and/or anticholinergics at presentation, or based on initial high-risk urodynamic findings by 1 year of age. Delayed management was defined as beginning management after 1 year of age or no intervention. Outcomes included incidence or diagnosis of secondary vesicoureteral reflux (VUR), urinary tract infection (UTI), and renal deterioration, which included renal scarring, loss of differential renal function on a nuclear scan, or a decrease in renal function defined by glomerular filtration rate or serum creatinine estimation. Forest plots were synthesized using the Inverse Variance method with random-effect model. The Risk of Bias was assessed using the ROBINS-I tool. RESULTS: We included 8 observational studies on 652 pediatric patients with spina bifida-associated NB (mean follow-up - 7 years). Proactive management following initial assessment was associated with significantly lower risks of secondary VUR (OR 0.37 [0.19, 0.74], p = 0.004), non-febrile UTI (OR 0.35 [0.19, 0.62], p = 0.0004), and renal deterioration (OR 0.31 [0.20, 0.47], p < 0.00001). DISCUSSION: Delayed management of NB potentially has 3 times higher risks of secondary VUR, non-febrile UTI, and renal deterioration. However, the evidence is limited by the high risk of bias due to lack of randomization and standardized reporting in observational studies. CONCLUSION: While further well-defined prospective studies with long-term follow-up should be conducted to confirm this finding, this study supports the EAU/ESPU recommendations for early intervention in children with NB.

Midline cutaneous anomalies of the craniospinal axis.

Patients with midline cutaneous anomalies of the craniospinal axis can be indicative of underlying embryonic defects, such as neural tube defects. Lack of familiarity with these midline aberrant skin findings may lead to misdiagnosis and delayed treatment. In this review, midline cutaneous anomalies of the craniospinal axis including aplasia cutis congenita, cranial and spinal dysraphism, and other developmental anomalies are explored in detail with emphasis on cutaneous clues to the diagnosis and appropriate workup.

Multi-Institutional Review From the Pediatric Colorectal and Pelvic Learning Consortium of Minor Spinal Cord Dysraphism in the Setting of Anorectal Malformations: Diagnosis, Treatment, and Outcomes.

BACKGROUND/RATIONALE: Anorectal malformations (ARM) are associated with congenital anomalies of the spine, but the impact of a minor spinal cord dysraphism (mSCD) on fecal continence in the setting of ARM remains unclear. MATERIALS/METHODS: A retrospective review was performed utilizing data from the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) registry. The patient cohort was reviewed for ARM type, mSCD screening/incidence/neurosurgical intervention and age-based BMP utilization. RESULTS: 987 patients with ARM were categorized into mild (38%), moderate (32%) or complex (19%). 694 (70%) had normal spinal (NS) status. 271 (27.5%) patients had mSCD. MRI alone (49%) was the most common screening test for mSCD. US screening had a positive predictive value of 86.3% and a negative predictive value of 67.1%. Surgical intervention rates for mSCD ranged between 13% and 77% at a median age of 0.6-5.2 years. 726 (73.6%) patients were prescribed BMP (74.4% NS, 77.5% mSCD). Laxatives were most utilized BMP in all groups <5yo. ≥5yo, enema utilization increased with ARM complexity independent of spine status (with or without neurosurgical intervention). Neurosurgical intervention did not affect BMP utilization at any age or with any ARM when mSCD was identified. CONCLUSIONS: MSCD influence on bowel function in the setting ARM remains unclear. No significant impact of mSCD was noted on ARM patient bowel management program utilization. Variability exists within PCPLC site with screening and intervention for mSCD in patients with ARM. Future studies with standardized care may be needed to elucidate the true impact of mSCD on long term patient outcomes in ARM patients. TYPE OF STUDY: Retrospective Comparative Study. LEVEL OF EVIDENCE: III.

Electrodiagnostic findings of retethering in children with spinal dysraphism.

PURPOSE: Retethering of the cord can occur after the initial untethering surgery. Typical neurological manifestations indicative of cord tethering are often difficult to determine in pediatric patients. Patients who had a primary untethering operation are likely to present with some degree of neurological deficits from a previous tethering event, and urodynamic studies (UDSs) and spine images are frequently abnormal. Therefore, more objective tools to detect retethering are needed. This study sought to delineate the characteristics of EDS of retethering, and therefore, could support the diagnosis of retethering. METHODS: Among 692 subjects who had an untethering operation, data from 93 subjects who had been suspected of retethering clinically were retrospectively extracted. The subjects were divided into two groups, a retethered group, and a non-progression group, according to whether or not surgical interventions had been performed. Two consecutive EDSs, clinical findings, spine magnetic resonance imaging scans, and UDSs before the development of new tethering symptoms were reviewed and compared. RESULTS: In the electromyography (EMG) study, the appearance of abnormal spontaneous activity (ASA) in new muscles was prominent in the retethered group (p < 0.01). The loss of ASA was more pronounced in the non-progression group (p < 0.01). Specificity and sensitivity of EMG for retethering were 80.4 and 56.5%, respectively. In the nerve conduction study, the two groups did not show differences. The size of fibrillation potential was not different between the groups. CONCLUSIONS: To provide support for a clinician's decision on retethering, EDS could be an advantageous tool with high specificity when the results are compared to previous EDS results. Routine follow-up EDS post-operatively is recommended as a baseline for comparison at the time when retethering is clinically suspected.

False-positive urine pregnancy screening tests are uncommon in the hospital setting among patients with bowel-containing urinary tract reconstruction.

PURPOSE: False-positive urine pregnancy screening tests (UPST) have been reported among patients with bowel-containing urinary reconstruction (BCUR). However, the true frequency of such inaccurate results, which have been attributed to urinary mucous or other proteins interfering with or mimicking the binding of beta-HCG in the assay, is unknown in this population. We sought to determine the incidence of false-positive pregnancy screening tests among this patient population at our institution. MATERIALS AND METHODS: Using existing databases of patients with spina bifida, bladder exstrophy, and genitourinary rhabdomyosarcoma, we identified female patients with BCUR who had UPST over a 10-year period as screening prior to procedures or imaging. Patient and test result information was recorded. RESULTS: A total of 120 patients with a history of BCUR were identified: 33 with spina bifida, 73 within the exstrophy-epispadias complex (EEC), and 14 with genitourinary rhabdomyosarcoma. Of this group, 46 patients (38%) had at least one UPST during the study period; 15 had 1 UPST, 6 had 2 UPSTs, 4 had 3 UPSTs, and 21 had greater than 3 UPSTs, for a total of 244 UPST in this cohort. UPSTs used at our institution included Sure-Vue brand and Alere brand (HCG sensitivity 20 mIU/ml). Types of BCUR included ileal enterocystoplasty in 25 patients, colon enterocystoplasty in 6, stomach enterocystoplasty in 5, composite enterocystoplasty in 7, and continent catheterizable channel alone (e.g. Yang-Monti, appendicovesicostomy) in 3 patients. Of the 244 UPSTs in patients with BCUR, zero (0%) were positive. CONCLUSIONS: Despite reports in the literature that false-positive UPST are common among patients with bowel-containing urinary diversions, we found no positive UPST among patients with BCUR in the healthcare setting. False-positive UPST in the home setting may be due to variability in sensitivity thresholds, binding agents, technical errors in test technique, kit quality control, or other factors.

Electrodiagnosis has a potential to identify neural damage in asymptomatic infants with closed spinal dysraphism.

PURPOSE: The presence and progression of symptoms is the basis for deciding to perform surgery in infants with closed spinal dysraphism (CSD); however, identifying symptoms could be limited, making it difficult to decide. This study investigated whether an electrodiagnostic study (EDS) can provide evidence of neural damage in asymptomatic infants with CSD. METHODS: The study group comprised infants with CSD suspected of having neural damage based on structural abnormalities in spinal ultrasound findings. The patients' medical records were reviewed retrospectively for their clinical presentation, neuroimaging findings, urodynamic study (UDS) results, EDS findings, and surgical status. RESULTS: Among 125 infants who underwent EDS and UDS, 117 (94%) had no clinical symptoms, except for cutaneous manifestations. Among these asymptomatic patients, 51 individuals (43.6%) had abnormal EDS findings; 33 subjects (28.2%) showed abnormal findings on EDS alone, while 37 (31.6%) on UDS alone, and 18 (15.4%) on both EDS and UDS. Chi-square test showed an opposite relationship between the two test results; when EDS was abnormal, UDS was often normal and vice versa (χ2 = 5.328, p = 0.021). In all cases with abnormal EDS, denervation potentials, such as fibrillation and positive sharp waves, were observed on needle electromyography. However, abnormal findings in the nerve conduction study were observed only in six cases. CONCLUSION: Subclinical neural damage was identified through EDS in asymptomatic infants with CSD. EDS could be necessary to determine whether follow-up monitoring only or surgical intervention is required for this patient group complementing UDS findings.

Correlation of urodynamic studies and somatosensory evoked potential and their prognostic value in children with closed spinal dysraphism.

INTRODUCTION: Somatosensory evoked potential (SSEP) and urodynamic studies (UD) are valuable tools for assessing patients with closed spinal dysraphism (CSD) before neurosurgical intervention. No studies have correlated their findings in this cohort and our aim is to study their correlation and prognostic value in pediatric patients with closed spinal dysraphism. METHODS: Retrospective review of all patients referred to a multidisciplinary clinic in a tertiary pediatric surgical center over a 17 years period between April 2004 to September 2021 was performed. Inclusion criteria were <18 years old, diagnosed with CSD, with SSEP and UD done within 1 year of each other. Demographics data collected include age at presentation/at referral/at neurosurgical operation, gender, symptoms at presentation and intra-operative diagnoses. Pre-operative SSEP and UD findings were documented. Primary outcome was UD results in the group with normal and abnormal SSEP. Secondary outcome was urological and bowel function outcome in 4 groups of patients (Group A-both normal SSEP and UD, Group B- abnormal SSEP only, Group C - abnormal UD only and Group D-both abnormal SSEP and UD). RESULTS: A total of 45 patients were included for analysis. Mean follow up time was 118.9 months (24-216 months, SD 55.8 months). SSEP was normal in 20 patients and abnormal in 25 patients. Baseline demographics, preoperative symptoms and imaging were similar between 2 groups. Primary outcome Patients with abnormal SSEP were more likely to have abnormal UD results with a statistically significant difference (84% vs 40%, p < 0.05). They have a significantly higher end-fill detrusor pressure (12% vs 0%, p < 0.05), abnormal bladder compliance (20% vs 0%, p < 0.05), abnormal cystometric capacity (48% vs 10%, p < 0.05), poor emptying efficiency (24% vs 5%, p < 0.05) and sphincter incompetence (8% vs 0%, p < 0.05). Secondary outcome When compared to Groups A to C, patients in group D were more likely to be on anti-cholinergic (33.3% vs 4.3%, p < 0.05), required clean intermittent catheterization (42.9% vs 4.3%, p < 0.05) and had intravesical botulinum injection (19% vs 0%, p < 0.05). All the patients who had augmentation cystoplasty were in this group as well. Bowel function in terms of regular enema use was also statistically significantly higher in this group (33.4% p < 0.05). CONCLUSION: Pre-operative SSEP and UD results correlate well in patients with closed spinal dysraphism. Patients with abnormal SSEP and UD preoperatively have higher risk of urological deterioration over time. Close monitoring in this group is warranted.

Ischemic myelomalacia and closed spinal dysraphism in multiple finishing swine.

Ischemic myelomalacia secondary to fibrocartilaginous emboli (FCE) is an idiopathic disease in humans and animals. On the other hand, congenital spinal cord malformations result from neural tube defects in fetal development (ie, spinal dysraphism), with structural anomalies referred to collectively as myelodysplasia. Spinal dysraphisms are frequently accompanied by skin and vertebral abnormalities because of the embryogenic relationship. In this observational case study, we report the pathologic findings of 13, 18- to 24-weeks-old pigs from a large conventional operation that presented with acute paraparesis. Ischemic myelomalacia secondary to FCE was observed in 5 of 13 examined pigs. Congenital spinal cord malformations located between the caudal thoracic and sacral spinal cord were identified in 7 pigs, with structural abnormalities that ranged from diplomyelia/split cord malformation to segmental spinal dysgenesis (myelodysplasia) to caudal agenesis. Concurrent myelomalacia and congenital spinal cord malformations in the same or different sites were noted in 2 pigs. No spinal lesion was observed in 3 pigs. Although gross vertebral abnormalities were not observed herein, intervertebral instability due to minor defects in the articular facets, as well as other unidentified factors, is suspected to contribute high incidence of FCE. It is likely that these congenital malformations were previously underdiagnosed or are possibly new conditions associated with continuous inbreeding and genetic improvement in the modern swine industry.

Evaluating the role for regional analgesia in children with spina bifida: a retrospective observational study comparing the efficacy of regional versus systemic analgesia protocols following major urological surgery.

INTRODUCTION: Regional techniques are a key component of multimodal analgesia and help decrease opioid use perioperatively, but some techniques may not be suitable for all patients, such as those with spina bifida. We hypothesized peripheral regional catheters would reduce postoperative opioid use compared with no regional analgesia without increasing pain scores in pediatric patients with spina bifida undergoing major urological surgery. METHODS: A retrospective review of a multicenter database established for the study of enhanced recovery after surgery was performed of patients from 2009 to 2021 who underwent bladder augmentation or creation of catheterizable channels. Patients without spina bifida and those receiving epidural analgesia were excluded. Opioids were converted into morphine equivalents and normalized to patient weight. RESULTS: 158 patients with pediatric spina bifida from 7 centers were included, including 87 with and 71 without regional catheters. There were no differences in baseline patient factors. Anesthesia setup increased from median 40 min (IQR 34-51) for no regional to 64 min (IQR 40-97) for regional catheters (p<0.01). The regional catheter group had lower median intraoperative opioid usage (0.24 vs 0.80 mg/kg morphine equivalents, p<0.01) as well as lower in-hospital postoperative opioid usage (0.05 vs 0.23 mg/kg/day morphine equivalents, p<0.01). Pain scores were not higher in the regional catheters group. DISCUSSION: Continuous regional analgesia following major urological surgery in children with spina bifida was associated with a 70% intraoperative and 78% postoperative reduction in opioids without higher pain scores. This approach should be considered for similar surgical interventions in this population. TRIAL REGISTRATION NUMBER: NCT03245242.

Why are pediatric urologists unable to predict renal deterioration using urodynamics? A focused narrative review of the shortcomings of the literature.

In this focused narrative review we set out to review the current literature addressing the utilization of UDS in patients with spina bifida (SB). We specifically analyzed 6 urodynamic parameters and their roles as predictors of upper tract deterioration in pediatric SB patients. The material available did not allow a systematic analysis or the usage of metanalysis methodology, due to the predominance of small retrospective cohorts, and high heterogeneity. We identified 10 retrospective chart reviews that met our study criteria. The results of each of these papers, as well as other studies deemed relevant to the discussion, are included in our narrative review of the literature. We summarize the current literature, offer explanations for divergences in opinion, and identify future research directions and emerging solutions with a focus on machine learning.

Split cord malformation concomitant with spinal teratoma without open spinal dysraphism.

PURPOSE: Split cord malformation (SCM) presenting concomitant with spinal teratoma without any open spinal dysraphism has rarely been reported in the literature. We aimed to make a systematic review and qualitative analysis of the literature about the topic and present the first case of SCM concomitant with spinal teratoma harboring papillary thyroid carcinoma (PTC) component. METHODS: Two big search tools (Pubmed/MEDLINE) and Scopus were used. The search strategy was compatible to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). An exemplary case of ours was also presented. RESULTS: There were 30 patients (15 pediatric and 15 adult). Female and male distribution was even. Median age of the patients was 18 years (range = 0-66 years). The most common presenting symptoms were back pain and lower limb weakness. Spinal teratoma and SCM mostly presented at thoracic/thoracolumbar region in children and lumbar region in adults. Surgical outcome was better in the children compared to the adults. CONCLUSION: Thoracolumbar region is the most common location for such entity in children, whereas lumbar region for the adults. Surgical resection should be done as much as possible under neuromonitorization. The resected material should be evaluated thoroughly not to miss any malign pathology. Surgical outcome is better when it is done at an early age.

Investigating the landscape and trajectory of spina bifida research in Asia: a bibliometric analysis.

BACKGROUND: Spina bifida is a type of a neural tube defect which affects 243.14 per 100,000 babies in Asia. Research articles on spina bifida have increased in the recent years. However, no study has focused on the research trends in this field in Asia. METHODS: A systematic review of literature on spina bifida in Asia was performed using the Scopus database from inception to 2020. All published studies on spina bifida conducted in or published by authors from Asia were included in our analysis. Bibliometric information was obtained from Scopus and bibliometrics diagrams were created using VOSviewer software. RESULTS: A total of 652 articles were obtained in this study. The number of publications showed an upward trend starting 2000s. The country with the greatest number of publications was Japan while All India Institute of Medical Sciences was the most productive institution in spina bifida research in Asia. The current focus of this field in Asia was prevalence of spina bifida, prenatal diagnosis, folic acid supplementation, and complications of spina bifida. Future areas of research in spina bifida include the genetic basis of neural tube defects and the use of stem cell technology as therapies for spina bifida. CONCLUSION: This is the first bibliometric analysis on spina bifida in Asia. It showed the trend and future areas of research on spina bifida in Asia. Despite the increase in scientific literature on spina bifida research, more research outputs and collaborations are needed especially in developing countries in Asia.

A case report of serpentine-like syndrome and review of literature.

BACKGROUND: Serpentine-like syndrome (SLS) is a rare foetal abnormality, characterized by brachioesophagus, secondary intrathoracic stomach and vertebral deformity. Herein, we report a case of SLS diagnosed based on imaging, genetic examination and autopsy findings. CASE PRESENTATION: From the 19th to 23rd weeks of gestation, the foetus presented with brachioesophagus, secondary intrathoracic stomach, intrathoracic spleen with poly-spleen malformation, spinal deformity and diaphragm dysplasia, and some abdominal organs were partly located in the thoracic cavity. After extensive counselling, the couple opted to terminate the pregnancy. Whole genome sequencing and autopsy were performed. Then, the foetus was diagnosed with SLS. DISCUSSION AND CONCLUSIONS: SLS is characterized by multiorgan deformities and is associated with poor prognosis. Multiorgan deformities can be detected on prenatal sonography using three-dimensional ultrasound technology.

Variation in Definitions of Kidney Abnormality in Patients with Spina Bifida: A Systematic Scoping Review.

PURPOSE: We systematically reviewed the variability in definitions of kidney abnormality (KA) outcomes in individuals with spina bifida (SB). MATERIALS AND METHODS: A systematic scoping review was conducted using MEDLINE, Embase™, Cochrane Library, CINAHL, PsycInfo®, Web of Science™ and ClinicalTrials.gov for articles from time of database inception to September 2020. No language or patient age restrictions were applied. Primary research articles involving individuals with SB where KA was assessed as an outcome were included. Means of assessing KA and defining KA severity were abstracted. RESULTS: Of 2,034 articles found, 274 were included in the review. Most articles were published after 1990 (63.5%) and included pediatric-only populations (0-18 years; 60.5%). KA outcomes were identified by imaging-based anatomical outcomes (84.7%), serum-based outcomes (44.9%), imaging-based functional outcomes (5.5%), urine-based outcomes (3.3%) and diagnoses of end-stage kidney disease (2.6%) or chronic kidney disease otherwise unspecified (1.8%). Hydronephrosis was the most commonly used specific outcome (64.6%, 177/274) with 19.8% (35/177) of articles defining hydronephrosis severity. Hydronephrosis was used more frequently in articles with pediatric-only populations. Creatinine and cystatin-C were used in 82.1% (101/123) and 17.9% (22/123) of articles reporting serum-based outcomes, respectively, with 32.7% and 50.0% of articles defining estimated glomerular filtration rate (GFR) severity. Serum-based outcomes were more common in articles including adults >18 years. Measured GFR was assessed in 9.9% (27/274) of articles, with 44.4% (12/27) of articles defining GFR severity. CONCLUSIONS: Significant variability exists in how authors define KA with few specifically defining KA severity. Consensus and consistency in defining KA outcomes are needed.

Maternal weight as an additional first trimester spina bifida screening marker.

OBJECTIVE: To evaluate first trimester maternal weight as a spina bifida screening marker. METHODS: Case-control study of spina bifida and unaffected pregnancies; cases were from national records and controls from women referred to prenatal screening centers in the Ukraine. The median and inter-quartile range of weight, body mass index (BMI) and the obesity rate (BMI ≥ 30 kg/m2) were compared. Discriminatory power was assessed by logistic regression. Gaussian modeling was used to predict the additional spina bifida detection when weight was included as a screening marker risk in addition to first trimester biparietal diameter (BPD) and serum α-fetoprotein (AFP). RESULTS: There were 97 cases and 274 controls. The distribution of maternal weight was increased in cases by 3 kg, on average, about 5% (p < .05); BMI was increased about 8% (p < .005). Some 15% of cases were obese compared with 6.9% of controls (p < .02). In logistic regression including BMI and maternal age, 29% cases and 9.8% controls had high risk of spina bifida. Modeling predicted that incorporating weight would increase the detection rate compared with BPD and AFP alone by 3% and BMI would increase it by 4%. CONCLUSION: Incorporating maternal weight into first trimester spina bifida screening protocols will increase detection.