Mar Meléndez

Prenatal ultrasound evaluation of segmental level of neurological lesion in fetuses with myelomeningocele: development of a new technique

To report our preliminary experience in the use of prenatal ultrasound examination to assess lower‐limb movements in fetuses with myelomeningocele. We aimed to determine the accuracy of this method to establish the segmental level of neurological lesion, as this is the best known predictor of the future ability to walk.

Prenatal ultrasound evaluation of the segmental level of neurological lesion in foetuses with myelomeningocele: a new technique developing

To report our preliminary experience in the use of prenatal ultrasound examination to assess lower‐limb movements in fetuses with myelomeningocele. We aimed to determine the accuracy of this method to establish the segmental level of neurological lesion, as this is the best known predictor of the future ability to walk.

Patients with spina bifida and bladder cancer. Our experience

Results We found four patients with a mean age of 32.75 years old, one man and three women. Any patient had undergone bladder augmentation. Three patients used as mode of bladder management intermittent catheterization or permanent urethral catheter, and the male had used collector and had been diagnosed of repetitive bladder calculi. Abdominal pain was the first presenting symptom in two patients, hematuria in another one and the last one was diagnosed because of an ureteral obstruction. All of them had previous history of recurrent urinary tract infections. 75% patients had locally advanced stage (T3 or greater) or lymph node metastases at the time of diagnosis. Two patients had died at the time of the study. Conclusions Bladder cancer should be considered on in this patient population, even in young adult women. Therefore a complete screening would be beneficial for earlier detection and improved outcomes in every spina bifida patient with hematuria or chronic infection.

Functional results in young adults with spina bifida. A comparative study among two therapeutic periods

Background At least 75–85% of children born with Spina Bifida (SB) are expected to reach their early adult years. The growing adult SB population encounters a variety of complications including neurological, neurosurgical, cognitive, psychosocial, renal/urological, bowel management, mobility, musculoskeletal/orthopedic, sexual function, skin, and others. Materials and methods We compared two cohorts of adult patients with the diagnosis of Myelomeningocele, Meningocele, and Sacral anomalies associated with Meningocele, regularly controlled in our multidisciplinary SB Unit and treated in two different therapeutical periods. The first series included patients born before 1974 whereas the patients in the second series were born between 1982 and 1989. We collected the data from the medical history, radiographic records and also performed an interview with each patient included in this study. We collected information concerning two aspects: medical and social variables. The medical data included diagnosis, functional neurological level, shunted hydrocephalus, vertebral malformations, intelligence quotient (IQ), urological and faecal incontinence, reeducation of incontinence, orthosis and type of gait. The social data included level of education, employment, marital status and driving licence. We developed a questionnaire with 15 secondary conditions associated with SB which had to be filled in order of importance by all patients with normal or borderline IQ as per Wechsler Scale. Results A total of 206 patients were included in this study: 104 patients in the first series and 102 in the second one. Both series were homogeneous in relation to gender, functional neurological level and diagnosis. We found differences statistically significant in both series in relation to shunted hydrocephalus (p=0.001), spinal deformity (p=0.043), urinary re-education and collection method (p= 0.000), faecal re-education method (p=0.000), gait orthosis (p= 0.045), and educational level (p=0.000) reflecting an improvement in the second series. We found no differences in both series in relation to IQ, incontinence, ambulation, work incorporation, marital status and driving license. Regarding the personal questionnaire, there were statistically significant differences in the perception of both samples in relation to pressure ulcers (p=0.022), urinary incontinence (p=0.001), faecal incontinence (p=0.014), urinary infection (p=0.019), IQ (p=0.012), and sexual activity (p=0.000). Conclusions Management of the musculoskeletal, bowel, bladder, renal, neurological systems, and other issues in SB patients have changed and improved in the last years, regarding not only diagnostic and therapeutic procedures but also a coordinated interdisciplinary team approach that has lead these patients to reach adulthood in better conditions.

Current situation of young adults in a multidisciplinary spina bifida unit

Results A total of 100 patients were included in this study: 57 male and 43 female. The mean age was 21.3 (± 2.3, range 18–25) years. Myelomeningocele represented 91% of the sample. Regarding functional neurological level, 70% of patients belonged to lumbar (medium and low) or sacral. Hydrocephalus was present in 92% of them and 65% required the placement of a shunt. A total of 48% of the sample had a normal IQ. The 59% of the cases had some type of spinal deformity. 57 patients reached community ambulation whereas 24 were wheelchair-dependent. All patients except one case of meningocele were urine incontinent. Faecal and urinary re-education was achieved in 77.5% and 75.3% of our patients respectively. On the social aspect, the majority of our patients were single, had no children, were living with their parents and had a low educational level and work incorporation.

Bladder cancer in patients with spina bifida: observation from an adult clinic

Study designRetrospective descriptive study of patients with spina bifida and bladder cancer.ObjectivesTo compare the medical characteristics of patients diagnosed with spina bifida and bladder cancer to those patients diagnosed with spina bifida only.SettingSpina Bifida Clinic in Hospital Universitari Vall d’Hebron (Barcelona, Spain).MethodsPatients registered in the Spina Bifida Clinic between 1990 and 2015 and born before 1996 were included. We analyzed patients with confirmed bladder cancer. Demographic data, type of spina bifida, functional level, mode of bladder management, and risk factors for bladder cancer were compared between patients who developed bladder cancer and the rest of patients from the Clinic using the exact Fisher’s test.ResultsThe study included 446 patients with spina bifida, all older than 20 years. From these, ten patients also presented a bladder cancer at a mean age of 36 years (range 27–43), and with a higher proportion of mid-lumbar functional level (L3 in six out of ten patients). Eight of them were female. A wide variability of presenting symptoms was observed with locally advanced disease in nine cases. The median survival time was 4 months.ConclusionsDespite the advances in surgical and medical treatments, the prognosis of bladder cancer in patients with spina bifida is still poor. Screening tools are needed to improve the outcomes.

Ultrasound functional evaluation of fetuses with myelomeningocele: study of the interpretation of results

Abstract Objective: To assess the reliability of the interpretation of a new technique for the ultrasound evaluation of the level of neurological lesion in fetuses with myelomeningocele. Methods: Observational study including myelomeningocele fetuses, referred to our center for the sonographic assessment of the fetal lower-limb movements, made and recorded by an expert in Maternal–fetal medicine and a specialist in Rehabilitation. Two observers, with different levels of expertise and blinded to each other’s results, interpreted each recorded scan two different times. The agreement for the segmental levels assigned between the observers and the gold standard, the inter-observer and intra-observer reproducibility were tested using the weighed Kappa (wκ) index. Results: Twenty-eight scans were recorded and evaluated. The agreement between the observers and the gold standard remained constant for the expert observer (wκ = 0.82) and increased (wκ = 0.66-wκ = 0.72) for the other one. The inter-observer and the intra-observer variability for the expert observer were wκ = 0.72 and wκ = 0.94, respectively. Discussion: The agreement for the prenatal evaluation of the segmental neurological level was excellent, after a short training period, for observers with different degrees of expertise. The interpretation of this technique is reproducible enough and this supports its value for the prediction of postnatal motor function in myelomeningocele fetuses.

Poster 361: Unilateral Lower-Limb Amputation During Childhood: Outcome and Psychosocial Situation as Adults

to stand and walk, although level of performance varied. Only minor skin irritations in areas of contact with ReWalk were noted as an adverse effect. Conclusions: ReWalk enabled all the trained persons with thoracic level spinal cord injuries to transfer and ambulate short distances with crutches. A high degree of performance variability was observed across individuals. The 2 slowest subjects were unable to fully clear the foot during swing, resulting in short strides. Level of injury and other factors not completely identified may have influenced this variability.

Poster 226: Comparative Study of the Functional Results in Young Adults With Spina Bifida During Two Therapeutic Periods. A Case Series

Disclosures: R. L. Kriel, Medtronic Inc, Research grants Objective: Using a dog model, assess the safety and tolerance of intravenous (IV) baclofen. Design: Prospective, crossover, non-blinded after approval from IACUC. Setting: University veterinary science laboratory. Participants: 6 adult hound-mix dogs, 19-25 kg. Interventions: The first 2 dogs received an oral baclofen dose of 10 mg. After a 1-day washout, the same dogs received a 10-mg dose as an IV bolus (2 mg/mL concentration) over 5 minutes. The oral tablet was crushed, suspended in 5 mL of water and administered into the posterior pharynx. The dogs were not sedated. The subsequent 4 dogs were entered into a dose escalation study to determine limits of clinical tolerance and to correlate clinical observation with blood level. The same clinical observation scales were used. Boluses of 0.5, 1.0 and 1.5 mg/kg were given followed by constant infusion of baclofen (rates of 0.1, 0.2 and 0.4 mg/kg/h). Main Outcome Measures: Vital signs, modified Glasgow Coma Scale for Dogs, Discomfort and Behavior Scale. Blood samples were obtained during the observation period for determination of baclofen levels. Results: The boluses of 0.5 mg/kg and 1.0 mg/kg were tolerated with little or no clinical effect, however within 30 minutes of beginning the constant infusion of 0.2 mg/kg/h after the second bolus (1.0 mg/kg) dogs became progressively sedated and ataxic. At the highest dosages, vomiting, profuse salivation, and stupor occurred. At that time infusions were stopped and the dogs were allowed to recover while receiving IV hydration. They recovered to their baseline status within 3-6 hours. Oral bioavailability in the first 2 dogs was 0.66 and 0.69. Half-lives after intravenous administration were 3.32 and 3.58 hours indicating that baclofen was accumulating during infusions. Conclusions: IV baclofen bolus doses of 0.5 and 1.0 mg/kg were tolerated. Definite, but reversible, toxicity occurred at higher plasma baclofen concentrations.

Measuring health outcome in young adults with spina bifida

boyfriend/girlfriend (p=0.017), and not having some of the conditions of the personal questionnaire such as obesity (p=0.008), urinary infections (p=0.004), and scoliosis (p=0.014). A higher score in the HRQL was associated with a better faecal reeducation (p=0.046), gait level (p=0.043), use of gait aids (p=0.017), family economic level (p=0.020), mobility, self-care, and usual activities. A lower score in the HRQL was related to had undergone spine surgery (p=0.016), having anxiety/ depression (p=0.000), and having some of the conditions of the personal questionnaire such us pressure ulcers (p=0.010), and low self-esteem (p=0.024). In the regression model, determinant factors of having worse quality of life measured with EQ VAS were anxiety/depression, obesity, having boyfriend/girlfriend, and urinary infection. Determinant factors of having worse quality of life measured with HRQL were self-care, anxiety/depression, IQ and low self-esteem. Conclusions Almost two thirds of the series referred no problems in the health outcome. The determinants factors related to young SB patients personal perception of quality of life are not those related to disability.