Randomized Clinical Trial Using Sterile Single Use and Reused Polyvinylchloride Catheters for Intermittent Catheterization with a Clean Technique in Spina Bifida Cases: Short-Term Urinary Tract Infection Outcomes.

PURPOSE: Urinary tract infections are common and severe complications in patients with spina bifida. Management includes intermittent bladder catheterization with single use or reused sterile catheters. There is insufficient evidence to set a standard among the different techniques. We determined whether single use polyvinylchloride catheters would reduce urinary tract infections compared to reused polyvinylchloride catheters in patients with neurogenic bladder due to spina bifida. MATERIALS AND METHODS: We performed a 2-arm randomized parallel clinical trial from 2015 to 2016 with an 8-week followup at our center in patients with neurogenic bladder caused by spina bifida. Patients were divided into single use and reused polyvinylchloride catheter groups. Evaluations were done on days 0, 7, 14, 28, 42 and 56. Participants reported symptoms and urine cultures were obtained. The primary outcome was urinary tract infection frequency, defined as positive urine culture plus fever, flank pain, malaise, or cloudy or odorous urine. Study eligibility criteria were age 2 years or greater, spina bifida diagnosis with regular clean intermittent bladder catheterization and no urinary tract infection at initial evaluation. RESULTS: The calculated sample size was 75. Of the patients 135 were screened, 83 were randomized and 75 completed followup. Mean age was 12.7 years (range 2-56) and there were 29 males and 46 females. No statistical difference was found between the single use vs reused catheter groups in the frequency of asymptomatic bacteriuria (32.4% vs 23.7%, p = 0.398) or urinary tract infections (35.2% vs 36.8%, p = 0.877). CONCLUSIONS: Single use polyvinylchloride catheters for intermittent bladder catheterization did not decrease the incidence of urinary tract infections in our patients with neurogenic bladder compared to reused polyvinylchloride catheters. These results are consistent with the 2014 Cochrane Review.

Cryopreserved human umbilical cord patch for in-utero spina bifida repair.

OBJECTIVE: To identify a patch system to repair surgically created spina bifida in a sheep model for its efficacy in healing the skin defect, protecting the underlying spinal cord and reducing the Chiari II malformation. METHODS: Spina bifida was created surgically in 16 fetuses from eight timed-pregnant sheep at gestational age of 75 days. Two fetuses did not survive the procedure. Repeat hysterotomy was performed at 95 days' gestation to cover the defect with either biocellulose film with underwater adhesive (BCF-adhesive) (n = 7) or human umbilical cord with suture (HUC-suture) (n = 7). Three fetuses without formation of the defect served as reference controls. The skin healing was examined by direct visualization after a planned Cesarean section at term, followed by histological analysis using hematoxylin and eosin and Masson's trichrome stains. Mid-sagittal sections of the fetal cranium and upper cervical spine were analyzed by a pediatric neuroradiologist who was blinded to the type of patch received. RESULTS: Three fetuses that received the BCF-adhesive and six fetuses that received the HUC-suture survived to term for final analysis. As a result of dislodgment of the BCF-adhesive, all spina bifida defects repaired using BCF-adhesive were not healed and showed exposed spinal cord with leakage of cerebrospinal fluid. In contrast, all spinal defects repaired by HUC-suture were healed with complete regrowth of epidermal, dermal and subdermal tissue components, with no exposed spinal cord. The maximal skin wound width was 21 ± 3.6 mm in the BCF-adhesive group but 3 ± 0.8 mm in the HUC-suture group (P < 0.001). The spinal cord area (P = 0.001) and the number of anterior horn cells (P = 0.03) was preserved to a greater degree in the HUC-suture group than in the BCF-adhesive group, whilst psammoma bodies, signifying neuronal degeneration, were only observed in the BCF-adhesive group. Anatomic changes, indicative of Chiari II malformation, were seen in all three fetuses of the BCF-adhesive group but in none of the HUC-suture group (P < 0.01). CONCLUSION: Cryopreserved umbilical cord graft is a promising regenerative patch for intrauterine repair of spina bifida.

Cotransplantation with specific populations of spina bifida bone marrow stem/progenitor cells enhances urinary bladder regeneration.

Spina bifida (SB) patients afflicted with myelomeningocele typically possess a neurogenic urinary bladder and exhibit varying degrees of bladder dysfunction. Although surgical intervention in the form of enterocystoplasty is the current standard of care in which to remedy the neurogenic bladder, it is still a stop-gap measure and is associated with many complications due to the use of bowel as a source of replacement tissue. Contemporary bladder tissue engineering strategies lack the ability to reform bladder smooth muscle, vasculature, and promote peripheral nerve tissue growth when using autologous populations of cells. Within the context of this study, we demonstrate the role of two specific populations of bone marrow (BM) stem/progenitor cells used in combination with a synthetic elastomeric scaffold that provides a unique and alternative means to current bladder regeneration approaches. In vitro differentiation, gene expression, and proliferation are similar among donor mesenchymal stem cells (MSCs), whereas poly(1,8-octanediol-cocitrate) scaffolds seeded with SB BM MSCs perform analogously to control counterparts with regard to bladder smooth muscle wall formation in vivo. SB CD34(+) hematopoietic stem/progenitor cells cotransplanted with donor-matched MSCs cause a dramatic increase in tissue vascularization as well as an induction of peripheral nerve growth in grafted areas compared with samples not seeded with hematopoietic stem/progenitor cells. Finally, MSC/CD34(+) grafts provided the impetus for rapid urothelium regeneration. Data suggest that autologous BM stem/progenitor cells may be used as alternate, nonpathogenic cell sources for SB patient-specific bladder tissue regeneration in lieu of current enterocystoplasty procedures and have implications for other bladder regenerative therapies.

Spina bifida and dental care: key clinical issues.

Spina bifida is a birth defect affecting the spinal column, resulting from failure of neural tube closure during the first month in utero. It is associated with varying degrees of neurologic and orthopedic impairment. This article presents an overview of spina bifida discussing its correlation with dental caries, latex allergy, pulmonary function, craniosynostosis, and morphology of sella turcica, and explains the role of the dentist in countering these problems.

Health care expenditures among children with and those without spina bifida enrolled in Medicaid in North Carolina.

BACKGROUND: National data on health care use among children with special needs are limited and do not address children with spina bifida (SB). One recent study examined health care costs during 2003 among privately insured individuals with SB. Our objective was to compare health care use and expenditures among publicly insured children with SB to children without a major birth defect and among children with SB with and without hydrocephalus. METHODS: Data from the North Carolina Birth Defects Monitoring Program and Medicaid were linked to identify continuously enrolled children with SB (case children) and children without a major birth defect (control children) born from 1995 to 2002. Medicaid expenditures per child for medical, inpatient, outpatient, dental, well-child care, developmental/behavioral services, and home health for those aged 0 to 4 years old were calculated for case and control children and for case children with and without hydrocephalus. RESULTS: Of 373 case children who survived infancy, 205 (55%) were enrolled in Medicaid. Expenditures were assessed for 144 case and 5674 control children aged 0 to 4 years old continuously enrolled in Medicaid. During infancy, mean expenditure was $33,135 per child with SB and $3900 per unaffected child. The biggest relative expenditures were for developmental/behavioral services (82 times higher for case than control child [$1401 vs. $17]) and home health services (20 times higher [$821 vs. $41]). Average expenditure for an infant with SB and hydrocephalus was 2.6 times higher than an infant with SB without hydrocephalus ($40,502 vs. $15,699). CONCLUSIONS: Expenditure comparisons by SB subtype are important for targeting health care resources.

Effects of latex avoidance on latex sensitization, atopy and allergic diseases in patients with spina bifida.

BACKGROUND: Ten years ago, avoidance measures such as the performance of latex-free operations were implemented in children with spina bifida. Since then, latex sensitization and latex allergy have decreased in this high-risk group. OBJECTIVE: To study the effect of primary latex-free prophylaxis on the prevalence of allergic diseases and atopy as a marker for sensitization spreading in children with spina bifida. METHODS: One hundred and twenty children with spina bifida born after the introduction of latex-free prophylaxis and operated on under latex-free conditions ('current group') were examined for latex sensitization, latex allergy, sensitization to aero- and food allergens and allergic diseases. Results were compared to a 'historic' (not latex-free operated) group of children with spina bifida and comparable age (n = 87) and to a recent sample of children from the general population (n = 12,403). RESULTS: In comparison with the 'historic group', latex sensitization (55% vs 5%, P < 0.001) and latex allergy (37% vs 0.8%, P < 0.001) were significantly reduced in the 'current group'. Furthermore, a significant reduction could be demonstrated for sensitization to aeroallergens (41.4% vs 20.8%, P = 0.001) and for allergic diseases (35% vs 15%, P = 0.001). The prevalence for atopy, sensitization to aero-/foodallergens and for allergic diseases in children of the 'current group' was similar to those in children of the weighted population sample. CONCLUSIONS: Latex avoidance in children with spina bifida prevents latex sensitization and latex allergy. Additionally, it also seems to prevent sensitization to other allergens and allergic diseases which might be explained by the prevention of sensitization spreading.

The Cole Midfoot Osteotomy: Clinical and Radiographic Retrospective Review of Five Patients (Six Feet) with Different Etiologies.

BACKGROUND: Cole osteotomy is performed in patients having a cavus deformity with the apex of the deformity in the midfoot. Correction of the deformity at this midfoot level improves foot and ankle stability by creating a plantigrade foot. We retrospectively reviewed the clinical and radiographic results of six feet (five patients) that underwent Cole midfoot osteotomy (2011-2015). METHODS: The patients had different etiologies (spastic cerebral palsy, burn sequelae, spina bifida, and Charcot-Marie-Tooth disease). Dorsal and slightly laterally based transverse wedge osteotomy through the navicular bone medially and the cuboid bone laterally was performed. Patients were under routine clinical follow-up. We evaluated clinical and radiographic results. RESULTS: Mean clinical follow-up was 15.7 months (range, 6-36 months). The mean preoperative and postoperative talo-first metatarsal angles on lateral radiographs were 29.9° and 8.7°, respectively (P < .05) and on anteroposterior radiographs were 30.3° and 8.6° (P < .05). The mean preoperative talocalcaneal angle on anteroposterior radiographs increased from 19.2° to 29.8° postoperatively (P < .05). The mean postoperative calcaneal pitch angle change was 10.8° on the lateral radiograph (P < .05). At final follow-up, all five patients were independently active, had plantigrade feet, and were able to wear conventional shoes. The mean American Orthopaedic Foot and Ankle Society questionnaire score was 38.8 preoperatively and 79.5 postoperatively (P < .05). Only one patient did not have full bony union. Achilles tightness was seen in one patient. CONCLUSIONS: Cole midfoot osteotomy is a laboring procedure to correct adult pes cavus deformity with the apex in midfoot, although having some complication risks.

Latex allergy: two educational cases.

Natural rubber latex (NRL) allergy is a significant problem both for health care workers and for children with complex medical and surgical conditions that require multiple surgical interventions. Primary and secondary prophylaxis are effective measures in identified high risk groups, such as spina bifida (SB). It is therefore likely that with proper attention to prevention and secondary prophylaxis in the highest risk groups that the numbers of paediatric SB patients with NRL allergy will continue to decrease. In contrast medical awareness of established latex allergy needs to be maintained. The issue of latex sensitisation via fruit and food allergy will also remain, so some of the attention that SB patients have received in the past may need to be refocussed onto other emerging high risk groups. Innovative immunomodulatory approaches may soon translate to the clinic for latex sensitised or allergic subjects.

Persistent torticollis, facial asymmetry, grooved tongue, and dolicho-odontoid process in connection with atlas malformation complex in three family subjects.

Congenital clefts and other malformations of the atlas are incidental findings identified while investigating the cervical spine following trauma. A persistent bifid anterior and posterior arch of the atlas beyond the age of 3-4 years is observed in skeletal dysplasias, Goldenhar syndrome, Conradi syndrome, and Down's syndrome. There is a high incidence of both anterior and posterior spina bifida of the atlas in patients with metabolic disorders, such as Morquio's syndrome [Baraitser and Winter in London dysmorphology database, Oxford University Press, 2005; Torriani, Lourenco in Rev Hosp Clin Fac Med Sao Paulo 53: 73-76, 2002]. We report two siblings and their mother, with congenital, persistent torticollis, plagiocephaly, facial asymmetry, grooved tongues, and asymptomatic "dolicho-odontoid process". All are of normal intelligence. No associated Neurological dysfunction, paresis, apnoea, or failures to thrive were encountered. Radiographs of the cervical spine were non-contributory, but 3D CT scanning of this area allowed further visualisation of the cervico-cranial malformation complex in this family and might possibly explain the sudden early juvenile mortality. Agenesis of the posterior arch of the atlas and bifidity/clefting of anterior arch of the atlas associated with asymptomatic "dolicho-odontoid process" were the hallmark in the proband and his female sibling. Some of the features were present in the mother. All the family subjects were investigated. To the best of our knowledge the constellation of malformation complex in this family has not been previously reported.

Non-traditional management of the neurogenic bladder: tissue engineering and neuromodulation.

Patients with spina bifida and a neurogenic bladder have traditionally been managed with clean intermittent catheterization and pharmacotherapy in order to treat abnormal bladder wall dynamics, protect the upper urinary tract from damage, and achieve urinary continence. However, some patients will fail this therapy and require surgical reconstruction in the form of bladder augmentation surgery using reconfigured intestine or stomach to increase the bladder capacity while reducing the internal storage pressure. Despite functional success of bladder augmentation in achieving a low pressure reservoir, there are several associated complications of this operation and patients do not have the ability to volitionally void. For these reasons, alternative treatments have been sought. Two exciting alternative approaches that are currently being investigated are tissue engineering and neuromodulation. Tissue engineering aims to create new bladder tissue for replacement purposes with both "seeded" and "unseeded" technology. Advances in the fields of nanotechnology and stem cell biology have further enhanced these tissue engineering technologies. Neuromodulation therapies directly address the root of the problem in patients with spina bifida and a neurogenic bladder, namely the abnormal relationship between the nerves and the bladder wall. These therapies include transurethral bladder electrostimulation, sacral neuromodulation, and neurosurgical techniques such as selective sacral rhizotomy and artificial somatic-autonomic reflex pathway construction. This review will discuss both tissue engineering techniques and neuromodulation therapies in more detail including rationale, experimental data, current status of clinical application, and future direction.

Intraoperative anaphylactic reaction due to latex hypersensitivity.

Severe anaphylactic reaction secondary to latex allergy has lately been recognized and reported especially in individuals with spina bifida. We report a case of severe intraoperative anaphylactic reaction due to latex allergy. Preoperative testing for latex allergy may be helpful in determining latex allergy. We suggest a preoperative management protocol for patients who are thought to have latex allergy. An increased awareness to latex allergy will help avoid this potentially catastrophic event.

A child with spina bifida, cerebral palsy and juvenile rheumatoid arthritis: rehabilitation challenge.

PURPOSE: A child with spina bifida, cerebral palsy and juvenile rheumatoid arthritis is presented, and strategies to approach a patient with multiple paediatric onset disabling conditions with possible overlaps are discussed. CONCLUSION: The value of multidisciplinary team approach including physiatrist, physical therapist, occupational therapist, rehabilitation nurse, prosthetist-orthotist, psychologist, speech-language pathologist, paediatric rheumatologist, social worker, kinesiotherapist, dietitian, recreation therapist, dentist and other disciplines as required is emphasized.

High risk of sensitization to latex in children with spina bifida.

The risk of per-operative anaphylactic shock in response to latex is established in children with spina bifida. This study focuses on 30 children with spina bifida and aims at evaluating the value of predictive tests (skin tests and RAST). No patient had shown a preoperative accident. Hypersensitivity to latex, sometimes suspected by questioning of the patient, was detected 12 times out of 30 (40%). 10 children of the 12 were atopic (83%). The incidence of atopy is significantly higher than that of the general pediatric population (14 out of 30; 46%). This study demonstrates the frequency of the latent hypersensitivity to latex and the subsequent potential risk of per-operative anaphylactic shock. This sensitization can be partly explained by multiple contacts with latex; urinary catheterization several times a day, daily use of latex gloves for routine care, repetitive surgery (more than 8 operations per child). However, atopy increases the risk of sensitization to latex. The authors underline the importance of taking preventive measures for these children such as the use of PVC, silicone or polyurethane catheters and of synthetic rubber. Hypoallergenic gloves are under investigation. A questionnaire and systematic preventive cutaneous assessment before each operation are strongly advised.

Latex allergy in the operating room: case report and a brief review of the literature.

A patient with a history of spina bifida and cerebral palsy was anesthetized for an ileal conduit. The procedure was uneventful until penetration of the abdominal cavity, immediately after which the patient suffered severe hypotension and her peak inspiratory pressure doubled. The patient's skin became flushed and the capnogram tracing was consistent with bronchospasm. The patient was treated with phenylephrine hydrochloride (Neo-Synephrine), and then epinephrine. This was followed with an epinephrine infusion, intravenous (i.v.) methylprednisolone sodium succinate (Solu-Medrol), inhaled albuterol sulfate, and diphenhydramine hydrochloride (Benadryl) i.v.. The patient responded to these interventions and the procedure was completed without further incident. A diagnosis of latex allergy was made based on the patient's clinical status, underlying diseases, and positive postoperative radioallergosorbent test (RAST). Anesthesiologists must be able to diagnose the signs and symptoms of allergic reactions in patients under anesthesia. This article will review the types of hypersensitivity reactions involved, define the risk groups, and examine the treatment protocols available for latex allergy.

Latex allergy--a clinical perspective.

Latex allergy has been increasingly recognized in the past 6 years. Defined risk groups are health-care workers, spina bifida children, and possibly food allergy patients. Powdered latex gloves and latex barium enema tips are products most commonly associated with allergy. Clinically, patients present with contact urticaria, allergic rhinoconjunctivitis, asthma, and anaphylaxis. At least 15 deaths are reported to have occurred because of latex allergic reactions. Severe occupational latex allergy can result in removal of the affected individual from the workplace. This will have important cost implications. The only presently available treatment of latex allergy is avoidance.

Latex allergies in children with spina bifida: relevance for the pediatric dentist.

Latex is ubiquitous in pediatric dentistry and medical practice. Children with spina bifida and other urogenital abnormalities are at great risk for hypersensitivity reactions during dental treatment. Four representative cases of children with latex allergies at one institution are presented. A latex-avoidance protocol is presented with suggested instrument and equipment alternatives.

Latex allergy in patients with spina bifida.

Latex recently has been associated with severe intraoperative IgE-mediated anaphylactic reactions. Pediatric patients with meningomyelocele (spina bifida) appear to be specifically at risk for this type of reaction. This article provides background information on the etiology of latex allergies, identifies some commonly used dental products containing latex, recommends some alternatives to use, and suggests precautions the dental practitioner should take when treating high-risk individuals such as spina bifida patients.

Dental surgery in pediatric patients with spina bifida and latex allergy.

Dental rehabilitation is a common outpatient pediatric surgical procedure. It requires the dentist to restore or extract the teeth of children with severe tooth decay or poor dental hygiene. Medical, physical, or emotional problems can make safe treatment in an outpatient setting impossible. These children require sedation or anesthesia for the dentist to gain access to their oral cavities. This article discusses caring for pediatric patients with spina bifida and latex allergy undergoing dental rehabilitation. Perioperative nurses must act as patient advocates in providing a latex-safe environment and also ensure quality care.

[Latex allergy and orthopedic surgery]. / Allergie au latex et chirurgie orthopédique.

PURPOSE OF THE STUDY: Latex allergy is becoming a subject of extreme concern, especially in patients who have undergone multiple operations and/or have after effects of myelodysplasia. Presenting their data, the authors wish to make it aware to orthopaedic surgeons focusing on different risk groups, and at the same time advising on medical examinations, of the various measures to take. MATERIAL AND METHODS: Twenty patients were tested (17 with spina bifida and 3 with cerebral palsy) for latex and ethylene oxide allergy, and with certain fruits known for their cross-reactivity. Atopic history has also been researched. The immunologic examination had been carried out by means of skin-tests with evidence of specific IgE antibodies being found. RESULTS: Out of the twenty patients tested, eight had clinical signs of a latex allergy while the twelve others were classified as being at risk. The results show that 11 out of 20 are allergic to latex, 4 of them have an allergy to ethylene oxide, and 7 have food allergies: banana (2), kiwi (1), chestnut (1), hazelnut (3). DISCUSSION: We confirm: 1) the increased risk of a latex allergy among patients with spina bifida: 47 per cent in our study; 2) the association of an ethylene oxide allergy, practically the only product used in the sterilisation of medical equipment; 3) a more and more frequent association to food allergies, in particular to fruits: 7 out of 11 patients in our study compared to 50 per cent in other published studies. The classical subject of atopy is not found here. CONCLUSION: Against the increase of latex allergy, it is necessary to propose a strategy: firstly to determine the groups at risk: patients who have undergone multiple operations, atopic persons, isolated cases of food allergies, spina bifida cases. Then, to test them and if necessary, taking draconian measures concerning their medical environment; to identify them; to provide them with auto-injectable epinephrine; to give premedication, though with no success guarantee in case of anticipated surgical procedure. Finally, we recommend to manufacturers that all equipment and products should be free of latex.

[Use of polydimethylsiloxane in the endoscopic treatment of urinary incontinence of neurologic origin in children]. / Utilisation du polydiméthylsiloxane dans le traitement endoscopique de l'incontinence urinaire d'origine neurologique chez l'enfant.

AIM: We report the use of polydimethylsiloxane (PDS-Macroplastique) in endoscopic treatment of urinary incontinence in children with neurogenic bladder and try to determine optimal criteria for patient selection. METHODS: Forty four children (19 males, 25 females) have been treated since 1995. Aetiology was mainly spina bifida (n = 36). Previous surgery had been performed in 26 patients, including bladder neck reconstruction in 21 cases and bladder augmentation in 15 cases. Mean age at injection was 13 years (7 to 17). Only one injection was performed in 27 patients, two injections in 4 cases, and three and more injections in 3 cases. Mean volume at each injection was 3.6 cc. Mean interval between two injections was 6 months (3-15 m). All injections were performed transurethrally. RESULTS: Follow-up ranged from 6 to 41 months (median: 23). Fifteen patients (34%) are dry (continence > 4 hours, no urinary pad during the day) and 11 (25%) are improved (continence from 2 to 3 hours, minimal pad). Eighteen patients obtained poor results. In the entire series previous bladder neck surgery or preoperative detrusor hyperactivity did not interfere with the results. The only difference concerns the sex-ratio: the good results were mainly seen in females: 44% of girls are cured versus 21% of boys. CONCLUSION: Injection of PDS in the bladder neck achieve the goal of continence in 34% of the cases in neurogenic bladder. Better results are seen in girls. Injection does not compromise other surgical procedures. The use of PDS seems more suitable than bovine collagen in view of the potential problems of prions.