The Timing of Primary Neurosurgical Repair and Wound-Site Infection in Children with Myelomeningocele

Background: The optimal time to closure of a newborn with a myelomeningocele has been the focus of a number of evaluations. The Timing of primary surgery has received significant attention due to its relationship to repair-site infection that can lead to increased morbidity and prolonged hospital stays. It is on this basis that recommendations have utilized 48 - 72 hours post birth as ideal time of closure. This is not only prevent infection at the site but also prevent ventriculitis and neural structure damage. We therefore, hypothesized an increase in wound infection rates in those patients with delays in myelomeningocele repair. Methods: We retrospectively reviewed the records of 103 children with myelomeningocele treated between 2016 and 2023. At discharge the patients were followed up at the post-operative clinic visit 2 weeks later. Children were assigned to 1 of 2 groups, those who underwent primary neurosurgical repair within 72 hours of delivery (Group 1) and those undergoing repair after 72 hours (Group 2). We compared the infection rates. Results: 103 children who underwent myelomeningocele repair were identified, with a median time from birth to treatment of 1 day. Eight (7.8 %) patients were noted to have post-repair surgical site complications. There was no significant difference in rates of infection between Group 1 and Group 2 repair times. The presence of infection was associated increased length of stay when compared to neonates without infection. Conclusion: In children with myelomeningocele, the timing of primary neurosurgical repair appears not to have a significant impact on surgical site infection. Closure of the spinal lesion within the first 72 hours of life may be more favorable for neural damage prevention. These results suggest that early myelomeningocele repair may not impart significantly on the rate of wound-site infection.

Recto-Anal Biofeedback Treatment and Quality of Life in Children with Myelomeningocele

Introduction: Myelomeningocele is one of the most complex birth defects that cause physical disability, with consequent fecal incontinence and therefore difficulty in social integration of these patients. Objective: To improve the quality of life and manometric values after biofeedback therapy. Method: Longitudinal, prospective, analytical and experimental study. Patients with myelomeningocele and fecal incontinence who were between 5 to 15 years old and their parents were included in the study. Child and parent reports of PedsQMtm generic questionnaire were collected after obtaining informed consent and assent. Anorectal manometry and first biofeedback sessions were held with each child. Following treatment completion, the PedsQMtm was applied again. Results: 17 children and their parents were included in the study. All the patients presented fecal incontinence and an inability to voluntarily evacuate rectal contents. After biofeedback, the totality of patients improved their fecal incontinence. Nine of them stop using diaper. All reported successful use of the toilet. Statistically significant differences were observed when comparing the quality of life of children and parents at the beginning and at the end of treatment. There was an improvement in quality of life after the treatment with biofeedback for both children and parents, which was more perceived by these. Conclusions: It is highly significant for the improvement both in clinic and manometric values. The improvement in quality of life is more evident in the parents.

Demographic and Clinical Characteristics of 63 Children with Myelomeningoceles

Background: Myelomeningocele (MMC) is the most common neural tube defect (NTD) characterized by the extrusion of the spinal cord into a sac filled with cerebrospinal fluid, resulting in lifelong disability. In the general population, the incidence of MMC ranges from 0.3 to 4.5/10,000 births. Live born infants with myelomeningocele have a death rate of approximately 10%. Many factors may play a role in the development of MMC such as environmental and genetic factors. In this study, we present our experience with a group of 63 children afflicted with MMC. Methods: This study was a retrospective analysis of 63 patients with MMC admitted to the neurosurgical department of Gabriel Touré Hospital from September 2017 to August 2018. A detailed history was obtained from the family at presentation. The family history and medical information before and during the pregnancy were compiled. Patients underwent complete physical and neurological examinations. Forty-seven (74.60%) patients underwent repair of the MMC and a ventriculoperitoneal shunt was placed in 12 (19%) patients with accompanying hydrocephalus. The risk factors, neurological status, and surgical results have been analyzed. Results: Of 63 children with MMC admitted to our neurosurgical department, 34 (54%) were boys and 29 (46%) were girls. Forty (63.49%) patients were the children of marriages of second cousins or closer. The mean age of the fathers was 34 years (16 - 65), while that of mothers was 26 years (16 - 38). The pregnancy was unplanned in all cases. Fourteen (22.22%) mothers had genitourinary infections, 9 (14.3%) had malaria and 57 (90.47%) mothers used analgesics and antibiotics during the pregnancy. Fifty-nine (93.65%) children were born at term, 58 (92%) were delivered via normal spontaneous vaginal delivery, and 5 (8%) via cesarean section. Lumbosacral lesions were the most frequent in 27 (42.86%) patients. Forty-seven (74.60%) patients underwent repair of the MMC and a ventriculoperitoneal shunt was placed in 12 (19%) patients with accompanying hydrocephalus. Wound infection developed in 2 cases in the postoperative period. The mortality rate was 4.3%. Conclusion: Myelomeningocele is a congenital anomaly for which several risk factors are known as well as environmental and genetic factors. This emphasizes the importance of prevention with folic acid supplementation and genetic advice.

Evaluation of the Neurodevelopmental Outcome of Toddlers with Spinal Dysraphism after Surgical Repair Using ASQ-3™ Scores

Background: Spinal dysraphism represents a wide spectrum of congenital abnormalities of the spine. Myelomeningocele is considered the most common malformation and the most common we saw in our community, with its morbidity problems seen commonly in the postoperative period. ASQ-3TM Scores are the ages and stages questionnaire, third edition, and represent a tool to assess the development progress, especially in toddlers. Objectives: Evaluation of neurodevelopmental outcome among Sudanese toddlers with spinal dysraphism after surgical closure with or without a VP shunt using ASQ-3TM Scores. Methodology: This is a retrospective hospital-based study of 84 patients who underwent myelomeningocele repair at the National Center for Neurological Sciences (NCNS) during the period from 2017 up to 2019. Data were collected through a constructed questionnaire, including ASQ-3TM Scores. Data were processed and analyzed using the Statistical Package for Social Science (SPSS) computer program. Version 25. Results: 84 patients were included in this study;all patients were diagnosed with spinal dysraphism. Out of them, 51 (60.7%) were 2 years old, 33 (39.3%) were 3 years old, 45 (53.6%) were male, 45 (53.6%) of patients mothers attended ANC irregularly, and 54 (64.3%) their mothers didn’t receive folate supplements. 44 (52.3%) of patients underwent MMC repair only, while 40 (47.7%) underwent MMC repair and VP shunt. The commonest postoperative complication was infection, reported in 12 (14.3%) of patients, followed by VP shunt revision in 9 (10.7%) of patients. Neurological assessment showed that the majority of patients need further assessment with a professional, 57 (67.9%) of children don’t walk, run, or climb like other toddlers as their parent’s state;also, half of patients (42, 50%) had medical problems, and 27 (32.1%) of their parent’s state that they do not talk like other toddlers their age. There was a statistically significant association between post-operative complications and communication development, problem-solving development, and personal social development (P value = 0.05), and a statistically significant association was found between age at repair and neurological development (P value = 0.05). Conclusion: The majority of patients had motor deficiency (particularly gross motor) and poor personal and social skills. Age at repair and postoperative complications significantly influenced the neurological development.

Lethal Consequences in an Infant with Myelomeningocele Following an Inadvertent Treatment

Myelomeningocele(MMC)is a protrusion of spinal cord contents and meninges through a vertebral defect.Iatrogenic deaths of patients with MMC are rarely encountered in forensic practice.In our case,a 3‑month‑old female was born with a lumbosacral cyst,the size of which had been increasing gradually over age.There was no neurological,orthopedic,or urologic dysfunction.On the day of her death,she received a repetitive and rapid lumbosacral cyst puncture drainage procedure,performed by an illegal medical practitioner.Postmortem autopsy findings confirmed a diagnosis of MMC and the cause of death to be cerebellar tonsillar herniation.This is a pathetic case of preventable infant death.This report suggests that the possibility of MMC should be considered in infants born with a lumbosacral cyst,and aspiration is inadvisable.Besides,forensic autopsy has a valuable role in determining the exact cause of death,identifying,or excluding iatrogenic factors that may be relevant to death following a medical procedure.A final point is that prevention programs should be developed,especially by the health care sectors to reduce such tragedy.

An Overview of Spina Bifida

A child born with untreatable birth defect encounters constant challenges in lifetime. Spina bifida is such type of defect mainly affecting neural tube. As a result, a child with spina bifida faces abnormal physical appearance to neurological dysfunctions. The incident rate of such birth defect is relatively common compared to other birth defects, therefore, an awareness among people is necessary to avoid such vulnerability. Therefore, this article provides a general outline of symptoms, types, risk factors, pathophysiology, preventive and therapeutic strategies of spina bifida which will help the general people for better understanding of the disease and be able to take precautions to combat such defect.

Pattern and Management of Neural Tube Defect in Cameroon

Objectives: The aim of study was to determine the pattern and management of neural tube defects (NTD). Methodology: It was a hospital based descriptive cross-sectional retrospective study on patients who consulted and/or were admitted at the Douala General hospital for neural tube defects from January 2005 to April 2015. Results: A total of forty-nine (49) patients were enrolled. Males constituted 59.8% and females 40.2% giving a sex ratio of 1.5 in favour of males. Most of the parents of the patients (71.5%) had a low socio-economic status. Myelomeningocele was the most common type (80.4%) followed by 17.4% cases of meningocele and 2.2% cases of lipomeningocele. Three cases (3) of encephaloceles were seen during this period. The commonest site of these defects was the lumbosacral region (47.8%). Other sites included lumbar (19;41.3%), sacral (3;6.5%) and thoracolumbar (2;4.3%) ones. About half of the patients (24;48.9%) presented with ruptured lesions. Hydrocephalus was also recorded in 65.3% of patients. Talipes equinovarus and talipes calcaneovalgus were the most common associated orthopedic birth defects found. Surgical closure was done for 44 (89.9%) patients. Ventriculoperitoneal shunting was done in 78.1% of those who presented with hydrocephalus. Post-operative complications were more frequent in patients with ruptured lesions (P = 0.001). The most common post-operative complications were wound infections (22;44.9%) and wound dehiscence (20;40.8%). Conclusion: Lumbosacral Myelomeningocele was the most common type of NTD in our region. Low socio-economic status was a common risk factor.

Assessment on self-care, mobility and social function of children with spina bifida in Turkey

The aim of the study was to investigate the functional performance in children with spina bifida, using the Pediatric Evaluation of Disability Inventory （PEDI） to look into capacity of twen- ty-eight children with spina bifida with lesions at different levels in different dimensions of self- care, mobility and social function. Mean age of the patients was 3.5 ± 2.3 （1-10） years. In the muscle test carried out, 13 patients （44.8%） had no movements including pelvic elevation in lower extremity muscles and they were at level 5. Sixteen patients （54%） were non-ambulatory according to the Hoofer ambulation classification. Raw and scale scores in the self-care, mobil- ity and social function domains both in the functional skill scale and in the caregiver scale were found to be lower compared to the data of the normal population. A statistically significant correlation was observed in the self-care values of the Functional Skills Scales and the Caregiver Assistance Scale measurements, which was positive for age and negative for Functional Ambu- lation Scale and muscle test （P 〈 0.05）. A positive relation was found between the Functional Skills Scales-mobility area and age while a negative relation was observed between Functional Ambulation Scale and muscle test （P 〈 0.005）. A negative relation was also found between Care- giver Assistance Scale-mobility and Functional Ambulation Scale and muscle test （P 〈 0.005）. In our study, the functional performance of the children was found to be low. Low-level lesions, encouraging muscular strength and independence in mobility are all very important factors for functional independence.

LL-37 induced cystitis and the receptor for advanced glycation end-products (RAGE) pathway

To elucidate pathways in bladder inflammation, we employed our physiologically relevant LL-37 induced cystitis model. Based on inflammatory studies involving other organ systems implicating the receptor for advanced glycation end-products (RAGE), we first hypothesized that RAGE is critically involved in LL-37 induced cystitis. We further hypothesized that?a common RAGE ligand high mobility group box 1 (HMGB1) is up-regulated in bladders challenged with LL-37. Finally, we hypothesized that NF-κB dependent inflammatory genes are activated in LL-37 induced cystitis. Testing our first hypothesis, C57Bl/6 mice were challenged with either saline (control) or 320 μM of LL-37 intravesically for 1 hr. After 12 or 24 hours, tissues were examined with immunohistochemistry (IHC) for RAGE, and both mRNA and protein isolation for respective qRT-PCR and Western Blot analysis. Our second hypothesis was tested by employing HMGB1 IHC. Testing our final hypothesis, qRT-PCR was performed investigating five genes: TNFα, IL-6, IL-1β, GM-CSF, COX-2. In control and LL-37 challenged tissues, IHC for RAGE revealed similar qualitative expression. Evaluation with qRT-PCR and Western Blot for RAGE revealed diminished expression at the mRNA and protein level within LL-37 challenged bladders. IHC for HMGB1 revealed a moderate qualitative increase within LL-37 challenged tissues. Finally, with the exception of TNFα, all NF-κB dependent inflammatory genes yielded substantial up-regulation. We have employed our LL-37 induced cystitis model to gain insight to wards a possible mechanistic pathway involved in bladder inflammation. This work provides data for future studies involving the inflammatory ligand HMGB1, RAGE, and receptor pathways that activate NF-κB.

In utero and exo utero fetal surgery on histogenesis of organs in animals

Until recently, fetal surgery was only used for fetuses with very poor prognosis who were likely to die without intervention. With advances in imaging, endoscopic techniques, anesthesia and novel interventions, fetal surgery is becoming a realistic option for conditions with less severe prognoses, where the aim is now to improve quality of life rather than simply allow survival. Until forty years ago, the uterus shielded the fetus from observation and therapy. Rapid changes in the diagnosis and treatment of human fetal anatomical abnormalities are due to improved fetal imaging studies, fetal sampling techniques(e.g., amniocentesis and chorionic villus sampling), and a better understanding of fetal pathophysiology derived from laboratory animals. Fetal therapy is the logical culmination of progress in fetal diagnosis. In other words, the fetus is now a patient. Now-a-days, in utero(IU) and exo utero(EU) surgical methods are popular for experimental analyses of the histogenesis of organ development. Using these surgical methods, developmental anomalies can be created and then repaired. By applying microinjection and/or fetal surgery with these methods, models of developmental anomalies such as neural tube defects, temporomandibular joint defects, hip joint defects, digit amputation, limb and digit development and regeneration, and tooth germ transplantation in the jaw could be created and later observed. After observing different types of anomalies, novel IU and EU surgical techniques would be the best approach for repairing or treating those anomalies or diseases. This review will focus on the rationale for the IU and EU creation of animal models of different organ defects or anomalies and their repair, based on analyses of organ histogenesis and pathologic observations. It will also focus in detail on the surgical techniques of both IU and EU methods.

Bone Mineral Density and Vitamin D Status in Children with Chronic Neurological Syndromes—Clinical Observations

Background: Some of the risk factors for osteoporosis population include: chronic immobilisation, insufficient dietary supply of calcium and vitamin D, decreased physical activity and long-term pharmacological treatment (glucocorticoids, anticonvulsant drugs). In disabled children and adolescents, the negative impact of these factors may cumulate to considerably impair the quality of life. Objectives: The aim of our study was to assess the vitamin D status and bone mineral density in children with chronic neurological syndromes. Material and Methods: A total of 34 children between 3 and 18 years of age were examined: 9 children with muscular dystrophy, 17 with cerebral palsy and 8 with lumbar myelomeningocele. All the subjects underwent the following assessments: measurement of the concentration of the hepatic metabolite of vitamin D and total body less head and/or lumbar spine densitometry by dual energy X-ray absorptiometry (DEXA). Low bone mass or low bone mineral density was diagnosed if the Z-score value was found to be equal to or below −2.0. Results: Indications for the above tests were chronic immobilisation or motor activity restriction, and—in 10/34 children—femoral or vertebral fracture. Vitamin D deficiency (81%). Low bone mass on densitometry was demonstrated in 27/34 and osteoporosis in 10/34 subjects (Z-score ≤−2.0). Conclusion: Bone densitometry should be included in the standard of care for children with chronic neurological syndromes, and early detection of low bone mass should be an indication for treatment with calcium and vitamin D.