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Child: Care, Health and Development Mar 2024Children with meningomyelocele may require continuous care. Consequently, there is a risk for caregiver burden and impact on family quality of life (QoL), including...
BACKGROUND
Children with meningomyelocele may require continuous care. Consequently, there is a risk for caregiver burden and impact on family quality of life (QoL), including siblings' QoL. Some studies analysed caregivers' burden and family QoL separately. However, none of these studies evaluated siblings' QoL and the associations between these three dimensions. This study investigated the associations between caregivers' burden, family QoL and siblings' QoL in Brazilian families of children with meningomyelocele and its correlations with sociodemographic, functional and clinical variables. Siblings' QoL was specifically assessed using as a parameter the QoL of typically developed Brazilian children.
METHODS
One hundred and fifty families, 150 caregivers and 68 siblings completed the Family Quality of Life Scale, Burden Interview, KIDSCREEN-27 Child and Adolescent Version and Parents Version questionnaires.
RESULTS
Most families and caregivers reported a high family QoL and a low caregiver burden. Family QoL was significantly lower as caregivers' burden increased. Caregiver's burden was significantly lower with increasing family QoL levels. Self-reported siblings' QoL was significantly worse than that of typically developed peers. There were no significant differences between self and parent-reported siblings' QoL. Self-reported siblings' QoL was significantly worse as their age increased and better with increasing family QoL levels. Parent-reported siblings' QoL was significantly worse with increasing levels of caregiver's burden and significantly better as family QoL increased. There were no significant associations with functional and clinical variables.
CONCLUSIONS
Despite the cross-sectional nature of the available data precludes any statements of causality, our results reinforce the relevance of knowing the factors that influence the QoL of families and siblings of children and adolescents with meningomyelocele and the relevance of actions aimed at reducing caregivers' burden, improving family QoL and meeting siblings' individual needs. Future multicenter studies may validate the generalizability of our findings.
Topics: Child; Humans; Adolescent; Quality of Life; Siblings; Meningomyelocele; Cross-Sectional Studies; Caregivers; Surveys and Questionnaires
PubMed: 38483061
DOI: 10.1111/cch.13246 -
Fetal Diagnosis and Therapy 2022Uterine incision based on the placental location in open maternal-fetal surgery (OMFS) has never been evaluated in regard to maternal or fetal outcomes.
INTRODUCTION
Uterine incision based on the placental location in open maternal-fetal surgery (OMFS) has never been evaluated in regard to maternal or fetal outcomes.
OBJECTIVE
The aim of this study was to investigate whether an anterior placenta was associated with increased rates of intraoperative, perioperative, antepartum, obstetric, or neonatal complications in mothers and babies who underwent OMFS for fetal myelomeningocele (fMMC) closure.
METHODS
Data from the international multicenter prospective registry of patients who underwent OMFS for fMMC closure (fMMC Consortium Registry, December 15, 2010-June 31, 2019) was used to compare fetal and maternal outcomes between anterior and posterior placental locations.
RESULTS
The placental location for 623 patients was evenly distributed between anterior (51%) and posterior (49%) locations. Intraoperative fetal bradycardia (8.3% vs. 3.0%, p = 0.005) and performance of fetal resuscitation (3.6% vs. 1.0%, p = 0.034) occurred more frequently in cases with an anterior placenta when compared to those with a posterior placenta. Obstetric outcomes including membrane separation, placental abruption, and spontaneous rupture of membranes were not different among the 2 groups. However, thinning of the hysterotomy site (27.7% vs. 17.7%, p = 0.008) occurred more frequently in cases of an anterior placenta. Gestational age (GA) at delivery (p = 0.583) and length of stay in the neonatal intensive care unit (p = 0.655) were similar between the 2 groups. Fetal incision dehiscence and wound revision were not significantly different between groups. Critical clinical outcomes including fetal demise, perinatal death, and neonatal death were all infrequent occurrences and not associated with the placental location.
CONCLUSIONS
An anterior placental location is associated with increased risk of intraoperative fetal resuscitation and increased thinning at the hysterotomy closure site. Individual institutional experiences may have varied, but the aggregate data from the fMMC Consortium did not show a significant impact on the GA at delivery or maternal or fetal clinical outcomes.
Topics: Female; Fetal Therapies; Gestational Age; Humans; Hysterotomy; Infant, Newborn; Meningomyelocele; Placenta; Pregnancy
PubMed: 34915495
DOI: 10.1159/000521379 -
Child's Nervous System : ChNS :... Jun 2021A total of 43 Italian children, aged between 6 and 16 years, diagnosed with spina bifida, myelomeningocele, and shunted hydrocephalus have been described clinically and...
PURPOSE
A total of 43 Italian children, aged between 6 and 16 years, diagnosed with spina bifida, myelomeningocele, and shunted hydrocephalus have been described clinically and completed a neuropsychological battery in order to evaluate their cognitive, personality, and behavior profile.
METHODS
Enrolled children underwent cognitive assessment by means of the Weschler WISC-IV cognitive test and assessment of the attention sustained through the LEITER test. In addition, parents were asked, in order to obtain a personality and behavior profile of the children, to fill in a "CBCL 6-18 years" questionnaire and to fill in a Barthel Index questionnaire.
RESULTS
Processing Speed Index of the WISC-IV QI scale was statistically significant (p = 0.027), with the highest value presented by autonomous patients (95.8 ± 12.8) and the lowest by patients using a wheelchair (75.5 ± 19). WISC-IV QI mean value is 98 (±15.7) for lipoma patients and 78.7 (±17.6) for LMMC and MMC patients (p = 0.001). In more detail, Perceptual Reasoning (p < 0.0005), Working Memory (p = 0.01), and Processing Speed Index (p = 0.001) highlighted a significant difference between the groups. The attention sustained subscale of the LEITER presented a mean of 6.9 (±3.1) for lipoma patients and a men value of 4.6 (±3.1) for LMMC and MMC patients (p = 0.024). Patients with hydrocephalus had statistically significant worse cognition and autonomy (Barthel Index) score (p < 0.001) compared with those without hydrocephalus, and normal scores regarding attention and depression scales.
CONCLUSION
These results can be useful in planning dedicated therapeutic protocols such as suitable rehabilitation treatments, speech therapy, psychomotor skills, and cognitive enhancement and to develop prevention protocols particularly tailored for children with hydrocephalus who appear to have the more deficient skills.
Topics: Adolescent; Child; Humans; Hydrocephalus; Male; Meningomyelocele; Neuropsychological Tests; Spinal Dysraphism; Wechsler Scales
PubMed: 33709156
DOI: 10.1007/s00381-021-05089-9 -
Neurosurgical Focus Oct 2019The goal of this study was to analyze the factors that have an impact on morbidity and mortality in patients with myelomeningocele (MMC).
OBJECTIVE
The goal of this study was to analyze the factors that have an impact on morbidity and mortality in patients with myelomeningocele (MMC).
METHODS
A retrospective cohort study was conducted to analyze factors associated with MMC that influence the morbidity and mortality of the disease. Data were collected from medical records of children who underwent the primary repair of MMC at the Fernandes Figueira Institute-Oswaldo Cruz Foundation (IFF-Fiocruz) between January 1995 and January 2015, with a minimum follow-up of 1 year. The following variables were analyzed: demographic characteristics (gestational age, sex, and birth weight); clinical features (head circumference at birth, anatomical and functional levels of MMC, hydrocephalus, symptomatic Chiari malformation type II, neurogenic bladder, and urinary tract infection [UTI]); and surgical details such as timing of repair of MMC, age at first shunt placement, shunt surgery modality (elective or emergency), concurrent surgery (correction of MMC and shunt insertion in the same surgical procedure), incidence and cause of shunt dysfunction, use of external ventricular drain, transfontanelle puncture, surgical wound complications prior to shunting, and endoscopic treatment of hydrocephalus.
RESULTS
A total of 231 patients with MMC were included in the analysis. Patients were followed for periods ranging from 1 to 20 years, with a mean of 6.9 years. The frequency of shunt placement was observed mainly among patients with MMC at the highest spinal levels (p < 0.01). The main causes of morbidity and mortality in patients with MMC were shunt failures, diagnosed in 91 of 193 cases (47.2%) of hydrocephalus, and repeated UTIs, in 129 of 231 cases (55.8%) of MMC; these were the main causes of hospitalization and death. Head circumference ≥ 38 cm at birth was found to be a significant risk factor for shunt revision (p < 0.001; 95% CI 1.092-1.354). Also, the lumbar functional level of MMC was associated with less revision than upper levels (p < 0.014; 95% CI 0.143-0.805). There was a significant association between recurrent UTI and thoracic functional level.
CONCLUSIONS
Macrocephaly at birth and higher levels of the defect have an impact on worse outcome and, therefore, are a challenge to the daily practice of pediatric neurosurgery.
Topics: Adolescent; Arnold-Chiari Malformation; Child; Child, Preschool; Female; Follow-Up Studies; Gestational Age; Humans; Hydrocephalus; Male; Meningomyelocele; Neurosurgical Procedures; Prognosis; Retrospective Studies; Ventriculoperitoneal Shunt
PubMed: 31574474
DOI: 10.3171/2019.7.FOCUS19462 -
European Spine Journal : Official... Dec 2023Lumbar kyphosis occurs in approximately 8-20% of patients with myelomeningocele (MMC). The purpose of this article is to analyze the risks and benefits of vertebrectomy...
PURPOSE
Lumbar kyphosis occurs in approximately 8-20% of patients with myelomeningocele (MMC). The purpose of this article is to analyze the risks and benefits of vertebrectomy and spinal stabilization in MMC children with severe lumbar kyphosis and to establish treatment guidelines.
METHODS
This is an IRB-approved retrospective analysis of 59 patients with MMC who underwent kyphectomy and posterior instrumentation in three centers. Average age at surgery was 7.9 years (2 weeks-17 years). Sitting trunk position, skin status, kyphosis angle, and thoracic lordosis were analyzed preoperatively, postoperatively, and at an average follow-up of 8.2 years (range 2.5-16). The correction was maintained by applying a short posterior instrumentation in 6 patients, and extending to the pelvis in 53 cases. Pelvic fixation was achieved using the Warner and Fackler technique in 24 patients, the Dunn-McCarthy in 8, Luque-Galveston in 8, sacral screws in 2, and ilio-sacral screws in 11.
RESULTS
Sitting position improved postoperatively in 47 of the 53 patients who underwent pelvic fixation and only in one patient with short instrumentation. All 6 patients with long instrumentation and poor postoperative sitting balance were in the Dunn-McCarthy fixation group. Skin sores at the apex of the deformity disappeared postoperatively in all patients but recurred in two patients with short instrumentations. Kyphosis angle improved from 109° (45°-170°) preoperatively to 10° (0°-45°) postoperatively and 21° (0°-55°) at last follow-up. The best results were seen in cases where a cross-k-wire fixation of the kyphectomy site was used, augmented with a long thoraco-pelvic instrumentation consisting of Luque sublaminar wires in the thoracic region and a Warner-Fackler type of pelvic fixation. Good results were also found with the bipolar technique and ilio-sacral screw fixation. Six over 24 patients with the Warner and Fackler technique showed gradual dislodgment or hardware failure, with subsequent nonunion of the kyphectomy site in four. Infection, with or without wound dehiscence and/or hardware exposure, occurred in 17 cases, necessitating hardware removal in 9 patients.
CONCLUSION
Lumbar kyphosis in MMC children is best managed by resection of enough vertebrae from the apex to produce a flat lumbar spine, with perfect bone-to-bone contact and long thoraco-pelvic instrumentation using the Warner and Fackler technique through the S1 foramina or the bipolar technique with ilio-sacral screw fixation. Additional local fixation of the osteotomy site using cross-wires with or without cerclage increases the stability of the construct. The majority of complications occurred in patients with short instrumentations or where residual kyphosis persisted postoperatively regardless of the type of pelvic fixation or hardware density. The Dunn-McCarthy technique for pelvic fixation following kyphectomy in MMC was less successful in producing stable pelvic fixation and should not be considered in this patient category.
Topics: Child; Humans; Meningomyelocele; Retrospective Studies; Treatment Outcome; Kyphosis; Scoliosis; Lumbar Vertebrae; Risk Factors; Spinal Fusion
PubMed: 37698696
DOI: 10.1007/s00586-023-07924-w -
Child's Nervous System : ChNS :... Apr 2020Fetal repair of myelomeningocele has been increasingly offered to mothers of children with myelomeningocele after the seminal Management of Myelomeningocele (MOMs)...
Fetal repair of myelomeningocele has been increasingly offered to mothers of children with myelomeningocele after the seminal Management of Myelomeningocele (MOMs) trial, which demonstrated decreased reliance on ventriculoperitoneal shunt following fetal closure. We present the case of a fetus diagnosed with a lumbar myelomeningocele in utero whose mother refused in utero closure and who was subsequently born with a skin-covered defect. A fetal MRI was obtained on a mother with a male fetus diagnosed with open neural tube defect at 20 weeks of gestation. The child demonstrated spinal dysraphism extending from L2 to L5 and associated Chiari II malformation with lateral and third ventriculomegaly. Based on our institutional criteria and the criteria of the MOMs trial, the parents were offered fetal repair of the myelomeningocele; however, they declined because of concerns about risks to the mother. At birth, the patient was found to have a skin-covered meningocele. He underwent elective repair of his occult meningocele and detethering of his spinal cord. Intraoperative findings demonstrated spinal nerve roots attached to the arachnoid within the defect, and a closed, tubularized neural placode. This represents a unique case in which a fetus with a clinical picture consistent with open spinal defect was found to have a lesion more consistent with meningocele on postnatal operative interrogation. Knowledge that this can occur should be taken into consideration when discussing fetal closure, although the frequency of this occurrence is not known. Additionally, identification of this case sheds light on the mechanism by which occult myelomeningoceles form.
Topics: Child; Female; Fetus; Humans; Hydrocephalus; Infant, Newborn; Male; Meningomyelocele; Spinal Dysraphism; Ventriculoperitoneal Shunt
PubMed: 32025870
DOI: 10.1007/s00381-019-04454-z -
Prenatal Diagnosis Jun 2023Maternal-fetal interventions-such as prenatal fetal myelomeningocele (MMC) repair-are at the forefront of clinical innovation within maternal-fetal medicine, pediatric... (Review)
Review
Maternal-fetal interventions-such as prenatal fetal myelomeningocele (MMC) repair-are at the forefront of clinical innovation within maternal-fetal medicine, pediatric surgery, and neonatology. Many centers determine eligibility for innovative procedures using pre-determined inclusion and exclusion criteria based on seminal studies, for example, the "Management of Myelomeningocele Study" for prenatal MMC repair. What if a person's clinical presentation does not conform to predetermined criteria for maternal-fetal intervention? Does changing criteria on a case-by-case basis (i.e., ad hoc) constitute an innovation in practice and flexible personalized care or transgression of commonly held standards with potential negative consequences? We outline principle-based, bioethically justified answers to these questions using fetal MMC repair as an example. We pay special attention to the historical origins of inclusion and exclusion criteria, risks and benefits to the pregnant person and the fetus, and team dynamics. We include recommendations for maternal-fetal centers facing these questions.
Topics: Pregnancy; Child; Female; Humans; Meningomyelocele; Fetus; Prenatal Care; Family; Decision Making
PubMed: 37139690
DOI: 10.1002/pd.6363 -
Child's Nervous System : ChNS :... Sep 2021Myelomeningocele (MMC), the commonest type of spina bifida (SB), occurs due to abnormal development of the neural tube and manifest as failure of the complete fusion of... (Review)
Review
Myelomeningocele (MMC), the commonest type of spina bifida (SB), occurs due to abnormal development of the neural tube and manifest as failure of the complete fusion of posterior arches of the spinal column, leading to dysplastic growth of the spinal cord and meninges. It is associated with several degrees of motor and sensory deficits below the level of the lesion, as well as skeletal deformities, bladder and bowel incontinence, and sexual dysfunction. These children might develop varying degrees of neuropsychomotor delay, partly due to the severity of the injuries that affect the nervous system before birth, partly due to the related cerebral malformations (notably hydrocephalus-which may also lead to an increase in intracranial pressure-and Chiari II deformity). Traditionally, MMC was repaired surgically just after birth; however, intrauterine correction of MMC has been shown to have several potential benefits, including better sensorimotor outcomes (since exposure to amniotic fluid and its consequent deleterious effects is shortened) and reduced rates of hydrocephalus, among others. Fetal surgery for myelomeningocele, nevertheless, would not have been made possible without the development of experimental models of this pathological condition. Hence, the aim of the current article is to provide an overview of the animal models of MMC that were used over the years and describe how this knowledge has been translated into the fetal treatment of MMC in humans.
Topics: Animals; Arnold-Chiari Malformation; Female; Fetal Therapies; Humans; Hydrocephalus; Meningomyelocele; Pregnancy; Spinal Dysraphism
PubMed: 34333685
DOI: 10.1007/s00381-021-05299-1 -
NeoReviews Dec 2019Spinal dysraphism, which includes conditions such as myelomeningocele and sacral agenesis, is one of the most common causes of congenital lower urinary tract... (Review)
Review
Spinal dysraphism, which includes conditions such as myelomeningocele and sacral agenesis, is one of the most common causes of congenital lower urinary tract dysfunction. Early evaluation of the neurogenic bladder serves to minimize renal damage, and the main goals of management include preserving renal function, achieving acceptable continence, and optimizing quality of life. The survival of patients with such conditions has improved to greater than 80% reaching adulthood, owing to advances in diagnostic and therapeutic modalities. The result is a real, and unfortunately often unmet, need for successful transitional care in this complex patient population. Clinicians must be able to identify the unique challenges encountered by patients with neurogenic bladder as they shift through different stages of their life.
Topics: Abnormalities, Multiple; Acetylcholine Release Inhibitors; Botulinum Toxins, Type A; Cholinergic Antagonists; Fetal Therapies; Humans; Hydronephrosis; Meningocele; Meningomyelocele; Sacrococcygeal Region; Spina Bifida Occulta; Spinal Dysraphism; Transition to Adult Care; Ultrasonography, Prenatal; Urinary Bladder; Urinary Bladder, Neurogenic; Urinary Catheterization; Urinary Tract Infections; Urodynamics; Urology; Vesico-Ureteral Reflux; Watchful Waiting
PubMed: 31792158
DOI: 10.1542/neo.20-12-e711 -
The Pan African Medical Journal 2022
Topics: Humans; Spinal Dysraphism; Meningomyelocele
PubMed: 36338552
DOI: 10.11604/pamj.2022.42.258.35894