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Investigations of hydrocephalus in myelomeningocele
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Hydrocephalus is one of the most common complications of spinal dysraphism. Although few patients require cerebrospinal fluid diversion immediately at birth or within the first few days of life, most patients with myelomeningocele, which comprises the most prevalent, clinically significant form of spina bifida, will eventually need surgical treatme...
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... Occipito-frontal circumference measurement is important because the patent fontanelles and calvarial sutures may mask overt signs of raised intracranial pressure due to increasing head size, though there may be significant pathology in the brain. When OFC crosses centiles or increases rapidly, surgical intervention is mostly indicated [11]. ...
... It is easy to carry out, cheap, and widely available. It can assess ventricular size, evaluate other anatomic anomalies, and detect other pathologies such as intraventricular hemorrhage [11]. ...
... It is a sensitive and widely available modality, but with exposure to radiation, there are concerns for the risk of tumors and adverse effects on cognition (Figure 4) [11]. ...
Hydrocephalus (HCP) is one of the most common associations of myelomeningocele, and it may be overt and present at birth or be latent and develop following the repair of myelomeningocele. In patients with myelomeningocele, aqueductal stenosis, fourth ventricular obstruction, subarachnoid obstruction at the tentorial hiatus, and the crowded posterior fossa, which are all related to Chiari II malformation, are the various causes of hydrocephalus. The clinical manifestations depend on the age at presentation, but most patients present with macrocephaly and craniofacial disproportion, increasing head size, bulging anterior fontanelle, calvaria sutural diastasis, distended scalp veins, poor feeding as well as signs of raised intracranial pressure such as vomiting, headache, and altered consciousness. Diagnosis is based on clinical features and supportive radiological investigations such as transcranial ultrasound, brain computerized tomographic scan, and brain magnetic resonance imaging. Prompt treatment is very important to obtain optimal clinical outcomes, and this may be by inserting a shunt or performing endoscopic third ventriculostomy with or without choroid plexus cauterization.
... Hydrocephalus (HCP) is associated with myelomeningocele (2,3) in 35% -90% of cases . It may be present at birth or be evident a few days to weeks following repa ir of MMC ; about 84% to 89% of those who develop hydrocephalus may (4,5) ultimately require diversion of cerebrospinal fluid . Studies in India and the United States evaluated different sonographic and computerized tomographic parameters such as bifrontal diameter, bicaudate diameter, and diameter of the body of the lateral ventricle, fronto-occipital horn ratio, ventricular index and thalamo-occipital distance and found them to be predictive of hydrocephalus post (3,6) myelomeningocele repair . ...
Background: Clinical and transcranial ultrasound parameters may be used to predict the risk of developing hydrocephalus after myelomeningocele in infants. Objective: To determine the risk of developing hydrocephalus after repair of myelomeningocele using pre- existing preoperative clinical and transcranial sonographic parameters.
Method: Prospective cohort study of 28 patients with myelomeningocele who presented to a tertiary hospital in southwestern Nigeria, from November 2016 to October 2018.
Transcranial ultrasound was done before myelomeningocele repair and patients followed up for six months to detect hydrocephalus. Analysis of the state of the anterior fontanelle, the occipitofrontal circumference (OFC), ventricular index, bicaudate diameter, lateral ventricular ratio, thalamo-occipital distance and maximum transverse lateral ventricular diameter were done to determine their predictive values for the development of hydrocephalus after myelomeningocele repair.
Result: Age range was 1 -120 days with a mean of 20 days and the average age at the time of myelomeningocele repair was 23 days. There were 16 (57.1%) females and 12(42.9%) males in the study. OFC range at presentation was 31- 42cm with a mean of 34.7cm ±2.6 SD. The odd ratio of OFC as a predictor of hydrocephalus after myelomeningocele repair was 1.74, p= 0.03. However, the state of the anterior fontanelle as well as the trans cranial ultrasound parameters were not found to be predictors of hydrocephalus post myelomeningocele repair.
Conclusion: The OFC of the patients before myelomeningocele repair was predictive of development of hydrocephalus post repair, but other clinical and ultrasonic parameters were not. Keywords; Hydrocephalus, myelomeningocele, trans-fontanelle ultrasound.
... However, it is not without complications. It is known to have a significant failure rate, particularly related to infective complications [10,19]. Furthermore, these children already have vulnerable CNS tissue and the development of additional infection further worsens cognitive function and other outcomes. ...
... Furthermore, these children already have vulnerable CNS tissue and the development of additional infection further worsens cognitive function and other outcomes. This has made the timing of VPS insertion an area of debate [19]. Whereas some authors suggest that VPS insertion at the same time or within a week of myelomeningocele closure increases the risk of shunt infection [10,15,20], others report no significant difference in the rate of shunt infection and dysfunction [21]. ...
Background
Myelomeningocoele (MMC) is common in the developing world. The purpose of this study was to investigate the clinical characteristics and management of myelomeningocoele and to identify factors contributing to outcomes.Methods
This was a retrospective, observational study of consecutive children diagnosed with MMC managed in the Paediatric Neurosurgery Unit at Inkosi Albert Luthuli Central Hospital. Multiple logistic regression analysis identified clinical characteristics, demographics and surgical variables that were associated with outcome.ResultsA total of 309 children were managed during this period (M:F 1.3:1). The most common sites were lumbar, lumbo-sacral and sacral. Mean age at surgical repair was 4.7 ± 15.6 months. Two hundred and eight children had ventriculomegaly, of whom 158 had symptomatic hydrocephalus, requiring CSF diversion. Fifty-eight (21%) patients developed wound sepsis, of whom 13 (22%) developed meningitis (p = 0.001). The time to wound sepsis was 9.5 ± 3.6 days. The commonest organism isolated was Staphylococcus aureus followed by MRSA. Thirty-two patients (23%) developed shunt malfunction and three (11%) developed ETV malfunction. Twenty children (9%) demised during the admission period. Death was associated with meningitis (p < 0.0001), and meningitis itself was associated with wound sepsis (p < 0.0001). Hospital stay was 20.4 ± 16 days. Wound sepsis (p = 0.002) and meningitis (p < 0.0001), respectively, were associated with prolonged hospital stay.Conclusion
There was a slight male preponderance and hydrocephalus occurred in two thirds of cases. Wound sepsis and meningitis were associated poor outcomes.
... There are several neurosurgical issues that need to be monitored, regardless of age, in the SB population. Approximately 80% of patients with SB require shunting for hydrocephalus [7]. Understanding the overt as well as subtle signs and symptoms of shunt malfunction is extremely important as they can be subtle [4]. ...
There are numerous multidisciplinary spina bifida (SB) clinics (typically including urology, orthopedics, neurosurgery, developmental pediatrics, physiatry, nursing, social work, and physical and occupational therapy) throughout the U.S. Many SB clinics have a nurse coordinator. The coordinator's role is truly multifaceted. It goes far beyond coordinating the clinic visit in which patients and families are seen for care. The frequency of clinical visits varies from program to program, from a few hours once a month to a full day every week. This role encompasses many aspects of care for this complex patient population, which will be described.
Was haben wir unter Mißbildungen zu verstehen ? Im Grunde genommen jede von der Norm abweichende Formbildung, mag sie nun die Folge von Erkrankungen, endogener oder sonst früh einwirkender Faktoren sein.
