Surgery For Myelomeningocele Research Articles

Infected dermoid tumor causing tethered cord after myelomeningocele repair

Intramedulary dermoid tumors are rare tumors mostly found associated with dermal sinus tracts. Spinal dermoid tumor can occur after myelomeningocele repair. Infected dermoid tumors are reported in patients with dermal sinus tracts as well, but have never been reported subsequent to myelomeningocele surgery. Here, we report a rare association of infected dermoid tumor with tethered cord without dermal sinus tract in a child who had been operated for myelomeningocele during infancy.

20: A cost effective analysis of prenatal surgery for myelomeningocele

20: A cost effective analysis of prenatal surgery for myelomeningocele

427: Risk factors for preterm delivery and uterine dehiscence following prenatal surgery for myelomeningocele

427: Risk factors for preterm delivery and uterine dehiscence following prenatal surgery for myelomeningocele

Preschool Neurobehavioral Outcome following Fetal Myelomeningocele Surgery

Objective: It was the aim of this study to assess the prevalence of preschool neurobehavioral problems in children following fetal myelomeningocele (fMMC) surgery. Methods: Prior to the Management of Myelomeningocele Study, 30 fMMC patients underwent a standardized neurodevelopmental examination at 5 years of age. The prevalence of behavioral problems was assessed by the Child Behavior Check List (CBCL), which includes a total problem score and 2 broad-band indices (internalizing and externalizing problems). fMMC children were classified as having normal, at-risk or clinically significant scores based on calculated T scores. Results: Twenty-two (73%) fMMC families completed the evaluation. Mean age at delivery following fMMC surgery was 35.5 ± 1.6 weeks. The prevalence of ‘at-risk’ or ‘clinically significant’ scores for internalizing, externalizing and total behavioral problems was similar to general population norms. No difference in overall internalizing (p = 0.19), externalizing (p = 0.54) and total behavioral (p = 0.18) scores was found between non-shunted and shunted fMMC children. However, shunted fMMC children were more likely to experience anxiety and depressive (p = 0.02), pervasive developmental (p = 0.03) and withdrawn (p = 0.06) behavior. Oppositional defiant, attention deficit and hyperactivity problems were not found. No correlation was found between overall neurodevelopmental outcome and abnormal CBCL scores. Conclusions: fMMC surgery and subsequent preterm delivery is not associated with increased behavioral problems, impaired social interactions and restricted behavior patterns. Shunted fMMC children were more likely to have scores in the ‘at-risk’ or ‘clinically significant’ range for multiple CBCL subindices. Early detection of behavioral problems following fMMC surgery provides an opportunity for targeted and timely intervention enabling affected fMMC children to be socially successful.

Anesthesia Literature Review

Anesthesia Literature Review

NewsCAP: Prenatal surgery is better than postnatal surgery for myelomeningocele

NewsCAP: Prenatal surgery is better than postnatal surgery for myelomeningocele

Fetal Surgery for Myelomeningocele?

The possibility of surgical repair of fetal anomalies in utero has long tantalized obstetricians and pediatric surgeons, especially since the development of high-resolution real-time ultrasonography. The presumption has been that earlier in utero repair would provide superior outcomes for the offspring than would postnatal surgery. However, postnatal treatment is much simpler and is not traumatic to the mother. Successful fetal surgery for any condition thus requires an accurate identification of which fetuses are at highest risk for a poor outcome if repair is delayed until after birth. Despite the promise of fetal surgical procedures, such repair of structural malformations has . . .

Fetal myelomeningocele surgery: preschool functional status using the Functional Independence Measure for children (WeeFIM)

To study preschool functional status in children following fetal myelomeningocele (fMMC) surgery. Prior to the NICHD-MOMS trial, 30 fMMC underwent standardized neurodevelopmental examination at 5 years of age. Functional status was determined with the Functional Independence Measure (WeeFIM), which assesses self-care, mobility, and cognitive independence. Evaluations were completed in 26 (87%). Mean cognitive (93.0 ± 21.9), self-care (66.5 ± 23.9), mobility (82.3 ± 19.5), and total (77.9 ± 20.3) functional quotient of fMMC children were significantly lower than age-matched population norms (P < 0.01). Complete caregiver independence was achieved by 22 (84%), 10 (38%), 16 (62%), and 15 (58%) fMMC children for cognition, self-care, mobility, and total functional outcome, respectively. Cognitive, mobility, and total independence were higher in non-shunted than shunted fMMC children (P = 0.02, P = 0.02, and P < 0.01, respectively) and in fMMC children with average neurodevelopmental scores (P < 0.001, P = 0.01, and P < 0.01, respectively). Self-care independence tended to be higher in the non-shunted group and in fMMC children with normal neurodevelopmental outcome (P = 0.07 and P = 0.09, respectively). The majority of fMMC children achieved cognitive and mobility independence, but continue to require significant assistance in self-care. Non-shunted and fMMC children with normal neurodevelopmental outcome were more likely to be independent in daily living activities. Better understanding of the extent of functional limitations following fMMC surgery will allow for more effective early interventions geared toward maximizing independence in everyday tasks in all environments.

404: Fetal myelomeningocele surgery: correlation of risk factors and preschool functional status using the Functional Independence Measure for Children (WeeFIM)

FETUS 83 51% 9 82% 0.06 Lethal congenital anomaly 45 28% 7 64% 0.01 Infection 3 2% 0 0% 0.7 Non-immune hydrops 8 5% 0 0% 1 Isoimmunization 1 1% 0 0% 0.7 Fetomaternal haemorrage Fetal growth restriction 25 15% 2 18% 0.8 Other 1 1% 0 0% 0.8 UMBILICAL CORD 15 9% 0 0% 0.3 PLACENTA 20 12% 0 0% 0.2 AMNIOTIC FLUID 12 7% 0 0% 0.4 UTERUS 1 1% 0 0% 0.8 MOTHER 1 1% 0 0% 0.8 INTRAPARTUM TRAUMA UNCLASSIFIED 26 16% 2 18% 0.8

405: Preschool neurobehavioral outcome following fetal myelomeningocele surgery

405: Preschool neurobehavioral outcome following fetal myelomeningocele surgery

The Relationship of Seizure Activity and Chronic Epilepsy in Early Infancy and Short-Term Neurodevelopmental Outcome Following Fetal Myelomeningocele Closure

We explored the relationship between seizure activity (SA) and/or chronic epilepsy (CE) and short-term neurodevelopmental outcomes following fetal myelomeningocele (fMMC) surgery. Retrospective databases and a parental questionnaire focusing on common complications of hindbrain herniation associated with MMC were used to determine the incidence of seizures following fMMC surgery. The Bayley Scales of Infant Development II was used to evaluate the neurocognitive outcomes. The available 3-year outcome data were used for analysis. 54 children underwent fMMC closure at our institution between 1998 and 2003. 48 (89%) families participated. The shunt rate was 50% (n=24). Seizures developed in 8/48 (17%) children, 2 (8%) non-shunted and 6 (25%) shunted (P=0.07). Of those six, 3 developed CE. Neurodevelopmental scores in the average range were found in both non-shunted and 3 shunted fMMC children. The remaining 3 shunted toddlers had CE and significant neurodevelopmental delays. Of those, 2 had severe intracranial hemorrhage and one developed frequent apneic spells in combination with epilepsy. The incidence of seizures in fMMC children was similar to previously reported data of postnatally repaired MMC patients. SA alone without CE was not associated with a worse neurocognitive outcome. The occurrence of severe acquired intracranial injury and CE, however, appeared to be correlated with adverse neurocognitive outcome following fMMC surgery.

Preschool neurodevelopmental outcome of children following fetal myelomeningocele closure

We sought to investigate the preschool neurodevelopmental outcomes of children following fetal myelomeningocele (fMMC) surgery. Prior to the Management of Myelomeningocele Study trial, 54 children underwent fMMC closure at our institution. Thirty (56%) returned at 5 years of age for standardized neurocognitive examination. Scores were grouped as high-average, average, mildly delayed, and severely delayed by SD intervals. Mean verbal intelligence quotient (VIQ), performance intelligence quotient (PIQ), and full intelligence quotient (FIQ) scores were within normal population range. High-average or average scores for VIQ, PIQ, FIQ, and processing speed were found in 93%, 90%, 90%, and 60%, respectively. Mean FIQ and processing speed of nonshunted children were significantly higher than for those who required shunt placement (P=.02 and P=.01, respectively). Mean VIQ and PIQ tended to be higher in nonshunted fMMC children (P=.05). The majority of fMMC children in this highly selective population had average preschool neurodevelopmental scores. fMMC children who did not require shunt placement were more likely to have better scores.

Characteristics and surgery of cervical myelomeningocele

Cervical myelomeningocele (CMMC) is a rare entity in neurosurgical practice, which presents different clinical characteristics compared with other more common lumbosacral variant. Since not much about this lesion has been reported in the literature, this study, herein, demonstrates by cases the clinical characteristics, methods, and techniques of surgical treatment of CMMC in children. A total of 10 children (six boys and four girls) with CMMC were recruited in this study. Their ages ranged from 9 days to 8 years with a median age of 3 months. All patients underwent neurological and radiological examinations. One was found to have had a mild unilateral arm weakness, and others were neurologically intact. Of these 10 patients, five had other associated neurological or orthopedic anomalies, including mild ventriculomegaly in two, cervical diastematomyelia in one, Chiari II malformation and hydrocephalus in one, and sacral spina bifida occulta in one. Surgical excision of the lesion with intradural exploration of the sac to release any potential adhesion bands was performed for all. No complications, such as cerebrospinal fluid leakage and infection, had been found after operation. During the follow-up of 1-7 years (mean of 3.9 years), all cases did not suffer from aggravation of nervous symptoms. None of the patients deteriorated postoperatively, and the one with left arm weakness improved following surgery. In the two children with mild ventriculomegaly, cerebral ventricle returned to be normal after surgery. The management strategies of CMMC are early surgical treatment with standard microneurosurgical techniques to prevent the development of neurological defects. It is safe and effective to adopt surgery excision of the lesions with intradural exploration of the sac to release any potential adhesion bands.

Inert patch with bioadhesive for gentle foetal surgery of myelomeningocele in a sheep model

Objective Current techniques used in foetal myelomeningocele repair can require considerable manipulation of fragile foetal tissues to obtain tension-free closure. The aim of this study was to assess the feasibility of a simple foetal coverage method without foetal tissue manipulation to provide closure of the neural tube defect in myelomeningocele. Study design This is an experimental study performed in 15 foetal sheep with lumbar myelomeningocele, surgically created on day 75 of gestation. Five foetuses remained untreated. Ten underwent coverage with inert sheeting (5 Silastic; 5 Silastic + Marlex) secured by surgical tissue adhesive without suturing on day 95; none of them underwent foetal muscle or skin manipulation. Clinical and subsequent histological examinations were performed at 48 h after birth. The Chi-square, Fisher exact, and Mann–Whitney U tests, when appropriate, were used for the comparisons. Results The mean operating time for foetal coverage was 7.1 (SD = 1.6) min. All untreated animals were unable to walk, had sphincter incontinence, showed an open defect, histological spinal cord damage, and a large Chiari malformation. All covered animals were able to walk, had sphincter continence, showed almost complete closure of the defect with regeneration of several soft tissue layers, and minimum Chiari malformation. Conclusion In a surgical myelomeningocele model in sheep, a simple, fast and gentle coverage method using a sealed patch avoids foetal tissue manipulation and enables adequate closure of the neural tube defect, providing regeneration of several tissue layers that protect the spinal cord, and significantly reducing Chiari II malformation.

Reversal of Hindbrain Herniation after Maternal-fetal Surgery for Myelomeningocele Subsequently Impacts on Brain Stem Function

The aim of our study was to delineate whether the reversal of hindbrain herniation (HH) following fetal myelomeningocele (fMMC) closure subsequently reduces the incidence and severity of HH-associated brainstem dysfunction (BSD). Prior to the NIH-sponsored Management of Myelomeningocele Study (MOMS) trial, 54 children underwent fMMC closure at our institution. Forty-eight (89%) families participated in a structured survey focusing on HH-associated BSD (e.g., apnea, neurogenic dysphagia [ND], gastro-esophageal reflux disease [GERD], neuro-ophthalmologic disturbances [NOD]). Median age at follow-up was 72 months (range: 46-98). Fifty-percent required shunting. HH-related symptoms were completely absent in 15 (63%) non-shunted and 10 (42%) shunted children (P=0.15). No HH-related death occurred and none developed severe persistent cyanotic apnea. ND was reported in 2 (8%) non-shunted and 9 (38%) shunted infants (P=0.03). Mild GERD (medically managed) developed in 2 (8%) without and 6 (25%) with shunt placement (P=0.24). NOD was found in 6 (25%) and 13 (54%) of non-shunted and shunted children, respectively (P=0.07). The majority of fMMC children developed no or only mild BSD at follow-up. Our data support the hypothesis that neurodevelopmental deficits associated with MMC are at least partially acquired and that reversal of HH following fMMC surgery may help to reduce the incidence and severity of BSD.

Fetal spina bifida in a mouse model: loss of neural function in utero

The devastating neurological deficit associated with myelomeningocele has previously been assumed to be a direct and inevitable consequence of the primary malformation-failure of neural tube closure. An alternative view is that secondary damage to the pathologically exposed spinal cord tissue in utero is responsible for the neurological deficiency. If the latter mechanism were shown to be correct, it would provide an objective rationale for the performance of in utero surgery for myelomeningocele, because coverage of the exposed spinal cord could be expected to alleviate or perhaps prevent neurodegeneration. To examine this question, the authors studied the development of neuronal connections and neurological function of mice during fetal and neonatal stages in a genetic model of exposed lumbosacral spina bifida. The persistently exposed spinal cord of mouse fetuses carrying both curly tail and loop-tail mutations exhibited essentially normal anatomical and functional hallmarks of development during early gestation (embryonic Days 13.5-16.5), including sensory and motor projections to and from the cord. A significant proportion of fetuses with spina bifida at early gestation exhibited sensorimotor function identical to that seen in age-matched healthy controls. However, at later gestational stages, increasing neurodegeneration within the spina bifida lesion was detected, which was paralleled by a progressive loss of neurological function. These findings provide support for the hypothesis that neurological deficit in human myelomeningocele arises following secondary neural tissue destruction and loss of function during pregnancy.

Bladder and External Urethral Sphincter Function After Prenatal Closure of Myelomeningocele

We compared urodynamic findings in patients who underwent prenatal closure of myelomeningocele with those of patients who underwent postnatal closure, and equivalent lower urinary tract evaluations. Urodynamic studies of 5 patients (2 boys, 3 girls) who underwent prenatal closure of myelomeningocele were compared to those of 88 patients with similar level lesions who underwent repair postnatally between 1979 and 2002. All 5 patients in the prenatally treated cohort had lower lumbosacral lesions on neurological examination. These patients displayed no evidence of electromyographic activity, indicating complete denervation of the external sphincter. In comparison 34 of the 88 patients in the postnatal cohort (39%) lacked sphincter activity at newborn examination, with similar findings noted at 1-year evaluation. In terms of bladder function all 5 patients in the prenatal cohort exhibited detrusor overactivity, compared to 33 of the 88 patients (38%) in the postnatal cohort at the newborn examination, with similar findings at 1-year evaluation. Fetal closure of myelomeningocele is associated with a higher incidence of complete denervation of the external urethral sphincter and detrusor overactivity compared to postnatal closure. Patients who undergo this novel procedure should undergo urodynamic studies in the immediate newborn period and should be under close postnatal surveillance to document possible tethering of the spinal cord, urinary incontinence and increased detrusor pressures.

Li JK: Neural Tube Defects – From Origin to Treatment Edited by: Diego F Wyszynski. Oxford University Press, New York; 2006.

Li JK: Neural Tube Defects – From Origin to Treatment Edited by: Diego F Wyszynski. Oxford University Press, New York; 2006.

Impact of maternal-fetal surgery for myelomeningocele on the progression of ventriculomegaly in utero

Intrauterine myelomeningocele (MMC) repair decreases hindbrain herniation and the need for postnatal ventriculoperitoneal shunting. We examined the impact of intrauterine repair on the progression of ventriculomegaly in utero. Fetuses with MMC were identified through computerized databases from June 1988 to April 2003. A retrospective cohort design was used to evaluate the impact of intrauterine repair on ventricular progression with a multivariate linear regression model that included baseline ventricle measurement, gestational age, level of lesion, and gender. Fourteen fetuses with intrauterine repair and 39 fetuses with postnatal repair were identified. The natural history of progression of ventricular diameter increased in a linear fashion throughout gestation (0.57 mm/week). After adjusting for confounding variables, no transient or sustained difference was observed in the rate progression of ventriculomegaly between intrauterine and postnatal repair (0.27 +/- 0.35 mm/week; P=.45). Intrauterine MMC repair does not impact the progression of ventriculomegaly.

Impact of maternal fetal surgery for myelomeningocele on the progression of ventriculomegaly in utero

Impact of maternal fetal surgery for myelomeningocele on the progression of ventriculomegaly in utero