Intelligence Quotient in Patients with Myelomeningocele: A Review

Alimi, Yusuf; Iwanaga, Joe; Oskouian, Rod J.; Loukas, Marios; Tubbs, R. Shane

doi:10.7759/cureus.3137

Cited by 10 publications

(12 citation statements)

References 35 publications

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“…This is similar to what has been published by various groups. 3,18 Our relatively high rate of shunt infection might also play a role in this. Interestingly, a more recent cohort series consisting of 108 cases from the MOMS trial did not confirm this association.…”

Section: Cognitionmentioning

confidence: 94%

Contemporary management and outcome of myelomeningocele: the Rotterdam experience

Spoor¹,

Gadjradj

Eggink³

et al. 2019

Neurosurgical Focus

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OBJECTIVEMyelomeningocele (MMC) is the most common form of spina bifida, with a lifelong impact on the quality of life for infants born with this condition. In recent decades, fetal surgery has evolved from an experimental therapy to standard of care for many centers in the world. In this study, the authors aimed to provide an overview of the current management and outcomes for infants with MMC managed at their institution. This then provides a center-specific historical cohort for comparison with future antenatal-treated MMC cases.METHODSThis is a retrospective, single-institution cohort study including all consecutive MMC cases between January 1, 2000, and June 1, 2018, at Erasmus MC. Outcome data included closure of the defect (location, timing, and surgical parameters), hydrocephalus management, Chiari malformation type II (CMTII) management, incidence of spinal cord tethering and outcome, motor outcomes, and continence.RESULTSA total of 93 patients were included with predominantly lumbosacral lesions. Two patients died during follow-up. Hydrocephalus was present in 84%, with a 71% ventriculoperitoneal shunt reoperation rate. Surgery was performed in 12% for a tethered spinal cord at a mean age of 8 years. Decompression surgery was performed in 3 patients for CMTII. Special education in 63% was significantly associated with hydrocephalus (p < 0.015). Nineteen percent of patients were able to walk independently, and 47% were nonambulators. Social continence for urine was obtained in 75% of patients, 4% had fecal incontinence.CONCLUSIONSThis study provides an overview of current MMC outcomes at the authors’ center and will serve as a historical cohort for comparison with future fetal surgery cases operated on at the center in the coming years. Apart from a relatively low surgical untethering rate, the authors’ outcome data are comparable to those in the literature. Hydrocephalus is highly prevalent in postnatally treated MMC patients; in this study as in much of the literature, hydrocephalus is correlated with a low cognitive function. Fetal surgery for MMC halves the need for shunt treatment in a select group of MMC pregnancies, constituting a major indication for us to undergo the transition to a fetal surgery center. The fetal benefits of open antenatal surgery for MMC are well established, yet long-term data on especially tethered spinal cord are eagerly awaited.

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Section: Cognitionmentioning

confidence: 94%

Contemporary management and outcome of myelomeningocele: the Rotterdam experience

Spoor¹,

Gadjradj

Eggink³

et al. 2019

Neurosurgical Focus

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“…To our knowledge there is no data on the brain pathology apart from Chiari II and hydrocephalus in such operated patients. Since hydrocephalus and shunting complications are associated with and potentially cause low IQ values 56 , 57 further data on brain morphology and the cognitive profile of individuals with SBA following in utero closure are urgently needed. Moreover, it needs to be considered that a hydrocephalus can occur early, and that ependymal denudation can indicate hydrocephalus onset and generate cerebral pathology before an intrauterine repair in the GW19-26.…”

Section: Discussionmentioning

confidence: 99%

Cerebral Abnormalities in Spina Bifida: A Neuropathological Study

et al. 2021

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Introduction Spina bifida (SB) is the most common neural tube defect in humans. Here, we analyzed systematically the neuropathological findings of the brain in SB cases. Methods 79 cases with SB aperta (SBA) and 6 cases with SB occulta (SBO) autopsied at the Charité Neuropathology from 1974 to 2000 were re-evaluated retrospectively. For this, case files and spinal cord as well as brain sections were studied. Results While no brain malformations were detected in SBO cases, 95% of SBA cases had brain malformations. Main brain anomalies identified were hydrocephalus (71%), Chiari II malformation (36%), heterotopia (34%), other cerebellar anomalies (36%), gyrification defects (33%), and ependymal denudation (29%). Hydrocephalus was observed as early as gestational week 17 and was highly associated to Chiari II and ependymal denudation. In 55% SBA was accompanied by further anomalies not primarily affecting the CNS. Conclusion We confirm using neuropathologic methods brain malformations in most SBA but none in SBO cases. In addition to our previous radiologic study, we now demonstrate the high prevalence of cerebellar malformations and cerebral heterotopias in SBA. The early detection of hydrocephalus and Chiari II malformation in fetuses raises the question whether these arise parallel rather than in strict temporal sequence.

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“…The IQ in an average myelomeningocele patient is in the normal range; however, those who suffer from shunt- and Chiari-related complications often fall to the low-normal or below average ranges. This is well reviewed recently [34]. Seizures, scoliosis, osteoarthritis, spinal tethering, bladder infections, upper renal tract damage, severe constipation, and latex allergies are but a few of the complications expected for these patients over their lifetimes [1].…”

Section: Discussionmentioning

confidence: 99%

Dual Myelomeningoceles in Twins: Case Report, Review, and Insights for Etiology

et al. 2020

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Introduction: Despite folate supplementation, neural tube defects (NTDs) still occur in 0.5–1.0/1,000 pregnancies, with 30–50% not preventable with folate. Twinning has increased due to artificial fertilization and in itself predisposes to NTDs at a rate of 1.6/1,000. The contributions of genetic and environmental factors to myelomeningocele development remain poorly understood. Expression patterns of congenital pathologies in twins can sometimes provide etiological insight. Concordance of NTDs in twins is 0.03/1,000, with dual myelomeningocele reported in only 23 pairs, only one of which survived. We present the 24th pair, the 1st to maintain lower extremity motor function. We review all prior cases and discuss implications of twin concordance on the interplay of genetic and environmental influences. Case Report and Review: A new case of female monozygotic twins born to a well-nourished 24-year-old female is reported with details of perioperative care. Prenatal ultrasound showed L3-S4 and L5-S4 myelomeningoceles, Chiari II malformations, and ventriculomegaly. Copy number microarray was unrevealing. Each underwent uncomplicated repair on day of life 1, and ventriculoperitoneal shunt placement on days of life 10 and 16. Both had movement in the legs upon 6-week follow-up. All prior reported cases of concordant twin myelomeningoceles were abstracted and analyzed, revealing persistence of occurrence despite folate supplementation and a majority occurring in dizygotic pairs. The literature is also reviewed to summarize current knowledge of myelomeningocele pathophysiology as it relates to genetic and environmental influences. Discussion: Meticulous surgical and perioperative care allowed for early positive outcomes in each twin. However, etiopathogenesis remains elusive. In general, only of a minority of cases have underlying genetic lesions or clear environmental triggers. Concordance in monozygotic twins argues for a strong genetic influence; yet, literature review reveals a higher rate of concordant dizygotic twins. This, along with the observation of differing resultant phenotypes in monozygotic twins as seen in this case, prompts further investigation into nonfolate environmental influences. While efforts in genetic investigation should continue, the role of teratogens and exposures should not be minimized in research efforts, public health, and family counseling. Clinical genetic testing remains of limited utility in the majority of patients until more is known.

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Intelligence Quotient in Patients with Myelomeningocele: A Review

Cited by 10 publications

References 35 publications

Contemporary management and outcome of myelomeningocele: the Rotterdam experience

Contemporary management and outcome of myelomeningocele: the Rotterdam experience

Cerebral Abnormalities in Spina Bifida: A Neuropathological Study

Dual Myelomeningoceles in Twins: Case Report, Review, and Insights for Etiology

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