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At least 19 recordsLinked to original sources
PubMed1999

Are screening echocardiograms warranted for neonates with meningomyelocele?

OBJECTIVE: To evaluate the incidence and types of congenital heart defects associated with meningomyelocele. DESIGN: All neonates who underwent meningomyelocele repair and had a perioperative echocardiogram from July 1990 to October 1998 were studied. Medical records were reviewed for age, weight, clinical cardiac examination results, meningomyelocele location, and associated noncardiac defects. Heart defects were identified from reviewing echocardiographic reports and videotapes. RESULTS: At meningomyelocele surgery, the 105 patients (53 female; 52 male) ranged in age from 1 to 20 days and in weight from 0.6 to 4.1 kg. Congenital heart disease was detected in 39 patients (37%). A secundum atrial septal defect was the most common defect (24%). A ventricular septal defect was found in 10 patients, 2 patients had anomalous pulmonary venous return, and 1 each had tetralogy of Fallot, bicuspid aortic valve, coarctation, and hypoplastic left heart syndrome. A patent ductus arteriosus and patent foramen ovale were not considered abnormal in these neonates. The cardiac examination was abnormal in only 5 of the 39 patients with heart defects (sensitivity = 13%). The presence of associated noncardiac defects (in addition to meningomyelocele) and location of the meningomyelocele (cervicothoracic vs lumbar) did not affect the incidence of heart disease. Of the patients with heart defects, girls were more frequently affected (25 of 39 vs 14 of 39, P<.05). CONCLUSIONS: Congenital heart defects are common in neonates, especially girls, with meningomyelocele and are unrelated to meningomyelocele location or associated noncardiac defects. Because the clinical examination is insensitive for detecting heart defects in this group, screening echocardiograms are warranted. This information has important implications for ventriculoatrial shunting, urinary tract instrumentation (antibiotic prophylaxis), and neurosurgical procedures (venous air embolism).

Abnormalities, Multiple↗
PubMed2001

The curly tail mouse model of human neural tube defects demonstrates normal spinal cord differentiation at the level of the meningomyelocele: implications for fetal surgery.

The paralysis associated with lumbosacral meningomyelocele has been attributed both to myelodysplasia and to degeneration of the exposed neural tissue. Surgically created dysraphism shows that exposure of an intact spinal cord in a genetically normal animal results in degeneration of the normal nervous tissue and subsequent paralysis. Our objective was to study neuronal differentiation in the curly tail mouse mutant model, which develops lumbosacral meningomyelocele naturally and is a phenocopy of nonsyndromic human neural tube defects. Prenatal repair of meningomyelocele assumes that the normal neuronal differentiation program occurs despite failure of neurulation. Here we demonstrate that this most suitable animal model has normal differentiation of neuronal structures at the level of the meningomyelocele. TuJ1, an antibody to neuronal specific class III beta-tubulin, an early marker of neuronal differentiation, was used to stain paraffin-embedded sections of curly tail mouse embryo meningomyelocele. Embryos were examined at embryonic day 13.5 (E13.5). The inbred mouse strain, C57BL6/J, which is genetically similar to the curly tail mouse, was used as a control in these studies. We show that early neuronal differentiation appears intact within the meningomyelocele. TuJ stains structures within the open neural tube. Motor neurons are present in the ventral horn and ventral roots. Dorsal root ganglia are present and of similar size to controls. The staining pattern is similar to that seen in the C57BL/6J control mouse, although dorsal structures are laterally displaced in the curly tail meningomyelocele. Based on this model, fetal surgery to repair human meningomyelocele may preserve neurological function in those cases where there is not an inherent genetic defect of the neural tissue.

Animals↗
PubMed1998

Outcome of patients with meningomyelocele: the Ege University experience.

The medical records of 190 patients with meningomyelocele operated on between 1979 and 1993 were reviewed. In 65 patients, psychometric tests were performed. The Denver Developmental Screening Test and the Wechsler Intelligence Scale for Children, Revised (WISC-R) were used in children under and over 6 years old, respectively. There were 82 boys (43.2%) and 108 girls (56.8%). The lumbar region was the site of the meningomyelocele in 113 patients (59.5%). Patients with cervical and sacral meningomyelocele had a higher rate of almost normal motor function than those with meningomyelocele at other levels (P=0.000). Only 36 (21.7%) of 166 patients followed up by us did not have hydrocephalus. We also noted that the higher the location of the meningomyelocele, the greater the control of both sphincters (P=0.014). Fifty-four percent of the hydrocephalic patients had a normal development or IQ. Psychometric tests were normal in 76% of those without hydrocephalus. Twenty-four patients were lost to follow-up. The follow-up of the remaining 166 patients ranged from 1 month to 180 months (mean 60.2 months). Fifty-three patients (32%) died, central nervous system infection being the most common cause of death (44%). The management of children with meningomyelocele needs a team approach.

Cerebrospinal Fluid Shunts↗
PubMed1998

The optimal route of delivery for fetal meningomyelocele.

OBJECTIVE: It has been proposed that cesarean section improves the long-term neurologic outcome of children with meningomyelocele. On the basis of this belief, a trial of labor is not offered in many centers. We hypothesized that there is no difference in immediate or long-term outcome by route of delivery for the fetus with meningomyelocele delivered in a tertiary care center. STUDY DESIGN: All fetuses (n = 60) with meningomyelocele delivered at the University of Iowa Hospitals and Clinics between 1971 and 1995 were analyzed. Thirty-six cases were available for long-term follow-up. Motor, sensory, and anatomic levels were converted to a numeric scale. Variables were compared by one-way analysis of variance, chi2 analysis, and Fisher's exact test with significance at P < .05. RESULTS: There were no significant differences by route of delivery for gestational age of delivery, birth weight, meningomyelocele size, or neonatal mortality (vaginal: 1/22 = 4.5%, cesarean section: 2/17 = 11.8%, P = .82). An antenatal diagnosis was made with similar frequency in the two groups (vaginal: 15/21 = 71.4%, cesarean section: 13/15 = 86.7%). In addition, the length of long-term follow-up was similar (vaginal: 54.7 +/- 11.1 months, cesarean section: 33.7 +/- 8.6 months). There was no difference in long-term neurologic outcome as determined by the change in motor level, the change in sensory level, or when comparing the final motor level with the anatomic level. CONCLUSIONS: This study was unable to detect differences between either immediate or long-term outcome for the infant with isolated meningomyelocele when stratified by route of delivery. A multicenter randomized trial should be required before the acceptance of cesarean section as the optimal route of delivery for the fetus with meningomyelocele.

Adult↗
PubMed1998

Anatomy of the spinal cord in patients with meningomyelocele with and without hypoplasia or hydromyelia.

We compared the cross-sectional areas of spinal cords of patients with normal cords to the area of patients with meningomyelocele. The control group consisted of examinations of 27 patients with normal spinal cords providing 1547 axial images at 20 levels, C2-L2. The meningomyelocele group consisted of 67 MRI examinations of 41 patients, providing 4,095 axial images at 23 levels C2 to S1. Thirty-four examinations were of 21 patients with minimal hydromyelia, 7 examinations were of 3 patients with operable hydromyelia, and 26 examinations were of 17 patients without hydromyelia. In an additional analysis, we selected those meningomyelocele patients with cord tethering but without hydromyelia or hypoplasia (53 examinations of 30 patients) and compared them to symptomatic hypoplasia cases (9 examinations of 6 patients). The symptomatic hypoplasia cases were chosen because of progressive loss of muscle strength and worsening spasticity not relieved by surgical adhesiolysis. The test, retest error was 5.6% with differences between the means of repeated readings not being significant. All tests for significance were paired T test. The areas of spine levels C7-L2 for the controls were significantly larger than for the meningomyelocele patients (p = 0.000007). Including all levels C2-S1, the minimal hydromyelia cases were not significantly different from those without hydromyelia (p = 0.5). The areas C2-S1 of operable hydromyelia cases were larger than both non-shunted minimal hydromyelia (p = 0.00009) and of meningomyelocele patients without hydromyelia (p = 0.00003). The areas C7-L2, of hypoplasia cases were significantly smaller compared to the "normal" meningomyelocele cases (p = 0.0004). These data suggest that hydromyelia stimulates overgrowth of the cord, as does hydrocephalus of the brain, and that adhesiolysis procedures are of no value with hypoplasia of the spinal cord.

Adolescent↗
PubMed1991

Cesarean section before the onset of labor and subsequent motor function in infants with meningomyelocele diagnosed antenatally.

Background. Meningomyelocele can now be detected before birth. Few data are available on its natural history, however, and optimal management at the time of delivery is controversial, although it has been suggested that labor and vaginal delivery may cause pressure on exposed nerve roots, resulting in additional loss of neural function. Methods. To assess the effect of labor and the type of delivery on the level of motor function in fetuses with uncomplicated meningomyelocele, we identified 200 cases of this disorder, accounting for 95 percent of the cases that occurred in the state of Washington during our 10-year study period. We compared the outcomes of 47 infants delivered by cesarean section before labor began, 35 delivered by cesarean section after a period of labor, and 78 who were delivered vaginally (another 40 were ineligible for the study). In cases of meningomyelocele detected prenatally, cesarean section was performed before the onset of labor if isolated meningomyelocele without severe hydrocephalus was present. The infants delivered in this manner were compared with those who were delivered either vaginally or by cesarean section after labor began. Results. At two years of age, the infants who had been exposed to labor were 2.2 times more likely to have severe paralysis than those delivered by cesarean section without labor (95 percent confidence interval, 1.7 to 2.8). Infants delivered by cesarean section before the beginning of labor had a mean (+/- SD) level of paralysis 3.3 +/- 3.0 segments below the anatomical level of the spinal lesion at two years of age, as compared with 1.1 +/- 2.3 for infants delivered vaginally and 0.9 +/- 4.1 for infants delivered by cesarean section after the beginning of labor (P less than 0.001 for both comparisons). Exposure to labor did not affect the frequency of neonatal complications or later intellectual performance. Conclusions. For the fetus with uncomplicated meningomyelocele, delivery by cesarean section before the onset of labor may result in better subsequent motor function than vaginal delivery or delivery by cesarean section after a period of labor.

Cesarean Section↗
PubMed2006

Osteoporosis in adults with meningomyelocele: an unrecognized problem at rehabilitation clinics.

OBJECTIVES: To assess the prevalence of osteoporosis and osteopenia in adults with meningomyelocele and to explore whether neurologic level, ambulatory status, and other medical problems are associated with bone mineral density (BMD). DESIGN: A cross-sectional study, including a self-administered questionnaire and clinical assessment. SETTING: Outpatient referral clinic in Sweden. PARTICIPANTS: Twenty-one adults (mean age, 30 y) with meningomyelocele admitted to the Young Adult Teams in Göteborg and Boras, Sweden. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: BMD in the lumbar spine and hip and forearm measured with dual x-ray absorptiometry. RESULTS: Seven (33%) subjects had osteoporosis in at least 1 of the measured sites. Three patients had osteopenia and 2 had osteoporosis in the lumbar spine. Among the 15 subjects whose BMD of the hip region could be reliably measured, 7 (47%) had osteoporosis in the femoral neck or trochanteric region of the hip. Subjects with other medical problems commonly occurring in meningomyelocele had lower BMD in the femoral neck and trochanteric region of the hip than subjects without such factors. Ambulation alone showed only a tendency to be associated with BMD of the femoral neck, whereas the effect of other medical risk factors on BMD of the femoral neck was stronger among the nonambulators than the ambulators. CONCLUSIONS: Our results show that osteoporosis is a medical problem to be considered when treating and rehabilitating patients with meningomyelocele.

Adult↗
PubMed2004

Using the sac membrane to close the flap donor site in large meningomyeloceles.

If a large transposition flap with or without muscle is used for closure of a large meningomyelocele defect, then, a part of the donor site of the flap can be closed by split thickness skin graft, which produces an additional donor wound for the patient. We used the sac membrane instead of split thickness skin graft for closure of donor sites of fasciocutaneous flaps and latissimus dorsi musculocutaneous flaps employed to cover large meningomyelocele defects. This technique was used in three thoracolumbar and in two lumbosacral meningomyelocele patients. The sac membrane was prepared like a full thickness skin graft. Follow-up in five patients has ranged from 1 to 18 months, with a mean of 10.6 months. The donor sites that were closed by the sac membrane exhibited complete healing in all patients. We conclude that the sac membrane supplies a reserve of epithelialised tissue that can be used for repair of the meningomyelocele defects.

Epithelial Cells↗
PubMed1995

Spinal anesthesia for repair of meningomyelocele in neonates.

The use of spinal anesthesia for meningomyelocele repair in neonates has received minimal attention. Spinal anesthesia may lessen the stress response to surgery and decrease postoperative respiratory complications. We therefore examined the efficacy of spinal anesthesia in 14 neonates requiring repair of lumbar or sacral meningomyelocele. All neonates were positioned prone with a small chest roll. Hyperbaric 0.5% tetracaine with epinephrine was injected into the caudal end of the meningomyelocele sac. If necessary, supplemental tetracaine was administered directly into the intrathecal space by the surgeon during the operation. Blood pressure, heart rate, and oxyhemoglobin saturation were measured throughout surgery. Neonates were monitored with transthoracic impedance apnea monitors, electrocardiogram (ECG), and pulse oximetry for 36 h after surgery. Spinal anesthesia was successful in all cases. Seven patients received one supplemental tetracaine injection; one patient received two supplemental injections. Arterial blood pressure decreased an average of 5 mm Hg with the largest decrease being 10 mm Hg. Two postoperative respiratory events occurred in the first 8 h after surgery. Both neonates had received intraoperative midazolam for sedation. Neurologic function was assessed pre- and postoperatively. Twelve patients had no change in neurologic function after surgery, while two infants demonstrated improved function. We conclude that spinal anesthesia can be safely used for meningomyelocele repair.

Anesthesia, Spinal↗
PubMed2006

Bilobed fasciocutaneous flap closure of large meningomyeloceles.

BACKGROUND: Closure of large meningomyelocele defects presents a challenging problem. In this paper, the procedure and outcome of bilobed fasciocutaneous flap closure of large meningomyeloceles have been discussed. MATERIALS AND METHODS: Bilobed fasciocutaneous flap was used in 20 patients with large meningomyelocele defects, the largest of which was 77 cm2. A fasciocutaneous dissection was performed and the defect area was covered by tension-free closure. RESULTS: In the follow-up period of 6 weeks, partial flap loss in a patient and cerebrospinal fluid leak inferior to the flap in another 2 were observed. These patients recovered by dressing without flap loss. CONCLUSIONS: Utilization of bilobed flaps for closure of large meningomyelocele defects seems to be an effective and reliable procedure, with advantages of decreased operative time, minimal bleeding, the suture lines for dura and flap not superimposed, and low morbidity.

Fascia↗
PubMed2006

Use of allogeneic skin graft for the closure of large meningomyeloceles: technical case report.

OBJECTIVE AND IMPORTANCE: To describe an alternative approach to the operative management of large meningomyeloceles, in which primary closure is not possible. CLINICAL PRESENTATION: Two full-term infants presented with very large meningomyeloceles for closure after an uncomplicated delivery. TECHNIQUE: An allogeneic skin graft was used in both cases to approximate the skin defect that remained after removal of the large meningomyelocele sacs. The surgical site was treated with dressing changes only during the postoperative period. CONCLUSION: The independent use of allogeneic skin grafts can lead to a successful cosmetic result for the treatment of large meningomyeloceles without the use of complicated skin incisions or flap advancements.

Humans↗
PubMedDate not supplied

Prominent choroid plexus in meningomyelocele: sonographic findings.

Of 18 infants with meningomyelocele who were examined by cranial sonography, 17 were found to have a prominent choroid plexus, especially in the region of the glomus. In almost half of the cases this prominence assumed the configuration of a stalk arising from the glomus and terminating in a clublike ending. This prominence can be easily confused with intraventricular hemorrhage. The literature dealing with meningomyelocele and the Arnold-Chiari malformation makes no mention of this finding, but literature related to choroid plexus pathology describes similar findings in hydrocephalic infants, some of whom had documented meningomyelocele. This unusual choroidal configuration seen on sonography might be part of the spectrum of brain malformations seen in infants with meningomyelocele.

Choroid Plexus↗
PubMed1987

Outcome of fetal meningomyelocele after vaginal delivery.

Eight neonates with meningomyelocele were delivered vaginally. Diagnosis of meningomyelocele was made by ultrasonography during the latter half of pregnancy in three patients. The remaining five did not have ultrasound examination during the antenatal period. None of the meningomyelocele sacs (less than or equal to 4 cm in diameter) was ruptured at the time of delivery. Cesarean section may not be necessary for all fetuses with meningomyelocele.

Adult↗
PubMed1996

Cervical meningomyelocele associated with spina bifida in a hydrocephalic miniature colt.

Cervical meningomyelocele, spina bifida, and hydrocephalus were diagnosed in a newborn miniature colt that was unable to stand and had a cystic mass in the caudal portion of the dorsal cervical region. Results of physical examination and ultrasonographic imaging of the brain supported the diagnosis of hydrocephalus. Results of radiographic evaluation of the vertebral column were consistent with spina bifida at C5-C6. Fluid aspirated from the cervical mass resembled CSF. Radiography of the cervical region after injection of iohexol into the mass revealed herniation of the spinal cord through a large bony defect, supporting the diagnosis of spina bifida and meningomyelocele. Meningomyelocele, spina bifida, and hydrocephalus are interrelated congenital anomalies in other species. Meningomyelocele should be suspected in foals that have neurologic abnormalities, especially if they have other congenital anomalies and a fluid-filled mass along the dorsal midline.

Animals↗
PubMed1998

[Brain changes in dysraphic syndromes occurring in cases of meningomyelocele].

Neural tube defects are the most common anomalies of the CNS in the group of dysraphic syndromes. They occur on both encephalic and spinal levels. According to the title of this article we discuss some characteristic examples of intracranial, encephalic changes occurring in cases of meningomyeloceles. The special position has to be attributed to Arnold-Chiari or Chiari malformation, being currently under a nosological discussion. It is often restricted to its most typical form-Chiari II syndrome. This is characterized by the neural tube defect and the posterior fossa and brain-stem-cerebellum malformation with a displacement into the foramen magnum. The compression of the fourth ventricle and even secondary necrotic changes are also seen. Clinical symptoms depend on the topography of pathological changes. Chiari II malformation coincides in the majority of cases with meningomyeloceles and sometime with other spinal cord malformations. Meningomyeloceles coincide often with anomalies within cerebral hemispheres. Among them hydrocephalus deserves particular attention, occurring in the majority of Chiari II cases and not being rare in cases of myeloceles. The anomalies and/or secondary lesions of encephalic structures, particularly of the cortical mantle are observed, depending upon the severity of hydrocephalus and the onset of its development. Meningomyeloceles can also be accompanied by cerebral hemispheres anomalies arising in the other pathomechanism and developmental period than dysraphic syndromes. The midline malformations and anomalies of commissural system are not rare among them. In conclusion we emphasize that each case of CNS malformations is unique. The observed anomalies have to be assessed whether they form a part of a leading syndrome or occur together with dysraphic changes.

Abnormalities, Multiple↗
PubMed2000

The effect of C677T mutation of methylene tetrahydrofolate reductase gene and plasma folate level on hyperhomocysteinemia in patients with meningomyelocele.

To evaluate the relationship between genotypes of methylene tetrahydrofolate reductase (MTHFR), and plasma folate and homocysteine (Hcy) levels in meningomyelocele, 21 Korean patients, 47 of their family members, and 43 healthy controls were recruited. The presence of C677T mutation in the MTHFR gene and plasma concentrations of folate/Hcy were investigated. The genotype frequency of C677T mutation was not higher in study groups (patients and family members). The plasma folate concentration showed no difference either between the study and the control groups or among MTHFR-genotypic groups. The plasma Hcy concentration in homozygotes in the study group was higher than that in the control group, and higher than that in heterozygotes when plasma folate levels were low (P=0.006). Although neither MTHFR genotype nor plasma folate/Hcy level plays a definite part on its own, they seem to have an additive effect on the occurrence of meningomyelocele. Our results support folate supplementation for the prevention of hyperhomocysteinemia and meningomyelocele.

Alleles↗
PubMed1995

Epilepsy in children with meningomyelocele.

The medical records of 89 children followed at a multidisciplinary Meningomyelocele Clinic at the Children's Clinics for Rehabilitative Services were reviewed. Almost all children in southern Arizona with meningomyelocele are followed at this clinic. Eight children (foreign nationals) were excluded because they were not eligible for neurosurgery/neurology services at the clinic. The remaining 81 children have been followed at the clinic from 0.25 to 21 years. Seventeen children (21%; age: 1.3-17 years, mean: 9.1 +/- 4.4 years; follow-up: 1.3-16 years) manifested seizures at some time during their course. All children with seizures had shunted hydrocephalus. Neonatal seizures occurred in 2 children currently not receiving medication. An additional 3 children had an acute symptomatic seizure associated with an intraventricular hemorrhage during ventriculoperitoneal shunt revision, 2 of whom later developed epilepsy. Fourteen children (17.3%) had epilepsy; 12 were taking antiepileptic drugs. Seizures were controlled on medication in 5 children. EEG abnormalities were present in 12 children (focal slowing 4, focal spikes 8, diffuse slowing 3, generalized or bilaterally synchronous spike-wave 4). Most of these children (12/14) had evidence of additional central nervous system (CNS) pathology (i.e., areas of encephalomalacia or past stroke 7, cerebral malformations 2, CNS calcifications 1, and frequent apneic spells/cardiac arrest 2). We conclude that epilepsy occurs in approximately 17% of children with meningomyelocele, and most have other CNS pathology to account for their seizures.

Adolescent↗
PubMed1990

Seizures in relation to shunt dysfunction in children with meningomyelocele.

To determine whether seizures are a reliable sign of increased intraventricular pressure in children with shunt-dependent hydrocephalus and meningomyelocele, we performed a retrospective chart review of 346 patients with meningomyelocele and shunt-dependent hydrocephalus. Fifty-one patients had seizures. Seizure episodes were investigated to determine whether they were temporally associated with shunt dysfunction. Episodes of actual or presumed shunt dysfunction were analyzed as to presenting symptoms, ventriculogram, computed tomography scan, and shunt film results. These 51 patients had 129 admissions for possible shunt dysfunction. One hundred one admissions were due to increased intraventricular pressure; nine (9%) of these, had a seizure as one of the presenting symptoms. All of the nine episodes had other common presenting symptoms of shunt dysfunction, such as headache, vomiting, lethargy, or respiratory compromise. We conclude that seizures alone are an inadequate predictor of shunt dysfunction in children with meningomyelocele, but can be seen as one of the presenting symptoms.

Cerebrospinal Fluid Shunts↗