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Sonographic determination of normal Conus Medullaris level and ascent in early infancy.
Rozzelle CJ, Reed GT, Kirkman JL, Shannon CN, Chern JJ, Wellons JC, Tubbs RS
(2014) Childs Nerv Syst 30: 655-8
MeSH Terms: Humans, Infant, Infant, Newborn, Lumbosacral Region, Neural Tube Defects, Reference Values, Spinal Cord, Ultrasonography
Show Abstract · Added March 20, 2014
INTRODUCTION - Controversial reports exist in the literature regarding both the spinal level of the conus medullaris (CM) in normal infants and the age at which the CM achieves its adult level. Autopsy studies have demonstrated ascent continuing into early infancy while more recent imaging study series' suggest the adult conus level is attained by the 40th postmenstrual week.
METHODS - The authors conducted a retrospective review of 1,273 screening lumbar ultrasound studies performed over 5 years at a pediatric tertiary referral center. All patients were infants referred for initial imaging to rule out the presence of a tethered spinal cord. Referral sources included urban academic, urban private practice, and rural private practice pediatricians. After excluding studies lacking sufficient documentation (n = 90) and those reported as abnormal (n = 106), 1,077 remained for review. The CM level and patient age in days were recorded from each study. Statistical analysis was performed using unpaired t testing and ANOVA for continuous variables; chi-square for categorical data.
RESULTS - The mean CM level for infants in group I (ages 0-30 days) was compared to those in groups II (31-60 days) and group III (61-100 days). Group I had a mean CM level of 0.125 and 0.2 vertebral segments lower than groups II and III (p = 0.0005 and <0.0001, respectively). ANOVA comparison of all three groups confirmed a rostral migratory trend (p < 0.001). The prevalence of CM level caudal to L2 in group I was 13 %, group II 11.4 %, and group III 4.7 %; also indicating a significant rostral trend (p = 0.004).
CONCLUSIONS - Rostral migration of CM level continues through the first few months of post-natal life, albeit of limited extent. Documentation of continued ascent in a neonate may obviate the need for magnetic resonance imaging.
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8 MeSH Terms
Use of lumbar ultrasonography to detect occult spinal dysraphism.
Chern JJ, Kirkman JL, Shannon CN, Tubbs RS, Stone JD, Royal SA, Oakes WJ, Rozzelle CJ, Wellons JC
(2012) J Neurosurg Pediatr 9: 274-9
MeSH Terms: Female, Humans, Infant, Infant, Newborn, Lumbosacral Region, Male, Neonatal Screening, Neural Tube Defects, Patient Selection, Predictive Value of Tests, Primary Health Care, Retrospective Studies, Skin, Ultrasonography
Show Abstract · Added March 20, 2014
OBJECT - Various cutaneous stigmata and congenital anomalies are accepted as sufficient reasons to perform lumbar ultrasonography as a screening tool to rule out occult spinal dysraphism (OSD). The purpose of this study was to correlate presenting cutaneous findings with lumbar ultrasonography results based on a large number of lumbar ultrasonography tests obtained by regional primary care providers.
METHODS - Over the course of 5 years, 1273 infants underwent lumbar ultrasonography screening at a major pediatric tertiary referral center. Of these infants, 1116 had adequate documentation for retrospective chart review. Referral sources included urban academic, urban private practice, and surrounding rural private practitioners. Presence of cutaneous stigmata and/or congenital anomalies and lumbar ultrasonography results were reviewed for all patients. When present, surgical findings were reviewed.
RESULTS - A total of 943 infants were referred for presumed cutaneous stigmata, the most common of which was a sacral dimple (638 patients [68%]) followed by hairy patch (96 patients [10%]). Other reported cutaneous findings included hemangioma, deviated gluteal fold, skin tag, and skin discoloration. In comparison, 173 patients presented with congenital anomalies, such as imperforate anus (56 patients [32%]) and tracheoesophageal fistula/esophageal atresia (37 patients [21%]), most of which were detected prenatally by fetal ultrasonography. A total of 17 infants underwent surgical exploration. Occult spinal dysraphism was diagnosed in 7 infants in the cutaneous stigmata group and in 10 infants in the group with congenital abnormalities. None of the cutaneous stigmata as recorded were found to be indicative of the presence of OSD.
CONCLUSIONS - Cutaneous markers as currently defined by general practitioners are not useful markers for predicting OSD. The vast majority of findings on lumbar ultrasonography studies performed under these circumstances will be negative.
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14 MeSH Terms
Long-term urological impact of fetal myelomeningocele closure.
Clayton DB, Tanaka ST, Trusler L, Thomas JC, Pope JC, Adams MC, Brock JW
(2011) J Urol 186: 1581-5
MeSH Terms: Child, Child, Preschool, Female, Fetal Diseases, Fetus, Follow-Up Studies, Humans, Infant, Newborn, Lumbosacral Region, Male, Meningomyelocele, Neurosurgical Procedures, Pregnancy, Retrospective Studies, Time Factors, Treatment Outcome, Urinary Bladder, Urinary Incontinence, Urodynamics
Show Abstract · Added February 19, 2015
PURPOSE - Between 1997 and 2002 a large number of fetal myelomeningocele closures were performed at our institution. Previously early reports showed little improvement in neonatal bladder function after fetal back closure. We evaluated the long-term urological impact of this procedure.
MATERIALS AND METHODS - Using a combination of retrospective review and survey questionnaire we reviewed the records of 28 patients in whom fetal myelomeningocele closure was done at our institution between 1997 and 2002. The areas addressed included medical management for neurogenic bladder and bowel, need for lower urinary tract reconstruction and functional bladder assessment by videourodynamics. Parameters after fetal myelomeningocele closure were compared to those of 33 age and sex matched patients with myelomeningocele who underwent standard postnatal closure.
RESULTS - We reviewed the records of 28 patients after fetal myelomeningocele closure. At a mean age of 9.6 years 23 used clean intermittent catheterization to manage the bladder, 24 required a bowel regimen to manage constipation and 6 underwent lower urinary tract reconstruction with enterocystoplasty and a catheterizable bladder channel. Videourodynamics performed in 14 patients at a mean age of 7.4 years revealed decreased bladder capacity in 71%, detrusor overactivity in 35% and increased detrusor pressure in 25%. Compared to age and sex matched children who underwent postnatal closure we noted no significant differences in bladder management, urinary tract surgery or urodynamics.
CONCLUSIONS - Neurogenic bowel and bladder management continues to be a significant issue for patients after fetal myelomeningocele closure. After fetal surgery patients should be followed closely, similar to patients who undergo postnatal closure.
Copyright © 2011 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.
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19 MeSH Terms
The usefulness of lumbosacral magnetic resonance imaging in the management of isolated dysfunctional elimination.
Broughton GJ, Clayton DB, Tanaka ST, Thomas JC, Adams MC, Brock JW, Pope JC
(2011) J Urol 186: 1715-20
MeSH Terms: Adolescent, Child, Child, Preschool, Diagnosis, Differential, Female, Humans, Lumbosacral Plexus, Lumbosacral Region, Magnetic Resonance Imaging, Male, Reproducibility of Results, Retrospective Studies, Spinal Cord, Spinal Cord Diseases, Urinary Bladder, Urination Disorders, Urodynamics
Show Abstract · Added February 19, 2015
PURPOSE - Magnetic resonance imaging is sometimes used to rule out spinal pathology in patients with dysfunctional elimination, although its usefulness in this setting is unclear. We determined the usefulness of magnetic resonance imaging in children with isolated dysfunctional elimination, and normal cutaneous, neurological and orthopedic examinations.
MATERIALS AND METHODS - We reviewed the records of children with dysfunctional elimination who underwent lumbosacral magnetic resonance imaging and identified those who were neurologically normal and who had normal cutaneous back examinations. Our primary goal was to determine the rate at which magnetic resonance imaging identified a spinal abnormality in this population. Univariate analysis was performed to assess the association of several clinical variables with a radiographic abnormality on magnetic resonance imaging.
RESULTS - Between 2000 and 2009 a total of 49 lumbosacral magnetic resonance imaging studies were performed in 29 females and 18 males with a mean age of 8.5 years (range 2.1 to 17). Four of the 49 imaging studies (8%) revealed a radiographic abnormality but in only 1 (2%) was clinically significant spinal pathology identified, that is filum lipoma causing cord tethering. Findings on the 3 remaining abnormal imaging studies were considered unrelated to bladder dysfunction. No clinical, demographic or videourodynamic findings were associated with a radiographic abnormality on magnetic resonance imaging.
CONCLUSIONS - In children with isolated dysfunctional elimination complaints, and normal neurological, orthopedic and back examinations the diagnostic yield of lumbosacral magnetic resonance imaging is low but not zero. We suggest that spinal magnetic resonance imaging be used judiciously and as a test of last resort in these children.
Copyright © 2011 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.
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17 MeSH Terms
Cytogenetic, telomere, and telomerase studies in five surgically managed lumbosacral chordomas.
Butler MG, Dahir GA, Hedges LK, Juliao SF, Sciadini MF, Schwartz HS
(1995) Cancer Genet Cytogenet 85: 51-7
MeSH Terms: Adult, Aged, Blotting, Southern, Chordoma, Chromosome Aberrations, DNA, Neoplasm, Female, Humans, Karyotyping, Lumbosacral Region, Male, Middle Aged, Repetitive Sequences, Nucleic Acid, Spinal Neoplasms, Telomerase, Telomere
Show Abstract · Added March 5, 2014
Lumbosacral chordomas are rare skeletal sarcomas of the spine that originate from the remnant notochord. The understanding of this human cancer is limited to observations of its clinical behavior and its embryonic link. Thus, we performed chromosome and molecular analyses from five surgically harvested chordomas in an effort to document genetic and biochemical abnormalities which might aid in understanding the tumor biology of this understudied neoplasm. Cytogenetic analysis of the five chordomas revealed normal results in four patients and random abnormalities in only one tumor cell in the 100 cells studied from the fifth patient. A repeat telomeric probe (TTAGGG)50 was hybridized to genomic DNA isolated from chordoma cells (and HeLa cells) and digested with HinfI. The tumor DNA was paired with leukocyte DNA from age-matched controls and revealed telomere elongation in four of the four chordoma patients studied with molecular genetic techniques. Conversely, telomere length reduction has been reported during in vitro senescence of human fibroblasts, giant cell tumor of bone, colon cancer, intracranial tumors, childhood leukemia, Wilms tumor, and in HeLa cells. Telomerase activity (telomerase is required to maintain telomere integrity) was also determined by visualizing the extension of radioactive telomeric repeats on DNA sequencing gels. The telomeric fragments were assembled during incubation of the cytoplasmic extract containing telomerase. Telomerase activity was observed in HeLa (positive control and commercially available cell line), giant cell tumor of bone (positive control tumor cells from living patients), and in chordoma cells from one of the two chordoma patients (but to a lesser degree compared with HeLa). As expected, the chordoma patients' fibroblasts exhibited no telomerase activity.
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16 MeSH Terms