Spinal Dysraphism In Children Research Articles

Spinal dysraphism in children: 3 years experience

Introduction: Spinal dysraphism (SD) is a congenital malformation of the spine and spinal cord resulting from congenital aberrations during the gastrulation, primary neurulation, and secondary neurulation stages. SD is the most common congenital anomaly of the CNS. Our study outlines the demography and clinical profile of this spectrum. Aim of the study: The aim of the study was to assess the demographic characteristics and clinical features of spinal dysraphism in children over a three-year period. Methods: This retrospective study involved 38 children with spinal dysraphism at the Department of Pediatric Surgery, Bangabandhu Sheikh Mujib Medical University (BSMMU) from January 2021 to December 2023. Inclusion criteria included complete clinical records and surgical intervention. Diagnostic methods comprised history, clinical exams, USG, CT, and MRI. Postoperative follow-up was assessed. Data were analyzed using SPSS version 22.0. Result: The study included 38 patients aged from 4 days to 7.5 years. The cohort exhibited a slight female predominance (57.89%). Myelomeningocele was the most predominant anomaly (52.63%), followed by lip myelomeningocele (31.58%) and meningocele (13.16%). Lesions were predominantly located in the lumbosacral region (92.11%). All patients presented with a swelling on the back. Associated conditions included hydrocephalus (34.21%), CSF leak (23.68%), and bowel and bladder incontinence (15.79%). All cases underwent an operative procedure with anatomical repair using standard techniques. Conclusion: Among spinal dysraphism, myelomeningocele was more common than other spinal defects. All patients presented with back swelling, with or without associated symptoms.

Use of ultrasound to estimate the prevalence of occult spinal dysraphism in children undergoing urogenital and anorectal surgeries: A cross-sectional study.

The use of ultrasound has immensely increased the safety toward regional blocks and central venous access and has been considered as the standard of care for securing central access. The aim of this study is to estimate the prevalence of occult spinal dysraphism using ultrasound in children less than 2 years of age undergoing elective urogenital or anorectal surgery. The lumbosacral region of 159 American Society of Anesthesiologists (ASA) category I/II patients, posted for elective urogenital and anorectal surgery was scanned with ultrasound, prior to giving caudal block. The prevalence of occult spina bifida was 3% in our study. There was no statistically significant association of cutaneous marker with abnormal scan. Prevalence of occult spina bifida was ten-times higher in our study than in the general population. Perioperative ultrasound screening of the lower spinal anatomy by anesthesiologist done prior to performing neuraxial block is worthwhile in ruling out occult spinal anomalies in high-risk children of occult spinal dysraphism.

The AIUM Practice Parameter for the Performance of an Ultrasound Examination of the Neonatal and Infant Spine.

The AIUM Practice Parameter for the Performance of an Ultrasound Examination of the Neonatal and Infant Spine.

604 - The prevalence and clinical features of spinal dysraphism in children with hypospadia

604 - The prevalence and clinical features of spinal dysraphism in children with hypospadia

AIUM Practice Parameter for the Performance of an Ultrasound Examination of the Neonatal and Infant Spine

AIUM Practice Parameter for the Performance of an Ultrasound Examination of the Neonatal and Infant Spine

AIUM Practice Parameter for the Performance of an Ultrasound Examination of the Neonatal and Infant Spine.

AIUM Practice Parameter for the Performance of an Ultrasound Examination of the Neonatal and Infant Spine.

Systematic Review of Urologic Outcomes from Tethered Cord Release in Occult Spinal Dysraphism in Children.

Tethered cord syndrome describes a condition of multisystem end organ dysfunction due to fixation of the spinal cord. This systematic review focuses on the closed skin variant of this condition, occult spinal dysraphism. The embryology, pathophysiology, presentation, and classification of occult spinal dysraphism are explained to develop a simple framework for discussions regarding this often confusing condition. Following Preferred Reporting Items for Systematic Review and Meta-Analyses (PRISMA) guidelines, we synthesized urologic outcome data after tethered cord release in children from 17 studies performed over the past 25years. These results prompted several conclusions. First, the different subgroups and different nomenclature of tethered cord syndrome are often confused, making interpretation of results difficult. Second, untethering has a positive effect on urologic symptoms and urodynamics parameters. Third, timing of untethering is important: early intervention prevents significant long-term traction aiming to avoid irreversible neurologic damage. Fourth, pediatric urologists and neurosurgeons have an important role in diagnosing and treating this condition and should work closely as part of a multidisciplinary team.

Ultrasonography reveals a high prevalence of lower spinal dysraphism in children with urogenital anomalies

Lower spinal dysraphism is frequently reported in anorectal anomaly combined with urogenital anomalies. The prevalence of the spinal dysraphism has not been comprehensively studied in children with simple urogenital anomalies. We evaluated the prevalence of the spinal dysraphism using ultrasound data of the lumbosacral area in children with urogenital anomalies. Lumbosacral ultrasound images of 259 children who underwent urological surgery with simple urogenital anomalies were reviewed by an ultrasound-specialized radiologist. The primary outcome measures were the conus medullaris (CM) level and the thickness of the filum terminale. The spinal ultrasonographic findings that were assessed in children showed abnormal spinal findings compared with the other children having normal findings. Two years later, the follow-up telephone interviews were made with the parents of the children with abnormal findings. Eighteen children were differentiated as the abnormal finding group. They were suspected of spinal cord tethering. The level of CM was lower, and the filum terminale was thicker compared to the normal group [L2(lower (L)) vs. L1(L), 2.2 mm vs. 0.8 mm]. Of eighteen children, four were confirmed as tethered spinal cord with lipoma on magnetic resonance imaging by the time of surgery, and two were strongly suspected of occult spinal dysraphism (OSD) based on ultrasound findings and follow-up interviews. The prevalence of OSD in children under 24 months of age with simple urogenital anomaly was higher than what was reported for the general population. Ultrasound examination of spinal structures before caudal block in children with urogenital anomaly should be considered.

Anterior sacral meningocele presenting as constipation

Anterior sacral meningocele (ASM) is a rare form of spinal dysraphism in children. Usually asymptomatic, it can present as constipation, urinary problems or rarely neurological symptoms. High index of suspicion with careful clinical examination is necessary to make early diagnosis. Magnetic resonance imaging is the investigation of choice. We describe a successfully managed young child with ASM associated with rib and vertebral defects.

The value of sacral skin lesions in predicting occult spinal dysraphism in children with voiding dysfunction and normal neurological examination

ObjectiveThe role of magnetic resonance imaging (MRI) in detecting occult spinal dysraphism (OSD) in children with voiding dysfunction and a normal neurological examination is still under debate. The aim of this study was to assess the correlation of sacral skin lesions with OSD detected on MRI, in a population of children with resistant lower urinary tract symptoms (LUTS). Patients and methodsA total of 114 children over 5 years of age with urinary tract infection (UTI) and/or LUTS and normal neurological examination were enrolled. All children underwent sacral neurological examination, urine analysis and cultures, renal/bladder ultrasound, voiding cystourethrogram and urodynamic examination. After a treatment period of 6 months, the patients were re-evaluated and spinal MRI was performed in 61 with ongoing LUTS or UTI. ResultsNineteen of 61 children (31%) had cutaneous stigmas. MRI detected spinal abnormality in 2/42 children with a normal sacral examination in comparison to 7/19 children with an abnormal sacral finding (Chi-squared test, P < 0.005). The sensitivity and specificity of an abnormal sacral finding in predicting MRI abnormality were 0.76 and 0.77, respectively. Urodynamic parameters did not predict an abnormal spinal MRI. ConclusionsAbnormal sacral findings, but not urodynamic studies, are strong predictors of OSD. A normal sacral examination does not rule out OSD.

A rare cutaneous sign of occult spinal dysraphism with tethered spinal cord

©2008, World J Pediatr. All rights reserved. A female, born after normal pregnancy and labor, was referred for consultation for a 1-cm long fi brous appendage in the midline of her lumbosacral region one day after birth (Fig. 1). Although she was neurologically intact, computed tomography at 3 months revealed L4-L5 spina bifi da (Fig. 2A), and magnetic resonance imaging (MRI) showed a low conus medullaris level (L3), and a tethered dorsal spinal cord that seemed to connect with the site of the skin lesion (Fig. 2B). Spina bifi da occulta with tethered spinal cord due to a dermal sinus tract was diagnosed and the patient was treated surgically. At operation, the sinus tract extending from the skin to the spinal cord was observed, but the thickening of the fi lum terminale was not evident. Occult spinal dysraphism with tethered spinal cord, if not recognized, may result in permanent neurologic defi cits. Moreover, the neurological disturbances can be diffi cult to demonstrate in a young baby and clinical evaluation of bladder function is equally diffi cult at this age. To prevent serious sequelae, early screening of high-risk infants with cutaneous markers that present in 50%-90% of patients, radiologic diagnosis using X-rays, either MRI or sonography, and urodynamic testing, and the appropriate timing of surgical untethering should be performed. However, dorsal cutaneous lesions may be ignored unless they are common lesions such as midline hemangiomas, hairy patches, dimples or sinuses, or pigmented macules. In order to screen effectively, the physician must have a high degree of suspicion for such a rare skin abnormality as this case. References 1 Guggisberg D, Hadj-Rabia S, Viney C, Bodemer C, Brunelle F, Zerah M, et al. Skin markers of occult spinal dysraphism in children: a review of 54 cases. Arch Dermatol 2004;140:1109-1115. 2 Cornette L, Verpoorten C, Lagae L, Van Calenbergh F, Plets C, Vereecken R, et al. Tethered cord syndrome in occult spinal dysraphism: timing and outcome of surgical release. Neurology 1998;50:1761-1765. 3 Yamada S, Won DJ, Siddiqi J, Yamada SM. Tethered cord syndrome: overview of diagnosis and treatment. Neurol Res 2004;26:719-721. Received March 31, 2008 Accepted after revision May 8, 2008 A rare cutaneous sign of occult spinal dysraphism with tethered spinal cord

Extensive Perineal Infantile Hemangioma with Associated Congenital Anomalies: An Example of the PELVIS Syndrome

Extensive Perineal Infantile Hemangioma with Associated Congenital Anomalies: An Example of the PELVIS Syndrome

Skin Markers of Occult Spinal Dysraphism in Children—Correction

Skin Markers of Occult Spinal Dysraphism in Children—Correction

Occult spinal dysraphism in children: the time and the surgical treatment of 50 children

The diagnosis and the surgical treatment of 50 children with occult spinal dysraphism followed from 1982 to 1992 are analyzed. The ages at the time of the surgery ranged from 9 days to 16 years. Sixty per cent (60%) were less than two years old, and neither sex was predominant […] OCCULT SPINAL DYSRAPHISM IN CHILDREN: THE TIME AND THE SURGICAL TREATMENT OF 50 CHILDREN (ABSTRACT).

The role of magnetic resonance imaging in the investigation of spinal dysraphism in the child with lower limb abnormality.

A review of magnetic resonance imaging (MRI) performed to exclude the presence of spinal dysraphism in children presenting with lower limb pathology is unrewarding in the absence of abnormal neurology. Over a 5-year period, 29 children ages 2 weeks to 15 years with a mean age of 6 years presenting with lower limb abnormalities were referred for MRI of the spine to exclude an occult neurologic cause for the deformities. More than one limb abnormality, for example pes cavus and limb length discrepancy, was present in 93% of the children, and 11 children had severe or recurrent talipes equinovarus deformity. Only two children (7%), both of whom had abnormal limb neurology, had abnormal MRI scans. In the absence of a demonstrable neurologic deficit in the lower limb, there appears to be no advantage in requesting MRI of the spine in children presenting with lower limb abnormality.

Long-term urological follow-up of occult spinal dysraphism in children: M.L. Capitanucci, B.D. Iacobelli, M. Silveri, et al. Eur J Pediatr Surg 6:25–26, (December), 1996 (suppl 1)

Long-term urological follow-up of occult spinal dysraphism in children: M.L. Capitanucci, B.D. Iacobelli, M. Silveri, et al. Eur J Pediatr Surg 6:25–26, (December), 1996 (suppl 1)

Long-term urological follow-up of occult spinal dysraphism in children.

From 1980 to 1996 we followed up 65 children with occult spinal dysraphism (OSD) out of 85 observed in our urodynamic laboratory. The average age at diagnosis was 4.7 years (range: 1-21 years). In 19 cases (22%) the OSD was associated with anorectal anomalies (ARA). Urinary incontinence or voiding anomalies with urinary tract infection were the presenting symptom in 34 children (49%). At diagnosis all children underwent complete neuro-urological and urodynamic evaluation. Thirty-eight patients underwent neurosurgical correction. Vesicoureteral reflux (VUR) was present or developed in 17 patients: 15 underwent endoscopic procedures and 2 required surgery; 2 needed bladder augmentation because of upper tract and renal function deterioration. Urinary incontinence was treated mainly by intermittent catheterization and anticholinergic drugs, but endoscopic treatment was performed in 3 children. At long-term follow-up (2 to 14 years), socially acceptable continence was achieved in 78% of 57 children (8 could not be assessed because they were less than 4 years old). Upper urinary tract deterioration occurred in 15% and renal failure in 7.5%.

Occult spinal dysraphism in children: need for early neurosurgical referral

The natural history of occult spinal dysraphism in children is one of progressive and unpredictable neurological deficit. The modern role of neurosurgery is in the prevention of deterioration rather than correction of established disability. A prospective study was carried out on 40 consecutive patients to analyse the referral pattern of children with this condition. The age, reason for referral, clinical history and source of referral was recorded in each case. The reason for referral varied with age. Of the 40 patients studied, only 12 were found to be without neurological deficit at the time of referral. The incidence of progressive neurological deterioration increased with increasing age. The referral source in different age groups varied, but in all age groups relatively few were referred by the general paediatricians or paediatric neurologists. For optimum surgical results, early referral and treatment is desirable and should become our objective.

Spinal dysraphism in children

Spina bifida occulta is one form of spinal dysraphism, a term used to refer to imcomplete formation of the midline structure of the dorsum. It is therefore related to all the other forms of spina bifida which have been described under different names, spina bifida aperta or cystica. The basic differences between spina bifida cystica and spinal bifida occulta result from the nature of their origino The spina bifida cystica is a failure of development of the spinal cord tissues intrinsically, with added destructive processes in utero, and cellular hyperplasia, as well as brain anormalies. By contrast, in spina bifida occulta there may be some dysplasia of the spinal cord but none of the other destructive processes associated with cystica; Arnold-Chiari malformation is very unusual in spina bifida occulta. In cystica, clinical abnormaIities are present at birth because of the absence of nervous tissue. However, in occulta the anomalies are extrinsic tho the spinal cord. Clinical abnormalities usualy develop later. Normally, the nerve tissues change position with movements of the spine, particularly flexion and extension. During a long time it was thought that clinical symptoms developped later because the conus was fixed in low position by extrinsic factors as lipomas, adherences and so on. This theory was based in part on the fact that in most cases of spina bifida occulta the conus medullaris is found under the L 2 leve!. In our statistic the conus medullaris is situated lower than normal in the vertebral canal in 81 % of the 88 cases. We know now that absence of «ascension» of the spinal cord as cause of neurologic deficit is incorrecto Barson, in 1970, exarnined the normal spinal cord of 252 foetuses and children aged between 13 weeks gestation and puberty. He concluded that the normal spinal cord ends L 3 at 30 weeks, L 2 at 40 weeks, reaching the normallevel at L 1 L 2 about 9 weeks afier fullterm gestation. We think that the main faetors of secondary neurologieal deficit are repeated traumatism on a rigid, a fixed spinal cord and-possibly vascular deficit. The abnormalities grouped under the term «spinal dysraphism» include: 1. Malformations of the meninges (excluding the posterior meningocele): anterior, lateral, intra sacral meningoceles. Arachnoidal cysts. 2 . Malforrnations ofspinal cord and roots: Fibrous bands between cord and dura, abnormal roots, tethered eord, diastematomyelia 3 . Lipomas (subeutaneous, extramedullary, mtramedullary, in the filum terminale). 4. Derrnal sinus, dermoid cysts. 5. Neurenteric cysts. It is interesting to note that in many pa~ents some of these lesions are found together. Spinal dysraphism must be separated from the most common myelomeningoeele or meningoeele whieh are grouped under the term spinal raehisehisis.

Magnetic resonance evaluation of spinal dysraphism in children

Magnetic resonance (MR) imaging of the spine was performed as the initial imaging technique in 20 children when spinal dysraphism was suspected clinically and plain radiographs showed spina bifida. The correlation with surgical findings indicated that MR provided accurate information preoperatively in all the cases. Some unusual observations in cases with spina bifida cystica and occulta are discussed. It is concluded that plain radiograph and MR complete the preoperative radiologic evaluation of cases with spinal dysraphism.