Occult Spinal Dysraphism: A Series of 73 Cases

PEDIATRICS ◽  
1975 ◽  
Vol 55 (6) ◽  
pp. 826-835 ◽  
Author(s):  
Frank M. Anderson
Keyword(s):  
Distal Part ◽  
Spinal Dysraphism ◽  
Clinical Manifestations ◽  
Dermal Sinus ◽  
Loss Of Function ◽  
Occult Spinal Dysraphism ◽  
Neural Tissues ◽  
The Subject ◽  
The Many ◽  
Developmental Variants

The subject of occult spinal dysraphism or myelodysplasia is reviewed from standpoints of embryology, clinical manifestations, and treatment, and the management of 73 cases summarized. In general, these concealed lesions arise from developmental variants in the most distal part of the neural tube, a situation which may cause distortion or partial absence of neural tissues and also lead to damage from compression or traction. Lipomyelomeningocele and congenital dermal sinus are two examples of the many types of such lesions, but some are more complicated, and borderline myelomeningocele-like forms occur. Incontinence, deformity or weakness of the feet, impaired gait, and other difficulties may appear late and increase with growth. Surgical treatment is advised to reduce chances of delayed or progressive loss of function.

scholarly journals Use of magnetic resonance imaging to detect occult spinal dysraphism in infants

2017 ◽  
Vol 19 (2) ◽  
pp. 217-226 ◽  
Author(s):  
Brent R. O'Neill ◽  
Danielle Gallegos ◽  
Alex Herron ◽  
Claire Palmer ◽  
Nicholas V. Stence ◽  
...  
Keyword(s):  
Congenital Anomalies ◽  
Imaging Modality ◽  
Spinal Dysraphism ◽  
Sinus Tract ◽  
Conus Medullaris ◽  
Dermal Sinus ◽  
Occult Spinal Dysraphism ◽  
Disc Space ◽  
Dermal Sinus Tract ◽  
Study Population

OBJECTIVE Cutaneous stigmata or congenital anomalies often prompt screening for occult spinal dysraphism (OSD) in asymptomatic infants. While a number of studies have examined the results of ultrasonography (US) screening, less is known about the findings when MRI is used as the primary imaging modality. The object of this study was to assess the results of MRI screening for OSD in infants. METHODS The authors undertook a retrospective review of all infants who had undergone MRI of the lumbar spine to screen for OSD over a 6-year period (September 2006–September 2012). All images had been obtained on modern MRI scanners using sequences optimized to detect OSD, which was defined as any fibrolipoma of the filum terminale (FFT), a conus medullaris ending at or below the L2–3 disc space, as well as more complex lesions such as lipomyelomeningocele (LMM). RESULTS Five hundred twenty-two patients with a mean age of 6.2 months at imaging were included in the study. Indications for imaging included isolated dimple in 235 patients (45%), asymmetrically deviated gluteal cleft in 43 (8%), symmetrically deviated (Y-shaped) gluteal cleft in 38 (7%), hemangioma in 28 (5%), other isolated cutaneous stigmata (subcutaneous lipoma, vestigial tail, hairy patch, and dysplastic skin) in 31 (6%), several of the above stigmata in 97 (18%), and congenital anomalies in 50 (10%). Twenty-three percent (122 patients) of the study population had OSD. Lesions in 19% of these 122 patients were complex OSD consisting of LMM, dermal sinus tract extending to the thecal sac, and lipomeningocele. The majority of OSD lesions (99 patients [81%]) were filar abnormalities, a group including FFT and low-lying conus. The rate of OSD ranged from 12% for patients with asymmetrically deviated gluteal crease to 55% for those with other isolated cutaneous stigmata. Isolated midline dimple was the most common indication for imaging. Among this group, 20% (46 of 235) had OSD. There was no difference in the rate of OSD based on dimple location. Those with OSD had a mean dimple position of 15 mm (SD 11.8) above the coccyx. Those without OSD had a mean dimple position of 12.2 mm (SD 19) above the coccyx (p = 0.25). CONCLUSIONS The prevalence of OSD identified with modern high-resolution MRI screening is significantly higher than that reported with US screening, particularly in patients with dimples. The majority of OSD lesions identified are FFT and low conus. The clinical significance of such lesions remains unclear.

Conflicting Diagnosis of Dermal Sinus Tract and Tethered Cord

2013 ◽  
Vol 40 (6) ◽  
pp. 889-891
Author(s):  
Chris J. Hong ◽  
Saleh A. Almenawer ◽  
Boleslaw Lach ◽  
Nina Stein ◽  
Benedicto Baronia ◽  
...  
Keyword(s):  
Spinal Dysraphism ◽  
Tethered Cord ◽  
Sinus Tract ◽  
Neural Tube Closure ◽  
Dermal Sinus ◽  
Lumbar Region ◽  
Occult Spinal Dysraphism ◽  
Developmental Abnormalities ◽  
Skin Tags ◽  
Dermal Sinus Tract

Dermal sinus tracts (DSTs) are an uncommon form of occult spinal dysraphism that is attributed to incomplete neural tube closure during fetal development. Dermal sinus tracts are found along the midline neuroaxis from the nasion to the coccyx, but they most commonly appear in the lumbar region. Dermal sinus tracts are more commonly associated with other developmental abnormalities such as skin tags, naevi, spinal dermoid cysts, meningocoele, lipomas and spinal cord tethering, and can be complicated by cerebrospinal fluid drainage, shedding of keratin from the epithelialized tract, and infection such as meningitis.

Dorsal dermal sinus and dermoid cysts in occult spinal dysraphism

1999 ◽  
Vol 134 (6) ◽  
pp. 793 ◽  
Author(s):  
Haruo Hattori ◽  
Yoshihisa Higuchi ◽  
Yuzuru Tashiro
Keyword(s):  
Spinal Dysraphism ◽  
Dermal Sinus ◽  
Occult Spinal Dysraphism

A rare case of Diastematomyelia with syrinx and tethered cord in a newborn

2021 ◽  
Vol 7 (3) ◽  
pp. 164-166
Author(s):  
Mahishma. K ◽  
Veeramalla Sandeep ◽  
Furkhan Hadi
Keyword(s):  
Bladder Dysfunction ◽  
Spinal Dysraphism ◽  
Skin Pigmentation ◽  
Tethered Cord ◽  
Conus Medullaris ◽  
Dermal Sinus ◽  
Loss Of Function ◽  
Longitudinal Splitting ◽  
Variable Extent ◽  
Bowel And Bladder Dysfunction

Diastematomyelia or Split cord syndrome is a rare form of spinal dysraphism characterized by longitudinal splitting of spinal cord, conus medullaris or filum terminale to a variable extent. Presence of SCM is suggested by certain superficial markers like skin pigmentation, hemangioma, lipoma, dermal sinus and hypertrichosis. Meningocele or myelomenigocele may also be present. Affected children usually present with progressive sensorimotor symptoms and bowel and bladder dysfunction. Development of sensorimotor symptoms and progressive loss of function emphasis the need for antenatal diagnosis of the spinal deformities which paves way for early intervention and management thus minimizing the morbidity.

Anterior Sacral Pyocele With Meningitis: A Rare Presentation of Occult Spinal Dysraphism With Congenital Dermal Sinus

2010 ◽  
Vol 25 (11) ◽  
pp. 1393-1397 ◽  
Author(s):  
Sonal Bhatia ◽  
Milind S. Tullu ◽  
Nitin B. Date ◽  
Dattatraya Muzumdar ◽  
Mamta N. Muranjan ◽  
...  
Keyword(s):  
Spinal Dysraphism ◽  
Dermal Sinus ◽  
Occult Spinal Dysraphism ◽  
Rare Presentation ◽  
Congenital Dermal Sinus

scholarly journals P.234 “Inappropriate” spinal ultrasounds for suspected occult spinal dysraphism in northern Alberta

2021 ◽  
Vol 48 (s3) ◽  
pp. S88-S88
Author(s):  
M Keough ◽  
A Thompson ◽  
V Mehta
Keyword(s):  
Imaging System ◽  
Spinal Dysraphism ◽  
Sinus Tract ◽  
Choosing Wisely ◽  
Dermal Sinus ◽  
Mri Imaging ◽  
Occult Spinal Dysraphism ◽  
Cutaneous Manifestations ◽  
Family Doctors ◽  
Dorsal Appendage

Background: Occult spinal dysraphism (OSD) may be associated with visible cutaneous manifestations. A common non-pathological mimic of a dermal sinus tract is an incidental sacrococcygeal dimple. Choosing Wisely Canada Guidelines recommend these dimples not be imaged. Methods: This study assessed the appropriateness of spinal ultrasounds performed for the investigation of an OSD. We interrogated our local imaging system to analyze spinal ultrasounds being performed in babies less than 6 months of age, from 2017-2018. Results: 429 children had spinal ultrasounds ordered by pediatricians (55%), family doctors (21%), and neonatologists (20%). 183 children (43%) had imaging indications that was deemed “inappropriate”. Some of this cohort had further MRI imaging (5/183) or neurosurgical referral (8/183). None of these children underwent neurosurgery. 231 (54%) children had appropriate indications for imaging. Within this cohort MRI imaging (23/231), neurosurgical referral (24/231) and neurosurgical intervention occurred (4/231). All four “surgical children” harboured either a dorsal appendage or a subcutaneous lipoma. We estimated, declining inappropriate scans would save $22, 500 annually. Conclusions: A significant portion of local spinal ultrasound requests for OSD are inappropriate. Collaboration with other sites is ongoing to investigate if the Choosing Wisely guidelines for imaging this patient population are being practiced more efficiently.

scholarly journals Spinal neurenteric cysts and their relation to more common aspects of occult spinal dysraphism

2001 ◽  
Vol 10 (1) ◽  
pp. 1-10 ◽  
Author(s):  
Michael J. Rauzzino ◽  
R. Shane Tubbs ◽  
Eben Alexander ◽  
Paul A. Grabb ◽  
W. Jerry Oakes
Keyword(s):  
Spinal Cord ◽  
Spinal Dysraphism ◽  
Postoperative Outcome ◽  
Cervical Region ◽  
Split Cord Malformation ◽  
Neurenteric Cyst ◽  
Dermal Sinus ◽  
Occult Spinal Dysraphism ◽  
Complete Excision ◽  
Thoracolumbar Region

Object Neurenteric cysts are infrequently reported congenital abnormalities believed to be derived from an abnormal connection between the primitive endoderm and ectoderm. The authors report a series of 13 patients treated over a 50-year period. Methods Of the 13 patients, seven were female and six were male. Their ages at presentation ranged widely from 5 weeks to 52 years of age. Children presented more commonly with cutaneous stigmata of occult spinal dysraphism (OSD) whereas adults presented primarily with pain. Neurological deficit as a presenting symptom was less common in our series, a finding that reflects the slow growth of these lesions. In all but one patient some form of vertebral anomaly was associated with the cystic lesions, including two patients with Klippel–Feil abnormalities. There was a high incidence of associated forms of OSD including split cord malformation, lipoma, dermal sinus tract, and tethered spinal cord. In previous reports the authors have suggested that neurenteric cysts are more common in the cervical region and in a position ventral to the cord. In the present series these cysts most commonly occurred as intradural, extramedullary masses in the thoracolumbar region, situated dorsal to the spinal cord. The median follow-up period was 7.5 years, and postoperative outcome reflected a patient's preoperative neurological status; in no patient was outcome worsened due to surgery. Conclusions Complete excision of the neurenteric cyst remains the treatment of choice, as subtotal excision is associated with recurrence.

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