Congenital Dermal Sinus Elements in Each Tethering Stalk of Coexisting Thoracic Limited Dorsal Myeloschisis and Retained Medullary Cord

2020 ◽  
pp. 1-8
Author(s):  
Takato Morioka ◽  
Nobuya Murakami ◽  
Masako Ichiyama ◽  
Takeshi Kusuda ◽  
Satoshi O. Suzuki
Keyword(s):  
Present Article ◽  
Dermoid Cyst ◽  
Spinal Dysraphism ◽  
Conus Medullaris ◽  
Dermal Sinus ◽  
Case Presentation ◽  
Upper Tract ◽  
Secondary Neurulation ◽  
Extradural Space ◽  
Congenital Dermal Sinus

<b><i>Introduction:</i></b> The embryogenesis of limited dorsal myeloschisis (LDM) likely involves impaired disjunction between the cutaneous and neural ectoderms during primary neurulation. Because LDM and congenital dermal sinus (CDS) have a shared origin in this regard, CDS elements can be found in the LDM stalk. Retained medullary cord (RMC) is a closed spinal dysraphism involving a robust, elongated, cord-like structure extending from the conus medullaris to the dural cul-de-sac. Because the RMC is assumed to be caused by impaired secondary neurulation, concurrent RMC and CDS cannot be explained embryologically. In the present article, we report a case in which CDS elements were noted in each tethering stalk of a coexisting LDM and RMC. <b><i>Case Presentation:</i></b> A 2.5-month-old boy with left clubfoot and frequent urinary and fecal leakage had 2 tethering tracts. The upper tract, which ran from the thoracic tail-like cutaneous appendage, had CDS elements in the extradural stalk and a tiny dermoid cyst in the intradural stalk immediately after the dural entry. In the lower tract, which ran from the lumbosacral dimple, the CDS as an extradural stalk continued to the RMC at the dural cul-de-sac. Both stalks were entirely resected through skip laminotomy/laminectomy at 1 stage to untether the cord and resect the CDS elements. <b><i>Conclusion:</i></b> Surgeons should be aware that CDS elements, in addition to LDM, may coexist with RMC that extends out to the extradural space.

Congenital dermal sinus with intracranial dermoid cyst

1994 ◽  
Vol 130 (2) ◽  
pp. 235-237 ◽  
Author(s):  
H. SAITO ◽  
R. OGONUKI ◽  
A. YANADORI ◽  
S. YAMAZAKI ◽  
M. NAGAI
Keyword(s):  
Dermoid Cyst ◽  
Dermal Sinus ◽  
Congenital Dermal Sinus

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.

Spinal Dermal Sinus Tract

2019 ◽  
pp. 47-55
Author(s):  
Nathan R. Selden
Keyword(s):  
Imaging Modality ◽  
Spinal Dysraphism ◽  
Cauda Equina ◽  
Sinus Tract ◽  
Conus Medullaris ◽  
Dermal Sinus ◽  
Dermal Sinus Tract ◽  
Long Term Follow Up ◽  
Operative Assessment ◽  
Spinal Dermal Sinus

Spinal dermal sinus tract is a rare form of spinal dysraphism that presents occasionally with signs of spinal tethering and rarely with repeated bouts of bacterial meningitis or fulminant pyogenic infection of the conus medullaris or cauda equina leading to serious loss of neurologic function. Physical examination is crucial to accurately diagnose cutaneous findings such as the presence of a pit or tract, in order to avoid subsequent neurological sequelae. Spinal MR imaging is the only definitive imaging modality for the diagnosis of spinal dermal sinus tract and surgical planning for its excision. Total removal of all dermoid tract material is necessary to prevent recurrence. Careful peri-operative assessment and long-term follow-up will optimize outcomes and minimize complications.

Dermoid cyst of the posterior fossa associated with congenital dermal sinus in a child

2008 ◽  
Vol 4 (1) ◽  
pp. 66-69 ◽  
Author(s):  
Chun-Quan Cai ◽  
Qing-Jiang Zhang ◽  
Xiao-Li Hu ◽  
Chun-Xiang Wang
Keyword(s):  
Dermoid Cyst ◽  
Posterior Fossa ◽  
Dermal Sinus ◽  
Congenital Dermal Sinus

scholarly journals Intramedullary Dermoid Cyst of the Cervical Spinal Cord – C5–C7 Level

2018 ◽  
Vol 37 (02) ◽  
pp. 140-144
Author(s):  
Palanisamy Seerangan ◽  
Aravinth Ashok ◽  
Jolarpettai Mahendran
Keyword(s):  
Spinal Cord ◽  
Dermoid Cyst ◽  
Histopathological Examination ◽  
Cervical Spinal Cord ◽  
Spinal Dysraphism ◽  
Operative Procedure ◽  
Hair Follicles ◽  
Cervical Region ◽  
Cervical Cord ◽  
Dermal Sinus

Introduction Inclusion cysts of the spinal cord are rarely intramedullary. Such cysts are commonly located in the lumbar and thoracic regions and are usually associated with congenital spinal dysraphism and dermal sinus. Intramedullary dermoid cysts in the cervical region without spinal dysraphism are extremely rare. To our knowledge, only seven such cases are reported in the literature to date. Materials and Methods An 18-year-old female patient presented with weakness in all four limbs, more distal than proximal muscle weakness, that had been progressing for 3 years. The magnetic resonance imaging (MRI) showed an intramedullary lesion from C5– C7 with peripheral ring enhancement. “Whorls” were observed within the lesion on T2 weighted image, with associated excavation of vertebral bodies C5– C7. Operative procedure and findings: partial laminectomy of C5– D1was performed. The dura was opened. A small myelotomy was made in the root entry zone. About 1.5 ml of yellowish colored fluid was drained. White shiny debris with hair, whitish pultaceous content and teeth were removed. Complete excision of cyst and its wall was performed. Results The histopathological examination revealed that the cyst wall was lined by stratified squamous epithelium with underlying dermis showing hair follicles, sebaceous glands, adipose tissue and cyst filled with keratin debris suggestive of dermoid cyst. Conclusion The intramedullary location of the dermoid cyst in the cervical cord and the absence of any congenital spinal dysraphism make this case a very unique and rare entity and warrants its inclusion in the reported cases of rare intramedullary space occupying lesions.

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
Sign in / Sign up

Export Citation Format

Share Document