An Official publication of The Asian Congress of Neurological Surgeons (AsianCNS)

Search Article
Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Advertise Subscribe Contacts Login  Facebook Tweeter
  Users Online: 2119 Home Print this page Email this page Small font sizeDefault font sizeIncrease font size  

   Table of Contents      
CASE REPORT
Year : 2020  |  Volume : 15  |  Issue : 1  |  Page : 172-175

Dermal sinus tract associated with type I and type II split cord malformation


1 Departament of Neurosurgery and Neurosciences, Hospital Militar Central, Universidad Militar Nueva Granada, Bogotá, Colombia
2 Departament of Pediatric Neurosurgery, Instituto Nacional de Pediatría, Mexico City, Mexico
3 Departament of Pediatric Neurosurgery, Chief of the Surgery Division, Instituto Nacional de Pediatría, Mexico City, Mexico

Date of Submission13-Sep-2019
Date of Acceptance11-Dec-2019
Date of Web Publication25-Feb-2020

Correspondence Address:
Dr. Juan Esteban Munoz Montoya
Departament of Neurosurgery, Hospital Militar Central, Universidad Militar Nueva Granada, Bogota
Colombia
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ajns.AJNS_287_19

Rights and Permissions
  Abstract 


The dermal sinus tract of the spine is associated with other occult spinal dysraphisms, such as the split cord malformation (diastematomyelia) in a 40% of the cases and embryologically is not clearly defined if the dermal sinus and split cord malformation have origin in gastrulation or late primary neurulation, but the most accepted theory of the dermal sinus tract consists in early incomplete disjunction, which explains the relation with other spinal dysraphisms. Here, we present two cases, with a dermal sinus tract of the spine associated with Type I and Type II split cord malformation.

Keywords: Dermal sinus, diastematomyelia, dysraphisms


How to cite this article:
Munoz Montoya JE, Jara MA, Vargas Osorio MP, Franco FR. Dermal sinus tract associated with type I and type II split cord malformation. Asian J Neurosurg 2020;15:172-5

How to cite this URL:
Munoz Montoya JE, Jara MA, Vargas Osorio MP, Franco FR. Dermal sinus tract associated with type I and type II split cord malformation. Asian J Neurosurg [serial online] 2020 [cited 2020 Apr 2];15:172-5. Available from: http://www.asianjns.org/text.asp?2020/15/1/172/279030




  Introduction Top


The dermal sinus tract of the spine is a rare spinal dysraphism which occurs in approximately 1 in 2500 newborns.[1],[2] The dermal sinus tract includes a stratified squamous epithelium, with variable depth, and it can end in any structure including the dural sac.[3],[4] The dermal sinus tract is associated with other occult spinal dysraphisms, as demonstrated by Gupta et al., that found an association of 11.34% with other dysraphisms.[5] The dermal sinus tract is associated with split cord malformation in 40% of cases.[4],[6] In addition to this, terminal syringomyelia is associated with split cord malformations although its incidence and natural history are not very clear.[7]

We present two cases with dermal sinus tract associated with split cord malformation.


  Case Reports Top


Case 1

A 10-month-old male presented with a delay in motor development and abnormal findings involving lower limbs.

At physical examination, him was noted a dorsal column deviation with left convexity and lumbosacral bluish spot, a thoracic midline cutaneous appendix at T10 level and another it at the right knee, in the left foot with presence of paraxial polydactyly by duplication of the first toe and in the right foot with third and fifth toe hypoplasia and absence of the fourth toe with appendix skin replacing it. Anal sphincter tone was decreased, and lower limb distal flaccid paraparesis was found [Figure 1]. Spine X-ray [Figure 2] and magnetic resonance imaging (MRI) were made and showed a dermal sinus tract with Type II split cord malformation [Figure 3]. Somatosensory evoked potential studies has identified compromise of the motor and sensory pathway in the lower limbs.
Figure 1: (a) Left foot polydactyly. (b) Third and fifth toe hypoplasia and absence of the fourth toe, (c) T10 dorsal column dermal appendix

Click here to view
Figure 2: (a) Simple X-ray showing lumbar spine rectification. (b) Scoliotic curve with left convexity, butterfly-like vertebrae at T3, T4, T5, T6, T8, T11, L3, and T10 hemivertebrae

Click here to view
Figure 3: (a) Sagittal T2 magnetic resonance imaging sequence showing terminal syringomyelia (arrow), (b) Sagittal T2 sequence revealing dermal sinus tract (arrow). (c) Axial T2, syringomyelia is identified with dilation of the spinal cord's central canal (white arrow). (d) Axial T2 white and black arrows, double spine cord

Click here to view


Thoracic dermal appendix was identified at the T10 level, and laminectomy was performed at that level, finding the dermal sinus tract termination at the dural sac. Dura was opened observing arachnoid adhesions in relation to fibrous septum formed in the dermal sinus tract causing split of the spinal cord into two parts. The septum and the dermal sinus tract were removed, and sent to pathology studies, added to the release of spinal cord adhesions [Figure 4]. Postoperatively, MRI was performed [Figure 5] and pathology report showed a stratified squamous epithelium.
Figure 4: (a) Dermal sinus tract with termination in the dural sac (black arrow) and arachnoid adherences (white arrow). (b) Opening of the dura with two split cord (black arrows) and (c) fibrous septum dividing spinal cord into two parts with unique dural sac (arrow)

Click here to view
Figure 5: Postoperative magnetic resonance imaging. (a) Syringomyelia decrease with respect to the previous (arrow). (b) Two spinal cords with without adhesions and without fibrous septum (white and black arrows)

Click here to view


In the patient's postoperative follow-up, distal and sensitive motor deficit was identified, showing sphincter involvement.

Case 2

One year old female presented a thoracolumbar protrusion which it has grown progressively, associated with sphineter compromise and lower limb weakness. Physical examination revealed a soft mass in the thoracolumbar region measuring 12 cm in diameter, with the presence of an dimple at its center [Figure 6], sphincter involvement, flaccid paraparesis, and bilateral equinovarus foot.
Figure 6: (a) Subcutaneous thoracolumbar 12 cm mass (arrow). (b) Dermal sinus in the thoracolumbar mass upper region

Click here to view


Somatosensory evoked potential studies has identified compromise of the motor and sensory pathway in the lower limbs. MRI [Figure 7] showed a dermal sinus tract with type I split cord malformation, associated with intraspinal lipoma and a lobulated intralesional arachnoid cyst.
Figure 7: Thoracolumbar spine magnetic resonance imaging. (a) T2 sequence sagittal section identifying complex occult spinal dysraphism with absence of the posterior arch from T10 to L2 (line and black arrow) and spinal cord protruding out the spinal canal, underlying to the dermal sinus tract, (b) T2 sequence, sagittal view which evidences a septum dividing the dural sac in two cavities (arrow), (c) T2 sequence, axial section: Evidence of bone septum that divides both spinal cords (arrow)

Click here to view


Surgery was performed for resection of the dermal sinus tract, opening of dural cavities, and resection of the osseous septum in relation to Type I split cord malformation [Figure 8]. Stratified squamous epithelium was confirmed by pathology. The patient after 1 month of follow-up dies from aspiration pneumonia.
Figure 8: (a) Thoracolumbar subcutaneous mass dissection identifying dermal sinus and dermal sinus tract (arrow). (b) Subcutaneous mass opening and which to identify a cord inside. (c) Each mass with a spinal cord (white and black arrows). (d) Dural reconstruction was performed in a single sheath. (e) A bone spur distal to the dural closure was identified (arrow)

Click here to view



  Discussion Top


Split cord malformations (diastematomyelia) and dermal sinus tract belong to the group of closed spinal dysraphisms, which are very heterogeneous, and they usually appear with cutaneous marks identified at birth in more than 50% of the cases such as skin appendages, hypertrichosis, lipomas, hemangiomas, or infection signs located at the spinal dysraphism site.[8]

Dermal sinuses can affect any neuraxis level, from the occipital to the sacral region, <1% affecting the cervical region, 10% thoracic, 40% lumbar, 12% lumbosacral, 23% sacral, and 13% sacrococcygeal.[9] In addition, its tract can end in soft tissues in the superficial dorsal region (6%–7%), in epidural space (10%–20%), and the great majority (58%–60%) in the dural sac, of these 50% can develop adhesions to the conus medullaris, terminal filum, and cauda equina.[1],[9],[10]

Embryologically, the dermal sinus tract origin is not clearly defined, but the most accepted theory consists in the incomplete early disjunction caused by a premature closure of the cutaneous ectoderm, and therefore, a permanent tract is formed between the skin and the neural tube, which occurs in late primary neurulation and in gastrulation phases.[11],[12] In split cord malformation, the defect is generated in the gastrulation phase, in which an endomesenchymal tract is produced by an accessory neuroenteric canal between the amnion and the yolk sac, that divides the notochord explaining the probable connection between split cord malformation with the dermal sinus tract.[10],[12],[13]

Split cord malformation is a rare form of spinal dysraphism, which is divided into Type I: A fibrocartilage or bone spur divides the spinal cord and Type II in which there is a fibrous or cartilaginous tissue that divides the spinal cord; finding a female predominance,[13],[14] most of them are located in the thoracic and mid-thoracic upper regions and are associated with skin abnormalities and other spinal dysraphisms, including the dermal sinus tract.[13],[15] It has been associated with low conus medullaris implantation in 83% of cases in addition to a thickened terminal filum.[13] Syringomyelia is an associated finding that has been found in 29%–55% of the cases[13],[16] and tethered cord.[17],[18]

For the diagnosis of occult spinal dysraphisms, MRI is fundamental because it allows to adequately characterize them, as well as to detect associated malformations.[4],[19]

For surgical management, it consist on the exploration of the dermal sinus tract until the dural sac, since there are several reports, showing that incomplete exploration could finish in neurological deterioration which can be observed after surgery, with the need to reexploration it.[1] In addition, arachnoid adhesions have been found intraoperatively, which contribute to tethered spinal cord in 33.3% of cases.[1]

In split cord malformation, it has been hypothesized that bone spur resection could prevent tethered spinal cord and duraplasty could contribute to an improvement in the circulation of cerebrospinal fluid and it would also lead to resolution of the terminal syringomyelia.[10],[18]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Ackerman LL, Menezes AH, Follett KA. Cervical and thoracic dermal sinus tracts. A case series and review of the literature. Pediatr Neurosurg 2002;37:137-47.  Back to cited text no. 1
    
2.
Kaufman BA. Neural tube defects. Pediatr Clin North Am 2004;51:389-419.  Back to cited text no. 2
    
3.
Ackerman LL, Menezes AH. Spinal congenital dermal sinuses: A 30-year experience. Pediatrics 2003;112:641-7.  Back to cited text no. 3
    
4.
Elton S, Oakes WJ. Dermal sinus tracts of the spine. Neurosurg Focus 2001;10:e4.  Back to cited text no. 4
    
5.
Gupta DK, Shastank RR, Mahapatra AK. An unusual presentation of lumbosacral dermal sinus with CSF leak and meningitis. A case report and review of the literature. Pediatr Neurosurg 2005;41:98-101.  Back to cited text no. 5
    
6.
Tubbs RS, Frykman PK, Harmon CM, Oakes WJ, Wellons JC 3rd. An unusual sequelae of an infected persistent dermal sinus tract. Childs Nerv Syst 2007;23:569-71.  Back to cited text no. 6
    
7.
Radmanesh F, Nejat F, El Khashab M. Dermal sinus tract of the spine. Childs Nerv Syst 2010;26:349-57.  Back to cited text no. 7
    
8.
Tortori-Donati P, Rossi A, Cama A. Spinal dysraphism: A review of neuroradiological features with embryological correlations and proposal for a new classification. Neuroradiology 2000;42:471-91.  Back to cited text no. 8
    
9.
Jindal A, Mahapatra AK. Spinal congenital dermal sinus: An experience of 23 cases over 7 years. Neurol India 2001;49:243-6.  Back to cited text no. 9
[PUBMED]  [Full text]  
10.
Gan YC, Sgouros S, Walsh AR, Hockley AD. Diastematomyelia in children: Treatment outcome and natural history of associated syringomyelia. Childs Nerv Syst 2007;23:515-9.  Back to cited text no. 10
    
11.
Erkan K, Unal F, Kiris T. Terminal syringomyelia in association with the tethered cord syndrome. Neurosurgery 1999;45:1351-9.  Back to cited text no. 11
    
12.
Albright AL, Pollack IF, Adelson PD. Normal and abnormal development of the nervous system. Principles and Practice of Pediatric Neurosurgery. 3rd ed., Ch. 2. Stuttgart, New York, Rio. Thieme: Thieme; 2014. p. 10-26.  Back to cited text no. 12
    
13.
Russell NA, Benoit BG, Joaquin AJ. Diastematomyelia in adults. A review. Pediatr Neurosurg 1990;16:252-7.  Back to cited text no. 13
    
14.
Pang D, Dias MS, Ahab-Barmada M. Split cord malformation: Part I: A unified theory of embryogenesis for double spinal cord malformations. Neurosurgery 1992;31:451-80.  Back to cited text no. 14
    
15.
Erşahin Y, Mutluer S, Kocaman S, Demirtaş E. Split spinal cord malformations in children. J Neurosurg 1998;88:57-65.  Back to cited text no. 15
    
16.
Scatliff JH, Hayward R, Armao D, Kwon L. Pre- and post-operative hydromyelia in spinal dysraphism. Pediatr Radiol 2005;35:282-9.  Back to cited text no. 16
    
17.
Jindal A, Mahapatra AK, Kamal R. Spinal dysraphism. Indian J Pediatr 1999;66:697-705.  Back to cited text no. 17
    
18.
Bekki H, Morishita Y, Kawano O, Shiba K, Iwamoto Y. Diastematomyelia: A surgical case with long-term follow-up. Asian Spine J 2015;9:99-102.  Back to cited text no. 18
    
19.
Rufener SL, Ibrahim M, Raybaud CA, Parmar HA. Congenital spine and spinal cord malformations-pictorial review. AJR Am J Roentgenol 2010;194:S26-37.  Back to cited text no. 19
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]



 

Top
 
 
  Search
 
<
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)  

 
  In this article
   Abstract
  Introduction
  Case Reports
  Discussion
   References
   Article Figures

 Article Access Statistics
    Viewed89    
    Printed2    
    Emailed0    
    PDF Downloaded10    
    Comments [Add]    

Recommend this journal