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| CASE REPORT |
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| Year : 2022 | Volume
: 9
| Issue : 2 | Page : 125-127 |
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A case report of a rare entity: Double dermal sinus with double intradural dermoid
Shanmugam Muthiah1, Vijay Kumar Sivaramamoorthy1, Muthukumar Natrajan2
1 Department of Neurosurgery, Neuro One Hospital, Trichy, Tamil Nadu, India
2 Department of Neurosurgery, Devadoss Multispeciality Hospital, Madurai, Tamil Nadu, India
| Date of Submission | 27-Nov-2021 |
| Date of Acceptance | 09-Jan-2022 |
| Date of Web Publication | 31-May-2022 |
Correspondence Address:
Shanmugam Muthiah
Department of Neurosurgery, Neuro One Hospital, Karur Bye Pass Road, Trichy - 620 002, Tamil Nadu
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/joss.joss_27_21
Congenital spinal dermal sinus tract is a rare entity and is usually solitary. Multiple dermal sinus tracts in a child are extremely rare; so far, only five cases have been published in the English literature to our knowledge. We report a case of double dermal sinus tracts with double intradural dermoid, and such a combination has not been reported. Meticulous excision of both dermal sinus tracts and intradural dermoid resulted in complete recovery. We present this rare entity and review the literature.
Keywords: Congenital spinal dermal sinus, double dermoid inclusion tumors, multiple dermal sinus tracts
How to cite this article:
Muthiah S, Sivaramamoorthy VK, Natrajan M. A case report of a rare entity: Double dermal sinus with double intradural dermoid. J Spinal Surg 2022;9:125-7 |
How to cite this URL:
Muthiah S, Sivaramamoorthy VK, Natrajan M. A case report of a rare entity: Double dermal sinus with double intradural dermoid. J Spinal Surg [serial online] 2022 [cited 2022 Jul 7];9:125-7. Available from: http://www.jossworld.org/text.asp?2022/9/2/125/346360 |
| Introduction | |  |
Congenital spinal dermal sinus is a rare entity seen in childhood with skin findings and neurological deficits.[1] These tracts terminate intradurally as dermoid, epidermoid, or teratoma. They often present as solitary lesions; multiple dermal sinuses (DSs) in a child reported in the literature are extremely rare with only five cases (excluding the present case) till date. After extensive search of the literature, we found only one case of double dermal sinus tracts at the rarer locations of the cervical and thoracic spine. We present the second case report of congenital dermal sinus tract occurring at two levels (cervical and thoracic). Our case is the first with double dermal sinus tracts with double intradural dermoid tumors.
| Case Report | | |
History
A 1½-year-old child presented with acute onset of difficulty in walking for 3 days with intermittent serous discharge from his back through a sinus. The sinus had been noticed at birth, but the discharge was noticed recently. Weakness was preceded by low-grade fever. There was no bowel/bladder involvement.
Clinical examination
The child had spastic paraplegia (power – grade 3/5) and sensory deficit below D10 level. He had two dermal sinus tracts, one high cervical level and another at D8–D9 [Figure 1]a. Hairy nevus was seen in the cervical dermal sinus tract [Figure 1]b. The dorsal dermal sinus tract was infected and had exuding serous discharge. There was no scoliosis or kyphosis. There were no signs of meningitis. | Figure 1: (a and b) Clinical photographs of the child showing double dermal dimple at cervical and lower dorsal spine levels and at the cervical level, a small hairy nevus seen
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Imaging
Magnetic resonance imaging (MRI) of the spine showed evidence of double dermal sinus tracts, one at D8–D9 level extending rostrally to end in an intramedullary dermoid, the margins of which enhanced with contrast [Figure 2]. MRI also showed a cervical dermal sinus tract at C2–C3 level which ended in an intradural extramedullary dermoid [Figure 3]. There was no block vertebra or scoliosis. | Figure 2: (a and b) Magnetic resonance imaging sagittal/axial images show double sinus tract with cervical (C2–C3) intradural mass and thoracic (D8–D9) intramedullary mass with enhancing wall
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Surgery
Operation was performed in two stages. In the first stage, after midline vertical skin incision and D8–D9 laminectomy, the sinus tract was carefully traced to the dura and excised. The intramedullary dermoid with hair and pus was completely excised through midline myelotomy. Postoperatively, the child had improvement in motor and sensory function.
Fifteen days later, the cervical dermal sinus was operated upon. The lamina and spinous processes of C1–C3 were aplastic. The sinus tract was followed into the dura. Intradural extramedullary dermoid was excised totally [Figure 4]a and [Figure 4]b. The child had complete return of sensory function and improved to grade 4+ power in the legs. He could walk with minimal support at the time of discharge. Postoperative cerebrospinal fluid leak after the second surgery was successfully managed.
Histopathology of both the specimens was consistent with dermoid cyst with no evidence of malignancy.
| Discussion | | |
Congenital dermal sinus occurs in one in 2500 live births and is most commonly located in the lumbosacral (75%) and rarely in the thoracic (<10%) or cervical region (<1%).[1],[2],[3],[4] They often present as a solitary midline lesion.[4] Mostly, the tracts end in dermoid (85%) and epidermoid (13%). Teratoma or malignant transformations are rare.
Multiple DSs are rare, and so far, only five cases have been reported. Among the five cases, there was one case of double cervical sinus tracts,[6] one of the double lateral retro-auricular sinus tracts,[7] one of the double occipital sinus tracts in the posterior fossa,[8] one of the double sinus tracts in the cervical and thoracic spine,[4] and one case of triple dermal sinus tracts.[5] The double DSs located in the cervical and thoracic regions have been reported only by Mrowczynski et al.[4] Here, we present a case of double dermal sinus tracts at cervical and thoracic levels. Our case is unique, as this is the first case of double dermal sinus tracts with double intradural dermoid, one being intramedullary and one being intradural extramedullary.
Clinical discussion
The failure of the neuroectoderm to separate from the cutaneous ectoderm between the third and eighth weeks of gestation is the embryological cause of these dermal sinus tracts.[1],[2],[3],[4] At birth, these children are seen neurologically intact, and later, they become symptomatic due to neural compression either from inclusion tumor or by tethering of cord, vascular compromise, or chronic arachnoiditis.[2] Ascending infection through the dermal sinus tract results in meningitis. Surgical excision of the tract and underlying cyst/tumor results in excellent prognosis[1],[2] as in our case.
Embryological discussion
A congenital dermal sinus is a tract lined by stratified squamous epithelium found on or near the midline and is thought to result from the abnormal adhesions (or incomplete disjunction) between the neuroectoderm and the cutaneous ectoderm.[9] The tract elongates during the development, due to ascent of the cord and may traverse several levels within the epidural space before entering the subarachnoid space. Disorder of the notochord formation with sagittal splitting of the spinal cord and persistence of the dorsal cutaneo-endo-mesenchymal fistula has also been suggested as a cause of dermal sinus formation.[10] SCDS may be associated with other abnormalities of the ectodermal, mesodermal or neural crest derivatives such as meningomyelocele or lipomeningomyelocele, reflecting a common ontogenic disorder.[9]
| Conclusion | | |
This case report highlights the rarity of double dermal sinus tracts, both being associated with dermoid and the importance of early surgical intervention in preventing neurological deficit and meningitis.
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 | | |
| 1. | Mete M, Umur AS, Duransoy YK, Barutçuoğlu M, Umur N, Gurgen SG, et al. Congenital dermal sinus tract of the spine: Experience of 16 patients. J Child Neurol 2014;29:1277-82.  |
| 2. | Kanev PM, Park TS. Dermoids and dermal sinus tracts of the spine. Neurosurg Clin N Am 1995;6:359-66. |
| 3. | Ackerman LL, Menezes AH. Spinal congenital dermal sinuses: A 30-year experience. Pediatrics 2003;112:641-7. |
| 4. | Mrowczynski OD, Lane JR, Shoja MM, Specht CS, Langan ST, Rizk EB. Double dermal sinus tracts of the cervical and thoracic regions: A case in a 3-year-old child and review of the literature. Childs Nerv Syst 2018;34:987-90. |
| 5. | Ansari S, Andrabi Y, El Khashab M, Bateni F, Dadmehr M, Iskandar BJ, et al. Triple lumbar dermal sinuses: Unusual presentation of a typically solitary midline lesion. Pediatr Neurosurg 2009;45:305-7. |
| 6. | El Khashab M, Nejat F, Ertiaei A. Double dermal sinuses: A case study. J Med Case Rep 2008;2:281. |
| 7. | Nejat F, Dias MS, Eftekhar B, Roodsari NN, Hamidi S. Bilateral retro-auricular dermal sinus tracts with intradural extension. Case report. J Neurosurg 2003;99:163-6. |
| 8. | Metre MG, Lakhdar F, Ndekha GJ, Benzagmout M, Chakour K, Chaoui F. Double paramedian occipital dermal sinus revealing an infected intra-dural dermoid cyst of the posterior cerebral fossa: Case report. Interdiscip Neurosurg 2018;14:28-31. |
| 9. | Jindal A, Mahapatra A K. Spinal congenital dermal sinus : an experience of 23 cases over 7 years. Neurol India 2001;49:243 |
| 10. | Pang D. Split cord malformation: Part II: Clinical syndrome. Neurosurgery. 1992 Sep;31(3):481-500 |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
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