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CORRESPONDENCE
Year : 2021  |  Volume : 66  |  Issue : 3  |  Page : 322-324
An unusual case of faun tail nevus with aplasia cutis, dermo-fascial sinus defect, diastematomyelia, and spinal cord syrinx


From the Department of Dermatology, Venereology & Leprosy, Dayanand Medical College, Ludhiana, Punjab 141001, India

Date of Web Publication13-Jul-2021

Correspondence Address:
Jaspriya Sandhu
From the Department of Dermatology, Venereology & Leprosy, Dayanand Medical College, Ludhiana, Punjab 141001
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_816_19

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How to cite this article:
Sandhu J, Gupta SK, Katha M. An unusual case of faun tail nevus with aplasia cutis, dermo-fascial sinus defect, diastematomyelia, and spinal cord syrinx. Indian J Dermatol 2021;66:322-4

How to cite this URL:
Sandhu J, Gupta SK, Katha M. An unusual case of faun tail nevus with aplasia cutis, dermo-fascial sinus defect, diastematomyelia, and spinal cord syrinx. Indian J Dermatol [serial online] 2021 [cited 2021 Jul 27];66:322-4. Available from: https://www.e-ijd.org/text.asp?2021/66/3/322/321329




Sir,

Faun tail nevus (FTN) is an uncommon congenital nevus characterized by circumscribed hypertrichosis usually present in the lumbosacral region, often a marker of underlying spinal dysraphism. The word faun tail is derived from the Roman mythological creature Faun, half human-half goat, believed to be a symbol of fertility. The spinal dysraphism is due to faulty closure of the neural tube during secondary neurulation occurring in the first trimester. Other markers include skin dimple, hemangioma, subcutaneous lipoma, and vestigial appendage. Spinal dysraphism occurring with cutaneous markers have been reported by various authors to vary from 50% to 95%.[1]

FTN has been reported to be associated with neurofibroma in a 6-year-old girl by Das et al.[2] Chander et al. have reported aplasia cutis congenita with diastematomyelia, tethered conus, asymptomatic spina bifida occulta with no neurological involvement.[3] FTN with diastematomyelia, cutaneous hemangioma has been reported in a 10-year-old girl with urinary incontinence.[4] We present here, a case of a 25-year-old male who presented to our outpatient department with an FTN associated with aplasia cutis along with a constellation of spinal cord abnormalities which have previously not been reported.

A 25-year-old male presented to us in the outpatient department with complaints of excessive hair on the lower back for which he wanted LASER hair reduction (LHR) treatment. He had been shaving the hair regularly, as he felt the growth unsightly. Since there were no symptoms of any kind, he had never sought medical attention for the same. A small bald patch at the center was present since birth.

On examination, a rhomboid tuft of coarse terminal hair (recently shaved) was present over the lumbosacral region; the center had aplasia cutis, an ovoid, atrophic, skin-colored plaque [Figure 1]. A thorough neurological exam revealed no focal neurological deficit. A clinical diagnosis of FTN with aplasia cutis was made based on history and clinical examination.
Figure 1: A rhomboid tuft of coarse terminal hair (recently shaved) was present over the lumbosacral region; the center had an ovoid, atrophic, skin-colored plaque of aplasia cutis

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Magnetic resonance imaging was done for the lumbosacral spine with serial sections. In addition, the entire spine was scanned for any other defect along its length. A linear T2 hypointense tract was seen extending from the skin and coursing oblique in subcutaneous fat, piercing the dorsolumbar fascia at L1 level with no intraspinal extension [Figure 2]. There was evidence of sagittal clefting of spinal cord into two hemi-cords at T12 level suggestive of diastematomyelia (Pang type II) and linear T2 hyperintense signal was seen in the T11-12 level cord suggestive of syrinx [Figure 3]. A neurosurgical consultation was taken and the patient was thoroughly evaluated by the neurosurgical team. However, being an asymptomatic condition, no specific treatment was advised for the syrinx and diastematomyelia.
Figure 2: Hypointense tract extending from the skin and coursing obliquely in subcutaneous tissue, piercing the lumbosacral facia at L1 level

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Figure 3: Diastematomyelia (Pang type II) was at T12 level (small arrow). Spinal cord syrinx seen at T11-T12 (large arrow)

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The patient underwent four sessions of LHR with Diode LASER (810 nm), with significant improvement.

FTN is a rare marker of spinal dysraphism. This abnormal tuft of hair may be present in the three forms:[1]

  1. Simple nevoid hypertrichosis: The tuft of hair is present away from the midline, usually benign
  2. Silky down: Soft, downy nonterminal hair present at the midline
  3. Faun tail: A broad patch of coarse, long, terminal hair present at the midline.


FTN may be associated with a number of spinal cord abnormalities; in this case, it was associated with diastematomyelia. Diastematomyelia is a congenital longitudinal split of the spinal cord. Russel et al. had previously reported that in 20 adult patients with diastematomyelia, 15 had associated FTN.[5] Diastematomyelia is asymptomatic in infancy and childhood, whereas several complications develop (i.e., pain, sensorimotor defects or sphincteric abnormalities) during adolescence/adulthood, at times requiring a surgical intervention.

Our patient also had an associated syrinx (T11-T12 level). A spinal cord syrinx is a rare fluid-filled neuroglial cavity within the spinal cord. This has been previously reported in a young girl with an FTN from India, but in that case, the child was paraplegic.[6]

Our patient had no neurological deficit and was therefore only treated for the hypertrichosis; Diode LASER was recommended which is the routine LHR device used in our center.

To the best of our knowledge, there have been no cases of FTN reported with aplasia cutis, dermo-fascial defect, diastematomyelia, and spinal cord syrinx in combination. The unusual features in this case were the presence of a constellation of spinal cord abnormalities without any associated neurological deficit.

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.
Scatliff JH, Kendall BE, Kingsley DP, Britton J, Grant DN, Hayward RD. Closed spinal dysraphism: Analysis of clinical, radiological, and surgical findings in 104 consecutive patients. AJR Am J Roentgenol 1989;152:1049-57.  Back to cited text no. 1
    
2.
Das A, Das N, Podder I, Biswas S. Faun tail nevus with neurofibroma: An uncommon cocktail…. Indian J PaediatrDermatol 2015;16:84-6.  Back to cited text no. 2
    
3.
Chander R, Jain A, Jaykar K, Garg T, Anand R. Faun tail nevus with aplasia cutis congenita. Pediatr Dermatol 2009;26:484-5.  Back to cited text no. 3
    
4.
Polat M, Polat F, Oztaş P, Kaya C, Alli N. Faun tail: A rare cutaneous marker of spinal dysraphism. Skinmed 2010;8:181-3.  Back to cited text no. 4
    
5.
Russell NA, Benoit BG, Joaquin AJ. Diastematomyelia in adults. A review. Pediatr Neurosurg 1990-1991;16:252-7.  Back to cited text no. 5
    
6.
Arora S, Rawat S, Banerjee A. Faun tail nevus - A case report. Med J Armed Forces India 2006;62:286-7.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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