The longest faun tail forming dreadlocks with underlying spina bifida occulta
Balvinder Kaur Brar MBBS MD, Bharat Bhushan Mahajan MBBS MD, Jyotisterna Mittal MBBS MD
Dermatology Online Journal 19 (4): 12

GGS Medical College, Faridkot, Punjab, India

Abstract

Spina bifida is a developmental anomaly characterized by defective closure of the bony encasement of the spinal cord through which the spinal cord and meninges may or may not protrude. We report a rare case of a very long faun tail, which was in the form of a 20 inch long tail originating from the lumbosacral area in a rhomboidal pattern, measuring 10 x 8 inches. The case is being reported for its rare presentation of a 20 inch long faun tail with underlying spina bifida occulta.



Introduction

Spinal dysraphism refers to a spectrum of congenital anomalies characterized by an incomplete fusion of the midline mesenchymal, bony, or neural elements of the spine through which the spinal cord and meninges may or may not protrude [1]. Spina bifida occulta refers to the failure of complete midline fusion of vertebral arches with no visible exposure of meninges or neural tissue [2]. A faun tail is abnormal lumbar hypertrichosis that is characterized by a wide, often triangular or lozenge-shaped patch of coarse hair, usually several inches long [3]. Faun tail itself is a rare entity. Whereas such a large faun tail has not been reported in the literature to the best of our knowledge, only four cases with faun tail have been reported in association with spina bifida occulta, mostly in the Korean literature [4, 5, 6, 7]. The earliest case reported from India is in 1994 [8].


Case report


Figure 1 Figure 2
Figure 1. 20 inches long faun tail arising from lumbosacral area with hair forming mats

Figure 2. CT scan showing non-fusion of lamina of the spinal cord at the level of faun tail.

A 13-year-old female child with normal intelligence presented with an asymptomatic tail of hair arising from the midline on the lower back. Dermatological examination revealed a tuft of coarse, light brown colored terminal hair arising from the lumbosacral region in a rhomboid pattern, with dimensions measuring 10 x 8 inches and about 20 inches in length forming mats (Figure 1). There was no loss of sensation or paralysis below the level of the lesion. There was no family history. She was a product of non-consanguinous marriage, normal gestation, and uneventful vaginal delivery. Routine investigations were normal. CT scan was done to detect any underlying anomalies and depicted an underlying isolated bony defect without any neurological component (Figure 2). The diagnosis of spina bifida occulta with faun tail was confirmed. The patient was advised to undergo laser hair reduction treatment or shaving of the area for psychological and cosmetic purposes. The case is being reported for its rare presentation of a matted faun tail with underlying spina bifida occulta.


Discussion

Spina bifida is one of the most common birth defects, with an average worldwide incidence of 1–2 cases per 1000 births. Spina bifida malformations fall into four categories: spina bifida occulta, spina bifida cystica (myelomeningocele), meningocele, and lipomeningocele. The most common locations of the malformations are the lumbar and sacral areas.

Occulta means hidden and the defect is not visible. Spina bifida occulta is rarely linked with complications or symptoms and is usually discovered accidentally on radiography. A midline cutaneous posterior anomaly such as a dimple, tuft of hair, sinus, hemangioma, hyperpigmentation, lipoma, or scar is often a clue for an underlying occult spinal dysraphism. Sacral hypertrichosis (faun tail nevus) is the most common skin lesion evident at birth [9], as was seen in our patient. Such a cosmetic deformity may lead to a considerable psychological stress especially in a young patient. The prevalence of the occulta type is not known, but it is probably the most common type of spina bifida. Although this patient demonstrated no underlying neurologic problem associated with her localized hypertrichosis, accurate screening modalities such as MRI are necessary to detect any underlying anomalies and to prevent the occurrence or progression of the neurologic deficit by timely intervention.

There is neither a single cause of spina bifida nor any known way to prevent it entirely. All women of childbearing age who are capable of becoming pregnant should consume 0.4 mg of folic acid per day from 3 months before conception to the first 12 weeks of pregnancy. As for the management of the hypertrichosis, for the long-term removal of unwanted hair, the currently available treatment methods include bleaching, trimming, shaving, waxing, physical and chemical depilatories, electrolysis, intense pulsed light therapy, and laser hair removal [10].

References

1. Guggisberg D, Hadj-Rabia S, Viney C, Bodemer C, Brunelle F, Zerah M. Skin Markers of Occult Spinal Dysraphism in Children: A Review of 54 Cases. Arch Dermatol 2004;140:1109-1115. [PubMed]

2. Harris HW, Miller OF. Midline cutaneous and spinal defects. Arch Dermatol 1976;112:1724-1728. [PubMed]

3. Lee HI, Rho YK, Kim BJ, Kim MN. A Case of Faun Tail Naevus Treated by Intense Pulsed Light. Ann Dermatol 2009; 21(2): 147-149. [PubMed]

4. Kim SW. Faun tail. Ann Dermatol 1993; 5: 47-50.

5. Bak H, Kim JY, Chung YL, Kim SC. A case of faun tail associated with tethered cord syndrome. Korean J Dermatol 2004; 42:781-783.

6. Choi JS, Lee WH, Lee JB. Circumscribed hypertrichosis and blue nevus with spina bifida occulta. Korean J Dermatol 1981;19:365-369.

7. Suhr GB, Lee JS, Lee JH, Park JK. A case of spina bifida occulta with faun-tail nevus. Korean J Dermatol 1988; 26:759-763.

8. Dhar S. Fauntail naevus. Indian J Dermatol Venereol Leprol 1994;60:47-48.

9. Tavafoghi V, Ghandchi A, Hambrick GW, Udverllelyi GB. Cutaneous signs of spinal dysraphism. Report of a patient with a tail like lipoma and review of 200 cases in the literature. Arch Derm 1978; 114: 573-577. [PubMed]

10. Trueb RM. Causes and management of hypertrichosis. Am J Clin Dermatol 2002; 3:617-627. [PubMed]

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Title:

The longest faun tail forming dreadlocks with underlying spina bifida occulta

Journal Issue:

Dermatology Online Journal, 19(4)

Author:

Brar, Balvinder Kaur;
Mahajan, Bharat Bhushan;
Mittal, Jyotisterna

Publication Date:

2013

Publication Info:

Dermatology Online Journal, UC Davis

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