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      Faun tail associated with bony tail like projecting dysplastic sacral vertebral segments in natal cleft: Unique twin tails

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          Abstract

          Dear Sir, A 8-year-old girl delivered at full-term with normal developmental milestones, presented with excessive growth of hair and bony hard protuberance in midline over the lumbosacral region since birth causing difficulty in prolonged sitting with intermittent low backache. Local examination revealed coarse dark terminal hair two inches long, over 12 cm × 14 cm circumscribed region in central lumbosacral region [Figure 1a]. She had exquisite hypersensitivity over patch of hair and rest of examination was normal. Figure 1 (a) Clinical photograph showing faun tail (b) X-ray lateral view of lumbosacral spine of 8-year girl showing bony tail like dorsal projection of dysplastic sacral segments (c) X-ray lumbosacral spine, AP view lateral view of 8-year girl showing absent coccygeal segments (d) magnetic resonance imaging of lumbosacral spine T2-Wi showing low lying conus with dural ectasia X-ray pelvis showed posteriorly directed tip of sacral vertebra resembling bony tail [Figure 1b and c]. Magnetic resonance imaging of spine showed conus lying at L4 vertebral level with hyperacute angulations of posterior directed sacral tip, spina bifida over sacral area with ectatic thecal sac [Figure 1d]. Human tail is classified into five types by Bartel, the first three representing soft tail, fourth bony tail originating from hypertrophy of sacrococcygeal vertebrae and last representing true tail containing additional vertebrae and extends from the coccyx.[1 2] During the 5th–6th week of intrauterine life, human embryo contains tail having 10–12 vertebrae, by 8 weeks it disappears. Tails are usually associated with occult spinal dysraphism. Clinically it needs differentiation from meningomyelocele, teratoma, and chordoma.[3] Faun tail is a wide patch of coarse terminal hair several inches long, distributed over adjoining areas with a large lumbosacral vertebral spinous process and represents cutaneous marker for underlying spinal dysraphism.[3] Surasak et al. observed true tails are midline protrusion attached to the skin of the sacrococcygeal region and usually comprises of normal skin, muscle, nerves, and vessels covered by healthy skin.[4] While pseudo tail is usually short, stump-like and its content may include adipose tissue, or cartilage.[5] As faun tail was her prime cosmetic concern, it planned for pulsed light treatment after undergoing detethering surgery with section of filum terminale for low lying conus with fatty filum.[6] Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

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          The human tail: rare lesion with occult spinal dysraphism--a case report.

          Human tail or tail-like caudal cutaneous appendage is a rare fingerlike, midline protrusion at the lumbosacrococcygeal region, often associated with occult spinal dysraphism. A 2-month-old male child presented here had a lumbosacral tail-like appendage with underlying spinal dysraphism without any appreciable neurological deficit. In contradiction to a previous report, true vestigial tails are not benign because they may be associated with underlying dysraphic state. About 50% of the cases were associated with either meningocele or spina bifida occulta. Management of such lesions must include complete neurological history and examination as well as magnetic resonance or computed tomographic imaging. After diagnosis, microsurgery should be performed if there is any intraspinal component to avoid any damage and neurological deficit.
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            The human tail and spinal dysraphism.

            Recent publications have endeavoured to differentiate between the true, or vestigial tail, and the pseudotail by clinical and pathological examination, and have indicated the benign nature of the true tail. The true tail arises from the most distal remnant of the embryonic tail, contains adipose, connective, muscle, and nerve tissue, and is covered by skin. Pseudotails represent a variety of lesions having in common a lumbosacral protrusion and a superficial resemblance to vestigial tails. A review of the case reports indicates spina bifida to be the most frequent coexisting anomaly with both. A review of occult spinal dysraphism shows it to be associated with cutaneous signs in more than 50% of instances. Three cases of spinal dysraphism with tail-like cutaneous structures are described and their radiological, operative, and pathological findings presented. The classification of each of the appendages into true tail or pseudotail remains obscure. Although the finding of these three tails was the subject of much curiosity, surgical treatment was clearly designed to adequately deal with the associated dysraphic state. The presence of a tail-like appendage in the lumbosacral region should alert the clinician to the possibility of underlying spinal dysraphism. Preoperative assessment must include a complete neurological history and examination as well as computed tomographic or magnetic resonance imaging.
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              Cutaneous signs of spinal dysraphism. Report of a patient with a tail-like lipoma and review of 200 cases in the literature.

              The term "spinal dysraphism" was coined in 1940 by Dr Lichtenstein to designate incomplete fusion or malformations of structures in the dorsal midline of the back, particularly congenital abnormalities of the vertebral column and spinal cord. Raphes develop on the face and head, brancheal arches, sternum, and spinal column. When dysraphism occurs in these sites, failure of closure of fontanelles, cleft lip and palate, brancheal cysts, and abnormalities of the ribs and spine result. A review of 200 cases of occult spinal dysraphism showed the condition to be more common in female patient and to be associated with cutaneous signs in more than 50% of instances. The age at which neurologic symptoms appeared in recorded cases is from birth to 76 years, the average being three years. A case of spinal dysraphism with a tail-like cutaneous structure is presented. The cutaneous manifestations accompanying spinal dysraphism that may lead to early recognition of this syndrome and early institution of treatment are discussed.
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                Author and article information

                Journal
                J Pediatr Neurosci
                J Pediatr Neurosci
                JPN
                Journal of Pediatric Neurosciences
                Medknow Publications & Media Pvt Ltd (India )
                1817-1745
                1998-3948
                Oct-Dec 2015
                : 10
                : 4
                : 411-412
                Affiliations
                [1]Department of Neurosurgery, All Institute of Medical Sciences, New Delhi, India
                Author notes
                Address for correspondence: Dr. Guru Dutta Satyarthee, Department of Neurosurgery, Room No. 714, Neurosciences Center, All India Institute of Medical Sciences, New Delhi - 110 029, India. E-mail: duttaguru2002@ 123456yahoo.com
                Article
                JPN-10-411
                10.4103/1817-1745.174439
                4770666
                26962360
                e7c6d680-6e9a-4176-bfef-d2178132e908
                Copyright: © Journal of Pediatric Neurosciences

                This is an open access article distributed under the terms of the Creative Commons Attribution NonCommercial ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non commercially, as long as the author is credited and the new creations are licensed under the identical terms.

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