|Year : 2008 | Volume
| Issue : 2 | Page : 142-145
The tale of a tail
NK Venkataramana1, Shailesh AV Rao1, Arun L Naik1, Neeraj Awasthy2, H Gupta2, Karamchand Sharma2
1 Department of Neurosurgery, BGS Global Neuroscience Institute, Kengeri, Bangalore, India
2 Department of Pediatrics and Neurosurgery, Vardhaman Mahavir Medical College and Safdarjung Hospital, Delhi, India
N K Venkataramana
Neurosurgeon, Director, BGS Global Neuroscience Institute, Vice Chairman, BGS Global Hospital, No. 67, Uttarahalli Road, Kengeri, Bangalore - 560 060
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Human tail refers to a congenital cutaneous appendage protruding at the lumbosacral region. This interesting anomaly is often associated with occult spinal dysraphism and presents with treatment dilemma. Management of such lesions must include complete neurological examination and magnetic resonance imaging. Appropriate management including early diagnosis and microsurgical intervention can prevent development or progression of severe neurological deficits in later life. In this article, three cases of human tails are reported along with their successful treatment and review of the literature.
Keywords: Human tail, spinal dysraphism
|How to cite this article:|
Venkataramana N K, Rao SA, Naik AL, Awasthy N, Gupta H, Sharma K. The tale of a tail. J Pediatr Neurosci 2008;3:142-5
| Introduction|| |
Human tail is a rare congenital anomaly which, when present in a young child, becomes a cause of immense anxiety and apprehension in parents and other family members. Because of its frequent association with complex intraspinal abnormalities, it also poses a threat to the patient's long-term neurological outcome and a challenge to the treating clinicians. Its poorly understood etiology, confused classification, and debatable management strategies are directly related to the relative rarity with which this abnormality is observed in the normal population. Reports of this uncommon and interesting anomaly have been available since the nineteenth century and more than 100 cases of human tails have been reported in Western literature so far. ,,,,,,,,, However, not much literature is available on this problem in our country. , In this report, we have compiled three case reports on human tails, from two different Indian institutions.
| Case Reports|| |
Human tail - in association with tethered cord and neurogenic bladder dysfunction
A twenty-six-year old male presented to the urological services at our institute with bladder dysfunction. He was evaluated and posted for cystoscopy and sphincter surgery. When a lumbar puncture was performed for spinal anesthesia, he developed sudden weakness of both lower limbs. The surgical procedure was abandoned and a neurosurgical consultation was sought. On examination, the patient was found to have a tail-like structure at the tip of the coccyx, in the midline, which was nontender and soft in consistency [Figure 1A]. MRI revealed the conus ending at S 2 level, with the filum terminale in continuity with the tail like appendage [Figure 1B]. He underwent lumbosacral laminectomy, with releasing of the tethered filum and excision of the tail-like process. Postoperatively, his bladder dysfunction improved significantly.
A 7-month old male child presented with a tail like appendage on the low back since birth which increased minimally in size at par with the general growth of the child. The child did not have leg weakness or incontinence of urine. Examination revealed a tail like appendage measuring 3 inches in length covered by normal skin with a dimple at its tip and no bony or cartilage structures within it [Figure 2A] and [Figure 2B]. A dimple was also noted below the appendage in the sacral area [Figure 2B]. MRI scan revealed no intraspinal abnormality except for tapering filum towards the dimple. At surgery, a fibrous track was observed extending from the tail like appendage to the extradural plane [Figure 2C]. There was no intradural abnormality and the appendage was excised completely. The filum was released and the wound was closed. Child recovered without any neurological deficits.
Human tail - An unusual case
A three-year-old healthy female child presented with an elongated tail like appendage since birth. On examination, there was 10 cm x 4 cm soft and non-fluctuant appendage, which was covered with normal skin [Figure 3A] and [Figure 3B]. There were no bony or cartilaginous elements in the appendage. There was no urinary or bowel incontinence and the child did not have any neurological deficit. There was no other congenital anomaly, except for bilateral congenital talipes eqino varus, without any paraperesis. Anal tone was normal.
An X-ray of the lumbosacral spine revealed spinal dysraphism in the form of spina bifida extending from L4 to S1 vertebral levels. An MRI scan showed that along with spina bifida, there was soft tissue in lumbosacral region having lipomatous component, extending from L2 to the sacral level. The spinal cord was dorsally displaced, could be traced up to L5-S1 (low lying) and was tethered to the lipomatous tissue. There was exophytic lipomatous mass in the sacral region. Neural tissue was seen extending through the spinal defect into subarachnoid space and also on the superior aspect (Lipomyelocele) in the sacral region [Figure 3C].
Our patient was treated with microsurgical resection of the intraspinal lipomatous tissue, untethering of the cord, and excision of the tail-like appendage. There was no neurological deficit postoperatively and the operative wound healed well.
| Discussion|| |
Considerable confusion exists in the terminology of these anomalies. The tail-like appendage could be either a true (vestigial) tail or a pseudotail. A true tail, by definition, is the most distal remnant of the embryonic tail that contains adipose and connective tissue, striated muscle, blood vessels and nerves, and is covered by normal skin.  It is in fact a vestigial structure and is present as a result of abnormal development and regression of the human tail. Pseudotail, on the other hand, contains bone, cartilage, notochord, and elements of the spinal cord. The most common cause of pseudotail is the protrusion of coccygeal vertebrae in the lumbosacral area. They contribute to what is known as "tail-like appendages".
Human tail is an infrequent malformation resulting from disturbance of caudal regression, around the sixth week of embryonal life. ,, The embryogenesis of this malformation is not very clear, but it is usually associated with spinal abnormalities. A lack of awareness about associated anomalies in a patient with a tail-like appendage may prevent accurate diagnosis and delay appropriate management of the condition. 
Association of spina bifida, tethered cord syndrome and intraspinal lipomas are frequently reported. ,,, There are several reports suggesting its association with intra spinal lipomas , and lipomyelomeningoceles , leading to tethering, almost indicating that the tail is a cutaneous marker, similar to the tuft of hair that is associated with split cord malformations.
Spinal dysraphism is an anomaly that is most frequently associated with human tail. Other congenital anomalies include lipomyelomeningocele, tethered cord, lumbosacral teratoma, terminal myelocystocele, and coccygeal vertebrae. According to Hoffman et al. ,  fatty mass in the lumbosacral region is the most common presenting feature in a series of 97 children, who had tail-like appendages. There are also reports wherein the spinal cord is in continuity with the tail and the filum is directly attached to the tail, causing tethering. ,,, Other associated anomalies like club feet have been reported in only 1.5-2.0% of reported cases of human tail. 
Magnetic resonance (MR) imaging is the modality of choice for diagnosis of a tail-like appendage. All such cases must be investigated with neuroimaging for spinal dysraphism, because of its frequent association with the human tail.  Spinal dysraphism is the main factor affecting the outcome and overall prognosis in these patients. MRI is also essential to plan surgical strategies.
All human tails are surgically correctable anomalies. Surgery is aimed at untethering the spinal cord as the first step, followed by complete excision of the tail. Human tail is a benign congenital anomaly but its associated underlying spinal lesions, if not recognized soon, can leave the affected child with permanent neurological disabilities. Early identification and prompt surgical treatment can avert these disastrous consequences.
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[Figure 1A], [Figure 1B], [Figure 2A], [Figure 2B], [Figure 2C], [Figure 3A], [Figure 3B], [Figure 3C]
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