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The human tail: rare lesion with occult spinal dysraphism—a case report
Journal of Pediatric Surgery (2008) 43, E41–E43
www.elsevier.com/locate/jpedsurg
The human tail: rare lesion with occult spinal dysraphism—a case report Deepak Kumar Singh a,⁎, Basant Kumar b , V.D. Sinha a , H.R. Bagaria a a
Department of Neurosurgery, S.M.S. Medical College, Jaipur, Rajasthan 302004, India Department of Pediatric Surgery (SPMCHI), S.M.S. Medical College, Jaipur, Rajasthan 302004, India
b
Received 27 March 2008; revised 18 April 2008; accepted 18 April 2008
Key words: Human tail; Occult spinal dysraphism; Cutaneous appendage
Abstract 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. © 2008 Elsevier Inc. All rights reserved.
Skin lesions associated with spinal dysraphism are a welldocumented finding. A “vestigial tail” describes a remnant of a structure found in embryonic life or in ancestral forms [1]. A skin appendage resembling tail in a human is a very rare condition and is usually consist of an underlying lipoma or lipomyelomeningocele [2]. This article will discuss our experience with a case of human tail with underlying lipomyelomeningocele and review the literature.
1. Case report A 2-month-old male child born after an uncomplicated full-term pregnancy, weighing 3.8 kg, was admitted in our institute with an elongated, midline, lumbosacral, cutaneous
⁎ Corresponding author. Tel.: +91 9352536809 (Mobile). E-mail address: [email protected] (D.K. Singh). 0022-3468/$ – see front matter © 2008 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2008.04.030
growth resembling a tail apparent since birth. He was the first offspring of a 32-year-old healthy mother. The baby had normal neurological examination without any deficit in both lower limbs, but some weakness in anal sphincter tone was appreciated. Local examination revealed a skin-covered, soft, nontender, nonfluctuating appendage 2.2 × 7.5 cm in diameter located in the midline of the lumbosacral region, appearing like a human tail (Fig. 1). Plain x-ray of the spine demonstrated normal bony structures. Magnetic resonance imaging (MRI) of the spine showed spinal dysraphism in the form of spina bifida at L5 and S1 vertebrae with lipomeningomyelocele and low-lying tethered spinal cord with prominent central canal of lower spinal cord (Figs. 2 and 3). Nerve conduction studies were suggestive of demyelination and axonal sensory motor polyneuropathy. During operation, a midline vertical incision was given encircling the base of appendage. The subcutaneous lipoma was excised along with its intradural components. The dural
E42
D.K. Singh et al.
Fig. 1
Lumbar tail measuring 2.2 × 7.5 cm.
opening was extended caudally, and detethering of filum terminale was done. The dura was closed in watertight fashion using fascial graft along with layered closure of
Fig. 3 The MRI T2 sagittal image showing lipomeningomyelocele (short arrow) with low-lying tethered cord (long arrow).
surgical wound according to standard protocol. Microsurgical techniques were applied during the whole procedure. Microscopic examination showed that the resected specimen had mature adipose tissue with occasional nerves.
2. Discussion
Fig. 2 The MRI T2 coronal image showing lipomeningomyelocele (lipomatous component passing through dura marked with arrow head) with dilated lower central canal (white arrow).
The human tail is an ill-defined, rare, fingerlike, midline, interesting anomaly and very difficult to classify as either a true (vestigial) tail or pseudotail [2,3]. Dao and Netsky [4] described the features to distinguish the true tails from the pseudotails. True tail contains adipose and connective tissue, striated muscle, blood vessels, and nerves and is covered by skin. Bone, cartilage, notochord, and spinal cord are lacking. It may be as long as 13 cm and can move and contract. The pseudotail is an anomalous prolongation of the coccygeal vertebra, lipoma, teratoma, condrodystrophy, or parasitic fetus. The true tail is the distal remnant of the embryologic tail, which is present between the fourth and seventh or eighth weeks of fetal development [5,6]. Harrison [6] suggested that vestigial tail arise from the distal unvertebrated mesodermal portion of the embryonic tail. There are only 66 cases that have been reported in modern literature [2]. Our case was associated with spina bifida with lipomeningomyelocele and tethered spinal cord. There are only 5
The human tail cases of human tail and intraspinal lipoma causing tethering of spinal cord reported in the literature [5]. Reviews of case reports indicate spina bifida to be the most frequent coexisting anomaly with both true tail and pseudotail [2,4]. Tavaghi et al [7] reviewed 200 cases of occult spinal dysraphism and showed it to be associated with cutaneous signs in more than 50% cases, and 41 cases had subcutaneous lipoma associated with spina bifida [1,5,7]. They reported only 1 case of spinal dysraphism with tail-like cutaneous structure consisting cutaneous and intrathecal lipoma [7]. In the case of spinal dysraphism with associated tail-like caudal appendages, the tail consists of underlying spinal lipoma or lipomyelomeningocele [1,2]. There have been 14 cases of such tail-like appendages with spinal dysraphism reported in the literature [1]. In contradiction to a previous report by Spiegelmann et al [3], true vestigial tails are not benign because they may be associated with underlying dysraphic state. According to previous a series of 48 skin-covered lumbosacral masses, 67% of cases were lipomas and lipomyelomeningocele, 25% lumbosacral and sacrococcygeal teratomas, and 4% meningocele and 4% terminal myelocystoceles [8]. Hoffman et al [9] had described that fatty mass in the lumbosacral region is the most common presenting complaint in a series of 97 patients, and 5 patients presented with tail-like caudal appendages. Lu et al [10] noted 59 cases with caudal appendages, and 50 % of the cases were associated with either meningocele or spina bifida occulta [10]. The distinction between the true tail and pseudotail on clinical examination is almost always dubious, and despite a
E43 normal neurological examination, normal plain x-ray of spine, and clinical classification of true tail, subfascial exploration may show tethered cord or lipomeningomyelocele as what occurred in our case. Hence, ignoring the type of tail, each individual case needs comprehensive preoperative investigation, and surgical exploration of the intraspinal content should be done microsurgically to avoid any damage or neurological deficit.
References [1] Belzberg AJ, Myles ST, Trevenen CL. The human tail and spinal dysraphism. J Pediatr Surg 1991;26:1243-5. [2] Chakraborty S, Shizuo O, Yoshida Y, et al. Myelomeningocele and thick filum terminale with tethered cord appearing as a human tail. J Neurosurg 1993;78:966-9. [3] Spiegelmann R, Schinder E, Mintz M, et al. The human tail: a benign stigma. J Neurosurg 1985;63:461. [4] Dao AH, Netsky MG. Human tails and pseudotails. Hum Pathol 1984; 15:449-53. [5] Feridun A, Ersin E, Muzeyyen G, et al. Human tail. A rare lesion and possible marker of intraspinal lipoma in adolescent and pediatric cases. WSJ 2006;1(2):84-7. [6] Harrison RG. On the occurrence of tail in man. Johns Hopkins Hosp Bull 1901;12:96-101. [7] Tavaghi G, Ghandchi A, Hambrick G, et al. Cutaneous signs of spinal dysraphism. Arch Dermatol 1978;114:573. [8] McLone DG, Naidich TP. Terminal myelocystocele. Neurosurgery 1985;16:36-43. [9] Hoffman HJ, Taecholarn C, Hendrick EB, et al. Management of lipomyelomeningoceles. J Neurosurg 1985;62:1-8. [10] Lu FL, Wang PJ, Teng RJ. The human tail. Pediatr Neurol 1988;1988: 230-3.
www.elsevier.com/locate/jpedsurg
The human tail: rare lesion with occult spinal dysraphism—a case report Deepak Kumar Singh a,⁎, Basant Kumar b , V.D. Sinha a , H.R. Bagaria a a
Department of Neurosurgery, S.M.S. Medical College, Jaipur, Rajasthan 302004, India Department of Pediatric Surgery (SPMCHI), S.M.S. Medical College, Jaipur, Rajasthan 302004, India
b
Received 27 March 2008; revised 18 April 2008; accepted 18 April 2008
Key words: Human tail; Occult spinal dysraphism; Cutaneous appendage
Abstract 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. © 2008 Elsevier Inc. All rights reserved.
Skin lesions associated with spinal dysraphism are a welldocumented finding. A “vestigial tail” describes a remnant of a structure found in embryonic life or in ancestral forms [1]. A skin appendage resembling tail in a human is a very rare condition and is usually consist of an underlying lipoma or lipomyelomeningocele [2]. This article will discuss our experience with a case of human tail with underlying lipomyelomeningocele and review the literature.
1. Case report A 2-month-old male child born after an uncomplicated full-term pregnancy, weighing 3.8 kg, was admitted in our institute with an elongated, midline, lumbosacral, cutaneous
⁎ Corresponding author. Tel.: +91 9352536809 (Mobile). E-mail address: [email protected] (D.K. Singh). 0022-3468/$ – see front matter © 2008 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2008.04.030
growth resembling a tail apparent since birth. He was the first offspring of a 32-year-old healthy mother. The baby had normal neurological examination without any deficit in both lower limbs, but some weakness in anal sphincter tone was appreciated. Local examination revealed a skin-covered, soft, nontender, nonfluctuating appendage 2.2 × 7.5 cm in diameter located in the midline of the lumbosacral region, appearing like a human tail (Fig. 1). Plain x-ray of the spine demonstrated normal bony structures. Magnetic resonance imaging (MRI) of the spine showed spinal dysraphism in the form of spina bifida at L5 and S1 vertebrae with lipomeningomyelocele and low-lying tethered spinal cord with prominent central canal of lower spinal cord (Figs. 2 and 3). Nerve conduction studies were suggestive of demyelination and axonal sensory motor polyneuropathy. During operation, a midline vertical incision was given encircling the base of appendage. The subcutaneous lipoma was excised along with its intradural components. The dural
E42
D.K. Singh et al.
Fig. 1
Lumbar tail measuring 2.2 × 7.5 cm.
opening was extended caudally, and detethering of filum terminale was done. The dura was closed in watertight fashion using fascial graft along with layered closure of
Fig. 3 The MRI T2 sagittal image showing lipomeningomyelocele (short arrow) with low-lying tethered cord (long arrow).
surgical wound according to standard protocol. Microsurgical techniques were applied during the whole procedure. Microscopic examination showed that the resected specimen had mature adipose tissue with occasional nerves.
2. Discussion
Fig. 2 The MRI T2 coronal image showing lipomeningomyelocele (lipomatous component passing through dura marked with arrow head) with dilated lower central canal (white arrow).
The human tail is an ill-defined, rare, fingerlike, midline, interesting anomaly and very difficult to classify as either a true (vestigial) tail or pseudotail [2,3]. Dao and Netsky [4] described the features to distinguish the true tails from the pseudotails. True tail contains adipose and connective tissue, striated muscle, blood vessels, and nerves and is covered by skin. Bone, cartilage, notochord, and spinal cord are lacking. It may be as long as 13 cm and can move and contract. The pseudotail is an anomalous prolongation of the coccygeal vertebra, lipoma, teratoma, condrodystrophy, or parasitic fetus. The true tail is the distal remnant of the embryologic tail, which is present between the fourth and seventh or eighth weeks of fetal development [5,6]. Harrison [6] suggested that vestigial tail arise from the distal unvertebrated mesodermal portion of the embryonic tail. There are only 66 cases that have been reported in modern literature [2]. Our case was associated with spina bifida with lipomeningomyelocele and tethered spinal cord. There are only 5
The human tail cases of human tail and intraspinal lipoma causing tethering of spinal cord reported in the literature [5]. Reviews of case reports indicate spina bifida to be the most frequent coexisting anomaly with both true tail and pseudotail [2,4]. Tavaghi et al [7] reviewed 200 cases of occult spinal dysraphism and showed it to be associated with cutaneous signs in more than 50% cases, and 41 cases had subcutaneous lipoma associated with spina bifida [1,5,7]. They reported only 1 case of spinal dysraphism with tail-like cutaneous structure consisting cutaneous and intrathecal lipoma [7]. In the case of spinal dysraphism with associated tail-like caudal appendages, the tail consists of underlying spinal lipoma or lipomyelomeningocele [1,2]. There have been 14 cases of such tail-like appendages with spinal dysraphism reported in the literature [1]. In contradiction to a previous report by Spiegelmann et al [3], true vestigial tails are not benign because they may be associated with underlying dysraphic state. According to previous a series of 48 skin-covered lumbosacral masses, 67% of cases were lipomas and lipomyelomeningocele, 25% lumbosacral and sacrococcygeal teratomas, and 4% meningocele and 4% terminal myelocystoceles [8]. Hoffman et al [9] had described that fatty mass in the lumbosacral region is the most common presenting complaint in a series of 97 patients, and 5 patients presented with tail-like caudal appendages. Lu et al [10] noted 59 cases with caudal appendages, and 50 % of the cases were associated with either meningocele or spina bifida occulta [10]. The distinction between the true tail and pseudotail on clinical examination is almost always dubious, and despite a
E43 normal neurological examination, normal plain x-ray of spine, and clinical classification of true tail, subfascial exploration may show tethered cord or lipomeningomyelocele as what occurred in our case. Hence, ignoring the type of tail, each individual case needs comprehensive preoperative investigation, and surgical exploration of the intraspinal content should be done microsurgically to avoid any damage or neurological deficit.
References [1] Belzberg AJ, Myles ST, Trevenen CL. The human tail and spinal dysraphism. J Pediatr Surg 1991;26:1243-5. [2] Chakraborty S, Shizuo O, Yoshida Y, et al. Myelomeningocele and thick filum terminale with tethered cord appearing as a human tail. J Neurosurg 1993;78:966-9. [3] Spiegelmann R, Schinder E, Mintz M, et al. The human tail: a benign stigma. J Neurosurg 1985;63:461. [4] Dao AH, Netsky MG. Human tails and pseudotails. Hum Pathol 1984; 15:449-53. [5] Feridun A, Ersin E, Muzeyyen G, et al. Human tail. A rare lesion and possible marker of intraspinal lipoma in adolescent and pediatric cases. WSJ 2006;1(2):84-7. [6] Harrison RG. On the occurrence of tail in man. Johns Hopkins Hosp Bull 1901;12:96-101. [7] Tavaghi G, Ghandchi A, Hambrick G, et al. Cutaneous signs of spinal dysraphism. Arch Dermatol 1978;114:573. [8] McLone DG, Naidich TP. Terminal myelocystocele. Neurosurgery 1985;16:36-43. [9] Hoffman HJ, Taecholarn C, Hendrick EB, et al. Management of lipomyelomeningoceles. J Neurosurg 1985;62:1-8. [10] Lu FL, Wang PJ, Teng RJ. The human tail. Pediatr Neurol 1988;1988: 230-3.