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Spinal Lipoma with Tibial Hemimelia—Incidental Or Causative?

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Spinal Lipoma with Tibial Hemimelia—Incidental Or Causative? [Downloaded free from http://www.neurologyindia.com on Thursday, March 05, 2015, IP: 202.177.173.189] || Click here to download free Android application for this journal Letters to Editor Neeraj N. Baheti, Dinesh Kabra, Nitin H. Chandak, B. D. Mehta1, Rajesh R. Agrawal2 Spinal lipoma with tibial Department of Neurology, Central India Institute of hemimelia—incidental or Medical Sciences, 1Department of Dermatology, NKP Salve Medical College, 2Department of Pediatrics, Colours Hospital, causative? Revisiting the Nagpur, Maharashtra, India E-mail: [email protected] McCredie-McBride hypothesis References Sir, 1. Damel CR 3rd, Scher RK. Nail changes secondary to systemic drugs. J Am Acad Deratol 1984;10:250-8. A 32-year-old female with congenitally short left lower limb 2. Silverman R, Baran R. Nail and appendageal abnormalities. In: [Figure 1] who was able to independently ambulate with Schachner LA, Hansen RC, editors. Pediatric Dermatology. Edinburgh: a crutch presented to the Department of Orthopedics for Mosby; 2003. p. 561-87. lower limb prosthesis. Her radiographs revealed a Type-1 3. Mishra D, Singh G, Pandey SS. Possible carbamazepine induced reversible onychomadesis. Int J Dermatol 1989;28:460-1. left tibial hemimelia and a dysplastic femur with a poorly 4. Prabhakara VG, Krupa DS. Reversible onychomadesis induced by developed femoral head and acetabulum [Figure 2]. Hence, a carbamazepine. Indian J Dermatol Venereol Leprol 1996;62:256-57. custom-made lower limb prosthesis with a pelvic support was 5. Grech V, Vella C. Generalized onycholysis associated with sodium planned. On examination, it was found that there was a tuft valproate therapy. Eur Neurol 1999;42:64-5. 6. Chopra A, Kaur M, Kular J, Chopra D. Nail changes after carbamazepine. of hair in her lower back [Figure 1] for which a neurosurgical Indian J Dermatol Venereol Leprol 2000;66:103-4. opinion was sought. On examination, she was continent with 7. Yasushi I, Hirokazu O, Takayoshi T, Kyoko T, Makiko O. no sphincteric dysfunction. The power was normal in the Carbamazepine-induced reversible onychomadesis: A case report. J Japan Epilepsy Soc 2005;23:14-7. right lower limb with normal reflexes. The left lower limb 8. You KA, Jeong JA, Nahm SA, Kim EA. A case of carbamazepine induced was flail although some movement was present at all joints. onychomadesis. J Allergy Clin Immunol 2006;117:P227. There was no sensory deficit in the limbs or in the perianal 9. Czajkowski R, Weiss-Rostkowska V, Wankiewicz A, Drewa T, Placek W, region. There was a varus deformity of the foot [Figure 1]. Biedka M, et al. Stevens-Johnson syndrome induced by carbamazepine. Acta Pol Pharm 2007;64:89-92. X-rays of the lumbosacral (LS) spine showed multilevel spina 10. Poretti A, Lips U, Belvedere M, Schmitt B. Onychomadesis: bifida [Figure 2]. Magnetic resonance imaging (MRI) of the A rare side-effect of valproic acid medication? Pediatr Dermatol LS spine showed an intraspinal lipoma at D11 to L1 levels 2009;26:749-50. 11. Icagasioglu D, Ayvaz A, Akyol M. Onychomadesis: A new side effect [Figure 3]. This was present more on the left side and the of sodium valproate therapy in children? Arch Neuropsychiatry cord too was thinned out on the left side [Figure 3]. The 2011;48:79-81. ultrasound abdomen revealed normal kidneys and bladder 12. Jenerowicz D, Szulczynska-Gabor J, Polanska A, Sadowska-Przytocka A, with no evidence of residual post-void urine. No surgery was Osmola-Mankowska A, Czarnecka-Operacz M. Finger-nail onycholysis, leukonychia and acrocyanosis in a patient treated with valproic performed but her family was counseled about the possibility acid – Case report. Postepy Dermatol Alergol 2011;28:522-4. 13. Clementz GC, Mancini AJ. Nail matrix arrest following hand-foot-mouth disease: A report of five children. Pediatr Dermatol 2000;17:7-11. 14. Convey JM, Kriel RL, Birnbaun AK. Antiepileptic drug therapy in children. In: Swaiman KF, Ashwal S, Ferriero DM, editors. Pediatric Neurology Principles and Practice. 4th ed. Philadelphia: Mosby; 2006. p. 1122-5. 15. Jones KL, Lacro RV, Johnson KA, Adams J. Pattern of malformations in the children of women treated with carbamazepine during pregnancy. a b N Engl J Med 1989;320:1661-6. Figure 1: Clinical photograph of the patient showing (a) shortened left lower 16. Lewis-Jones MS, Evans S, Culshaw MA. Cutaneous manifestations of limb with varus deformity of foot and (b) tuft of hair in the lower back on zinc deficiency during treatment with anticonvulsants. Br Med J (Clin the left side Res Ed) 1985;290:603-4. 17. Altunbasak S, Biatmakoui F, Baytok V, Herguner O, Burgut HR, Kayrin L. Serum and hair zinc levels in epileptic children taking valproic acid. Biol Trace Elem Res 1997;58:117-25. Access this article online Website: Quick Response Code www.neurologyindia.com DOI: 10.4103/0028-3886.152687 a b c PMID: Figure 2: X-rays showing (a) complete agenesis of left tibia with deformed, shortened femur, (b) dysplastic femoral head, and acetabulum with poorly xxxxx formed ilium, and (c) spina bifida in the lumbar spine 122 Neurology India / January 2015 / Volume 63 / Issue 1 [Downloaded free from http://www.neurologyindia.com on Thursday, March 05, 2015, IP: 202.177.173.189] || Click here to download free Android application for this journal Letters to Editor the left side corresponded to the cord segments innervating the dysmelic lower limb. However, the incidence of spinal lipoma is far more common (1 in 4,000 patients)[7] than that of hemimelia; and, while spinal lipomas may cause tethering or pressure effect on the cord, they have not been reported to completely replace the neural tissue or cause gross agenesis of the nerves. In fact, the cord-lipoma junction is said to be medial to the dorsal root entry zone.[8] Hence, an explanation of lipoma-induced a b c dysgenesis of posterior columns leading to dysmelia may not Figure 3: Magnetic resonance imaging showing (a) a lipoma extending from be tenable (unlike in cases with thalidomide toxicity) and their D11 to L1 levels on sagittal T1 images, (b) the lipoma is present posteriorly and to the left compressing the cord anteriorly and to the right on axial T1 association, in this case, may merely be a random occurrence images, and (c) thinned cord on the left side in coronal T2 images of two unrelated congenital abnormalities. of neurological deterioration in the future and the patient was told to review periodically in the Neurosurgery Department. Prasad Krishnan, Sutirtha Chakraborty1 Department of Neurosurgery, National Neurosciences Centre, Tibial hemimelia is a rare congenital abnormality with an Calcutta, 1Peerless Hospital and B. K Roy Research Centre, incidence of one in 1,000,000 live births.[1] It can be of four Calcutta, West Bengal, India types[1]—with a complete absence of the tibia, as in our E-mail: [email protected] case, being classified as Type-1. While it can be associated References with several other congenital abnormalities, the commonest [2] 1. Salinas-Torres VM, Barajas-Barajas LO, Perez-Garcia N, Perez-Garcia spinal problem reported with dysmelia is scoliosis. We G. Bilateral tibial hemimelia type 1 (1a and 1b) with T9 and T10 encountered one report describing its association with hemivertebrae: A novel association. Sao Paulo Med J 2013;131:275-8. hemivertebrae[1] and the other with a spinal lipoma.[3] 2. Powers TA, Haher TR, Devlin VJ, Spencer D, Millar EA. Abnormalities of the spine in relation to congenital upper limb deficiencies. J Pediatr Orthop 1983;3:471-4. The influence of the development of the neural crest on 3. Sanyal SK, Mahapatra AK. Transverse hemimelia in a child with spinal limb morphogenesis came to light in experimental studies in dysraphism. Pediatr Neurosurg 2004;40:203-4. the wake of the thalidomide disaster where it was noticed 4. McBride WG. Studies of the etiology of thalidomide dysmorphogenesis. that ultrastructural changes predating the development Teratology 1976;14:71-87. 5. Stokes PA, Lykke WJ, McBride WG. Ultrastructural changes in the of limb formation appeared in those dorsal root ganglia dorsal root ganglia evoked by thalidomide in rabbits. Experientia and their neurons that corresponded to the deformed 1976;32:597-9. limb segments.[4,5] This theory of “embryonic sensory 6. McCredie J. Segmental embryonic peripheral neuropathy. Pediatr Radiol neuropathy”[6] contributing to the genesis of dysmelia is called 1975;3:162-8. 7. Wykes V, Desai D, Thompson DN. Asymptomatic lumbosacral lipomas-a the McCredie-McBride hypothesis. natural history study. Childs Nerv Syst 2012;28:1731-9. 8. Chapman PH. Congenital intraspinal lipomas: Anatomic considerations The vital question in our case was whether the lipoma had and surgical treatment. Childs Brain 1982;9:37-47. contributed to the dysmelia by interfering with the functions of the dorsal horn cells; or, was it an incidental finding? Access this article online It is worth noting that the side of the limb deformity and Website: Quick Response Code the side of the lipoma were the same (i.e., the left side). www.neurologyindia.com Moreover, the lipoma was posteriorly placed, i.e., in the DOI: region where the dorsal columns of the spinal cord should 10.4103/0028-3886.152688 have been present and the MRI also showed asymmetrical cord atrophy (more on the left side). Furthermore, the PMID: location of the lipoma at D11 to L1 vertebral segments on xxxxx Neurology India / January 2015 / Volume 63 / Issue 1 123 Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission.
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