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LETTER TO EDITOR
Year : 2017  |  Volume : 65  |  Issue : 1  |  Page : 214-216

Spinal dysraphism with tripedus: A child with three legs


1 Department of Neurosurgery, G. B. Pant Institute of Postgraduate Education and Research (GIPMER), New Delhi, India
2 Department of Anaesthesia, G. B. Pant Institute of Postgraduate Education and Research (GIPMER), New Delhi, India

Date of Web Publication12-Jan-2017

Correspondence Address:
Shaam Bodeliwala
Department of Neurosurgery, G. B. Pant Institute of Postgraduate Education and Research (GIPMER), New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.198209

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How to cite this article:
Bodeliwala S, Singh D, Singh H, Iqbal M, Agarwal A, Khurana P. Spinal dysraphism with tripedus: A child with three legs. Neurol India 2017;65:214-6

How to cite this URL:
Bodeliwala S, Singh D, Singh H, Iqbal M, Agarwal A, Khurana P. Spinal dysraphism with tripedus: A child with three legs. Neurol India [serial online] 2017 [cited 2017 Mar 24];65:214-6. Available from: http://www.neurologyindia.com/text.asp?2017/65/1/214/198209


Sir,

An accessory limb associated with spinal dysraphism is rare with only a few cases reported so far. However, its actual incidence is unknown. There are many hypotheses regarding the embryogenesis of an accessory limb. It may be a primary mesodermal defect involving paraxial mesoderm or a failure of regression of limb bud of a conjoined twin (rachipagus).[1] As it occurs as a result of deranged embryogenesis, it is commonly associated with other malformations including vertebral body malformations, spina bifida, clubfoot, anorectal malformations, accessory pelvis, or genitalia.[2] Here, we report a case of an accessory limb arising from midline dorsal region, attached to the lumbar vertebral column. This was associated with a meningomyelocoele protruding into the accessory limb as well as a subcutaneous lipoma.

A one-and-a-half year old female child presented with an accessory lower limb at the lower back growing with the growth of the child. The child was born to a 25-year-old lady after a nonconsanguineous marriage. Her antenatal and postnatal periods were uneventful. Antenatal ultrasonographic examination was not performed as per recommendations; however, the 11 and 18 week antenatal ultrasonographic examinations failed to detect any abnormality. The child was born by a normal vaginal delivery at home. At birth, the baby was healthy with a finger size projection arising from the lower back. She also had a left club foot. This appendage kept growing to attain the current size (14 cm). At presentation, the child had become conscious of the appendage and her mother had problems in dressing her. There was neither previous history of twins being born nor any congenital anomalies prevalent amongst members in the family. There was no history of maternal illness, drug intake, or radiation exposure during pregnancy. The child completed the entire recommended immunization.

Clinical examination revealed an accessory lower limb arising from the back at the level of L2 and L3 vertebrae. The accessory limb showed a partially developed foot with syndactyled toes and nails [Figure 1]. The limb measured approximately 14 cm in length. No evidence of any neurological deficit was noted. She also had left-sided congenital talipus equinovarus (club foot). The patient could walk without support, and other developmental milestones were normal. X-ray and computed tomography showed a soft tissue appendage with rudimentary bones attached to the L2-L3 vertebrae, appearing like bones of the lower limb [Figure 2]. Magnetic resonance imaging (MRI) confirmed the meningomyelocele with multilevel spina bifida [Figure 3]. The patient underwent excision of the accessory limb along with detethering of the cord and dural repair [Figure 4]. The proximal bone of the accessory limb was firmly adhered to the posterior elements of the L2 and L3 vertebrae. The postoperative period was uneventful, and MRI showed no residual accessory bone or dural sac.
Figure 1: Photograph showing the accessory limb with the magnified view of the syndyctyled toes and nails (at the left lower corner)

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Figure 2: (a) Lateral view X-ray, and (b) non-contrast computed tomography with three-dimensional reconstructed images show the soft tissue mass (a cutaneous appendage) with rudimentary bones (shown by arrows) appearing like an underdeveloped limb

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Figure 3: Magnetic resonance (T2 weighted) images revealed the dural sac protruding into the accessory limb containing a part of the spinal cord and roots

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Figure 4: Intraoperative photograph showing the neural tissue (shown by an arrow) entering the dural sac which has been opened up. Here, the dural sac was protruding into the base of the accessory limb, which was covered with lipomatous tissue

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Spinal dysraphism is a broad term depicting the defect in closure of the caudal neuropore, occurring in the second to sixth week of gestation. The incidence of spinal dysraphism has been decreasing in developed countries; however, it is still a major problem in developing countries. Incidence of spinal dysraphism in India is 1.9 per 1000 births.[3] Some of the closed spinal dysraphism with secondary neurulation defect are generally associated with mesodermal anomalies such as an accessory limb or a tail-like structure containing mesenchymal tissue covered with skin (human tail).[4] There are reports suggesting its association with fully formed organs, such as an ectopic breast and supernumery ribs, or differentiated tissues, such as salivary glandular, renal, intestinal or respiratory tissues.[5],[6],[7] The first case operated for an accessory limb was reported by Jones and Larkin in 1889;[8] however, earlier reports of supernumerary limbs have been noted. This condition has been given different names over time, including heterotopic redundancies, dysraphic appendages, polymelia, tripedus, heteropegus, aborted twinning, and rudimentary or aborted accessory limb.

There are different hypotheses postulated for the development of an accessory limb with spinal dysraphism. Solak et al., believed in fusion hypothesis.[1] This hypothesis suggests that an accessory limb represents a failure of regression of the limb bud of the underdeveloped fused twin. Krishna et al., supported Gardner's theory for the development of accessory appendages, which suggested an overexpansion and subsequent rupture of the neural tube under the intact cutaneous ectoderm causing the dispersion of neural fluid and infiltration of mesodermal tissue, leading to an anomalous mesenchymal development.[9] One theory proposed that it is a primary mesodermal defect involving para-axial mesoderm and limb bud whereas another theory suggested early splitting of the limb bud.[10] None of the theories have been proven yet. Bayri et al., suggested that an accessory limb is associated with different types of neural tube defects, i.e., spinal lipoma (50%), meningocele (36%) or myelomeningocele (14%).[2] It is most commonly found in the thoracic-interscapular region (35%) followed by the lumbar (22%), sacral (14%), and cervical (7%) regions.[2] Most commonly, female infants are affected, as in our case. Such anomalies have also been identified with anorectal malformations, ectopic intestinal loops, and rudimentary external genitalia. Hence, these patients have to be completely investigated for associated congenital anomalies and treatment should be offered to them for these anomalies as well as for cosmetic concerns.

Acknowledgment

This article is dedicated to Francesco Lantini (1884), a famous “three legged football player,” who worked in a circus and made people laugh with his deformity.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Solak A, Ergün S, Polat İ, Şahin N, Genç B. A rare form of heteropagus twinning: Three-armed infant with spinal dysraphism. Case Rep Pediatr 2012;2012:1-4.  Back to cited text no. 1
    
2.
Bayri Y, Tanrıkulu B, Ekşi M, Dağçınar A. Accessory lower limb associated with spina bifida: Case report. Childs Nerv Syst 2014;30:2123-6.  Back to cited text no. 2
    
3.
Bhide P, Sagoo G, Moorthie S, Burton H, Kar A. Systematic review of birth prevalence of neural tube defects in India. Birth Defects Res A Clin Mol Teratol 2013;97:437-43.  Back to cited text no. 3
    
4.
Salunke P, Sura S, Tripathi M, Mukherjee K. Human tail continuing as transitional lipoma. Neurol India 2011;59:637-8.  Back to cited text no. 4
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5.
Gupta VK, Kapoor I, Punia RS, Attri AK. Dorsal ectopic breast in a case of spinal dysraphism: A rare entity. Neurol India 2015;63:392-4.  Back to cited text no. 5
[PUBMED]  Medknow Journal  
6.
Shubha A M, Mohanty S, Das K, Garg I. Spinal teratoma with salivary glandular differentiation. Neurol India 2009;57:821-2.  Back to cited text no. 6
[PUBMED]  Medknow Journal  
7.
Krishnan P, Saha M. Osseous dysraphic hamartoma - A completely formed, aberrantly located, supralaminar rib in a lumbar lipomyelocele. Neurol India 2016;64:182-4.  Back to cited text no. 7
[PUBMED]  Medknow Journal  
8.
Jones R, Larkin F. Removal of accessory limb and meningocele from the back of a child, and its anatomy. BMJ 1889;2:310-1.  Back to cited text no. 8
    
9.
Krishna A, Lal P. Accessory limbs associated with spina bifida - A second look. Pediatr Surg Int 1999;15:248-50.  Back to cited text no. 9
    
10.
Wasnik A, Shinagare A, Lalchandani U, Gujrathi R, Pai B. Rudimentary third lower limb in association with spinal dysraphism: Two cases. Indian J Orthop 2007;41:72.  Back to cited text no. 10
[PUBMED]  Medknow Journal  


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