PICTURE OF THE MONTH

Multiple Neural Tube Defect with Split CordMalformation — A Rare Entity

Kanwaljeet Garg & Vivek Tandon &

Deepak Kumar Gupta & Bhawani Shankar Sharma

Received: 6 November 2013 /Accepted: 17 February 2014# Dr. K C Chaudhuri Foundation 2014

A 4-mo-old girl presentedwith complaints of two swellings, overthe occipital region and dorsal region since birth. This child wasborn to non-consanguineous parents, at full term by vaginaldelivery. Perinatal period was uneventful but mother had nottaken folic acid supplements in the periconceptional period.Physical examination revealed a 3×3 cm swelling on the backof head and a 6×6 cm swelling in the mid back. Both theswellings were soft, cystic, transilluminant andwell coveredwithskin. Anterior fontanel was open and lax. Child had paraparesiswith lower motor neuron type of bladder involvement.

MRI revealed meningomyelocele with split cord malfor-mation (SCM) type 1 with bony spur at D 10-11 (Figs. 1 and

2). There was an associated occipital cephalocele and chiaritype 2 malformation. There was holocord syrinx and corpuscallosum agenesis. The final diagnosis of this patient wascervical cephalocele, lipomeningomyelocele, SCM type I,and Chiari type 2 malformations.

Multiple neural tube defects is of rare occurrence, reportedto represent <1 % in all cases of neural tube defects [1–3]. Theoccurrence of multiple neural tube defects, like in this case,has lent credence to ‘multisite closure theory’, which has nowreplaced single closure ‘zipper’ theory. According to ‘multi-site closure theory’, the closure of neural tube initiates at fivesites and the “collision sites” of neural tube closure with

Fig. 1 MRI T1 WI and T2 WIsagittal sections showing occipitalmeningocele (thin white arrow)with Chiari type 2 malformation(thick white arrow), and D10-11meningomyelocele (star) withsplit cord malformation type 1(black thin arrow) (a, b). Note ismade of cervicodorsal syrinx andlow-lying conus with cord endingat S 1 (thick black arrow) (a, b).CT spine sagittal section showinglarge bony spur extendinginferiorly and dorsally (black thinarrow) (c)

K. Garg :V. Tandon :D. K. Gupta : B. S. SharmaDepartment of Neurosurgery, All India Institute of Medical Sciences,Ansari Nagar, New Delhi, India

V. Tandon (*)R No-720, 7th Floor, All India Institute of Medical Sciences,Ansari Nagar, New Delhi 110029, Indiae-mail: drtandonvivek@gmail.com

Indian J PediatrDOI 10.1007/s12098-014-1383-x

opposing closure directions are the sites of neural tube defects[4].

Less than 8 % of meningomyelocele are known to beassociated with SCM type I [5]. Split cord malformation,though a relatively common association of meningomyelocelehas been reported rarely in the published literature with mul-tiple neural tube defects [2].

Contributions All authors were involved in the treatment of the pa-tient. VT will act as guarantor for this paper.

Conflict of Interest None.

Role of Funding Source None.

References

1. Ahmad FU, Dwarakanath S, Sharma BS, Mahapatra AK. Multipleneural tube defects: A clinical series of seven cases and their embry-ological basis. Pediatr Neurosurg. 2008;44:280–7.

2. Singh N, Singh DK, Aga P, Singh R. Multiple neural tubedefects in a child: A rare developmental anomaly. Surg NeurolInt. 2012;3:147.

3. Singh DK, Singh N. Occipital encephalocele and spinalmeningomyelocele in same patient: New theories hold true? Indian JPediatr. 2012;79:1393–4.

4. Van Allen MI, Kalousek DK, Chernoff GF, Juriloff D, Harris M,McGillivray BC, et al. Evidence for multi-site closure of the neuraltube in humans. Am J Med Genet. 1993;47:723–43.

5. Ansari S, Nejat F, Yazdani S, Dadmehr M. Split cord malformationassociated with myelomeningocele. J Neurosurg. 2007;107:S281–5.

Fig. 2 MRI brain T1 WI axial sections showing occipital meningocele(white star) (a), MRI dorsal spine T2 WI axial sections showing twohemicords with syrinx in right hemicord (thin white arrow) (b), CT spine

axial section showing bony spur (thick white arrow) dividing spinal canalinto two halves

Indian J Pediatr