Congenital Interparietal Encephalocele: A Case Report AShok NAyAk1, SAtyAdeo ShArmA2, rAkeSh kumAr VAdher3, SourAbh dixit4, rohAN

Encephalocele is a mesodermal defect in the skull bones and duramater. Parietal encephalocele is a rare congenital anomaly of newborn with variable prognostic value. The authors report a case of a very large inter parietal encephalocele with no associated other system malformations. A midline inter parietal encephalocele is much rare, earlier reported cases were posterior parietal in location. Such cases can be successfully operated upon with a very good outcome. A unique case of a 18 day neonate, with swelling over scalp was evaluated by the neurosurgical team and the patient underwent neurosurgery. In planning the strategy for management of encephalocele, one needs to take into consideration the site, size, contents, patency of CSF pathway, neurological status and other associated anomalies. Inspite of such a big encephalocele in an atypical location, excision and repair gave excellent results. keywords: Congenital anomaly, Parietal encephalocele, Vertex encephalocele SCAN head showed a focal defect involving high parietal bone with herniation of CSF filled sac and neural tissue through this defect suggestive of encephalocele. There was forced sutural separation [table/Fig-1]: Neonate with cystic scalp swelling and a daughter cyst [table/Fig-2]: Antero-posterior view of skull showing a mid-line bony gap [table/Fig-3]: 3D CT scan of head antero-lateral view showing the location of defect [table/Fig-4]: 3D CT scan of head postero-lateral view showing the location of defect [table/Fig-5]: CT scan coronal sections showing the herniated contents of the cyst Ashok Nayak et al., Congenital Interparietal Encephalocele : A Case Report www.jcdr.net Journal of Clinical and Diagnostic Research. 2015 Apr, Vol-9(4): PD09-PD10 10 ConClusion Among encephaloceles, inter parietal encephaloceles represents rare cases. CT image morphology is required to make a diagnosis of the underlying brain malformations. In planning the strategy for the management of encephalocele, one need to take into consideration the site, size, contents, patency of CSF pathway, neurological status and other associated anomalies. Timely surgical intervention, as in our case has a good outcome. In spite of such a big size encephalocele excision and repair gave very good results, child was moving all four limbs and reflexes were intact. Postoperative follow up should be done at regular interval to look for hydrocephalous or other neurological deficit. Postoperatively this case doesn’t revealed significant neurofocal deficit however, left sided lateral rectus paresis was noted on 6 month follow up. ReFeRenCes [1] Bano S, Chaudhary V, Yadav S (2012). Congenital Malformation of the Brain, Neuroimaging – Clinical Applications, Prof. Peter Bright (Ed.), ISBN: 978953-51-0200-7, InTech, Available from: http://www.intechopen.com/books/ neuroimaging-clinical-applications/congenital-malformations-of-thebrain [2] Yokota A, Kajiwara H, Kohchi M, Fuwa I, Wada H. Parietal cephalocele: clinical importance of its atretic form and associated malformations. J Neurosurg. 1988;69:545–51. [3] Mahapatra AK. 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A giant occipital encephalocele with spontaneous hemorrhage into the sac: A rare case report. Asian J Neurosurg. 2014;9:158-60. [11] Nardich TP, Altman NR, Braffman BH, et al. Cephaloceles and related malformatons. AJNR. 1991;13:655-90. [12] Mealey J, Dzenitis AJ, Hockey AA. The prognosis of encephaloceles. J Neurosurg. 1970;32:209-18. in the midline and anterior fontanel was enlarged enormously due to the pedicle of the cyst [Table/Fig-2-5]. Excision and repair of the sac was done under general anaesthesia with prone position. Elliptical incision encircling the pedicel of the cyst was made and duramater was defined all around the sac. Sac was opened and about 800 cc dark brown fluid came out with pressure. Cyst wall was excised all around and aberrant atrophied brain tissue partially filling the cyst was excised. Superior sagittal sinus was aberrant, midline superior sagittal sinus could not be located during the surgical procedure, and probably it was obliterated or atrophied due to mass effect of tumor. Dural defect was meticulously repaired, to get a watertight closure. Child stood surgery well and post operatively child could move all four limbs without any gross neurological deficit. No ventilator support was needed post operatively. Post-operative recovery was uneventful till 4th postoperative day.