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Table of Contents    
Year : 2012  |  Volume : 60  |  Issue : 2  |  Page : 247-249

Neurenteric cyst of the cerebello-pontine angle in an infant presenting with obstructive hydrocephalus

1 Department of Neurosurgery, Sri Satya Sai Institute of Higher Medical Sciences, Bangalore, India
2 Department of Pathology, Sri Satya Sai Institute of Higher Medical Sciences, Bangalore, India

Date of Submission29-Nov-2011
Date of Decision02-Dec-2011
Date of Acceptance15-Jan-2012
Date of Web Publication19-May-2012

Correspondence Address:
Ravi Dadlani
Department of Neurosurgery, Sri Satya Sai Institute of Higher Medical Sciences, Bangalore
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.96429

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How to cite this article:
Dadlani R, Ghosal N, Khetan R, Hegde AS. Neurenteric cyst of the cerebello-pontine angle in an infant presenting with obstructive hydrocephalus. Neurol India 2012;60:247-9

How to cite this URL:
Dadlani R, Ghosal N, Khetan R, Hegde AS. Neurenteric cyst of the cerebello-pontine angle in an infant presenting with obstructive hydrocephalus. Neurol India [serial online] 2012 [cited 2023 Jun 4];60:247-9. Available from:


Neurenteric cysts (NCs) are congenital epithelial lined cystic lesions presumably derived from the endoderm and most often occur anterior to the spinal cord in the lower cervical or thoracic spine. [1],[2],[3],[4],[5] These lesions are rare in the intracranial compartment, most often located in the midline anterior to the brainstem [1] and a laterally placed NC is exceptional. [1],[2],[3] This lesion is usually misdiagnosed as arachnoid cyst or epidermoid cyst. [1] Magnetic resonance imaging (MRI) features may help in accurate preoperative diagnosis, thus facilitating better surgical planning and complete excision of the lesion. [1] Because these cysts have a chronic course and increase in size gradually, they usually present in mid-adult life with clinical features attributable to the adjacent structures. [1],[2],[3],[4],[5] NC in the cerebello-pontine angle (CPA) in an infant has not been documented till date, probably this is the first such report.

An 11-month-old infant presented with history of gradually increasing head circumference since the age of two months and delayed social and motor milestones. On examination the child had an increased occipito-frontal circumference of 67 cm with a patent anterior fontanelle. MRI brain revealed a right CPA cyst measuring 4.5×4×3.5 cm. The cyst was hypointense on T1-weighted images and hyperintense on T2- weighted image. The signal characteristics were similar to that of cerebrospinal fluid (CSF) on all sequenceswith no restriction on diffusion-weighted imaging (DWI). Fluid-attenuated inverse recovery (FLAIR), however, demonstrated a hyperintensity of the fluid. There was no enhancement of the cyst wall [Figure 1]a-e. The child underwent a retro-mastoid suboccipital craniectomy and decompression of the cyst. The cyst fluid was clear and CSF-like. The cyst wall was gradually peeled off the nerves and the brainstem and excised near completely. In the postoperative period the child had a single episode of chemical meningitis. Follow-up imaging showed considerable reduction in the size of the cyst [Figure 2]a- c. Histopathology revealed an NC with pseudostratified columnar epithelium with few ciliated cells [Figure 3]a-c. Immunohistochemistry for Epithelial Membrane Antigen EMA was positive in the lining epithelial cells [Figure 3]b.
Figure 1: Preoperative MRI revealing a - 4.5 × 3.5 × 4 cm cyst in the right CPA. The lesion is of CSF intensity on both T1-weighted images (b) and on T2-weighted images (a and c). There was no restriction on diffusion-weighted images (d) but did show significant hyperintensity on FLAIR images (e)

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Figure 2: Follow-up radiology of the child at one month (a and b) and one year (c). The first two images (T2WI coronal and axial) revealed considerable decrease in the size of the cyst. The third image is a CT scan of the brain done one-year interva

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Figure 3: (a) Paraffin section of the enterogenous cyst showing pseudostratified columnar epithelium (Hematoxylin and Eosin ×100). (b) Immunohistochemistry for epithelial membrane antigen is positive in the lining epithelial cells (Avidin Biotin Complex immunoperoxidase ×400). (c) Same as 3a but higher resolution, i.e. H and E, ×400

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Developmental cysts occurring in the CPA and posterior fossa cysts may be of four possible types based on the germ cell origin of the cyst [Table 1]. 'Enterogenous cyst' was previously the accepted term for these rare entities [1] but more recent literature seems to favor the term NC. [2] NC of the CPA is extremely rare, representing about 0.1% of all CPA lesions. [1],[2],[3],[4] Only 18 such cases have been reported, the youngest patient in a Pubmed review was 14 years of age and the oldest 71. [1] There have been none reported in an infant. [1] The foremost theory of embryogenesis outlines NC as a minor degree of endodermal-ectodermal malformation beginning at the time of notochordal development. [1] It represents failed closure of the transient neurenteric canal between the foregut or the respiratory buds and the notochord during the third week of development. [1],[4] However, this therapy fails to explain cysts located laterally such as in the CPA. An extradural NC in the CPA has also been described necessitating a new hypothesis of embryogenesis. [6] MRI aids the diagnosis preoperatively. The arachnoid cyst is most often confused with the NC, both cysts demonstrate signal intensities similar to CSF on all sequences however FLAIR sequence usually clinches the diagnosis; on FLAIR, NC appears hyperintense as in this patient. [1],[2] NCs are endodermal lined cysts; histological variants were described and classified by Wilkins and Odome into three distinct types. [7] Our patients fits into Type I. NCs are known to recur, cause chemical meningitis and rarely, malignant change [8] necessitating adequate surgical management and complete excision of the lesion.
Table 1: A simplified classification of congenital intracranial cysts

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 » References Top

1.Perrini P, Rutherford SA, King AT, du Plessis D, Di Lorenzo N. Enterogenous cysts of the cerebellopontine angle short review illustrated by two new patients. Acta Neurochir (Wien) 2008;150:177-84; discussion 184.  Back to cited text no. 1
2.Wang L, Zhang J, Wu Z, Jia G, Zhang L, Hao S, Geng S. Diagnosis and management of adult intracranial neurenteric cysts. Neurosurgery 2011;68:44-52.  Back to cited text no. 2
3.Novegno F, Di Rocco F, Tamburrini G, Massimi L, Lauriola L, Caldarelli M, et al. Unusual presentation of intradural endodermal cysts in young children under 2 years of age. Report of two cases. Eur J Pediatr 2006;165:613-7.  Back to cited text no. 3
4.Gopalakrishnan CV, Dhakoji A, Shrivastava A, Nair S. Neurenteric cyst of the cerebellopontine angle with fetal-type posterior circulation. Neurosurg Q 2010;20:311-3.  Back to cited text no. 4
5.Batuk D, Koichiro Y, Tsukasea K, Tetsuo K, Ando M. Cerebellopontine angle endodermal cyst: A rare occurrence. Neurol India 2010;58:676-7.  Back to cited text no. 5
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6.Inoue T, Kawahara N, Shibahara J, Masumoto T, Usami K, Kirino T. Extradural neurenteric cyst of the cerebellopontine angle. Case report. J Neurosurg 2004;100:1091-3.  Back to cited text no. 6
7.Sreedhar M, Menon S, Varma G, Ghosal N. Cervico-thoracic neurenteric cyst - a case report. Ind J Radiol Imag 2006;16:99-102.  Back to cited text no. 7
8.Wang W, Piao YS, Gui QP, Zhang XH, Lu DH. Cerebellopontine angle neurenteric cyst with focal malignant features. Neuropathology 2009;29:91-5.  Back to cited text no. 8


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]

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