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LETTER TO EDITOR
Year : 2012  |  Volume : 60  |  Issue : 5  |  Page : 552-554

Synchronous occurrence of a ruptured skull base teratoma and an expansile porencephalic cyst presenting with proptosis


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

Date of Web Publication3-Nov-2012

Correspondence Address:
Sumit Thakar
Department of Neurosurgery, Sri Sathya Sai Institute of Higher Medical Sciences, Whitefield, Bangalore
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.103224

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How to cite this article:
Thakar S, Sai Kiran NA, Ghosal N, Alangar H. Synchronous occurrence of a ruptured skull base teratoma and an expansile porencephalic cyst presenting with proptosis. Neurol India 2012;60:552-4

How to cite this URL:
Thakar S, Sai Kiran NA, Ghosal N, Alangar H. Synchronous occurrence of a ruptured skull base teratoma and an expansile porencephalic cyst presenting with proptosis. Neurol India [serial online] 2012 [cited 2020 Apr 9];60:552-4. Available from: http://www.neurologyindia.com/text.asp?2012/60/5/552/103224


Sir,

Porencephalic cysts (PCs) typically do not affect flow or pressure dynamics of the cerebrospinal fluid (CSF) and are often asymptomatic. Neurological deficits in these patients are due to associated developmental anomalies or preceding parenchymal insult(s). Although local mass effect and raised intracranial pressure have been described in some cases, herniation of a PC into the extracranial compartment is anecdotal. [1],[2],[3] We describe a singular case of a spontaneous herniation of a PC into the orbit. The added peculiarity was its synchronous association with a ruptured skull base teratoma.

A seven-year-old boy presented with a spontaneous, progressive, and painless proptosis of the the right eye of a duration of six months. His mother recollected a temporary regression in his gait skills around the age of one year. Neurological examination revealed nonpulsatile and nontender proptosis of the right eye. Computed tomography (CT) brain [Figure 1]a and b revealed a 3.0 Χ 3.5 Χ 4.0 cm sized, nonenhancing, heterogeneous lesion on the anterior cranial fossa floor. There was a large bifrontal cyst associated with the lesion [Figure 1]a. Bone windows demonstrated a defect in the right orbital roof [Figure 1]b. Magnetic resonance image (MRI) [Figure 2]a-f revealed specks of T1 hyperintensity, suggestive of fat dissemination into right temporal horn, fourth ventricle, and right cerebellopontine angle cistern [Figure 2]b. The bifrontal cyst was of CSF intensity on all sequences, and was communicating with the ventricle on the right and with the convexity subarachnoid space on the left. It had a component in the superomedial aspect of the right orbit [Figure 2]e. At surgery, the cyst was found to be lined by a thin layer of brain tissue on all sides. The ventricular communication with the cyst was noted to exist through a small opening, and this was substantially enlarged. A bony defect was identified in the right orbital roof through which the cyst was herniating out. The skull base lesion was extending posteriorly into the suprasellar region where its capsule was densely adherent to the anterior cerebral arteries (ACAs). The lesion was resected piecemeal and the bony defect in the orbital roof sealed with a fat plug and reinforced with a pericranial patch and fibrin glue. MRI [Figure 2]f showed total excision of the lesion and decompression of the PC. At discharge from the hospital a week later, the degree of proptosis had significantly decreased. Grossly, the grayish and fleshy tumor had solid and cystic areas. Histologically, it was composed of a haphazard array of differentiated trigerminal elements [Figure 3] consistent with the diagnosis of a mature teratoma. The wall of the cyst was identified to be white matter with areas of gliosis, and had no teratomatous elements. This confirmed the cyst to be a PC, and not a teratomatous cyst.
Figure 1: Preoperative non-contrast-enhanced CT brain showing: (a) a lesion of heterogeneous density in the anterior skull base, in the midline and onto the right side. The medial component is hypodense, with an attenuation of -40 HU, suggestive of fat. Local mass effect of a surrounding cystic lesion is noted in the form of thinned out adjacent calvarium and a posteriorly displaced right anterior cerebral artery. (b) Coronal section (bone window) demonstrating a defect in the right orbital roof (white arrow)

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Figure 2: (a) Axial T1 weighted image (WI) showing a large hyperintense component in the lesion, (b) Specks of hyperintensity (black arrows) corresponding to fat globules seen in the right temporal horn, fourth ventricle and right cerebello-pontine angle cistern, (c) Axial T2 WI showing the lesion in relation to a bifrontal cyst of CSF intensity and both the anterior cerebral arteries, (d) The cyst's communication with the lateral ventricle and convexity subarachnoid space, (e) A component of the cyst within the orbit, (f) MR section showing total excision of the lesion and decompression of the cyst

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Figure 3: Paraffin section of the lesion showing: (a) gastric mucosa (straight arrow), (b) respiratory mucosa (straight arrow), (c) nerve (curved arrow), (d) adipose tissue. [H and E, (a-d) x400]

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Our case is unique for its combination of clinicopathological peculiarities. The synchronous occurrence of a PC with a tumor has been only rarely described. [4],[5] Given the fact that ruptured dermoids are known to induce ischemia, [6],[7] it is conceivable that the teratoma in the patient had a similar effect on the ACAs following a spontaneous rupture in infancy. It may have been that the temporary regression of gait skills was related to bifrontal, ACA territory ischemia following leakage of the cyst contents. Proptosis had occurred secondary to a herniation of the PC through a nontraumatic bony defect. This spontaneous bone erosion would have been related to the chronic pressure of a water hammer effect [8] induced by a ball valve mechanism at the site of the small ventricular communication. It was for this reason that we prophylactically enlarged the fenestration as an additive surgical measure.

 
  References Top

1.Abrunhosa J, Gonalves P, dos Santos JG, Moreira F, Resende M, dos Santos AG. Traumatic porencephalic cyst and cholesteatoma of the ear. J Laryngol Otol 2000;114:864-6.  Back to cited text no. 1
    
2.Ranjan M, Arivazhagan A, Chandrajit P, Chandramouli BA. Unusual manifestation of an orbital roof fracture in a child: acute orbital leptomeningeal porencephalic cyst. Neurol India 2010;58:974-5.  Back to cited text no. 2
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3.Shevach I, Manor RS, Rappaport ZH. Orbital porencephalic cyst following penetrating orbitocranial trauma. Childs Nerv Syst 1992;8:297-9.  Back to cited text no. 3
    
4.Nelson AS, Jafari A, Shah P, Eljamel S, O'Riordan JI. Pneumoencephaly following lumbar puncture in association with an ethmoidal osteoma and porencephalic cyst. J Neurol Neurosurg Psychiatry 2007;78:1149-51.  Back to cited text no. 4
    
5.Tada Y, Kannuki S, Kuwayama K, Kouyama Y. Atypical presentation of a dermoid cyst with porencephaly. No Shinkei Geka 2003;31:1215-20.  Back to cited text no. 5
    
6.Liu JK, Gottfried ON, Salzman KL, Schmidt RH, Couldwell WT. Ruptured intracranial dermoid cysts: Clinical, radiographic, and surgical features. Neurosurgery 2008;62:377-84; discussion 384.  Back to cited text no. 6
    
7.Zheng K, Mao BY, Ma L, Jiang S. Ruptured intracranial dermoid cyst with infarction in the basal ganglia--case report. Neurol Med Chir (Tokyo) 2010;50:254-6.  Back to cited text no. 7
    
8.Ryzenman JM, Rothholtz VS, Wiet RJ. Porencephalic cyst: A review of the literature and management of a rare cause of cerebrospinal fluid otorrhea. Otol Neurotol 2007;28:381-6.  Back to cited text no. 8
    


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