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      Quadrigeminal plate cistern lipoma presenting with seizures in a child

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          Abstract

          Intracranial lipomas are neither hamartomatous nor neoplastic lesions. They result from abnormal persistence and development of meninx primitiva.[1] They are extremely rare and are seen in around 0.08-0.2% of all autopsy cases and constitute only 0.06-0.3% of incidental lesions.[2] Rarely, they have been associated with seizures, headache, and behavioral disturbances.[3] These lesions are managed conservatively owing to their indolent course. A 3-year-old child from rural background was referred for CT scan of head for evaluation of single episode of generalized tonic-clonic seizures 1 day back. There was associated loss of consciousness and postictal confusion. The patient denied any history of prior similar episodes, and his neurological development was normal. On examination, the patient was in overall good general health and systemic examination of respiratory, cardiovascular, and nervous system revealed no anomaly. Routine hematological investigations were within normal limits. Axial non-contrast CT scan of the head revealed a well-defined lobulated hypodense lesion, with smooth margins and showing fat attenuation (CT value of -30 HU), in the quadrigeminal plate cistern [Figure 1]. There was no evidence of calcification within the lesion, and the lesion exhibited no enhancement on contrast administration. No other abnormality was detected. Figure 1 (a) Axial non-contrast CT scan of the patient showing a well-defined lobulated hypodense lesion, with no evidence of calcification and showing smooth margins and fat attenuation, in the quadrigeminal plate cistern. (b) On contrast administration, there is no enhancement of the lesion A provisional diagnosis of quadrigeminal plate lipoma was made, and patient was treated with oral anti-epileptic drugs. The patient is on regular follow up for 1 year, with no evidence of progression of symptoms. Meckel was the first to report intracranial lipomas, way back in 1818.[1] Since then, intracranial lipomas have being routinely described in literature as incidental lesions. Yilmaz et al. reported that these lesions were occasionally associated with headache, epilepsy, and neurological dysfunction.[3] Lipomas of the quadrigeminal plate have been associated with obstructive hydrocephalus due to compression of cerebral aqueduct.[4] These lesions are usually seen in children and young adults, but to the best of our knowledge, this is the youngest case of a child with quadrigeminal plate cistern lipoma. The most common location of these lesions is pericallosal, where 45% of these lesions are found. Other uncommon locations include suprasellar cistern, quadrigeminal plate cistern, cerebellopontine angle, and the sylvian fissure. On CT scans, they appear as well demarcated non-enhancing lesions with fat attenuation. The lesions in pericallosal location may show calcifications. On MRI, they appear hyperintense on both T1W and T2W sequences and show signal drop on fat saturated images. The common differentials of these lesions on imaging include dermoid and epidermoid cysts, tectal plate cysts, arachnoid cysts and, rarely, lipomatous transformation of neoplasms like DNET and tectal gliomas. An association of these lesions has been described with neurocutaneous syndromes like encephalocraniocutaneous lipomatosis, epidermal nevus syndrome, or congenital infiltrating lipomatosis.[5] Quadrigmenial plate lipomas have also been associated with hypoplasia of inferior colliculus. Vascular abnormalities including kinking and narrowing along with arteriovenous malformations have also been linked with intracranial lipomas.[6] These lesions are usually managed conservatively[7] as surgical removal of these lesions is difficult owing to their close contiguity with adjacent neurovascular structures. Attempts of surgical evacuation often result in marked neurological deterioration.[8] In conclusion, quadrigeminal plate lipomas are extremely rare entities with varied clinical presentations. A thorough knowledge of the imaging features is essential to rule out other conditions as it is usually asymptomatic and rarely requires aggressive management.

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          Most cited references 8

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          Pathogenesis of intracranial lipoma: an MR study in 42 patients.

          Intracranial lipomas are uncommon lesions whose development remains poorly understood. To clarify the anatomic and embryologic features of intracranial lipomas, we retrospectively reviewed the MR scans of 42 patients with 44 intracranial lipomas. Interhemispheric lipomas were the most common, accounting for 45% of cases. The remainder of the lesions were clustered in the quadrigeminal/superior cerebellar (25%), suprasellar/interpeduncular (14%), cerebellopontine angle (9%), and sylvian (5%) cisterns. Fifty-five percent of the lesions were associated with brain malformations of varying degrees. Intracranial vessels and nerves were noted to course through 16 (36%) of the lesions. The relative frequencies of the locations of the lipomas correspond to the temporal sequence of dissolution of the meninx primitiva, the mesenchymal anlage of the meninges. This finding supports the concept of lipoma formation as a result of abnormal persistence and maldifferentiation of the meninx. This embryologic concept of the development of intracranial lipomas explains the high frequency of callosal and other brain hypoplasias. Intracranial lipomas are neither hamartomas nor true neoplasms; rather, they are congenital malformations.
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            Differential diagnosis of intracranial lesions with high signal on T1 or low signal on T2-weighted MRI.

            Magnetic resonance imaging (MRI) is the preferred technique for examining intracranial pathology in the non-acute setting in most cases. The aim of this review is to demonstrate the imaging appearances on unenhanced spin-echo MRI of lesions that show characteristic, unusual signal changes, which occur due to the presence of fat, cholesterol, paramagnetic substances, rapidly flowing fluids, air, cortical bone or calcification. The nature of the MRI signal, together with the anatomical location of the lesion should allow the provision of a narrow differential diagnosis, before the use of intravenous contrast medium or additional imaging sequences.
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              Intracranial lipomas--a clinical study.

              This study aimed to investigate the localization of the lipoma, as well as associated intracranial and extracranial lesions in 14 patients immediately following hospital admission. The pathological findings from the neurological examinations of these patients are also investigated. Fourteen patients who were admitted to our clinic with a variety of symptoms and diagnosed with intracranial lipoma were included in the study. Problems presented upon admission, neurological findings, and other existing system abnormalities were evaluated. Localization of the lipomas and accompanying pathologies were determined by using computerised tomography and magnetic resonance imaging. The most frequent reasons for admission of patients with intracranial lipomas were: headache 7 (50%), trauma 3 (21.5%), epilepsy 3 (21.5%) and one with symptoms due to the local mass effect of tumor (7%). Although the pericallosal region is accepted as the region where lipomas commonly occur, this study found the most frequent occurrence in the quadrigeminal cistern. Intracranial lipoma calcification was only evident in 1 of the 14 patients. In addition, contrary to the expectations, intracranial and extracranial lesions accompanying lipomas were rare. All patients received systematic treatment. This study showed that intracranial lipomas are more frequent in the quadrigeminal region of the brain; most are asymptomatic, generally caught incidentally; and accompanying intracranial and extracranial pathologies are less common than expected.
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                Author and article information

                Journal
                J Pediatr Neurosci
                J Pediatr Neurosci
                JPN
                Journal of Pediatric Neurosciences
                Medknow Publications & Media Pvt Ltd (India )
                1817-1745
                1998-3948
                May-Aug 2013
                : 8
                : 2
                : 172-173
                Affiliations
                Department of Radiodiagnosis, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India
                [1 ]Department of General Surgery, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India
                [2 ]Department of Medicine, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India
                Author notes
                Address for correspondence: Dr. Abhishek Jha, C/O defender Locks and Hardware, Achal Tank, Aligarh - 202 001, Uttar Pradesh, India. E-mail: abhi.jnmc@ 123456gmail.com
                Article
                JPN-8-172
                10.4103/1817-1745.117865
                3783734
                24082945
                Copyright: © Journal of Pediatric Neurosciences

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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