scispace - formally typeset
Search or ask a question

Showing papers on "Intracranial Lipoma published in 2013"


Journal ArticleDOI
TL;DR: In this small retrospective analysis of 50 cases undergoing brain CT for various reasons small intracranial lipomas where found in nine patients, in contrast to previous reports lipomas may be a frequent finding on CT imaging of the brain.
Abstract: Intracranial lipomas are described as a rare finding. In this small retrospective analysis of 50 cases undergoing brain CT for various reasons small intracranial lipomas where found in nine patients. In contrast to previous reports lipomas may be a frequent finding on CT imaging of the brain. In particular, these small lipomas seem to be incidental findings lacking clinical relevance. Radiologists should be aware of intracranial lipomas to establish proper differential diagnosis.

11 citations


Journal ArticleDOI
TL;DR: To the authors' knowledge, this is the first case of ECCL having a large temporal exostosis, and the objective of this article is to better understand the phenotypic spectrum of this syndrome whose molecular basis is still unknown.
Abstract: Encephalocraniocutaneous lipomatosis (ECCL), also known as Haberland syndrome, is a rare syndrome with unknown etiology. The syndrome is characterized by a triad of unique cutaneous, ocular, and central nervous system (CNS) manifestations. The cutaneous hallmark, nevus psiloliparus (NP), along with overlying alopecia is a constant feature. Choristoma of the eyelid is the most common ocular manifestation, while intracranial lipoma is the predominant CNS finding. Genetic counseling is required to emphasize that the disorder, although congenital, is not inheritable. We present a 21-year-old female with cutaneous, ocular, and CNS features satisfying the diagnostic criteria for ECCL. To our knowledge, this is the first case of ECCL having a large temporal exostosis. The objective of this article is to better understand the phenotypic spectrum of this syndrome whose molecular basis is still unknown.

11 citations


Journal Article
TL;DR: A case with prenatal detection of a pericallosal lipoma and a skin tag on the forehead and after delivery, the diagnosis was confirmed as a case of Pai syndrome.
Abstract: Summary: Prenatal detection of Pai syndrome without cleft lip and palate: a case report: Pai syndrome is a rarely encountered disease characterized by findings of median cleft lip, facial skin polyps, nasal mucosal polyps and midline central nervous system lipoma. We report a case with prenatal detection of a pericallosal lipoma and a skin tag on the forehead. After delivery, the diagnosis was confirmed as a case of Pai syndrome.Key-words: Pericallosal lipoma - Pai syndrome - Prenatal ultrasoundINTRODUCTIONIntracranial lipomas are rarely observed congenital malformations. They usually occur secondary to the abnormal differentiation of meninx primitiva layer, arachnoid and the inner layer of dura mater (5). They make up less than 0. 1% of all intracranial tumors in the literature. Twenty five-fifty percent of all intracranial lipomas are pericallosal lipomas with partial or complete agenesis of the corpus callosum in some of the patients (5). They can be observed in nodular or curvylinear forms and are usually asymptomatic (5).When an intracranial lipoma is coupled with a facial polyp, it may be suspicious for Pai syndrome. Pai syndrome is a rare disorder in which there are some other associated findings such as median cleft lip, facial skin and nasal mucosal polyps in addition to a midline intracranial lipoma (1,2, 6).CASE REPORTA 40 years old woman (gravida 13, para 12) at 28th week of pregnancy was referred to our perinatology unit because of a hyperechogenic structure in the brain of the fetus. Fetal biometrie measurements were appropriate for gestational age. Cranial ultrasonography revealed a hyperechogenic structure right above the septum pellucidum, neighboring the corpus callosum. Pericallosal artery was intact (Fig. 1). Further, an abnormally shaped nose and a polyp like structure were detected on the forehead (Fig. 2). There was no sign of cleft lip and palate. The rest of the neurosonographical examination was within normal limits. A preliminary diagnosis of pericallosal lipoma was made. The family history did not reveal any findings regarding mental retardation or congenital malformations and an offer of fetal karyotyping was declined by the parents.A male fetus (2740 g / 49 cm) was delivered by cesarean section on the 40th week of pregnancy. The Apgar scores at 1st and 5th minutes were 6 and 8 respectively. The baby did well without the need for neonatal intensive care unit. On physical examination there were dysmorphic features (Fig. 3) such as bifid nose, bilateral nostril polyps, skin tags at the midline of the forehead and on the preauricular area, wide nasal bridge, absence of right eyelid, lipoma on the conjunctiva and big and wide earlobes.Abdominal ultrasonography and otoaccustic emission tests revealed no pathology. On cranial MRI, a 30 mm wide lesion was observed in the pericallosal area, concordant with pericallosal lipoma and characterized with hyperintensity in all sequences. Chromosomal analysis of the peripheral blood cultures revealed a 46,XY normal karyotype.DISCUSSIONPai syndrome was first defined by Pai et al. in 1987 (10). Central nervous system lipomas, median cleft of the upper lip and facial skin polyps form the classic triad to define the syndrome, but the phenotype may be variable (13). A median cleft lip is a frequent finding in the previously reported Pai syndrome cases in the literature (3, 6, 13), however, it was not present in our case. Although the only cranial MRI finding in our patient was a pericallosal lipoma, computerized tomography and/or MRI show additional cranial structural anomalies in 85% of the cases with Pai syndrome (5, 9). For example, Guion et al. reported a Pai syndrome with a huge encephalocele, and the computerized tomography scan demonstrated a general disorganization of the cerebral parenchyma in addition to hydrocephaly in this patient (4). Despite this high rate of central nervous system pathologies, thus far serious neurological disorders such as mental retardation have been reported in only a few cases (8, 3). …

7 citations


Journal ArticleDOI
TL;DR: A rare case of midline craniofacial dysraphism with interhemispheric calcified lipoma together with subcutaneous lipoma and agenesis of the corpus callosum is presented.
Abstract: Frontonasal dysplasia (FND) or craniofacial dysraphism includes a variety of craniofacial defects. FNDs are rarely associated with intracranial lipoma. The majority of intracranial lipomas are incidentally identified on radiological examinations. They are commonly accompanied by other congenital intracranial malformations. Moreover, they are rarely associated with subcutaneous lipoma. We present a rare case of midline craniofacial dysraphism with interhemispheric calcified lipoma together with subcutaneous lipoma and agenesis of the corpus callosum.

3 citations


Journal ArticleDOI
01 Jun 2013
TL;DR: Improvements in the neuroimaging field may lead to higher diagnostic rates, even in asymptomatic individuals, despite currently the best method for diagnosis being magnetic resonance imaging.
Abstract: Intracranial lipoma - Review of literature Intracranial lipomas are considered to be congenital malformations, originated from primitive mesenchymal meninge persistence and later differentiation into fatty tissue. They represent 0.06% to 0.1% of all intracranial tumors. Neuroimaging is useful for diagnosing symptomatic patients. The lesion is often associated with other congenital anomalies such as agenesis of the corpus callosum, however it may be found incidentally with atypical clinical manifestations. Surgical treatment is not recommended due to high complication rates and to the condition's benign course. The present paper reviews the literature through the online databases Cochrane, PubMed/MedLine, Lilacs and SciELO. The reviewed articles were published from 1974 to 2010; descriptors included "cerebral lipoma" and "intracranial lipoma" associated to "brain tumor" and/or "treatment". Intracranial lipomas result from primitive mesenchymal meninge's persistence and later abnormal differentiation into fatty tissue. Computed tomography scan reveals a hypodense lesion, with no enhancement after intravenous application of contrast media or perilesional edema. Magnetic resonance imaging is currently the best method for diagnosis. In case of incidental finding in a previous asymptomatic patient, no surgical treatment is indicated. Intracranial lipomas are rare malformations originated from development abnormalities and usually associated with dysraphisms. This lesion is often either asymptomatic or an incidental imaging finding. Improvements in the neuroimaging field may lead to higher diagnostic rates, even in asymptomatic individuals. No surgical treatment is indicated for asymptomatic or incidental cases.