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Increased Sylvian fissure angle as early sonographic sign of malformation of cortical development.

ABSTRACT: OBJECTIVE:To evaluate Sylvian fissure development by assessing Sylvian fissure angles in fetuses with malformation of cortical development (MCD). METHODS:This was a retrospective study of 22 fetuses with MCD. Cases with a stored three-dimensional (3D) brain volume acquired at 18?+?0 to 30?+?6?weeks of gestation at an ultrasound-based research clinic between January 2010 and December 2017 were identified through a database. Of the 22 fetuses, seven had an extracranial abnormality, such as cardiac, renal, gastrointestinal and/or digital anomalies, and five had a minor abnormality such as micrognathia, low-set ears and/or single umbilical artery. To confirm the final clinical diagnosis of brain abnormality, postmortem histological findings or prenatal or postnatal magnetic resonance images were used. For measurement of Sylvian fissure angle, an anterior coronal plane of the fetal brain on transvaginal 3D volume multiplanar imaging was visualized as a single image from the three orthogonal views. The right and left Sylvian fissure angles were measured between a horizontal reference line (0°) and a line drawn along the upper side of the respective Sylvian fissure. The Sylvian fissure angle on both sides was plotted on the graphs of the reference ranges for gestational age in weeks. RESULTS:In 21 (95.5%; 95% CI, 86.8-100.0%) of 22 fetuses with MCD, the Sylvian fissure angle on one or both sides was larger than the 90th percentile of the normal reference. There was one case with apparent focal MCD in the parietal lobe, but the Sylvian fissure angles were normal. A case with apparent unilateral cortical dysplasia and one with apparent unilateral schizencephaly had conspicuous discrepancies between the left and right Sylvian fissure angles. Abnormal genetic test results were obtained in six cases, including four cases with a mutation in a single gene. CONCLUSIONS:This study has shown that the Sylvian fissures, as defined by the Sylvian fissure angle, have delayed development in most MCD cases prior to the diagnosis of the condition. The Sylvian fissure angle may potentially be a strong indicator for the subsequent development of cortical malformation, before the time point at which the gyri and sulci become obvious on the fetal brain surface. Further research is required to validate these findings. © 2018 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of the International Society of Ultrasound in Obstetrics and Gynecology.

SUBMITTER: Pooh RK

PROVIDER: S-EPMC6772089 | biostudies-literature | 2019 Aug

REPOSITORIES: biostudies-literature

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Increased Sylvian fissure angle as early sonographic sign of malformation of cortical development.

Pooh R K RK Machida M M Nakamura T T Uenishi K K Chiyo H H Itoh K K Yoshimatsu J J Ueda H H Ogo K K Chaemsaithong P P Chaemsaithong P P Chaemsaithong P P Poon L C LC

Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology 20190708 2

<h4>Objective</h4>To evaluate Sylvian fissure development by assessing Sylvian fissure angles in fetuses with malformation of cortical development (MCD).<h4>Methods</h4>This was a retrospective study of 22 fetuses with MCD. Cases with a stored three-dimensional (3D) brain volume acquired at 18 + 0 to 30 + 6 weeks of gestation at an ultrasound-based research clinic between January 2010 and December 2017 were identified through a database. Of the 22 fetuses, seven had an extracranial abnormality, ...[more]

PMID: 30381845

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Project description:Pediatric meningeal tumors are rare, but those in the region of the sylvian fissure without dural attachment are extremely rare, with only 24 previously reported cases in the world literature. In this series, we report two additional cases of sylvian fissure meningioma without dural attachment and one case of perisylvian meningioangiomatosis in the medial temporal lobe. All three patients presented with complex partial seizures, but the diagnosis was delayed in each case because the symptoms were misinterpreted to be behavioral rather than epileptic. The seizures were eventually confirmed with electroencephalogram, and subsequent imaging showed enhancing masses within the sylvian fissure region that were at least partially calcified in all three cases. Each patient underwent craniotomy. In the first case, gross total resection was achieved, and in the second case, a small residual portion of tumor was densely calcified and adherent to the middle cerebral artery branches. Both of these were World Health Organization (WHO) grade I meningiomas. The third patient underwent biopsy and limited resection of meningioangiomatosis. No dural attachments were noted in any of the tumors, but one of the meningiomas was intraparenchymal in location, surrounding the sylvian fissure in both the frontal and temporal lobes, which has been described in only a small number of these cases previously. The patients underwent pre- and postsurgical neuropsychiatric testing and did not experience any significant cognitive deficits. At 10-year follow-up, the patient who had gross total resection of the tumor has had no recurrence and is seizure-free without anticonvulsant medications. The incompletely resected intraparenchymal meningioma in the second patient recurred after 5 years, however, and at repeat surgery was found to have transformed to a WHO grade II tumor. Radiation therapy was delivered and the tumor has been stable for 2 years, but the patient continues to have occasional seizures despite medication. The patient with meningioangiomatosis has had no further growth and has excellent control of seizures but remains on medication. We review the clinical presentation of these rare tumors and discuss the treatment, outcomes, and possible relationship of meningiomas to meningioangiomatosis.

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Increased Sylvian fissure angle as early sonographic sign of malformation of cortical development.

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Increased Sylvian fissure angle as early sonographic sign of malformation of cortical development.

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