We describe a case of atretic cephalocele (AC) characterized by the presence of various cerebral anomalies of different midline structures. In our patient the presence of a parietal AC was associated with an embryonic position of the straight sinus, fenestration of the superior sagittal sinus, an abnormal insertion of the cerebellar tentorium with prominence of the superior cerebellar cistern and a septum pellucidum cyst. These findings, associated with AC, could lead to a worse prognosis with regard to neurodevelopmental milestones. This suggests that even if AC is a benign lesion, a complete evaluation of the brain structures should always be performed in these young patients.
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http://dx.doi.org/10.1177/1971400915581744 | DOI Listing |
Neuroradiol J
August 2024
Department of Diagnostic Radiology and Nuclear Medicine, Neuroradiology Section, University of Maryland Medical Center, USA.
Any posterior midline cystic or soft tissue scalp mass in an infant needs evaluation with MRI brain and MRV brain to look for intracranial extension of the lesion. One of the differential considerations is an atretic cephalocele, which can be a difficult diagnosis, especially when the cystic lesions are small. The presence of a small calvarial defect adjacent to the cystic mass and a persistent primitive falcine vein, which points towards the cystic mass, are imaging clues to this diagnosis.
View Article and Find Full Text PDFChilds Nerv Syst
February 2024
Division of Neurosurgery, National Center for Child Health and Development, Okura 2-10-1, Setagaya-ku, Tokyo, Japan.
Purpose: The purpose of this report is to investigate the association of atretic cephalocele (AC) with sinus pericranii (SP) in order to improve the management of AC.
Methods: Pediatric patients with AC who underwent repair surgeries were retrospectively analyzed. Anomalies including SP were evaluated using computed tomography angiography and venography (CTA/V) as well as magnetic resonance imaging.
J Neurosci Rural Pract
March 2023
Department of Radio-Diagnosis, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India.
Atretic cephaloceles (ACs) are congenital skull defects with herniation of rudimentary intracranial structures through the defect and associated with persistent falcine sinus or embryonic positioning of straight sinus. We describe five cases of ACs, out of which only one had embryonic straight sinus. Three cases had other intracranial malformations such as hypoplasia of corpus callosum, dysplastic tectum in one child and parieto-occipital polymicrogyria with falcotentorial dehiscence in the other, and frontal horn deformity and cortical dysplasia in the third.
View Article and Find Full Text PDFJ Cutan Pathol
July 2023
Pathology Service, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Background: Encephaloceles are neural tube defects characterized by herniation of meninges, neural tissue and cerebrospinal fluid, while atretic cephaloceles denote a rudimentary connection to the intracranial space with absence of herniated neural tissue and represent an infrequent dermatopathologic diagnosis. Limited reports of these entities confound the challenge in their histopathologic distinction. Accurate classification is important given associated anomalies and neurologic manifestations that impact prognosis.
View Article and Find Full Text PDFRadiol Case Rep
March 2023
Department of Radiology, Tripler Army Medical Center, Medical Center, Honolulu, 1 Jarrett White Rd Medical Center, HI 96859, USA.
The falcine sinus is a normal embryonic structure that is situated between the 2 layers of the falx cerebri and drains the deep cerebral venous system into the superior sagittal sinus. It normally involutes after birth and is uncommon in adults. Although it is often an isolated and incidental finding, it can also be associated with a number of other conditions including but not limited to vein of Galen arterial malformations (VGAM), atretic parietal cephaloceles, acrocephalosyndactyly (Apert syndrome), absence of the corpus callosum, absence of the tentorium, osteogenesis imperfecta, or Chiari II malformations.
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