Atretic cephalocele is a small skin-covered lesion, usually located at or near the mid-line of the scalp. Histologically, it is composed of syncytial cells expressing neurone-specific enolase and epithelial membrane antigen. The syncytial cells form capillary-like structures *(pseudovascular areas) and collagenic fibrosis with densely packed collagen bundles (fibrous areas). Such findings suggest that the atretic cephalocele is a mild form of cephalocele, with its pathogenesis lying in the spectrum of neural tube closure abnormalities. However, few descriptions of abnormalities of the skin overlying and surrounding atretic cephalocele are available. We report two cases of atretic cephalocele that showed hamartomatous change in the surrounding cutaneous appendages. These findings suggest that atretic cephalocele is associated with abnormalities not only of the neural tube, but also of the surrounding skin.
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Atretic cephalocele and differential considerations: A small case series.
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 PDFAtretic cephalocele associated with sinus pericranii: a single-center analysis.
Childs 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.
Atretic cephaloceles with different imaging phenotypes - Case series with review of literature.
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 PDFAtretic cephalocele and encephalocele: A single-institution clinicopathological study.
J 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 PDFPersistent falcine sinus in the newborn: 3 case reports of associated anomalies.
Radiol 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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