Related Experiment Videos
Atretic parietal cephaloceles revisited: an enlarging clinical and imaging spectrum?
R J Patterson1, J C Egelhoff, K R Crone
1Department of Radiology, Children's Hospital Medical Center and the University of Cincinnati College of Medicine, Ohio 45229, USA.
Insights
Atretic cephalocele diagnosis is aided by characteristic CT and MR imaging findings, including specific sinus and cistern abnormalities. Some affected children may still achieve normal development despite these imaging clues.
Area of Science:
- Radiology
- Pediatric Neurology
- Developmental Biology
Background:
- Atretic cephalocele is a rare congenital condition.
- Accurate diagnosis is crucial for appropriate management.
Purpose of the Study:
- To identify and describe imaging features indicative of atretic cephalocele.
- To correlate imaging findings with clinical and pathological data.
- To explore potential mechanisms of development.
Main Methods:
- Retrospective review of imaging (CT/MR) and medical records of eight children with midline subscalp lesions.
- Surgical excision and pathological examination of lesions.
- Analysis of radiologic findings and clinical outcomes.
Main Results:
- Six of eight children exhibited specific imaging findings: vertical embryonic positioning of the straight sinus, prominent superior cerebellar cistern, "spinning-top" tentorial incisura, "cigar-shaped" CSF tract, sagittal sinus fenestration, and tentorial "peaking".
- Pathology revealed glial, meningeal, fibrous, and dermal elements.
- Developmental outcomes varied, with most children being normal, one with mild delay, and one mortality.
Conclusions:
- Characteristic CT and MR imaging findings can suggest the diagnosis of atretic cephalocele.
- Despite abnormal imaging, some children with atretic cephalocele may have normal developmental trajectories.
Purpose:
We describe imaging features that are clues to the diagnosis of atretic cephaloceles and discuss clinical findings and a possible mechanism by which these lesions develop.
Methods:
Eight children (five girls and three boys) ranging in age from 1 day to 3 years 4 months with midline subscalp lesions underwent radiologic examination with CT or MR imaging. In all cases, the lesions were surgically excised and subjected to pathologic examination. Imaging studies and medical records were reviewed retrospectively.
Results:
Six of eight children had vertical embryonic positioning of the straight sinus with a prominent superior cerebellar cistern. A "spinning-top" configuration of the tentorial incisura, a "cigar-shaped" CSF tract within the interhemispheric fissure, fenestration of the superior sagittal sinus, and "peaking" of the tentorium were associated findings helpful in making this diagnosis. Two of the eight children had findings indistinguishable from focal dermoid, six were developmentally normal, one had mild motor delay, and one died at the age of 3 years. Pathologic examination revealed glial, meningeal (arachnoid), fibrous, and dermal elements.
Conclusion:
Characteristic findings on MR images and CT scans provide clues to the diagnosis of atretic cephalocele. However, even in the presence of abnormal imaging findings, these children may be developmentally normal.