Lewis Heidi,  Kucera Jennifer

Final Pr. ID: Poster #: CR-033

Atretic cephalocele is a rare type of neural tube defect that occurs in the parietal or occipital scalp. Most cases present postnatally with a scalp nodule which may also have associated skin and hair abnormalities. We will present a case of atretic cephalocele diagnosed with fetal MRI as well as two additional cases of postnatally diagnosed atretic cephalocele in different patients.

A 26 week gestational age male fetus was found to have ventriculomegaly on routine OB ultrasound and was referred to maternal fetal medicine. Further ultrasound imaging demonstrated features suspicious for a posterior fossa encephalocele. A fetal MRI was then performed which demonstrated imaging findings consistent with an atretic cephalocele.

The imaging findings associated with atretic cephalocele including embryologic positioning of the straight sinus, the “spinning top” appearance of the tentorial incisura, and the “cigar shaped” CSF tract in the interhemispheric fissure will be illustrated in the exhibit using both fetal and postnatal MRI. Although the exact embryological development of atretic cephaloceles is not completely understood, the proposed mechanisms will be discussed. A brief summary of the associated syndromes and clinical outcomes reported in the literature will be presented.

Atretic cephalocele is a rare condition that is usually diagnosed postnatally with several characteristic imaging findings. These same abnormalities can also be seen on fetal MRI which can lead to an earlier diagnosis and more appropriate family counseling. Read More

Authors:  Lewis Heidi , Kucera Jennifer

Keywords:  atretic cephalocele, Fetal MRI

Ortega García Diana,  Rodríguez Garza Claudia,  Canales Nañez Sofìa,  Terán Helda Estrella,  Montemayor Martínez Alberto

Final Pr. ID: Poster #: CR-005

Atretic cephalocele is a rare disruption of neural tube closure. The reported incidence ranges from 1 in 3500 to 1 in 5000 live births. Is a small subscalp lesion that consist of dura, fibrous tissue, and dysplastic brain tissue. Its most common presentation it is a palpable midline parietal soft tissue mass, occasionally occipital, with a wide range of clinical presentations, the patient may have normal neurological development or have severe mental retardations, since it could be associated with multiple intracranial malformations including a fenestrated superior sagittal sinus (the most common), persistent falcine sinus, vertical embryonic positioning of the straight sinus, holoprosencephaly, hydrocephalus, eye anomalies, agenesis of the corpus callosum, among others, therefore is important to know about this condition as well as to identify its radiological characteristics using different diagnostic imaging methods to make a certain diagnostic. Transfontanellar brain ultrasound could be used as the first imaging method as it enables a non-invasive method, in addition the patients are not exposed to ionizing radiation, being the most consistent finding a subcutaneous, hypoechoic mass, with a solid fibrous tract connecting the extracranial lesion, through a small bony cranium defect, to the intracranial space; additionally in order to evaluate a further extension of the fibrous tract or the presence of other anomalies, CT or MRI can be performed, considering to the MRI as a better study because it can be used in pediatric population since there is no exposure to radiation. Main differential diagnoses of atretic cephalocele are sinus pericranii and dermoid cyst, other possible causes include haemangioma, sebaceous cyst, inclusion cyst, lipoma, tumors (Langerhans cell histiocytosis, sarcomas, etc.), bone and soft tissue infections and traumatic lesions. This educational poster pretends to review the radiological findings of two cases of patients with atretic cephalocele moreover a literature review since the published works regarding is limited and few case series or case reports have been documented. Read More

Authors:  Ortega García Diana , Rodríguez Garza Claudia , Canales Nañez Sofìa , Terán Helda Estrella , Montemayor Martínez Alberto

Keywords:  ATRETIC CEPHALOCELE, CYST, SCALP MASS