| Autor: |
Yengo-Kahn, Aaron M., Plackis, Andreas C., Bonfield, Christopher M., Reddy, Srijaya K. |
| Zdroj: |
BMJ Case Reports; 3/31/2020, Vol. 13 Issue 3, p1-2, 2p, 2 Color Photographs, 1 Black and White Photograph |
| Abstrakt: |
An encephalocele is a neural tube defect characterised by the displacement of cranial contents outside of the skull. Often, these congenital malformations are due to a primary axial mesodermal defect and occur in 0.8–3.0 of 10000 live births. 1 2 In rare cases, encephaloceles are manifestations of amniotic band syndrome (ABS), best defined as multiple and often-diverse secondary deformities caused by the formation of thin membranous bands in utero. 3 While encephaloceles related to primary neural tube defects are often found at a midline occipital or frontal location, ABS-related encephaloceles may involve multiple defects with variable locations. 1 2 The craniofacial defects associated with ABS usually involve nasal deformity and asymmetry. In contrast to typical encephaloceles, those related to ABS may only be covered with rudimentary meninges rather than true skin. 4 Correction of the lesion is necessary to allow for more normal brain growth and development, as the presence of the excess tissue may differentially drive cranial skeleton formation. 1 2 Empiric antibiotic therapy and surgical correction must be undertaken promptly to reduce the risk of infection. 2 Operative objectives include removal of the herniated cranial contents, closure of the dural defect and closure of the cranial defect, when feasible. Large encephaloceles can present a challenge to the neurosurgeon, especially in terms of positioning and obtaining a watertight dural closure. We present a case of a large atypical vertex encephalocele in a premature neonate with associated complex craniofacial malformations believed to be related to ABS. [ABSTRACT FROM AUTHOR] |
| Databáze: |
Complementary Index |
| Externí odkaz: |
|
