Managing an open nasofrontal encephalocele after birth

Abstract

Encephaloceles are relatively uncommon in western countries. Most of the reported cases involve occipital encephaloceles. Open frontal encephaloceles comprise a rare entity. Most of them will be detected during early prenatal diagnostic, whereas the majority of the pregnancies will be terminated after the consent of the parents. Open frontal encephaloceles pose a great challenge to neurosurgeons as well as anesthesiologists, as these infants present with a microcephaly, non-physiological intracranial anatomy, and low birth weight, thus making the infant prone to excessive blood loss, hypothermia, and death. Neonates born with an incomplete cutaneous coverage are exposed to an imminent threat to life due to the risk of meningitis, necessitating surgical repair in the first days of life. We represent a rare case of an open nasofrontal encephalocele managed surgically in the first day of life. Surgery did not influence the neurological outcome of the patient.

Keywords: Case report; Encephalocele; Nasofrontal.

Fig. 1

Preoperatively MRI Scans. (Sagittal, Axial and Coronal). In the T2-weighted image scans the encephalocele is identified together with the monoventricle. On the coronal slide the 90-degree hemispheric rotation is to be noted

Fig. 2

Preoperatively MR-Angiography

Fig. 3

Intraoperative photographs showing the defect before the operation presenting the (a) nasofrontal encephalocele. Furthermore (b, c) presenting the midfacial incision and use of Raney Clips to avoid blood loss. The skin closure with 5-0 Monocryl sutures

Fig. 4

A series of images showing the intraoperative Steps. At first mobilising the Encephalocele, followed by resecting it en toto. The closure of the bony defect using a bone flap from the parietal bone. Lumbar Drainage used as an external ventricle drainage

Fig. 5

MRI Scan ( axial and coronal ) 6 months after Operation demonstrating the Hydrocephalus