A Clinicopathological Classification Of Encephalocoeles Based On 207 Patients

Encephalocoeles are relatively rare congenital abnormalities. There have been a few classifications of encephalocoeles, but these are predominantly anatomical. A more clinical classification system would assist in planning treatment, surgical procedures and assessing outcomes.

All encephalocoeles presenting at the Craniofacial Unit at Inkosi Albert Luthuli Central Hospital were reviewed. There were 207 patients with 224 encephalocoeles. The clinical presentation and CT findings were analysed and used to group these encephalocoeles.

There were five distinct groups with some having subgroups.1.Cranial (n= 43). These were located on the calvarium and were subdivided into subgroups according to their anatomical location. They are occipital, parietal, frontal, temporal and acrania.2.Nasal (n= 122). These were located in the nasal region and were classified into two large subgroups (supranasal and infranasal) depending on whether the pathway and defect were above or below the nasal bones.3.Orbital (n= 21). These presented with the displacement of the globe and were subdivided into two subgroups: anterior and posterior.4.Basal (n= 11). The pathway of these encephalocoeles was through the floor of the anterior cranial fossa often with no visible deformity of the face.5.Cleft related (n= 27). The pathway of these encephalocoeles was through an existing craniofacial cleft.

This classification system demonstrated good clinicopathological correlation. This allowed one to better appreciate the pathway and assess concomitant deformities. It also directed one to plan the procedure and detail the surgical corrections required to produce satisfactory outcomes.