It would appear, therefore, that a cephalocele results from the incomplete cutting off of the neural canal in the head region of the embryo from the overlying epiblast, with consequent fusion between the primary neural tube (from which the brain is developed) with the primordial mesoblastic membranous cranium (from which the membranes are developed), and with the overlying epiblastic layer (from which the epidermal portion of the scalp is developed). In consequence of this localized fusion of layers, the outer dermic coat (from which the membranous skull is developed) fails to form. Bony defects therefore exist through which brain-matter protrudes, the projecting portion often being intimately attached to the skin, and containing not only epiblastic elements, but also mesoblastic tissue from irregular occlusion of the same. The mesoblastic involvement is proved by the presence in the tumour of muscle tissue, vessels, &c.

Position of the tumour.

The tumour may project through the vault or base of the skull. In the former case, it is almost invariably situated in close relation to the middle line of the skull, from nasion to below the inion.

Fig. 20. An Occipital Cephalocele. (For further description, see text.)

1. Occipital cephaloceles—the commonest variety—occupy, anatomically, two positions (1) between the two lower segments of the occipital bone (inferior occipital cephaloceles), often involving the foramen magnum and sometimes complicated by a condition of cervical spina bifida, and (2) between the two upper segments of the occipital bone (superior occipital cephaloceles), occasionally involving the posterior fontanelle.

The tumour may possess a broad base or may be definitely pedunculated. In the former instance the gap in the bone may be of considerable size and the margins everted: in the latter case, the hole may be quite small.

The deformity is frequently associated with other congenital defects—hydrocephalus, microcephalus, spina bifida, hare lip, hernia, and talipes.

2. Sincipital cephaloceles occur next in order of frequency. The tumour projects between the nasal bones and the nasal process of the superior maxilla (naso-frontal), between the nasal process of the maxilla and the orbital plates of the ethmoid (naso-ethmoidal), or between the nasal bones (nasal).