A Morphological Classification of Sincipital Encephalomeningoceles
A Morphological Classification of Sincipital Encephalomeningoceles
sincipital encephalomeningoceles
1 2 3 4 5 6
7 8 9
0Go10 11 12
FIG. I. Drawings to illustrate the findings in all 12 patients. The appearance and location of the mass
are shown in the upper row, the defects of the bones of the face in the middle row. The intracranial open-
ings in Cases 1 through 10 and the defects of the cranial vault in Cases 11 and 12 are shown in the lower
row,
1 mo. M glabella =x2x5 thin & shiny skin FE 2.5X2.5 small (2 X2 X3 small frontal lobes, long
thin wall of sac NF cm) quaddgeminal plate &
aqueduct
2 mos. F glabella 5X3X2 ulcerated, infec- FE 3X3 moderate right unilateral hydro-
ted, thin wall of NF (3 X4 X4 cm) cephalus, reverse direc-
sac tion of optic nerve &
internal carotid artery,
long brain stem, abnor-
mal position of falx
cerebri
2 mos, M glabella lOXl0Xl5 thin&shiny skin, FE 4 X2 about one-half fairly well preserved
(neck 5 X2) thin wall of sac NF cortical gyri, stretched
cortex & brain stem
I.
3 inos. F wide base of 0.5XI X l . 5 thin skin over FE 2 X2 small (1 X2X2 bilateral hydrocephalus,
nose with mass, shiny at NE cm) elongated brain stem
I right mass cyst
2 yrs F both sides of 2XI XI thin skin over FE 2 X3 moderate right unilateral hydro-
base of nose mass, shiny at NE (2 X2 X4 cm) cephalus, elongated
cysts brain stem, abnormal
position of falx cerebri
10 1 too. M both infra- 2 X I X0.5 normal skin, thick FE; NO minimal normal
orbital 1.5 XI XO.5 wall of sac O. 7 X0.7 left (olfactory bulb)
0.4X0.2 right
II 8 days F midforehead 7 X5 X2 normal skin, thick interfrontal moderate normal cortex, con-
wall of sac 6X7 (2 X6 X6 era) stdcted ring at frontal
lobes
12 newbn. F whole head 15XIOX8 thin & shiny, righl craniofacial more than half severe damage, partly
5X4X3 normal, left 5X6 macerated, two constric-
tions at cerebrum
two patients had a thin skin covering which from the size of a fingertip to almost half of
had ruptured and become infected. The last the brain (Fig. 6). When a large amount of
two patients had cystic masses covered with brain was herniated, the cortical gyri and
thin and shiny skin. sulci converged at the neck of the opening.
Upon dissection of the brain, one patient Certain areas of cortex were smooth with
(Case 1) had a single cerebrum without a faint or no convolutions. The cerebral blood
falx cerebri or corpus callosum, that is, a ho- vessels also followed the brain into the herni-
lotelencephaly (Fig. 5). The remaining five ation. In one patient, the anterior communi-
patients had herniation of both frontal tips cating artery was displaced to the level of the
and the intervening falx cerebri into the sac. crista galli (Fig. 7). The olfactory apparatus
The amount of the herniated brain varied was almost invariably herniated and the ol-
FIG. 5. Case 1. Superior view (left) and coronal section (right) of the brain in an 8-day-old boy with a
nasofrontal encephalomeningocele showing holotelencephaly.
rotation of the brain. Unilateral hydrocepha- ally seen on top of the ethmoidal bone in the
lus was seen in two of these five patients. depths of the hole. The lateral wall, which at
The herniated portion of the brain under- times was membranous instead of bony, was
went variable degrees of change, from one formed by the medial wall of the orbit (Fig.
that was rather well preserved to one that 8).
had become a firm nodular mass of adherent In this subgroup, the neck of the encepha-
scar. lomeningocele was long. Its intracranial ori-
In one patient (Case 5) the falx cerebri fice was between the frontal and ethmoidal
attached to the tentorium cerebelli along the bones, and its outer end lay between the na-
left apex of the petrous pyramid instead of sal bones and the nasal cartilage. The dis-
the median portion. The right cerebral hemi- tance between the nasal bones and the nasal
sphere, which was hydrocephalic, was larger
than the left one and occupied the right half
as well as the posterior portion of the left
half of the cranial cavity. The superior sagit-
tal sinus also deviated to the left and lay
close to the point of attachment of the falx
cerebri.
Naso-ethmoidaI Type (Cases 7 through 9)
The cranial defect in three patients was
between the nasal bones and the nasal carti-
lage as seen from the face. The nasal bones
and a portion of the frontal process of the
maxillary bone were attached to the frontal
bone above the sac of the encephalomenin-
gocele, forming the anterosuperior wall of
the canal for the sac. The posteroinferior Fro. 6. Case 6. Brain showing constriction in the
middle corresponding to the neck of a nasofrontal
wall was formed by the nasal cartilage and encephalomeningocele. About one-half of the brain
nasal septum which attached superiorly to was outside the cranial cavity. Note the rather well-
the ethmoidal bone. The crista galli was usu- formed cortical gyri.