Cranium bifidumおよびその特殊型Cephalocele basalisについて

CRANIUM BIFIDUM AND ITS SPECIAL TYPE (CEPHALOCELE NASOPHARYNGEALIS)

I．緒言

　中枢神経系をおかす先天奇形ないし発生異常にはいろいろの種類があり，これらのうちには成人期になつて発症するものもあるが，多くは乳幼児期，ないし小児期に見出される。

　Cephalocele, cranium bifidumは出生と同時に容易に発見されるが，正しい治療をうけないと種々の合併症を併発しやすい。特に鼻腔内に発展したcephaloceleは，鼻腔内腫瘍と間違えられやすく，誤つた治療をうけ，死の転帰をとることが少なくなかつた。

Since 1942, 33 cases of cephalocele were en-countered in this department, in which 26 cases were meningocele and 7 cases were meningoencephalocele. The incidence was 23 cases in male baby and 10 cases in female baby. Twenty-eight cases were cepha-locele occipitalis. There were a case of cephalocele frontalis, a case of cephalocele parietalis, a case of cephalocele sagittalis, a case of cephalocele fronto-ethmoidalis (nasofrontalis) and a case of cephalocele nasopharyngealis (transethmoidalis). There was no case of cephalocele lateralis.

Cephalocele tends to accompany various congenital abnormalities. Twenty-two cases were found to ac-company various malformations. Hydrocephalus and mental retardation were the major important com-plications. Among 26 cases of meningocele, there were only 7 cases with hydrocephalus, but there were 3 cases with hydrocephalus among 7 cases of menin-goencephalocele.

Operation was performed on 32 cases. Ventric-ulopleurostomy or ventriculoatriostomy was per-formed on 5 cases which accompanied progressive hydrocephalus.

The mortality rate directly attributable to surgery for cephalocele was 3.1%.

In follow-up study over 5 years of 26 cases, 6 cases (23.1%) died, disturbance of mental or physical devel-opment was found in 4 cases (15.4%) and the other 14 cases (53.8%) have lived in good condition. In 4 cases of meningoencephalocele, 3 cases were died and one case showed disturbance of mental and physical developement.

A case of cephalocele nasopharyngealis (trans-ethmoidalis) of 7 month old male baby was reported in the present paper. In the left nasal cavity was found a reddish compressible and fluctuating tumor which turned out to contain C. S. F. By tomography, this radiopaque tumor was found to occupy the anterior half of the left nasal cavity.

Under general anesthesia, bilateral frontal cranio-tomy was performed and a oval bone about 5 mm in diameter was disclosed in the lamina cribrosa.

A dura flap obtained at the edge of the bone defect was reflected to cover the defect and was sutured with falx cerebri. The intranasal meningocele did not disappear but diminished in size postoperatively. Literatures related to cephalocele nasopharyngealis were reviewed.