脊髄髄膜瘤，水頭症，脳梁発育不全ならびに巨大な透明中隔腔を伴つたCraniolacuniaの1例

A CRANIOLACUNIA ASSOCIATED WITH A MYELOMENINGOCELE, A HYDROCEPHALUS. A HUGE CAVUM SEPTI PELLUCIDI AND A HYPOPLASIA OF THE CORPUS CALLOSUM:REPORT OF A CASE

I．はじめに

　Craniolacunia （Lueckenschaedel, Lacullarskull）は，頭部単純写で容易に診断される疾患であり，かつ本症だけの場合は治療を必要としないものであるから，めずらしいとはいえないが，他に中枢神経系の発育異常を伴うことが多く，^{1）2）3）5）8）9）}，その高頻度の合併症としては，水頭症，脊髄髄膜瘤といわれており，これら合併症の種類，およびその数によつて，治療ならびに予後に影響を与えるという点について，注目すべき疾患であるとおもう。それは近年，抗生物質，ならびにshunt opera—tionの進歩によつて，水頭症の予後が希望的になつてきているからである。

　われわれは最近，脊髄髄膜瘤，水頭症，脳梁の発育不全と，巨人な透明中隔腔を合併した，craniolacuniaに遭遇し，外科的処置を加えたが，敗血症をおこして生後5カ月半で不幸の転帰をとった1例を経験した。幸いにして剖検の機会が与えられたのでここに報告するしだいである。

A craniolacunia is characterized with multiple, honey comb-like areas of diminished density alter-nating with increased density of the skull ; it is very commonly associated with a hydrocephalus, a my-elomeningocele, a meningocele of the skull and the spine. The authors are reporting our experience with an infant who had a craniolacunia associated with a myelomeningocele, a hydrocephalus, by-poplasia of the corpus callosum, and a huge cavum septi pellucidi.

Case Presention : On December 18, 1967 a female infant of two months of age was admitted to our department with enlargement of the head followingan operation upon myelomeningocele. The circum-ference of the head was 46 cm, The anterior and the posterior fontanels were left open remarkably. The plain skull films showed a craniolacunia. On December 23, 1967 ventricular " bubble " study was performed, revealing a communicating hydrocephalus and a large cavum septi pellucidi. On January 5. 1968 a ventriculo-atrial shunt with Pudenz's valve was carried out via the right internal jugular vein. On the 21 st clay after the operation the patient developed a subcutaneous abscess in the cervical cision area and developed a CSF leakage in the same area on the 56 th day ; the right cardiac tube was removed, then a new cardiac tube being inserted via the left internal jugular vein. Unfor-tunately the infant died of sepsis five and a half months after her birth.

Autopsy disclosed a huge non-communicating cavum septi pellucidi accompanied with a hypoplasia of the corpus callosum.