Noshita N, Shirane R, Yoshimoto T, Onuma T
Department of Neurosurgery, Tohoku University School of Medicine.
No Shinkei Geka. 1997 Jun;25(6):549-53.
We report a case of "functional aqueductal stenosis" which reveals dilatation of the lateral and 3rd ventricles without stenosis at the aqueduct in MRI. This case shows a pineal teratoma which presents one year later with symptoms of hydrocephalus caused by "functional aqueductal stenosis". A seven-year-old boy was admitted to our department owing to headache and vomiting. CT and MRI showed hydrocephalus. The lateral and 3rd ventricles were dilated while the 4th ventricle was normal. Furthermore, tumoral obstruction of the aqueduct was not found. After a ventriculoperitoneal shunt, he recovered well without neurological deficits. One year later, symptoms of precocious puberty, that is the appearance of public hair and deepening of his voice, were found. A follow-up MRI demonstrated a pineal region tumor. Although human chorionic gonadotropin level in the serum and urine was transiently elevated, it normalized before surgery. The operation was performed by the occipital transtentorial approach and the tumor was totally removed. Histological examination proved this tumor to be a mature teratoma, showing three germ cell layers. About two weeks later, he was discharged without any neurological deficit. In this case, although hydrocephalus occurred, MRI didn't demonstrate aqueductal obstruction caused by the tumor. However, one year later, a pineal region tumor was confirmed by MRI. This suggests that hydrocephalus might have some association with the appearance of the pineal region tumor. Therefore, it is necessary to be aware of the possibility of the occurrence of tumors whenever we encounter "functional aqueductal obstruction", when MRI doesn't demonstrate aqueductal obstruction caused by a tumor.
我们报告一例“功能性导水管狭窄”病例，该病例在MRI上显示侧脑室和第三脑室扩张，而导水管无狭窄。此病例显示为松果体畸胎瘤，一年后出现由“功能性导水管狭窄”导致的脑积水症状。一名7岁男孩因头痛和呕吐入院。CT和MRI显示脑积水。侧脑室和第三脑室扩张，而第四脑室正常。此外，未发现导水管有肿瘤阻塞。脑室腹腔分流术后，他恢复良好，无神经功能缺损。一年后，发现性早熟症状，即阴毛出现和声音变低沉。随访MRI显示松果体区有肿瘤。虽然血清和尿液中的人绒毛膜促性腺激素水平短暂升高，但在手术前恢复正常。手术采用枕下经小脑幕入路，肿瘤被完全切除。组织学检查证实该肿瘤为成熟畸胎瘤，可见三个胚层。大约两周后，他出院，无任何神经功能缺损。在本病例中，虽然发生了脑积水，但MRI未显示肿瘤导致的导水管阻塞。然而，一年后，MRI证实了松果体区肿瘤。这表明脑积水可能与松果体区肿瘤的出现有一定关联。因此，当我们遇到“功能性导水管阻塞”且MRI未显示肿瘤导致的导水管阻塞时，有必要意识到发生肿瘤的可能性。
