Association of Craniovertebral Junction Anomalies, Klippel-Feil Syndrome, Ruptured Dermoid Cyst and Mirror Movement in One Patient: A Unique Case and Literature Review.

The Klippel-Feil syndrome (KFS) has been reported to be associated with intracranial neoplasms, most frequently epidermoid or dermoid cysts. To our knowledge, however, patients who present with a posterior fossa dermoid cyst (DC) and KFS are extremely rare with only 24 previously reported cases in the English literature worldwide. Therefore, we present the first report of a patient with a craniocervical ruptured DC accompanied by craniovertebral junction (CVJ) anomalies, KFS and mirror movement. Meanwhile, a literature review of KFS accompanying with posterior fossa DC discusses these conditions from the embryological, anatomical, clinical and therapeutic perspectives. Additionally, the combination of CVJ anomalies, KFS and DC may represent a new syndrome that has previously gone unnoticed.

Optimal microsurgical treatment of dorsum sellae meningioma.

BACKGROUND: There are few reports of microsurgical treatment of dorsum sellae meningiomas-which, because of location, size, and differences in growth direction, clinical presentations, degree of surgical difficulty, have varied posttreatment sequelae. In pursuit of an optimal microsurgical treatment option for dorsum sellae meningioma patients, we performed a retrospective analysis of eight microsurgery-treated patients in our set up. METHODS: Clinical data of eight microsurgery-treated dorsum sellae meningioma patients were analyzed. Dorsum sellae meningiomas were classified into 2 types based on tumor location, size, and direction of growth. Type I tumors (dorsum sellae-inferior third ventricle type, four cases) were resected by craniotomy via the frontotemporal or orbitozygomatic approach. Type II tumors (dorsum sellae-third ventricle type, 4 cases) were resected by frontal craniotomy via the transcallosal-interforniceal approach. RESULTS: Complete tumor resection was achieved in all the eight patients. In Type I tumor patients, the only postoperative complication was oculomotor nerve palsy. In Type II tumor patients, the postoperative complications included hyperthermia, electrolyte imbalances, endocrinologic disturbances, and hydrocephalus. The mean follow-up was 2.1 years. Four patients returned to normal life and found a job, two were able to live independently, one required assistance, while one died. CONCLUSIONS: Dorsum sellae meningioma surgery is challenging, and resection of Type II tumors is more difficult than Type I tumors. The selection of a suitable microsurgical approach based on tumor type, and the active treatment of postoperative complications are important means of increasing therapeutic efficacy.