Multiple-site neural tube defects: embryogenesis with complete review of existing literature.

OBJECTIVE: Multiple-site neural tube defects (MNTDs) are very uncommon, with the predominant number of cases being reported in developing countries. The classic theory of neural tube closure fails to explain the occurrence of these defects. Multisite closure theory, first proposed in 1995, explains most of the occurrences with a few modifications specific to a few defects. In this paper, the authors endeavor to explain all the defects, along with their genetic and embryological bases, and to review the available literature and discuss their own experience in the management of these complex cases. METHODS: The authors retrospectively reviewed the data of all the patients treated surgically for MNTDs over that past 14 years. All possible demographic data, clinical details, and radiological imaging data were reviewed. In addition, surgical parameters, complications, and status at follow-up of more than 12 months were evaluated. All previously reported cases of MNTD were analyzed, and comparisons with the present series were made. RESULTS: A total of 3 major series (including the present one) on MNTDs have been from India. A total of 57 such cases (including those of the present series) have been reported in the available literature. While previous series reported a higher incidence of spinal defects, the present series had a higher rate of cephalic defects (55%). Among the reported cases, insertion of a ventriculoperitoneal shunt was necessary in 12 (26%), and only 4 patients were operated on in 2 stages. Neurological status at presentation dictated outcome. CONCLUSIONS: MNTDs are extremely rare, and their embryogenesis is different from that of single neural tube defects. Simultaneous repair of 2 or even 3 defects is possible in a single-stage surgery. The requirement of a shunt is uncommon, and complications following surgery are rare. Folic acid supplementation may reduce the incidence of defects.

Surgical implementation and efficacy of endoscopic endonasal extradural posterior clinoidectomy.

OBJECTIVEThe endoscopic endonasal approach (EEA) for skull base tumors has become an important topic in recent years, but its use, merits, and demerits are still being debated. Herein, the authors describe the nuances and efficacy of the endoscopic endonasal extradural posterior clinoidectomy for maximal tumor exposure.METHODSThe surgical technique included extradural posterior clinoidectomy following lateral retraction of the paraclival internal carotid artery and extradural pituitary transposition. In cases with prominent posterior clinoid process, a midline sellar dura cut was added to facilitate extradural exposure. Forty-four consecutive patients, in whom this technique was performed between 2016 and 2018 at Osaka City University Hospital, were reviewed. The pathology included 19 craniopharyngiomas, 7 chordomas, 6 meningiomas, 6 pituitary adenomas, 4 chondrosarcomas, and 2 miscellaneous. Utilization and effectiveness of this approach were further demonstrated with neuroimaging.RESULTSExtradural posterior clinoidectomies were successfully applied in all patients without permanent neurovascular injury and with better maneuverability and greater resection rate of the tumors. Four patients experienced transient postoperative abducens nerve paresis, and 1 patient experienced transient postoperative oculomotor nerve paresis; however, the patients with deficits recovered within 3 months. On radiological examination, the surgical field was 2.2 times wider in cases with bilateral posterior clinoidectomy than in cases without posterior clinoidectomy.CONCLUSIONSThe extended EEA with extradural posterior clinoidectomy creates an extra working space and allows adequate accessibility with safe surgical maneuverability to remove tumors that extend behind the posterior clinoid and dorsum sellae.

Ipsilateral Hemichorea-hemiballism in a Case of Postoperative Stroke.

BACKGROUND: Ipsilateral hemiballismus refers to the rare occurrence of hemiballism developing on the same side of a brain lesion. CASE REPORT: We describe a rare case of postoperative ipsilateral hemiballism in a patient who underwent pituitary adenoma resection and experienced a right internal cerebral artery territory infarct. We review the literature on hemichorea hemiballismus (HCHB) and explore various mechanisms for its occurrence. DISCUSSION: Only three cases of ipsilateral hemiballism have been described, and the exact pathophysiology remains unknown. A dominant left hemisphere with corpus callosal connections to the right basal ganglia is the most probable explanation for this unusual event.