医学
外科
发育不良
第四脑室
Chiari畸形
磁共振成像
脊髓空洞症
放射科
作者
Maria Luisa Gorgoglione,Roberta Laera,Antonello Curcio,Gerardo Caruso,Gabriele Delia,Antonino Germanò
标识
DOI:10.1016/j.wneu.2023.01.020
摘要
Chiari malformation (CM)-III is the rarest anomaly among CMs.1 Treatment of choice is surgical repair,2 although poor outcome and postoperative mortality has been reported.3 Surgical timing is still debated.4,5 We present the case of a male infant with a prenatal diagnosis of encephalocele. Presentation was characterized by hemodynamic instability, horizontal nystagmus, and left shoulder dystocia due to caesarean section, with a 64 mm × 49 mm × 76 mm soft, fluctuant, and translucent suboccipital-cervical sac. Magnetic resonance imaging revealed a median occipital bone defect with the meningoencephalic sac communicating with the vermian cistern and the fourth ventricle, moderate hydrocephalus, reduction of the posterior cranial fossa volume, hypoplasia of cerebellar hemispheric, vermian structures, and corpus callosum hypoplasia. The patient underwent surgery on day 4 with the use of a 4K 3D ORBEYE exoscope (Video 1). Surgery consisted of disengagement of nervous structures and repair of the neurocutaneous defect, followed on day 12 by a ventriculoperitoneal shunt with a programmable valve. The procedures were well tolerated. At the 14-month follow-up visit he was in range with growth charts (weight, height, and cranic circumference) and gained the physiologic stages of growth. He had no motor impairment but still present were convergent strabismus and mild left C5-C6 radiculopathy, secondary to shoulder dystocia. This is the first case reported in the literature of CM-III treated with the 4K 3D ORBEYE exoscope. Advantages of the exoscope were ergonomic positions for operative staff, possibility for the team to assist in the 4K 3D view, especially in cases with a narrow operative field, with a clear and detailed vision, although a learning curve is required6 to become a valid alternative in pediatric neurosurgery.
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