Preoperative evaluation and surgical management of the Arnold-Chiari II malformation

The authors report their recent experience with 14 meningomyelocele patients with the Arnold-Chiari II malformation. Three major types of fourth ventricle anomalies seen in the Arnold-Chiari II malformation are defined, based on preoperative magnetic resonance imaging and intraoperative ultrasound studies. The Type A deformity is defined as no cystic dilatation of the fourth ventricle. In the Type B anomaly, there is intracranial dilatation of the fourth ventricle. The Type C deformity involves intraspinal dilatation of the fourth ventricle, either dorsal to the cord or within the substance of the cord. The Type A deformity was most common in infants, and in two cases progression from a Type A to Type B deformity was documented. Recognition of the type of Arnold-Chiari II malformation aids in designing an operative approach more specific to that structural abnormality. Intraoperative ultrasound is a valuable adjunct in localization of the underlying anomalies and permits safe decompression of the fourth ventricle. The authors' indications for surgery now include failure to thrive due to either early respiratory and swallowing dysfunction, progressive spasticity, or upper-extremity weakness. Nine patients significantly improved following surgery and three patients with a progressively deteriorating course were stabilized by surgery. Decompression of the fourth ventricle by fenestration and internal shunting appears to be well tolerated, even in young infants, and is recommended in the treatment of the Arnold-Chiari II deformity.