The image-guided transoral approach (IGTOA) provides a safe exposure to skull base midline lesions and the ventral aspect of the craniovertebral junction (CVJ). The IGTOA has several advantages: the head being placed in the extended position, it decreases the brainstem angulation during surgery; the approach being done through the avascular median pharyngeal raphe, not only lowers the bleeding risk but also provides a direct access to the bony pathology and granulation tissue accessible only via the ventral route. Wide field exposure and maneuverability are necessary to deal with the entire ventral brainstem compression in case of severe CVJ malformation to safely perform partial clivectomy and odontoidectomy.

We illustrate the cases of two patients, 52-year-old and 42-year-old males, who presented with an impressive craniovertebral junction malformation, confirmed on CT and MRI images. They first underwent surgery by IGTOA, later completed by occipitocervical fixation. For the two patients, outcomes were assessed respectively at 4 and 5 years and showed satisfactory results both clinically and radiologically.

In patients with marked ventral compression, the IGTOA provides direct and guided access to the anterior aspect of the CVJ and effective means for odontoidectomy and clivectomy. This approach is more easily maneuverable compared to the endonasal endoscopic approach. The IGTOA approach is quite a complex technique, requiring multidisciplinary skills, but it should primarily be used in difficult situations. We suggest that endonasal endoscopy is over-utilized. We consider that endoscopy should not be routinely performed and kept only for well-selected cases.