This 15-yr-old girl presented with nasal obstruction and dysphagia of duration 3 mo and 8 to 10 pounds of weight loss. On examination, she had a hoarse voice and left… Click to show full abstract
This 15-yr-old girl presented with nasal obstruction and dysphagia of duration 3 mo and 8 to 10 pounds of weight loss. On examination, she had a hoarse voice and left tongue deviation without weakness or myelopathy. Computed tomography (CT) demonstrated an erosive lesion arising from the clivus and left occipital condyle. Magnetic resonance imaging (MRI) demonstrated a T1-isointense, T2-hyperintense, enhancing mass centered at the occipital condyle and extending into the craniovertebral junction (CVJ), causing severe brainstem compression and extending inferiorly to C2 and anteriorly into the retropharyngeal space. The patient underwent transoral biopsy to confirm the diagnosis of chordoma and complete tumor resection via a left extreme lateral transodontoid (ELTO) approach. This approach was chosen because it provides bilateral exposure to the ventral CVJ and retropharyngeal space and allows for complete tumor removal using a single approach, although it requires an experienced surgeon. The ELTO incision should provide adequate exposure for occipitocervical fusion (OCF) after the destabilization of the CVJ. Transposition of the vertebral artery and odontoidectomy are key maneuvers that provide exposure to the ventral CVJ bilaterally. Dural closure is performed primarily and augmented with fat, fibrin glue, and temporary cerebrospinal fluid diversion. Postoperative MRI showed a gross-total resection and decompression of the brainstem at the CVJ. The patient remained in a cervical collar until OCF. Postoperatively, she had left vocal cord paralysis and moderate weakness with left arm abduction at the deltoid. At 2-mo follow-up, she had improved lower cranial neuropathies, tolerated oral intake, and was scheduled to begin proton beam therapy. The patient provided consent for publication.
               
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