Nasi Davide, Dobran Mauro, di Somma Lucia, Santinelli Alfredo, Iacoangeli Maurizio
Department of Neurosurgery, Università Politecnica delle Marche, Umberto I General Hospital, Ancona, Italy.
Section of Pathological Anatomy and Histopathology, Department of Neuroscience, Università Politecnica delle Marche, Umberto I General Hospital, Ancona, Italy.
Case Rep Neurol. 2019 Jan 30;11(1):24-31. doi: 10.1159/000496419. eCollection 2019 Jan-Apr.
Metastases involving the clivus and craniocervical junction (CCJ) are extremely rare. Skull base involvement can result in cranial nerve palsies, while an extensive CCJ involvement can lead to spinal instability. We describe an unusual case of clival and CCJ metastases presenting with VI cranial nerve palsy and neck pain secondary to CCJ instability from metastatic bladder urothelial carcinoma. The patient was first treated with an endoscopic endonasal approach to the clivus for decompression of the VI cranial nerve and then with occipitocervical fixation and fusion to treat CCJ instability. At the 6-month follow-up, the patient experienced complete recovery of VI cranial nerve palsy. To the best of our knowledge, the simultaneous involvement of the clivus and the CCJ due to metastatic bladder carcinoma has never been reported in the literature. Another peculiarity of this case was the presence of both VI cranial nerve deficit and spinal instability. For this reason, the choice of treatment and timing were challenging. In fact, in case of no neurological deficit and spinal stability, palliative chemo- and radiotherapy are usually indicated. In our patient, the presence of progressive diplopia due to VI cranial nerve palsy required an emergent surgical decompression. In this scenario, the extended endoscopic endonasal approach was chosen as a minimally invasive approach to decompress the VI cranial nerve. Posterior occipitocervical stabilization is highly effective in avoiding patient's neck pain and spinal instability, representing the approach of choice.
累及斜坡和颅颈交界区（CCJ）的转移瘤极为罕见。颅底受累可导致脑神经麻痹，而广泛的CCJ受累可导致脊柱不稳定。我们描述了一例不寻常的斜坡和CCJ转移瘤病例，该病例表现为因转移性膀胱尿路上皮癌导致CCJ不稳定继发的Ⅵ脑神经麻痹和颈部疼痛。患者首先接受了经鼻内镜入路至斜坡的手术，以解除Ⅵ脑神经受压，然后进行枕颈固定融合术以治疗CCJ不稳定。在6个月的随访中，患者的Ⅵ脑神经麻痹完全恢复。据我们所知，文献中从未报道过转移性膀胱癌同时累及斜坡和CCJ的情况。该病例的另一个特殊之处在于同时存在Ⅵ脑神经功能缺损和脊柱不稳定。因此，治疗方法和时机的选择具有挑战性。实际上，在没有神经功能缺损和脊柱稳定的情况下，通常采用姑息性化疗和放疗。在我们的患者中，由于Ⅵ脑神经麻痹导致进行性复视，需要紧急手术减压。在这种情况下，选择扩大经鼻内镜入路作为一种微创方法来解除Ⅵ脑神经受压。枕颈后路稳定术在避免患者颈部疼痛和脊柱不稳定方面非常有效，是首选的治疗方法。
