Escobar-Moreno Julio, Ramirez-Loera Cristopher, Durán-Villalobos Alfonso, Reyes Navarro Daniel Alberto, Ruiz-Treviño Armando S
Neurological Surgery, High Specialty Regional Hospital Bajio, León, MEX.
Cureus. 2024 Dec 5;16(12):e75177. doi: 10.7759/cureus.75177. eCollection 2024 Dec.
Intradural extramedullary bronchogenic cysts (IEBCs) are exceedingly rare congenital entities, composed of respiratory epithelial cells derived from the anomalous development of the embryonic foregut. Due to their exceptionally low morbidity, only limited cases are available. Consequently, the clinical features and optimal therapeutic approach remain poorly understood. We report a very unusual case of a 54-year-old Mexican male who initially presented with paraparesis seven years ago, leading to a significant gait disturbance. Conservative therapy was employed, with notable improvements seen after four months of treatment. No additional ambulation assistance was required over the following six years, reflecting a stable progression of the condition. However, the patient experienced a worsening recurrent paraparesis, along with episodic dorsal pain in the last year. Physical examination revealed a reduction in lower extremity strength, with preservation of sensory function. Magnetic resonance imaging (MRI) showed an intraspinal, intradural extramedullary lesion in the dorsal region, extending for at least 50% of the canal. Areas of hyperintensity on the T2 sequence, with a fusion of vertebral bodies at the T3-T4 levels, were observed. A posterior dorsal approach was performed with T2-T4 laminectomy, durotomy, and resection of the cystic lesion, draining the cyst and removing the capsule. Histopathology from the capsule reported a ciliated cyst morphologically characterized by bronchogenic features; immunohistochemistry revealed positivity for cytokeratin cocktail AE1/AE3. Follow-up MRI showed no residual lesion, without further complications. Surgical resection is the most effective treatment for intraspinal bronchogenic cysts, providing significant symptom relief. Complete removal is ideal, while partial resection may reduce complications in cases with severe adhesions; however, it increases the risk of recurrence. Due to the rarity of the disease, the number of cases is relatively limited. Future studies should strongly consider employing a larger sample size and extending the follow-up period to better understand the spectrum of the disease.
硬脊膜内髓外支气管源性囊肿（IEBCs）是极其罕见的先天性病变，由胚胎前肠异常发育衍生的呼吸道上皮细胞组成。由于其发病率极低，仅有有限的病例报道。因此，其临床特征和最佳治疗方法仍知之甚少。我们报告了一例非常罕见的病例，一名54岁的墨西哥男性，7年前最初表现为双下肢轻瘫，导致明显的步态障碍。采用了保守治疗，治疗4个月后有显著改善。在接下来的6年里无需额外的行走辅助，表明病情进展稳定。然而，患者在去年出现了复发性双下肢轻瘫加重，伴有发作性背痛。体格检查发现下肢力量减弱，但感觉功能保留。磁共振成像（MRI）显示脊髓背侧有一个硬脊膜内髓外病变，至少占据椎管的50%。在T2序列上观察到高信号区，T3 - T4水平椎体融合。采用后路背侧入路，进行T2 - T4椎板切除术、硬脊膜切开术和囊性病变切除术，引流囊肿并切除囊壁。囊壁的组织病理学报告显示为一个形态上具有支气管源性特征的纤毛囊肿；免疫组织化学显示细胞角蛋白混合抗体AE1/AE3呈阳性。随访MRI显示无残留病变，无进一步并发症。手术切除是治疗脊髓支气管源性囊肿最有效的方法，能显著缓解症状。完全切除是理想的，而在粘连严重的病例中部分切除可减少并发症；然而，这会增加复发风险。由于该疾病罕见，病例数量相对有限。未来的研究应大力考虑采用更大的样本量并延长随访时间，以更好地了解该疾病的全貌。
