Department of Neurosurgery, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Department of Otorhinolaryngology-Head and Neck Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
Childs Nerv Syst. 2024 Dec;40(12):3915-3921. doi: 10.1007/s00381-024-06595-2. Epub 2024 Sep 2.
Non-hypothalamic glioneural hamartomas are rare entities known to cause medically refractory epilepsy. Olfactory bulb hamartomas, in particular, are exceptionally rare.
We describe a case of an olfactory bulb hamartoma that was surgically resected at our institution. We also performed a literature review of all glioneural hamartomas and discuss the clinical presentation, diagnosis, and management of these lesions.
Herein, we present the unusual case of a typically developing 17-year-old boy with a near life-long history of drug-resistant epilepsy, found to have a 0.8 × 1.0 cm right olfactory bulb hamartoma. Endoscopic endonasal trans-cribriform resection of the lesion led to seizure freedom in the 6-month follow-up period (Engel class 1 outcome). Comprehensive literature review revealed only one other sporadic case, which was also successfully treated with total surgical resection.
Our case of an olfactory bulb hamartoma adds to the limited literature currently available, illustrating key clinical characteristics of these exceedingly rare lesions and outlining an effective, minimally invasive, and low-morbidity treatment strategy.
非下丘脑神经胶质神经细胞瘤是一种罕见的实体,已知会导致药物难治性癫痫。特别是嗅球神经胶质细胞瘤极其罕见。
我们描述了一例在我院行手术切除的嗅球神经胶质细胞瘤。我们还对所有神经胶质细胞瘤进行了文献复习,并讨论了这些病变的临床表现、诊断和治疗。
在此,我们介绍了一例不典型的病例,一名通常发育正常的 17 岁男孩,有近一生的药物难治性癫痫病史,发现右侧嗅球有 0.8×1.0 厘米的神经胶质细胞瘤。经鼻内镜经筛状突切除病变,术后 6 个月随访时癫痫无发作(Engel 分级 1 级)。全面的文献复习仅发现另一例散发性病例,也通过完全手术切除成功治疗。
我们的嗅球神经胶质细胞瘤病例增加了目前有限的文献,说明了这些极其罕见病变的关键临床特征,并概述了一种有效、微创和低并发症的治疗策略。