Roszkowski Marcin, Drabik Krzysztof, Grajkowska Wiesława, Jurkiewicz Elzbieta, Daszkiewicz Paweł
Kliniki Neurochirurgii, Instytutu Pomnika Centrum Zdrowia Dziecka w Warszawie.
Neurol Neurochir Pol. 2003;37(4):847-60.
In the study surgical indications are reviewed and the effect of the applied surgical technique on long-term treatment outcome in children with large cystic brainstem cavernous malformations is evaluated.
Clinical data of 5 patients treated surgically for large cystic cavernous malformations of the pons in the years 1995-2001 were retrospectively evaluated. In all the cases the transsylvian approach to the ventrolateral pons with splitting of the crus cerebri was used to reach cavernoma within the cystic cavity. The long-term follow up (mean 4.9 years) included a complete neurological examination and annual MRI studies.
At least one hemorrhagic episode was diagnosed in all the cases, while bleeding to the pons, with clinical course mimicking that of stroke, was noted twice in 3 patients. All the episodes confirmed by MRI imaging appeared to be intralesional. The malformations ranged in size from 30 to 50 mm (mean 36.9 mm). Their most characteristic MRI features were not only the localization in the pontine structure exclusively i.e. within the natural anatomical barriers separating the pons from the midbrain and medulla, but also the growth pattern corresponding to that of brainstem benign gliomas. The patients' mean preoperative KPS score was 60 (30 to 90). Cavernous hemangiomas were totally removed in 3 out of 5 cases. One patient with partially removed lesion presented after a year recurrent hemorrhage and new neurological deficits that required a second stage surgery. In all but one case transient complications included some new cranial nerve, motor and sensory deficits that influenced the patients' immediate postoperative KPS assessment, with the mean score of 48. In a follow-up assessment at 1.5 to 7 years, a significant amelioration of both their neurological status and quality of life was found, with KPS scores from 70 to 90 (mean score 80). Annual MRI examinations showed extensive hemosiderin deposits within the pontine tissue in 3 cases of totally removed cavernous hemangiomas and this image was stable during the follow-up period.
The findings suggest that resection of large pontine cavernomas in children is indicated in cases of symptomatic hemorrhage with lesions approaching the pial surface, or surrounded by a small margin of normal tissue. Recurrent hemorrhages are intralesional (i.e. limited to pontine structures by the natural anatomical barriers), resulting in a "cyst-like" growth of malformations mimicking that of focal neoplasms and in compression of the brain stem tissue rather than in a direct hemorrhagic insult.
本研究回顾手术指征,并评估所应用的手术技术对患有大型囊性脑干海绵状畸形儿童的长期治疗效果。
回顾性评估1995年至2001年间接受手术治疗的5例桥脑大型囊性海绵状畸形患者的临床资料。在所有病例中,采用经侧裂入路至桥脑腹外侧,劈开大脑脚,以到达囊性腔内的海绵状血管瘤。长期随访(平均4.9年)包括全面的神经系统检查和每年的MRI检查。
所有病例均诊断出至少一次出血事件,3例患者中有2例出现桥脑出血,临床过程类似中风。MRI成像证实的所有出血事件似乎均为瘤内出血。畸形大小范围为30至50毫米(平均36.9毫米)。其最典型的MRI特征不仅是仅位于桥脑结构内,即在将桥脑与中脑和延髓分隔开的自然解剖屏障内,还包括与脑干良性胶质瘤相对应的生长模式。患者术前平均KPS评分为60(30至90)。5例中有3例海绵状血管瘤被完全切除。1例病变部分切除的患者在一年后出现复发出血和新的神经功能缺损,需要进行二期手术。除1例病例外,所有病例的短暂并发症均包括一些新的颅神经、运动和感觉功能缺损,这影响了患者术后即刻的KPS评估,平均评分为48。在1.5至7年的随访评估中,发现患者的神经状态和生活质量均有显著改善,KPS评分从70至90(平均评分80)。每年的MRI检查显示,3例完全切除海绵状血管瘤的病例中,桥脑组织内有广泛的含铁血黄素沉积,且该影像在随访期间保持稳定。
研究结果表明,对于有症状性出血、病变靠近软脑膜表面或被小范围正常组织包绕的儿童大型桥脑海绵状血管瘤,建议进行手术切除。复发出血为瘤内出血(即受自然解剖屏障限制在桥脑结构内),导致畸形呈“囊肿样”生长,类似局灶性肿瘤,并压迫脑干组织,而非直接的出血性损伤。