Samples Derek C, Thoms Dewey J, Tarasiewicz Izabela
Department of Neurological Surgery, University of Texas Health San Antonio, 7703 Floyd Curl Drive MC7843, San Antonio, TX, 78229, USA.
Childs Nerv Syst. 2018 Aug;34(8):1549-1555. doi: 10.1007/s00381-018-3787-9. Epub 2018 Apr 2.
Chiari I malformation, defined as herniation of the cerebellar tonsils at least 5 mm below the foramen magnum, can result from congenital or acquired pathology. While the mechanism is not well understood, an association between Chiari I and cystic fibrosis has been described in the literature. The lifelong respiratory status management necessitated by cystic fibrosis creates a greater risk of Chiari symptomatology as well as post-operative CSF-related complications in the setting of duraplasty secondary to recurrent transient increases in intracranial pressure. We will review the literature, describe our experience with these patients, and propose bony decompression as an approach to treatment.
A retrospective review of pediatric patients treated at our institution with both cystic fibrosis and Chiari I was performed. Since our first case in 2016, our department has evaluated four patients carrying that dual diagnosis. All four underwent posterior fossa decompression surgery. Two patients had incidental pathology. Two symptomatic patients exhibited headaches and/or coordination difficulty. Half of the patients had associated syringomyelia. All patients were offered posterior fossa decompression utilizing intraoperative ultrasound.
All four patients underwent posterior fossa decompression without duraplasty. Average operative time was 128 min. There were no complications post-operatively. Average hospital stay was 3.8 days. Average surgical length of stay was 2.3 days. Morbidity and mortality were 0%. The longest follow-up to date is 20 months. The two asymptomatic patients remained so post-operatively. The child with headaches and imbalance had complete resolution of his symptoms after surgery, as did the toddler with headaches. Both patients with syringomyelia demonstrated significant decrease in the size of their syrinxes on imaging performed at least 3 months post-operatively.
Based on the literature and our experience, we recommend considering posterior fossa decompression without duraplasty as treatment for pediatric cystic fibrosis patients with Chiari I malformation. This approach can be effective for symptomatic and prophylactic cases in this particular patient demographic because their comorbidities predispose them to Chiari pathology and symptomatology as well as certain post-operative complications.
Chiari I畸形定义为小脑扁桃体疝至枕大孔以下至少5毫米，可由先天性或后天性病变引起。虽然其机制尚不完全清楚，但文献中已描述了Chiari I与囊性纤维化之间的关联。囊性纤维化所必需的终身呼吸状态管理，在因颅内压反复短暂升高继发硬脑膜成形术的情况下，会增加Chiari症状以及术后脑脊液相关并发症的风险。我们将回顾文献，描述我们对这些患者的治疗经验，并提出骨减压作为一种治疗方法。
对在我们机构接受囊性纤维化和Chiari I治疗的儿科患者进行回顾性研究。自2016年我们的第一例病例以来，我们科室评估了四名患有双重诊断的患者。所有四人都接受了后颅窝减压手术。两名患者有偶然发现的病变。两名有症状的患者表现出头痛和/或协调困难。一半的患者伴有脊髓空洞症。所有患者均在术中超声引导下接受后颅窝减压手术。
所有四名患者均接受了后颅窝减压手术，未进行硬脑膜成形术。平均手术时间为128分钟。术后无并发症。平均住院时间为3.8天。平均手术住院时间为2.3天。发病率和死亡率均为0%。迄今为止最长的随访时间为20个月。两名无症状患者术后仍无症状。患有头痛和平衡障碍的儿童术后症状完全缓解，患有头痛的幼儿也是如此。两名脊髓空洞症患者在术后至少3个月进行的影像学检查中，脊髓空洞大小均显著减小。
基于文献和我们的经验，我们建议考虑对患有Chiari I畸形的儿科囊性纤维化患者采用不进行硬脑膜成形术的后颅窝减压术作为治疗方法。这种方法对于该特定患者群体的有症状和预防性病例可能有效，因为他们的合并症使他们易患Chiari病变和症状以及某些术后并发症。
