Beuriat Pierre-Aurélien, Szathmari Alexandru, Rousselle Christophe, Sabatier Isabelle, Di Rocco Federico, Mottolese Carmine
Department of Pediatric Neurosurgery, Hôpital Femme Mère Enfant, Lyon, France.
Department of Pediatric Neurosurgery, Hôpital Femme Mère Enfant, Lyon, France.
World Neurosurg. 2017 Dec;108:62-68. doi: 10.1016/j.wneu.2017.08.152. Epub 2017 Sep 1.
It was believed that Chiari type II malformation (CM-II) was always present in a myelomeningocele (MMC). In fact, it is associated in about 80% of cases. Improvement of the hindbrain herniation after prenatal closure of MMC has challenged the idea that this condition was irreversible. Only 2 studies report ascent of the cerebellar tonsil after postnatal closure. This work aimed to study a large group of patients with MMC who benefited from a postnatal repair to evaluate the rate of long-term total reversibility of CM-II.
Sixty-one patients were included. Mean time of follow-up was 8.1 years. The presence of CM-II after closure of the MMC was assessed on the most recent brain scan available for each patient.
Forty-seven patients (77%) had a CM-II at birth (confirmed before the MMC repair). There was a significant correlation between the level of the malformation and the presence of a CM-II at birth (P = 0.003). After MMC closure, only 28 (45.9%) patients had a remaining CM-II. The reversibility rate was 40.4%. The reversibility was higher in lower level malformations (P = 0.004). The number of patients treated for hydrocephalus was significantly higher in the group of patients with remaining CM-II (P = 0.004). Only 11.5% of the children needed surgery for a symptomatic CM-II.
MMC is not always associated with CM-II. The outcome of CM-II has improved. Postnatal closure can reverse the CM-II. This must be kept in mind when analyzing the result of prenatal series.
过去人们认为脊髓脊膜膨出（MMC）患者总是伴有Chiari II型畸形（CM-II）。事实上，约80%的病例存在这种关联。产前闭合MMC后脑疝的改善对这种情况不可逆转的观点提出了挑战。仅有两项研究报告了产后闭合MMC后小脑扁桃体上移的情况。本研究旨在对一大组受益于产后修复的MMC患者进行研究，以评估CM-II长期完全可逆的比例。
纳入61例患者。平均随访时间为8.1年。根据每位患者最新的脑部扫描评估MMC闭合后CM-II的存在情况。
47例患者（77%）出生时即存在CM-II（在MMC修复前确诊）。畸形程度与出生时CM-II的存在之间存在显著相关性（P = 0.003）。MMC闭合后，仅有28例（45.9%）患者仍存在CM-II。可逆率为40.4%。较低程度畸形的可逆率更高（P = 0.004）。仍存在CM-II的患者组中接受脑积水治疗的患者数量显著更多（P = 0.004）。仅有11.5%的儿童因有症状的CM-II需要手术治疗。
MMC并非总是与CM-II相关。CM-II的预后有所改善。产后闭合可使CM-II逆转。在分析产前系列研究结果时必须牢记这一点。
