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先天性腰骶部脂肪瘤。

Congenital lumbosacral lipomas.

作者信息

Pierre-Kahn A, Zerah M, Renier D, Cinalli G, Sainte-Rose C, Lellouch-Tubiana A, Brunelle F, Le Merrer M, Giudicelli Y, Pichon J, Kleinknecht B, Nataf F

机构信息

Department of Pediatric Neurosurgery, Groupe Hospitalier Necker-Enfants Malades, Paris, France.

出版信息

Childs Nerv Syst. 1997 Jun;13(6):298-334; discussion 335. doi: 10.1007/s003810050090.

Abstract

Congenital lumbosacral lipomas can be responsible for progressive defects. The general feeling is that tethering of roots, filum, or cord probably explains this evolution, and that untethering of these structures could prevent late deterioration. Like the vast majority of neurosurgeons, we too have routinely and systematically operated on lumbosacral lipomas, even in the absence of neurological deficits. This policy stemmed from our belief that spontaneous neurological deterioration was frequent, recovery from preoperative deficits rare, and surgery both efficient and benign in nature. After 22 years of experience, we felt that it was necessary to review our series of 291 lipomas (38 lipomas of the filum and 253 of the conus) operated on from 1972 to 1994. To reassess the value of prophylactic surgery, we attempted an accurate evaluation of (1) the risk of pathology, (2) the risks involved in surgery, (3) the postoperative outcome with respect to preoperative deficits, and (4) the postoperative outcome in asymptomatic patients at 1 year and at maximum follow-up. Special attention was paid to 93 patients whose postoperative follow-up was more than 5 years (average 8.7, median 8, range 5-23 years). Of these 93 patients, 39 were asymptomatic preoperatively (7 with lipoma of the filum and 32 with lipoma of the conus). Lipomas of the filum and of the conus are entirely different lesions and were studied separately. In 6 cases prenatal diagnosis had been possible. The mean age at surgery was 6.4 years. Low back skin stigmata were present in 89.4% of cases. Preoperative neurological deficits existed in 57% of the patients and were congenital in 22%. Clinical signs and symptoms recorded were pain in 13.3% of the patients and/or neurological deficits affecting sphincter (52%), motor (27.6%) and sensory (22.4%) functions. Deficits were progressive in 22.4% of cases, slowly progressive in 58.8% of these and rapidly progressive in the remaining 41.2%. In 36 patients (13.2%) the lipomas were seen to grow either subcutaneously or intraspinally. Among these patients, 21 were infants, 2 were obese adolescents, and 10 were pregnant women. The metabolism of the fat within the lipomas was studied in 11 patients and found to be similar to that at other sites. Lipomas were associated with various other malformations, either intra- or extraspinal. These associated anomalies were rare in the case of lipomatous filum (5.2%) but frequent with lipomas of the conus, except for intracranial malformations (3.6%). Therapeutic objectives were spinal cord untethering and decompression, sparing of functional neural tissue and prevention of retethering. Procedures used to achieve these goals were subtotal removal of the lipoma, intraoperative monitoring, duroplasty, and sometimes closure of the placode. Histologically, lipomas consisted of normal mature fat. However, 77% of them also included a wide variety of other tissues, originating from ectoderm, mesoderm, or entoderm. This indicates that lipomas are either simple or complex teratomas. The results of the study are as follows. (1) Surgery was easy and safe when performed for treatment of lipomas of the filum (no complications), but difficult and hazardous in the case of lipomas of the conus (20% local, 3.9% neurological complications). (2) All types of deficit could be improved by surgery, which was beneficial in all cases of lipoma of the filum and 50% of cases of lipoma of the conus. (3) In asymptomatic patients long-term surgical results depended on the anatomical type of the lipoma. They were excellent in lipomas of the filum. In lipomas of the conus they were good in the short term but eroded with time. At more than 5 years of follow-up only 53.1% of the patients were still free of symptoms. (4) Reoperations were performed in 16 patients (5.5%), 5 (31.2%) of whom improved postoperatively, while in 7 (43.7%) progression stopped, in 3 (18.7%) deterioration continued and in 1 (6.2%) the condition was wor

摘要

先天性腰骶部脂肪瘤可导致进行性缺陷。一般认为神经根、终丝或脊髓的拴系可能是这种病情发展的原因,解除这些结构的拴系可防止病情后期恶化。和绝大多数神经外科医生一样,我们也常规且系统地对腰骶部脂肪瘤进行手术,即便患者没有神经功能缺损。这一策略源于我们的信念,即神经功能自发恶化很常见,术前缺损恢复罕见,且手术本质上有效且安全。经过22年的经验积累,我们觉得有必要回顾一下我们在1972年至1994年间手术治疗的291例脂肪瘤(38例终丝脂肪瘤和253例圆锥脂肪瘤)。为重新评估预防性手术的价值,我们试图准确评估:(1)病变风险;(2)手术相关风险;(3)术后相对于术前缺损的结果;(4)无症状患者术后1年及最长随访期的结果。我们特别关注了93例术后随访超过5年的患者(平均8.7年,中位数8年,范围5 - 23年)。在这93例患者中,39例术前无症状(7例终丝脂肪瘤,32例圆锥脂肪瘤)。终丝脂肪瘤和圆锥脂肪瘤是完全不同的病变,分别进行了研究。6例患者进行了产前诊断。手术时的平均年龄为6.4岁。89.4%的病例存在腰骶部皮肤体征。57%的患者术前存在神经功能缺损,其中22%为先天性缺损。记录的临床症状和体征包括13.3%的患者有疼痛和/或影响括约肌(52%)、运动(27.6%)和感觉(22.4%)功能的神经功能缺损。22.4%的病例缺损呈进行性,其中58.8%进展缓慢,其余41.2%进展迅速。36例患者(13.2%)的脂肪瘤可见于皮下或椎管内生长。这些患者中,21例为婴儿,2例为肥胖青少年,10例为孕妇。对11例患者脂肪瘤内脂肪的代谢进行了研究,发现与其他部位相似。脂肪瘤与各种其他脊柱内或脊柱外畸形相关。这些相关畸形在终丝脂肪瘤中很少见(5.2%),但在圆锥脂肪瘤中很常见,颅内畸形除外(3.6%)。治疗目标是解除脊髓拴系、减压、保留功能性神经组织并防止再次拴系。用于实现这些目标的手术步骤包括脂肪瘤次全切除、术中监测、硬脊膜成形术,有时还包括封闭神经基板。组织学上,脂肪瘤由正常成熟脂肪组成。然而,其中77%还包含多种其他组织,起源于外胚层、中胚层或内胚层。这表明脂肪瘤要么是单纯性要么是复杂性畸胎瘤。研究结果如下:(1)手术治疗终丝脂肪瘤时简单且安全(无并发症),但治疗圆锥脂肪瘤时困难且有风险(局部并发症20%,神经并发症3.9%)。(2)所有类型的缺损均可通过手术改善,手术对所有终丝脂肪瘤病例和50%的圆锥脂肪瘤病例有益。(3)无症状患者的长期手术结果取决于脂肪瘤的解剖类型。终丝脂肪瘤的结果极佳。圆锥脂肪瘤短期内结果良好,但随时间推移逐渐变差。随访超过5年时,只有53.1%的患者仍无症状。(4)16例患者(5.5%)进行了再次手术,其中5例(31.2%)术后改善,7例(43.7%)病情进展停止,3例(18.7%)病情继续恶化,1例(6.2%)病情恶化。

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