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库里亚里诺三联征:肛门直肠畸形、骶骨骨质异常和骶前肿块——11例病例回顾

Currarino triad: anorectal malformation, sacral bony abnormality, and presacral mass--a review of 11 cases.

作者信息

Lee S C, Chun Y S, Jung S E, Park K W, Kim W K

机构信息

Department of Pediatric Surgery, Seoul National University Children's Hospital, Korea.

出版信息

J Pediatr Surg. 1997 Jan;32(1):58-61. doi: 10.1016/s0022-3468(97)90094-4.

DOI:10.1016/s0022-3468(97)90094-4
PMID:9021570
Abstract

Currarino et al, in 1981, described an association of a congenital anorectal stenosis, or another type of low anorectal malformation, an anterior sacral defect, and presacral mass. Eleven patients with this anomaly were treated at the Department of Pediatric Surgery, Seoul National University Children's Hospital from 1984 to 1995. Among these patients, low-type imperforate anus (IA) was seen in three cases and anorectal stenosis was present in eight cases. Presacral masses included seven teratomas, two meningoceles, one dermoid cyst, and one enteric cyst with dermoid cyst. All had a deformed sacrum. Among the eight with anorectal stenoses, posterior sagittal anorectoplasty (PSARP) with diverting colostomy was performed in seven cases, and repeated rectal dilatation was performed in one case. Among the three low-type IA, anoplasty was performed in two cases and PSARP was performed in one case. Although PSARP is a safe and satisfactory method facilitating the excision of the presacral mass, in meningoceles the repair should be performed before correction of anorectal malformation because of the risk of meningitis that can occur when surgeries are done simultaneously. In two cases, untethering of a tethered spinal cord was performed. All patients are continent. Because the incidence of Currarino triad is high when there is an anorectal stenosis (38% in the present series), the Currarino triad should be suspected in anorectal stenosis. Magnetic resonance imaging is a preferred diagnostic method because the incidence of association of tethered cord in this triad is high (18% in the present series).

摘要

1981年,库里亚里诺等人描述了一种先天性肛门直肠狭窄或另一种类型的低位肛门直肠畸形、骶骨前部缺损和骶前肿块的关联。1984年至1995年期间,首尔国立大学儿童医院小儿外科治疗了11例患有这种异常的患者。在这些患者中,3例为低位无肛畸形(IA),8例存在肛门直肠狭窄。骶前肿块包括7例畸胎瘤、2例脊膜膨出、1例皮样囊肿和1例伴有皮样囊肿的肠囊肿。所有患者的骶骨均有畸形。在8例肛门直肠狭窄患者中,7例行后矢状位肛门直肠成形术(PSARP)并加做转流性结肠造口术,1例行反复直肠扩张术。在3例低位IA患者中,2例行肛门成形术,1例行PSARP。虽然PSARP是一种安全且令人满意的方法,有助于切除骶前肿块,但对于脊膜膨出,由于同时进行手术时可能发生脑膜炎的风险,应在纠正肛门直肠畸形之前进行修复。有2例患者进行了脊髓栓系松解术。所有患者均能自主控制排便。由于存在肛门直肠狭窄时库里亚里诺三联征的发生率较高(本系列中为38%),因此在肛门直肠狭窄时应怀疑库里亚里诺三联征。磁共振成像(MRI)是首选的诊断方法,因为该三联征中脊髓栓系的关联发生率较高(本系列中为18%)。

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