影像学诊断Currarino综合征

Imaging diagnosis of Currarino syndrome

目的探讨Currarino综合征的影像学表现。方法回顾性分析经手术证实的9例Currarino综合征患儿的临床及影像学资料。对9例患儿均行腹部X线平片及消化道造影(或瘘管造影),8例行MSCT扫描,5例行骶尾部MR扫描。结果 9例中,6例为肛门闭锁,其中4例伴直肠会阴瘘;3例为肛门直肠狭窄。6例患儿一侧骶骨缺如,表现为"弯刀征";2例表现为椎体融合;1例表现为骶尾骨短小,发育不良。5例骶前包块为畸胎瘤,4例为骶前脊髓脊膜膨出伴脊髓栓系。结论 Currarino综合征临床少见,但具有特征性影像学表现。对Currarino综合征患儿进行影像学检查并发现肛门直肠畸形时,应注意骶尾骨发育情况及有无骶前包块。

Objective To investigate imaging performances of Currarino syndrome. Methods Clinical and imaging data of 9 Currarino syndrome patients confirmed by operation and pathology were retrospectively analyzed. All patients underwent KUB and gastrointestinal contrast （or fistula angiography）, 8 underwent abdominal MSCT, 5 underwent sacrococcygeal MR scanning. Results In all patients, 6 had anal atresia, including 4 with scaphoid fossa fistula, 3 with anoreetal stenosis. Six patients showed sacral agenesis on one side, displaying as ＂scimitar syndrome＂, 2 showed partial bone interbody fusion, 1 showed stunted sacrocoecygeal bone. Presacral mass was proved teratoma in 5 patients, while in 4 patients were proved anterior sacral myelomeningoeele with tethered cord. Conclusion. Currarino syndrome is a rare disorder with characteristic imaging features. When anorectal malformation was detected, sacral bone development and presacral mass should be considered.