Erratum: Currarino Triad: Surgical Management and Follow-Up Results of Three Cases

The Currarino syndrome is a rare triad that is a combination of a presacral mass, a congenital sacral bony abnormality and an anorectal malformation. We present 4 children with complete Currarino triad who were diagnosed using MRI. Our aim was to report the neurosurgical management of Currarino syndrome in children. All of the patients had chronic constipation and pain in the lumbosacral region. In the plain radiograph, 3 patients had a sacral scimitar-shaped bony abnormality, and 1 patient had total sacral agenesis. There was a narrow anal canal or narrow ventrally displaced anus in all patients. Their anorectal malformations were characterized as anal stenoses (4 patients), associated with Hirschsprung’s disease in 2 cases. In 3 patients, MRI showed tethered cord syndrome in addition to the presacral mass. There was hydrocephalus in 1 patient. Anal stenosis was treated by anal dilatation. In 2 patients, rectal biopsy and temporary colostomy (2 patients) had been performed previously due to Hirschsprung’s disease. We performed a posterior procedure via lumbar and sacral partial laminectomy-laminoplasty and transdural ligation of the neck of the meningocele for anterior sacral meningoceles, or alternatively, tumor excision for other types of presacral lesions. Histopathologically, 3 were cases of anterior sacral meningoceles and 1 was a teratoma. One of them also had a spinal abscess. He required reoperation (twice) and appeared at the time to have improved with medical therapy. All patients improved and stabilized. There were no additional neurological deficits and no recurrence of the presacral mass over the follow-up period (6 years, on average). The family pedigree did not reveal any familial transmission pattern. In cases of Currarino triad, MRI can allow the characterization of the presacral masses. If it is an anterior sacral meningocele or a solid tumor without severe anorectal malformation, it can be managed with posterior lumbar and sacral procedures. Such approaches are performed easily by transdural ligation of the neck of the anterior sacral meningocele or through tumor excision.

库雷拉综合征（Currarino syndrome）是一种罕见的三联征，由骶前肿物、先天性骶骨骨质异常及肛肠畸形三者联合构成。本研究纳入4例经磁共振成像（MRI）确诊的完全性库雷拉三联征患儿，旨在探讨儿童库雷拉综合征的神经外科治疗方案。所有患者均存在慢性便秘及腰骶部疼痛。平片检查显示，3例患者存在骶骨弯刀状骨质异常，1例患者为完全性骶骨发育不全。所有患者均存在肛管狭窄或肛门腹侧移位。患者的肛肠畸形表现为肛门狭窄（4例），其中2例合并先天性巨结肠（Hirschsprung’s disease）。3例患者的MRI检查除骶前肿物外，还可见脊髓栓系综合征（tethered cord syndrome）；1例患者合并脑积水。肛门狭窄采用肛门扩张术治疗。2例患者此前因先天性巨结肠接受过直肠活检及临时结肠造口术。针对骶前脊膜膨出（meningocele）患者，本研究采用腰骶部部分椎板切除-椎板成形术经硬膜内结扎脊膜膨出颈口的后路手术方案；对于其他类型骶前病变，则采取肿瘤切除术。组织病理学检查显示，3例为骶前脊膜膨出，1例为畸胎瘤（teratoma）。其中1例患者还合并脊柱脓肿，需接受2次再次手术，经药物治疗后病情有所改善。所有患者的病情均得到改善并趋于稳定。平均随访6年期间，未出现新增神经功能缺损，骶前肿物也无复发。家系调查未发现明确的家族遗传模式。对于库雷拉三联征患者，MRI可明确骶前肿物的性质。若为骶前脊膜膨出或无重度肛肠畸形的实体肿瘤，可采用腰骶部后路手术治疗，此类手术可通过经硬膜结扎骶前脊膜膨出颈口或肿瘤切除术顺利完成。