• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제2권1호
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• pp.104-108
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• 1999

Currarino triad is a hereditary condition diagnosed when three abnormalities are noted: (1) an anorectal malformation; (2) an anterior sacral defect and (3) a presacral mass. We experienced 3 cases of Currarino triad with severe constipation. All cases had presacral mass and sacral bony defect. One case had rectovaginal fistula, other one case had holoprosencephaly. Presacral masses were lipomeningomyelocele and epidermoid or dermoid cyst. We report three cases of Currarino triad with brief review of the related literature.

• Journal of Korean Neurosurgical Society
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• 제37권1호
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• pp.63-66
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• 2005

The authors report a case of Currarino triad which had a congenital anorectal stenosis associated with a sacral defect and a presacral mass. A 1-year-old female presented with constipation since birth. Neurological deficits were not found on admission. She had had a diverting colostomy due to anorectal stenosis at another hospital before admission. Lumbar X-ray films showed bony defect caudal to the third sacral vertebra. Magnetic resonance image demonstrated a round cystic pelvic mass which was connected with a dural sac via anterior sacral defect. Posterior approach with Intradural removal of the presacral cystic mass was performed and followed by anoplasty by a pediatric surgeon. The cystic mass was verified histologically as mature teratoma with cystic change. Postoperatively, the urinary function and bowel movement remained intact. Currarino triad should be suspected and evaluated physically and radiographically in a case of congenital anorectal stenosis. Prompt recognition and close cooperation between pediatric surgeons and neurosurgeons is advisable to ensure adequate surgical treatment.

• Advances in pediatric surgery
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• 제17권1호
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• pp.65-71
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• 2011

Currarino syndrome is a hereditary syndrome characterized by the triad of a sacral bony defect, presacral mass and anorectal malformation. We retrospectively reviewed 13 Currarino syndrome patients who were treated in our center between 1997 and 2010. Demographic data, initial symptoms, initial diagnosis. pathologic diagnosis of presacral mass, associated anomalies and managements were analyzed. There were 8 boys and 5 girls. Four patients were diagnosed as Currarino syndrome immediately after birth with failure of passage of meconium and abdominal distension. Four patients underwent surgery for imperforate anus immediately after birth and W8re diagnosed as Currarino syndrome later and underwent reoperation. Three patients were diagnosed during work-up and management with of the tentative diagnosis of Hirschsprung's disease. Diagnosis of the remaining two patients was at the age of 26 months and 9 years and anorectal malformation was not associated. Twelve patients showed hemi-sacrum and one patient showed bilateral sacral subtotal agenesis. Two patients without anorectal malformation underwent presacral mass excision, untethering of spinal cord and repair of myelomeningocele. Six out of 8 patients, excluding 3 that expired or were lost to follow up, with anorectal malformation underwent colostomy, presacral mass excision, untethering of spinal cord, repair of myelomeningocele, posterior sagittal anorectoplasty and colostomy repair. One patient underwent only posterior sagittal anorectoplasty after colostomy. One waits the scheduled operation only with Hegar dilatation. Pathologic examation of presacral masses showed myelomeningoceles in 4 patients, lipomyelomeningoceles in 3 patients and dermoid Cyst in one patient. Teratoma was combined in 2 patients. Eight patients needed neurosurgical operation for spinal cord problems. Seven patients had urologic anomalies and two of them underwent operation. Currarino syndrome should be considered as a differential diagnosis in pediatric patients with abdominal distension, constipation and anorectal malforlnation. For proper evaluation and treatment, a multi-disciplinary approach is recommended.

• Journal of Korean Neurosurgical Society
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• 제37권1호
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• pp.29-33
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• 2005

Objective: Congenital dermal sinus is a rare congenital disease that results from the failure of the neuroectoderm to separate from the surface ectoderm during the process of neurulation, where there is communication between the skin and the deeper structures. Their pathogenesis, clinical course and treatment strategy are well known. We analyze our series and compare our results with other series. Methods: Twenty patients were diagnosed as congenital dermal sinus and confirmed pathologically from October 1986 to July 2003 at our hospital. We studied the patients' clinical manifestations, radiological findings and pathological profiles. Results: Seven cases were located in the suboccipital area and 13 cases were located in the spinal area. Interestingly, 4 of 13 spinal lesion cutaneous openings were located lower than the 3rd sacral body level. 8 of 20 lesions were terminated at neural structures, 4 of 20 lesions were terminated at the intradural portion and others terminated at the extradural portion. Nine anomalies were combined with the dermal sinus, including 4 lipomas, 2 Currarino's triad, 1 encephalocele, 1 myelomeningocele and 1 diastematomyelia. Eleven patients had dermoid tumors. Conclusion: Congenital Dermal Sinus must be surgically removed immediately if they are diagnosed. The surgical procedure of congenital dermal sinus is complete removal, but in some cases, complete removal is impossible. In those cases, we removed all epithelial tissues. We consider sacrococcygeal dimple almost invariably have no connection with intraspinal structures. But, if other cutaneous manifestations are combined with cutaneous pits, it can communicate with the sacrococcygeal dimple.