• 제목/요약/키워드: Sacral agenesis

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Caudal Agenesis : Understanding the Base of the Wide Clinical Spectrum

  • Lee, Ji Yeoun;Shim, Youngbo;Wang, Kyu-Chang
    • Journal of Korean Neurosurgical Society
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    • 제64권3호
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    • pp.380-385
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    • 2021
  • Caudal agenesis refers to the congenital malformation with the essential feature of the agenesis of the sacrococcygeal bone. It is associated with various types of spinal cord anomaly as well as with complex anomalies of genitourinary or gastrointestinal system. The wide spectrum of the disease can be attributed to its pathoembryological origin, the secondary neurulation. This review presents the definition, etiology, classification, and clinical features of the disease.

Imaging Diagnosis of Sacrocaudal Dysgenesis in a Shih-tzu Dog

  • Choi, Soo-Young;Lee, In;Cho, Na-Young;Shin, Bong-Hun;Lee, Ki-Ja;Choi, Ho-Jung;Lee, Young-Won
    • 한국임상수의학회지
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    • 제33권6호
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    • pp.389-391
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    • 2016
  • A 10-month-old, intact female Shih-tzu dog with a pelvic limb ataxic gait, and urinary and fecal incontinence since birth, was examined by computed tomography and magnetic resonance imaging. The butterfly hemivertebra of the fourth lumbar vertebra, agenesis of the third sacral and coccygeal vertebrae, and spina bifida of the remaining sacral vertebra were observed on the computed tomography images. Magnetic resonance imaging revealed traction of the conus medullaris. The dog was diagnosed with sacrocaudal dysgenesis with presumptive tethered cord syndrome, and concurrent infectious cystitis was found by ultrasonography and urinary analysis. The Shih-tzu recovered from the cystitis, but still has the ataxic gait and urinary and fecal incontinence. This report summarizes the case of a dog with sacrocaudal dysgenesis that was identified by imaging diagnosis.

큐라리노 증후군의 임상 경험 (Clinical Experience of Currarino Syndrome)

  • 김태훈;조민정;김대연;김성철;김인구
    • 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.

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