• Childhood Kidney Diseases
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• 제1권2호
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• pp.155-160
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• 1997

목적 : Tethered cord 증후군은 척수내 지방종이나 종사의 섬유대 등에 의해 척수 원추가 천골에 부착, 견인됨으로 하지의 신경 결함이나 족부의 변형을 일으키는 질환군으로 척추 유합부전증의 경우에서 흔히 나타난다. 신경학적 소견 외에 빈뇨, 요실금, 유뇨증, 급뇨, 배뇨 감각 장애 등의 비뇨기계 증상도 높은 빈도로 나타난다고 하며 수술적 치료의 예후는 조기에 진단될수록 양호하다고 알려져 있다. 그러나 tethered cord 증후군에서 나타나는 비뇨기계 증상 및 소견에 관하여 체계적으로 보고된 것이 많지 않다. 저자들은 tethered cord 증후군에서의 비뇨기계 증상을 여러 특징을 파악하여 tethered cord 증후군 환자에서의 조기 진단 및 치료 방침 설정에 도움을 얻고자 본 연구를 시행하였다. 대상 및 방법 : 1991년 11월부터 1997년 7월까지 5년 9개월간 영남 대학 병원에서 tethered cord 증후군으로 진단받은 소아 환자 9례를 대상으로 연령 및 성별분포, 비뇨기계 이상에 관한 증상 및 비정상 검사소견의 여부와 수술 후 경과에 대하여 의무 기록을 조사하는 후향적 연구로 조사하였다. 결 과: 1) 대상 환아 9례의 연령 분포로는 0-2세가 5례, 2-6세가 1례. 6-10세가 3례였으며 남아가 2례, 여아는 1례였다. 2) 요실금, 빈뇨, 유뇨증, 급뇨, 배뇨 감각 장애 등의 비뇨기계 증상을 가진 경우가 6례였으며, 비뇨기계의 방사선학적 검사에서 신경성 방광을 보인 경우가 5례, 수신증과 수뇨관증이 동반된 경우가 3례, 방광-요관 역류를 보인 경우가 3례였다. 3) 요동력학적 검사는 3례에서 시행되었으며 3례 모두에서 이상 소견이 발견되었다. 4) 진단시 비뇨기계 증상 및 이상 검사 소견을 가진 7례 중 5례에서 detethering이 행해졌으나 증상의 호전은 1례에서만 관찰되었다. 결론 : Tethered cord 증후군에서 비뇨기계의 증상 및 비정상 검사 소견이 동반되는 경우가 흔하며 비뇨기계의 이상을 시사하는 증상이 없는 경우에도 검사상 이상이 발견되는 경우가 적지 않다. Tethered cord 증후군 환아의 평가는 신경학적 이상 유무는 물론이고 비뇨기계의 이상 여부에 대한 평가도 필히 포함되어야 할 것으로 생각된다.

• Journal of Korean Neurosurgical Society
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• 제54권4호
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• pp.363-365
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• 2013

Split cord malformations (SCMs) usually present in childhood, and are rarely reported in adults. And also, a cervicothoracic SCM associated with tethered cord syndrome has very rarely been reported in the literature. We report a case of SCM associated with tethered cord and spina bifida in an adult. This report describes the case of a 34-year-old woman who presented for evaluation of neck pain, back pain, and intermittent paraparesis of several months duration. The MRI and CT showed a SCM at the cervicothoracic level and a fibrous septum at the thoracic level. She underwent surgery for the SCM and tethered cord syndrome, and was followed for 7 years. Patient presented complete recovery in the follow-up. The authors discuss this unusual lesion and describe the anatomical relationship of the level of cord duplication and fibrous septum.

• Childhood Kidney Diseases
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• 제6권1호
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• pp.109-113
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• 2002

저자들은 야뇨증을 주소로 내원한 12살 남아에서 천추 부위의 피부 보조개를 발견하여 정밀 검사를 통한 계류 척수 증후군을 확인하였고 이에 따른 신경원성 방광과 만성 신부전을 경험하였기에 문헌 고찰을 통한 증례 보고를 하는 바이다.

• Journal of Korean Neurosurgical Society
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• 제39권4호
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• pp.281-285
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• 2006

Objective : The tethered cord syndrome results in progressive neurological deficits. Although it may remain controversial, many physicians recommend definitive surgery to untether the cord as soon as this condition is identified. We retrospectively evaluate the pre-operative and post-operative course of 38 tethered cord patients with spinal dysraphism in an attempt to learn the natural history of the disease and to determine the effectiveness of the surgical treatment. Methods : The medical records, operation notes and radiographs were evaluated. The follow up period ranged from 4 months to 12 years with a mean follow-up of 28.6 months. Twenty-seven patients were younger than 15 years of age. Results : At presentation, 26 of the patients were asymptomatic. In three of 11 adult symptomatic patients, their neurological deficits worsened after trauma or exercise. Improvement of motor strength was documented in two out of 5 patients. Five of nine patients with bladder symptoms improved, however, none had a complete return of their bladder function. Conclusion : Childhood patients were less symptomatic than the adult patients. Adult patients showed progression of their symptoms that were not improved even after the operation in most of the cases. Asymptomatic tethered spinal cord can be symptomatic as time passes by and even at an old age. Future research should be focused on the operative methods to prevent the delayed deterioration after surgery, rather than on the usefulness of preventive surgery in asymptomatic patients.

• Journal of Korean Neurosurgical Society
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• 제63권3호
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• pp.314-320
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• 2020

Retained medullary cord (RMC) is a relatively recent term. Pang et al. newly defined the RMC as a late arrest of secondary neurulation leaving a non-functional vestigial portion at the tip of the conus medullaris. RMC, which belongs to the category of closed spinal dysraphism, is a cord-like structure that is elongated from the conus toward the cul-de-sac. Because intraoperative electrophysiological confirmation of a non-functional conus is essential for the diagnosis of RMC, only a tentative or an assumptive diagnosis is possible before surgery or in cases of limited surgical exposure. We suggest the term 'possible RMC' for these cases. An RMC may cause tethered cord syndrome and thus requires surgery. This article reviews the literature to elucidate the pathoembryogenesis, clinical significance and treatment of RMCs.

• 대한근전도전기진단의학회지
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• 제20권2호
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• pp.119-123
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• 2018

A 14-year-old student presented with a 2-year history of progressive left foot deformity. High elevated medial arch, hindfoot varus deformity and second to fourth claw toes were idenfied in the left foot without a familial history. Neurologic examinations showed left distal lower extremity weakness and bilateral increased deep tendon reflexes. MRI of whole spine demonstrated thickened filum terminale and spinal defect covered with pulled skin which findings consistent with tethered cord syndrome (TCS). He was referred to neurosurgery department and had a detethering operation of the spinal cord. Two years later, he underwent foot surgery because his foot deformity progressed despite the detethering operation. It is very rare for TCS to present with pes cavus as the only symptom, also in Korea. We suggest that TCS should be considered as one of the differential diagnoses associated with unilateral pes cavus in adolescence so as not to miss the proper period of surgery.

• Journal of Korean Neurosurgical Society
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• 제63권3호
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• pp.342-345
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• 2020

Some types of spinal dysraphism can be accompanied by extraspinal cysts, including myelomeningocele, myelocystocele, myelocele, meningocele, limited dorsal myeloschisis, lipomyelomeningocele, and terminal myelocystocele. Each disease is classified according to the developmental mechanism, embryologic process, site of occurrence, or internal structure of the extraspinal cyst. In most cystic spinal dysraphisms except meningocele, part of the spinal cord is attached to the cyst dome. Most open spinal dysraphisms pose a risk of infection and require urgent surgical intervention, but when the cyst is accompanied by closed spinal dysraphism, the timing of surgery may vary. However, if the extraspinal cyst grows, it aggravates tethering by pulling the tip of the cord, which is attached to the dome of the cyst. This causes neurological deficits, so urgent surgery is required to release the tethered cord.

• Journal of Korean Neurosurgical Society
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• 제46권5호
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• pp.468-471
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• 2009

Objective : The adolescent presentation of tethered cord syndrome (TCS) is well-recognized, but continues to pose significant diagnostic and management controversies. The authors conducted a retrospective study of clinical outcomes after surgical intervention in 24 school-aged children, adolescents, and young adults with TCS. Methods : All 83 patients with a lipomyelomeningocele (LMMC) underwent untethering surgery for caudal cord tethering between 1987 and 2007. The clinical charts and follow-up data were reviewed. Of these patients, 24 school-aged children, adolescents, and young adults with TCS were studied with respect to the clinical, radiologic, pathologic features, and surgical outcomes. Results : Untethering procedures were performed in 24 patients (age range, 7-25 years) for TCS of various origins (lipoma, lipomyelomeningocele, and tight filum terminale). Specific circumstances involving additional tugging of the already tight conus, and direct trauma to the back precipitated the onset of symptom in 50% of the patients. Diffuse and non-dermatomal leg pain, often referred to the anorectal region, was the most common presenting symptom. Progressive sensorimotor deficits in the lower extremities, as well as bladder and bowel dysfunction, were also common findings, but progressive foot and spinal deformities were noted less frequently. The most common tethered lesions were intradural lipomas, thickened filum and fibrous band adhesions into the placode sac. The surgical outcome was gratifying in relation to pain and motor weakness, but disappointing with respect to resolution of bowel and bladder dysfunction. Of the 24 patients with TCS, pre-operative deficits improved after surgery in 14 (58.3%). remained stable in 8 (33.4%). and worsened in 2 (8.3%). Conclusion : The pathologic lesions of tethered cord syndrome in school-aged children, adolescents, and young adults, are mostly intradural lipomas and tight filum. It is suggested that the degree of cord traction results in neurologic dysfunction in late life due to abnormal tension, aggravated by trauma or repeated tugging of the conus during exercise. Early diagnosis and adequate surgical release might be the keys to the successful outcome in school-aged children, adolescents, and young adults with TCS.

• Clinical and Experimental Pediatrics
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• 제62권2호
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• pp.68-74
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• 2019

Purpose: To investigate the prevalence of occult spinal dysraphism (OSD) and subsequent neurosurgery in pediatric patients with isolated or combined dorsal midline cutaneous stigmata with or without other congenital malformations. Methods: We carried out a retrospective review of patients who underwent sonography or magnetic resonance imaging (MRI) for OSD because of suspicion of dorsal midline cutaneous stigmata (presumed to be a marker for OSD) between January 2012 and June 2017. Information about patient characteristics, physical examination findings, spinal ultrasound and MRI results, neurosurgical notes, and accompanying congenital anomalies was collected. Results: Totally 250 patients (249 ultrasound and one MRI screening) were enrolled for analysis. Eleven patients underwent secondary MRI examinations. The prevalence of OSD confirmed by an MRI was 2.4% (6 patients including one MRI screening). Five patients (2%) had tethered cord and underwent prophylactic neurosurgery, 3 of whom had a sacrococcygeal dimple and a fibrofatty mass. Prevalence of tethered cord increased as markers associated with a sacrococcygeal dimple increased (0.5% of the isolated marker group, 8.1% of the 2-marker group, and 50% of the 3-marker group). Incidence of OSD with surgical detethering in 17 other congenital anomaly patients was 11.8%, which was higher than the 1.3% in 233 patients without other congenital anomalies. Conclusion: Our results suggest that the presence of dorsal midline cutaneous stigmata, particularly fibrofatty masses, along with a sacrococcygeal dimple is associated with OSD or cord tethering requiring surgery. OSD should be suspected in patients with concurrent occurrence of other congenital anomalies.

• Journal of Korean Neurosurgical Society
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• 제42권2호
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• pp.77-82
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• 2007

Objective : The authors tried to reveal some unique features of lipomeningomyelocele (LMMC), including clinical presentation, factors precipitating onset of symptoms, pathologic entities of LMMC associated with tethered cord syndrome, and surgical outcome in LMMC patients. Methods : Seventy-five patients with LMMC were enrolled in this study. Neuro-imaging and intraoperative findings allowed classification of LMMC into three Types. The patients were divided into two groups by age : A (51 patients), from birth to 3 years, and B (24 patients), from 3 to 24 years. For prevention of retethering of the cord, a mega-dural sac rebuilding procedure was performed in 15 patients. Results : During a mean postoperative follow-up period of 4 years, the surgical outcome was satisfactory in terms of improved pain and motor weakness, but disappointing with reference to the resolution of bowel and bladder dysfunction. Among these 75 patients with LMMC, preoperative deficits were improved after surgery in 29 (39%), remained stable in 28 (37%), changed slightly in 13 (17%), and worsened in 5 (7%). Patients in group A achieved better outcomes than those in group B. Depending on the type of lesion, patients with types I and II LMMC have better outcomes than those with type III LMMC. Finally, retethering of the cord with neurological deterioration occurred in 4 (5.3%) of the 75 patients, but no retethering was found in the 15 patients who were recently treated with a mega-dural sac rebuilding procedure. Conclusion : Our data continue to support the opinion that early diagnosis and optimal surgery are still essential for the treatment of patients with LMMC, since there is a high likelihood of residual neurological functions that can be preserved. Based on our surgical experience of untethering and decompression of lipomas, a mega-dural sac repair is useful to prevent retethering of the cord.