• Childhood Kidney Diseases
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• v.1 no.2
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• pp.155-160
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• 1997

Purpose : Tethered cord syndrome is characterized by progressive motor and sensory disturbances in lower extremities, foot deformities caused by a pathologic fixation of spinal cord resulting in excessive stretching of the spinal cord. It is also frequently associated with urological symptoms include urinary frequency, incontinence, enuresis, urgency and recurrent urinary tract infection. Because there is few report in the literature about urological manifestations of theterd cord syndrome, we conducted a retrospective study on the patients diagnosed as tethered cord syndrome to delineate the characteristics of urologic manifestations in tethered cord syndrome and to establish the policy to evaluate patients who is suspected of tethered cord syndrome. Method : A retrospective study was conducted by reviewing the medical records of nine patients who was diagnosed as tethered cord syndrome from November 1991 to July 1996 in Yeungnam University Hospital. Result : 1) The age distribution of nine patients was as follows; 5 patients were under 2 years, 1 case from 2 to 6 years and 3 cases from 6 to 10 years. 2) Of 9 patients 6 had voiding frequency, urinary incontinence, enuresis, urgency and loss of micturation sense. Radiologic urodynamic studies revealed neurogenic bladder in 5 patients, hydronephrosis and hydroureter in 3, vesicoureteral reflux in 3. 3) Of 6 patients with urological abnormal manifestations 4 underwent spinal cord surgery (detethering). In spite of surgical intervention, the urological manifestations improved in only one patient. Conclusion : Urological abnormalities were common in tethered cord syndrome. Abnormal laboratory findings including urodynamic study were found even before the onset of urological symptoms. We have to concern tethered cord syndrome as one of common causes of voiding frequency and enuresis and to evaluate urological abnormalities as thetered cord syndrome is diagnosed.

• Journal of Korean Neurosurgical Society
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• v.54 no.4
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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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• v.6 no.1
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• pp.109-113
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• 2002

The tethered cord syndrome is characterized by sensory and motor disturbances of the lower extremities and incontinence. We report a 12-year-old boy with sacral lipoma and a tethered cord syndrome, whose chief complaint is enuresis. This complication of tethered cord syndrome was most likely due to a renal failure and hydronephrosis secondary to a neurogenic bladder. Thus, the spinal lesions have to be considered in patients with enuresis.(J Korean Soc Pediatr Nephrol 2002 ;6 : 109-13)

• Journal of Korean Neurosurgical Society
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• v.39 no.4
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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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• v.63 no.3
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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.

• Journal of Electrodiagnosis and Neuromuscular Diseases
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• v.20 no.2
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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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• v.63 no.3
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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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• v.46 no.5
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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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• v.62 no.2
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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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• v.42 no.2
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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.