• 제목/요약/키워드: spinal dysraphism

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선천성 척추 피부동관 증례보고 (A Case Report of Congenital Spinal Dermal Sinus Tract)

  • 심병관;김용배;남승민;최환준
    • Archives of Plastic Surgery
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    • 제37권6호
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    • pp.827-830
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    • 2010
  • Purpose: Congenital spinal dermal sinus tract is a rare lesion connecting skin to deeper structures including neural tissue. It results from the failure of the neuroectoderm to separate from the cutaneous ectoderm in the third to fifth week of gestation. The common locations are the lumbosacral and occipital regions. Sometimes it extends to spinal canal. In this paper we report a case of congenital spinal dermal sinus tract in the coccyx. Methods: A 21-month-old male child born after an uncomplicated full-term pregnancy was admitted to our institute with a midline dermal sinus and a cartilaginous protrusion in the coccygeal region. There were no signs of infection. Neurologic examination showed no functional deficit in both lower limbs. He was treated with complete excision of the tract and an underlying accessory cartilage. Results: The spinal dermal sinus tract was extended from the skin to the coccyx. The stalk was loosely attached to the accessory cartilage of coccyx. At that point, it was dissected from the accessory cartilage and resected. The accessory cartilage was also resected at the bone and cartilage junction. During the follow-up period of 6 months, the wound healed well without any complication nor recurrence. Conclusion: Congenital spinal dermal sinus tract is known as a form of spinal dysraphism. In order to prevent complications, timely surgical intervention including complete resection of sinus tract with correction of associated abnormalities is of utmost importance.

Surgical Management of Complex Spinal Cord Lipomas : A New Perspective

  • Pang, Dachling
    • Journal of Korean Neurosurgical Society
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    • 제63권3호
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    • pp.279-313
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    • 2020
  • This review summarises the classification, anatomy and embryogenesis of complex spinal cord lipomas, and describes in some detail the new technique of total lipoma resection and radical reconstruction of the affected neural placode. Its specific mission is to tackle two main issues surrounding the management of complex dysraphic lipomas : whether total resection confers better long term benefits than partial resection, and whether total resection does better than conservative treatment, i.e., no surgery, for asymptomatic lipomas. Accordingly, the 24 years progression-free survival data of the author and colleagues' series of over 350 cases of total resection are compared with historical data from multiple series (including our own) of partial resection, and total resection data specifically for asymptomatic lesions are compared with the two known series of non-surgical treatment of equivalent patients. These comparisons amply support the author's recommendation of total resection for most complex lipomas, with or without symptoms. The notable exception is the asymptomatic chaotic lipoma, whose peculiar anatomical relationship with the neural tissue defies even our aggressive surgical approach, and consequently projects worse results (admittedly of small number of cases) than for the other two lipoma subtypes of dorsal and transitional lesions. Prophylactic resection of asymptomatic chaotic lipomas is therefore not currently endorsed.

편측 첨족을 첫 증상으로 보인 계류척수증후군: 증례 보고 (Unilateral Pes Cavus as an lnitial Sign of Tethered Cord Syndrome in an Adolescent: A Case Report)

  • 박승완;문정보;류병주
    • 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.

신경근 압박증상을 동반한 Tarlov씨 낭종 2예 - 증 례 보 고 - (Symptomatic Tarlov's Cyst(Sacral Meningeal Cyst) - Case Report -)

  • 임강택;조병문;신동익;박세혁;오세문
    • Journal of Korean Neurosurgical Society
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    • 제29권4호
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    • pp.569-573
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    • 2000
  • Spinal meningeal cyst of the sacrum is uncommon congenital lesion. We experienced two cases of sacral meningeal cyst, so called Tarlov's cyst, who presented with radiating pain. Magnetic resonance imaging is a highly effective way of locating and approximating the size of these entities, which generally appear as intraspinal masses of low intensity on T1-weighted and high intensity on T2-weighted images, similar to cerebrospinal fluid(CSF). We evaluated 2 patients who had Tarlov's cyst diagnosed with conventional MRI. The clinical features, radiological findings, gross appearances of the lesion at surgery, surgical technique, histopathological features of the cyst wall, and surgical outcome are described. We conclude that excellent result can be expected in the case of symptomatic Tarlov's cyst by surgical decompression.

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Junctional Neural Tube Defect

  • Eibach, Sebastian;Pang, Dachling
    • Journal of Korean Neurosurgical Society
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    • 제63권3호
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    • pp.327-337
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    • 2020
  • Junctional neurulation represents the most recent adjunct to the well-known sequential embryological processes of primary and secondary neurulation. While its exact molecular processes, occurring at the end of primary and the beginning of secondary neurulation, are still being actively investigated, its pathological counterpart -junctional neural tube defect (JNTD)- had been described in 2017 based on three patients whose well-formed secondary neural tube, the conus, is widely separated from its corresponding primary neural tube and functionally disconnected from corticospinal control from above. Several other cases conforming to this bizarre neural tube arrangement have since appeared in the literature, reinforcing the validity of this entity. The cardinal clinical, neuroimaging, and electrophysiological features of JNTD, and the hypothesis of its embryogenetic mechanism, form part of this review.

Catastrophic Intramedullary Abscess Caused by a Missed Congenital Dermal Sinus

  • Dho, Yun-Sik;Kim, Seung-Ki;Wang, Kyu-Chang;Phi, Ji Hoon
    • Journal of Korean Neurosurgical Society
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    • 제57권3호
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    • pp.225-228
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    • 2015
  • Congenital dermal sinus (CDS) is a type of occult spinal dysraphism characterized by a midline skin dimple. A 12-month-old girl presented with fever and ascending quadriparesis. She had a midline skin dimple in the upper sacral area that had been discovered in her neonatal period. Imaging studies revealed a holocord intramedullary abscess and CDS. Overlooking CDS or misdiagnosing it as benign sacrococcygeal dimple may lead to catastrophic infection and cause serious neurological deficits. Therefore, further imaging work-up or consultation with a pediatric neurosurgeon is recommended following discovery of any atypical-looking dimples in the midline.

Retethering : A Neurosurgical Viewpoint

  • Lee, Ji Yeoun;Kim, Kyung Hyun;Park, Kwanjin;Wang, Kyu-Chang
    • Journal of Korean Neurosurgical Society
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    • 제63권3호
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    • pp.346-357
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    • 2020
  • During the follow-up period after surgery for spinal dysraphism, a certain portion of patients show neurological deterioration and its secondary phenomena, such as motor, sensory or sphincter changes, foot and spinal deformities, pain, and spasticity. These clinical manifestations are caused by tethering effects on the neural structures at the site of previous operation. The widespread recognition of retethering drew the attention of medical professionals of various specialties because of its incidence, which is not low when surveillance is adequate, and its progressive nature. This article reviews the literature on the incidence and timing of deterioration, predisposing factors for retethering, clinical manifestations, diagnosis, surgical treatment and its complications, clinical outcomes, prognostic factors after retethering surgery and preventive measures of retethering. Current practice and opinions of Seoul National University Children's Hospital team were added in some parts. The literature shows a wide range of data regarding the incidence, rate and degree of surgical complications and long-term outcomes. The method of prevention is still one of the main topics of this entity. Although alternatives such as spinal column shortening were introduced, re-untethering by conventional surgical methods remains the current main management tool. Re-untethering surgery is a much more difficult task than primary untethering surgery. Updated publications include strong skepticism on re-untethering surgery in a certain group of patients, though it is from a minority of research groups. For all of the abovementioned reasons, new information and ideas on the early diagnosis, treatment and prevention of retethering are critically necessary in this era.

Outcome of Surgical Management for Tethered Spinal Cord

  • Lee, Jeong-Ho;Park, Seong-Hyun;Sung, Joo-Kyung;Hwang, Sung-Kyoo
    • 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.

이분척추 환아의 어머니가 인식한 질병관리의 어려움: 질적 서술적 접근 (Mothers' Perceived Difficulties in the Management of Chronic Conditions of Children with Spina Bifida: A Qualitative Descriptive Approach)

  • 임승화;이명선
    • Child Health Nursing Research
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    • 제22권4호
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    • pp.247-256
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    • 2016
  • Purpose: The purpose of the study was to identify and describe the caregiving difficulties that mothers of children with spina bifida experience from their own perspectives. Methods: A qualitative descriptive study was designed. Data were collected from five mini-focus group interviews and four individual interviews using open-ended questions. Nineteen mothers of children with mild spina bifida participated in the study during 2014-2015. Data were analyzed using qualitative content analysis to identify major difficulties perceived by the mothers. Results: Five domains were identified with 12 subdomains. "Daily routine continence management" describes difficulties arising in bladder and bowel management for the child with spina bifida. "Management of school life of child" deals with difficulties in relation to the school facilities, such as the toilets, as well as teachers and friends. "Relationship with family and neighbors" illustrates problems in relations with their spouse, normal children, relatives and neighbors. "Maintaining physical and psychological health" includes physical and psychological problems of the mothers. "Finance" describes economic burden that the mothers face in the management of child's illness. Conclusion: The findings from this study provide insight into the practical issues related to the management of chronic conditions of children with spina bifida from the mothers' perspectives.

Myelomeningocele defect reconstruction with keystone flaps: vascular rationale for the design and operative technique

  • Kushida-Contreras, Beatriz Hatsue;Gaxiola-Garcia, Miguel Angel
    • Archives of Plastic Surgery
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    • 제48권3호
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    • pp.254-260
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    • 2021
  • Background Myelomeningocele is a frequently seen condition at tertiary care hospitals. Its treatment involves a variety of plastic reconstructive techniques. Herein, we present a series of myelomeningocele patients treated using keystone flaps. Methods We gathered information regarding soft tissue reconstruction and the use of bilateral keystone flaps to treat myelomeningocele patients. We obtained data from clinical records and recorded the demographic characteristics of mothers and children with the condition. The size, level of defect, and complications detected during the follow-up were analyzed. Results A series of seven patients who underwent bilateral keystone flaps for myelomeningocele closure was analyzed. There were no cases of midline or major dehiscence, flap loss, necrosis, surgical site infections, or cerebrospinal fluid leakage. No revision procedures were performed. Minor complications included one case with minimal seroma and three cases with areas of peripheral dehiscence that healed easily using conventional measures. Conclusions The use of keystone flaps is an adequate option for closure of dorsal midline soft tissue defects related to myelomeningocele. This technique offers predictable results with an acceptable spectrum of complications. Robust blood flow can be predicted based upon anatomical knowledge.