With advancements in corrective surgery for spina bifida since the 1960s, affected women are now reaching adulthood and achieving pregnancies. However, the implications on reproductive health?especially in pregnancy?are rarely studied. We are presenting a case of a woman born with spina bifida who has undergone surgical repair and closure of the defect at 4 years old, as well as surgical management for tethered cord at 13 years old. She lived productively into adulthood, became pregnant and delivered vaginally to a healthy baby despite various obstetrical challenges. In the care for this patient, we faced recurrent urinary tract infections, the risk of preterm delivery and the task of coordinated multidisciplinary care to solve dilemmas in decision making. Through this case report, we were able to share our experience, explore the most recent evidence to support our clinical decisions and hopefully serve as a basis for future clinical practice recommendations.
Pregnancy ; Female ; Spinal Dysraphism

Infantile hemangioma (IH) usually presents solely as a cutaneous manifestation, and rarely accompanies diverse anomalies such as spinal dysraphism. A 2-month-old girl presented with IH on her lumbar skin as a coin-sized red plaque with adjacent depressed skin and a child-palm-sized red plaque on her left ankle since birth. Considering the coexistence of IH and depressed skin on the midline in her lumbosacral area, magnetic resonance imaging of her spine was performed, which showed intraspinal/dermal vascular tumors with spina bifida occulta at the 12th thoracic vertebrae level. Furthermore, no neurologic deficits were observed. She has been taking oral propranolol with topical timolol to prevent neural complications and the lesions clinically improved. However, additional surgery for the intraspinal lesions was considered due to urination/defecation abnormalities since she was 13 months of age. In cases of midline IH, particularly with additional skin lesions, appropriate imaging studies to identify accompanying anomalies should be performed, and referrals to neurosurgical specialists should be considered.
Ankle ; Female ; Hemangioma ; Humans ; Infant ; Magnetic Resonance Imaging ; Neurologic Manifestations ; Parturition ; Propranolol ; Referral and Consultation ; Skin ; Specialization ; Spina Bifida Occulta ; Spinal Dysraphism ; Spine ; Thoracic Vertebrae ; Timolol

The stenosing foramen of L5–S1 by several degenerative diseases is one of the challenging areas on surgical approaching because of the deeper depth and steep slope in the lumbosacral junction. The floating view using unilateral biportal endoscopic spine surgery rather than docking into the Kambin’s zone can make the foraminal structures seen panoramically and permit dynamic handling of various instruments without destroying the facet joint and causing iatrogenic instability. Fine discrimination of structural margins in helps of the higher magnification and gentle manipulation of neural structures just as in open spine surgery could be guaranteed using floating technique from the target structures. Selective decompression with preserving innocent structures including facet joints could relieve foraminal lesions at the L5–S1 and decrease the necessity of fusion surgery caused by wider decompression and iatrogenic instability.
Decompression ; Discrimination (Psychology) ; Endoscopy ; Lumbosacral Region ; Minimally Invasive Surgical Procedures ; Spinal Dysraphism ; Spinal Stenosis ; Spine ; Zygapophyseal Joint

STUDY DESIGN: A retrospective cohort study. PURPOSE: To examine the validity of prophylactic surgery for children with tethered cord syndrome (TCS). OVERVIEW OF LITERATURE: Prophylactic surgery for pediatric patients with TCS remains controversial. METHODS: We retrospectively analyzed the surgical outcomes of 14 children (nine boys and five girls) with asymptomatic TCS who were surgically treated at Hokkaido University Hospital between 1989 and 2015. RESULTS: The median age at the time of initial surgery for asymptomatic TCS was 28.6 months (range, 0–66 months). The median final follow-up period was 142 months (range, 7–232 months). Of the 14 children with asymptomatic TCS, 12 had lumbosacral lipoma and two had meningocele. According to the classification of spinal lipoma, two children had dorsal type, four had caudal type, two had transitional type, and four had filar type. There were no children with lipomyelomeningocele. All children were free of neurological symptoms until 94 months after the initial surgery. Subsequently, one child exhibited delayed neurological deficits and underwent a second surgery because of motor and sensory disturbances; slight sensory disturbance was noted at the final follow-up examination. Another child later showed bowel and bladder dysfunction. However, a second surgery was not performed for this child because his motor and sensory functions were normal; hence, we chose to avoid nerve injury in the case of dissecting adhesion. CONCLUSIONS: All 14 children with asymptomatic TCS were free of neurological symptoms until 94 months after the initial surgery. However, two children exhibited delayed neurological deficits at 94 months and 177 months. We believe that prophylactic surgery for asymptomatic TCS is effective for a certain period. However, because the natural history of TCS is poorly understood, strict follow-up after surgery is necessary.
Child ; Classification ; Cohort Studies ; Follow-Up Studies ; Humans ; Lipoma ; Meningocele ; Natural History ; Neural Tube Defects ; Retrospective Studies ; Sensation ; Spinal Dysraphism ; Urinary Bladder

Fibrous hamartoma of infancy (FHI) is a rare entity with a benign nature. The typical clinical features are a single, slowly growing, painless mass on the trunk that appears within the first 2 years of life. We report a 13-month-old boy who presented with a plaque on the lower back since 4 months of age. The plaque had gradually become larger and firm, and hyperhidrosis and hypertrichosis were noticed. No visible connection between the spinal cord and the lesion was found in radiologic studies, indicating a disease other than spinal dysraphism. Histopathological findings showed well-defined fibrous trabeculae, mature adipose tissue, and primitive mesenchymal cells, all consistent with FHI. This is the first case of FHI presenting with hyperhidrosis and hypertrichosis reported in Korea.
Adipose Tissue ; Hamartoma ; Humans ; Hyperhidrosis ; Hypertrichosis ; Infant ; Korea ; Male ; Spinal Cord ; Spinal Dysraphism

STUDY DESIGN: We established induced pluripotent stem cells (iPSCs) and neural stem/progenitor cells (NSPCs) from three newborns with spina bifida aperta (SBa) using clinically practical methods. PURPOSE: We aimed to develop stem cell lines derived from newborns with SBa for future therapeutic use. OVERVIEW OF LITERATURE: SBa is a common congenital spinal cord abnormality that causes defects in neurological and urological functions. Stem cell transplantation therapies are predicted to provide beneficial effects for patients with SBa. However, the availability of appropriate cell sources is inadequate for clinical use because of their limited accessibility and expandability, as well as ethical issues. METHODS: Fibroblast cultures were established from small fragments of skin obtained from newborns with SBa during SBa repair surgery. The cultured cells were transfected with episomal plasmid vectors encoding reprogramming factors necessary for generating iPSCs. These cells were then differentiated into NSPCs by chemical compound treatment, and NSPCs were expanded using neurosphere technology. RESULTS: We successfully generated iPSC lines from the neonatal dermal fibroblasts of three newborns with SBa. We confirmed that these lines exhibited the characteristics of human pluripotent stem cells. We successfully generated NSPCs from all SBa newborn-derived iPSCs with a combination of neural induction and neurosphere technology. CONCLUSIONS: We successfully generated iPSCs and iPSC-NSPCs from surgical samples obtained from newborns with SBa with the goal of future clinical use in patients with SBa.
Cells, Cultured ; Ethics ; Fibroblasts ; Humans ; Induced Pluripotent Stem Cells* ; Infant, Newborn* ; Meningomyelocele ; Plasmids ; Pluripotent Stem Cells ; Regenerative Medicine ; Skin ; Spina Bifida Cystica* ; Spinal Cord ; Spinal Dysraphism* ; Stem Cell Transplantation ; Stem Cells

Surgical treatment of the degenerative disc disease has evolved from traditional open spine surgery to minimally invasive spine surgery including endoscopic spine surgery. Constant improvement in the imaging modality especially with introduction of the magnetic resonance imaging, it is possible to identify culprit degenerated disc segment and again with the discography it is possible to diagnose the pain generator and pathological degenerated disc very precisely and its treatment with minimally invasive approach. With improvements in the optics, high resolution camera, light source, high speed burr, irrigation pump etc, minimally invasive spine surgeries can be performed with various endoscopic techniques for lumbar, cervical and thoracic regions. Advantages of endoscopic spine surgeries are less tissue dissection and muscle trauma, reduced blood loss, less damage to the epidural blood supply and consequent epidural fibrosis and scarring, reduced hospital stay, early functional recovery and improvement in the quality of life & better cosmesis. With precise indication, proper diagnosis and good training, the endoscopic spine surgery can give equally good result as open spine surgery. Initially, endoscopic technique was restricted to the lumbar region but now it also can be used for cervical and thoracic disc herniations. Previously endoscopy was used for disc herniations which were contained without migration but now days it is used for highly up and down migrated disc herniations as well. Use of endoscopic technique in lumbar region was restricted to disc herniations but gradually it is also used for spinal canal stenosis and endoscopic assisted fusion surgeries. Endoscopic spine surgery can play important role in the treatment of adolescent disc herniations especially for the persons who engage in the competitive sports and the athletes where less tissue trauma, cosmesis and early functional recovery is desirable. From simple chemonucleolysis to current day endoscopic procedures the history of minimally invasive spine surgery is interesting. Appropriate indications, clear imaging prior to surgery and preplanning are keys to successful outcome. In this article basic procedures of percutaneous endoscopic lumbar discectomy through transforaminal and interlaminar routes, percutaneous endoscopic cervical discectomy, percutaneous endoscopic posterior cervical foraminotomy and percutaneous endoscopic thoracic discectomy are discussed.
Adolescent ; Athletes ; Cicatrix ; Constriction, Pathologic ; Diagnosis ; Diskectomy ; Diskectomy, Percutaneous ; Endoscopy ; Fibrosis ; Foraminotomy ; Humans ; Intervertebral Disc Chemolysis ; Length of Stay ; Lumbosacral Region ; Magnetic Resonance Imaging ; Quality of Life ; Spinal Canal ; Spinal Dysraphism ; Spine* ; Sports

Ultrasonography (US) is a useful screening method for the diagnosis of developmental dysplasia of the hip (DDH) and congenital spinal anomalies in infants. In addition, US is a useful, noninvasive imaging modality for the diagnosis of transient synovitis in children. The early detection of DDH leads to a better prognosis without surgical intervention. Moreover, spinal US allows the detection of congenital malformations and can also demonstrate normal anatomy and normal variants that may resemble disorders. Therefore, radiologists should be familiar with the sonographic anatomy of the normal infant hip, scanning and measurement techniques for DDH, the US features of transient synovitis in children, spinal US techniques, and the US features of normal anatomical structures, normal variants, and congenital spinal anomalies.
Child ; Diagnosis ; Hip Dislocation, Congenital ; Hip* ; Humans ; Infant ; Mass Screening ; Methods ; Prognosis ; Spinal Dysraphism ; Spine* ; Synovitis ; Ultrasonography*

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.
Child* ; Friends ; Humans ; Interviews as Topic ; Mothers ; Qualitative Research ; Spinal Dysraphism* ; Spouses ; Urinary Bladder

STUDY DESIGN: Retrospective cohort study. PURPOSE: To compare long-term results of surgery with the outcomes of symptomatic and asymptomatic tethered cord syndrome (TCS) in children and adolescents and to assess the surgery duration for those with TCS. OVERVIEW OF LITERATURE: Pediatric patients with TCS continue to pose significant diagnostic and management challenges. METHODS: We retrospectively analyzed the outcomes of 31 patients (16 males, 15 females) with TCS, including 21 with lumbosacral lipoma. All were surgically treated between 1989 and 2015. They were divided into symptomatic and asymptomatic TCS groups. The results of the treatment were summarized and analyzed using a non-parametric Mann–Whitney U test. RESULTS: Nineteen patients had symptomatic TCS and 12 had asymptomatic TCS. Patients had a median age of 34 months (range, 0–201 months). The median follow-up period was 116 months (range, 7–223 months). Of the 19 symptomatic TCS patients, preoperative deficits improved after surgery in two (10.5%) and remained stable in 17 (89.5%) patients. One of the 12 asymptomatic TCS (8.3%) patients showed an exacerbated illness after surgery, and one in 11 patients remained stable (11.7%). There were significant differences in monthly age at surgery, preoperative bowel and bladder dysfunction, neurological function, presence/absence of clean intermittent catheterization introduction, and presence/absence of motor disorder at final follow-up (all p<0.05). In the univariate analysis, the presence/absence of preoperative bowel and bladder dysfunction, and symptoms were strongly associated with the risk of children and adolescents with TCS (p<0.05). CONCLUSIONS: Early accurate diagnosis and adequate surgical release might lead to successful outcomes in children and adolescents with TCS. Surgical untethering is a safe and effective method for treatment of children with TCS.
Adolescent* ; Child* ; Cohort Studies ; Diagnosis ; Follow-Up Studies ; Humans ; Intermittent Urethral Catheterization ; Lipoma ; Male ; Methods ; Neural Tube Defects* ; Retrospective Studies ; Spinal Dysraphism ; Urinary Bladder