Surgical Outcomes of Pediatric Patients with Asymptomatic Tethered Cord Syndrome

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.

Usage Patterns of Surveillance, Chemoprevention and Risk-Reducing Surgery in Korean BRCA Mutation Carriers: 5 Years of Experience at a Single Institution / 한국유방암학회지

PURPOSE: Options for BRCA mutation carriers include close surveillance, chemoprevention, and risk-reducing surgery (RRS) for breast and ovarian cancer. However, chemoprevention and RRS for cancer prevention are not widely performed in Korea. The aim of this study was to investigate the usage patterns of surveillance, chemoprevention and RRS of breast and ovary in Korean BRCA mutation carriers. METHODS: We retrospectively reviewed the medical record of 67 women who were diagnosed with BRCA1 or BRCA2 mutation between January 2005 and May 2009 at Seoul National University Bundang Hospital. RESULTS: Mean age was 46 years old (range, 27-73 years), and median follow-up period was 10 months. The numbers of affected and unaffected carriers were 50 (74.6%) and 17 (25.4%). In 47 women affected with breast cancer excluding 3 cases of concurrent breast/ovarian cancers, 42 (89.4%) have received intensive surveillance only, 2 (4.3%) have taken tamoxifen for chemoprevention, and 3 (6.4%) have undergone contralateral prophylactic mastectomies to prevent breast cancer. For ovarian cancer prevention, risk reducing salpingo-oophorectomy was performed in 11 (24.4%) of 45 affected carriers excluding 5 patients who had bilateral salphingo-oophorectomy previously. In 17 unaffected carriers, chemoprevention and RRS were not performed. Only 4 (23.5%) of these unaffected carriers have chosen surveillance for breast or ovarian cancer. Old age and no family history are related to the poor compliance (no follow-up) of the carriers only in the univariate analysis but not in the multivariate analysis. CONCLUSION: Most of the Korean affected BRCA mutation carriers in our study chose intensive surveillance rather than chemoprevention or RRS. We should take special effort to follow and educate unaffected carriers, especially for those with old age or no family history.

Outcome of Surgical Treatment for Lipomeningomyelocele

Lipomeningomyelocele(LMMC) is one of the most common forms of occult spinal dysraphism seen in clinical practice. It is now widely accepted that prophylactic surgery is indicated in most cases, but areas of controversies were remained. From January 1986 to December 1996, long term data are available for 57 patients who underwent surgery for LMMC repair. The most common presenting symptom of these patients were mass on back which was followed by weakness of lower extremities and bladder-bowel symptoms. Transitional type was most common(53%) and followed by caudal(28%) and dorsal type(19%). Surgical repair was performed at age of 1 month to 40 years(mean age: 48months old, median 5 months old). Follow up for these patients ranged from 10 to 130 months(mean 50.3 months). None of the patients who underwent surgery before deficits had occured had ever developed new neurological deficits at the end of the follow up. However, 8 of 57 patients(14%) had aggravation of thier initial neurologic status by history preoperatively. Those progressive symptoms were somewhat reversed or stabilized in all of them postoperatively. In addition, surgical correction in infancy provides a degree of reversibility but do not in older children. We concluded that early diagnosis and treatment should be taken to prevent these progression and permanency of neurological changes.

Lipomyelomeningocele: Clinical analysis of 14 Cases

Lipomyelomeningocele is the most common malformation leading to spinal cord tethering. Nonetheless, these lesion continues to pose a challenge in patient management. The purpose of this study was to identify the clinical feature, candidate for surgical repair, and outcome of lipomyelomeningocele in young children and adults. We studied the magnetic resonance images and/or lumbar myelo-CT of lipomyelomeningocele performed between January 1987 and September 1995 at our institutions and reviewed the pertinent medical records and radiologic stuies to detemine clinical feature, surgical candidate, and outcomes of these patients. Of the 14 cases(11 female and 3 males) studied, the most common factor that caused these patients to seek help was the cosmetic effect of the mass on their back, followed by urinary incontinence, and weakness of lower extremities. The patients' age ranged from 2 months to 21 years(mean, 5.0 years). All the patients underwent immediate surgical management that consisted of removal of lipoma, untethering of the cord, and complete dural sac repair. Follow up for these patients ranged from 6 to 82 months(median, 54 mo). None of the patients that underwent surgical correction before the emergence of neurological deficit had ever developed neurological deficits or uninary incontinence at the time of surgery, improved significantly. In conclusion, early detection and prophylactic surgery is imperative to prevent permanent neurological deficits and urinary dysfunction.

Surgical Treatment of Lipomyelomeningocele

From January 1987 to December 1989, Six patients with lipomyelomeningocele were operated. There were 4 females and 2 males. The chief complaints that caused these patients to seek help were cosmetic effect of the mass on back, urinary incontinence, ankle deformity and weak leg. The postoperative results were as follows : Patient treated before developing neurological deficit remained unchanged neurologically. Patient treated after developing neurological deficitwere left with neurological sequelae. lipomyelomeningoceles are serious lesion which recommand early prohylactic surgery.