Selective Dorsal Rhizotomy For Treatment of Spasticity After Hemispherectomy In Children: A Case Report.

Performing a hemispherotomy or hemispherectomy is known to treat medically intractable epilepsy successfully, yet contralateral hemiparesis and increased muscle tone follow the epilepsy surgery. Spasticity and coexisting dystonia presumably cause the increased muscle tone in the lower extremity on the opposite side of epilepsy surgery. However, the extent of the role of spasticity and dystonia in high muscle tone is unknown. A selective dorsal rhizotomy is performed to reduce spasticity. If a selective dorsal rhizotomy is performed in the affected patient and muscle tone is reduced, the high muscle tone is not due to dystonia. Two children, who previously underwent a hemispherectomy or hemispherotomy, had a selective dorsal rhizotomy (SDR) performed in our clinic. Both children underwent orthopedic surgery to treat heel cord contractures. To study the extent of the role of spasticity and dystonia in high muscle tone, the mobility of the two children was examined pre- and post-SDR. The children had follow-ups 12 months and 56 months after SDR to study long-term effects. Before SDR, both children showed signs of spasticity. The SDR procedure removed spasticity, and muscle tone in the lower extremity became normal. Importantly, dystonia did not surface after SDR. Patients started independent walking less than two weeks after SDR. Sitting, standing, walking, and balance improved. They could walk longer distances while experiencing less fatigue. Running, jumping, and other more vigorous physical activities became possible. Notably, one child showed voluntary foot dorsiflexion that was absent before SDR. The other child showed improvement in voluntary foot dorsiflexion that was present before SDR. Both children maintained the progress at the 12 and 56-month follow-up visits. The SDR procedure normalized muscle tone and improved ambulation by removing spasticity. The high muscle tone following the epilepsy surgery was not due to dystonia.

Selective Dorsal Rhizotomy for Treatment of Hereditary Spastic Paraplegia-Associated Spasticity in 37 Patients.

Background A limited number of publications have described a reduction of spasticity associated with hereditary spastic paraplegia (HSP) after selective dorsal rhizotomy (SDR). Typically, the SDR procedure is performed on patients with spastic cerebral palsy to remove spasticity and to help these patients with ambulatory function. Whether SDR has similar effects on HSP patients, requires further investigation. Thus, we are providing a personal experience of the effects of SDR on this specific cohort of patients. Objectives To examine the safety of SDR, changes in spasticity, and ambulatory function after SDR on patients with HSP. Methods The Institutional Review Board of Washington University School of Medicine approved this study (#201704003). A total of 37 children and adults received SDR for the treatment of HSP-associated spasticity between 1988 and 2021. SDR was performed through an L1 laminectomy, as we previously described in an earlier publication. The patients took part in the follow-up examination either in-person or by email. The follow-up focused on the patients' motor functions (primarily ambulation), adverse effects of SDR, and orthopedic treatments after SDR. Results Of the total 37 patients who participated in this study, 46% were female and 54% were male. The age range of when HSP was diagnosed was one month to 34 years. Six of the patients' diagnoses were made, based on the family history of HSP in six patients and the remaining 31 patients' diagnoses were confirmed by genetic tests. The most common genetic mutations were SPG4 and SPG3A. Of the patients with positive genetic tests, 40% had no family history of HSP. SDR was performed at the age of 2 to 45 years (mean: 14.7 years). The follow-up period ranged from 0 to 33 years (mean: 3.8 years). One patient developed a spinal fluid leak requiring surgical repair. Two patients reported mild numbness in parts of the lower limbs. Spasticity was removed in 33 patients (89%). Four patients (11%) experienced a return in spasticity. Regarding ambulatory function, 11% of patients reported a decline in function. Two patients walked independently before surgery but declined, requiring a wheelchair eight years and seven years, respectively, after surgery for each patient. In contrast, 16% saw an improvement in ambulatory function, improving from walking with a walker to walking independently. The remaining 73% of patients maintained their level of ambulation. These two groups of patients showed improvement in other motor functions and independence.  Conclusions The present analysis suggests the potential role of SDR in the management of spasticity in HSP patients. We found no sign of SDR being a direct cause of deleterious effects on patients with HSP.

Parent-Reported Outcomes of Early Childhood Selective Dorsal Rhizotomy for the Treatment of Spastic Diplegia.

Background A selective dorsal rhizotomy (SDR) is employed to treat spastic cerebral palsy. The surgical techniques and patient care protocols vary among hospitals. One of the variations is the age cut-off for SDR. We have been advocating SDR to be performed early - especially at ages 2 and 3. With this study, we are reporting the feasibility and parent-reported surgical outcomes of receiving SDR at an early age for the treatment of spastic diplegia. Objectives Our aim is to examine the safety and benefits of receiving SDR at the ages of 2 and 3 for the treatment of spastic diplegia. Methods The Institutional Review Board (IRB) of Washington University School of Medicine approved this retrospective quality of life survey and chart review (approval #202009056). The subjects of this study were children and teens (ages: 3.9-18.1) with spastic diplegic cerebral palsy who underwent SDR at ages 2 or 3 between years 2005 and 2019 at St. Louis Children's Hospital. Only domestic patients that were minors at the time of the study were selected to be participants in compliance with IRB regulations to protect patient health information that could potentially be breached by sending information to an incorrect or dated email. Thus, all contact was made through postal mail. The study included 141 patients from a total of 362 eligible patients. Parents of eligible patients were sent the research survey via postal mail. Only patients who responded to the survey were included in this study. The survey included questions on demographic information, quality of life, health perception, motor and ambulatory functions, braces and orthotics, pain issues, side effects of SDR, and post-SDR treatment. Results The study included 141 diplegic patients. Of all patients at the time of the study, 91% reported an improvement in walking, 92% in standing, and 89% in sitting. In daily life activities, 87% of patients became more independent after SDR. 65% of patients were able to walk without a walking aid and about 4% were not able to walk. 11% of all patients relied mostly on a wheelchair. Moreover, 43% of patients were able to run independently. Regarding post-SDR orthopedic surgery, 48% of patients received at least one type of orthopedic surgery, with Achilles tendon lengthening, hamstring lengthening, and calf muscle release being the most common types.  Conclusions SDR performed at an early age through a single-level laminectomy was proved feasible and safe. A follow-up until the adult age (18 years) showed improvements in walking and other motor functions. The results support the implementation of early-age SDR for the treatment of spastic diplegia.

Selective Dorsal Rhizotomy for the Treatment of Spastic Hemiplegic Cerebral Palsy.

Background Selective dorsal rhizotomy (SDR) can remove spasticity in cerebral palsy (CP). Spastic hemiplegia is associated with spasticity in the upper and lower limbs on one side. Only a single report described the outcome of SDR specifically in patients with spastic hemiplegic CP. The effect of SDR on spastic hemiplegia requires further investigation. Objectives To analyze the outcomes of motor functions, the quality of life, and satisfaction of patients who received SDR for the treatment of spastic hemiplegia. Methods A total of 29 children and 1 adult who received SDR were surveyed. The survey questionnaire asked about demographic information, patient's perception of SDR, functional outcomes, SDR surgical outcomes, pain, braces/orthotics, and post-SDR treatment. Results Our study included 30 patients. The age at the time of surgery was 2 to 36 years. The follow-up period ranged from one to six years. Of all parents, 90% of parents reported that SDR benefited their children, and 93% stated that they would recommend the SDR procedure to other families of children with hemiplegic CP. Of all patients, 90% reported improved walking, 63% reported improved sitting, and 87% reported improved balance and posture. In daily life functioning after the SDR, 67% were more independent and confident. Moreover, 33% of patients were pain-free and 43% had reduced pain in their legs and back. In activities of daily living, 93% transferred independently from one position to another. A majority of the patients reported regular strengthening and stretching of the lower limb, and 50% of the patients played sports. A majority (73%) of patients underwent post-SDR orthopedic surgery for heel cord, hamstring, and adductor contractures. Five patients experienced numbness in the small part of the lower limb after SDR. None reported that the numbness affected their daily activities. One child required surgical repair of the cerebrospinal fluid leak. Conclusions In our 29 children and 1 adult with spastic hemiplegia, SDR improved motor function and daily life function. Nearly all parents of children and the one adult felt that SDR was beneficial and that they would recommend surgery to other children with spastic hemiplegia.

Selective Dorsal Rhizotomy for the Treatment of Spastic Triplegic Cerebral Palsy.

Background Spastic triplegia is a recognized subtype of cerebral palsy (CP). In the course of treating spastic triplegic children with selective dorsal rhizotomy (SDR), we found that some children who had "minimal or mild involvement" in the stronger arm improved strikingly after undergoing SDR. Some of them became independent ambulators, which was an outcome that is not usually achieved in spastic quadriplegic children. However, the literature currently contains no data on the natural clinical course and the effects of CP interventions on spastic triplegia. Objectives Our aim was to elucidate the clinical characteristics of spastic triplegia and the effects of SDR on functional outcomes and the quality of life after childhood SDR. Methods The Institutional Review Board of the Washington University School of Medicine approved this quality of life survey (number: 201908177). The subjects of this study were children and adults (ages: 3.9-23.8 years at the time of the survey completion; mean: 12.1 ± 5.1 years) with spastic triplegic CP who had undergone SDR (ages: 2.2-15.9 years; mean: 6.1 ± 3.2 years) between 2003 and 2018 at the St. Louis Children's Hospital. The follow-up period ranged from 1-16 years (mean: 6.0 ± 4.3 years). The study included a 76-patient cohort selected from a total of 253 spastic triplegic CP patients who had undergone SDR. All 253 patients were contacted via email or postal mail soliciting their participation in the study including the survey. The cohort included all patients who responded. The survey included questions on demographic information, quality of life, perceptions of health and the SDR procedure, motor and ambulatory functions, braces and orthotics, pain issues, side effects of SDR, and post-SDR treatment. Results Thirteen patients had presented with scissored gait, and these patients had undergone partial ventral rhizotomy (PVR) of L1-3 ventral roots immediately after the completion of SDR. Of note, 91% of 76 patients reported that SDR improved their quality of life, and 93% would recommend the procedure to other patients. After SDR, 21 more patients were able to run, 14 more played recreational sports, and 18 more could walk without using walking aids. Sixteen fewer patients used a wheelchair for long-distance walking and in crowds; 37 and 32 patients reported an improvement in the more affected arm and hand, respectively. Sixty-eight patients were able to regularly strengthened their muscles at least once a week, and 60 patients regularly stretched their legs. However, 53 patients required assistance with bathing or showering, 50 with getting dressed, and 56 with grooming or hygiene. Forty-eight patients had orthopedic surgery after SDR. Percutaneous hamstring-lengthening was the most common type of orthopedic surgery performed. Three of 13 patients who received PVR and SDR required adductor release. Six patients used medications for spasticity or dystonia. No late side effects of SDR were observed. Conclusions Our report elucidates the clinical features of spastic triplegia before and after SDR. A distinct clinical feature was the wide variation in ambulatory functions, ranging from total independent walking to wheelchair mobility. The vast majority of patients felt that SDR improved their motor functions and quality of life. PVR also resulted in favorable outcomes, with only three of 13 patients requiring additional adductor release surgery. There were no late complications related to SDR surgery.

Functional Outcome of Adulthood Selective Dorsal Rhizotomy for Spastic Diplegia.

Objective The medical evidence supporting the efficacy of selective dorsal rhizotomy (SDR) on children with spastic diplegia is strong. However, the outcome of SDR on adults with spastic diplegia remains undetermined. The aim is to study the effectiveness and morbidities of SDR performed on adults for the treatment of spastic diplegia.  Methods Patients who received SDR in adulthood for the treatment of spastic diplegia were surveyed. The survey questionnaire addressed the living situation, education level, employment, health outcomes, postoperative changes of symptoms, changes in ambulatory function, adverse effects of SDR and orthopedic surgery after SDR.  Results The study included 64 adults, who received SDR for spastic diplegia. The age at the time of surgery was between 18 and 50 years. The age at the time of the survey was between 20 and 52 years. The follow-up period ranged from one to 28 years. The study participants reported post-SDR improvements of the quality of walking in 91%, standing in 81%, sitting in 57%, balance while walking 75%, ability to exercise in 88%, endurance in 77%, and recreational sports in 43%. Muscle and joint pain present before surgery improved in 64% after surgery. Concerning the level of ambulatory function, all patients who walked independently in all environments maintained the same level of ambulatory function. Eighteen percent of the patients who walked independently in some environments improved to the independent walking in all environments. All patients who walked with an assistive device before SDR maintained the assistive walking after SDR. Concerning adverse effects of SDR, 50% (32 of 64 patients) developed numbness in the various parts of the legs. Two patients reported a complete loss of sensation in parts of the legs, and one patient reported numbness and constant pain in the bilateral lower extremities. Ten patients (16%) reported recurrent spasticity after SDR, and three patients (5%) reported ankle clonus, which is an objective sign of spasticity. Tendon lengthening surgery after SDR was needed in 27% and hip and knee surgery in 2% and 6%, respectively.  Conclusions The great majority of our 64 patients, who received adulthood SDR for spastic diplegia, improved the quality of ambulation and abated signs of early aging. Numbness and diminished sensation in the lower extremity was the most common adverse effect of the adulthood SDR.

Forkhead box O3 promotes cell proliferation and inhibits myotube differentiation in chicken myoblast cells.

1. In the poultry industry, growth performance is important due to its effects on economic value. Much effort has been put forth to achieve introgression of specific genes and DNA markers related to muscle proliferation and differentiation in selective breeding approaches. 2. This study investigated the biological functions of the gene Forkhead box O3 (FOXO3) during myogenic differentiation in chicken myoblast cells. FOXO3 was downregulated in primary chicken myoblast (pCM) cells by the piggyBac transposon-mediated microRNA (miRNA) knock-down (KD) system. 3. The pCM cells that were stably integrated into the FOXO3 KD expression vector showed significant downregulation of FOXO3 protein and mRNA levels. Expression levels of paired box protein Pax7 (Pax7) and target genes such as CCAAT/enhancer binding protein beta and serum response element decreased in FOXO3 KD pCM cells. In addition, in the undifferentiated myoblast stage, there were no significant differences in cell morphology; however, proliferation rate in FOXO3 KD pCM cells was significantly lower during d 4 and 5 of in vitro culture. By contrast, when myotube differentiation was induced, FOXO3 KD pCM cells exhibited rapid initiation of myotube formation, higher expression of myogenin and desmin as myogenic indicators and a further differentiated phenotype than observed in regular pCM cells. 4. These results demonstrated that FOXO3 promotes cell proliferation and inhibits myotube differentiation in chicken myoblast cells. Therefore, the regulation of FOXO3 could be applied to improve muscle differentiation in commercial poultry.

Activity measurement of 222Rn gas for a key comparison.

The Korea Research Institute of Standards and Science (KRISS) participated, in 2015, in an international Key Comparison (KC) of gaseous radon-222 activity named CCRI (II) -K2.Rn-222 to confirm international equivalence of KRISS-established gaseous radon-222 measurement standards. LNHB acted as KC pilot laboratory. This paper describes the KC measurement procedure followed at KRISS using the defined solid angle counting method together with auxiliary relative measurement methods and presents the results.

The MLL recombinome of acute leukemias in 2017.

Chromosomal rearrangements of the human MLL/KMT2A gene are associated with infant, pediatric, adult and therapy-induced acute leukemias. Here we present the data obtained from 2345 acute leukemia patients. Genomic breakpoints within the MLL gene and the involved translocation partner genes (TPGs) were determined and 11 novel TPGs were identified. Thus, a total of 135 different MLL rearrangements have been identified so far, of which 94 TPGs are now characterized at the molecular level. In all, 35 out of these 94 TPGs occur recurrently, but only 9 specific gene fusions account for more than 90% of all illegitimate recombinations of the MLL gene. We observed an age-dependent breakpoint shift with breakpoints localizing within MLL intron 11 associated with acute lymphoblastic leukemia and younger patients, while breakpoints in MLL intron 9 predominate in AML or older patients. The molecular characterization of MLL breakpoints suggests different etiologies in the different age groups and allows the correlation of functional domains of the MLL gene with clinical outcome. This study provides a comprehensive analysis of the MLL recombinome in acute leukemia and demonstrates that the establishment of patient-specific chromosomal fusion sites allows the design of specific PCR primers for minimal residual disease analyses for all patients.

Functional Outcomes of Childhood Selective Dorsal Rhizotomy 20 to 28 Years Later.

BACKGROUND: Selective dorsal rhizotomy (SDR) is a surgical method used to treat childhood spastic cerebral palsy (CP). However, the effects of early SDR on functional outcomes and quality of life decades later in adulthood remains to be elucidated. OBJECTIVES: To evaluate the long-term outcomes in terms of satisfaction and mobility of adult patients who received childhood SDR. METHODS: Adult patients who received SDR in childhood were surveyed. The survey questionnaire asked about demographic information, quality of life, health outcomes, SDR surgical outcomes, ambulation, manual ability, pain, braces/orthotics, post-SDR treatment, living situation, education level, and work status. RESULTS: Our study included 95 patients. The age that patients received SDR was between two and 18 years. The age at the time of survey was between 23 and 37 years (mean ± S.D., 30.2 ± 3.6 years). Post-SDR follow-up ranged from 20 to 28 years (mean ± S.D., 24.3 ± 2.2 years). Seventy-nine percent of patients had spastic diplegia, 20% had spastic quadriplegia, and one percent had spastic triplegia. Ninety-one percent of patients felt that SDR impacted positively the quality of life and two percent felt that the surgery impacted negatively the quality of life after SDR. Compared to pre-operative ambulatory function, 42% reported higher level of ambulation and 42% ambulated in the same level. Eighty-eight percent of patients would recommend the procedure to others and two percent would not. Thirty-eight percent reported pain, mostly in the back and lower limbs, with mean pain level 4.2 ± 2.3 on the Numeric Pain Rating Scale (NPRS). Decreased sensation in patchy areas of the lower limbs that did not affect daily life was reported by eight percent of patients. Scoliosis was diagnosed in 31%. The severity of scoliosis is unknown. Only three percent of them underwent spinal fusion. Fifty-seven percent of patients required some orthopedic surgery after SDR. The soft-tissue tendon lengthening procedures included lengthening on hamstrings, Achilles tendons or adductors. Out of all bone procedures, 24% of patients had hip surgery, five percent had knee surgery, and 10% had derotational osteotomies. No late side effects of SDR surgery were reported in this survey. CONCLUSIONS: In our 95 adult patients who received SDR in childhood, the surgery had positive effects on the quality of life and ambulation 20-28 years later. There were no late complications of SDR surgery.

Beneficial Effects of Childhood Selective Dorsal Rhizotomy in Adulthood.

BACKGROUND:  Selective dorsal rhizotomy (SDR) has been used to treat children with spastic cerebral palsy (CP) for over three decades. However, little is known about the outcomes of childhood SDR in adults.  Objectives: 1) To study the effects of childhood SDR on the quality of life and ambulatory function in adult life. 2) To determine late side effects of SDR in adults.   Methods: Adults (> 17.9 years) who underwent SDR in childhood (2 - 17.9 years) between 1987 and 2013 were surveyed in 2015. Patients completed a survey, including questions on demographic information, quality of life, health, surgical outcomes, motor function, manual ability, pain, braces/orthotics, post-SDR treatment, living situation, education level, work status, and side effects of SDR.  Results: In our study population of 294 patients (18.0 - 37.4 years), patients received SDR during the ages of 2.0 - 17.9 years and were followed up 2.2 to 28.3 years after surgery. Eighty-four percent had spastic diplegia, 12% had spastic quadriplegia, and 4% had spastic triplegia. The majority (88%) of patients reported improved post-SDR quality of life and 1% considered the surgery detrimental. Most (83%) would recommend the procedure to others and 3% would not. However, patients who would not recommend SDR to others ambulated with a walker or were not ambulatory at all prior to SDR. The majority (83%) of patients improved (30%) or remained stable (53%) in ambulation. Twenty-nine percent of patients reported pain, mostly in the back and lower limbs, with a mean pain level of 4.4 ± 2.4 on the Numeric Pain Rating Scale (NPRS). Decreased sensation in small areas of the lower limbs was reported by 8% of patients, though this did not affect daily life. Scoliosis was diagnosed in 28%, with 40% of these patients pursuing treatment. Whether scoliosis was related to SDR is not clear, though scoliosis is known to occur in patients with CP and also in the general population. Only 4% of patients underwent spinal fusion.  Orthopedic surgeries were pursued by 59% of patients. The most common orthopedic surgeries were hamstring lengthenings (31%), Achilles tendon lengthenings (18%), adductor lengthenings (16%), and derotational osteotomies (16%). Twenty-four percent of all patients later underwent hip surgery and 8% had surgeries on their knees.  Conclusion: Results of this study indicate that the beneficial effects of childhood SDR extend to adulthood quality of life and ambulatory function without late side effects of surgery.

THE INFLUENCE OF TOTAL OR SUB-TOTAL GASTRECTOMY ON GLUCOSE CONTROL IN DIABETIC AND NON-DIABETIC PATIENTS.

OBJECTIVE: Although bariatric surgery including gastrectomy has recently emerged as a useful treatment for type 2 DM with obesity, it is not clear whether gastrectomy itself can have beneficial effects on glucose metabolism. Therefore, in this study, we investigated changes in blood glucose in patients with and without diabetes who underwent gastrectomy. METHODS: From Jan 2010 to May 2014, 77 patients with diabetes and 77 patients without diabetes who underwent gastrectomy at Chonbuk National University Hospital, South Korea, were included. We compared fasting plasma glucose levels and HbA1c value before and after gastric surgery. RESULTS: After gastrectomy, 59 patients (38.3%) showed reduced fasting plasma glucose levels at the 1 year point, and 80 patients (51.9%) exhibited reduced fasting plasma glucose at 3 years, irrespective of their diabetes status. Among 77 patients with diabetes, decreased fasting plasma glucose was observed in 22 (28.6%) and 46 patients (59.7%) 1 and 3 years after gastrectomy, respectively. In patients who exhibited reduced fasting plasma glucose after gastrectomy, the degree of reduced glucose was as follows: 56.4±48.5 vs 23.2±16.1 mg/dL after 1 year, 58.3±52.3 vs 18.4±13.7 mg/dL after 3 years, in DM and non-DM patient respectively. CONCLUSIONS: Although there was a significant drop in mean fasting glucose after gastrectomy, not all patients experienced a drop in fasting glucose. Gastrectomy did not show a consistent association with glucose reduction in patients with and without diabetes, and in about half of the patients, fasting plasma glucose levels increased after gastrectomy. Therefore, bariatric surgery including gastrectomy needs to be performed with care in diabetes, and glucose monitoring including oral glucose tolerance tests should be done for assessing or prediction of the glucose state after gastric surgery in non-DM patients.

Risks and outcomes of spinal deformity surgery in Chiari malformation, Type 1, with syringomyelia versus adolescent idiopathic scoliosis.

BACKGROUND CONTEXT: Chiari malformation, Type 1, with syringomyelia (CIM+SM) is often associated with spinal deformity. The safety of scoliosis surgery this population is controversial and has never been directly compared with adolescent idiopathic scoliosis (AIS). PURPOSE: The purpose of this study was to compare the safety and subjective outcomes of spinal deformity surgery between patients with Chiari malformation Type 1-associated scoliosis and a matched AIS cohort. STUDY DESIGN: This study is based on a retrospective matched cohort analysis. PATIENT SAMPLE: Patients with CIM+SM and treated with spinal fusion for spinal deformity were identified in the surgical records of a single institution and were matched, 1:1, with AIS patients undergoing spinal fusion at the same institution. OUTCOME MEASURES: The outcome measures were neurological monitoring data quality and integrity, radiographic parameters, and Scoliosis Research Society Questionnaire-22 (SRS-22) scores. METHODS: A clinical database was reviewed for patients undergoing spinal reconstruction for CIM+SM-associated spinal deformity at our institution from 2000 to 2012. Thirty-six CIM+SM patients were identified and matched to an AIS cohort (1:1) based on age, gender, major curve magnitude, fusion length, and revision status. Demographics, deformity morphology, surgical details, neuromonitoring data, and preoperative and postoperative SRS-22 scores were recorded at a minimum of 2-year follow-up. Changes in SRS-22 scores were compared within and between groups. Complications and neurological monitoring data issues were compared between groups. RESULTS: Mean age was 14.5±5 years (CIM+SM: 14.6±5; AIS: 14.4±5), and 42% of patients were male. Preoperative mean major coronal Cobb measured 58°±25° versus 57°±17° (p=.84) with mean kyphosis 52°±17° versus 41°±20° (p=.018). An average of 10.4±2.6 vertebral levels were fused (10.4±2.8 vs. 10.4±2.3, p=.928). No differences existed in surgical approach (p=.336), estimated blood loss (680±720 vs. 660±310 mL, p=.845), or duration of surgery (6.0±2.2 vs. 5.6±2 hours, p=.434). Complication rate was comparable between the two groups (33% vs. 14%, p=.052). Chiari malformation, Type 1, with syringomyelia experienced more neurological complications (11% vs. 0%, p=.04) and neuromonitoring difficulties (28% vs. 3%, p=.007) than the AIS cohort. Mean curve correction was comparable at 2 years (58% CIM+SM vs. 64% AIS, p=.2). At follow-up, both CIM+SM and AIS groups demonstrated improved cumulative SRS-22 outcome subscores (CIM+SM: +0.4, p=.027; AIS: +0.3, p<.001). No difference in outcome subscores existed between CIM+SM and AIS groups. CONCLUSIONS: Although CIM+SM patients undergoing spine reconstruction can expect similar deformity corrections and outcome scores to AIS patients, they also experience higher rates of neuromonitoring difficulties and neurological complications related to surgery. Surgeons should be prepared for these difficulties, particularly in children with larger syrinx size.

Vertebral compression fractures may increase mortality in male patients with chronic obstructive pulmonary disease.

BACKGROUND: Vertebral compression fracture (VCF) is frequent in chronic obstructive pulmonary disease (COPD) patients. However, little is known about whether VCF affects mortality in COPD patients. OBJECTIVE: To investigate whether VCFs might increase death in COPD patients. METHODS: In this retrospective cohort study, we enrolled 254 COPD patients with a recent history of hospitalisation due to respiratory problems. Patients were assessed for VCF using quantitative morphometric analyses of lateral chest radiographs; 211 patients received follow-up examinations for 2 years. RESULTS: Of the 211 COPD patients analysed, 60 (28.4%) had VCF at enrolment. During the follow-up period, 33/60 (55.0%) patients with and 46/151 patients (30.5%) without VCF died (P = 0.003, log-rank test). Cox proportional hazard analysis revealed that VCF is an independent risk factor for death after adjusting for age, sex, body mass index, smoking, dyspnoea scale, forced expiratory volume in 1 sec (FEV1) and comorbidities (hazard ratio for VCF = 1.79, 95%CI 1.11-2.89, P = 0.02). CONCLUSION: VCF might be an independent risk factor for death in male COPD patients.

Surgical management of complex multiloculated hydrocephalus in infants and children.

OBJECTIVE: Multiloculated hydrocephalus may occur as a consequence of intraventricular hemorrhage or infection and is characterized by enlargement of multiple noncommunicating intraventricular and/or periventricular cysts. In this study, we report the outcomes of open and endoscopic fenestration for multiloculated hydrocephalus at our institution. METHODS: Records of children who underwent endoscopic or open fenestration at St. Louis Children's Hospital from 1999 to 2011 were analyzed. The cause of MLH, operative parameters, length of hospital stay, and subsequent shunt intervention rate were recorded. RESULTS: Twenty-five subjects were identified for study. Twelve subjects underwent open craniotomy and 13 underwent endoscopic fenestration. Endoscopic fenestration was associated with decreased blood loss, operative time, and length of stay (p = 0.003, 0.002, 0.02, respectively). Subjects undergoing craniotomy had an average of 5.1 ± 4.5 subsequent shunt-related interventions versus 3.1 ± 4.0 in the endoscopy group (p = 0.25). The craniotomy group's median subsequent shunt revision rate was 0.74 interventions per year versus 0.50 interventions per year in the endoscopy group (p = 0.51). Fifty percent of subjects in the open fenestration group required additional fenestration surgery compared to 38.5% in the endoscopic group (p = 0.70). CONCLUSION: Both open and endoscopic fenestration appeared effective at improving shunt management. The endoscopic technique may offer advantages in operative time, blood loss, and length of hospital stay. These data suggest that endoscopic fenestration may be used as the initial approach for treatment of multiloculated hydrocephalus, with craniotomy and open fenestration used for more severe or refractory cases.

Smoking cession decreases mean platelet volume in healthy Korean populations.

BACKGROUND: Smoking is considered as a major modifiable risk factor for cardiovascular diseases. It has been shown that smoking cessation drops the risk of cardiovascular diseases such as myocardial infarction and also improves platelet function. Because mean platelet volume (MPV) is a simple and convenient indicator for platelet activation, we planned to investigate the effect of smoking status on MPV in healthy populations. METHODS: This study was conducted on 398 individuals who visited our hospital for regular medical check-ups and were confirmed not to have diabetes or hypertension. MPV was measured using EDTA blood on an Advia 2120 (Siemens Healthcare Diagnostics Inc., Tarrytown, NY, USA) within 2 hours. RESULTS: Present smokers showed higher MPV levels than present non-smokers. When MPV was compared by taking previous smoking history and present smoking status into account, the smoking cessation group showed significantly lower MPV levels than other groups. CONCLUSIONS: Because this finding was significant only in the female group, the change in MPV according to smoking status was found to be different by gender. We carefully suggest that smoking cessation can lower the risk of cardiovascular diseases through the change in MPV, which can be more effective for women than men.

The MLL recombinome of acute leukemias in 2013.

Chromosomal rearrangements of the human MLL (mixed lineage leukemia) gene are associated with high-risk infant, pediatric, adult and therapy-induced acute leukemias. We used long-distance inverse-polymerase chain reaction to characterize the chromosomal rearrangement of individual acute leukemia patients. We present data of the molecular characterization of 1590 MLL-rearranged biopsy samples obtained from acute leukemia patients. The precise localization of genomic breakpoints within the MLL gene and the involved translocation partner genes (TPGs) were determined and novel TPGs identified. All patients were classified according to their gender (852 females and 745 males), age at diagnosis (558 infant, 416 pediatric and 616 adult leukemia patients) and other clinical criteria. Combined data of our study and recently published data revealed a total of 121 different MLL rearrangements, of which 79 TPGs are now characterized at the molecular level. However, only seven rearrangements seem to be predominantly associated with illegitimate recombinations of the MLL gene (≈ 90%): AFF1/AF4, MLLT3/AF9, MLLT1/ENL, MLLT10/AF10, ELL, partial tandem duplications (MLL PTDs) and MLLT4/AF6, respectively. The MLL breakpoint distributions for all clinical relevant subtypes (gender, disease type, age at diagnosis, reciprocal, complex and therapy-induced translocations) are presented. Finally, we present the extending network of reciprocal MLL fusions deriving from complex rearrangements.

Radiation therapy for pilocytic astrocytomas of childhood.

PURPOSE: Though radiation therapy is generally considered the most effective treatment for unresectable pilocytic astrocytomas in children, there are few data to support this claim. To examine the efficacy of radiation therapy for pediatric pilocytic astrocytomas, we retrospectively reviewed the experience at our institution. METHODS AND MATERIALS: Thirty-five patients 18 years old or younger with unresectable tumors and without evidence of neurofibromatosis have been treated since 1982. Patients were treated with local radiation fields to a median dose of 54 Gy. Six patients were treated with radiosurgery to a median dose of 15.5 Gy. Five patients were treated with initial chemotherapy and irradiated after progression. RESULTS: All patients were alive after a median follow-up of 5.0 years. However, progression-free survival was 68.7%. None of 11 infratentorial tumors progressed compared with 6 of 20 supratentorial tumors. A trend toward improved progression-free survival was seen with radiosurgery (80%) compared with external beam alone (66%), but this difference did not reach statistical significance. Eight of the 9 patients progressing after therapy did so within the irradiated volume. CONCLUSIONS: Although the survival of these children is excellent, almost one third of patients have progressive disease after definitive radiotherapy. Improvements in tumor control are needed in this patient population, and the optimal therapy has not been fully defined. Prospective trials comparing initial chemotherapy to radiation therapy are warranted.