Musculoskeletal Deformities Research Articles

Charcot neuroarthropathy of the knee due to idiopathic sensory peripheral neuropathy

BackgroundCharcot neuroarthropathy is a systemic disease that generates pathological changes in the musculoskeletal system, causing instability, dislocations, and deformities. Charcot neuroarthropathy of the knee, due to either diabetes mellitus or syringomyelia, is anecdotally reported with the epidemic of the diseases. However, idiopathic sensory peripheral neuropathy can inflict osteoarticular structures directly, inducing a dysfunctional Charcot neuroarthropathy. An early diagnosis and effective relief of the symptomatic deformity is essential for the treatment.Case presentationWe report the case of a patient with idiopathic sensory peripheral neuropathy who presented with a swelling right knee, as well as distorted and painless gait disorder, diagnosed as Charcot neuroarthropathy of the knee. Partial weight bearing with a hinged knee brace was used to correct the abnormal alignment and gait posture, and bisphosphonates were prescribed to decrease pathological bone resorption. Although the alignment and Knee Society Score got a gradual deterioration, the combination of orthosis and pharmacy could alleviate the symptom to a certain extent.ConclusionThe diagnosis of Charcot neuroarthropathy of the knee is rare that requiring early diagnosis. The presence of features, including painlessness, numbness, and deformed arthropathy following chronic-onset algesthesia loss should be taken carefully.

The Role of Index and Ring Fingers in Gender Identification and Height Estimation

Background: It is a challenge in forensic medicine to identify deceased bodies when body dismembered from remaining body parts, such as hands, arms, and feet. We aimed to determine gender and the correlation between stature, and index and ring fingers in an Iranian population. Methods: In this cross-sectional study, 200 Iranian students aged between 18 and 25 years were included (2016-2017). The length of the nondominant index and ring fingers were measured from proximal crease to the tip. The exclusion criteria were a history of heavy physical work by hand, hand vocational sports, anatomical musculoskeletal deformities, and chronic internal diseases (diabetes, thyroid disorders, renal failure, etc.). The obtained data were analyzed using SPSS. t-test, Pearson’s correlation coefficient, and correlation and regression models were used to analyze the achieved data. Results: The mean height was 179 cm in males and 164 cm in females. The mean index and ring fingers lengths were 73 mm and 74 mm in males, and 68 mm and 68 mm in females, respectively. The mean sum of index and ring fingers lengths were 147 mm in males and 136 mm in females. Height, index and ring fingers length, and the sum of them significantly differed between genders (P<0.0001). The accuracy of gender determination was 92%, 71%, 73% and 74.5% in terms of stature, index finger length, ring finger length, and the sum of index and ring fingers length, respectively. The correlation between height and index finger length, as well as the height and ring finger length, were significant in males, females, and total cases (P<0.0001). Conclusion: Based on this study, index and ring fingers lengths can be used to predict height and determine gender.

Postural stability in people with visual impairment.

BackgroundThe visual system enables the brain to assess information regarding the position of the body in space. Congenital or acquired blindness leads to the development of abnormal sensory‐motor interactions that results in development of typical musculoskeletal deformities and gait patterns that cause disability. Diabetes and related complications are expected to increase exponentially in the next 10 years; thus, the number of people with visual impairment is expected to increase. However, there have been few studies regarding etio‐pathogenesis of postural alteration and balance impairment in people with visual impairment; moreover, no previous study has investigated postural stability in this population. This study aimed to assess the center of gravity (COG) velocity in subjects with visual impairment and compared with that in sighted subjects.MethodsSeventy male subjects, 20–40 years of age, participated in this study; they were divided into sighted (control) and visually impaired groups. COG velocity while standing on an unstable surface was measured using the NeuroCom® Balance Master Version 8.5.0. For the sighted group, data were recorded with eyes open and then with eyes closed. For the visually impaired group, no instructions were given with respect to eyes during data collection.ResultsMean COG velocity was significantly higher in the visually impaired group than in the sighted group with eyes open. However, there was no difference in mean COG velocity between the visually impaired group and the sighted group with eyes closed. The difference in mean COG velocity between sighted subjects with eyes open and eyes closed was also significant. Mean COG velocity while standing on a foam surface varied among visually impaired subjects and sighted subjects with eyes open and closed.ConclusionThis study showed that subjects with visual impairment, regardless of eye opening or closure, behave in the same manner as sighted subjects with eyes closed.

Can a reduction approach predict reliable joint contact and musculo-tendon forces?

Musculoskeletal models generally solve the muscular redundancy by numerical optimisation. They have been extensively validated using instrumented implants. Conversely, a reduction approach considers only one flexor or extensor muscle group at the time to equilibrate the inter-segmental joint moment. It is not clear if such models can still predict reliable joint contact and musculo-tendon forces during gait.Tibiofemoral contact force and gastrocnemii, quadriceps, and hamstrings musculo-tendon forces were estimated using a reduction approach for five subjects walking with an instrumented prosthesis. The errors in the proximal-distal tibiofemoral contact force fell in the range (0.3–0.9 body weight) reported in the literature for musculoskeletal models using numerical optimisation. The musculo-tendon forces were in agreement with the EMG envelops and appeared comparable to the ones reported in the literature with generic musculoskeletal models.Although evident simplifications and limitations, it seems that the reduction approach can provided quite reliable results. It can be a useful pedagogical tool in biomechanics, e.g. to illustrate the theoretical differences between inter-segmental and contact forces, and can provide a first estimate of the joint loadings in subjects with limited musculoskeletal deformities and neurological disorders.

Left congenital diaphragmatic hernia-associated musculoskeletal deformities.

With the advancement in the treatment strategies of congenital diaphragmatic hernia (CDH), there is an increase in the survival rates. This fact leads to an increase in the morbidity and extrapulmonary complications in the long term such as failure to thrive, hernia recurrence, neurodevelopmental delay, gastrointestinal problems, and musculoskeletal anomalies. Herein, we aim to investigate the association between the long-term musculoskeletal complications in CDH patients regarding the defect size, repair type, and perinatal parameters. After Institutional Review Board approval was obtained (2017-6361), a retrospective chart review was performed on CDH patients from 2003 to 2016. Patients who were operated due to left-sided isolated congenital diaphragmatic hernia and survived to date were included in the study. Data were collected on demographics, preoperative characteristics, operative interventions, and postoperative outcomes. Statistical analysis was performed with IBM SPSS Statistics 20.0.0 (Chicago, IL). There were 98 patients with left CDH of whom 33 (33.7%) had primary repair, 25 (25.5%) had patch repair, and 40 (40.8%) had muscle flap repair. The median age of the patients was 6.00 ± 3.83years. 45 patients (45.9%) had large diaphragmatic defects, 28 patients (28.6%) had at least one type of musculoskeletal deformities, 2 of which were pectus carinatum, 16 were pectus excavatum, and 18 were scoliosis. CDH patients who had small diaphragmatic defects and repaired with a patch were less likely develop musculoskeletal deformities while who had primary abdominal closure after ventral hernia significantly have more pectus excavatum. Although there was a trend towards an increased risk of the pectus deformity and scoliosis in patients repaired with muscle flap, it did not reach statistical significance. There is a correlation between musculoskeletal deformities and the severity of the CDH.

An ultra-rare mutation (C.181C>T) in GALNS gene associated with Morquio syndrome: A case report

MotivationOne of the rare inherited diseases is the mucopolysaccharidoses disorders (MPS) associated with accumulation of glycosaminoglycan in several organs, leading to abnormalities in musculoskeletal, respiratory, cardiac, neurological, ophthalmological, otolaryngological, and gastrointestinal. Nowadays, an increasing number of patients with MPS are reaching adulthood and are involved in family planning due to improvements in diagnosis, multi-disciplinary care, and therapies such as enzyme replacement therapy and hematopoietic stem cell transplantation. Knowledge on skeletal deformity in MPS remains relatively uncommon and includes a few case reports. To assess more, we present a case report on musculoskeletal deformity in one subject with MPS. CaseThis case revealed that subject with MPS has generalized osteoporosis, Degenerative Joint Disease (DJD) of both hips, general skeletal deformity alongside normal mental status. On physical examination, disproportionate short stature, skeletal dysplasia, osteoporosis, prognathism, negative ulnar variance, angle blunting and ulnar styloid were detected. In the present research work, C.181C>T (p.Arg61Trp) variant of GALNS gene was found through whole exome sequencing and the mutation was confirmed using Sanger sequencing method, which is associated with MPS4A phenotype. ConclusionsPathogenic variant in the GALNS gene associated with hereditary Mucopolysaccharidosis IVA, (MPS4A, Morquio syndrome A) which encodes N-acetylgalactosamin-sulfate sulfatase, may be useful as a biomarker to early diagnosis and treatment.

Safety and Efficacy of a Propofol and Ketamine Based Procedural Sedation Protocol in Children with Cerebral Palsy Undergoing Botulinum Toxin A Injections.

Pediatric patients with cerebral palsy often undergo intramuscular botulinum toxin (BoNT-A) injections. These injections can be painful and may require procedural sedation. An ideal sedation protocol has yet to be elucidated. To investigate the safety and efficacy of a propofol and ketamine based sedation protocol in pediatric patients with cerebral palsy requiring BoNT-A injections. Retrospective chart review. The sedations took place in a procedural sedation suite at a tertiary children's hospital from February 2013 through September 2017. 164 patients with diagnoses of cerebral palsy undergoing propofol and ketamine based sedation for injections with botulinum toxin A. An initial bolus of 0.5 mg/kg ketamine followed by a 2 mg/kg bolus of propofol was administered with supplemental boluses of propofol as needed to achieve deep sedation during the intramuscular BoNT-A injections. Propofol dosages, adverse events, serious adverse events, and sedation time parameters were reviewed. 345 sedations were successfully performed on 164 patients. The median total dose of propofol was 4.7 mg/kg (interquartile range [IQR]: 3.5, 6.3). Adverse events were encountered in 10.1% of procedures including hypoxemia responsive to supplemental oxygen (9.6%) and transient apnea (1.4%). The mean procedure time, recovery time, and total sedation time were 10, 11 and 33 minutes, respectively. With regard to patient variables, including age, weight, dose of propofol, sedation time, and Gross Motor Function Classification System classification, there was no association with increased incidence of adverse events. Our sedation protocol of propofol and ketamine is safe and effective in children with cerebral palsy undergoing procedural sedation for intramuscular injections with BoNT-A. The adverse events encountered appeared to be related to airway and respiratory complications secondary to musculoskeletal deformities, emphasizing the importance of airway monitoring and management in these patients. IV.

Outcomes after Split Abdominal Wall Muscle Flap Repair for Large Congenital Diaphragmatic Hernias.

Repair of large congenital diaphragmatic hernias (CDHs) is challenging. As primary repair is not always feasible, patches are commonly used. An alternative treatment is split abdominal wall muscle flap repair, which uses vascularized autologous tissue. The aim of this study was to analyze the long-term outcome of large CDH defects undergoing split abdominal wall muscle repair. This is a retrospective review (2003-2016) of large CDH treated by split abdominal wall muscle flap repair. In a total of 107 CDH patients, the abdominal muscle flap technique was used in 10 (9.3%); 7 had been prenatally treated with tracheal occlusion. Two patients experienced recurrence at 2 months and 6 years, respectively. Only one patient required abdominoplasty due to abdominal wall muscle weakness. Two patients developed progressive scoliosis; one of them required orthopaedic treatment. Minor chest wall deformities were detected in seven, but only one required orthopaedic treatment. The lung-to-head ratio was 0.79 in patients developing musculoskeletal deformities, and 1.5 in those without this complication (p < 0.05). Median follow-up was 11.2 years (3.5-14.2), and all patients were alive at the time of writing this article. The split abdominal wall muscle flap technique is a valid option for repair of large CDH. Associated musculoskeletal deformities seem to be influenced not only by the repair technique used but also by the degree of pulmonary hypoplasia and inherent pathophysiological changes.

Single-Event Multilevel Surgery: Contender or Pretender.

* Abbreviation: SEMLS — : single-event multilevel surgery Single-event multilevel surgery (SEMLS) refers to the surgical management of children and adult patients with cerebral palsy and spastic diplegia to address muscle imbalance, contractures, and musculoskeletal deformities secondary to untreated or inadequately treated spasticity.1–3 Spasticity is a movement disorder in which there is injury to upper motor neurons comprising the pyramidal tract. The upstream inhibitory influence of the central nervous system is interrupted, causing the local reflex arc in the spinal cord to become disinhibited and leading to continuous muscle contraction. The spasticity is characterized by a velocity-dependent increase in muscle tone with hyperreflexia. Surgical management consists of concurrent orthopedic procedures targeting the musculoskeletal system at ≥2 levels (eg, feet, ankles, knees, and hips). Procedures include tendon lengthening to correct contractures, tendon transfers for muscle imbalance, rotational osteotomies for torsional deformities, and stabilization of the hip and foot. The advantages of SEMLS include a single hospital … Address correspondence to Andrew Jea, MD, MHA, FAAP, Section of Pediatric Neurosurgery, Indiana University School of Medicine, Riley Hospital for Children, 705 Riley Hospital Dr, 1134, Indianapolis, IN 46202. E-mail: ajea{at}goodmancampbell.com

Postpolio Syndrome: A Review of Lived Experiences of Patients.

Postpolio syndrome (PPS) refers to a group of conditions that are present in patients, years after recovery from initial acute paralytic poliomyelitis. About 15%–80% of 20 million polio survivors worldwide will experience exacerbation of symptoms which typically appear 15–30 years after the resolution of initial poliomyelitis. Symptoms include new muscle weakness, fatigue, myalgia, joint pain, dysphagia, and difficulty breathing. Other reported symptoms include cold intolerance, sleep disorder, dysphonia, loss of stamina, musculoskeletal deformities, cardiovascular disorders, psychosocial problems, and restless legs syndrome. These symptoms are attributed to the superimposed neuronal loss of aging with inflammatory mechanisms, but without any convincing evidence of viral reactivation. Risk factors include female gender, respiratory symptoms, normal aging, permanent disability caused by motor neuron damage, muscle overuse and disuse, aging, and immunologic mechanisms. Hypothyroidism-induced myopathy and fibromyalgia are a differential diagnosis for PPS, and exclusion diagnosis is required as confirmatory criteria for PPS. The symptoms of PPS presented determine the course of management.

Prevalence of Musculoskeletal Pain and Postural Deformity in Papad Making Women —A Cross-Sectional Observational Study

Aim and Objective: To study musculoskeletal problems like musculoskeletal pain, postural deformity and nerve radiculopathy in women engaged in Papad making by clinical assessment. Methodology: A cross-sectional observational survey carried out at Shri. Mahila Gruh Udyog Lijjat Papad, Nandanvan, Nagpur (2014). 50 female subjects were selected for the study. After taking permission from factory in-charge, the survey was conducted at the factory site and subjects were explained about the study in details in language they can understand. Consent form was obtained by subjects to participate. Data were collected by interview and clinical examination of women engaged in this occupation, As per proforma of musculoskeletal examination. Result: Almost 37 subjects out of 50 complaints about low back pain as a chief complaint followed by neck pain in 34, thoracic kyphosis was more profound postural deviation seen in 10 subjects. Paraspinal and calf muscle spasm is more common. Radiating pain and numbness more in dominant hand suggestive of involvement of median nerve were confirmed by Upper Limb Traction Test [ULTT]. Discussion: Socioeconomic status greatly influences the working needs of women. Shri. Mahila Gruh Udyog Lijjat Papad factory provides a platform for Women Empowerment. Faulty posture adoption for longer duration during work gives rise to various musculoskeletal problems. Conclusion: 74% of the study population has low back pain as a chief musculoskeletal problem. 34% present with postural deviation and 82% belong to poor socioeconomic scale.

Preferential Associated Malformation in Patients With Anotia and Microtia

Few epidemiological studies have investigated the external ear malformations anotia and microtia. The authors' study aimed to investigate the relationships between age, sex, type of microtia, and birth place and associated malformation as seen in 1 private Japanese clinic. Cases of anotia/microtia that presented in Nagata Microtia and Reconstructive Plastic Surgery Clinic (Saitama, Japan) between 2005 and 2018 were included in the study. The authors identified 1896 patients with anotia/microtia with or without associated malformation. Most were primary cases, with some secondary reconstruction cases wherein the primary surgery was performed at another hospital. Cases were classified with Nagata classification; lobule type, small concha type, concha type, and anotia. Cryptotia was also observed in this study. Among the patients, 61.1% were male, 85.4% had unilateral defects (69.0% LB), and 59.1% had a right-sided defect. Most patients were less than 1 year old (15.0%) or 8 to 10 years old (5.5%-6.3%) on first examination, while 58% were from the Kanto region, including Tokyo. Regarding concomitant disorders, 32.7% had an accompanying malformation, while 13.3% had associated syndromes (eg, craniofacial microsomia, Treacher Collins syndrome). To conclude, in the authors' clinic, most cases of anotia/microtia were LB, unilateral, and seen in male patients. Information regarding several characteristic clinical features was obtained, especially that clefts and musculoskeletal deformities of the skull/face and jaw were the main accompanying malformations.

Outcome in prenatal diagnosis of isolated or complex clubfoot – systematic review and case series

Clubfoot represents a congenital limb deformity, with a frequency of 1/1000 pregnancies, and it is one of the most common musculoskeletal deformities presenting at birth. Although it is diagnosed at birth, many cases are detected from the antenatal period, following an ultrasound examination. In about half of the cases, both feet are affected, boys being twice more likely than girls to have the deformity. Objective. This study aims to review the literature regarding the outcome of isolated clubfoot and the risk that stems from its association with other anomalies. Materials and method. The online database was used to detect studies that have evaluated the outcome of isolated clubfoot or associated with other abnormalities. The information from literature was compared with our experience at the Antenatal Diagnostic Unit from the Emergency County Clinical Hospital of Craiova, based on the ultrasound assessments performed during 2018. Results. If the malformation is isolated, it will have a good prognosis. It may be postural, especially in pelvic presentations associated with a reduced amniotic fluid. In complex clubfoot cases, associated with other structural abnormalities, a chromosomal abnormality may be diagnosed, or the cause may be neurological or muscular, and it is part of a syndrome. Conclusions. Because of the significant different prognosis, it is important to carry out an antenatal ultrasound diagnosis, as complex as possible, to discriminate between the presence of an isolated clubfoot from the cases where this abnormality is associated with other malformations.

Pain and health status in adults with myelomeningocele living in Sweden.

PURPOSE: To increase knowledge about pain and general health in adults with myelomeningocele, a health condition with several risk factors for pain such as musculoskeletal deformities, shunt dysfunctions, bowel problems, and urinary tract infections/stones.METHODS: Descriptive correlational pilot study ( 51, 53% males). Chi-square tests were used to test associations among presence, impact, severity, and pain site in relation to sex and age. ANOVA was used to analyze associations between sex, age, and general health (today).RESULTS: Seventy-three percent reported pain in the past four weeks. No significant sex or age differences were associated with the presence of pain. Women were significantly more likely to report that pain interfered with work, (1, 41) 5.53, 0.02. There were significant main effects for sex and age on general health (today), (2, 44) 5.63, 0.007, adjusted 0.17. Women scored lower on general health (today) (mean 63.58) than men (mean 76.33). Older individuals reported worse general health (today) than did younger (0.89, 2.79, 0.008).CONCLUSIONS: Pain was frequent, and pain sites differed widely. Women were more likely to report that pain interfered with work, and scored lower on health, as did older persons.

PREVALENCE OF ANATOMICAL/ORTHOPEDIC DISORDERS AND EFFECTIVE FACTORS IN CHILDREN AGED 6–14 YEARS

Background: Musculoskeletal disorders are common among children. Community- and school-based studies have revealed that the complaint of pain in childhood associated with musculoskeletal deformities is common. Objective: The present study aims to examine the prevalence of extremity, spinal and chest wall deformities and other disorders in children aged 6–14 years, and to determine the relationship between these disorders and nutrition, benefiting from sunlight, and familial factors. Method: The present cross-sectional study was conducted among 625 children aged 6–14 years attending elementary and secondary schools in rural, urban, and suburban areas. Children were evaluated for kyphosis, scoliosis, lordosis, chest wall disorders, and other disorders. Families were also asked about the factors that may be associated with these disorders. Chi-square test and binary logistic regression test were used for data analysis and p<0.05 was considered statistically significant. Results: Anatomical/orthopedic disorders were detected in 30% of the children. The most common anatomical disorders are genu varum and chest wall deformities. The most important risk factor for the development of anatomical/orthopedic disorders was family history. In addition, being male and living in the city center are important risk factors. After 2 years of age, yogurt and cheese consumption decrease the risk of genu varum. Conclusions: The important risk factor in the development of anatomical/orthopedic disorders is familial-genetic features. Gender and nutrition are effective factors as well.

Bone Marrow-Based and Longitudinal Blood-Based MRD Tracking in Newly Diagnosed Multiple Myeloma Patients Treated with Daratumumab, Carfilzomib, Lenalidomide and Dexamethasone (DKRd): A Correlative and Clinical Phase II Study

INTRODUCTIONUsing Carfilzomib, Lenalidomide and Dexamethasone (KRd) combination therapy in newly diagnosed multiple myeloma patients lead to ~40% minimal residual disease (MRD) negativity rate. Here, we use KRd in combination with daratumumab (DKRd); and treatment response is assessed with extensive correlative science including parallel bone-marrow-based and blood-based MRD tracking, together with targeted DNA sequencing of baseline bone marrow samples. Primary end-point is to rule out 60% and to target up to 80% MRD negativity rate.METHODSThis is a single-arm, Phase II clinical trial based on Simon's optimal two-stage design. The first cohort (twice-a-week carfilzomib) (N=41) has the following treatment schedule: 8 cycles of treatment; 28-day cycles with carfilzomib 20/36 mg/m2 days 1, 2, 8, 9, 15 and 16; lenalidomide 25 mg days 1-21; dexamethasone 40 mg weekly cycles 1-4, 20 mg after cycle 4; and daratumumab 16 mg/kg days 1, 8, 15, and 22 cycles 1-2, days 1 and 15 cycles 3-6, and day 1 cycles 7-8. The second cohort (once-a-week carfilzomib) (N=41): 8 cycles of treatment; 28-day cycles with carfilzomib 20/56 mg/m2 days 1, 8, and 15; lenalidomide, dexamethasone, and daratumumab are given at the same doses/schedules as the first cohort. For fit patients, stemcell collection is recommended after 4 to 6 cycles of therapy; DKRd therapy is resumed after collection to a total of 8 cycles DKRd. Treatment response is being assessed with parallel bone-marrow-based (10-color single tube flowcytometry, invivoscribe V(D)J sequencing) as well as blood-based (MALDI-TOF and QTOF-mass spectrometry [MS]) for MRD tracking. Baseline bone marrow samples are evaluated with targeted DNA sequencing for FISH-Seq and somatic mutational characteristics (myTYPE). Here, we present the first stage (N=28) of the first cohort (twice-a-week carfilzomib). We are waiting for the results to mature before the second stage (N=13) of the first cohort can open. The second cohort (once-a-week carfilzomib) is opening for enrollment in August 2018 (N=41).RESULTSThe first stage of the first cohort is fully enrolled; 28 patients meeting eligibility criteria were enrolled onto study (14 males, 14 females) between October 2017 and July 2018. Baseline characteristics include; median age 60 years (range 32-80 years); 12(43%) patients had high-risk FISH/SNP signature defined as one or more of the following: 1q+, t(4,14), t(14,16), t(14,20), and 17p-. At the submission of this abstract, 20 patients have completed one or more cycles DKRd; among these, 3 patients have completed all 8 cycles. The median number of cycles delivered is currently 4 (range 1-8). Full assessments with MRD assays have been completed in 3 patients:-Pt #1 obtained complete response (CR) after 3 cycles, and workup after the last cycle of therapy showed MRD-negativity (by 10-color single tube flowcytometry and V(D)J sequencing) in the bone marrow; and peripheral blood (serum) was negative by MALDI-TOF MS after completion of cycle 2.-Pt#2 obtained CR after 4 cycles, however, workup after cycle 5 showed MRD-positivity (by 10-color single tube flowcytometry and V(D)J sequencing) in the bone marrow; and peripheral blood (serum) was positive by MALDI-TOF MS throughout the end of the last cycle.-Pt#3 obtained CR after 4 cycles and after 6 cycles both 10-color single tube flowcytometry and V(D)J sequencing showed MRD-negativity in the bone marrow. However, MALDI-TOF MS detected small abnormal serum proteins in peripheral blood and remained positive throughout the end of cycle 8.Overall, the DKRd therapy is well tolerated and it has similar toxicity profile as KRd. Grade >3 adverse events were hypotension, musculoskeletal deformity, back pain, dyspnea, lung-infection, and febrile neutropenia. So far, 5 patients underwent dose reductions of lenalidomide.CONCLUSIONSIn this pre-planned interim analysis of our phase II study, we show that DKRd is a highly effective and well tolerated combination therapy for newly diagnosed multiple myeloma patients. Based on small numbers of patients who have completed the planned DKRd cycles and been evaluated by bone marrow-based MRD and peripheral-blood based assays, we show that highly sensitive protein assays may allow longitudinal MRD tracking in peripheral-blood. At the meeting, we will present updated results using longitudinal testing with MALDI TOF-MS and QTOF-MS on the entire cohort. DisclosuresLandgren:Takeda: Consultancy, Membership on an entity's Board of Directors or advisory committees, Research Funding; Janssen: Consultancy, Membership on an entity's Board of Directors or advisory committees, Research Funding; Amgen: Consultancy, Research Funding; Celgene: Consultancy, Research Funding; Pfizer: Consultancy; Karyopharm: Consultancy; Merck: Membership on an entity's Board of Directors or advisory committees. Lesokhin:Takeda: Consultancy, Honoraria; Janssen: Research Funding; Squibb: Consultancy, Honoraria; Bristol-Myers Squibb: Consultancy, Honoraria, Research Funding; Genentech: Research Funding; Serametrix, inc.: Patents & Royalties: Royalties. Mailankody:Juno: Research Funding; Physician Education Resource: Honoraria; Takeda: Research Funding; Janssen: Research Funding. Smith:Celgene: Consultancy, Patents & Royalties: CAR T cell therapies for MM, Research Funding. Hassoun:Oncopeptides AB: Research Funding. Shah:Amgen: Research Funding; Janssen: Research Funding. Arcila:Invivoscribe, Inc.: Consultancy, Honoraria. Ho:Invivoscribe, Inc.: Honoraria. Korde:Amgen: Research Funding.

Postpolio syndrome and the late effects of poliomyelitis. Part 1. pathogenesis, biomechanical considerations, diagnosis, and investigations.

Postpolio syndrome (PPS) is characterized by new muscle weakness and/or muscle fatigability that occurs many years after the initial poliomyelitis illness. Many theories exist regarding the pathogenesis of PPS, which remains incompletely understood. In contrast, the late effects of poliomyelitis are often a consequence of biomechanical alterations that occur as a result of polio-related surgeries, musculoskeletal deformities, or weakness. Osteoporosis and fractures of the polio-involved limbs are common. A comprehensive clinical evaluation with appropriate investigations is essential to fulfilling the established PPS diagnostic criteria. PPS is a diagnosis of exclusion in which a key clinical feature required for the diagnosis is new muscle weakness and/or muscle fatigability that is persistent for at least 1 year. Electromyographic and muscle biopsy findings including evidence of ongoing denervation cannot reliably distinguish between patients with or without PPS. Muscle Nerve 58:751-759, 2018.

Wednesday, September 26, 2018 7:35 AM–9:00 AM ePosters

BACKGROUND CONTEXT With the increasing frequency of spinal fusion surgery, there has been increased pressure on providers and hospital systems to decrease cost and increase the value of this procedure. One potential area of improvement is decreasing length of hospital stay. Minimally invasive surgery (MIS) of the spine is designed to improve outcomes by minimizing approach. Previous studies looking at length of stay after MIS spine surgery are limited by their retrospective nature, single-intuition, small sample size, poor long-term follow-up, as well as their ability to collect outcomes. Identification of pre-operative factors that can predict shorter length of stay would allow surgeons to stratify what surgeries can be performed in an outpatient setting or in an ambulatory surgery center as a means of further cost containment. PURPOSE The goal of this study was to identify pre-operative factors that would be predictive of shorter length of stay (LOS) after minimally invasive transforaminal interbody fusion (MIS TLIF) or lateral lumbar interbody fusion (LLIF). STUDY DESIGN/SETTING The MSSIC is a statewide, prospective, longitudinal, multicenter quality improvement registry. We retrospectively analyzed a subset of MSSIC patients who had undergone TLIF or LLIF from a single surgeon's experience. PATIENT SAMPLE Patients that underwent one or two level MIS TLIF or LLIF from 2015 to 2016 were eligible for enrollment into this study. Inclusion criteria include: spondylosis; intervertebral disc disorders; other and unspecified disorders of the back; other acquired musculoskeletal deformity (Grade 1 or 2 spondylolisthesis); other congenital musculoskeletal anomalies (Grade 1 or 2 spondylolisthesis); and complications peculiar to certain specified procedures. Exclusion criteria include: patient age is younger than 18 years; patient is incarcerated; patient's medical records are unavailable or inaccessible; and cases of moderate (25°–50°) and severe (>50°) scoliosis. Additional exclusion criteria include: pure thoracic cases; tumor; meningitis; preexisting spinal infection related to surgery; other disorders of bone and cartilage, spinal deformity, Grades 3 and 4 spondylolistheses; congenital anomalies of the nervous system; traumatic fracture; and spinal cord injury. OUTCOME MEASURES The primary outcome measure for this study was LOS. A LOS ≥4days was considered prolonged. METHODS Variables recorded prospectively include medical history, indications for surgery, surgical details, postoperative hospital course and any comorbidities. The modified frailty index was calculated for each patient; briefly, it is a numerical score that is dependent on the patient's past medical history. In addition, daily oral morphine equivalents (OME) were also calculated preoperatively (in milligrams) and considered for analysis. Multivariable logistic regression models were constructed to identify potential risk factors for increased LOS after MIS TLIF or LLIF. We assigned length of stay ≥4days as prolonged. RESULTS We identified 73 patients who met the inclusion criteria. Of these patients, 80.8% underwent a 1 level procedure, 19.2% underwent a 2 level procedure, and 13.6% experienced prolonged discharge. Preoperative OME did not affect length of stay (17.9 ±2.1 mg vs. 18.4±5.6 mg, p=.998). Number of levels of fusion did not affect length of stay. Modified frailty index was significantly higher in patients with delayed discharge (2.6±0.4vs. 1.6±0.15, p=.021). Following a multivariable regression, every 1-point on a cormorbidity scale increased the odds of belated discharge by 79% (p=.048). No affect was observed with morphine milligram equivalents. CONCLUSIONS Modified frailty index predicts length of stay after MIS TLIF and LLIF. Preoperative opioid use does not affect length of stay.

Clinical management of Friedreich's Ataxia: a report of two cases.

Friedreich's ataxia (FDRA) is the most common autosomal recessive, early-onset ataxia. FDRA is a progressive neurodegenerative disease that mainly affects the posterior (dorsal) columns of the spinal cord resulting in sensory ataxia. It manifests in initial symptoms of poor coordination and gait disturbance. We present two cases, a brother (54 years old) and sister (56 years old), with FDRA that are chronically institutionalized for incomplete quadriplegia without spasticity. Gait and postural ataxia, cerebellar dysarthria, oculomotor dysfunction, musculoskeletal deformities, hearing impairment, hypertrophic cardiomyopathy, and diabetes mellitus are also present. Neurological examination reveals extensor plantar responses and diminished to absent tendon reflexes. Both are wheelchair bound, cannot perform daily tasks and need assistance. Although there is no cure that can alter the natural course of the disease physiotherapy, management of spasticity and neuropathic pain, symptomatic treatment of heart failure and diabetes and nursing care can grant the patients quality of life.

Charcot Neuroarthropathy of the Foot and Ankle.

➤ Charcot neuroarthropathy (CN) is a systemic disease that generates pathological changes in the musculoskeletal system, causing fractures, dislocations, and deformities involving the foot and ankle.➤ A common underlying cause of CN is diabetes mellitus; a substantial risk of ulceration and deep infection contributes to a 15% rate of major amputation (i.e., at the level of the ankle or higher).➤ The goal of both nonsurgical and surgical treatment of CN is to obtain a stable, plantigrade foot free from ulcerations; an equally important goal is to manage the patient’s diabetes and obesity.➤ The primary indications for surgery are recurrent ulceration, substantial deformity, deep infection, and pain; common complications include nonunion, wound breakdown, and infection.➤ The principles of thorough joint preparation, deformity correction, and minimizing soft-tissue trauma are essential when arthrodesis is performed for CN, which requires more robust (internal and/or external) fixation and prolonged off-loading.