scholarly journals Klippel–Feil Syndrome with Sprengel Deformity and Extensive Upper Extremity Deformity: A Case Report and Literature Review

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
John W. Stelzer ◽  
Miguel A. Flores ◽  
Waleed Mohammad ◽  
Nathan Esplin ◽  
Jonathan J. Mayl ◽  
...  

Introduction. Klippel–Feil syndrome (KFS) is a congenital anomaly resulting from fusion of cervical vertebral bodies secondary to the dysregulation of signaling pathways during somite development. It is commonly associated with scoliosis and Sprengel deformity. We present a case of KFS with commonly associated abnormalities as well as deformities that have not yet been reported in the literature. Case Presentation. A 3-year-old girl presented for further evaluation of a left upper extremity deformity following a negative genetic workup. Upon physical exam and radiographic imaging, the patient was diagnosed with KFS and associated abnormalities including cervical scoliosis, Sprengel deformity, and congenital deformity of the left upper extremity. Deformities of the left upper extremity include radioulnar synostosis, a four-rayed hand, and absent thenar musculature. The Sprengel deformity was corrected surgically with a Woodward procedure. Discussion. Congenital musculoskeletal deformities can be differentiated based upon spinal and limb embryology. The presence of extraspinal abnormalities not originating from somite differentiation may suggest a severe form of KFS. Important considerations in the workup of the KFS patient include looking for deformities of the shoulder girdle and upper extremities to identify abnormalities for intervention at a young age.

2021 ◽  
pp. 074880682110310
Author(s):  
Angelo Cuzalina ◽  
Pasquale G. Tolomeo

The aim of this study was to determine the efficacy of cool atmospheric plasma (Renuvion/J-plasma) in promoting skin tightening and soft tissue contouring following liposuction of the upper extremities. The study was a retrospective review of upper extremity liposuction with associated Renuvion therapy performed by the same surgeon. Patients were made aware of Renuvion therapy to assist with skin laxity and offered adjunctive treatment following liposuction. While a majority of patients elected to have Renuvion therapy performed bilaterally, a small subset of patients elected for unilateral treatment. This subset of patients pursued delayed treatment on the control side. The inclusion criteria for the study included patients with moderate fat excess of the upper extremity with associated mild to moderate cutaneous laxity. Exclusion criteria for the study included severe medical comorbidities, body mass index greater than 35 kg/m2 and those below the age of 30. The study included 5 female patients between the ages of 46 to 52. The method of treatment was liposuction of the bilateral upper extremities with removal of equal proportions of fat. The recipient site for Renuvion treatment was randomly selected by the study coordinator; the surgeon and clinical staff remained blinded to the selection. Following treatment, the patients were evaluated at 1 week, 6 weeks, and 6 months postoperatively to assess surgical outcomes subjectively. The surgeon and clinical staff were unblinded at the final visit. Patients were evaluated based on subjective criteria and photographic evaluation at each postoperative visit. At the 1-week visit, no significant differences were noted in all subjects. At the 6-week visit, two patients demonstrated improved results to the treatment site when compared with the control site. At the 6-month visit, four out of the five patients demonstrated a significant improvement in contour and laxity at the treatment site when compared with the control site. One patient demonstrated equal results on both treatment and control sites with no major abnormalities. Following the final evaluation, the patients underwent a secondary procedure to the control site with Renuvion to obtain similar results as the recipient site. One patient demonstrated equal results on both test and control sites with no major abnormalities. The use of plasma energy via Renuvion in conjunction with liposuction has demonstrated esthetic results with proposed long-term benefits. The plasma energy device, as an adjuvant therapy, may be beneficial in cases where liposuction alone may not address tissue laxity concerns. Additional studies with a larger sample size, objective criteria, and extended follow-ups are necessary to statistically analyze the results and determine its significance.


2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Shahana Perveen ◽  
Karmaine A. Millington ◽  
Suchitra Acharya ◽  
Amit Grag ◽  
Vita Boyar

AbstractObjectivesTo describe challenges in diagnosis and treatment of congenital neonatal gangrene lesions associated with history of maternal coronavirus disease 2019 (COVID-19) infection.Case presentationA preterm neonate was born with upper extremity necrotic lesions and a history of active maternal COVID-19 infection. The etiology of his injury was challenging to deduce, despite extensive hypercoagulability work-up and biopsy of the lesion. Management, including partial forearm salvage and hand amputation is described.ConclusionsNeonatal gangrene has various etiologies, including compartment syndrome and intrauterine thromboembolic phenomena. Maternal COVID-19 can cause intrauterine thrombotic events and need to be considered in a differential diagnosis.


1999 ◽  
Vol 91 (5) ◽  
pp. 885-888 ◽  
Author(s):  
Yasushi Miyagi ◽  
Fumio Shima ◽  
Katsuya Ishido ◽  
Masashi Moriguchi ◽  
Kazufumi Kamikaseda

✓ This 49-year-old man gradually developed a disabling action tremor in the proximal right upper extremity 8 months after suffering a pontine tegmental hemorrhage. The intraoperative microrecording in the nucleus ventralis intermedius (VIM) of the left thalamus revealed tremor-synchronous grouped discharges with a vigorous (2.7 Hz) action tremor predominantly in the shoulder and upper arm. High frequency electrical stimulation in the VIM did not affect the tremor. A posteroventral pallidotomy (PVP) was performed and resulted in the successful alleviation of all tremor activity. Posteroventral pallidotomy is known to alleviate parkinsonian tremors, especially those occurring in the contralateral lower extremity, trunk, and proximal segment of the contralateral upper extremity. The authors consider the pallidoreticular pathway to be an important tremor-mediating pathway for the proximal segment of the upper extremities and believe it can be controlled more effectively by PVP than by VIM thalamotomy, as demonstrated by the PVP-induced resolution of the midbrain tremor observed in this case.


Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000011992
Author(s):  
David J Lin ◽  
Kimberly S Erler ◽  
Samuel B Snider ◽  
Anna K Bonkhoff ◽  
Julie A DiCarlo ◽  
...  

Objective:To test the hypothesis that cognitive demands influence motor performance during recovery from acute stroke, we tested acute stroke patients on two motor tasks with different cognitive demands and related task performance to cognitive impairment and neuroanatomic injury.Methods:We assessed the contralesional and ipsilesional upper extremities of a cohort of 50 patients with weakness after unilateral acute ischemic stroke at three timepoints with two tasks: the Box & Blocks Test, a task with greater cognitive demand, and Grip Strength, a simple and ballistic motor task. We compared performance on the two tasks, related motor performance to cognitive dysfunction, and used voxel-based lesion symptom mapping to determine neuroanatomical sites associated with motor performance.Results:Consistent across contralesional and ipsilesional upper extremities and most pronounced immediately post-stroke, Box & Blocks scores were significantly more impaired than Grip Strength scores. The presence of cognitive dysfunction significantly explained up to 33% of variance in Box & Blocks performance but was not associated with Grip Strength performance. While Grip Strength performance was associated with injury largely restricted to sensorimotor regions, Box & Blocks performance was associated with broad injury outside sensorimotor structures, particularly the dorsal anterior insula, a region known to be important for complex cognitive function.Conclusions:Altogether, these results suggest that cognitive demands influence upper extremity motor performance during recovery from acute stroke. Our findings emphasize the integrated nature of motor and cognitive systems and suggest that it is critical to consider cognitive demands during motor testing and neurorehabilitation after stroke.


Author(s):  
Anand Hammad ◽  
Anil Kalra ◽  
Prashant Khandelwal ◽  
Xin Jin ◽  
King H. Yang

Injuries to the upper extremities that are caused by dynamic impacts in crashes, including contact with internal instrument panels, has been a major concern, especially for smaller female occupants, and the problem worsens with increasing age due to reduced strength of the bones. From the analysis of 1988–2010 CDS unweighted data, it was found that risk of AIS ≥ 2 level for the arm was 58.2±20.6 percent higher in females than males, and the injury risk for a 75-year-old female occupant relative to a 21-year-old subjected to a similar physical insult was 4.2 times higher. Although injuries to upper extremities are typically not fatal, they can have long-term effects on overall quality of life. Therefore, it is important to minimize risks of injuries related to upper extremities, especially for elderly females, who are most at risk. Current anthropomorphic surrogates, like crash-test dummies, cannot be directly used to study injury limits, as these dummies were developed mainly to represent the younger population. The current study is focused on the development of a finite element (FE) model representing the upper extremity of an elderly female. This can be further used to analyze the injury mechanisms and tolerance limits for this vulnerable population. The FE mesh was developed through Computer Tomography (CT) scanned images of an elderly female cadaver, and the data included for validation of the developed model were taken from the experimental studies published in scientific literature, but only the data directly representing elderly females were used. It was found that the developed model could predict fractures in the long bones of elderly female specimens and could be further used for analyzing injury tolerances for this population. Further, it was determined that the developed segmental model could be integrated with the whole body FE model of the elderly female.


2022 ◽  
Vol 20 ◽  
Author(s):  
Mary M Czech ◽  
William Ogden ◽  
Rashmi Batra ◽  
Joseph D Cooper

Background: Multilocular thymic cysts (MTCs) in adults with human immunodeficiency virus (HIV) are rarely reported. Case Presentation: We describe a case of symptomatic MTC in a male with untreated HIV. A presumptive diagnosis was established based on radiographic imaging and biopsy. Pathologic diagnosis and exclusion of malignancy were ultimately confirmed the following thymectomy. In conjunction with starting antiretroviral therapy, the patient recovered well post-operatively with a resolution of his presenting symptoms. Conclusion: Our case report and review of the literature serve to highlight MTCs as an important clinical entity occurring in persons with HIV.


2021 ◽  
Author(s):  
Grigore Burdea ◽  
Nam H. Kim ◽  
Kevin Polistico ◽  
Ashwin Kadaru ◽  
Namrata Grampurohit ◽  
...  

BACKGROUND BrightArm Compact is a new rehabilitation system for upper extremities. It provides bimanual training with gradated gravity loading and mediates interactions with serious games. OBJECTIVE To design and test a robotic rehabilitation table-based virtual rehabilitation system for training upper extremities early post-stroke. METHODS A new robotic rehabilitation table, controllers and adaptive games were developed. Participants underwent 12 experimental sessions in addition to the standard of care. Standardized measures of upper extremity motor impairment and function, depression severity, and cognitive function were administered pre- and post-intervention. Non-standardized measures included game variables and subjective evaluations. RESULTS Two case study participants attained high total arm repetitions per session (504 and 957, respectively), and achieved high grasp and finger extension counts. Training intensity contributed to marked improvements in affected arm shoulder strength (225% and 100%, respectively), grasp strength (27% and 16% increase), 3-finger pinch strength (31% and 15% increase). Shoulder active flexion range increased 17% and 18%, respectively, and elbow active supination was larger by 75% and 58%, respectively. Improvements in motor function were at/above Minimal Clinically Important Difference for Fugl-Meyer Assessment (11 and 10 points), Chedoke Inventory (11 and 14 points) and Upper Extremity Functional Index (19 and 23 points). Cognitive/emotive outcomes were mixed. CONCLUSIONS The design of the robotic rehabilitation table was successfully tested on two participants early post-stroke. Results are encouraging. CLINICALTRIAL ClinicalTrials.gov NCT04252170


2018 ◽  
Vol 42 (1) ◽  
pp. 26-29
Author(s):  
Jason Roberts ◽  
Vaqar Ali ◽  
Mian Ahmed Hasan

Upper extremity pathology is uncommon and generally involves the brachial artery in about 12% of the cases. Of chronic upper limb ischemia, trauma, fibromuscular dysplasia, giant cell arteritis, and atherosclerosis are among the most reported cases. After a thorough review of the literature, there is agreement with duplex ultrasound sensitivity and specificity for predicting >70% stenosis in the subclavian arteries with a sensitivity of >82% and specificity of >90%. This article not only correlates the relationship between duplex ultrasound and severe peripheral arterial disease, but it also proves with 100% accuracy the ability to detect latent and abnormal pathology of the upper extremities post endovascular intervention.


2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Hiroki Kitaoka ◽  
Jun Inatomi ◽  
Hayato Chikai ◽  
Keiko Watanabe ◽  
Tadayuki Kumagai ◽  
...  

Abstract Background Renal abscess in children is a rare and severe form of infectious kidney disease that is responsible for several serious complications. In this report, we describe a previously healthy 5-year-old girl with a renal abscess caused by extended-spectrum β-lactamase (ESBL)-producing Escherichia coli (E. coli), which led to bacteremia and renal scarring. Case presentation The patient presented to our department with high fever, headache, vomiting for 2 days and high inflammatory response. We diagnosed her with a urinary tract infection and initiated treatment with ampicillin and cefotaxime. Gram-negative bacilli bacteremia was noted on day 3. On day 4, her fever persisted, and a computed tomography (CT) scan revealed a renal abscess in the left kidney. After identifying the bacteria as ESBL-producing E. coli from the blood culture, we switched to the antibiotic meropenem and continued treatment for 3 weeks. The renal abscess was not drained. Although the renal abscess was successfully treated and it disappeared, a low-density area remained in same lesion on subsequent CT scans and a dimercaptosuccinic acid renal scan performed 4 months after onset revealed renal scarring. Conclusion Given the increasing prevalence of ESBL-producing microorganisms, clinicians should be aware of the possibility of renal abscesses caused by community-acquired ESBL-producing organisms even in previously healthy children. Once a renal abscess is suspected, early diagnosis and management are important for reducing the risk of life-threating complications and renal scarring.


Sign in / Sign up

Export Citation Format

Share Document