Pott Disease in a 13-Month-Old: Case Report

Neurosurgery ◽  
2011 ◽  
Vol 68 (5) ◽  
pp. E1485-E1490 ◽  
Author(s):  
Giac Consigilieri ◽  
Udaya K. Kakarla ◽  
Nicholas Theodore
Keyword(s):  
Clinical Presentation ◽  
Posterior Instrumentation ◽  
The United States ◽  
Cord Compression ◽  
Common Disease ◽  
Unique Challenge ◽  
Very Young Children ◽  
Anterior Tibial ◽  
Steinmann Pin

Abstract BACKGROUND AND IMPORTANCE: Tuberculosis (TB) is a common disease worldwide that is caused by Mycobacterium tuberculosis. TB of the spine is the most common site of bony infection and is often referred to as Pott disease. To the best of our knowledge, our case represents the youngest patient with naturally acquired Pott disease in the United States. CLINICAL PRESENTATION: A 13-month-old boy presented with paraplegia and a known diagnosis of TB. His evaluation revealed a kyphotic gibbus deformity in the midthoracic region associated with severe spinal cord compression. The patient underwent urgent decompressive laminectomies, T2-T4 transpedicular corpectomies, placement of an anterior tibial strut graft, and posterior instrumentation with sublaminar wires and a Steinmann pin. At his 18-month follow-up, the patient was ambulating 5 steps at a time independently, and his kyphotic deformity showed no sign of progression. CONCLUSION: Pott disease can occur in very young children and presents a unique challenge when a patient presents with a neurological deficit and unstable deformity requiring surgical intervention.

scholarly journals Spinal angiolipoma: a report of two cases and review of the literature

2020 ◽  
Vol 48 (9) ◽  
pp. 030006052095469
Author(s):  
RuiDeng Wang ◽  
Hai Tang
Keyword(s):  
Standard Treatment ◽  
Clinical Presentation ◽  
Cord Compression ◽  
Benign Tumors ◽  
Fatty Tissue ◽  
Resonance Imaging ◽  
Total Resection ◽  
Vascular Channels ◽  
Spinal Angiolipomas

Spinal angiolipomas (SALs) are extremely rare benign tumors composed of both mature fatty tissue and anomalous vascular channels. We present two cases of SALs and review the clinical presentation, radiological appearance, pathological aspects, and treatment of this distinct clinicopathological mass. The patients’ neurologic symptoms improved postoperatively and follow-up revealed no signs of tumor recurrence or neurological deficit. SAL should be considered as a differential diagnosis in patients with spinal cord compression. Magnetic resonance imaging is important for detecting and characterizing SALs. The gold standard treatment modality should be total resection.

scholarly journals Multiple spinal extradural cysts causing progressive paraparesia: case report and review of literature

2012 ◽  
Vol 31 (01) ◽  
pp. 46-49
Author(s):  
Elton Gomes da Silva ◽  
Vinícius Teixeira Ribeiro ◽  
Bruno Vieira Scarpim ◽  
Yvens Barbosa Fernandes
Keyword(s):  
Spinal Cord ◽  
Clinical Presentation ◽  
Cord Compression ◽  
Subtotal Resection ◽  
Review Of Literature ◽  
Partial Removal ◽  
Asymptomatic Patients ◽  
History Of ◽  
Progressive Paraparesis

AbstractMultiple meningeal extradural cysts are extremely rare. The clinical presentation varies from asymptomatic patients to important symptoms due to spinal cord compression. This article reports the case of a girl with multiple meningeal extradural cysts with progressive paraparesis and hypoesthesia on inferior limbs. The MRI showed multiple extradural cysts between C7 and L1. A partial resection was made at the cystis, reflecting a improve at the postoperative follow up of the patient. The management of asymptomatic cases is usually followed up clinical and radiologically. At the management of the symptomatic cases, the resection of the cysts is the most indicated treatment, even for patients with a long-standing history of compression. However the partial removal has as good results as the total one. The authors review the literature and show a subtotal resection of the cysts did not change the evolution of the presentation.

scholarly journals Clinical presentation, genotype–phenotype correlations, and outcome of pancreatic neuroendocrine tumors in Von Hippel–Lindau syndrome

Endocrine ◽  
2021 ◽  
Author(s):  
F. Penitenti ◽  
L. Landoni ◽  
M. Scardoni ◽  
M. L. Piredda ◽  
S. Cingarlini ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Clinical Presentation ◽  
Genetic Mutation ◽  
Common Disease ◽  
Tumor Diameter ◽  
Pancreatic Neuroendocrine Tumors ◽  
Von Hippel Lindau ◽  
Node Metastases ◽  
Von Hippel Lindau Syndrome

Abstract Purpose Data regarding the clinical management and follow-up of pancreatic neuroendocrine tumors (PanNETs) associated with Von Hippel–Lindau (VHL) syndrome are limited. This study aimed to assess clinical presentation, genotype–phenotype correlations, treatment and prognosis of PanNETs in a series of VHL syndrome patients. Methods Retrospective analysis of data of patients observed between 2005 and 2020. Results Seventeen patients, including 12 probands and 5 relatives (mean age 30.8 ± 18.4; 7 males), were recruited. PanNETs were found in 13/17 patients (77.5%) at a median age of 37 years: 4/13 (30.7%) at the time of VHL diagnosis and 9 (69.3%) during follow up. Six (46.1%) PanNET patients underwent surgery, whereas seven were conservatively treated (mean tumor diameter: 40 ± 10.9 vs. 15 ± 5.3 mm respectively). Four patients (30.7%) had lymph node metastases and a mean tumor diameter significantly larger than the nonmetastatic PanNETs (44.2 ± 9.3 vs. 17.4 ± 7 mm, p = 0.00049, respectively). Five (83.3%) operated patients had stable disease after a median follow up of 3 years whereas one patient showed liver metastases. Six (85.7%) non-resected PanNETs were stable after a median follow-up of 2 years, whereas one patient developed a new small PanNET and a slight increase in diameter of a pre-existing PanNET. No correlation was found between the type of germline mutation and malignant behavior of PanNETs. Conclusions PanNETs are a common disease of the VHL syndrome and can be the presenting feature. Tumor size rather than genetic mutation is a prognostic factor of malignancy.

scholarly journals Differences in Clinical Presentation and Outcomes between Metropolitan and Rural Myeloma Patients

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 44-45
Author(s):  
Stephanie Clugston ◽  
Portia Smallbone ◽  
Duncan Purtill ◽  
Dustin Hall ◽  
Rebecca De Kraa ◽  
...  
Keyword(s):  
Overall Survival ◽  
Western Australia ◽  
Clinical Presentation ◽  
Statistical Significance ◽  
The United States ◽  
Early Mortality ◽  
First Line ◽  
Remote Areas ◽  
Significant Difference

Introduction: Australia's largest state, Western Australia (WA), comprises a land area of more than 2.5 million square kilometres, an area than larger than that of Texas and Alaska in the United States combined, with a population of more than 2.6 million. Whilst a large proportion of Western Australians live in the capital city Perth, approximately 20% are dispersed across the state in regional and remote areas. The diagnosis and treatment of myeloma require specialist Haematologist management and frequent follow-up. Access to Haematology specialist services and treatment in WA is centred in metropolitan Perth, with outreach services visiting regional and remote areas limited in location and frequency. Some patients are required to travel long distances or relocate to access treatment. The aim of our study was to assess difference in clinical presentation, treatment and outcomes of myeloma patients living in regional or remote Western Australia compared to metropolitan Perth. Methods: A retrospective chart review of new cases of symptomatic multiple myeloma diagnosed between January 2008 and December 2019 and referred to Royal Perth Hospital and Fiona Stanley Hospital, two tertiary metropolitan hospitals was conducted. Data was obtained regarding patient demographics, disease characteristics, treatment, response and survival outcomes, through review of patient paper and electronic medical records. Patients were grouped into those living inside or outside the Perth metropolitan area (metro or non-metro) according to area codes obtained from the WA government data suite. Results: Two hundred and seventy-five cases were identified, 218 (79%) metro and 57 (21%) non-metro. Baseline characteristics for the two groups are listed in Table 1. The median age at diagnosis was 68.4 years (range 30-91.5 years) and 47% were female, with no significant difference between the groups. There were a higher number of patients with lytic bone disease at diagnosis in the non-metro cohort (75.4% non-metro vs 60.2% metro, p=0.03) as well as a higher proportion of patients with international staging system (ISS) stage II or III disease (77.8% non-metro vs 55.8% metro, p=0.005). Sixty three percent of patients overall received first line bortezomib based therapy and 27% first line imid based therapy, with no significant difference by location. Overall 41% of patients underwent autologous stem cell transplantation, 70% of those ≤70 years of age, with no significant difference between the groups (33.3% non-metro vs 42.5% metro, p=0.21). The median overall survival (OS) was 47 months for the entire cohort. Survival was lower in the non-metro cohort, although this did not reach statistical significance (median OS 52 months for metro vs 40 months for non-metro, p=0.05) Figure 1. Progression free survival (PFS) was similar between the two groups (median PFS 23 months metro vs 12 months non-metro, p=0.12) Figure 2. Early mortality at 6 and 12 months was higher in the non-metro cohort (Six-month mortality was 21.1% non-metro vs 8.3% metro, p=0.01. Twelve-month mortality was 28.1% non-metro vs 13.4% metro, p=0.01) Figure 3. There was a trend in cause of early mortality due to infection being higher in the metro cohort, and cause of early mortality due to renal failure being higher in the non-metro cohort, Table 1. Conclusions: In our cohort, patients living in non-metropolitan locations were more likely to present with higher ISS stage and lytic lesions at diagnosis. Rates of early mortality were significantly higher in the non-metropolitan cohort. There was a trend towards shorter overall survival although this did not meet statistical significance. These differences may represent delays in clinical presentation and diagnostic workup and highlight the need for optimisation of follow up of patients in non-metropolitan areas particularly during the early time period post diagnosis. Periods of resource constraint and travel restrictions as is faced currently may accentuate these disparities. In addition, the nature of myeloma therapy is evolving with addition of treatments requiring expertise to deliver, such as monoclonal antibodies and chimeric antigen receptor T cells. As these therapies become commercial further studies are needed to assess adequacy of access for patients from non-metropolitan centres. Disclosures Leahy: Pfizer: Membership on an entity's Board of Directors or advisory committees. Sidiqi:Celgene: Honoraria, Other: Travel grant; Amgen: Honoraria; Janssen: Honoraria.

scholarly journals The Natural History and Clinical Presentation of Cervical Spondylotic Myelopathy

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Chester K. Yarbrough ◽  
Rory K. J. Murphy ◽  
Wilson Z. Ray ◽  
Todd J. Stewart
Keyword(s):  
Spinal Cord ◽  
Natural History ◽  
Cervical Spondylotic Myelopathy ◽  
Clinical Presentation ◽  
Clinical Course ◽  
The United States ◽  
Cord Compression ◽  
Asymptomatic Patients ◽  
History Of ◽  
Electrophysiologic Studies

Cervical spondylotic myelopathy (CSM) refers to impaired function of the spinal cord caused by degenerative changes of the cervical spine resulting in spinal cord compression. It is the most common disorder in the United States causing dysfunction of the spinal cord. A literature review of the natural history of mild cervical myelopathy is undertaken. Clinical presentation and current concepts of pathophysiology are also discussed. While many patients with mild signs of CSM will stabilize or improve over time with conservative treatment, the clinical course of a specific individual patient cannot be predicted. Asymptomatic patients with cervical stenosis and abnormalities on electrophysiologic studies may be at higher risk for developing myelopathy.

Adjacent Segment Disease: An Uncommon Complication after Cervical Expansile Laminoplasty: Case Report

Neurosurgery ◽  
2003 ◽  
Vol 53 (3) ◽  
pp. 770-773 ◽  
Author(s):  
Michael Y. Wang ◽  
Barth A. Green ◽  
Elizabeth Vitarbo ◽  
Allan D.O. Levi
Keyword(s):  
Clinical Presentation ◽  
Facet Joint ◽  
Posterior Instrumentation ◽  
Cord Compression ◽  
Adjacent Segment Disease ◽  
Adjacent Segment ◽  
Joint Motion ◽  
Disc Disease ◽  
Adjacent Level Degeneration ◽  
Uncommon Complication

Abstract OBJECTIVE AND IMPORTANCE Adjacent segment disc disease is a well-described phenomenon that occurs after anterior cervical spinal fusion. One of the advantages of cervical laminoplasty over anterior approaches is that although the treated segments are stiffened, no formal fusion is performed. This is thought to reduce the biomechanical stresses placed on adjacent levels and thus decrease the likelihood of adjacent level degeneration. CLINICAL PRESENTATION A 62-year-old man presented with myelopathy attributable to cervical spondylosis and underwent a C3–C7 laminoplasty. Improvements in gait were followed 2 years later by symptomatic disc degeneration and spinal cord compression at T1–T2, which rendered him wheelchair bound. INTERVENTION The patient was treated with a laminectomy at the level of stenosis accompanied by posterior instrumentation and fusion from C5 to T3. This resulted in clinical improvement, and the patient was returned to his baseline ambulatory status. CONCLUSION Adjacent segment disease is an uncommon complication that occurs after laminoplasty. Careful attention to preserving facet joint motion in the cervical spine may minimize the stresses placed on adjacent motion segments.

scholarly journals Needs, Expectations, and Experiences of International Students in Pathway Programs in the United States

2019 ◽  
Vol 9 (1) ◽  
pp. 192-210 ◽  
Author(s):  
Eman Elturki ◽  
Yang Liu ◽  
Justyna Hjeltness ◽  
Kate Hellmann
Keyword(s):  
International Students ◽  
Social Relationships ◽  
Support Services ◽  
The United States ◽  
Cultural Barriers ◽  
Individual Student ◽  
Unique Challenge ◽  
Additional Support ◽  
Sociocultural Experiences

This research assesses the first cohort of pathway students’ needs and their academic and sociocultural experiences at a U.S. university. A needs analysis survey, individual student interviews, and a follow-up survey were used for data collection. Understanding lectures, completing assignments, and building social relationships with domestics were among the challenges faced by pathway students due to linguistic and cultural barriers. An additional unique challenge for this particular population of international students centered around the fact that undergraduate and graduate students tackle discipline-related courses while still refining their English skills. Additional support services to help students in pathways academically and socio-culturally are crucial.

scholarly journals Obsessive compulsive disorder in very young children – a case series from a specialized outpatient clinic

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Veronika Brezinka ◽  
Veronika Mailänder ◽  
Susanne Walitza
Keyword(s):  
Young Children ◽  
Obsessive Compulsive Disorder ◽  
Clinical Presentation ◽  
Age Of Onset ◽  
Diagnosis And Treatment ◽  
Obsessive Compulsive ◽  
Family Accommodation ◽  
Compulsive Disorder ◽  
Very Young Children

Abstract Background Paediatric obsessive-compulsive disorder (OCD) is a chronic condition often associated with severe disruptions of family functioning, impairment of peer relationships and academic performance. Mean age of onset of juvenile OCD is 10.3 years; however, reports on young children with OCD show that the disorder can manifest itself at an earlier age. Both an earlier age of onset and a longer duration of illness have been associated with increased persistence of OCD. There seems to be difficulty for health professionals to recognize and diagnose OCD in young children appropriately, which in turn may prolong the interval between help seeking and receiving an adequate diagnosis and treatment. The objective of this study is to enhance knowledge about the clinical presentation, diagnosis and possible treatment of OCD in very young children. Case presentation We describe a prospective 6 month follow-up of five cases of OCD in very young children (between 4 and 5 years old). At the moment of first presentation, all children were so severely impaired that attendance of compulsory Kindergarten was uncertain. Parents were deeply involved in accommodating their child’s rituals. Because of the children’s young age, medication was not indicated. Therefore, a minimal CBT intervention for parents was offered, mainly focusing on reducing family accommodation. Parents were asked to bring video tapes of critical situations that were watched together. They were coached to reduce family accommodation for OCD, while enhancing praise and reward for adequate behaviors of the child. CY-BOCS scores at the beginning and after 3 months show an impressive decline in OCD severity that remained stable after 6 months. At 3 months follow-up, all children were able to attend Kindergarten daily, and at 6 months follow-up, every child was admitted to the next level / class. Conclusions Disseminating knowledge about the clinical presentation, diagnosis and treatment of early OCD may shorten the long delay between first OCD symptoms and disease-specific treatment that is reported as main predictor for persistent OCD.

scholarly journals Incidence, Clinical Features, and Outcomes of Langerhans Cell Sarcoma in the United States

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 3031-3031
Author(s):  
Anuhya Kommalapati ◽  
Sri Harsha Tella ◽  
Ronald S. Go ◽  
Gaurav Goyal
Keyword(s):  
United States ◽  
Radiation Therapy ◽  
Clinical Presentation ◽  
The United States ◽  
Langerhans Cell ◽  
Seer Database ◽  
Newly Diagnosed ◽  
Cell Sarcoma ◽  
Langerhans Cell Sarcoma

Abstract Background: Langerhans cell sarcoma (LCS) is a rare malignant neoplasm of Langerhans cells characterized by multi-organ involvement and an aggressive clinical course.Given the rarity of the disease, the true epidemiological data on LCS are lacking, and most of our knowledge is obtained from institutional case series. In this study, we utilized two national databases- the Surveillance, Epidemiology, and End Results (SEER) Program database (https://seer.cancer.gov/) and National Cancer Data Base (NCDB) to study the incidence, clinical presentation, and outcomes of LCS. Methods: We calculated LCS incidence (cases/10, 000, 000 individuals) from SEER database using SEER*Stat (v 8.3.4; https://seer.cancer.gov/seerstat/) statistical software. SEER is a population-based registry program of the United States National Cancer Institute that covers approximately 28% of the population. We identified LCS cases that were confirmed histologically using International Classification of Diseases for Oncology edition 3 (ICD-0-3) histology code 9756/3 from SEER 18 (2000-2014) registry. As the disease was mandatory reportable only after 2001, we included cases that were diagnosed after 2001 in the analysis. Demographic patterns, clinical presentation, and overall survival (OS) were calculated utilizing the patient level data from the NCDB Participant User File. NCDB is a joint program by the American College of Surgeons and American Cancer Society that represents approximately 70% of newly diagnosed cancer cases in the nation. All patients aged > 18 years with a diagnosis LCS (ICD-0-3 Code 9756/3) from 2004 to 2015 were included. To ensure accuracy of follow-up, we excluded the patients that were not treated at the same facility of diagnosis from survival analysis. Results: A total of 25 cases of LCS were reported to SEER between 2000 and 2014. The overall incidence of the disease was 0.2 per 10,000,000 [95% CI:0.1-0.3] and was similar among males and females (p=0.33). The incidence of the disease did not differ significantly among different races (whites, blacks and other races) (p=0.56). Between 2004 and 2015, 52 patients with newly diagnosed LCS were identified from NCDB and 60% were males. The median age of diagnosis was 62 (range, 19-90 years). The most common sites of the presentation were connective tissue (29%; n=15), reticuloendothelial system (RES) and hematopoietic system (25%; n=13), skin (19%, n=10), gastrointestinal (10%, n=5), bones and joints (8%, n=4), brain (2%, n=1) and breast (2%, n=1). Site specific details were missing in 27% (n=14) of cases. Out of the 52 patients, twenty patients (39%) received chemotherapy as first-line therapy, while 24 (46%) received surgery and 15 (29%) received radiation therapy. After a median follow up of 18 (range, 1-144) months, 27 patients (52%) had died. The one- year survival rate was 62%, and the median OS was 19 months (Fig. 1A). We also calculated the 1-year disease-specific survival and OS using SEER database, and was 74% and 63%, respectively. After censoring the patients with hematopoietic and RES involvement, the patients who were managed with radiation therapy (median dose 3960 cGy, range 750-6400) had a better OS as compared to those who received no radiation therapy (p=0.03, Fig. 1B). On the contrary, after excluding the patients with hematopoietic and RES as primary site, no significant difference in OS was noted between the patients who were managed with and without surgery (17 vs 21 months; p = 0.75; Fig. 1C). In addition, post-surgical radiation or chemotherapy had no significant benefit in median OS (p=0.25). Among patients with hematopoietic and RES involvement, there was no association between OS and receipt of chemotherapy (p = 0.16; Fig. 1D); however, the number of patients who received chemotherapy was small (n=6). Conclusion: This dual-national registry study shows that LCS is extremely rare, and has a poor prognosis. Although the data regarding optimal management of LCS are limited, our study demonstrates that radiation therapy may offer survival advantage in patients without hematopoietic and RES involvement. Disclosures No relevant conflicts of interest to declare.

Surgical treatment of thoracic Pott disease in a 3-year-old child, with vertebral column resection and posterior-only circumferential reconstruction of the spinal column

2012 ◽  
Vol 9 (4) ◽  
pp. 447-451 ◽  
Author(s):  
Leonardo Rangel-Castilla ◽  
Steven W. Hwang ◽  
William E. Whitehead ◽  
Daniel J. Curry ◽  
Thomas G. Luerssen ◽  
...  
Keyword(s):  
Young Children ◽  
Vertebral Column ◽  
Spinal Column ◽  
Technical Difficulty ◽  
Bed Rest ◽  
Cord Compression ◽  
Common Disease ◽  
Vertebral Column Resection ◽  
Safe Alternative ◽  
Very Young Children

Tuberculosis (TB) is a common disease worldwide that is caused by Mycobacterium tuberculosis. Tuberculosis of the spine, also called Pott disease, is the most common site of bony dissemination. Although children are disproportionately affected, spinal TB is nonetheless rare in very young children. Cases involving infants requiring surgical intervention have been previously reported, and they are often associated with greater management challenges given the technical difficulty with instrumentation in very young children. This case involved a 3-year-old girl with TB centered at T-6, who presented with myelopathy from spinal cord compression and a severe kyphotic deformity (> 60°). She underwent a single-stage costotransversectomy for vertebral column resection, followed by reconstruction with an anterior expandable titanium cage and posterior pedicle screw instrumentation. At last follow-up, the patient was clinically and radiographically stable. The authors report on the youngest patient with spinal TB treated surgically with this strategy and review the literature regarding prior cases involving young children. Although limited by the paucity of cases in the literature, surgical debridement and spinal fusion appear to provide a safe alternative to prolonged bed rest or casting and may offer additional benefits of a faster recovery and ambulation.

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