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1.
Int J Surg Case Rep ; 120: 109885, 2024 Jun 06.
Article in English | MEDLINE | ID: mdl-38851061

ABSTRACT

INTRODUCTION: Radial head dislocations in children can result from congenital anomalies, traumatic events, or as part of more complex injuries like Monteggia fractures. These dislocations are rare and may be overlooked unless considered in differential diagnoses. CASE PRESENTATION: We describe a unique instance of a post-traumatic isolated lateral radial head dislocation in a 5-year-old boy with no previous medical concerns. The child presented with persistent pain and limited mobility in the left elbow following an unwitnessed fall at home. Diagnosis was confirmed via radiographs showing the dislocation without associated fractures. Closed reduction under anesthesia was successfully performed. CLINICAL DISCUSSION: This case underscores the importance of a high index of suspicion and thorough imaging in diagnosing pediatric radial head dislocations. Our discussion includes a review of the literature and the clinical approach for managing such injuries, highlighting the specifics of the closed reduction technique used. CONCLUSION: Isolated lateral radial head dislocations, although rare, can occur and are amenable to closed reduction. This case contributes to the limited reports of such dislocations in pediatric patients, emphasizing the need for awareness and precise management strategies to prevent long-term complications.

2.
J Int Med Res ; 50(3): 3000605221082852, 2022 Mar.
Article in English | MEDLINE | ID: mdl-35264045

ABSTRACT

OBJECTIVES: Most epidemiologic studies on soft tissue sarcomas (STS) and bone sarcomas (BS) are performed in western countries, with few in the Middle East and North Africa region. We describe the epidemiology of sarcomas in Lebanon using the medical records database at the American University of Beirut Medical Center (AUBMC). METHODS: This single-center retrospective cohort study included patients with sarcomas registered in the database between 2015 and 2019. Their charts were reviewed for baseline characteristics, tumor biology and location, treatment modalities, recurrence, metastasis, and death. RESULTS: The cohort included 234 patients with STS and 99 patients with BS. Most tumors were <10 cm in size. The most common subtypes were liposarcoma for STS and osteosarcoma for BS. The most common location of STS was the thigh. The most frequent sites of STS metastasis were the lungs. Histological subtype, smoking status, and tumor size and grade were significant for progression-free survival (PFS) in patients with STS. By multivariable analysis, smoking was significantly associated with poorer PFS in STS. For BS, only tumor grade was significant for PFS. CONCLUSION: The epidemiology of sarcomas at AUBMC is similar to that previously reported. Smoking history was associated with poorer survival in patients with STS.


Subject(s)
Bone Neoplasms , Osteosarcoma , Sarcoma , Bone Neoplasms/epidemiology , Humans , Lebanon/epidemiology , Osteosarcoma/epidemiology , Retrospective Studies , Sarcoma/epidemiology , Sarcoma/pathology
3.
J Pediatr Orthop B ; 31(2): e258-e263, 2022 Mar 01.
Article in English | MEDLINE | ID: mdl-34101677

ABSTRACT

Expandable endoprostheses provide a limb salvage option for skeletally immature patients with bone sarcoma of the lower extremities. Initial reports of the Repiphysis prosthesis were encouraging; however, medium-term follow-up revealed high complication rates. We report on the long-term follow-up of a cohort of patients treated with the Repiphysis prosthesis. Eleven patients were included in the study. Data collected included sex, age at surgery, duration of follow-up, site of disease, histologic diagnosis, number of lengthening sessions, amount lengthened, postoperative complications, endoprosthetic failure, mode of endoprosthetic failure, duration from index surgery to failure and to revision, type of revision surgery and final limb-length discrepancy. The average duration of follow-up from the time of surgery was 180 months (range, 144-215 months). Fifteen Repiphysis implants were used in 11 patients. All implants failed with an average time from surgery to failure of 36 months (range, 3-72 months). Twenty-four complications were observed: one wound dehiscence, two deep infections, 18 mechanical failures, implant collapse with destruction of proximal tibia epiphysis in two and one periprosthetic proximal femur fracture with dislodgement of the stem. Despite being an option for limb salvage, the Repiphysis prosthesis has a high rate of mechanical failure and need for revision, similar to other expandable implants. The authors, therefore, recommend full disclosure of the potential short- and long-term complications and need for revision, as well as alternative treatment options if their use is considered. Level of evidence: IV (Therapeutic).


Subject(s)
Bone Neoplasms , Femoral Neoplasms , Sarcoma , Bone Neoplasms/diagnostic imaging , Bone Neoplasms/surgery , Child , Femoral Neoplasms/surgery , Follow-Up Studies , Humans , Limb Salvage , Lower Extremity , Prosthesis Design , Prosthesis Failure , Prosthesis Implantation , Retrospective Studies , Sarcoma/diagnostic imaging , Sarcoma/surgery , Treatment Outcome
4.
Clin Med Res ; 19(3): 132-137, 2021 09.
Article in English | MEDLINE | ID: mdl-34531270

ABSTRACT

Sever's disease is an underreported prevalent pediatric condition that causes heel pain in children worldwide. It is often described as an overuse injury that can present with either unilateral or bilateral heel pain. Even though the exact mechanism of injury is unknown, it is often thought it involves repetitive stress and pressure on the calcaneal growth plate. Diagnosing Sever's disease mainly relies on a thorough clinical investigation and physical examination, with a positive squeeze test usually sufficient to establish diagnosis. Nevertheless, radiographic imaging can help exclude other differential diagnoses. Therapeutic options of Sever's disease are mostly conservative, and these include rest, physical therapy, kinesiotherapy, and orthoses. Educating parents and coaches on the symptomatology and presentation of Sever's disease is pivotal for the establishment of efficient preventive interventions and earlier diagnoses. This study presents a case of a pediatric patient with Sever's disease and offers medical insight into the diagnostic, clinical, pathologic, and therapeutic characteristics of this condition, in light of the current existing literature.


Subject(s)
Calcaneus , Child , Foot , Humans , Pain , Physical Examination
5.
Oncol Lett ; 21(5): 354, 2021 May.
Article in English | MEDLINE | ID: mdl-33747211

ABSTRACT

Extraskeletal Ewing sarcoma (EES) is a relatively uncommon primary tumor of the soft tissues, which accounts for 20-30% of all reported cases of ES. Being uncommon, all members of the ES family tumors are treated following the same general protocol of sarcoma tumors. The present review summarizes the diagnosis, management and prognosis of EES, focusing on the differences between the subtypes of ESS. The clinical features and imaging of EES are also discussed. Magnetic resonance imaging is the modality of choice for diagnostic imaging and local staging, while core-needle biopsy with pathological testing is used to obtain a definitive diagnosis. Although several oncology groups endorse that ES family of tumors should be treated with similar algorithm and protocols, some studies have demonstrated that surgery and radiotherapy may be used as a form of local control. However, further studies are required to conclude the optimum treatment option for EES.

6.
J Foot Ankle Surg ; 60(1): 163-166, 2021.
Article in English | MEDLINE | ID: mdl-33162292

ABSTRACT

Giant cell tumor of the tendon sheath is a slowly growing benign tumor. It usually arises from the tendon sheath and periarticular soft tissue of small joints. However, it may infrequently involve the large joints emerging around the knee, elbow, and hip joints. Giant cell tumor of the tibialis tendon sheath is rarely reported in the foot and ankle joint. Here, we report the first case in the medical literature of bilateral mirror-symmetrical giant cell tumor of the tendon sheath in the foot and ankle. A 12-year-old male presented with a bilateral and mirror-image mass on his ankles extending to the foot. It was painless but affected his gait and footwear. Staged complete resection was done first on the right then on the left side, with no recurrence after 1 year. The role of genes can be argued for this presentation and giant cell tumor's etiology, owing to the bilateralism and mirror-image presentation. Studies are needed to explore this genetic aspect and its role in management.


Subject(s)
Ankle , Giant Cell Tumors , Ankle Joint/diagnostic imaging , Ankle Joint/surgery , Child , Giant Cell Tumors/diagnostic imaging , Giant Cell Tumors/surgery , Humans , Male , Neoplasm Recurrence, Local , Tendons/diagnostic imaging , Tendons/surgery
7.
Pediatr Blood Cancer ; 68(1): e28784, 2021 01.
Article in English | MEDLINE | ID: mdl-33128443

ABSTRACT

BACKGROUND: Patients with sickle cell disease are at higher risk of infections with encapsulated bacteria due to immature immune responses and functional asplenia. We aimed to study our patient population for the emergence of gram-negative organisms other than Salmonella as the cause of osteomyelitis and document a vast decrease in Streptococcus pneumoniae bacteremia rates. METHODS: We conducted a retrospective chart review of 158 patients with sickle cell disease registered at our hospital. Over a period of 13 years, every patient presenting to the emergency department (ED) with fever had their medical record reviewed for blood cultures, wound cultures, and magnetic resonance imaging results for osteomyelitis. RESULTS: The number of patients presenting to the ED with fever was 105, with 581 febrile episodes and 893 blood cultures. Among those, no culture grew Streptococcus pneumoniae, 14 grew coagulase-negative staphylococci (1.5%), one grew Salmonella enterica Paratyphi B, and three grew Salmonella enterica group C (in the same patient). The total number of osteomyelitis episodes in patients with sickle cell disease presenting with fever and documented by imaging was nine (1.5%). In patients with osteomyelitis, organisms were isolated in four patients (44%), including Enterobacter cloacae, Bacteroides, Pseudomonas aeruginosa, and Salmonella enterica group C. CONCLUSIONS: Immunization against Streptococcus pneumoniae and the use of prophylactic penicillin has virtually eliminated pneumococcal bacteremia among our patients. We observed the emergence of gram-negative organisms other than Salmonella as the cause of osteomyelitis in patients with sickle cell disease.


Subject(s)
Anemia, Sickle Cell/epidemiology , Gram-Negative Bacteria/pathogenicity , Gram-Negative Bacterial Infections/complications , Hospitalization/statistics & numerical data , Osteomyelitis/epidemiology , Adolescent , Anemia, Sickle Cell/pathology , Anemia, Sickle Cell/virology , Child , Child, Preschool , Female , Follow-Up Studies , Gram-Negative Bacterial Infections/virology , Humans , Incidence , Infant , Infant, Newborn , Lebanon/epidemiology , Male , Osteomyelitis/pathology , Osteomyelitis/virology , Prognosis , Retrospective Studies
8.
J Pediatr Orthop B ; 29(5): 510-513, 2020 Sep.
Article in English | MEDLINE | ID: mdl-31356507

ABSTRACT

The surgical treatment of bone sarcoma involves wide surgical resection and endoprosthetic replacement. Surgical resection for proximal tibia bone sarcoma includes the patellar tendon. The secondary extension lag is a common complication and, although many have been described, the options for reconstruction of the patellar tendon are suboptimal. We propose adding a biological reinforcement, the hamstring tendons, to our extensor mechanism reconstruction technique (polyprolene mesh and gastrocnemius flap). We describe the surgical technique and, using an illustrative case, compare the outcome of this technique with the outcome following reconstruction without reinforcement. Level of evidence: Level III.


Subject(s)
Bone Neoplasms/surgery , Hamstring Tendons/transplantation , Osteosarcoma/surgery , Patella/surgery , Surgical Mesh , Tibia , Bone Neoplasms/diagnostic imaging , Follow-Up Studies , Humans , Osteosarcoma/diagnostic imaging , Surgical Flaps , Transplantation, Autologous , Treatment Outcome
9.
J Glob Oncol ; 5: 1-8, 2019 04.
Article in English | MEDLINE | ID: mdl-30946633

ABSTRACT

PURPOSE: Outcomes in pediatric osteosarcoma have dramatically improved over the past few decades, with overall survival rates of 70% and 30% for patients with localized and metastatic disease, respectively. PATIENTS AND METHODS: We retrospectively reviewed clinical characteristics and outcomes of 38 patients treated between 2001 and 2012 at a single institution in Lebanon. All patients received a uniform three-drug chemotherapy regimen consisting of cisplatin, doxorubicin, and methotrexate. Ifosfamide and etoposide were added to the adjuvant treatment regimen in case of metastatic disease and/or poor degree of tumor necrosis (< 90%). RESULTS: After a median follow-up of 61 months (range, 8 to 142 months), patients with localized disease had 5-year overall and event-free survival rates of approximately 81% and 68%, respectively, whereas for metastatic disease, they were approximately 42%. The most common primary site was the long bones around the knee (n = 34; 89.5%). Six patients (15.8%) had metastatic disease to lungs, and three (7.9%) had synchronous multifocal bone disease with lung metastases. Adverse prognostic factors included nonlower extremity sites, metastasis, poor degree of necrosis, and delay of more than 4 weeks in local control. In bivariable analysis, only degree of necrosis was a prognostic predictor for survival and disease recurrence. CONCLUSION: Treatment of pediatric osteosarcoma in a multidisciplinary cancer center in Lebanon resulted in survival similar to that in developed countries. Delay in local control was associated with worse outcome. The only statistically significant inferior outcome predictor was poor degree of necrosis at the time of local control.


Subject(s)
Bone Neoplasms/therapy , Cisplatin/therapeutic use , Doxorubicin/therapeutic use , Etoposide/therapeutic use , Methotrexate/therapeutic use , Osteosarcoma/therapy , Adolescent , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Bone Neoplasms/mortality , Child , Child, Preschool , Disease-Free Survival , Female , Humans , Infant , Lebanon , Male , Neoadjuvant Therapy , Neoplasm Metastasis , Osteosarcoma/mortality , Prognosis , Retrospective Studies , Risk Assessment , Treatment Outcome
10.
J Glob Oncol ; 3(1): 23-30, 2017 Feb.
Article in English | MEDLINE | ID: mdl-28717738

ABSTRACT

BACKGROUND: Children with malignant bone tumors have average 5-year survival rates of 60% to 70% with current multimodality therapy. Local control modalities aimed at preserving function greatly influence the quality of life of long-term survivors. In developing countries, the limited availability of multidisciplinary care and limited expertise in specialized surgery and pediatric radiation therapy, as well as financial cost, all form barriers to achieving optimal outcomes in this population. METHODS: We describe the establishment of a collaborative pediatric bone tumor program among a group of pediatric oncologists in Lebanon and Syria. This program provides access to specialized local control at a tertiary children's cancer center to pediatric patients with newly diagnosed bone tumors at participating sites. Central review of pathology, staging, and treatment planning is performed in a multidisciplinary tumor board setting. Patients receive chemotherapy at their respective centers on a unified treatment plan. Surgery and/or radiation therapy are performed centrally by specialized staff at the children's cancer center. Cost barriers were resolved through a program development initiative led by St Jude Children's Research Hospital. Once program feasibility was achieved, the Children's Cancer Center of Lebanon Foundation, via fundraising efforts, provided continuation of program-directed funding. RESULTS: Findings over a 3-year period showed the feasibility of this project, with timely local control and protocol adherence at eight collaborating centers. We report success in providing standard-of-care multidisciplinary therapy to this patient population with complex needs and financially challenging surgical procedures. CONCLUSION: This initiative can serve as a model, noting that facilitating access to specialized multidisciplinary care, resolution of financial barriers, and close administrative coordination all greatly contributed to the success of the program.

11.
Front Genet ; 8: 217, 2017.
Article in English | MEDLINE | ID: mdl-29326753

ABSTRACT

Despite an obvious role for consanguinity in congenital heart disease (CHD), most studies fail to document a monogenic model of inheritance except for few cases. We hereby describe a first-degree cousins consanguineous Lebanese family with 7 conceived children: 2 died in utero of unknown causes, 3 have CHD, and 4 have polydactyly. The aim of the study is to unveil the genetic variant(s) causing these phenotypes using next generation sequencing (NGS) technology. Targeted exome sequencing identified a heterozygous duplication in CSRP1 which leads to a potential frameshift mutation at position 154 of the protein. This mutation is inherited from the father, and segregates only with the CHD phenotype. The in vitro characterization demonstrates that the mutation dramatically abrogates its transcriptional activity over cardiac promoters like NPPA. In addition, it differentially inhibits the physical association of CSRP1 with SRF, GATA4, and with the newly described partner herein TBX5. Whole exome sequencing failed to show any potential variant linked to polydactyly, but revealed a novel missense mutation in TRPS1. This mutation is inherited from the healthy mother, and segregating only with the cardiac phenotype. Both TRPS1 and CSRP1 physically interact, and the mutations in each abrogate their partnership. Our findings add fundamental knowledge into the molecular basis of CHD, and propose the di-genic model of inheritance as responsible for such malformations.

12.
Spine (Phila Pa 1976) ; 40(17): E971-7, 2015 Sep 01.
Article in English | MEDLINE | ID: mdl-25929208

ABSTRACT

STUDY DESIGN: Cross-sectional validation and reliability assessment study of Arabic version of Scoliosis Research Society-22 (SRS-22r) Questionnaire. OBJECTIVE: To develop and validate the Arabic version of the SRS-22r questionnaire. SUMMARY OF BACKGROUND DATA: The diagnosis and treatment of adolescent idiopathic scoliosis may influence patient quality of life. SRS-22r is an internationally validated questionnaire used to assess function/activity, pain, self-image, and mental health of patients with scoliosis. It has been translated into several languages but not into Arabic language. Therefore, a valid health-related quality-of-life outcome questionnaire for patients with spinal deformity is still lacking in Arabic language. METHODS: The English version of SRS-22r questionnaire was translated, back-translated, and culturally adapted to Arabic language. Then, 81 patients with idiopathic adolescent scoliosis were allocated randomly into either the reliability testing group (group 1) or the validity testing group (group 2). Group 1 patients completed Arabic version of SRS-22r questionnaire twice with 1-week interval in-between. Cronbach α and intraclass correlation coefficient were measured to determine internal consistency and temporal reliability. Group 2 patients completed the Arabic version of SRS-22r questionnaire and the previously validated Arabic version of 36-Item Short Form Health Survey (Short Form-36) questionnaire concurrently, and Pearson correlation coefficient was obtained to assess validity. RESULTS: Content analysis, internal consistency reliability, test/retest reproducibility (intraclass correlation coefficient range: 0.82-0.90), and test of concurrent validity showed satisfactory results. Function/activity and satisfaction with management domains had a lower Cronbach α (0.58 and 0.44, respectively, vs. 0.71-0.85 range for others). Self-image/appearance and satisfaction with management had a lower correlation with domains of the 36-Item Short Form Health Survey. CONCLUSION: An Arabic version of the SRS-22r questionnaire has been developed and validated. This questionnaire will aid health care workers and researchers in evaluation of patient perception of the deformity, satisfaction with treatment, and quality of life in Arabic-speaking populations. LEVEL OF EVIDENCE: 3.


Subject(s)
Mental Health , Quality of Life , Scoliosis/surgery , Adolescent , Adult , Cross-Sectional Studies , Female , Humans , Language , Male , Psychometrics/methods , Reproducibility of Results , Scoliosis/diagnosis , Self Concept , Societies, Medical , Surveys and Questionnaires
13.
J Med Liban ; 62(3): 180-2, 2014.
Article in English | MEDLINE | ID: mdl-25306800

ABSTRACT

Nontuberculous mycobacteria are rare causes of skin, soft tissue, and musculoskeletal infections. Mycobacterium marinum remains one of the most commonly encountered mycobacterial species in humans, causing superficial cutaneous as well as deep infections. We are reporting a case of M. marinum osteomyelitis involving two primary noncontiguous sites in an immunocompetent host, which was successfully treated with surgical drainage and antibiotic therapy.


Subject(s)
Immunocompetence , Metacarpal Bones/microbiology , Metatarsophalangeal Joint/microbiology , Mycobacterium Infections, Nontuberculous/diagnosis , Osteomyelitis/microbiology , Humans , Magnetic Resonance Imaging , Male , Metacarpal Bones/pathology , Metatarsophalangeal Joint/pathology , Middle Aged , Mycobacterium marinum , Osteomyelitis/drug therapy
14.
J Pediatr Orthop B ; 23(4): 354-7, 2014 Jul.
Article in English | MEDLINE | ID: mdl-24406811

ABSTRACT

Discoid meniscus of the knee is a well-known anatomic and congenital variant of the lateral meniscus. It is often asymptomatic, but can be associated with knee pain and other symptoms. Posterior dislocation of the discoid meniscus is an extremely rare finding; to the best of our knowledge, only one case has been reported in the literature. Here, we report a case of 10-year-old girl who presented with right knee pain with no history of trauma. The radiograph of her right knee was negative but MRI showed bilateral lateral discoid menisci with posterior dislocation in the right knee and some subluxation in the left. She underwent right knee arthroscopic lateral meniscoplasty with excellent outcome. This case demonstrates the possibility of torn and dislocated discoid lateral menisci management through arthroscopic reduction and repair.


Subject(s)
Knee Dislocation/etiology , Menisci, Tibial/abnormalities , Child , Female , Humans , Knee Dislocation/diagnosis , Knee Dislocation/surgery
15.
Pediatr Blood Cancer ; 61(8): 1472-5, 2014 Aug.
Article in English | MEDLINE | ID: mdl-24395458

ABSTRACT

Treatment of Ewing sarcoma (ES) necessitates coordinated multi-disciplinary care. We analyzed outcome for 39 patients treated at a single institution in Lebanon, a developing country with available multidisciplinary treatment modalities, where financial barriers to care are overcome by a fundraising system. Median follow-up was 58 months. Five-year overall and event-free survival were 76% and 58%, respectively, for localized disease, and 40% and 38%, respectively, for metastatic disease. We conclude that, in a country with emerging economy, by following international protocols and ensuring availability of needed resources, outcome of patients with ES is similar to that in developed countries.


Subject(s)
Sarcoma, Ewing/mortality , Sarcoma, Ewing/therapy , Adolescent , Child , Child, Preschool , Disease-Free Survival , Female , Follow-Up Studies , Humans , Lebanon/epidemiology , Male , Retrospective Studies , Survival Rate
17.
Musculoskelet Surg ; 97(1): 85-7, 2013 Apr.
Article in English | MEDLINE | ID: mdl-22441672

ABSTRACT

Propionibacterium acnes detection in culture media was previously considered a contamination but recently its infectious role was discovered in post-spinal surgery infections. P. acnes might be introduced during surgery. Its diagnosis is based on non-specific clinical signs, image indications of infection, and the conclusive microbiological sign. Furthermore, its diagnosis is difficult because of slow growth rate and low virulence, delaying its presentation. Usually, the infection is manifested after a couple of months or years. Here, a 65-year-old man presented with drainage at the site of instrumented spinal surgery performed 13 years ago. P. acnes infection was confirmed by culture with extended incubation. Our review of the literature revealed only two other reported cases of delayed P. acnes infection presenting a decade following a spinal surgery with instrumentation. This article sheds light on such delayed infections and discusses their presentation and management.


Subject(s)
Fistula/microbiology , Gram-Positive Bacterial Infections/complications , Neurosurgical Procedures/adverse effects , Propionibacterium acnes/isolation & purification , Spinal Fractures/surgery , Surgical Wound Infection/microbiology , Aged , Anti-Bacterial Agents/therapeutic use , Ciprofloxacin/therapeutic use , Delayed Diagnosis , Disease Progression , Fistula/diagnosis , Fistula/therapy , Follow-Up Studies , Gram-Positive Bacterial Infections/diagnosis , Gram-Positive Bacterial Infections/microbiology , Gram-Positive Bacterial Infections/therapy , Humans , Male , Neurosurgical Procedures/methods , Surgical Wound Infection/diagnosis , Surgical Wound Infection/therapy , Thoracic Vertebrae/surgery , Treatment Outcome
18.
Endocr Pract ; 18(6): e135-7, 2012.
Article in English | MEDLINE | ID: mdl-22784836

ABSTRACT

OBJECTIVE: To report a case of slipped capital femoral epiphysis in a young patient with Turner syndrome (TS) receiving growth hormone therapy and to emphasize the importance of keeping this orthopedic condition in mind during management of this patient group. METHODS: Clinical, laboratory, and radiographic findings are presented, and risk factors for slipped capital femoral epiphysis are discussed. RESULTS: A child with TS presented for medical assessment because of a limp but with no history of trauma or febrile illness. Growth hormone therapy had been administered for 1 year because of her short stature. Physical examination and pelvic radiography of the patient showed the presence of bilateral slipped capital femoral epiphysis. She underwent bilateral pinning in situ, and growth hormone therapy was terminated. At follow-up after more than 2 years, no sequelae were noted. CONCLUSION: Patients with TS are at high risk for developing certain orthopedic conditions, such as slipped capital femoral epiphysis. Furthermore, slipped capital femoral epiphysis is a known complication of growth hormone therapy in growing children. A limp, hip pain, knee pain, or thigh pain might be a symptom of slipped capital femoral epiphysis in patients with TS, especially those receiving growth hormone therapy. Prompt recognition and treatment of this condition are important for prevention of sequelae.


Subject(s)
Growth Hormone/therapeutic use , Slipped Capital Femoral Epiphyses/diagnostic imaging , Turner Syndrome/drug therapy , Child , Female , Growth Hormone/adverse effects , Humans , Radiography , Risk Factors , Slipped Capital Femoral Epiphyses/epidemiology , Slipped Capital Femoral Epiphyses/etiology , Withholding Treatment
19.
Skeletal Radiol ; 41(11): 1453-61, 2012 Nov.
Article in English | MEDLINE | ID: mdl-22406919

ABSTRACT

OBJECTIVE: The aim of this work was to determine the role of MRI in interpreting abnormal signals within bones and soft tissues adjacent to tumor bulk of osteosarcoma and Ewing's sarcoma in a pediatric population by correlating MR findings with histopathology. MATERIALS AND METHODS: Thirty patients met the inclusion criteria, which included (1) osteosarcoma or Ewing's sarcoma, (2) MR studies no more than 2 months prior to surgery, (3) presence of abnormal MR signal surrounding the tumor bulk, (4) pathological material from resected tumor. The patients received standard neoadjuvant chemotherapy. Using grid maps on gross pathology specimens, the abnormal MR areas around the tumor were matched with the corresponding grid sections. Histopathology slides of these sections were then analyzed to determine the nature of the regions of interest. The MR/pathological correlation was evaluated using Mann-Whitney U test and Fisher's exact test. RESULTS: Twenty-seven patients had osteosarcoma and three patients had Ewing's sarcoma. Of the studied areas, 17.4% were positive for tumor (viable or necrotic). There was no statistically significant correlation between areas positive for tumor and age, gender, signal extent and intensity on MRI, or tissue type. There was, however, a statistically significant correlation between presence of tumor and the appearance of abnormal soft tissue signals. A feathery appearance correlated with tumor-negative areas whereas a bulky appearance correlated with tumor-positive regions. CONCLUSIONS: MR imaging is helpful in identifying the nature of abnormal signal areas surrounding bone sarcomas that are more likely to be tumor-free, particularly when the signal in the soft tissues surrounding the tumor is feathery and edema-like in appearance.


Subject(s)
Bone Neoplasms/pathology , Magnetic Resonance Imaging/methods , Osteosarcoma/pathology , Sarcoma, Ewing/pathology , Adolescent , Bone Neoplasms/therapy , Child , Female , Humans , Male , Neoadjuvant Therapy , Osteosarcoma/therapy , Retrospective Studies , Sarcoma, Ewing/therapy , Statistics, Nonparametric , Young Adult
20.
Spine (Phila Pa 1976) ; 37(4): 334-9, 2012 Feb 15.
Article in English | MEDLINE | ID: mdl-21494197

ABSTRACT

STUDY DESIGN: Literature review. OBJECTIVE: To present a comprehensive overview of spinal involvement in patients with ß-thalassemia, aiming to orient the spinal surgeon to these potentially disabling complications. SUMMARY OF BACKGROUND DATA: ß-thalassemia, an inherited disorder of hemoglobin synthesis, is the most common monogenetic disease worldwide. Patients with thalassemia major (TM) suffer a severe anemia that requires regular blood transfusions for survival and iron chelation therapy to control transfusional iron overload. Thalassemia intermedia is a phenotype of thalassemia with a milder course and anemia, wherein most patients remain transfusion independent. Spinal involvement related to disease course and treatment is common in patients with thalassemia syndromes, yet it has not been constructively reviewed in the literature. METHODS: Potentially relevant studies were identified from an electronic search of MEDLINE (1966 to the second week of May 2010). RESULTS: In patients with TM, genetic and acquired risk factors lead to osteoporosis, pathologic fractures of the spine, and back pain. Osteoporosis in TM patients is progressive; thus, early diagnosis and treatment are recommended. Bisphosphonates are relatively safe and effective in this patient population. Characteristic intervertebral disc degeneration is also seen in patients with TM who have evidence of severe iron overload or those who receive the subcutaneous iron chelator deferoxamine. Spinal asymmetry and overt scoliosis are common in patients with TM. The prognosis seems favorable, with many patients showing spontaneous resolution without the need for intervention. In patients with thalassemia intermedia, ineffective erythropoiesis drives extramedullary hematopoietic tissue formation, which is mostly evident on magnetic resonance imaging. Paraspinal involvement is of greatest concern because of the associated spinal cord compression. Several treatment options have been described, including transfusion therapy, laminectomy, radiotherapy, and the use of fetal hemoglobin-inducing agents that decrease the hematopoietic drive. CONCLUSION: Current knowledge supports that spinal involvement in patients with ß-thalassemia is common and diverse yet still requires further prospective evaluation.


Subject(s)
Back Pain/pathology , Spinal Diseases/pathology , Spine/pathology , beta-Thalassemia/pathology , Back Pain/epidemiology , Back Pain/genetics , Bone Density Conservation Agents/therapeutic use , Bone Remodeling/physiology , Comorbidity , Diphosphonates/therapeutic use , Humans , Intervertebral Disc Degeneration/epidemiology , Intervertebral Disc Degeneration/genetics , Intervertebral Disc Degeneration/pathology , Osteoporosis/drug therapy , Osteoporosis/epidemiology , Osteoporosis/genetics , Osteoporosis/pathology , Risk Factors , Spinal Diseases/epidemiology , Spinal Diseases/genetics , beta-Thalassemia/epidemiology , beta-Thalassemia/genetics
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