Rosai-Dorfman: Rare Manifestations of a Rare Disease.

Rosai-Dorfman disease (RDD) is an exceedingly rare non-Langerhans cell histiocytosis of uncertain etiology that most commonly presents in children as self-limited, painless, massive cervical lymphadenopathy. However, extranodal disease occurs in 43% of cases and has a wide range of phenotypic presentations. The pathogenesis has not been clearly understood in the literature and coupled with a wide range of clinical manifestations, early diagnosis and initiation of an appropriate treatment modality have been challenging. Herein, we describe a cohort of five cases that occurred at the same institution within a 12-month period. These cases highlight unique and atypical presentations of an already rare disease, outline the varying and tailored diagnostic and therapeutic approaches, and propose a novel environmental predisposing factor given the exceptionally high incidence at our institution over a short period of time. We emphasize the need for further investigation of predisposing factors and to discern targeted therapies that may offer benefits.

Metastatic Endometrioid Carcinoma Mimicking a Subungual Melanoma.

CASE: We report a case of a 76-year-old female with a stage IB, grade I endometrioid endometrial carcinoma who presented with right-hip pain and an enlarging black, exophytic, subungual lesion on her right-small-finger distal phalanx. Clinically, the distal phalanx lesion was suspicious for a subungual melanoma; however, advanced imaging suggested metastatic disease, with lesions in the acetabulum, lungs, brain, vulva, and vagina. CONCLUSION: Partial amputation of the right, small finger and vulvar biopsies confirmed an endometrial carcinoma. To our knowledge, this is the first described case of endometrial adenocarcinoma metastasis to the phalanx of an upper extremity, mimicking a subungual melanoma.

Vitamin D Deficiency in the Military: It's Time to Act!

Vitamin D is critically important to numerous physiologic functions, including bone health. Poor vitamin D status is a common but underrecognized problem that predisposes the military population to stress fracture and completed fracture. This has significant implications for force health protection, warfighter readiness, attrition, and cost. Despite this, vitamin D deficiency is still underdiagnosed and undertreated in the military. This is a major hindrance to military readiness and one that could easily be modified with awareness, prevention, and early treatment. In this commentary, we review the literature on vitamin D deficiency and critically examine the current status of policies and clinical practice related to vitamin D in the military health system. We offer several practical recommendations to increase awareness and readiness while decreasing musculoskeletal injury and the associated costs.

Tumor-induced hypophosphatemic osteomalacia caused by a mesenchymal tumor of the mandible managed by a segmental mandibulectomy and microvascular reconstruction with a free fibula flap.

BACKGROUND: Tumor-induced osteomalacia is a rare paraneoplastic syndrome in which patients develop hypophosphatemia and osteomalacia. METHODS AND RESULTS: Here, we report a unique case of a 42-year-old man who presented to our institution with a 1-year history of pain in his ribs, hips, lower back, and feet. Radiologic examination revealed a decrease in bone density and multiple insufficiency fractures. Laboratory evaluation revealed hypophosphatemia, low serum 1,25 dihydroxy vitamin D3 , and elevated fibroblast growth factor 23 (FGF23). A positron emission tomography/CT scan showed increased uptake in the right mandibular third molar region. Panoramic radiography and CT scanning showed a lytic expansile bone lesion. A mandibular bone biopsy revealed a mixed connective tissue tumor. A right segmental mandibulectomy was performed, followed by microvascular reconstruction. The resection was confirmed by normalization of serum phosphate and FGF23. CONCLUSION: Successful management of this condition was achieved, with complete surgical resection of the tumor and reconstructive surgery.

Recurrent Shoulder Instability in a Young, Active, Military Population and Its Professional Implications.

BACKGROUND: Shoulder instability is a topic of significant interest within the sports medicine literature, particularly regarding recurrence rates and the ideal treatment indications and techniques. Little has been published specifically addressing the occupational implications of symptomatic recurrent shoulder instability. HYPOTHESIS: Previous arthroscopic repair will continue to be a significant predisposing factor for recurrent instability in a young, active population, and that recurrent instability may have a negative effect on college graduation and postgraduate occupational selection. STUDY DESIGN: Case series. LEVEL OF EVIDENCE: Level 4. METHODS: We conducted a retrospective review of approved medical waivers for surgical treatment of anterior shoulder dislocation or instability prior to matriculation at the US Military Academy or the US Naval Academy for the graduating classes of 2010 to 2013. Statistical analysis was performed to determine the incidence and risk factors for recurrence and to determine the impact on graduation rate and occupation selection. RESULTS: Fifty-nine patients were evaluated; 34% developed recurrent anterior instability. Patients with previous arthroscopic repair had a significantly higher incidence of recurrence (38%, P = 0.044). Recurrent shoulder instability did not significantly affect graduation rates or self-selected occupation ( P ≥ 0.05). CONCLUSION: There is a significant rate of recurrent shoulder instability after primary surgical repair, particularly among young, active individuals. In addition, arthroscopic repair resulted in a significantly higher recurrence rate compared with open repair in our population. Surgical repair for shoulder instability should not necessarily preclude young individuals from pursuing (or being considered for) occupations that may place them at greater risk of recurrence. CLINICAL RELEVANCE: The risk of recurrent instability is greater than the rate typically described, which may suggest that some subpopulations are at greater risk than others. A unique data point regarding instability is the effect on occupation selection.

Defining the terms acute and chronic in orthopaedic sports injuries: a systematic review.

BACKGROUND: Although many authors report on acute injuries and chronic injuries in the orthopaedic literature, the actual terms are seldom explicitly defined. HYPOTHESIS: Much of the literature pertaining to sports injuries that are acute or chronic does not define these terms. It is believed that definitions will provide clarity and specificity in future literature. STUDY DESIGN: Systematic review. METHODS: A systematic review of 116 articles was conducted to determine whether and how the terms acute and chronic were defined as they pertain to several commonly treated conditions: Achilles tendon rupture, distal biceps tendon rupture, pectoralis major tendon rupture, anterior cruciate ligament (ACL) tear, anterior shoulder instability, and acromioclavicular (AC) joint dislocation. Articles were isolated from various databases and search engines by use of keywords to identify relevant literature. RESULTS: This study determined that the terms acute and chronic for each injury are defined, respectively, as follows: Achilles tendon rupture: <1 week, >4 weeks; distal biceps tendon rupture: <6 weeks, >12 weeks; pectoralis major tendon rupture: <6 weeks, >6 weeks; ACL tear: <6 weeks, >6 months; anterior shoulder instability: <2 weeks, >6 months; AC joint dislocation, <3 weeks, >6 weeks. CONCLUSION: The current literature varies greatly in defining the terms acute and chronic in common sports injuries. The vast majority of authors imply these terms, based on the method of their studies, rather than define them explicitly. Injuries involving tendons showed greater consistency among authors, thus making a definition based on consensus easier to derive. The literature on ACL and shoulder instability in particular showed great variability in defining these terms, likely representing the more complex nature of these injuries and the fact that timing of surgery in the majority of patients does not particularly affect the complexity of the surgical approach and treatment. CLINICAL RELEVANCE: Defining injuries as acute or chronic is clinically relevant in many cases, particularly concerning tendon injuries, where these terms have implications regarding the anatomic pathologic changes and tissue quality, which may necessitate augmentation and alter the initial surgical plan. In cases where these terms are less pertinent to operative treatment considerations, they bring clarity to the discussion of the acuity of the injury (as it pertains to time from insult).

Bone mineral density loss after combat-related lower extremity amputation.

OBJECTIVES: Determine the incidence, severity, and associated risk factors for the development of low bone mineral density (BMD) after combat-related lower extremity amputation. DESIGN: Retrospective case-control comparison. SETTING: Tertiary care military treatment facility. PATIENTS/PARTICIPANTS: One hundred fifty-six lower extremity amputees, representing 182 amputations (121 unilateral, 35 bilateral). INTERVENTION: All patients underwent dual energy x-ray absorptiometry scanning during the treatment period. MAIN OUTCOME MEASUREMENTS: The Z score was the main outcome measure. We identified all patients with low BMD (Z < -1.0) and conducted multivariate analysis to identify significant risk factors for low BMD development. RESULTS: The observed rate of low BMD was 42%. The average Z score was -0.6 ± 1.1 among unilateral amputations and -1.2 ± 1.0 among bilateral amputations (P = 0.005). Risk factors for the development of low BMD were prolonged time to first ambulation [odds ratio (OR) = 1.39; 95% confidence interval (CI): 1.003-1.93; P = 0.048], prolonged time to dual energy x-ray absorptiometry (OR = 1.10; 95% CI: 1.02-1.18; P = 0.009), and more proximal amputation level (OR = 7.27; 95% CI: 3.21-16.49; P < 0.001). Among unilateral amputees, we detected a significant difference in the BMD of the intact and amputated limbs (-1.0; 95% CI: -1.1 to -0.8; P < 0.001). CONCLUSIONS: Proximal amputation level and delayed ambulation demonstrated a significant relationship with low BMD after traumatic and trauma-related amputation. We conclude that transfemoral amputees are at greater risk of BMD loss and that disuse atrophy is a primary factor in the development of low BMD. Prevention should focus on early and aggressive weight bearing rehabilitation and assessing levels and appropriately supplementing calcium and vitamin D.

Bizarre parosteal osteochondromatous proliferation and periosteal chondroma: a comparative report and review of the literature.

Bizarre parosteal osteochondromatous proliferation and periosteal chondroma are rare, benign, bony lesions that may present in the hand. Bizarre parosteal osteochondromatous proliferation was first reported in the literature by Nora in 1983, and periosteal chondroma was first reported by Lichtenstein in 1952. This report provides a unique side-by-side comparison of these lesions, illustrating each with a clinical vignette. This report, coupled with a thorough review of the literature, serves to demonstrate that the history and physical exam characteristics, as well as the radiographic and gross appearances, are insufficient to differentiate between the two lesions. Histopathologic examination is required to confirm the diagnosis.