Primary versus trauma-induced Gorham-Stout disease.

Gorham-Stout disease - also known as "disappearing bone disease" is currently considered a single entity with varying clinical manifestations. We reviewed the existent literature from the earliest historic description(Jackson in 1838) and Gorham and Stout's original series of patients, multiple case reports and series since. After analyzing 212 reported cases, we identified 76 cases with details that recorded either a history of multifocal disease or an identifiable history of preceding trauma. From this review, we have defined two distinct Gorham-Stout entities - those characteristically associated with lymphangiomatosis [a form of GLA (generalized lymphangiomatosis) questionably distinguishable by bone biopsy and radiologic appearance] with multifocal distributed bone lesions, and those others, usually self-limited, first appearing after a traumatic event and always confined to a single bone or closely adjacent one. Multifocal disease is more likely to have chylothorax as a complication. These two Gorham-Stout entities differ in their demographic distribution, clinical history and manifestations, and they follow divergent clinical courses. The prognosis differs, and so should approaches to monitoring as well as acute and long-term treatment. Further research should seek to identify and define the differences in pathology and molecular mechanisms.

Unexplained "massive osteolysis of femoral head" (MOFH) after acetabular fracture: occurrence and suggested patho-etiology.

Background We observed cases of extensive osteolysis of the femoral head following acetabular fractures even though the original fracture did not involve the femoral head or neck. This observation has been called massive osteolysis of the femoral head (MOFH). Purpose To evaluate the clinical and imaging features of MOFH to gain a better understanding of its patho-etiology. Material and Methods We retrospectively collected cases of acetabular fractures seen over a period of 10 years and evaluated the clinical features and imaging characteristics. The findings were compared with the features of other complications such as osteonecrosis, rapidly progressive osteoarthritis, or post-traumatic osteolysis. Results Fifteen patients (M:F ratio 9:6; mean age, 61.6 years) out of 244 had MOFH (prevalence: 6.1%). Motor vehicle collision and falls were the most common mechanisms of injury. The time interval for developing MOFH was in the range of 1-18 months after the initial injury. Patterns of femoral head osteolysis varied from eccentric (12 cases) to transcervical (3 cases). Four cases of eccentric osteolysis developed high-degree of osteolysis. MOFH was observed near the surgical hardware in 6/15 cases. One biopsy specimen did not reveal typical features of osteoarthritis or avascular necrosis. Conclusion MOFH appears to be a distinct entity from avascular necrosis or rapidly progressive osteoarthritis. It is suggested that MOFH is a variant of post-traumatic osteolysis that is evident in a subset of patients.

Abdominal lymphangiomatosis in a 38-year-old female: case report and literature review.

Lymphangioma is an uncommon benign tumor that develops in the lymphatic system. Abdominal lymphangiomatosis is extremely rare in adult patients, and the clinical symptoms of this condition are complicated and atypical. We report a case of abdominal lymphangiomatosis in a 38-year-old female who presented with intestinal bleeding and protein-losing enteropathy, as well as lesions in the lung and bones. A computed tomography scan revealed multiple small cystic lesions without enhancement. Histological examination revealed microscopic cysts were submucosal, with walls composed of thin fibrous tissue, and D2-40 stained highlight the lining of the lymphatic channels by immunohistochemical method. We make a comparison with the cases reported before, and also discuss the diagnose of diffuse pulmonary lymphangiomatosis and Gorham's disease.

Novel molecular pathways in Gorham disease: implications for treatment.

Rapid advances in evidence-based treatment schedules are a hallmark of modern oncology. In rare neoplastic diseases, however, clinical expertise is hard to build and evidence based on randomized trials almost impossible to collect. Gorham disease is a rare form of lymphatic proliferation accompanied by osteolysis, which usually occurs in young adults. Despite the fact that the clinical course of Gorham disease is often devastating and occasionally fatal, insights into its biological background are sparse and standardized treatment unavailable. Interestingly, recent knowledge on the mechanisms of lymphangiogenesis may help elucidate the pathophysiology of Gorham disease and lead to novel treatment targets. Here, we discuss our current understanding of Gorham disease, discuss established and emerging therapeutic strategies, and attempt to frame a treatment rationale.

Effect of proximal femoral bone support on the fixation of a press-fit noncemented total hip replacement femoral component.

PURPOSE: Proximal femoral bone loss is a common challenge in revision hip arthroplasty. In this study, in-vitro fixation of a non-cemented, rectangular, dual-tapered, press-fit femoral component designed to achieve metadiaphyseal fixation was analyzed using an accelerated proximal femoral bone loss model to assess the potential use in revision cases. METHODS: The press-fit AlloclassicTM femoral stem was implanted in ten cadaveric femurs and tested under cyclic biomechanical loading in an intact state, and then again after sequential proximal femoral bone resections, simulating increasing amounts of bone deficiency. Anterior-posterior and medial-lateral interface motions were measured at the distal stem tip throughout loading. 
 RESULTS: Three specimens remained stable throughout testing, with initial and peak per-cycle motions of less than 50 µm. Six specimens were destabilized under loading with higher per-cycle motions, specifically at the distal stem tip during peak loading in the anterior-posterior direction, with motions of 78±69 µm, compared to 12±9 µm in the stable specimens (P<.05). Total migration of the destabilized specimens was also significantly higher, specifically at the proximal stem tip in the medial-lateral direction, with migrations of 101±34 µm (P<.05) and at the distal stem tip in the anterior-posterior direction, with migrations of 155±179 µm (P<.05), compared to 33±12 µm and 13±11 µm for the stable specimens. CONCLUSION: The results indicate that when strong initial fixation is achieved, long-term success is possible given substantial proximal femoral bone loss.

Surfer's toe: trauma-induced idiopathic acro-osteolysis in the toes of a 46-year-old surfer: a case report.

Acquired acro-osteolysis (AOL) is defined as the resorption of bone from the tufts or shafts of the terminal phalanges. Acquired acro-osteolysis can manifest as a primary osteolysis syndrome and also appears in a number of disease states including rheumatologic disorders, neuropathic diseases, the result of prolonged exposure to polyvinyl chloride, and in rare cases, as a response to repeated mechanical stress. In this report, a 46-year-old surfer was evaluated for AOL as a complication of sports-related repetitive trauma to the right second and third toes. Radiography showed the bony tips of his right second and third toes had been eroded away. Acquired acro-osteolysis in the surfer's toes resulted from increased blood flow initiated to repair microdamage caused by repeated trauma to the distal ends of his second and third right toes due to the habitual dragging of the affected toes across a surfboard. The always initial lytic phase of bone repair was magnified by the increased arterial input to warm the extremities after prolonged exposure to cold. At 6-years' follow-up, the use of a protective bandage while surfing has permitted full regeneration of the affected toes.

Chylous hip joint effusion and bone absorption after total hip arthroplasty in a patient with chylocolporrhoea: a case of Gorham's disease.

Chyle is a sterile, milky fluid consisting of lymph and emulsified fats that is formed in the small intestines and taken up by lymph vessels. Chylous effusions usually occur after destruction or obstruction of lymphatic channels, and chylous joint effusions have been reported in association with rheumatoid and/or septic arthritis, and as the result of penetrating trauma to subsynovial fatty tissue and the intra-articular fat pad. We report a case of bone absorption and lytic change in the femur associated with a chylous hip joint effusion after a total hip arthroplasty (THA) in a patient with chylocolporrhoea and a history of chylous ascites.

Neuropathic arthropathy of the shoulder associated with syringomyelia: a report of six cases.

Here, we report a series of 5 patients (6 shoulders) diagnosed with neuropathic arthropathy of the shoulder joint in our clinic between 2005 and 2008. Initial diagnosis, previous treatment, and radiological and clinical follow-up findings were reviewed. The mean age at diagnosis was 44.2 years. Four patients had unilateral and 1 patient had bilateral involvement. The presenting symptoms were pain, swelling, and loss in range of motion. Active forward flexion and abduction ranged from 0° to 90°. Hypoesthesia and loss of temperature sense was evident in 3 patients. Radiographs showed massive osteolysis of humeral head and glenoid process, and magnetic resonance imaging showed periarticular fluid collection, and degeneration at the rotator cuff and shoulder joint, resembling chronic septic arthritis or sarcoma. Biopsy was performed in 4 patients before definitive diagnosis, and synovial hypertrophy and necrotic bone was found. Two patients had a history of operated cervical syringomyelia, and the remaining 3 patients were later diagnosed to have syringomyelia and referred to neurosurgery clinic, where 2 of those were operated. Four patients were followed-up with symptomatic therapy, and 1 patient underwent an unsuccessful shoulder arthroplasty in another clinic. As a conclusion, neuropathic arthropathy of the shoulder is rare, and correct diagnosis is possible by careful physical and neurological examination and pathologic evaluation when needed.

Síndrome de Gorham-Stout: doença do osso fantasma / Gorham-Stout syndrome: phantom bone disease

A síndrome de Gorham-Stout é uma doença que apresenta osteólise idiopática de um osso ou área contígua próxima. A etiologia é desconhecida, sendo uma condição rara, de difícil diagnóstico e tratamento controverso. Acomete pessoas sem distinção quanto à idade e ao sexo. Neste trabalho realizamos uma revisão bibliográfica da doença, dando enfoque específico no diagnóstico diferencial, e demonstramos o acompanhamento de um paciente com esta síndrome, desde o seu diagnóstico, tratamento e estado atual de evolução.

Gorham-stout syndrome is a disease that presents idiopathic osteolysis of a bone or contiguous area. The etiology is unknown. It is a rare condition, difficult to diagnose and with controversial treatment. This condition affects persons with no distinction as to age or sex. In this study, we conduct a bibliographic review of the disease, specifically focusing on differential diagnosis, and follow a patient with this syndrome from the time of its diagnosis, through treatment, to its current state of evolution.

Massive bone loss from fungal infection after anterior cruciate ligament arthroscopic reconstruction.

Although there are numerous reports of septic pyogenic arthritis after arthroscopic anterior cruciate ligament (ACL) reconstruction, there is limited information regarding the outcomes of fungal infection. We determined the outcomes of six patients with mycotic infection after regular ACL reconstruction. There were four males and two females with a mean age of 33 years. We determined the number of procedures performed, bone loss originating to control infection, and final reconstruction in these patients. An average of five arthroscopic lavage procedures had been performed at the referring centers. Fungal infection was diagnosed based on pathologic samples; five infections were the result of mucormycosis and one was Candida. After final débridement, the mean segmental bone loss was 12.8 cm. All patients were treated with intravenous antifungal coverage and cement spacers before final reconstruction. At final followup, all patients were free of clinical infection. Three had reconstruction with an allograft-prosthesis composite, two with hemicylindrical allografts, and one with an intercalary allograft arthrodesis. Despite the extremely unusual presentation of this complication, surgeons should be aware of potential and catastrophic consequences of this severe complication after ACL reconstruction.

Mid-term results using a cementless hip prosthesis in young Chinese patients: a five- to seven-year follow-up study.

A retrospective study was undertaken to evaluate the clinical and radiographic outcomes of 74 cementless total hip arthroplasties (THA) in 69 young Chinese patients. The Asian size and MMA AML stems with smoothly tapered tip were used, and the patients were followed up for at least five years. The mean Harris hip score was 45.4 preoperatively and 95.3 at the last follow-up. The incidence of thigh pain was 5.4%, and was related to the short stature of the patient (<160 cm) (P = 0.030). Six patients (6 hips, 8.1%) had acetabular osteolysis in zone 2; reoperation was performed in one patient because of osteolysis and wear of the polyethylene liner. The survival rate of the metal acetabular and femoral components was 100% (95% confidence interval, 0.95-1.0). Primary THA with this AML prosthesis had an acceptable mid-term result in young Chinese patients.

Gorham's disease: an osseous disease of lymphangiogenesis?

Gorham's disease, also known as massive osteolysis, Gorham-Stout disease, vanishing bone disease, or, phantom bone disease is a rare disorder of the musculoskeletal system. The disease is characterized by osteolysis in bony segments, with localized proliferation of lymphatic channels. The presence of abundant, leaky systemic lymphatic vessels is often accompanied by chylous ascites. There is no standardized treatment available for Gorham's disease, and its molecular mechanisms remain unclear. Future strategies for understanding Gorham's disease should emphasize its apparent identity as a disease of disordered lymphangiogenesis. Breakthroughs in lymphatic research have identified several lymphangiogenic pathways that may play a relevant role in Gorham's disease.