Gorham's disease: a literature review and case reports

Gorham's disease (disappearing bone disease, massive osteolysis, idiopathic osteolysis, essential osteolysis, progressive atrophy of bone, spontaneous absorption of bone, phantom bone, hemangiomatosis/lymphangiomatosis of bone, progressive osteolysis) is an extremely rare occurrence. There are fewer than 150 reported cases in the literature. This disorder can be characterized by spontaneous or posttraumatic progressive resorption of bone. The etiology is still very speculative, the prognosis unpredictable, and any effective therapy still unknown. This paper presents a review of the literature and two case reports of suspected Gorham's disease of the bones of the foot.     

The effect of prostaglandin synthesis inhibitors and diphosphonates on tumour-mediated osteolysis

Previous studies have shown that the prostaglandin synthesis inhibitor indomethacin reduced osteolysis produced by the experimental VX2 carcinoma, probably by inhibiting the stimulation of osteoclasts by prostaglandin E2. This study was carried out to determine whether prostaglandin inhibitors affect the tumour osteolysis produced by human mammary carcinoma as well as the VX2 carcinoma. The effect of diphosphonates on reducing tumour osteolysis was also investigated, since diphosphonates directly affect bone resorption. The results indicated that various non-steroidal, anti-inflammatory agents which inhibit prostaglandin synthesis reduced the osteolysis produced by human mammary or rabbit VX2 carcinomas. The diphosphonate compounds also produced a significant inhibition of tumour osteolysis. The results confirm the findings of Powles and his colleagues (1) that aspirin (also a prostaglandin synthesis inhibitor) reduced the osteolysis induced by human mammary carcinoma. It is suggested that these agents be evaluated as adjuvant therapy in patients with apparently 'early' mammary cancer in a controlled clinical trial.     

Periprosthetic acetabular fracture associated with extensive osteolysis

Periprosthetic fracture of the acetabulum is an uncommon complication of total hip arthroplasty. The management is reported to be difficult, and complications such as nonunion and implant loosening are common. We described herein a case of nontraumatic periprosthetic acetabular fracture associated with significant osteolysis, which was successfully managed by addressing the fracture and osteolysis independently.     

Membrane electrical properties associated with insulin receptor downregulation in human erythrocytes

We reviewed 29 consecutive patients after cemented femoral revision of cemented hip arthroplasties for osteolysis. After an average follow-up of 8.5 years, osteolysis had recurred in only two cases (6.9%) and 25 femoral components (86%) remained well fixed.     

Massive craniofacial osteolysis

Involvement of the craniofacial skeleton by the process of massive osteolysis has been infrequently recorded. We describe a patient whose extent of craniomaxillofacial bony loss defied combined reconstruction by combined autogenous tissue and alloplastic materials. The clinical and radiographic features and management approaches to massive osteolysis are reviewed.     

Frequent isolation of human herpesvirus 7 from saliva

BACKGROUND: The purpose of this study was to investigate the influence of a prophylactic bone protective treatment with the bisphosphonate dichloromethane/diphosphonic acid (Cl2MBP) in an experimental model of osteolysis with intraosseous implantation of the Walker carcinosarcoma 256 B. METHODS: The biphosphonate was given as a high-dose, short-term treatment (30 mg/5 days sc) followed by a treatment-free interval (1-7 weeks) or as a low-dose, long-term prophylactic treatment (2.5 or 5.0 mg/day/3 weeks sc). Osteolysis was measured with a radiographic and histologic grading system. RESULTS: The high-dose short-term prophylactic treatment was shown clearly to inhibit tumor osteolysis. The osteoprotective effect decreases with increasing length of the treatment-free interval. A similar positive result could be achieved following the low-dose long-term prophylactic treatment. Dosage could not be shown to influence the inhibition of tumor osteolysis in the long-term bone protective treatment. A possible direct influence of the treatment on tumor growth could be ruled out. The prophylactic treatment does not inhibit body weight increase. Animals treated prophylactically showed less weight loss than the controls after tumor implantation. CONCLUSIONS: These results show that a prophylactic treatment with Cl2MBPs protects the skeleton effectively against tumor osteolysis.     

Osteoclasts are required for bone tumors to grow and destroy bone

It has been hypothesized that bone resorption during tumor osteolysis is performed by osteoclasts. Data supporting this hypothesis have been provided from analysis of human biopsy specimens obtained from sites of tumor osteolysis, as well as from experimentation with in vivo animal models. Experiments in this report take this concept one step further by testing the hypothesis that osteoclasts are required for bone tumors to grow and destroy bone. To test this hypothesis, the influence of an osteolytic sarcoma tumor, NCTC clone 2472 (2472), on bone was studied in animals that are osteoclast deficient (microphthalmic, strain B6C3Fe-a/a-Mitf(mi)) but whose osteoclast deficiency can be reversed following bone marrow transplantation. Femora of these mice and unaffected wild-type siblings were injected with 10(5) 2472 cells, and after 14 days the femora were analyzed by radiographic and histomorphometric analysis. Macroscopic tumor, tumor-induced osteolysis, and increased osteoclast number were noted in femora of normal mice but not in femora of osteoclast-deficient mice (p < 0.001). Bone marrow transplantation converted osteoclast-deficient mice to mice with femora that contained osteoclasts in 4 weeks. Femora of these mice were then injected with 10(5) 2472 tumor cells; after 14 days, in contrast to the findings in the original osteoclast-deficient mice, macroscopic tumor was present, tumor-induced osteolysis was noted on roentgenograms, and osteoclast number was increased when tumor-bearing limbs were compared with sham-injected limbs (p < 0.001). These data prove the hypothesis that osteoclasts are required for 2472 tumor-induced osteolysis, and they introduce the exciting possibility that osteoclasts are also required for tumors to grow in bone.     

Osteolysis associated with cemented total knee arthroplasty

Osteolysis has not been mentioned as a complication or cause of failure of cemented total knee arthroplasties in long-term follow-up studies. We are aware of a single case report of osteolysis after cemented total knee arthroplasty. We report the case of an 87-year-old woman with massive osteolysis beneath a cemented tibial component.     

Clinical assessment of posttraumatic headaches

The evaluation of the person with posttraumatic headache remains a difficult clinical task. The rehabilitation clinician seeing the person with posttraumatic headache may encounter a large spectrum of potential etiologies for cephalgia. We believe an ordered approach to the history, physical, and laboratory examination may lead to a more accurate and focused diagnosis in some cases. In an attempt to provide a directed approach to posttraumatic headaches, this discussion is divided into extracranial and intracranial sources. A brief table guide to the etiology of pain by site is presented, and the key eight steps of examination are reviewed.     

Direction of influence between posttraumatic and depressive symptoms during prolonged exposure therapy among children and adolescents.

Objective: Our objective in the present study was to examine the temporal sequencing of posttraumatic and depressive symptoms during prolonged exposure therapy for posttraumatic stress disorder (PTSD) among children and adolescents. Method: Participants were 73 children and adolescents (56.2% female) between the ages of 8 and 18. Participants completed self-report measures of posttraumatic stress and depression prior to every session. Measures included the Child PTSD Symptom Scale, Beck Depression Inventory, and Children's Depression Inventory. Results: Multilevel mediational analyses indicated reciprocal relations during treatment: Changes in posttraumatic symptoms led to changes in depressive symptoms and vice versa. Posttraumatic symptoms accounted for 64.1% of the changes in depression, whereas depressive symptoms accounted for 11.0% of the changes in posttraumatic stress. Conclusions: Prolonged exposure therapy may work primarily by reducing posttraumatic stress, which in turn reduces depression. (PsycINFO Database Record (c) 2011 APA, all rights reserved)     

Fixation with bioabsorbable pins in chevron bunionectomy

Fixation with bioabsorbable pins in distal chevron bunionectomy reduces the inconvenience and the risk of infection associated with fixation with stainless-steel Kirschner wires, which leaves a portion of the wires protruding from the skin. However, use of bioabsorbable implants has been reported to be associated with osteolysis and formation of a sinus with a sterile discharge. We studied the outcome and complications seen with use of poly-p-dioxanone pins and those seen with use of stainless-steel Kirschner wires after chevron bunionectomy in 114 patients (144 feet). We found no difference between the treatment groups with regard to the prevalence of complications or the stability of fixation. Notably, the prevalence of osteolysis was quite similar between the treatment groups; none of the feet that had had fixation with bioabsorbable pins had formation of a sinus with a sterile discharge. We believe that bioabsorbable pins can be used reliably to fix the site of the osteotomy for a distal chevron bunionectomy without undue risk of osteolysis or other complications.     

Self-evaluative appraisals of coping capability and posttraumatic distress following motor vehicle accidents.

This study tested the importance of coping self-efficacy (CSE) perceptions and change in perceptions of CSE for recovery from motor vehicle accident (MVA) trauma. Data were collected 7 days following the accident (Time 1; n = 163), 1 month after the accident (Time 2; n = 91), and 3 months after the accident (Time 3; n = 70). Early changes in CSE (i.e., from Time 1 to Time 2) predicted posttraumatic distress at 3 months after MVA trauma, even after controlling for Time 1 or Time 2 posttraumatic distress and other trauma-related variables (i.e., accident responsibility, litigation involvement, and peritraumatic dissociation). Early changes in CSE perceptions, however, neither moderated nor mediated the effects of early posttraumatic distress (Time 1) on 3-month posttraumatic distress. Time 2 CSE levels, however, did mediate the relationship between acute posttraumatic distress (Time 1) and 3-month posttraumatic distress (Time 3). These findings highlight the importance of early interventions aimed at strengthening self-efficacy after MVA trauma. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Demonstration of a new dopamine-containing cell group in the primate rostral telencephalon

An unusual case of rapid-growing posttraumatic gluteal epidermal cyst with ischiorectal and presacral extension is reported. Pertinent clinical history and magnetic resonance imaging allowed accurate preoperative diagnosis. Cautious complete excision was important to prevent recurrence. To our knowledge, posttraumatic gluteal epidermal cyst with this unusual clinical presentation has not been described previously.     

The fate of pelvic osteolysis after reoperation. No recurrence with lesional treatment

Twenty-three hips (21 patients) with 30 pelvic osteolytic lesions underwent reoperation and were observed prospectively for 25 to 74 months (average, 40 months) to assess the fate of pelvic osteolysis after reoperation. The average radiographic dimensions of the lytic lesions were 2.4 x 1.9 cm with the largest lesion measuring 7 x 5 cm. The porous ingrowth acetabular component shell had been left in situ in 15 hips and had been revised in eight. There was no difference in the average lesional size for hips with revised shells compared with those with unrevised shells. In cases where the shell was left in situ, the osteolytic lesions were curetted by working around the component perimeter or through holes in the shell. In 18 hips the bone defect(s) were grafted with autograft or allograft. Regardless of the management of the acetabular shell or the absence or presence of bone graft, none of the osteolytic lesions have progressed. Twenty-six of the 30 lesions have increased radiographic density. All acetabular components remain radiographically well fixed. There were no new osteolytic lesions. All hips were functioning well, and none have required subsequent reoperation for any reason. There was a statistically significant reduction in the operative time and the amount of blood loss when the acetabular component was not revised. It does not appear necessary to remove a well fixed and well positioned cementless acetabular component for the treatment of pelvic osteolysis. Debridement of periarticular inflammatory tissue and lesional curettage, either with or without bone graft, is effective in managing this type of bone loss. Revision of the acetabular component shell was associated with a significant increase in operative time and blood loss. These results support routine radiographic evaluation after total hip arthroplasty to monitor the development of osteolysis. On the basis of this experience, the authors recommend lesional treatment of progressive pelvic osteolysis to avoid more difficult surgery and minimize patient morbidity.     

Kasabach-Merritt syndrome of the leg associated with osteolysis or Gorham sign

INTRODUCTION: Kasabach-Merritt syndrome and Gorham's sign are two uncommon and severe, sometimes life-threatening, complications in infants with vascular lesions. Their association has been described in rare cases. CASE REPORT: An infant with a vast congenital angiomatous lesion including an extensive lymphatic component, developed active regional osteolysis then suddenly suffered disseminated intravascular coagulation of the leg. Medical treatment was unsatisfactory. After unsuccessful use of low molecular weight heparin, pentoxifyllin and alpha interferon, amputation of the leg was required to avoid a fatal outcome. DISCUSSION: Kasabach-Merritt syndrome does not develop on classic immature hemangiomas, despite some contradictory statements in the literature. In our case, a complex tumor developed in association with a lymphatic malformation. The association of Kasabach-Merritt syndrome with osteolysis (Gorham's sign) does not appear to be fortuitous. Therapeutic management of these severe complications is difficult and requires case by case analysis.     

National service in rural areas: the case of Colombia

A rational approach to the management of spontaneous fracture of the severely atrophie mandible is illustrated by means of a case report. A study of the literature reveals that some confusion exists concerning the terms "atrophy" and "osteolysis", and an attempt is made to distinguish between them.     

The relationship between posttraumatic stress symptoms and sexual risk: Examining potential mechanisms.

Little is known about behavioral and psychological mechanisms that may explain relationships between posttraumatic stress and sexual risks. As rates of HIV infection among African American women remain significantly higher than for other female subgroups, research on sexual risk among African American women is needed. The present study examines the relationships of posttraumatic stress symptoms as measured by the Posttraumatic Stress Disorder Checklist-Civilian Version with sexual risk behaviors, sexual sensation-seeking, and sexual compulsivity in 30 undergraduate African American women with any reported history of sexual intercourse. Higher posttraumatic stress symptoms were associated with more sexual partners, greater frequency of vaginal sex without a condom, and endorsement of sex while under the influence of a substance. Posttraumatic stress symptoms were negatively correlated with perceived sexual control, but were not significantly correlated with sexual compulsivity or sensation-seeking. Perceived sexual control was negatively associated with frequency of unprotected sex and sex under the influence. The preliminary evidence from this small sample suggests sexual control may mediate the relationship of the posttraumatic stress symptoms with unprotected sex. These results are generally consistent with previous findings suggesting posttraumatic stress is associated with sexual risk. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Posttraumatic stress symptoms and depression in mothers of children with severe burn injuries

This study examined posttraumatic stress symptoms and depressive symptoms in mothers of children with burn injuries from accidentally falling into a bathtub filled with hot water. Subjects were 16 pairs, children with burn injuries and their mothers. Psychiatric interviews were administered to the mothers to check the presence or absence of mental disorders. The severity of mothers' depressive symptoms was rated on the Hamilton Depression Scale. Prevalence rates of DSM-IV posttraumatic stress disorder and major depression were 6.3% and 0% in children with burn injury and 12.5% and 18.8% in their mothers, respectively. For three symptoms of posttraumatic stress disorder (intense distress at similar event, restricted range of affect, and hypervigilance), prevalence rates were significantly higher for the mothers than for the children. Ratings of the three symptoms of posttraumatic stress disorder for the mothers were significantly and positively correlated with scores for guilt feelings. Compared with children with burn injury, mothers are prone to posttraumatic stress symptoms mixed with guilt feelings for children with burn injury.     

Perceived beneficial and detrimental postwar responses of Israeli adults: Are they positively or negatively linked to each other?

This study first compares two theoretical perspectives on the links between beneficial and detrimental posttraumatic responses. One group of studies claims that positive posttraumatic responses tend to be positively related with negative stress-related responses. Others posit that, after a severe stress, these responses are negatively correlated with each other. We argue that these contradictory results represent the different theoretical positions on the nature of favorable postwar outcomes. A second issue investigated is the associations of beneficial and detrimental postwar responses with demographic variables as well as with exposure to the stress of war. An Israeli adult sample (n = 870) was administered two different measures of negative posttraumatic outcomes (resource loss and postwar symptoms) and two different measures of positive posttraumatic outcomes (resource gains and posttraumatic recovery) one year after they were badly affected by a war. Resource gains correlated positively with resource loss, whereas PTR correlated negatively with postwar symptoms. There were also positive associations between the two measures of stress as well as the two beneficial posttraumatic outcomes. Older age, being a woman, lower economic status, and higher exposure to traumatic events tended to correlate positively with both measures of detrimental outcomes. These variables correlated negatively with PTR, and three of them (excluding economic status) were not significantly linked with resource gains. (PsycINFO Database Record (c) 2011 APA, all rights reserved)