1/22. bacteremia and skin/bone infections in two patients with X-linked agammaglobulinemia caused by an unusual organism related to Flexispira/helicobacter species.Two patients with Bruton's X-linked agammaglobulinemia are described with bacteremia and skin/bone infection due to an organism which by 16S rRNA gene sequence analysis was most closely related to "Flexispira" rappini (and thus designated a Flexispira-like organism, FLO) and more distantly related to the helicobacter species. The organism required microaerobic conditions and, supplemental H(2) gas for growth and was reliably stained with acridine orange. In common with helicobacter cinaedi infections, the focus of the FLO infection was in one case in the blood vessels or lymphatics of an extremity and in the other case in the skin and adjacent bone of an extremity. In both cases, prolonged IV antibiotic therapy was necessary to clear the infection. The susceptibility of XLA patients to FLO infection appears to be related to the fact that XLA is associated with severe B cell (humoral) immunodeficiency and thus these patients have difficulty with intravascular or intralymphatic infection. These findings elucidate the nature of FLO infections in humans and point the way to their detection and treatment.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/22. Humeral hydatid cyst complicated with extraosseous involvement: a case of unusual location of echinococcosis.Hydatid disease is a parasitic disease most commonly caused by echinococcus granulosus that seldom involves the skeleton and is still common in the countries of the temperate zones. We present an extremely rare case report of a young patient with humeral hydatid disease complicated with extraosseous involvement. Plain film of the left humerus demonstrated distortion of the axis, regional expansion of the humeral shaft with minimal thinning areas of the cortex. Additionally, numerous radiolucent areas of the humeral shaft were observed especially at the distal part of the bone. No calcification of the soft tissues was noticed. Computed tomography examination showed significant destruction of the trabecular bone of the humeral head and polycystic appearance of the bone marrow with regional calcifications into the lumen. Additionally, a large cystic lesion between medial and lateral head of the triceps muscle with some foci of calcifications were observed. magnetic resonance imaging revealed the multiocular nature of the lesion into the humeral lumen with a cystic lesion involving the soft tissues of the upper arm. The cyst showed contrast enhancement at the pericyst capsule of the cyst after gadolinium-DTPA administration.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/22. An algorithm for management of residual posttraumatic calvarial defects in adults.For the discussion of options in late reconstruction of residual posttraumatic calvarial defects in adults, the calvaria is divided into three reconstructive zones. Zone 1 comprises the frontal sinus region and the contour of the supraorbital brow; Zone 2 comprises the smooth, cosmetically visible prehairline forehead; Zone 3 comprises the posthairline area and the calvaria. The particular reconstructive requirements (autogenous bone versus alloplastic material) of each zone are described and illustrated with clinical cases. The merits of bone from various donor sites and those of alloplastic material are discussed. The authors present an algorithm of reconstructive choices for residual posttraumatic calvarial defects in adults based on the nature of the defect and the aesthetic reconstructive zone.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/22. Intraosseous proliferative sparganosis: a case report and review of the literature.Intraosseous proliferative sparganosis is an extremely rare parasitic disease in which the larvae of incomplete differentiated sparganum proliferate in the human bone. We present the first case of intraosseous proliferative sparganosis arising in the long bone. The patient was a 51-year-old man who complained of a slow growing painful mass on his right leg. The radiographic findings showed an infiltrative osteolytic lesion with speckled calcification at the proximal tibia the clinical diagnosis of which favored chondrosarcoma. Incisional biopsy revealed an innumerable number of small globular shapes, whitish parasites. Histologically, the parasites were composed of a few layers of smooth muscle and several calcerous bodies that were enclosed within a single row of tegumental cells. The latter exhibited a wavy appearance and coated with microvilli. These morphologic findings confirmed the nature of these maldifferentiated larvae. The patient was treated by partial resection of the lesion. This should remind clinicians that parasitic infection of the bone can produce a tumor-like lesion.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/22. Congenital partial aplasia of the posterior arch of the atlas causing myelopathy: case report and review of the literature.STUDY DESIGN: A case report of a patient with a congenital anomaly of the posterior arch of the atlas and review of the literature are reported. OBJECTIVE: To describe a unique presentation of cervical myelopathy caused by a mobile isolated bone fragment in an otherwise healthy young male. The description is supplemented by a video showing the repeated trauma the bone fragment caused to the dorsal spinal cord with neck extension. SUMMARY OF BACKGROUND DATA: Congenital anomalies of the posterior arch of C1 are well described and are almost always asymptomatic and found incidentally. Neurologic symptoms, either of a chronic nature or developing acutely following head or neck trauma, have been described in patients with isolated posterior arch tubercles (Types C or D). methods: A 17-year-old male developed sensory deficit in his distal lower extremities acutely that progressed over several weeks proximally and into his left upper extremity. He also described a Lhermitte sign, only with extension of his neck and an episode of temporary quadriparesis with a minor fall. Plain radiographs and a computed tomography scan of his neck revealed a congenital deformity of the posterior arch of the atlas. magnetic resonance imaging showed T1 and T2 signal abnormality within the posterior spinal cord at the level of C1 to C2. RESULTS: The patient underwent flexion and extension movements under fluoroscopy, which showed the posterior tubercle moving anteriorly and compressing the spinal cord every time he extended his neck. The fragment was resected, and, intraoperatively, dense fibrous tissue was found to be bridging the bony defect. After surgery, his sensory deficits almost completely resolved, and he no longer had a Lhermitte phenomenon. CONCLUSION: This case illustrates a symptomatic congenital deformity of the posterior arch of the atlas due to a mobile, isolated fragment. These anomalies are exceedingly rare. To date, only 17 patients, including ours, have been described in the literature as having myelopathy related to an isolated posterior tubercle. Of these, only two patients had documented movement of this tubercle before our report. Recognizing the anomaly is crucial because treatment is relatively simple, produces resolution of symptoms, and prevents major neurologic deficits from occurring after trauma.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/22. eosinophilic granuloma: resolution of maxillofacial bony lesions following minimal intervention. Report of three cases and a review of the literature.INTRODUCTION: Langerhans' cell histiocytosis is a collective term used to describe a group of enigmatic proliferative disorders. The natural history of the disease varies from a slow, benign, localized symptomatic bony or soft tissue lesion, to a rapidly progressive widespread multiple organ disorder which is often fatal. eosinophilic granuloma accounts for 60-70% of all cases of Langerhans' cell histiocytosis and can present as solitary (50-75%) or multifocal defects in bone. It occasionally presents as a localized soft tissue lesion. There are multiple treatment options but the response is unpredictable. AIMS: We present three separate cases, of the maxillofacial skeleton where the lesions of eosinophilic granuloma resolved following incisional biopsy only. patients AND RESULTS: Three patients presented with solitary lesions of the maxillofacial skeleton. All were diagnosed as Langerhans' cell histiocytosis following open curettage, which also resulted in resolution of the lesions. Follow-up has thus far been disease free. CONCLUSION: For some solitary Langerhans' cell histiocytosis lesions, simple curettage is the only treatment required. The paper discusses the need to confirm the solitary nature of the disease and the need for follow-up. Reviewing the literature on the disease, the authors suggest that perhaps cellular immaturity holds the cells of the lesion in a disease state until pushed to maturity by the trauma of open curettage surgery, resulting in a complete resolution of the disease.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/22. Intradiploic primary epithelial inclusion cyst of the skull.Intradiploic inclusion cyst of the skull presenting as a calvarial defect is fairly uncommon in the neuroscience practice. They are benign in nature, slow to grow, and clinically difficult to differentiate from other causes of calvarial defects. We present a case of a healthy 55-year-old woman who presented clinically for an asymptomatic lump over the right parieto-occipital region of the scalp of 4-month duration. The radiological features were suggestive of presence of a large intradiploic solitary cyst and additional multiple lytic defects with sclerotic borders, destroying the underlying bone. The gross and histomorphological features of the right parieto-occipital craniectomy specimen received were pathognomic of an intradiploic primary epithelial inclusion cyst of the skull bone. The importance of its benign nature, embryogenic origin, and differentiation from other cystic lesions with similar histology is stressed. We report this case for its unique histomorphology and first of its kind in the Indian literature.- - - - - - - - - - ranking = 2keywords = nature (Clic here for more details about this article) |
8/22. Synovial chondromatosis with intracranial extension. A case report.A case of unilateral synovial chondromatosis of the temporomandibular joint with intracranial extension is presented. Extracapsular extension of these lesions is rare, and few cases have previously been reported. The case presentation will also reflect the inadequacy of routine preoperative temporomandibular joint x-ray films to reflect the extent of the lesion. Despite the benign nature of the lesion, excision was done because of the clinical aggressive behavior.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/22. Giant cell reaction in a phalangeal bone.A case of the rare and non-neoplastic lesion, giant cell reaction of bone, is described. The pathological and radiological features of this lesion are reviewed and an aetiological mechanism is proposed to account for its unique histopathological features. Recognition of this lesion and its non-neoplastic nature are important so as to avoid surgical ablation of the affected bone.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/22. Lymphographic patterns in systemic mastocytosis with diffuse bone involvement and hematological signs.lymphography revealed the nature of diffuse lymph node involvement in systemic mastocytosis with diffuse bone involvement and hematological signs, similar to that seen in some malignant hematological diseases or in osteomyelosclerosis. The final stages of transformation into acute lymphoblastic leukemia must also be considered.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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