ABSTRACT
Angiokeratomas consist of ectasias of dermal capillaries associated with an acanthotic and hyperkeratotic overlying epidermis. These dark red-to-purple, papular vascular anomalies can vary considerably in size, depth, and location. It is a skin disorder that rarely involves oral cavity. It can occur in localized or generalized form and is often associated with underlying metabolic disorder such as Fabry's disease and fucosidosis. It has many clinical variants with the same underlying histopathology. Mucosal involvement, including the oral cavity, is occasionally found either as a component of the systemic variety, called angiokeratoma corporis diffusum, or associated with cutaneous lesions in more locations. Isolated oral involvement seems to be rather infrequent, and only eighteen cases have been described in the world literature thus far. Isolated multiple angiokeratomas of tongue without plaque formation have been reported only four times before this. Here, we report a fifth case of isolated multiple angiokeratomas of tongue in a 16-year-old female which was confirmed by immunohistochemical pattern in consonance with a blood vessel origin, with expression of CD31, CD34, and von Willebrand factor. The lesion did not express D2-40 and CD45. No other malformation or metabolic disorder was found in the patient.
ABSTRACT
Ewing's Sarcoma of bone (ESB) is a rare primary malignant tumor of bone, belonging to Ewing's Sarcoma Family of Tumors (EFT) and are neuro-ectodermal in origin. These tumors are characterized histopathologically as small round blue cell tumors (SRBCT) containing cytoplasmic glycogen, cytogenetically by a (t:11;22) or (q:24;12) translocation and molecularly by the presence of EWS and FLI1 fusion transcripts. ESB is primarily a pediatric tumor, uncommon in the Asian population, affecting the axial skeleton and rarely the jaw bones. ESB poses a diagnostic challenge as it shares many features with other malignant tumors whose managements are substantially different. We present the clinical, radiographic histopathological and immuno histochemical features of ESB involving the left rhinomaxillary complex in a young Indian male. We also discuss the differential diagnosis and current treatment modalities in management of ESB.
ABSTRACT
Aim: To compare the efficacy of part-time versus full-time occlusion for treatment of amblyopia in children aged 7-12 years. Materials and Methods: Prospective interventional case series. One hundred children between 7-12 years of age with anisometropic (57), strabismic (25) and mixed (18) unilateral amblyopia were randomized (simple randomization) into four groups (25 each) to receive two hours, four hours, six hours or full-time occlusion therapy. Children were regularly followed up at six-weekly intervals for a minimum of three visits. Statistical Analysis: Intragroup visual improvement was analyzed using paired t-test while intergroup comparisons were done using ANOVA and unpaired t-test. Results: All four groups showed significant visual improvement after 18 weeks of occlusion therapy ( P < 0.001). Seventy-three (73%) of the total 100 eyes responded to amblyopia therapy with 11 eyes (44%), 17 eyes (68%), 22 eyes (88%) and 23 eyes (92%) being amblyopia responders in the four groups respectively, with the least number of responders in the two hours group. In mild to moderate amblyopia (vision 20/30 to 20/80), there was no significant difference in visual outcome among the four groups ( P =0.083). However, in severe amblyopia (vision 20/100 or worse), six hours ( P =0.048) and full-time occlusion ( P =0.027) treatment were significantly more effective than two hours occlusion. Conclusion: All grades of part-time occlusion are comparable to full-time occlusion in effectiveness of treatment for mild to moderate amblyopia in children between 7-12 years of age unlike in severe amblyopia, where six hours and full-time occlusion were more effective than two hours occlusion therapy.