We report a case of the Antley-Bixler syndrome in an 11-year-old girl. She presented with bilateral proximal femoral focal deficiency, right clubfoot, left radiohumeral synostosis, bilateral ear hypoplasia, cleft palate, tongue tie, missing teeth, congenital heart disease, a pelvic kidney with hydronephrosis, and mental retardation. Proximal femoral focal deficiency has never been reported before as a manifestation of Antley-Bixler syndrome. Her mother was exposed to radiation during an intravenous urogram done in the first trimester of pregnancy. Exposure to radiation has not been implicated as a cause of Antley-Bixler syndrome.
The Osteoporosis Self-Assessment Tool for Asians (OSTA) score has been developed to identify women at risk of osteoporosis. It can be used as a screening tool for patients at risk who would benefit from bone mineral density measurement and treatment. It was developed based on data from eight countries including Malaysia. However, most subjects were of Chinese (59%). This study evaluated the performance of OSTA among 152 post-menopausal Malay women. OSTA score calculation and DEXA scan were performed. Our results showed that the OSTA score is a good predictor of patients at risk of osteoporosis based on BMD measurements at the proximal femur. Instrument sensitivity was 87.5%, specificity was 95.8%, positive predictive value (PPV) was 0.538, negative predictive value (NPV) was 0.993, and the area under the receiver operating characteristic curve (ROC) was 0.895. We conclude that use of the OSTA score in postmenopausal Malay women is effective and has adequate sensitivity and specificity.
The use of 'spare part' tissue from an amputated segment to cover a stump with soft tissue deficiency is a viable option for augmenting the shape of the stump and preserving functional length of the stump without additional donor morbidity. The procedure requires no microsurgical skill if the tissues are used as pedicle fillet flaps transfer. This concept of fillet flap makes the amputation distal to an unfavourable definitive amputation site such as in congenital constriction band possible. We report herein a case of amputation distal to constriction ring to illustrate the application of fillet flap in reconstruction surgery.
We compared the result of radiofrequency (RF)-assisted valvotomy and balloon dilation with closed surgical valvotomy and Blalock Taussig (BT) shunt as primary treatment in selected patients with pulmonary atresia and intact ventricular septum (PA-IVS).