RESUMO
The Antley-Bixler syndrome is a rare multiple congenital anomaly with a high mortality rate. The characteristic manifestations include craniosynostosis, radiohumeral synostosis, midface hypoplasia, joint contractures and arachnodactyly. We report two new cases of this syndrome and address the diagnostic features, associated malformations, inheritance patterns, prenatal findings, and briefly review the literature.
Assuntos
Anormalidades Múltiplas/genética , Contratura/genética , Craniossinostoses/genética , Humanos , Lactente , Masculino , Síndrome de Marfan/genética , Síndrome , Sinostose/genéticaRESUMO
Out of 321 consecutive cases of Percutaneous Transluminal Coronary Angioplasty (PTCA), 28 (8.7%) patients underwent PTCA for nonacute total occlusion with no visible antegrade flow. All patients had evidence of reversible ischaemia in the territory of totally occluded artery. Overall initial success rate was 66% and was mainly related to the duration of the occlusion (85% for occlusion of 1 month or less, 70% for 1-6 months, and nil for more than 6 months duration). In 21 (75%) cases where collateral circulation to the occluded vessel could be demonstrated before PTCA, was not visible after successful PTCA. Failure was mainly due to inability to cross the lesion with the guide wire (8 out of 10 failures). Emergency Coronary Artery Bypass Graft (CABG) surgery was required in 2 cases where dissection of nontarget vessels occurred during manipulation of the guide wire. Acute reocclusion occurred in 2 cases, both were redilated successfully. There was no death. Although the primary success rate is lower than that associated with conventional stenotic lesions, with improvement in the hardware-balloon catheters and guide wires, coronary angioplasty can be performed successfully in majority of the patients with total coronary occlusion.