
Anaesthetic management of a patient with Laurence Moon Biedl syndrome undergoing ostium primum atrial septal defect closure
Author(s) -
Vithal K Dhulkhed,
Akshaya N Shetti,
Pavan Dhulkhed
Publication year - 2013
Publication title -
al-banǧ. maqālāt wa abḥāṯ fī al-taẖdīr wa-al-in’āš
Language(s) - English
Resource type - Journals
ISSN - 0259-1162
DOI - 10.4103/0259-1162.118980
Subject(s) - polydactyly , medicine , genetic disorder , hypoplasia , pediatrics , retinitis pigmentosa , ciliopathy , surgery , anatomy , ophthalmology , genetics , disease , gene , phenotype , biology , retinal
Laurence-Moon-Biedl Syndrome is primarily a rare, recessively inherited genetic disorder. The cardinal features are polydactyly, obesity and retinitis pigmentosa, mental retardation, genital hypoplasia and cardiac anomalies. This spectrum may extend and may also involve renal anomalies. Here, we report a rare case of 15-year-old boy who was overweight and suffering from Laurence Moon Biedl syndrome scheduled for closure of atrial septal defect.