Anaesthetic management of a patient with Laurence Moon Biedl syndrome undergoing ostium primum atrial septal defect closure | Zendy

AkshayaN Shetti | Zendy; VithalK Dhulkhed | Zendy; PavanV Dhulkhed | Zendy

Open Access

Anaesthetic management of a patient with Laurence Moon Biedl syndrome undergoing ostium primum atrial septal defect closure

Author(s) -

AkshayaN Shetti,

VithalK Dhulkhed,

PavanV Dhulkhed

Publication year - 2013

Publication title -

anesthesia essays and researches

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.

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