The incidence of Marfan syndrome (MFS) is as high as 2-3every 1000 newborns. As the diagnosis of MFS is sometimeschallenging and not infrequently delayed, only an accuratepreoperative assessment may increase the index of suspicion.Therefore, when the anesthesiologist, preoperatively, encoun-ters a tall young patient with an abnormal length of the ex-tremities relative to the trunk, heart murmur and history ofspontaneously detached retina or lens dislocation, the clinicalsuspicion of Marfan misdiagnosis should arise. In such pa-tients a more accurate preoperative screening with a detailedanesthesiological and surgical workup is mandatory. Fre-quently undiagnosed, patients may be difficult to approachfrom the anesthetist's perspective mainly due to skeletal de-formities, aortic vascular disease and heart valve associateddisorders. Referral to a highly experienced center for MFS isstrongly suggested also for minor procedures to avoid major complications
Monaco, F., De Luca, M., Sala, A. (2015). Anesthesia in Marfan patients. ITALIAN JOURNAL OF VASCULAR AND ENDOVASCULAR SURGERY, 22(suppl. 3 to No.3), 37-41.
Anesthesia in Marfan patients
MONACO F;
2015
Abstract
The incidence of Marfan syndrome (MFS) is as high as 2-3every 1000 newborns. As the diagnosis of MFS is sometimeschallenging and not infrequently delayed, only an accuratepreoperative assessment may increase the index of suspicion.Therefore, when the anesthesiologist, preoperatively, encoun-ters a tall young patient with an abnormal length of the ex-tremities relative to the trunk, heart murmur and history ofspontaneously detached retina or lens dislocation, the clinicalsuspicion of Marfan misdiagnosis should arise. In such pa-tients a more accurate preoperative screening with a detailedanesthesiological and surgical workup is mandatory. Fre-quently undiagnosed, patients may be difficult to approachfrom the anesthetist's perspective mainly due to skeletal de-formities, aortic vascular disease and heart valve associateddisorders. Referral to a highly experienced center for MFS isstrongly suggested also for minor procedures to avoid major complicationsI documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.



