ESRA Academy. Silva E. Sep 8, 2016; 138582
Eva Silva
Eva Silva

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Background and Aims:

The Léri-Weill Dyschondrosteosis (LWD) is a skeletal dysplasia characterized by disproportionate short stature with Madelung deformity in the wrist and multiple skeletal deformities. It’s a very rare syndrome, eventhough its prevalence is unknown. There are no indications that a difficult airway must be expected, however, it should be carefully assessed. One of the very common skeletal deformities is scoliosis. Careful intraoperative positioning is difficult but necessary. 


A 38 year-old G2P1, 144cm, 76kg, with LWD was previously evaluated in anaesthesia consultation for anaesthetic technique decision. Considering her medical history, it was decided that she should not be subjected to neuraxial blockade due to the highly probable technical difficulties that the anaesthesiologist would face.
Despite the above, on the delivery day, patient underwent analgesia with epidural technique. She was positioned in the left lateral decubitus and technique was performed using the median approach at the L3-L4 interspace with Tuohy needle, 18G.
A test dose with lidocaíne 2% was conducted. After five minutes 16mg of ropivacaíne 0,2% and 10µg of sufentanil were administered. The catheter was introduced about 4cm in the epidural space. A ropivacaíne 0,2% perfusion of 4 to 6 ml/hr was left until the second stage of labor. 


The epidural technique was performed uneventfully and at the first attempt.
Analgesia lasted about 2 hours and elapsed witthout any complications.


Although performing an epidural technique in a patient with LWD may represent an anaesthetic challenge, regional anesthesia is not contraindicated and can be performed safely.

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