///2019 Abstract Details
2019 Abstract Details2019-07-13T07:45:15-05:00

Anesthetic Management of a Parturient with Jarcho-Levin Syndrome Undergoing Cesarean Delivery

Abstract Number: RF3BC-280
Abstract Type: Case Report Case Series

Nathalia Torres Buendia MD1 ; Daria Moaveni MD2; Reine Zbeidy MD3

Jarcho-Levin Syndrome (JLS) is a rare autosomal recessive condition with two subtypes: spondylothoracic and spondylocostal dysostosis. Abnormal number and/or structure of vertebrae and costal arches create a short torso with “crab-like” thorax, short neck and disproportionate dwarfism. Several congenital malformations may coexist in JLS.

This case presents a 30 year old G2P1 woman with JLS who underwent cesarean delivery (CD) at 37 weeks gestation due to a congenital heart defect in the fetus. Her medical history was also significant for repeated pneumonias throughout childhood, asthma and history of difficult intubation in a previous CD. The patient presented with tachypnea, subcostal retractions and did not tolerate lying supine. Her height was 120 cm, weight 49 kg. She had a short neck with limited extension, macroglossia and Mallampati 4 airway. Her thorax was short, protruding and “crab-like” shaped. Her back and spine were distorted. No recent spine imaging was available. Pulmonary function tests showed severe restrictive lung disease. Echocardiogram showed preserved biventricular function with mildly increased right ventricle systolic pressure.

Following a multidisciplinary discussion, which included pulmonology, CD under general anesthesia with awake intubation was planned. Preoperatively, albuterol nebulization followed by 4% lidocaine nebulization and gargles were done. Once in the OR, patient was positioned with blankets to support her kyphosis. Vocal cords were sprayed with 1 ml of 4% lidocaine using a laryngotracheal topicalization anesthesia kit. Sedation with remifentanil infusion was started. Using a video laryngoscope, an endotracheal tube size 6.0 was placed with excellent tolerance by the patient. She was subsequently induced with propofol. Lung protective ventilation strategy was used in the context of restrictive lung disease in pregnancy, parameters were adjusted after delivery due to the change in lung mechanics. Estimated blood loss was 1 L, thus 1 unit of PRBC was administered based on hemodynamics and considering her low body weight. She was monitored postoperatively in ICU and extubated 24 hours later. Postoperative course was uneventful.

Patients with JLS present challenges for management of the airway, positioning, and ventilation.

Traditionally, awake fiberoptic intubation was indicated for potential difficult airway management. This case demonstrates that thorough topicalization of the airway can also allow for awake intubation with video laryngoscopy, which may be easier and more familiar to anesthesia providers. Highly complex cases such as this one should be performed in centers with expertise in high risk obstetric anesthesia care and availability of maternal fetal medicine and pulmonology specialists. The successful outcome of this case is attributed to thorough multidisciplinary planning and management.

References:

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Am. J. Med. Genet. 2004:128A: 120-126

SOAP 2019