Join now to get access to this content and more.
Become a SOAP member and have access to our benefits.
- 2020 SOAP Virtual Meeting Series Videos
- For Review: SOAP Consensus Statement on Neuraxial Procedures in Thrombocytopenic Parturients
- Sample Centers of Excellence Applications
- ASA Corner
- SOAP Policy and Procedure Manual (P&P Manual)
- SOAP Expert Opinions
- SOAP's Learning Modules
- 2019 Annual Meeting Lecture Videos
- December 2018 - SOAP Unofficial Guide to ASA Committees Webinar
- Submit a Position
- View Job Postings
- Previous Meeting Archives
- Previous Meeting Abstract Search
- CMS Guidelines
- Member Benefits
- Newsletter Clinical Articles
- ACOG Documents
- Search our Patient Safety Archive
- Ask SOAP a Question
- Global Health Opportunities
- And more…
Anesthetic Management of a Parturient with Jarcho-Levin Syndrome Undergoing Cesarean Delivery
Abstract Number: RF3BC-280
Abstract Type: Case Report Case Series
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.
PRHSJ 2004; 23(1):65-67
Am. J. Med. Genet. 2004:128A: 120-126