2024 FSA Podium and Poster Abstracts
P065: PEDIATRIC DIFFICULT AIRWAY IN A PATIENT WITH DESBUQUOIS DYSPLASIA
Madison Rhodes, DO1; Adriana Grossman, MD, MPH, MHA2; Patricia Pozo, BS3; Luis Rodriguez, MD1; 1Nicklaus Children's Hospital; 2UM/Jackson Memorial Hospital; 3University of Miami Miller School of Medicine
Introduction: Patient is a 9 year-old male presenting or scheduled osteotomy as a result of a past medical history of Desbuquois Dysplasia. This condition is an autosomal recessive chondrodysplasia with two subtypes: DBQD1 and DBQD2. Proteomic dysfunction is the result of biallelic variants in CANT1 and XYLT1. Phenotypic traits can include cardiopulmonary defects such as VSD and ASD, but predominantly osteodysmorphias such as growth retardation, joint laxity, osteoarthritis, microretrognathia, bell-shaped shaped thorax, short neck, hypotonia, round flat face, midface hypoplasia and progressive scoliosis.
Methods: This particular patient had many radiographic images showing he was not prone to atlantoaxial instability, the concern remained given the patient’s age. This patient also had further indications of a difficult airway, short broad neck and flat facies, the plan for intubation was to attempt with video laryngoscopy (VDL) rather than direct laryngoscopy (DL) and have the room prepared with a fiberoptic scope and several supraglottic mask airways. Planned size endotracheal tube was a 6.0 with half-size larger and smaller tubes immediately available.
Results: Ventilation was moderately difficult, thus quick video laryngoscopy showed an extremely narrow and anterior larynx but vocal cords were well visualized with a Grade 1 view. The patient’s head and shoulders were held by the attending anesthesiologist to limit flexion of the neck and maintain cervical stabilization. The endotracheal tube was sized down to a 5.0 and the patient was successfully intubated without issue. Throughout the procedure the patient was ventilated with 6 mL/kg of tidal volumes to decrease the risk of barotrauma with his mildly bell-shaped thorax. Osteotomy was performed by the orthopedics team and, though uneventful, took much longer than expected.
Discussion: In case reports involving Desbuquois Dysplasia LMAs are the predominant airway as most cases present with multiple failed intubation attempts with a fiberoptic scope. Due to joint laxity, short neck, and osteoarthritis, extension of the neck for DL is compromised; more advanced techniques are required. Additionally the airway is generally anterior making it difficult to visualize the vocal cords and confirm endotracheal placement. Once the airway is secured the bell-shaped thorax and scoliosis severity are harbingers of difficult ventilation as lung anatomy is unchanged, however the chest cavity cannot accommodate higher volumes. Oxygen and pressure will flow in the direction of least resistance, hyper-expanding one lung causing barotrauma and decreased flow to the other causing mismatch/shunting. The risk of barotrauma is increased in this population. Overall these patients present difficult though manageable obstacles to anesthetic management if the practitioner is given anticipatory guidance.
References:
- Use of CobraPLA for airway management in a neonate with desbuquois syndrome. Case report and anesthetic implications. Szmuk, P. M.D.
- Antenatal Phenotype of Desbuquois Dysplasia. Biji, Ishpreet
- Anesthesia Management in an Adult Patient with Desbuquois Syndrome. Tumer, Murat.
- Anesthetic management in a child with Rolland-Desbuquois type dyssegmental dysplasia