Abstract

AET-28

Severe Tracheal Abnormality in Morquio (MPS IVA) Patients Complicating Anesthetic Management

¹DiCindio S, ¹Theroux M, ¹Pizzaro C, ¹Mackenzie W, ¹Bober M, ¹Tomatsu S, ¹Brown M, ¹Barth P, ¹Schmidt R, ¹Tetsu U, ¹Averill L, ²Passi V
¹Nemours Alfred I. duPont Hospital for Children, Wilmington, DE, USA; ²AI duPont Hospital for Children, Wilmington, DE, USA

Introduction: Severe tracheal abnormalities have been reported in mucopolysaccharidosis (MPS) IVA patients. (1,2) MRI images of the cervical spine report 67.9% of patients have at least 25% tracheal narrowing, which worsens with age. (1) Tracheal obstruction, along with difficulties in managing the upper airway, of MPS IVA patients can lead to life-threatening complications during anesthesia. (3) We present the results of MPS IVA patients who had CT/Angiogram (CTA) and report on tracheal abnormalities with a plan to assess and manage the airway.
Methods: IRB approval and patient/parental consent was obtained. History and physical was performed with attention to the need to keep the head and neck extended to improve airway patency, change in voice quality or difficulty in speaking due to the need to pause to inspire. Intraoperative airway management, CTA and pulmonary function (PFT) data were also collected.
Results: Twenty-seven patients (mean age 18 years, range 5-36 years) with MPS IVA had CTA studies. Twenty-six of the patients had tracheal narrowing, buckling, and twisting. (Figure 1) Narrowing was recognized as early as age five. Information regarding airway management was available for 21 patients. (Table 1) Of the 15 patients requiring a Glidescope intubation, five were difficult to mask ventilate and seven required several attempts with different blades. Six patients had their trachea resected and anastomosed and are clinically functioning better than prior to their surgery. Relocation of the innominate artery was necessary in 5/6 due to the crowded thoracic inlet and compression of the trachea by the innominate artery. Two of the six patients who had a tracheal resection were difficult to mask ventilate and intubate. PFT studies are not diagnostic for severity of obstruction. Patient’s symptoms have been the best indicator to proceed to surgery.
Discussion: MPS IVA patients commonly have tracheal narrowing, tortuosity and buckling, which significantly impact anesthetic management. CTA, which requires no sedation, is the imaging study of choice to quantitate tracheal abnormality. Patient’s symptoms are the primary criteria for determining timing of tracheal and vascular reconstruction to treat the stenosis as well as compression caused by the innominate artery.
References:
1)Tomatsu 2016
2)Pizzaro 2016
3)Theroux 2012