Urgent Airway Management and Postoperative Complications in a Patient with Trichorhinophalangeal Syndrome

Abstract

Trichorhinophalangeal syndrome (TRPS) is a genetic disorder that may pose anesthetic challenges. We present a case of airway management for urgent surgery in a 56-year-old female with TRPS and difficult airway (macroglossia, narrow glottic opening, and hypoplastic epiglottis). Intubation was successful with video laryngoscopy using a size 2.5 pediatric blade and size 5.0 endotracheal tube. During emergence, she experienced bronchospasm and persistent urosepsis, necessitating intensive care unit (ICU) admission. Her pulmonary reserve was hindered by a Morgagni hernia causing lung compression. Our case demonstrates challenges in TRPS including challenging airway, decreased pulmonary reserve, and joint laxity introducing potential for spinal cord injury.

Figure 1

Axial (a) and coronal (b) views of CT (computed tomography) thorax, abdomen, and pelvis which revealed 6 mm left obstructing ureterolithiasis with hydronephrosis, as well as incidentally noted large 13 cm heterogeneous mass within the pelvis resulting in distortion of local structures. Additionally visualized was a large Morgagni hernia containing a large majority of the small bowel, the transverse colon, a portion of the descending colon, and a portion of the stomach with significant adjacent atelectasis of right lower lung lobe.

Figure 2

Chest X-ray revealing large right Morgagni hernia containing fat and several loops of small bowel. Associated right lung volume loss and atelectasis.