Ho Michelle,  Mercan Ezgi,  Evans Kelly,  Sheller Barbara,  Otjen Jeffrey,  Friedman Seth

Final Pr. ID: Poster #: EDU-067

Robin sequence (RS) is a craniofacial condition characterized by micrognathia, glossoptosis, and upper airway obstruction. The primary clinical concerns for infants and children with RS include feeding difficulties and obstructive sleep apnea. Patients with RS often undergo extensive testing to understand the type and severity of upper airway obstruction including flexible fiberoptic laryngoscopy, polysomnography, and computed tomography (CT) imaging.

Historically, surgical options to alleviate airway compromise have included tongue-lip adhesion, mandibular distraction osteogenesis, and tracheotomy, while less invasive treatments, including positioning and nasopharyngeal airways, have been insufficient. An effective non-surgical option is the orthodontic airway plate (OAP), which was initially developed and studied in Tübingen, Germany, and recently introduced in the United States. An OAP is an intraoral appliance comprised of two main components: a palatal base covering the hard palate and a dorsal spur extending just proximal to the epiglottis, thereby positioning the tongue forward to increase airway patency and improve the obstruction.

The design and manufacturing process of an OAP can be time and resource intensive depending upon the complexity and quality of scans and requires a multidisciplinary team and approach. Spur position and length are critical components to the OAP effectiveness. Small variations can cause discomfort and intolerance of the appliance. During the initial fitting and adjustment period, patients are admitted to the hospital for airway support and monitoring. Adjustments to the spur are guided by endoscopic and clinical examinations.

We present patients at our institution who underwent OAP therapy. We will review characteristic imaging findings of patients with RS. We will also summarize the evolving imaging and design workflow for constructing an OAP using intraoral scanning and dynamic 4D CT images. Our workflow is unique in its integration of intraoral scan with dynamic 4D CT images to help define initial spur position and length. By using patient-specific imaging to optimize spur positioning, a well-fitting OAP appliance can be constructed and facilitate rapid transition to outpatient care. Read More

Authors:  Ho Michelle , Mercan Ezgi , Evans Kelly , Sheller Barbara , Otjen Jeffrey , Friedman Seth

Keywords:  Pierre-Robin, Airway, Dynamic Airway CT

Liu Xiaozhou,  Mehta Akshita,  Pfeifer Cory

Final Pr. ID: Poster #: EDU-076

Poland Syndrome is a classic differential consideration for a unilateral hyperlucent hemithorax on chest radiograph due to pectoralis aplasia or hypoplasia. Additional associations include brachysyndactyly, simian crease, dextrocardia, and liver/biliary abnormalities. Isolated pectoral hypoplasia or aplasia without limb involvement is generally cosmetic and can be corrected with plastic surgery, but Mobius syndrome should be ruled out due to its association. Children with Mobius syndrome can exhibit an expressionless affect, excessive drooling, and/or eye paralysis due to cranial nerve deficiencies. This educational exhibit discusses the pediatric radiologist's role in the imaging approach to these supposedly separate diseases which may represent a continuum of one pathology. Read More

Authors:  Liu Xiaozhou , Mehta Akshita , Pfeifer Cory

Keywords:  Poland, Mobius, Pierre-Robin