Complete Tracheal Rings with Tracheal Stenosis

John Dahl, MD, PhD
MBA Indiana University School of Medicine

Complete tracheal rings are a rare and difficult-to-manage entity described in the pediatric airway literature. Management largely depends on the patient’s severity of symptoms, degree of stenosis, and the affected levels of the trachea. Many different surgical techniques have been described as a form of tracheoplasty.

Our team was consulted on a newborn with a suspected trisomy 21 gene disorder who initially presented in respiratory distress. During intubation, the endotracheal tube was difficult to pass. A bronchoscopy revealed complete tracheal rings with long segment tracheal stenosis. He was also diagnosed with a complete arteriovenous canal defect. The patient was taken by Cardiothoracic surgery for a complete AV canal defect repair and a pericardial patch tracheoplasty. The Pediatric Otolaryngology team endoscopically identified the stenosis intra-operatively. Video documentation presented here was recorded from both an external and intra-luminal perspective.

Endoscopic Treatment of a Tracheal Tumor, A Rare Cause of Chronic Cough

Gabriel Gomez, MD
University of North Carolina - Chapel Hill

The outpatient evaluation of chronic cough in the pediatric population has a broad differential. Often, empiric treatments are attempted early in management. Diagnostic testing such as pulmonary function testing, in-office flexible laryngoscopy, chest radiography, and allergy testing are commonly utilized modalities. Direct laryngoscopy and bronchoscopy in the operating room are utilized in refractory cases. This video demonstrates the case of a 4-year-old with a chronic cough who underwent a nonemergent bronchoscopy, revealing an obstructing right mainstem bronchus mass that was removed with a combination of instrumentation via rigid bronchoscopy.

Extended Partial Cricotracheal Resection with Thyrotracheal Anastomosis in Grade IV Subglottic Stenosis with Posterior Glottic Involvement

Sohit Paul Kanotra, MD
Pediatric Aerodigestive Center, Louisiana State University

Partial Cricotracheal resection (PCTR) refers to the resection of an isolated subglottic stenosis (Grade III or IV) with normal vocal cords. When PCTR is combined with an additional open-airway procedure, it is referred to as extended PCTR. The procedure is reserved for patients presenting with glottis involvement in the form of posterior glottis stenosis.

The surgical video describes the steps of extended PCTR in an 8-year-old child with Grade 4 subglottic stenosis with posterior glottic involvement. The steps include initial exposure of the Laryngotracheal complex, resection of the subglottic stenosis with exposure of the posterior cricoid plate, thinning of the posterior cricoid plate with a drill, posterior cricoid split with placement of the costal cartilage graft, posterior anastomosis, placement of a stent and anterior thyrotracheal anastomosis with a tracheohyoidpexy.

Mandibular Distraction for Micrognathia in a Neonate

Megan Gaffey, MD
University of Arkansas for Medical Sciences

Introduction: Patients with Pierre-Robin Sequence (PRS) suffer from micrognathia, glossoptosis, and upper airway obstruction, which is sometimes associated with cleft palate and feeding issues.  To overcome these symptoms in our full-term male neonate patient with PRS, mandibular distraction osteogenesis was performed. 

Methods: The patient was intubated after airway endoscopy.  A submandibular incision was carried down to the mandible. A distractor was modified to fit the osteotomy site that we marked, and its pin was pulled through an intraarticular incision.   Screws secured the plates, and the osteotomy was performed.  The mandible was distracted 1.8 mm daily for twelve days.

Results: During distraction, the patient worked with speech therapy.  Eventually, he was adequately fed orally. He showed no further glossoptosis or obstruction after distraction was completed. 
Discussion: In our experience, mandibular distraction is a successful way to avoid a surgical airway and promote oral feeding in children with PRS and obstructive symptoms.

Myoepithelial Carcinoma of the Mandible: Reconstruction with Rib Cartilage and Supraclavicular Artery Island Flap

Joseph Park, MD
Children's National Medical Center

Case: A four-year-old female presents with a rapidly growing myoepithelial carcinoma of the anterior mandible. A large segmental mandibulectomy was performed for tumor resection through combined high apron and gingival buccal sulcus incisions. The defect was reconstructed using a supraclavicular artery island flap (SCAIF) for the floor of the mouth and rib cartilage using absorbable reconstruction plates for the mandible. 

Discussion: Myoepithelial carcinoma exhibits aggressive behavior and carries a poor prognosis, requiring wide local excision and adjuvant therapy. Cartilage reconstruction was chosen over fibular free flap reconstruction due to the patient’s age, as a bone graft will not be able to adapt to her growth and potential growth complications from fibular reconstructions. She will require revision reconstruction in the future with a fibular-free flap, which will allow dental implantation for the final reconstruction. The combined cervical and intraoral incisions avoided morbidity and cosmetic defects associated with lip split.

Transoral Excision of Floor of Mouth Teratoid Cyst

Kaalan Johnson, MD
Seattle Children's Hospital, University of Washington

9yo F presents with a 3-month history of an enlarging palpable bilateral floor of mouth mass. The overlying submental skin is full with no erythema or tenderness.
MRI reveals a 4cm x 5cm cystic lesion displacing the midline floor of mouth musculature laterally. Ddx: Plunging Ranula, Epidermoid Cyst, Teratoma.
The family consented to transoral mass excision (possible transcervical) and bilateral sublingual gland excision. 
Intraoperatively, the bilateral floor-of-mouth hockey stick incisions were connected at the midline. 
The sublingual glands were normal but were removed for access to the mass. 
The submandibular ducts and lingual nerves were preserved.
The genioglossus muscles were laterally retracted, and a large cystic mass encompassing the floor of the mouth extending through the mylohyoid musculature was bluntly removed in its entirety. 

Pathology: Benign Developmental Cyst/TERATOID CYST (Congenital germline fusion cyst): thin-walled cyst wall lined focally by hyperkeratotic squamous mucosa with underlying smooth muscle and focal associated sebaceous glands.