CRP7.Congenital Absence of Tracheal Rings - A Video Case Report
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Dr. Angeles graduated with honors from medical school in the Dominican Republic at the Instituto Tecnologico de Santo Domingo after growing up in Leonia, New Jersey. During medical school, she earned a research project award that allowed her to present her work on bioethics in Bogota, Colombia. She also experienced the needs of the DR by volunteering on many medical missions. She remained in the DR working as surgical assistant at a cardiac and neurosurgery renowned center, where she was involved in a number of important research and clinical projects including helping with one of the first heart transplants in the DR. Upon her return to the US, she obtained a master’s degree in neuroscience at the University of Hartford in Connecticut, concentrating on cancer treatments. During this time, she also worked as an adjunct professor for the University of Hartford and Quinnipiac University. Next, she joined Westchester Medical Center in Valhalla, New York where she completed general surgery residency with an excellent clinical exposure, especially to minimally invasive thoracic surgery and participate in numerous research projects. She was the recipient of the Women in Thoracic Surgery Scholarship and the Brigid Scanlan Traveling Mentorship Award that allowed her to visit MD Anderson Cancer Center in Houston, Texas for a clinical rotation which solidified her commitment to becoming a minimally invasive academic thoracic oncologist. Dr. Angeles is now at University of Wisconsin in Madison for cardiothoracic surgery fellowship. She enjoys skiing, rollerblading, yoga, tennis and dancing away with her husband and two children.
Description
A 3-week-old, ex-31-week, 2.5kg baby with tachypnea and stridor since birth underwent a rigid bronchoscopy which demonstrated significant narrowing of the distal trachea. A CT scan revealed severe localized airway narrowing and no other cardiovascular abnormalities. Patient was taken for bronchoscopy evaluation with possible dilation. She was found to have severe dynamic narrowing at the distal trachea 1 cm above the carina. The stenotic airway measuring 5 mm in length, appeared to be compliant and distensible with positive pressure ventilation without evidence of complete tracheal rings. A 1.9 mm telescope on a 2.5 ETT bypassed this region. Overnight, patient had worsening ventilation and high peak airway pressures, she required reintervention. This was noted to be unsustainable and tracheal resection was planned.
The infant was taken for a median sternotomy with initiation of CPB. Using a flexible scope, the proximal and distal margins of the narrowing were marked. The diseased trachea was completely excised and it was noted to have absence of tracheal rings. A small incision was made anteriorly proximally and a mirror image incision was made posteriorly in the distal trachea at the membranous portion. Then, the two segments were anastomosed using a posterior running 7-0 Prolene suture and interrupted single and horizontal mattress anterior sutures. Post repair bronchoscopy showed no evidence of residual stenosis. Patient was extubated successfully on POD1 and transferred to the NICU for continued care of prematurity. Gross and histologic findings were consistent with congenital absence of tracheal rings. Subsequent bronchoscopy evaluations have shown no granulation, anastomotic contraction or narrowing.
Tracheal rings are normally "C-shaped," with cartilage anterior and lateral, and a membranous posterior wall. Absent tracheal rings is an extremely rare intrinsic tracheal defect that presents as severe focal tracheomalacia. The absence of the cartilaginous rings leads to airway collapse during fluctuations of pressure associated with normal ventilation. Patients will present with stridor and airway compromise, and become symptomatic much earlier in life. A resection with a primary spatulated anastomosis using everting sutures can result in an unobstructed airway and may have a low risk of recurrence.
Here we present a video of a unique case of a premature, low-birth weight neonate that underwent a successful resection of a short segment of tracheal stenosis from absence of tracheal rings with primary anastomosis of the unaffected trachea.