Congenital Absence of Tracheal Rings - A Video Case Report
Presented During:
Saturday, May 6, 2023: 5:00PM - Monday, May 8, 2023: 3:45PM
Los Angeles Convention Center
Posted Room Name:
ePoster Area, Exhibit Hall
Abstract No:
6374
Submission Type:
Cardiothoracic Resident Case Report Competition
Authors:
Clara Angeles (1), Petros Anagnostopoulos (2), Joshua Hermsen (3), Malcolm DeCamp (4), Tony Kille (5)
Institutions:
(1) University of Wisconsin, United States, (2) American Family Children's Hospital, Madison, WI, (3) University of Wisconsin, Madison, Madison, WI, (4) UW Hospitals and Clinics, Madison, WI, (5) University of Wisconsin, Madison, WI
Submitting Author:
Clara Angeles
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University of Wisconsin
University of Wisconsin
Co-Author(s):
*Petros Anagnostopoulos
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American Family Children's Hospital
American Family Children's Hospital
Joshua Hermsen
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University of Wisconsin, Madison
University of Wisconsin, Madison
*Malcolm DeCamp
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UW Hospitals and Clinics
UW Hospitals and Clinics
Tony Kille
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University of Wisconsin
University of Wisconsin
Presenting Author:
Clara Angeles
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N/A
Abstract:
Congenital absence of tracheal rings is a rare cause of congenital tracheal stenosis, only reported a few times previously. Here we present a case of a neonate with symptomatic tracheal stenosis, treated successfully with a resection and primary anastomosis.
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.
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.
Category:
Congenital
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