Congenital absence of tracheal rings: A video case report

Central MessageWe present a case of neonate with functional tracheal stenosis due to congenital absence rings who was treated successfully short-segment resection and anastomosis.See Commentary on page XXX. We anastomosis. See Congenital (CTS) is potentially life-threatening disorder that leads early life severe airway compromise. Common causes include complete or presence vascular ring secondary tracheobronchomalacia.1Hofferberth S.C. Watters K. Rahbar R. Fynn-Thompson F. Management stenosis.Pediatrics. 2015; 136: e660-e669Crossref PubMed Scopus (55) Google Scholar an exceedingly rare cause CTS2Seymour F.K. Roebuck D.J. McLaren C.A. Bailey C.M. segmental rings.Int J Pediatr Otorhinolaryngol. 2006; 1: 45-49Crossref (9) Scholar,3Smith M.M. Kou Y.F. Schweiger C. Lehenbauer D.G. de Alarcon A. Rutter M.J. bronchial rings.Otolaryngol Head Neck Surg. 2021; 164: 422-426Crossref (4) presents as focal tracheomalacia.2Seymour Here we (Video 1). The patient(s) parents provided informed written consent for the publication study data; institutional review board approval not required. A 3-week-old, ex-31-week premature female, weighing 2.5 kg, transferred from outside facility tachypnea stridor since birth. rigid bronchoscopy had demonstrated significant narrowing distal trachea. computed tomography scan revealed localized confirmed no other cardiovascular abnormalities (Figure 1).4Children’s Hospital Philadelphia, Center Pediatric Airway Disorders.https://www.chop.edu/treatments/bronchoscopyDate accessed: August 2, 2023Google patient taken evaluation. She found have dynamic at trachea 1 cm above carina. stenotic airway, measuring 5 mm in length, appeared be compliant distensible positive pressure ventilation without evidence rings. 1.9-mm telescope 2.5-mm endotracheal tube bypassed this region. Overnight, child decompensated requiring reintervention. tip moved proximally longer stenting open narrowed area. This noted unsustainable planned. After median sternotomy, cardiopulmonary bypass initiated. Using bronchoscopy, proximal margins were marked. diseased completely excised it small incision made anterior mirror image posteriorly membranous portion E1). 2 segments anastomosed using posterior running 7-0 Prolene suture E2) interrupted horizontal mattress everting sutures E3). Postrepair showed residual stenosis. extubated postoperative day neonatal intensive care unit continued prematurity. Gross histologic findings consistent Figure shows comparison normal cartilaginous pathologic finding specimen foci cartilage embedded respiratory epithelium subepithelial fibrosis. underwent subsequent bronchoscopies granulation formation anastomotic narrowing. discharged home stable conditions 52, equivalent 41-week gestation. Six-month follow-up healing 3).Figure 3Six-month bronchoscopy.View Large Image ViewerDownload Hi-res Download (PPT) Absent extremely intrinsic defect.3Smith collapse during fluctuations associated ventilation, coughing, crying. Patients will stridor, difficulty breathing, compromise become symptomatic much earlier compared patients defects. Smith colleagues3Smith presented 4 All repaired slide tracheoplasty carinal reconstruction; none required reinterventions. elected perform primary spatulated anastomosis described by Hobbs colleagues.5Hobbs R.D. Moon J. Murala Ohye R.G. Novel technique treatment long-segment stenosis.Semin Thorac Cardiovasc 2020; 32: 930-934Abstract Full Text PDF (8) segment abnormal must resected. performed nonabsorbable (Prolene) portion, anteriorly absorbable (PDS; Ethicon) used facilitate mucosal apposition. can result unobstructed may lower risk recurrence than standard technique. It avoids figure-of-8 deformity decrease incidence complications reinterventions tissue contraction5Hobbs E4). Known reported reconstruction children reintubation, failed extubation tracheostomy, ballooning stenting, well perioperative mortality.6Stephens E.H. Eltayeb O. Mongé M.C. Forbess J.M. Rastatter J.C. Rigsby C.K. et al.Pediatric surgery: 25-year resection.Ann 109: 148-153Abstract (23) demonstrates complexity these need improvement surgical techniques management tracheobronchial tree. unique highlights importance keeping mind different neonates. Absence recognized dyspnea life. Some critical concepts learned are ringless such Additionally, use applied period well. premature, low-birth weight successful short unaffected eyJraWQiOiI4ZjUxYWNhY2IzYjhiNjNlNzFlYmIzYWFmYTU5NmZmYyIsImFsZyI6IlJTMjU2In0.eyJzdWIiOiJhMWY3YjgwYzJmOTNmMGQ3NzQ4Yzk4NWQyNjQzNDI2NSIsImtpZCI6IjhmNTFhY2FjYjNiOGI2M2U3MWViYjNhYWZhNTk2ZmZjIiwiZXhwIjoxNjk0OTU1OTQwfQ.kS58LD5EbQ7jaSyrb3wq2enodmwTHQk-jzNq3TZhXgctq82DLXPAN7csoBSYPZvEaoBajpROQlSK0SAEq3BfwySyLuIS3o4Lez_jA-4u-nBYIQ_fbuO2xPnK0bt9vYSQFXC7371c2OoVWfFNIhPGodRvP5HLjjWUARIHncwsMb5qYdccqduYGQcopZUV4IyeUiBUCSFXmHlREG8k63SzR6RyVi41ucOh0htZIdS4OqAm3RIDQW6KX_n7n5NIqDKgeZCLOYQPXQsdaWWfb8IylRzL9VUgFyv318SLjdJRhcVYDAxNQXCPNHZ-9tMxp5K4EncqewV74GIZ7zWMxzheQQ .mp4 (64.42 MB) Help files Video 1Congenital available at: https://www.jtcvs.org/article/S2666-2507(23)00226-2/fulltext. .jpg (.16 E2The elements 7–0 technique.Illustration Margaret Greco, MD.View (PPT)Figure E3The locked either end sutures. Three additional PDS (Ethicon) completed connection anteriorly.Illustration E4Final anastomosis.Illustration