Comorbidity and long-term clinical outcome of laryngotracheal clefts types III and IV: Systematic analysis of new cases.

BACKGROUND: Long segment laryngotracheoesophageal clefts (LTECs) are very rare large-airway malformations. Over the last 40 years mortality rates declined substantially due to improved intensive care and surgical procedures. Nevertheless, long-term morbidity, comorbidity, and clinical outcomes have rarely been assessed systematically. METHODS: In this retrospective case series, the clinical presentation, comorbidities, treatment, and clinical outcomes of all children with long-segment LTEC that were seen at our department in the last 15 years were collected and analyzed systematically. RESULTS: Nine children were diagnosed with long segment LTEC (four children with LTEC type III and five patients with LTEC type IV). All children had additional tracheobronchial, gastrointestinal, or cardiac malformations. Tracheostomy for long-time ventilation and jejunostomy for adequate nutrition was necessary in all cases. During follow-up one child died from multiorgan failure due to sepsis at the age of 43 days. The clinical course of the other eight children (median follow-up time 5.2 years) was stable. Relapses of the cleft, recurrent aspirations, and respiratory tract infections led to repeated hospital admissions. CONCLUSIONS: Long-segment LTECs are consistently associated with additional malformations, which substantially influence long-term morbidity. For optimal management, a multidisciplinary approach is essential.

Multicentric study applying the european laryngological society classification of benign laryngotracheal stenosis in adults treated by tracheal or cricotracheal resection and anastomosis.

OBJECTIVES/HYPOTHESIS: Introduction and widespread use of cricotracheal resection and anastomosis (CTRA) as routine treatment for high-grade benign laryngotracheal stenosis (LTS) led to the need for a new classification system that could accurately predict surgical outcomes by integrating crucial stenosis and patient-related information. In 2015, the European Laryngological Society (ELS) proposed a new classification for benign LTS. We retrospectively tested it in adults treated at three referral centers to assess its reliability in predicting surgical outcomes. STUDY DESIGN: Retrospective cohort study. METHODS: We included 166 adults treated by open tracheal resection and anastomosis (TRA) and CTRA procedures, restaged according to the ELS classification evaluating grade of stenosis (I-IV, Myer-Cotton), number of subsites involved, and presence of systemic comorbidities. We correlated these parameters with decannulation, number of retreatments, and complications. RESULTS: Final decannulation was predicted by a proposed ELS score ≥ IIIb, history of previous treatment, and length of resection (P < .05). Decannulation was achieved in 99% of patients without and in 88% of patients with surgical complications (P < .01). The incidence of surgical complications was related to the proposed ELS score (P < .01); an ELS score < IIIb showed a lower complication rate compared to patients with a ≥ IIIb score (32.8% vs. 57.7%, P < .01). Additional treatment was required in 73 (44%) patients (mean = 2.7 ± 2.2, range = 1-11). ELS score ≥ IIIb, length of resection, and occurrence of surgical complications predicted the number of such treatments (P < .05, P < .05, and P < .001, respectively). CONCLUSIONS: ELS classification of benign LTS is able to accurately predict success in adult TRA/CTRA procedures and may be helpful in choice of therapy and patient counseling. LEVEL OF EVIDENCE: 2b Laryngoscope, 130:1640-1645, 2020.

Implementation of the European Laryngological Society classification for pediatric benign laryngotracheal stenosis: a multicentric study.

PURPOSE: The European Laryngological Society (ELS) has published a revised classification for benign laryngotracheal stenosis (LTS), based on their degree, longitudinal extension, and associated comorbidities. We retrospectively applied this classification to pediatric patients treated in four referral centers to assess its reliability in predicting surgical outcomes. METHODS: We included 191 pediatric LTS patients treated by segmental resection, restaged according to the degree of stenosis (I-IV according to Myer-Cotton grading system), number of subsites involved ("a" to "d" for 1-4 subsites among supraglottis, glottis, subglottis and trachea), and presence of systemic comorbidity ("+" sign). We analyzed the ability of this scoring system in predicting the rates of decannulation and complications, as well as the number of re-treatments. RESULTS: The mean decannulation rate was 88%; a higher rate was observed in patients without comorbidities (95.7% vs. 78.1%, p < 0.001), with two or fewer vs. three or four subsites involved (89% vs. 72%, p < 0.01), and in those with an ELS score of IIIa+ or less vs. patients with IIIb or more (96% vs. 82%, p < 0.001). Surgical complications were not dependent on the degree of stenosis, but rather on the number of affected subsites (p < 0.05), as well as on the presence of associated comorbidities (RR 7.5, p < 0.01). The number of re-treatments was dependent on length of resection (p < 0.05), stage according to the revised ELS classification (p < 0.001), and presence of surgical complications (RR 17, p < 0.001). CONCLUSIONS: The revised ELS classification system is easy to apply in everyday practice and offers a sound contribution in the decision-making process.

Hormone receptors analysis in idiopathic progressive subglottic stenosis.

OBJECTIVE: Idiopathic subglottic stenosis predominantly affects fertile and perimenopausal women. Estrogens and/or progesterone have been proposed as mediators of its pathogenesis by stimulating collagen deposition within the upper airway. We evaluated the presence and expression of estrogen-alpha (ER-α), estrogen-beta (ER-ß), and progesterone receptors (PR) in idiopathic stenotic patients. STUDY DESIGN: A retrospective analysis on 42 surgical specimens from idiopathic stenosis female patients (mean age, 52.4; age range, 31-79) and 28 gender- and age-matched controls. METHODS: Immunoreactivity of ER-α, ER-ß, and PR was calculated as the product of intensity (1 = weak, 2 = moderate, 3 = strong) and positive cell percentage (1-4, for < 10/10-50/50-80/ > 80%). This score was calculated on the stenotic and peristenotic tissues. Influence of menopausal status on hormonal expression and stenotic grade was tested. RESULTS: Stenosis showed ER-α overexpression versus peristenotic tissue and controls (score 6.6 ± 4.4, 0.3 ± 0.5, and 2.2 ± 1.5, respectively; P < 0.001). Overexpression was even more marked for progesterone receptors (score 8.3 ± 3.6, 0.8 ± 0.6, and 1.0 ± 0.7, respectively; P < 0.001). There was no expression of ER-ß in stenosis (score 0), whereas it was normally expressed in peristenotic tissue and controls (score 0.7 ± 0.5 and 0.5 ± 0.5; P < 0.001 vs. stenosis). Expression of ER-α was higher in postmenopausal stenotic patients (P < 0.01). This subgroup included a higher proportion of Cotton-Myer grade III stenosis than in premenopausal subjects (P < 0.001). CONCLUSION: An imbalance between ER-α, ER-ß, and PR is present in idiopathic stenosis patients. The hormonal background may be involved in inappropriate inflammation and increased stenosis susceptibility. Menopausal changes seem to play a role in both stenosis grade and receptor patterns. LEVEL OF EVIDENCE: NA. Laryngoscope, 128:E72-E77, 2018.