Airway
Introduction
Long laryngeal clefts (Benjamin-Inglis Grades 3 and 4) are rare congenital anomalies with significant morbidity. At Great Ormond Street Hospital, we repair these using an open approach. We present outcomes of our single-centre series over two decades.
Methods
Retrospective review of patients with Grade 3 or 4 laryngeal clefts repaired 2001–2023. Fisher's exact and Mann-Whitney U tests used as appropriate.
Results
42 patients underwent repair (24 male); 33 Grade 3, 9 Grade 4; median follow-up 47 months (IQR 19–71). Age at operation differed by grade: Grade 3 patients were older (median 204 days), typically presenting with and maintaining a pre-operative tracheostomy, whereas Grade 4 patients presented earlier (median 29 days, p=0.036), usually intubated rather than tracheostomised pre-operatively. Comorbidity burden was high: tracheomalacia (67%), GORD (55%), cardiac anomalies (50%). Half required pre-operative fundoplication for reflux control; 72% of Grade 3 patients (13/18) were decannulated at median 31 months (IQR 19–48), compared with none of the 3 tracheostomy-dependent Grade 4 patients. Oral feeding was achieved in 62% overall. Revision surgery was required in 45% (39% Grade 3 vs 67% Grade 4). Mortality was 7%.
Conclusion
Severe laryngeal clefts carry substantial morbidity but are generally survivable with open repair in selected cases with multidisciplinary decision-making. Grade 3 and 4 patients follow distinct airway trajectories, with Grade 4 disease associated with earlier presentation but poorer decannulation outcomes. GORD is near-universal and despite fundoplication in half, aspiration persists in half post-operatively, highlighting ongoing MDT need.