Type IV Laryngeal Cleft: A Therapeutic Challenge

Type IV laryngeal cleft (LC) is a rare congenital malformation often associated with poorer prognosis compared to lower grades LC. The difficulty in managing LC lies in the ventilation prior to cleft repair, intraoperative anesthesia, technical difficulties to repair a longer cleft and higher rates of post-operative complications. We report a case of a premature neonate with type IV LC who underwent early gastrostomy at birth and survived with optimal medical management despite suffering bouts of pneumonia and life-threatening event. After a late cleft repair at 4-month old, she survived for 3 months post-operatively without complication of relapse fistula. Despite our cleft repair, she was unable to be weaned off from ventilator attributed to her poor neurological recovery and subsequently succumbed to death at 7 months. We would like to discuss the management challenges with regards to ventilation, approach of surgery as well as post-operative complication to improve the management of such complicated disease.

Posterior laryngeal clefts

The posterior laryngeal cleft is a rare congenital malformation of the larynx, with an estimated incidence of 1 in 10,000–20,000 children born alive. Despite the apparent obviousness of the pathology, the diagnosis of the posterior cleft of the larynx often causes difficulties, which is associated with a variety of clinical symptoms, primarily due to the varying prevalence of the pathological process. Objective: To analyze the results of treatment of patients with congenital laryngeal cleft. Materials and methods: A retrospective analysis of case histories of children in the department of otolaryngology of the clinic of the St. Petersburg State Pediatric Medical University, from 2003 to 2018, diagnosed with congenital malformation of the larynx, posterior laryngeal cleft, was performed. Results: Normal respiration and nutrition through the natural pathways was achieved in 13 children with type I cleft and in 2 with type IIIa. Three patients with IIIa and IIIb types of clefts could not be rehabilitated due to severe concomitant somatic and neurological pathology, for which they continue treatment with relevant specialists at the present time. Conclusions: Rehabilitation of patients with laryngeal cleft of type IIIa and IIIb, especially in the presence of severe concomitant somatic and neurological pathology, does not always give positive results.