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Posterior cleft larynx associated with hamartoma
Insights
This study presents a rare laryngeal hamartoma with a posterior cleft, causing severe airway obstruction and recurrent pneumonia in two children. Endoscopic removal successfully resolved obstruction and pneumonia, though aspiration persisted with rapid fluid intake.
Area of Science:
- Otolaryngology
- Pediatric Surgery
- Medical Genetics
Background:
- Laryngeal hamartomas are rare benign tumors.
- Posterior laryngeal clefts can cause significant respiratory and feeding issues.
- This case report details a unique combination of these anomalies.
Observation:
- Two pediatric patients presented with severe obstructive laryngeal disease.
- Symptoms included airway obstruction, aspiration of liquids, recurrent bronchitis, and pneumonia.
- Associated conditions included polysplenia and Opitz-Frias (G) syndrome.
Findings:
- A rare laryngeal hamartoma associated with an isolated posterior cleft was identified.
- Endoscopic removal of the hamartoma provided immediate relief from obstruction and recurrent pneumonia.
- Persistent aspiration occurred with rapid fluid consumption, indicating residual swallowing dysfunction.
Implications:
- Surgical management of laryngeal hamartomas can effectively alleviate obstructive symptoms.
- The combination of hamartoma and posterior cleft presents a complex pediatric airway challenge.
- Further management strategies may be needed for residual aspiration post-operatively.
Abstract:
Two patients with a thus far unreported unusual lesion of the larynx, a hamartoma in association with an isolated posterior cleft, are presented. Obstruction in association with aspiration of liquids, recurrent bronchitis with pneumonia, and severe obstructive laryngeal disease were cardinal symptoms of this anomaly. One child had 13 pairs of ribs and the other had Opitz-Frias (G) syndrome, a rare genetic disorder. The cases are reviewed in detail, endoscopic findings are described, and the literature and embryology are reviewed. After endoscopic removal of the hamartoma, both children were relieved of the obstruction, thrived, and no longer had recurrent pneumonia. The aspiration of liquid foods continued when fluids were gulped or taken too rapidly.