Posterior cleft larynx associated with hamartoma.
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.
D000402:Airway Obstruction / Q000209:etiology; D001991:Bronchitis / Q000209:etiology; D002675:Child, Preschool; D004724:Endoscopy; D006222:Hamartoma / Q000150:complications* / Q000235:genetics / Q000601:surgery; D006801:Humans; D007223:Infant; D007822:Laryngeal Neoplasms / Q000150:complications* / Q000235:genetics / Q000601:surgery; D007830:Larynx / Q000002:abnormalities*; D008297:Male; D011015:Pneumonia, Aspiration / Q000209:etiology; D012272:Ribs / Q000002:abnormalities; D013577:Syndrome