[Congenital laryngotracheo-esophageal cleft: diagnosis and surgical treatment by anterior, translaryngeal approach].

BACKGROUND

Laryngotracheoesophageal cleft is a rare but potentially life-threatening anomaly. Less than 200 cases have been published to date. Both the diagnostic and therapeutic recommendations are discussed controversially in the international literature.

PATIENT

We report on the diagnostic and surgical management of a type III cleft larynx in a one month old male presenting with aspiration, pneumonia, and aphonia. Hypoplasia of the cricoarytenoid muscles was associated to the cleft. Rigid endoscopy was found to be the best tool for the diagnostic exploration of the cleft, whereas flexible endoscopy failed to detect the defect. The cleft was broadly exposed using a modified anterior translaryngeal approach that included a tracheostomy. After debriding the mucosal margins, the defect was closed in two layers, and a t-shaped Montgomery tube was implanted. Two further revisions using the mentioned translaryngeal approach and one endoscopic procedure were necessary to achieve complete and permanent closure of the cleft.

RESULTS

Twenty months after birth the boy is able to swallow thick and liquid food without any problems. Stable scar tissue has grown within the former cleft region. The vocal cords are somewhat thickened but mobile in a reduced range. Both the trachea and the esophagus show quite normal diameters.

CONCLUSION

Considering the fact that the arytenoid cartilages touch or overlap each other a congenital defect within the posterior midline of the larynx can only be diagnosed by rigid endoscopy that spreads the cleft apart. In addition to our positive experiences with traumatic fistulas and stenosis of the juvenile trachea we recommend now the anterior vertical laryngeal incision for the operative management of the congenital type III cleft larynx. This direct open approach provides excellent exposure of all components of the defect without the risk of recurrent laryngeal nerve injury. Subglottic stenosis or impaired stability of the larynx described by other authors did not occur in this case. However, the postoperative period is relatively short and careful follow-up for a period of several years is therefore required.