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The CHARGE Association
The Role of Tracheotomy
Gilles Roger, MD;
Marie-Paule Morisseau-Durand, MD;
Thierry Van Den Abbeele, MD;
Richard Nicollas, MD;
Jean-Michel Triglia, MD;
Philippe Narcy, MD;
Veronique Abadie, MD;
Yves Manac'h, MD;
Erea-Noël Garabedian, MD
Arch Otolaryngol Head Neck Surg. 1999;125:33-38.
Objectives To evaluate the need for a tracheotomy and its timing during the evolution of an association of malformations, including coloboma, heart defects, choanal atresia, developmental and growth retardation, genitourinary malformation, and ear anomalies (CHARGE association).
Design Retrospective study from January 1988 through December 1997.
Setting Four academic tertiary care centers.
Patients and Methods Forty-five patients with CHARGE association having at least 3 cardinal malformations (growth retardation excluded) and review of the malformations and respiratory manifestations encountered. All the patients underwent endoscopic exploration on several occasions. We reviewed the nature and the timing of therapeutic interventions performed on the airway.
Results Two patients died (one patient of septicemia, the other of unknown causes). Abnormalities of blood gas levels and/or sleep were found in 30 patients (67%), were responsible for cardiorespiratory arrest in 9 (20%), and required admission to the intensive care unit in 21 (47%). Pharyngolaryngeal anomalies leading to dyspnea (discoordinate pharyngolaryngomalacia, glossoptosis, retrognathia, laryngeal paralysis, cleft, stenosis, and difficult intubation) were found in 26 patients (58%). Tracheobronchial anomalies (esophagotracheal fistula, esophageal atresia, and tracheomalacia) were present in 18 patients (40%). Resection of the aryepiglottic folds was attempted 3 times, but without success. Tracheotomy was necessary in 13 patients (29%) at a median age of 2.4 months (mean duration, 25 months). Among these infants, the posterior nasal choanae were patent in 10 patients at the time of tracheotomy. Gastroesophageal reflux was encountered in 36 patients (80%). Prolonged enteral feeding was necessary in 21 patients (47%), with gastrostomy in 16 (of whom 9 needed a tracheotomy). These feeding difficulties and airway problems were highly correlated.
Conclusions We encountered multiple, complicated airway abnormalities. Resection of aryepiglottic folds was inadequate. Often, a tracheotomy could not be avoided in these patients, regardless of choanal patency. Tracheotomy needs to be performed early to avoid hypoxic events. In some selected patients, ventilation using bilevel positive airway pressure may be an alternative.
From the Ear Nose and Throat Departments, Hôpital d'Enfants Armand Trousseau (Drs Roger and Garabedian), and Hôpital Robert Debré (Drs Van Den Abbeele and Narcy), and the Ear Nose and Throat Department (Drs Morisseau-Durand and Manac'h) and Department of Pediatrics (Dr Abadie), Hôpital Necker Enfants Malades, Paris, France; and the Ear Nose and Throat Department, Hôpital d'Enfants La Timone, Marseille, France (Drs Nicollas and Triglia).
THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES
Charge Association and Infantile Hypertrophic lyloric Stenosis
Al Shamsan et al.
CLIN PEDIATR 2000;39:623-624.
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