"; d" "/ ." SURGICAL TREATMENT OF CONGENITAL LARYNGEAL MALFORMATIONS JEAN-YVES SICHEL, MD, RON ELIASHAR, MD, ERIC DANGOOR, MB, MRCGP, DORON HALPERIN, MD Congenital malformations of the larynx are relatively scarce, but may be life threatening. The most common causes include laryngomalacia, vocal cord paralysis, and subglottic stenosis. The last 20 years have seen major advances in the field of surgical correction of such anomalies, also serving to reduce the number of tracheotomies in children and the inherent dangers they pose. Success rates for the most popular surgical procedures have been favorable. These include supraglottoplasty for cases of severe laryngomalcia, where relief of respiratory symptoms has been shown to occur in excess of 80% of cases. Complication rate is low, although postoperative death has been reported. Failure usually occurs in cases with concomitant airway abnormalities, including pharyngomalacia. Vocal cord lateralization for vocal cord paralysis with airway compromise is achieved by means of arytenoidopexy or arytenoidectomy using the lateral approach. Arytenoidectomy can also be performed using laryngofissure or endoscopic laser excision. Subglottic stenosis is the third most common congenital anomaly. Anterior or multiple cricoid splitting with cartilage graft interpositioning is usually performed. Success rates for these procedures have been shown to be around 90%. Congenital malformations of the larynx are relatively scarce, their main complication being upper airway obstruc- tion manifested by stridor, tachypnea, and intercostal or suprasternal recession. The severity of these symptoms may vary from life-threatening neonatal respiratory dis- tress requiring immediate intervention to mild symptoms that can be managed with parental reassurance and follow- up. Laryngomalacia (LM) is the most frequent cause of congenital laryngeal malformations, quoted at around 52%. 1 This estimate is probably higher, because only cases requiring endoscopic assessment and/or treatment were reviewed. The second most common cause is vocal cord paralysis, accounting for about 22% of cases, two thirds of which are unilateral. Subglottic stenosis, which accounts for 10% of cases, is the next most common category. Other etiologies include posterior laryngeal clefts, laryngeal webs or atresias, and abnormalities that are not considered to be "true" malformations such as subglottic hemangiomata or tumors. 1 New surgical techniques have been developed in the last 2 decades, not only in an attempt to treat the underlying abnormality, but also to avoid tracheotomies, which are known to have high mortality rates in young children. Pneumothorax, subcutaneous emphysema, granulation tis- sue formation, and tracheal stenosis may also complicate tracheotomy. Furthermore, the care of tracheotomized children is difficult and many require prolonged hospital- ization because of parental inability to manage care at home. Home care also poses social and family disruption. 2 The drive to develop new surgical techniques is attribut- From the Department of Otolaryngology, Head and Neck Surgery, Hadassah University Hospital Jerusalem, Israel. Address reprint requests to Jean-Yves Sichel, MD, Department of Otolaryngology, Head and Neck Surgery, Hadassah University Hospital Jerusalem, Israel. Copyright © 1999 by W.B. Saunders Company 1043-1810/99/1004-0004510.00/0 able in part to an attempt to overcome these difficulties and complications. This article reviews the surgical treatment of the most common laryngeal anomalies, namely LM, vocal cord paralysis, and subglottic stenosis, with emphasis on indica- tions and results of different surgical techniques. LARYNGOMALACIA Laryngomalacia is the most common congenital laryngeal abnormality. It is usually self-limiting and resolves sponta- neously; however, fatal outcomes have been reported. Severe cases may require intubation or tracheotomy to secure the airway. The first effective surgical treatment for LM was re- ported in 1922, when Iglauer 3 carried out partial epiglottec- tomy. Lane et al, 4 were the first to publish a case of supraglottic trimming (1984). Different names are given today to procedures that are almost identical (in which essentially supraglottic tissue is excised). Terms such as supraglottic trimming, epiglottoplasty, and aryepiglotto- plasty may be confusing. In our opinion, supraglottoplasty as described by Holinger et al 5 seems to be an appropriate term. SUPRAGLOTTOPLASTY: SURGICAL TECHNIQUE Polonovsky et al 6 described a suction test for estimating the amount of tissue to be removed (Fig 1). An aspiration cannula is introduced into the supraglottic inlet to create a negative pressure, thereby reproducing collapse of the area (Fig 1D). The test is repeated after surgical excision to confirm improvement (Fig 1E). The surgery is performed under general anesthesia with orotracheal intubation, jet ventilation, or insuffiation tech- nique (whereby a nasotracheal tube is introduced into the oro/hypopharynx superior to the larynx). The larynx is exposed with the aid of a laryngoscope and an operating OPERATIVE TECHNIQUES IN OTOLARYNGOLOGY--HEAD AND NECK SURGERY, VOL 10, NO 4 (DEC), 1999: PP 259-263 259