Volume 1, Issue 3 , Pages 204-206, September 2006
Delayed failure of open repair of an anterior glottic web in the neonatal time period
Article Outline
Summary
Laryngeal webs are rare congenital laryngeal malformations, the management of which are fraught with controversy. A case is presented of an unusually large web for which an open repair was attempted at a young age. The literature on this topic is reviewed.
Keywords: Glottic web, Airway obstruction, Tracheostomy, Laryngotracheal stenosis, Neonatal intensive care
1. Introduction
Incomplete recanalization of the laryngotracheal passage during embryonic life can result in the formation of a laryngeal web. These webs constitute a spectrum of developmental laryngeal disorders, and range from a small incomplete anterior glottic web (AGW) causing minimal dysphonia with obstruction to the extreme of a complete glottic web causing total laryngeal atresia. The characteristic presenting symptoms of an AGW are airway obstruction and dysphonia, the relative contributions of which vary with the size and extent of the web. Posterior glottic webs cause limitation of vocal fold abduction without concomitant dysphonia. The most common location for a web is in the anterior glottis [1].
Various treatment strategies have been devised to correct AGWs. These repairs generally involve either endoscopic web division with or without stenting, or open web division with post-operative glottic stenting with a keel [1]. A tracheostomy is always needed while a stent is present. We present a case of a severe congenital AGW in a newborn treated in an open fashion by employing the principles used for anterior cricoid split (ACS) repair of neonatal acquired laryngotracheal stenosis. The procedure was an initial success, but later failed due to web regrowth.
2. Case report
The patient was a healthy male born at term at an outside institution without complication and with APGAR scores of 5 and 10 at 1 and 5
min, respectively. However, a stridor was noted at approximately 1h of age, associated with tracheal tug and intercostal retractions. The stridor was moderate at rest, but exacerbated by distress. At the same time a dysphonic cry was noted. He was transferred to the Royal Children's Hospital Neonatal Unit for further investigations. A flexible endoscopic examination of the larynx performed on day 2 of life revealed a significant AGW obstructing close to 90% of the laryngeal lumen. An echocardiogram and chest X-ray showed normal findings.
The patient was taken to operating theatre on the same day. Under general anesthetic, with the patient in supine position, a laryngoscopy and rigid bronchoscopy was performed. The web was relatively thick and appeared to extend partially into the subglottis (Fig. 1). A 2.7mm Hopkins-Rod 0° telescope could be passed with some difficulty through the airway lumen; a 4mm telescope could not be passed. The trachea and main-stem bronchi were normal. The web's thickness precluded endoscopic division, thus we proceeded to undertake an open repair. The patient was intubated with a tight fitting size #2 endotracheal tube with an outer diameter of 2.9mm. The laryngeal cartilages were exposed and stay sutures were placed. The cricoid cartilage was then split in the midline, at which point the inferior extent of the web was identified. The thyroid cartilage was then divided vertically in the midline with preservation of glottic soft-tissue, and the full extent of the web delineated. Under direct vision the web was divided from below with a scalpel precisely at its midpoint and through the anterior commissure. The #2 endotracheal tube was then exchanged for a #3.5 nasotracheal tube, with an outer diameter of 4.8mm, and the larynx closed over the new tube, which was then used as a stent. The neck wound was closed in standard fashion without a suction drainage tube.
Fig. 1.
The anterior glottic web prior to surgical repair.
The patient remained intubated post-operatively. For the first 5 days he was sedated and paralysed, after which the paralysis ceased and sedation was weaned. Pre-extubation steroids (Dexamethasone 0.2mg/kg/dose) were administered starting on post-operative day 8. An uneventful extubation ensued on post-operative day 10. Immediately after extubation a flexible endoscopic examination of the larynx was performed, which demonstrated normal VC motion, a normal subglottis and only trace blunting of the anterior commissure. A normal sounding cry was heard. The child was observed in the neonatal unit for the following 3 days, after which he was uneventfully discharged home.
At routine follow-up 2 months later the parents gave a history of increasingly noisy breathing and development of an unusual arched back posture at rest. Both these changes had appeared over the preceding 3 weeks, however in this same time period he continued to gain weight normally for his age. An awake flexible endoscopic examination of the larynx demonstrated significant glottic swelling, and the suggestion of web reformation in the anterior glottis. Only a small airway could be seen in the posterior glottis. The likely need for a tracheostomy was raised with the parents, but in light of the fact that the patient continued to gain weight and grow normally, the parents wished to hold off until it became absolutely necessary.
Two weeks later, 10 weeks after the initial surgical repair, the patient presented to the Emergency Department with shortness of breath and increasing stridor. This was precipitated by an upper respiratory tract infection several days prior. A tracheostomy was performed uneventfully later that day. Rigid bronchoscopy demonstrated near complete reformation of the web, with only a 3mm posterior glottic opening as an airway. Distal to the web the subglottis and trachea appeared normal.
The patient was discharged home uneventfully 3 weeks after the tracheostomy. Future management will include a prolonged period of observation followed by a follow-up bronchoscopy at age 2 years, at which point hopefully plans can be made for web treatment and decannulation.
3. Discussion
In the management of a severe congenital AGW in the neonatal time period, we applied the principles of ACS by utilizing a laryngofissure approach with an endotracheal tube as a short-term stent. The open procedure enabled to the patient to be discharged home from the hospital in a minimal amount of time, and to initially remain tracheostomy free. However, despite early short-term success ultimately the repair failed and a tracheostomy was required.
There are no case series in the literature assessing the results of congenital AGW repair in an open laryngotracheal fashion during the neonatal time period. In the setting of an AGW causing airway compromise at birth, many authors promote performing a tracheotomy on the neonate to relieve the airway obstruction, and then waiting until early childhood before dividing the web, thus allowing for laryngeal growth and theoretically facilitating corrective surgery [1], [2]. However, the children in these cases are thus tracheostomy dependant until definitive surgery is undertaken to remove the web. In order to avoid the need for tracheostomy, some authors thus advocate staged endoscopic divisions of the web with either steel or laser, while others are proponents of single-stage open laryngotracheal surgery [1], [2], [3]. Wyatt and Hartley in 2005 published a series assessing their experience in open laryngotracheal surgery for infants and children with subglottic stenosis; within their series 15 patients also had a congenital AGW that was repaired simultaneously using either anterior or combined anterior–posterior cartilage grafts [2]. Note was made that 12 of the patients required staged surgical procedures to achieve decannulation, and in all cases a Silastic stent was left in situ for 6–8 weeks. However, the median age for web repair in their series was 23 months, far beyond the neonatal age of our patient. Milczuk et al. described in 2000 a small series of five patients with congenital laryngeal webs that required open laryngotracheal repair [3]. However, in these cases the repair was again delayed until later in the first quadrennium, and thus the results are not precisely comparable with ours. Considering that the bulk of our patient's pathology was in the glottic region, we did not feel that a cartilaginous expansion of the subglottis would have proven beneficial.
The most consistent problem facing laryngeal surgeons is that of persistant postoperative webbing at the anterior commissure due to contact adhesion of closely apposed raw surfaces. Stent placement is therefore always essential to keep the cut web surfaces apart [1]. Removable Teflon keels are the most commonly used stents, although other types of stents have been developed for use in specific situations [4], [5]. However, a short-term tracheostomy is still needed to maintain airway patency for the patient while the stent is in place, and an eventual second anesthetic is mandatory in order to remove the stent. With the keel and tracheostomy being complicating factors in AGW treatment, alternate therapeutic modalities have been discussed in the literature. Unal in 2004 described the use mitomycin-C in a 10-month-old patient [6], and there have been several reports on the use of buccal mucosal grafts in adult patients [7], [8]. In both instances promising results have been obtained, but it is debatable as to whether these protocols could be extrapolated to neonatal patients with congenital AGWs. Within the pediatric population some authors have advocated local intralaryngeal flaps to prevent postoperative adhesions; however no large series or long-term results exist to assess the efficacy of this procedure [1].
In conclusion, we attempted definitive repair of a congenital severe AGW in the neonatal period. Despite adequate web division and placement of a stent, ultimately our procedure failed in its goal of complete web obliteration. This case was instructive to us, and we advocate that in neonates with large anterior glottic webs causing airway obstruction at birth, priority be given to relief of the obstruction, with definitive repair deferred until a later date. It is possible that a prolonged period of stenting, on the order of 6–8 weeks, may have resulted in a more positive outcome.
Acknowledgements
Research by the senior author is supported by the Bertalli Otolaryngology Research Centre and the Murdoch Children's Research Institute.
References
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PII: S1871-4048(06)00059-1
doi:10.1016/j.pedex.2006.05.005
© 2006 Elsevier Ireland Ltd. All rights reserved.
Volume 1, Issue 3 , Pages 204-206, September 2006
