International Journal of Pediatric Otorhinolaryngology Extra 10 (2015) 22–24
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Case Report
Combined endoscopic and open approach in treating congenital laryngeal web Kishore Sandu *, Philippe Monnier, K. Lambercy, L. De Trey Department of Otorhinolaryngology, Lausanne University Hospital, Switzerland
A R T I C L E I N F O
A B S T R A C T
Article history: Received 16 November 2014 Received in revised form 15 January 2015 Accepted 19 January 2015
Laryngeal webs are rare and present with varied symptoms depending on the length of the web occupying the glottis. The patients present with voice and airway problems. Advanced webs have a longer glottic length involvement and web-associated cartilaginous subglottic stenosis. Adequate treatment of a laryngeal web must restore an optimal airway and give a near normal quality of voice. Both endoscopic and open procedures are used for the treatment of congenital laryngeal webs, each having their share of advantages and drawbacks. Transoral CO2 laser was used in the initial part of the surgery to cut an advanced glottic web precisely in the midline. Subsequently, the web-associated stenosis was treated by an external cartilage expansion laryngoplasty. By combining the endoscopic and open approaches, we obtained a sharp anterior commissure and also an adequate airway lumen. This assured optimal voice and airway results. ß 2015 Elsevier Ireland Ltd. All rights reserved.
Keyword: Congenital laryngeal webs
1. Introduction Congenital laryngeal web is a rare malformation of the larynx in which there is an abnormal tissue between the two vocal folds near the anterior commissure that could extend to the subglottis. Cohen classified congenital glottic webs into 4 types depending on the extent of the glottic length affected – type 1: web occupying 35% of the glottic length; type 2: 35–50%; type 3: 50–75%; type 4: 75–90% [1]. Patients with limited webbing of the anterior glottis present with vocal symptoms. Longer webs having subglottic extension cause respiratory problems and dysphonia. Several endoscopic and open surgical procedures have been described in the treatment of congenital glottic webs [2,4,5]. The aims in the surgical treatment of a congenital laryngeal web are: 1. To restore an optimal glotto-subglottic geometry and relieve respiratory symptoms. 2. Achieve a sharp-angle anterior commissure that would ensure adequate vocalisation. 3. Allow decannulation if tracheostomy is done in early life. 2. Clinical case An 18-month-old, Moroccan girl was referred to us with a preliminary diagnosis of congenital laryngeal web and was
* Corresponding author. Tel.: +41 213117657. E-mail address:
[email protected] (K. Sandu). http://dx.doi.org/10.1016/j.pedex.2015.01.001 1871-4048/ß 2015 Elsevier Ireland Ltd. All rights reserved.
transported to our institution for treatment by a non-government organisation. In her country, she presented with severe respiratory distress at birth and was immediately intubated using a size 2.0 endotracheal tube. The diagnosis of a laryngeal web was made during the intubation; however no additional details were available. The tube was removed after 3 weeks. Thereafter, she had a prolonged and stormy stay in the intensive care, but was not reintubated. In the following months, she had 4 episodes of upper respiratory tract infection with stridor, which were managed conservatively without performing a tracheostomy. On arrival at our hospital, she was aphonic, weighed 8 kg, in average general condition and had extreme fatigue on exertion. The transnasal fibreoptic laryngoscopy showed semi-obstructive adenoids, good mobility of both vocals cords and a thick band of web occupying 75% of the glottic length (type 4 according to the Cohen’s classification – Fig. 1). A thin 08, 1.9 mm Storz endoscope was passed in the posterior commissure without traumatising the surrounding mucosa to further evaluate the web extension. The web-associated cartilaginous subglottic stenosis extended up to the lower border of cricoid cartilage and the subglottic diameter was reduced by 50%. The trachea and bronchi were normal. Screening fluorescence in situ hybridisation (FISH Test) showed deletion at the chromosome 22q11.2 locus. There were no velopharyngeal and cardiac problems. The endoscopic findings clearly showed a pure congenital web without any sequelae of prior intubation. There were no other associated anomalies. Surgery began with laryngeal mask ventilation and a tracheotomy was done between the 5th and 6th tracheal rings. The larynx was suspended using a Benjamin–Lindholm microlaryngoscope.
K. Sandu et al. / International Journal of Pediatric Otorhinolaryngology Extra 10 (2015) 22–24
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Fig. 1. Pre-operative view of a Cohen type 4 laryngeal web.
Fig. 2. Post-operative view of the Cohen type 4 laryngeal web.
The vocal cords were separated using a vocal fold retractor to maintain tension on the glottic web. Using CO2 laser (Lumenis1) with the AcuBladeTM micromanipulator in UltraPulse chopped mode (4 mm straight-line laser delivery, 100–150 mJ/cm2) the web was incised from the back to front until the anterior commissure. Just 4 laser shots were enough to incise the complete web thickness exactly in the midline. On reaching the anterior commissure the straight-line laser delivery was changed to 250 mm spot, and the incision continued until the inner perichondrium of the thyroid and cricoid cartilages. The micro-spot laser allowed us to be precise and avoid accidental laser strikes while working below the vocal cords in the narrow anterior subglottis. Next, we proceeded with an open complete laryngofissure, which would allow us to manage the web-associated stenosis. Staying in the midline from the first tracheal ring upwards, the cricoid and thyroid cartilages were incised in the caudo-cranial direction. The anterior cricoid split was done meticulously to join the prior endolaryngeal laser incision, and then progressively extended upwards to incise the anterior commissure. The vocal ligaments were identified on both sides and fixed anteriorly to the thyroid cartilage using 6.0 Vicryl. This kept the vocal folds securely attached to the future anterior commissure. The cartilaginous subglottic component of the web was excised submucosally by meticulous dissection and using a fine diamond burr. While closing the laryngofissure, the vocal folds were sutured meticulously using 5.0 Vicryl with the beak of an appropriate size (no. 8) Monnier’s LTMold1 and the thyroid cartilage, so as to achieve a sharp-angle anterior commissure. A costal cartilage graft was harvested and used for an anterior airway expansion starting from just below the new anterior commisure, cricoid and up to the first tracheal ring. The LT-Mold was fixed within the larynx using two 3.0 prolene sutures, and was subsequently removed 4 weeks later during a suspension microlaryngocopy. The check endoscopy done at 1 month post-operative showed a well epithelised glottis with a sharp-angle anterior commissure (Fig. 2). The airway was optimal and the anterior graft seen well integrated in the subglottis (Fig. 3). The child was decannulated at 2 months and returned back to her parent country at 3 months after the first surgery. At discharge, the child fed normally without bronchoaspirations and had started comprehensive vocalisation. The non-government organisation that had organised for the treatment is regularly in contact with the child’s parents and her
treating local doctors. As per their information, presently, at 8 months after the surgery she is breathing well and progressing with appropriate speech and language acquisition. 3. Discussion Congenital glottic webs are rare laryngeal malformations and have varied clinical presentations. Anterior glottic webs can be thin and diaphragmatic or thick due to deposition of mesodermal elements (cartilage, muscle, fat) that could extend inferiorly up to the cricoid cartilage. Longer the glottic length of the web, more severe is the associated cartilaginous subglottic stenosis [3,4]. Depending on the extent of glottic webbing, the patient can present with symptoms varying from minimal voice complaints to extreme respiratory distress. Children can present with dysphonia, multiple episodes of laryngotracheitis (croup), feeding difficulties
Fig. 3. Adequate lumen of the subglottis with a well integrated anterior costal cartilage graft.
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and cyanosis. It is estimated that at least 50% of these patients have chromosome deletion at the 22q11.2 locus that is associated with the velo-cardio-facial, Di George, or Shprintzen syndrome [2,4]. Management of a congenital glottic web is surgical. The objectives of surgery are precise division of the web in the midline up to the anterior commissure, preservation of the vocal ligaments, adequate treatment of the associated subglottic stenosis, and a well mucosalised sharp-angle adhesion free anterior commissure. Only then, can we achieve a satisfactory airway with optimal vocalisation. Blanchard et al. [5] report excellent results in 7 out 8 patients who had an endoscopic anterior cricoid split with cold steel instruments followed by balloon dilation. They claim their technique to be safe and effective for congenital laryngeal stenosis grade II–IV and propose this procedure as an alternative option to open laryngeal expansion surgery, even as a first-line procedure. They conclude that an endoscopic procedure does not preclude the possibility for an open laryngeal procedure in case of failure. Though, in their cases, the endoscopic group had more dilations as against the open expansion procedure group. One of the advantages of balloon over rigid bougies during such dilations is that a deflated balloon is small in diameter and can be passed through a narrow airway thus being less traumatic and easy to use. A congenital laryngeal web can be treated either by an open or endoscopic technique. In a conventional external laryngofissure technique, the web is approached from the outside to inside and its division is done under endoscopic guidance provided by a second surgeon. Sometimes, in very thick webs this could go off-midline causing damage to the vocal ligaments. Meticulous dissection, delicate handling of tissues and precise hemostasis can be time consuming, thus inducing glottic and subglottic oedema. This can make the final re-approximation of the vocal folds difficult, thus failing to achieve a sharp-angle anterior commissure. As against the open procedure, an endoscopic approach is less invasive and provides an excellent axial view of the entire glottis during web division. However, contrary to the external approach, this approach does not allow a submucosal excision of the cartilaginous subglottic component of the stenosis (types 3 and 4) and an anterior cartilage graft expansion – both desirable for an optimal airway. Our technique combines the advantages of both open and endoscopic techniques. During the early part of our surgery, the endoscopic transoral approach offers an axial view of the glottis thereby allowing a precise midline incision of the web. The AcuBladeTM micromanipulator laser delivery system allows precise control for uniform web division and the straight-line laser delivery conforms well to the vocal cord surface anatomy. The surgeon has a user-defined control over the incision length, ablation area and penetration depth. The microvascular hemostasis by the laser enables practically no or very minimal bleeding allowing a clear visual field while cutting through the entire web thickness. Web division by the straight line laser scan is quick and can be achieved only by few laser strikes, thus causing minimal heat dispersion through the larynx. As we approach the perichondrium of the thyroid cartilage, we change the laser line to a microspot that prevents accidental strikes in the narrow ¨ nal commissure and anterior subglottis below the vocal cords. U [6] reported use of CO2 laser in web lysis followed by mitomycin C application with favourable results. In our technique, we use the laser on low settings to incise the web and further cut through the web associated subglottic extension. We do not advocate the use of mitomicin C on young and exposed cartilage fearing its cytotoxic effect on the chondroctes. In the later part of our technique, we perform a complete laryngofissure. While going from the outside to inside, the
laryngofissure is easily joined to the prior endolaryngeal cuts that had already been taken during the transoral laser division of the web. This avoids an off-midline cutting through the anterior commissure and damage to the vocal ligaments. Further, a submucosal excision of the cartilaginous component of the subglottic stenosis and a cartilage graft expansion guaranties an optimal airway. We use the Monnier’s LT-Mold1 to support the reconstruction. The stent is made of soft silicone, corresponds best to the inner laryngeal contours and allows excellent epithelisation of the endolarynx. The beak of the prosthesis sits in the anterior commissure, thus ensuring no vocal fold adhesions and a sharp angle after complete healing. Our patient had a very favourable result – both from the respiration and speech point of view. She had a sharp anterior commissure, optimal glotto-subglottic airway, excellent endolaryngeal epithelisation and finally minimal scar tissue in the anterior commissure and subglottis. The tracheotomy was only transitory to allow the procedure and the child was quickly decannulated as soon as the LT-Mold was endoscopically removed. Commenting on the overall treatment outcome, it would be only fair to make the final judgement at 12–18 months postoperatively. We are limited in our contact with the patient; given the organisational difficulties one faces during treatment of a patient that is motivated by a humanitarian cause. Her follow up is based on periodic telephone checks with her parents and the local doctors which is the only possibility in such a scenario. As per the last telephonic conversation with her treating ENT doctor, the child has made enormous progress as regards the voice quality. In spite of being mildly dysphonic, she is quite comprehensible. This is a great progress compared to her aphonic status pre-operatively. 4. Conclusions Laryngeal webs are rare and present with varied symptoms depending on the length of the web occupying the glottis. Patients can present with voice (types 1 and 2) and airway problems in extreme cases (types 3 and 4). Aim of treatment in these cases is to restore an optimal airway and obtain a near-normal quality of voice. Both endoscopic and open approaches have been proposed for the treatment of congenital laryngeal webs, each having their share of advantages and drawbacks. Our technique combines advantages of the two techniques to achieve best possible results. Conflict of interest None declared. Financial disclosures None. References [1] R. Cohen, Congenital glottic webs in children: a retrospective review of 51 patients, Ann. Otol. Rhinol. Laryngol. 121 (Suppl.) (1985) 2–16. [2] A.T.L. Cheng, E.J. Beckenham, Congenital anterior glottic webs with subglottic stenosis: surgery using perichondrial keels, Int. J. Pediatr. Otorhinolaryngol. 73 (2009) 945–949. [3] M.E. Wyatt, B.E.J. Hartley, Laryngotracheal reconstruction in congenital laryngeal webs and atresias, Otolaryngol. Head Neck Surg. 132 (2005) 232. [4] H.A. Milczuk, J.D. Smith, E.C. Everts, Congenital laryngeal webs: surgical management and clinical embryology, Int. J. Pediatr. Otorhinolaryngol. 52 (2000) 1–9. [5] M. Blanchard, N. Leboulanger, B. Thierry, J.-P. Blancal, F. Glynn, F. Denoyelle, E.N. Garabedian, Management of congenital laryngeal stenosis: external and endoscopic approaches, Laryngoscope 124 (2014) 1013–1018. ¨ nal, The successful management of congenital laryngeal web with endoscopic [6] M. U lysis and topical mitomycin-C, Int. J. Pediatr. Otorhinolaryngol. 68 (2004) 231–235.