International Journal of Pediatric Otorhinolaryngology 79 (2015) 31–35
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Low inter-arytenoid height: A subclassification of type 1 laryngeal cleft diagnosis and management Niall D. Jefferson *, Eldar Carmel, Alan T.L. Cheng Department of paediatric otorhinolaryngology, Westmead childrens hospital, Westmead, Sydney, NSW, Australia
A R T I C L E I N F O
A B S T R A C T
Article history: Received 5 May 2014 Received in revised form 21 October 2014 Accepted 29 October 2014 Available online 4 November 2014
Objective: To report our experience of patients with type 1 laryngeal cleft, (including low inter-arytenoid height) who failed conservative management over a five year period. We describe the diagnostic elements of the history, examination at laryngobronchoscopy and provide a management algorithm including the use of inter-arytenoid submucosal injection of gelfoam as a temporary therapeutic as well as diagnostic tool. Methods: A retrospective case note review over a five year period was undertaken to review all cases of type 1 laryngeal cleft who failed conservative management. Presenting symptoms, diagnostic procedures, surgical interventions and clinical outcomes were reviewed. Results: Seventeen patients were identified. Chronic cough was the most consistent feature in the history (100%). All patients underwent a microlaryngoscopy with binocular microlaryngeal assessment. Six patients (35%) underwent gelfoam injection; four of these went on to a formal repair. The remaining 11 all had a repair performed without injection. The success of surgical repair was 80% (12/15) however in the other three, all had improvement in symptoms. Conclusions: Type 1 laryngeal cleft anomalies may extend beyond that described by Benjamin and Inglis. An appropriate history as well as binocular inspection at the time of laryngoscopy is essential. Injection augmentation offers a safe tool in the assessment and management, and endoscopic surgical repair remains the standard for definitive therapy in those that fail conservative management. Crown Copyright ß 2014 Published by Elsevier Ireland Ltd. All rights reserved.
Keywords: Laryngeal cleft Endoscopic Injection augmentation Repair
1. Introduction First described by Richter in 1792 [1], posterior laryngeal clefts (PLCs), have been extensively described. Multiple classification systems have been developed [2–4] and a variety of management paradigms have been proposed [5,6]. What remains a relatively rare congenital anomaly with a quoted incidence overall of between 1 in 10,000 to 20,000 live births [7], an increasing awareness in particular of the type I cleft (according to the Benjamin Inglis classification [8]) has led to a relative increase in diagnosis [6,7,9]. The diagnosis of a type 1 cleft remains challenging. Described as supraglottic inter-arytenoid clefts above the level of the glottis, the question of ‘‘how low is too low?’’ becomes significant. Bakthavachalam et al. [10] describe the normal inter-arytenoid height as
* Corresponding author. Tel.: +61 403850218. E-mail addresses: [email protected], [email protected] (N.D. Jefferson).
approximately 3 mm, with a height of 0–3 mm indicative of a type I PLC. The presenting symptoms are typically non-specific, including chronic cough, dysphonia, cyanosis, aspiration pneumonia and stridor; consequently the diagnosis can be delayed or missed altogether if either is not suspected or inadequately assessed. Successful conservative management of type I laryngeal clefts with diet modification, anti-reflux medications, optimization of respiratory function and speech therapy has been quoted between 25% and 91% [10,11]. Despite this however, some fail conservative management and require surgical intervention. Patient selection and appropriate timing of surgical repair can be challenging, severe aspiration pneumonia and failure of conservative therapy are further complicated by the need for intensive care management post-operatively following a repair and the availability of hospital beds. In our institution, those cases of type I laryngeal cleft that are obvious based on both the clinical history as well as intra-operative assessment, who then fail conservative management go on to have an endoscopic repair. In those cases where the cleft is higher than
http://dx.doi.org/10.1016/j.ijporl.2014.10.040 0165-5876/Crown Copyright ß 2014 Published by Elsevier Ireland Ltd. All rights reserved.
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N.D. Jefferson et al. / International Journal of Pediatric Otorhinolaryngology 79 (2015) 31–35
3 mm above the glottis and or there is a higher degree of suspicion based on the clinical history, inter-arytenoid injection of gelfoam is used to ‘‘plump’’ the posterior cleft area. If there is significant improvement in the pre-operative symptoms post-operatively, and after the gelfoam has reabsorbed, typically 6 weeks [12], the clinical symptoms begin to recur, the patient is offered a formal endoscopic repair. The aim of this study was to review all cases of type I laryngeal clefts (including those with low inter-arytenoid height and those treated initially with gelfoam injection) who failed conservative treatment over a five year period at a tertiary children’s hospital, and to provide an outline of our diagnostic and management algorithm. 2. Methods This is a retrospective case note review of all cases identified with a type I PLC who failed conservative management and went on to undergo surgical intervention. Ethics review board approval was granted and standard demographic data including age at diagnosis, sex, prematurity, concurrent airway and other general medical problems was collected. In addition, symptoms of cough, stridor, aspiration pneumonias both pre-operatively and post-operatively was included. All cases in the series presented were diagnosed with a type I cleft intra-operatively using suspension microlaryngoscopy and an operating microscope, the patients breathe spontaneously utilizing an insufflation technique general anaesthetic. The posterior inter-arytenoid area is probed under binocular vision with a 2 mm Storz beaded rigid metal sucker as the beaded end aided in determining the height above the level of the glottis. The main outcome measure used to define surgical success was no further aspiration on thin fluids following intervention. This was assessed both through parental and patient questioning as well as in some cases fluoroscopic modified barium swallow conducted by the Speech Pathology department. 3. Results During the five year period, 17 paediatric patients were identified as having a type I PLC that had failed conservative management. The mean age at diagnosis was 41 months (standard deviation 27months) with a range of 12–96 months. There were 10 males and seven females. Chronic cough was the most common presenting symptom seen in all 17 patients (100%), see Table 1. Cough and aspiration with thin fluids was also common 15 (88%). Recurrent aspiration pneumonia was seen in 10 (59%) and stridor seen in six (35%). Six (35%) were premature and in many cases there were a number of co-morbidities (see Table 2). Concurrent airway abnormalities were reviewed and are presented in Table 3. Eleven (65%) underwent a modified barium swallow (MBS) preoperatively to assess the presence and severity of aspiration. Aspiration with thin fluids was seen in one (9%), silent aspiration was seen in two (18%) cases, with the remainder being negative for signs
Table 1 Presenting symptoms of patients with type I PLC. Symptom
Number of patients
%
Chronic cough Aspiration on thin fluids Aspiration pneumonias Stridor
17 15 10 6
100 88 59 35
Table 2 Concurrent co-morbidities. Airway
Number
%
Laryngeal oedema Laryngomalacia Subglottic stenosis Tracheomalacia Bronchomalacia Vocal fold abnormalities Tracheal stenosis
9 4 3 3 2 2 1
53 23 18 18 12 12 6
10 4 3 2 1 1 1 1 1
59 23 18 12 6 6 6 6 6
Co-morbidities Gastro-oesophageal reflux Neurological abnormalities Prematurity Cardiac Asthma Perinatal CMV Chronic lung disease Cystic hygroma Obstructive sleep apnoea
of aspiration on thin fluids. All patients underwent suspension laryngoscopy and binocular bimanual assessment with photodocumentation and diagnosed with either a confirmed type I laryngeal cleft or low inter-arytenoid height (Table 4). Following diagnosis, a course of conservative management and diet modification was undertaken in most cases, however, in a number of patients, diet modification and speech therapy involvement had already been instituted and failed. Two (11.7%) had undergone a fundoplication, six (35%) were either NG or PEG feed dependent and four (23%) were tracheostomy dependent. Six (35%) had a gelfoam injection with suspension laryngoscopy in the posterior cleft region in order to raise the height of the posterior inter-arytenoid cleft. Four (23%) of these patients went on to a formal repair, the other two were not present at the time of submission of this paper. Fifteen (88.2%) underwent a formal surgical repair. Endoscopic repair of a posterior laryngeal cleft has been well described elsewhere [10,13]. Our preference is to use a technique similar to that described by Chien et al. [6]. Post-operatively 14 (82%) were admitted to PICU for monitoring during the initial recovery period. It is the preference of the senior author AC to leave the patients intubated for three days to reduce the possibility of vigorous laryngeal activity in the post-operative period resulting in failure of the repair. Four (23%) had a tracheostomy in-situ and so did not require formal airway monitoring. All intubated patients were extubated three days after repair without complication; all were commenced on soft diet initially and then progressed to thin fluid trial prior to discharge. Success was defined as lack of aspiration on thin fluids and resolution of aspiration pneumonia. This was achieved in 14 (80%) cases. In the three (20%) who continued to aspirate, all had improvement in their symptoms. One went on to have a revision which was successful and another is awaiting revision surgery. The relatively high rate of residual symptoms compared to other Table 3 Patients demonstrating ongoing aspiration on thin fluids. Patient
Co-morbidities
1
Laryngomalacia, tracheomalacia, developmental delay, gastrostomy dependent, tracheostomy dependent and cardiac abnormalities Holoprosencephaly, diabetes insipidis, developmental delay, subglottic stenosis, piriform aperture stenosis and PEG dependent Lymphatic malformation (macrocystic) and tracheal stenosis
2
3
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Table 4 Management algorithm.
Recurrent aspiration pneumonia Cough with thin fluids Stridor
History and physical examination Flexible nasendoscopy
Suspension microlaryngoscopy and binocular assessment and bimanual palpation
Diet modification Speech therapy involvement +/- MBS, PPI
Failure of conservative management
Low-inter-arytenoid height or not diagnostic of cleft but supportive history
Diagnostic of laryngeal cleft – formal endoscopic repair ICU post-op
Endoscopic injection of gelfoam into inter-arytenoid area
Resolution of symptoms and gradual recurrence during follow up
reported series was felt to be partially attributable to the underlying complexity of the patient’s co-morbid state (see Table 3). All patients were followed up at a minimum of three months, mean follow up period of 23 weeks (range 12–96). 4. Discussion Classically, posterior laryngeal clefts, or laryngotracheoesophageal clefts, (LTOC) represent a failure or arrest of fusion of the tracheoesophageal septum which first begins to appear at Carnegie stages 12 and 13, (25–28 days) [14]. Ongoing scepticism exists and numerous theories as to the genesis of LTOC have been proposed [15,16]. An inter-arytenoid (type I) cleft however likely develops
from failure of dorsal fusion of the inter-arytenoid musculature with an intact cricoid [17]. As discussed previously, the differing grading systems have a differing view of a type I laryngeal cleft. The normal inter-arytenoid notch height has been quoted as between 3 and 3.5 mm in young children [18]. The significance of a low inter-arytenoid notch height however is controversial. As noted by Smith et al., not only is the presence, absence or function of the inter-arytenoid muscle important, but so is the implications of a low notch height in the presence of reflux. Whilst classically associated with Opitz–Frias and Pallister– Hall syndrome [19], none of the patients in this series had that diagnosis. Diagnosis of particularly type I laryngeal cleft requires a degree of clinical suspicion. Many cases present at an older age with a
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long-standing diagnosis of asthma or gastro-oesophageal reflux (GOR). Investigations such as MBS and functional endoscopic evaluation of swallow both have limitations but can be helpful in selected cases [20]. In the series presented, only one patient demonstrated functional aspiration on thin fluids during the MBS, two demonstrated silent aspiration prior to the commencement of the study. MBS provides little in the way of detailed laryngeal anatomy [6], only evaluates a few cycles of a swallow [13], and in many cases, the patient only aspirates intermittently thereby resulting in a potentially high false negative rate. That being said, close examination of an MBS may give prognostic information regarding the likely success of surgical repair of the laryngeal cleft. Two of the three patients who continued to aspirate on the preoperative MBS had evidence of silent aspiration, whilst this does not represent a contra-indication to surgical repair, it does suggest a more complex problem, one that surgical repair in isolation may not cure. FEES can be a useful diagnostic tool giving information on both penetration as well as the consistencies responsible, however, it is limited in its application in the paediatric population. Chien et al. describe the age range one to four years as the age least likely to be compliant with an assessment, given that the average age in our cohort is 41 months, child distress, not only compromises the evaluation but also can be very distressing for both the patient, family and staff. As in other series, there was a higher incidence of type I laryngeal clefts in boys than girls [10,21]. The significance of gastro-oesophageal reflux remains a significant component in both the diagnosis as well as the management of the type 1 laryngeal cleft as well as the LIAH patient group. The fact that GOR in the paediatric population is so high with a reported associated incidence of up to 44% [9] raises the importance of appropriate diagnosis and institution of antireflux measures both pre-operatively as well as post-operatively until resolution of symptoms. Intra-operative assessment remains the gold standard, as previously stated, the benchmark remains suspension laryngoscopy, binocular visualization with bimanual palpation. We utilise a Storz 2 mm beaded sucker as this gives useful information as to the height of the inter-arytenoid mucosa. It has been the experience of the senior author that there are a number of features often seen at the time of microlaryngoscopy – these include a narrowed inter-arytenoid distance with a paramedian position of the vocal folds. Inter-arytenoid erythema and tracheal cobblestoning. A prospective study is currently underway evaluating these clinical findings. Once the diagnosis is either suspected based on the clinical history and physical examination or microlaryngoscopy – a period of conservative management is undertaken, typically a minimum of three months, during this time diet modification, thickened feeds and a therapeutic course of a proton pump inhibitor is trialled. As described elsewhere, in many cases this is adequate to manage the symptoms related to the cleft, however, in those cases that fail, endoscopic repair should be considered. As discussed above, a number of patients in our series had already undergone dedicated periods of anti-reflux medications and diet modification prior to microlaryngoscopy and therefore were managed surgically from the outset either by injection or formal repair. Gelfoam [22], sodium carboxycellulose aqueous gel, SCCAG (Radiesse voice Gel)[4] and collagen injection [23] into the laryngeal cleft have been described previously and represent both a therapeutic as well as diagnostic tool in cases of type I clefts. As gelfoam and SCCAG are absorbable, they will resorb over time leading to recrudescence of symptoms, but, this also allows time to confirm the diagnosis as well as plan a definitive repair.
Whilst different surgical approaches exist [24,25], our preference is for an endoscopic repair akin to that described by Waltzman and Bent [26]. Our preference is to intubate at the end of the procedure and for the patient to remain intubated in ICU for three days with extubation in ICU and subsequent transfer to the ward. There is contention in the literature at present as to the need [27] and duration [28] of intubation after repair, this warrants review and is something we are prospectively assessing. Defining surgical success in the context of laryngeal cleft repair is worth discussing; in the case of repair of type I laryngeal clefts, Chien et al. describe success as ‘‘parental report of lack of aspiration with thin liquids and further pulmonary infections after the first three months post-operatively’’. Using these criteria, our series had a success rate of 80%. It is important to note however that in those cases that did not meet that standard of success, all reported an improvement in symptoms compared to the preoperative state. Objective measures such as MBS and FEES have limitations as outlined above, the utility of a quality of life questionnaire in relation to laryngeal clefts may be more beneficial in assessing outcomes in relation to laryngeal cleft repairs – this needs further research. There were no complications related to the surgical management of the patients in this series, either the gelfoam injection or the formal repair. Being a tertiary paediatric institution a large number of the children had very complex medical histories ultimately impacting upon the rate of residual disease. One of the limitations of this study is its retrospective design; previously there was no standardized measure of inter-arytenoid height, nor was there consistent reporting in the history or intraoperatively of the features we now recognize as suggestive of a symptomatic cleft or LIAH. In addition there was not a standardized follow up schedule making time to resolution of aspiration difficult to assess. There was no comparison group to compare long term outcomes with, though due to the small numbers and ethical concerns this would be difficult to achieve. Finally the number of cases is relatively low, although comparable to other case series reported in the literature [4,6,10]. Strengths of this paper include a clearly defined management algorithm, as well as the fact that a consistent approach was taken to both the endoscopic repair as well as the post-operative management. Conforming to a strict criteria of surgical success led to a lower than expected rate of ‘‘success’’ but the presence of consistent improvement in all cases is encouraging. We are currently undertaking a prospective assessment of all confirmed cases, additional elements currently under review include whether an elevated body mass index may be indicative of a cleft in a subpopulation of LIAH and type I clefts.
5. Conclusion Type I laryngeal clefts are an uncommon entity. Diagnosis begins with a heightened degree of suspicion based on clinical grounds including the history and physical examination. Diagnosis is confirmed by binocular assessment at the time of suspension laryngoscopy with bimanual palpation. In cases of low interarytenoid height that do not meet the criteria for a type I laryngeal cleft, inter-arytenoid injection augmentation is a safe diagnostic and therapeutic option, its temporary nature is of benefit in securing the diagnosis as well as allowing time to arrange a definitive repair. Endoscopic repair is a safe and reproducible surgical technique associated with minimal morbidity and a high success rate. Even in cases where complete resolution in aspiration is not achieved, improvement is often seen. Quality of life questionnaires may help in better defining the overall impact of laryngeal clefts rather than other difficult to define criteria.
N.D. Jefferson et al. / International Journal of Pediatric Otorhinolaryngology 79 (2015) 31–35
Financial disclosures The authors have no specific financial interests, relationship and affiliations relevant to the subject of the manuscript to declare. These include employment, consultancies, honoraria, stock ownership, etc. Conflict of interest The authors have no conflict of interest to declare: this includes any financial or personal relationships with other people or organizations that could inappropriately influence the authors’ actions. References [1] C.F. Richter, Dissertation medico de infanticide in artis obstetriciae (thesis for doctor of medicine), Leipzig, 1792. [2] G. Petterson, Inhibited separation of larynx and the upper part of trachea from oesophagus in a newborn: report of a case successfully operated on, Acta. Chir. Scand. 110 (1955) 250–254. [3] K.L. Evans, R. Courtney-Harris, M. Bailey, J.N. Evans, D.S. Parsons, Management of posterior laryngeal and laryngotracheoesophageal clefts, Arch. Otolaryngol. Head Neck Surg. 121 (1995) 1380–1385. [4] M.S. Cohen, L. Zhuang, J.P. Simons, D.H. Chi, R.C. Maguire, D.K. Mehta, Injection laryngoplasty for type I laryngeal cleft in children, Otolaryngol. Head Neck Surg. 144 (5) (2011;) 89–783. [5] S.M. Pezzettigotta, N. Leboulanger, G. Roger, F. Denoyelle, E.N. Garabedian, Laryngeal cleft, Otolaryngol. Clin. N. Am. 41 (2008) 913–933. [6] W. Chien, J. Ashland, K. Haver, S.C. Hardy, P. Curren, C.J. Hartnick, Type I laryngeal cleft: establishing a functional diagnostic and management algorithm, Int. J. Pediatr. Otorhinolaryngol. 70 (2006) 2073–2079. [7] B. Roth, K.G. Rose, G. Benz-Bohm, H. Gunther, Laryngotracheoesophageal cleft: clinical features, diagnosis and therapy, Eur. J. Pediatr. 140 (1983) 41–46. [8] B. Benjamin, A. Inglis, Minor congenital laryngeal clefts: diagnosis and classification, Ann. Otolarynol. Head Neck Surg. 98 (1989) 417–420. [9] K. Watters, J. Russell, Diagnosis and management of type 1 laryngeal cleft, Int. J. Pediatr. Otorhinolaryngol. 67 (6 Jun) (2003) 591–596. [10] S. Bakthalachalam, J.W. Schroeder, L.D. Holinger, Diagnosis and management of type I posterior laryngeal clefts, Ann. Otol. Rhinol. Laryngol. 119 (4) (2010) 239–248.
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[11] H.P. Van der Doef, J.B. Yntema, F.J. van den Hoogen, H.A. Marres, Clinical aspects of type I posterior laryngeal clefts: literature review and a report of 31 patients, Laryngoscope 117 (5) (2007) 859–863. [12] H.P. Jenkins, E.H. Senz, H.W. Owen, R.W. Jampolis, Present status of gelatin sponge for the control of haemorrhage, J. Am. Med. Assoc. 132 (1946) 614–619. [13] R. Rahbar, I. Rouillon, G. Roger, A. Lin, R.C. Nuss, F. Denoyelle, et al., The presentation and management of laryngeal cleft: a 10-year experience, Arch. Otolaryngol. Head Neck Surg. 132 (2006) 1335–1341. [14] G. Mouthgong, L.D. Holinger, Laryngotracheoseophageal clefts, Ann. Otol. Rhinol. Laryngol. 106 (12) (1997) 1002–1011. [15] D. Kluth, G. Steding, W. Seidl, The embryology of foregut malformations, J. Pediatr. Surg. 22 (5) (1987) 389–393. [16] J.M. Merei, J.M. Hutson, Embryogenesis of tracheoesophageal anomalies: a review, Pediatr. Surg. Int. 18 (5–6) (2002) 319–326. [17] E.N. Armitage, Laryngotracheo–oesophageal cleft. A report of three cases, Anaesthesia 39 (1984) 706–713. [18] R.J. Smith, M.B. Neville, N.M. Bauman, Interarytenoid notch height relative to the vocal folds. Pilot study, Ann. Otol. Rhinol. Laryngol. 103 (10 (Oct)) (1994) 753–757. [19] D.C. Tyler, Laryngeal cleft: report of eight patients and review of the literature, Am. J. Med. Genet. 21 (1985) 61–78. [20] H. Kubba, D. Gibson, M. Bailey, B. Hartley, Techniques and outcomes of laryngeal cleft repair: an update to the Great Ormond Street Hospital series, Ann. Otol. Rhinol. Laryngol. 114 (2005) 309–313. [21] J. Andrieu-Guitranacourt, P. Nancy, J. Desnos, S. Bobin, D. Dehesdin, J. Dubin, Diastema or laryngeal or posterior laryngotracheal cleft. Analysis of 16 cases, Chir. Pediatr. 25 (1984) 219–227. [22] C.A. Kennedy, M. Heimbach, F.L. Rimell, Diagnosis and the determination of the clinical significance of type 1A laryngeal clefts by gelfoam injection, Ann. Otol. Rhinol. Laryngol. 109 (11) (2000) 991–995. [23] S. Nakahara, N. Tayama, Y. Tsuchida, Aminor laryngeal cleft (type 1A) diagnosed in infancy, Int. J. Pediatr. Otorhinolaryngol. 32 (2) (1995) 187–191. [24] C.M. Myer, R.T. Cotton, D.K. Holmes, R.K. Jackson, Laryngeal and laryngotracheoesophageal clefts: role of early surgical repair, Ann. Otol. Rhinol. Laryngol. 99 (1990) 98–104. [25] D.S. Parsons, S.E. Stivers, D.R. Giovanetto, S.E. Phillips, Type I posterior laryngeal clefts, Laryngoscope 108 (1998) 403–410. [26] M. Waltzman, J. III. Bent, Repair of type 1-A and 1-B posterior laryngeal clefts. Operative techniques in otolaryngology-head and neck surgery 12 (4): 252–255. [27] P.J. Koltai, D. Morgan, J.N. Evans, Endoscopic repair of supraglottic laryngeal clefts, Arch. Otolaryngol. Head Neck Surg. 117 (3) (1991) 273–278. [28] J.P. Bent, N.M. Bauman, R.J. Smith, Endoscopic repair of type IA laryngeal clefts, Laryngoscope 107 (2) (1997) 282–286.
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Low inter-arytenoid height: A subclassification of type 1 laryngeal cleft diagnosis and management Niall D. Jefferson *, Eldar Carmel, Alan T.L. Cheng Department of paediatric otorhinolaryngology, Westmead childrens hospital, Westmead, Sydney, NSW, Australia
A R T I C L E I N F O
A B S T R A C T
Article history: Received 5 May 2014 Received in revised form 21 October 2014 Accepted 29 October 2014 Available online 4 November 2014
Objective: To report our experience of patients with type 1 laryngeal cleft, (including low inter-arytenoid height) who failed conservative management over a five year period. We describe the diagnostic elements of the history, examination at laryngobronchoscopy and provide a management algorithm including the use of inter-arytenoid submucosal injection of gelfoam as a temporary therapeutic as well as diagnostic tool. Methods: A retrospective case note review over a five year period was undertaken to review all cases of type 1 laryngeal cleft who failed conservative management. Presenting symptoms, diagnostic procedures, surgical interventions and clinical outcomes were reviewed. Results: Seventeen patients were identified. Chronic cough was the most consistent feature in the history (100%). All patients underwent a microlaryngoscopy with binocular microlaryngeal assessment. Six patients (35%) underwent gelfoam injection; four of these went on to a formal repair. The remaining 11 all had a repair performed without injection. The success of surgical repair was 80% (12/15) however in the other three, all had improvement in symptoms. Conclusions: Type 1 laryngeal cleft anomalies may extend beyond that described by Benjamin and Inglis. An appropriate history as well as binocular inspection at the time of laryngoscopy is essential. Injection augmentation offers a safe tool in the assessment and management, and endoscopic surgical repair remains the standard for definitive therapy in those that fail conservative management. Crown Copyright ß 2014 Published by Elsevier Ireland Ltd. All rights reserved.
Keywords: Laryngeal cleft Endoscopic Injection augmentation Repair
1. Introduction First described by Richter in 1792 [1], posterior laryngeal clefts (PLCs), have been extensively described. Multiple classification systems have been developed [2–4] and a variety of management paradigms have been proposed [5,6]. What remains a relatively rare congenital anomaly with a quoted incidence overall of between 1 in 10,000 to 20,000 live births [7], an increasing awareness in particular of the type I cleft (according to the Benjamin Inglis classification [8]) has led to a relative increase in diagnosis [6,7,9]. The diagnosis of a type 1 cleft remains challenging. Described as supraglottic inter-arytenoid clefts above the level of the glottis, the question of ‘‘how low is too low?’’ becomes significant. Bakthavachalam et al. [10] describe the normal inter-arytenoid height as
* Corresponding author. Tel.: +61 403850218. E-mail addresses: [email protected], [email protected] (N.D. Jefferson).
approximately 3 mm, with a height of 0–3 mm indicative of a type I PLC. The presenting symptoms are typically non-specific, including chronic cough, dysphonia, cyanosis, aspiration pneumonia and stridor; consequently the diagnosis can be delayed or missed altogether if either is not suspected or inadequately assessed. Successful conservative management of type I laryngeal clefts with diet modification, anti-reflux medications, optimization of respiratory function and speech therapy has been quoted between 25% and 91% [10,11]. Despite this however, some fail conservative management and require surgical intervention. Patient selection and appropriate timing of surgical repair can be challenging, severe aspiration pneumonia and failure of conservative therapy are further complicated by the need for intensive care management post-operatively following a repair and the availability of hospital beds. In our institution, those cases of type I laryngeal cleft that are obvious based on both the clinical history as well as intra-operative assessment, who then fail conservative management go on to have an endoscopic repair. In those cases where the cleft is higher than
http://dx.doi.org/10.1016/j.ijporl.2014.10.040 0165-5876/Crown Copyright ß 2014 Published by Elsevier Ireland Ltd. All rights reserved.
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N.D. Jefferson et al. / International Journal of Pediatric Otorhinolaryngology 79 (2015) 31–35
3 mm above the glottis and or there is a higher degree of suspicion based on the clinical history, inter-arytenoid injection of gelfoam is used to ‘‘plump’’ the posterior cleft area. If there is significant improvement in the pre-operative symptoms post-operatively, and after the gelfoam has reabsorbed, typically 6 weeks [12], the clinical symptoms begin to recur, the patient is offered a formal endoscopic repair. The aim of this study was to review all cases of type I laryngeal clefts (including those with low inter-arytenoid height and those treated initially with gelfoam injection) who failed conservative treatment over a five year period at a tertiary children’s hospital, and to provide an outline of our diagnostic and management algorithm. 2. Methods This is a retrospective case note review of all cases identified with a type I PLC who failed conservative management and went on to undergo surgical intervention. Ethics review board approval was granted and standard demographic data including age at diagnosis, sex, prematurity, concurrent airway and other general medical problems was collected. In addition, symptoms of cough, stridor, aspiration pneumonias both pre-operatively and post-operatively was included. All cases in the series presented were diagnosed with a type I cleft intra-operatively using suspension microlaryngoscopy and an operating microscope, the patients breathe spontaneously utilizing an insufflation technique general anaesthetic. The posterior inter-arytenoid area is probed under binocular vision with a 2 mm Storz beaded rigid metal sucker as the beaded end aided in determining the height above the level of the glottis. The main outcome measure used to define surgical success was no further aspiration on thin fluids following intervention. This was assessed both through parental and patient questioning as well as in some cases fluoroscopic modified barium swallow conducted by the Speech Pathology department. 3. Results During the five year period, 17 paediatric patients were identified as having a type I PLC that had failed conservative management. The mean age at diagnosis was 41 months (standard deviation 27months) with a range of 12–96 months. There were 10 males and seven females. Chronic cough was the most common presenting symptom seen in all 17 patients (100%), see Table 1. Cough and aspiration with thin fluids was also common 15 (88%). Recurrent aspiration pneumonia was seen in 10 (59%) and stridor seen in six (35%). Six (35%) were premature and in many cases there were a number of co-morbidities (see Table 2). Concurrent airway abnormalities were reviewed and are presented in Table 3. Eleven (65%) underwent a modified barium swallow (MBS) preoperatively to assess the presence and severity of aspiration. Aspiration with thin fluids was seen in one (9%), silent aspiration was seen in two (18%) cases, with the remainder being negative for signs
Table 1 Presenting symptoms of patients with type I PLC. Symptom
Number of patients
%
Chronic cough Aspiration on thin fluids Aspiration pneumonias Stridor
17 15 10 6
100 88 59 35
Table 2 Concurrent co-morbidities. Airway
Number
%
Laryngeal oedema Laryngomalacia Subglottic stenosis Tracheomalacia Bronchomalacia Vocal fold abnormalities Tracheal stenosis
9 4 3 3 2 2 1
53 23 18 18 12 12 6
10 4 3 2 1 1 1 1 1
59 23 18 12 6 6 6 6 6
Co-morbidities Gastro-oesophageal reflux Neurological abnormalities Prematurity Cardiac Asthma Perinatal CMV Chronic lung disease Cystic hygroma Obstructive sleep apnoea
of aspiration on thin fluids. All patients underwent suspension laryngoscopy and binocular bimanual assessment with photodocumentation and diagnosed with either a confirmed type I laryngeal cleft or low inter-arytenoid height (Table 4). Following diagnosis, a course of conservative management and diet modification was undertaken in most cases, however, in a number of patients, diet modification and speech therapy involvement had already been instituted and failed. Two (11.7%) had undergone a fundoplication, six (35%) were either NG or PEG feed dependent and four (23%) were tracheostomy dependent. Six (35%) had a gelfoam injection with suspension laryngoscopy in the posterior cleft region in order to raise the height of the posterior inter-arytenoid cleft. Four (23%) of these patients went on to a formal repair, the other two were not present at the time of submission of this paper. Fifteen (88.2%) underwent a formal surgical repair. Endoscopic repair of a posterior laryngeal cleft has been well described elsewhere [10,13]. Our preference is to use a technique similar to that described by Chien et al. [6]. Post-operatively 14 (82%) were admitted to PICU for monitoring during the initial recovery period. It is the preference of the senior author AC to leave the patients intubated for three days to reduce the possibility of vigorous laryngeal activity in the post-operative period resulting in failure of the repair. Four (23%) had a tracheostomy in-situ and so did not require formal airway monitoring. All intubated patients were extubated three days after repair without complication; all were commenced on soft diet initially and then progressed to thin fluid trial prior to discharge. Success was defined as lack of aspiration on thin fluids and resolution of aspiration pneumonia. This was achieved in 14 (80%) cases. In the three (20%) who continued to aspirate, all had improvement in their symptoms. One went on to have a revision which was successful and another is awaiting revision surgery. The relatively high rate of residual symptoms compared to other Table 3 Patients demonstrating ongoing aspiration on thin fluids. Patient
Co-morbidities
1
Laryngomalacia, tracheomalacia, developmental delay, gastrostomy dependent, tracheostomy dependent and cardiac abnormalities Holoprosencephaly, diabetes insipidis, developmental delay, subglottic stenosis, piriform aperture stenosis and PEG dependent Lymphatic malformation (macrocystic) and tracheal stenosis
2
3
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Table 4 Management algorithm.
Recurrent aspiration pneumonia Cough with thin fluids Stridor
History and physical examination Flexible nasendoscopy
Suspension microlaryngoscopy and binocular assessment and bimanual palpation
Diet modification Speech therapy involvement +/- MBS, PPI
Failure of conservative management
Low-inter-arytenoid height or not diagnostic of cleft but supportive history
Diagnostic of laryngeal cleft – formal endoscopic repair ICU post-op
Endoscopic injection of gelfoam into inter-arytenoid area
Resolution of symptoms and gradual recurrence during follow up
reported series was felt to be partially attributable to the underlying complexity of the patient’s co-morbid state (see Table 3). All patients were followed up at a minimum of three months, mean follow up period of 23 weeks (range 12–96). 4. Discussion Classically, posterior laryngeal clefts, or laryngotracheoesophageal clefts, (LTOC) represent a failure or arrest of fusion of the tracheoesophageal septum which first begins to appear at Carnegie stages 12 and 13, (25–28 days) [14]. Ongoing scepticism exists and numerous theories as to the genesis of LTOC have been proposed [15,16]. An inter-arytenoid (type I) cleft however likely develops
from failure of dorsal fusion of the inter-arytenoid musculature with an intact cricoid [17]. As discussed previously, the differing grading systems have a differing view of a type I laryngeal cleft. The normal inter-arytenoid notch height has been quoted as between 3 and 3.5 mm in young children [18]. The significance of a low inter-arytenoid notch height however is controversial. As noted by Smith et al., not only is the presence, absence or function of the inter-arytenoid muscle important, but so is the implications of a low notch height in the presence of reflux. Whilst classically associated with Opitz–Frias and Pallister– Hall syndrome [19], none of the patients in this series had that diagnosis. Diagnosis of particularly type I laryngeal cleft requires a degree of clinical suspicion. Many cases present at an older age with a
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long-standing diagnosis of asthma or gastro-oesophageal reflux (GOR). Investigations such as MBS and functional endoscopic evaluation of swallow both have limitations but can be helpful in selected cases [20]. In the series presented, only one patient demonstrated functional aspiration on thin fluids during the MBS, two demonstrated silent aspiration prior to the commencement of the study. MBS provides little in the way of detailed laryngeal anatomy [6], only evaluates a few cycles of a swallow [13], and in many cases, the patient only aspirates intermittently thereby resulting in a potentially high false negative rate. That being said, close examination of an MBS may give prognostic information regarding the likely success of surgical repair of the laryngeal cleft. Two of the three patients who continued to aspirate on the preoperative MBS had evidence of silent aspiration, whilst this does not represent a contra-indication to surgical repair, it does suggest a more complex problem, one that surgical repair in isolation may not cure. FEES can be a useful diagnostic tool giving information on both penetration as well as the consistencies responsible, however, it is limited in its application in the paediatric population. Chien et al. describe the age range one to four years as the age least likely to be compliant with an assessment, given that the average age in our cohort is 41 months, child distress, not only compromises the evaluation but also can be very distressing for both the patient, family and staff. As in other series, there was a higher incidence of type I laryngeal clefts in boys than girls [10,21]. The significance of gastro-oesophageal reflux remains a significant component in both the diagnosis as well as the management of the type 1 laryngeal cleft as well as the LIAH patient group. The fact that GOR in the paediatric population is so high with a reported associated incidence of up to 44% [9] raises the importance of appropriate diagnosis and institution of antireflux measures both pre-operatively as well as post-operatively until resolution of symptoms. Intra-operative assessment remains the gold standard, as previously stated, the benchmark remains suspension laryngoscopy, binocular visualization with bimanual palpation. We utilise a Storz 2 mm beaded sucker as this gives useful information as to the height of the inter-arytenoid mucosa. It has been the experience of the senior author that there are a number of features often seen at the time of microlaryngoscopy – these include a narrowed inter-arytenoid distance with a paramedian position of the vocal folds. Inter-arytenoid erythema and tracheal cobblestoning. A prospective study is currently underway evaluating these clinical findings. Once the diagnosis is either suspected based on the clinical history and physical examination or microlaryngoscopy – a period of conservative management is undertaken, typically a minimum of three months, during this time diet modification, thickened feeds and a therapeutic course of a proton pump inhibitor is trialled. As described elsewhere, in many cases this is adequate to manage the symptoms related to the cleft, however, in those cases that fail, endoscopic repair should be considered. As discussed above, a number of patients in our series had already undergone dedicated periods of anti-reflux medications and diet modification prior to microlaryngoscopy and therefore were managed surgically from the outset either by injection or formal repair. Gelfoam [22], sodium carboxycellulose aqueous gel, SCCAG (Radiesse voice Gel)[4] and collagen injection [23] into the laryngeal cleft have been described previously and represent both a therapeutic as well as diagnostic tool in cases of type I clefts. As gelfoam and SCCAG are absorbable, they will resorb over time leading to recrudescence of symptoms, but, this also allows time to confirm the diagnosis as well as plan a definitive repair.
Whilst different surgical approaches exist [24,25], our preference is for an endoscopic repair akin to that described by Waltzman and Bent [26]. Our preference is to intubate at the end of the procedure and for the patient to remain intubated in ICU for three days with extubation in ICU and subsequent transfer to the ward. There is contention in the literature at present as to the need [27] and duration [28] of intubation after repair, this warrants review and is something we are prospectively assessing. Defining surgical success in the context of laryngeal cleft repair is worth discussing; in the case of repair of type I laryngeal clefts, Chien et al. describe success as ‘‘parental report of lack of aspiration with thin liquids and further pulmonary infections after the first three months post-operatively’’. Using these criteria, our series had a success rate of 80%. It is important to note however that in those cases that did not meet that standard of success, all reported an improvement in symptoms compared to the preoperative state. Objective measures such as MBS and FEES have limitations as outlined above, the utility of a quality of life questionnaire in relation to laryngeal clefts may be more beneficial in assessing outcomes in relation to laryngeal cleft repairs – this needs further research. There were no complications related to the surgical management of the patients in this series, either the gelfoam injection or the formal repair. Being a tertiary paediatric institution a large number of the children had very complex medical histories ultimately impacting upon the rate of residual disease. One of the limitations of this study is its retrospective design; previously there was no standardized measure of inter-arytenoid height, nor was there consistent reporting in the history or intraoperatively of the features we now recognize as suggestive of a symptomatic cleft or LIAH. In addition there was not a standardized follow up schedule making time to resolution of aspiration difficult to assess. There was no comparison group to compare long term outcomes with, though due to the small numbers and ethical concerns this would be difficult to achieve. Finally the number of cases is relatively low, although comparable to other case series reported in the literature [4,6,10]. Strengths of this paper include a clearly defined management algorithm, as well as the fact that a consistent approach was taken to both the endoscopic repair as well as the post-operative management. Conforming to a strict criteria of surgical success led to a lower than expected rate of ‘‘success’’ but the presence of consistent improvement in all cases is encouraging. We are currently undertaking a prospective assessment of all confirmed cases, additional elements currently under review include whether an elevated body mass index may be indicative of a cleft in a subpopulation of LIAH and type I clefts.
5. Conclusion Type I laryngeal clefts are an uncommon entity. Diagnosis begins with a heightened degree of suspicion based on clinical grounds including the history and physical examination. Diagnosis is confirmed by binocular assessment at the time of suspension laryngoscopy with bimanual palpation. In cases of low interarytenoid height that do not meet the criteria for a type I laryngeal cleft, inter-arytenoid injection augmentation is a safe diagnostic and therapeutic option, its temporary nature is of benefit in securing the diagnosis as well as allowing time to arrange a definitive repair. Endoscopic repair is a safe and reproducible surgical technique associated with minimal morbidity and a high success rate. Even in cases where complete resolution in aspiration is not achieved, improvement is often seen. Quality of life questionnaires may help in better defining the overall impact of laryngeal clefts rather than other difficult to define criteria.
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Financial disclosures The authors have no specific financial interests, relationship and affiliations relevant to the subject of the manuscript to declare. These include employment, consultancies, honoraria, stock ownership, etc. Conflict of interest The authors have no conflict of interest to declare: this includes any financial or personal relationships with other people or organizations that could inappropriately influence the authors’ actions. References [1] C.F. Richter, Dissertation medico de infanticide in artis obstetriciae (thesis for doctor of medicine), Leipzig, 1792. [2] G. Petterson, Inhibited separation of larynx and the upper part of trachea from oesophagus in a newborn: report of a case successfully operated on, Acta. Chir. Scand. 110 (1955) 250–254. [3] K.L. Evans, R. Courtney-Harris, M. Bailey, J.N. Evans, D.S. Parsons, Management of posterior laryngeal and laryngotracheoesophageal clefts, Arch. Otolaryngol. Head Neck Surg. 121 (1995) 1380–1385. [4] M.S. Cohen, L. Zhuang, J.P. Simons, D.H. Chi, R.C. Maguire, D.K. Mehta, Injection laryngoplasty for type I laryngeal cleft in children, Otolaryngol. Head Neck Surg. 144 (5) (2011;) 89–783. [5] S.M. Pezzettigotta, N. Leboulanger, G. Roger, F. Denoyelle, E.N. Garabedian, Laryngeal cleft, Otolaryngol. Clin. N. Am. 41 (2008) 913–933. [6] W. Chien, J. Ashland, K. Haver, S.C. Hardy, P. Curren, C.J. Hartnick, Type I laryngeal cleft: establishing a functional diagnostic and management algorithm, Int. J. Pediatr. Otorhinolaryngol. 70 (2006) 2073–2079. [7] B. Roth, K.G. Rose, G. Benz-Bohm, H. Gunther, Laryngotracheoesophageal cleft: clinical features, diagnosis and therapy, Eur. J. Pediatr. 140 (1983) 41–46. [8] B. Benjamin, A. Inglis, Minor congenital laryngeal clefts: diagnosis and classification, Ann. Otolarynol. Head Neck Surg. 98 (1989) 417–420. [9] K. Watters, J. Russell, Diagnosis and management of type 1 laryngeal cleft, Int. J. Pediatr. Otorhinolaryngol. 67 (6 Jun) (2003) 591–596. [10] S. Bakthalachalam, J.W. Schroeder, L.D. Holinger, Diagnosis and management of type I posterior laryngeal clefts, Ann. Otol. Rhinol. Laryngol. 119 (4) (2010) 239–248.
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[11] H.P. Van der Doef, J.B. Yntema, F.J. van den Hoogen, H.A. Marres, Clinical aspects of type I posterior laryngeal clefts: literature review and a report of 31 patients, Laryngoscope 117 (5) (2007) 859–863. [12] H.P. Jenkins, E.H. Senz, H.W. Owen, R.W. Jampolis, Present status of gelatin sponge for the control of haemorrhage, J. Am. Med. Assoc. 132 (1946) 614–619. [13] R. Rahbar, I. Rouillon, G. Roger, A. Lin, R.C. Nuss, F. Denoyelle, et al., The presentation and management of laryngeal cleft: a 10-year experience, Arch. Otolaryngol. Head Neck Surg. 132 (2006) 1335–1341. [14] G. Mouthgong, L.D. Holinger, Laryngotracheoseophageal clefts, Ann. Otol. Rhinol. Laryngol. 106 (12) (1997) 1002–1011. [15] D. Kluth, G. Steding, W. Seidl, The embryology of foregut malformations, J. Pediatr. Surg. 22 (5) (1987) 389–393. [16] J.M. Merei, J.M. Hutson, Embryogenesis of tracheoesophageal anomalies: a review, Pediatr. Surg. Int. 18 (5–6) (2002) 319–326. [17] E.N. Armitage, Laryngotracheo–oesophageal cleft. A report of three cases, Anaesthesia 39 (1984) 706–713. [18] R.J. Smith, M.B. Neville, N.M. Bauman, Interarytenoid notch height relative to the vocal folds. Pilot study, Ann. Otol. Rhinol. Laryngol. 103 (10 (Oct)) (1994) 753–757. [19] D.C. Tyler, Laryngeal cleft: report of eight patients and review of the literature, Am. J. Med. Genet. 21 (1985) 61–78. [20] H. Kubba, D. Gibson, M. Bailey, B. Hartley, Techniques and outcomes of laryngeal cleft repair: an update to the Great Ormond Street Hospital series, Ann. Otol. Rhinol. Laryngol. 114 (2005) 309–313. [21] J. Andrieu-Guitranacourt, P. Nancy, J. Desnos, S. Bobin, D. Dehesdin, J. Dubin, Diastema or laryngeal or posterior laryngotracheal cleft. Analysis of 16 cases, Chir. Pediatr. 25 (1984) 219–227. [22] C.A. Kennedy, M. Heimbach, F.L. Rimell, Diagnosis and the determination of the clinical significance of type 1A laryngeal clefts by gelfoam injection, Ann. Otol. Rhinol. Laryngol. 109 (11) (2000) 991–995. [23] S. Nakahara, N. Tayama, Y. Tsuchida, Aminor laryngeal cleft (type 1A) diagnosed in infancy, Int. J. Pediatr. Otorhinolaryngol. 32 (2) (1995) 187–191. [24] C.M. Myer, R.T. Cotton, D.K. Holmes, R.K. Jackson, Laryngeal and laryngotracheoesophageal clefts: role of early surgical repair, Ann. Otol. Rhinol. Laryngol. 99 (1990) 98–104. [25] D.S. Parsons, S.E. Stivers, D.R. Giovanetto, S.E. Phillips, Type I posterior laryngeal clefts, Laryngoscope 108 (1998) 403–410. [26] M. Waltzman, J. III. Bent, Repair of type 1-A and 1-B posterior laryngeal clefts. Operative techniques in otolaryngology-head and neck surgery 12 (4): 252–255. [27] P.J. Koltai, D. Morgan, J.N. Evans, Endoscopic repair of supraglottic laryngeal clefts, Arch. Otolaryngol. Head Neck Surg. 117 (3) (1991) 273–278. [28] J.P. Bent, N.M. Bauman, R.J. Smith, Endoscopic repair of type IA laryngeal clefts, Laryngoscope 107 (2) (1997) 282–286.
