Fig. 8.1
Anterior view of the laryngeal cartilages showing difference between mature and immature larynx
Fig. 8.2
Posterior view of the laryngeal cartilages showing difference between mature and immature larynx
Fig. 8.3
Endoscopic view of the larynx showing difference between mature and immature larynx that presents a more anterior insertion of the vocal process
8.3 Vocal Fold Paralysis
Vocal fold paralysis (VFP) is defined as the absence or reduction of motion of the true vocal fold secondary to impairment of the recurrent laryngeal nerve, fixation of the cricoarytenoid joint, or scarring of the vocal fold itself. It can be unilateral (UVFP) or bilateral (BVFP), and they are distinct clinical entities that can have different presenting symptoms and causes and require different treatment techniques [10].
VFP represents 10–20 % of all congenital laryngeal abnormalities, second only to laryngomalacia [11–13], with bilateral representing 30–60 % [14]. VFP is an infrequent diagnosis, about 5 % of patients seen in a tertiary care voice clinic in a children’s hospital [15].
8.3.1 Presentation
Symptoms of VFP include stridor, dyspnea, apnea, feeding difficulties or dysphagia, aspiration, cyanosis, hoarseness, and dysphonia. Children with bilateral VFP generally present early symptoms because they often have significant airway compromise, with stridor which is the most common presenting symptom, present in almost all children with bilateral paralysis and approximately 75 % with unilateral VFP [11]. Up to 50 % of children with bilateral VFP require a tracheotomy to stabilize the airway [12].
History and physical examination should first determine the stability of the airway; after that the symptoms in relation with birth, intubation, surgery, recent illness, or medical therapy can be explored [13].
8.3.2 Etiology
The cause of VFP in children differs from that in adults. The leading causes of unilateral VFP are iatrogenic; cardiothoracic surgery is the most common, with patent ductus arteriosus ligation (PDA) accounting for the majority of identified cases [11]. As would be expected, the majority of these paralyses are left sided; it may be explained by the fact that the left recurrent laryngeal nerve presents a longer path [16]. Other pediatric surgical procedures involved with VFP are the tracheoesophageal fistula or esophageal atresia repair with incidence estimated at 12 % [17]. Transcervical excision of branchial anomalies may also place the recurrent laryngeal nerve (RLN) at risk [18]. Mediastinal surgery, neck surgery, and thyroidectomy are other surgical procedures which may be responsible of RLN injury.
Congenital left VFP that occurs in association with thoracic, vascular, or cardiac anomalies is known as cardiovocal syndrome [19].
Neonates who suffer cervical trauma in a complicated breech or vertex delivery with or without forceps may present VFP [11–13].
Other causes include intubation trauma, inflammatory causes, prolonged esophageal foreign body, and infections as Epstein–Barr virus, varicella-zoster virus, polio, and Lyme disease [13, 20–22].
Neurologic diseases are the other important cause of pediatric bilateral VFP. The Arnold–Chiari malformation is the most classic central nervous system phenomenon associated with bilateral VFP. The herniating contents of the posterior fossa exhibit direct pressure on the vagus nerve as it exits the skull bas, or indirect pressure via secondary hydrocephalus [14]. Other central neurologic causes include severe hypoxia, corpus callosum agenesis, congenital hydrocephalus, and neurofibromatosis. In literature 2 cases of UVFP in two children with presumed peripheral neurologic disease as hereditary distal spinal muscular degeneration and Horner syndrome are described [11].
Vincristine chemotherapy in children may be related to RLN neuropathy and UVFP subsequent to treatment [23, 24]. The majority of reported cases of VFP after chemotherapy administration have been bilateral, but there have been some cases of unilateral VFP reported [13].
In many cases also no specific etiological factors could be identified; the real incidence of idiopathic cause is unknown, but it is around 20–35 % [19].
Some children with VFP are also affected by upper airway disease as laryngomalacia, tracheobronchomalacia, or subglottic stenosis, and rarer are laryngeal web, cricoarytenoid joint fixation, or intubation granuloma [11].
8.3.3 Diagnosis
The diagnosis of VFP is typically made with flexible fiberoptic laryngoscopy. In many centers, microdirect suspension laryngoscopy and bronchoscopy under general anesthesia are usually performed to find vocal fold fixation, to palpate the cricoarytenoid joint, and to report any comorbid conditions that may be contributing to symptoms such as subglottic stenosis, laryngotracheomalacia, and laryngeal web or cleft [14]. In a small infant who presents in frank distress or who has a significant cardiac history, it is better to do examination in the operating room [13].
8.3.4 Treatment
The treatment of VFP depends not only upon age and symptoms but also on the skills and experience of the otolaryngologist surgeon and upon parent’s/child’s desires [13].
Spontaneous recovery of vocal fold function is more common in pediatric than in adult patients. Unilateral VFP secondary to iatrogenic causes has the poorest documented long-term recovery rate of 50 %. Prognosis is best for neurologic cases followed by idiopathic and iatrogenic in which percentage of recovery is approximately 75 %. Prematurity was associated with a worse prognosis for spontaneous recovery [11]. Time to recovery is highly variable, from 6 months in RLN injury subsequent to cardiac surgery [25] till 2 years in neurologic cause [1] and 11 years in idiopathic cause [11]. Recommended observation time before surgery is 8–12 months [1].
Goals of therapy in children with VFP should focus around establishing and maintaining safe and stable airway, obtaining or preserving intelligible speech, swallowing without aspiration, and feeding and growth [25]. Impact of dysphonia upon social interaction in education or workplace can be severe; this could be, as described in the next paragraph, an indication for injection laryngoplasty.
The treatment in newborn or children younger than scholar age should be focused on airway protection and alimentation. Tracheotomy is usually necessary if there is a bilateral VFP. Speech therapy evaluation and modified barium swallow could set up correct dietary recommendations as thickening of feed or enteral nutrition via gastric or nasogastric tube [13].
In many cases of UVFP (80 %), there is an effective compensation by contralateral vocal fold, making surgical treatment unnecessary. For those who have persistent glottal aperture defect (20 %) speech therapy to strengthen compensatory method of glottis closure while minimizing risk of hyperfunctional dysphonia may be useful. It has shown good results in older children; utility in the younger age group is limited by their ability to participate [26]. The ability of the child to interact and participate with the speech pathologist is the key, rather than the absolute age [14].
Surgical treatment options include injection laryngoplasty (vocal fold augmentation), laryngeal framework surgery (medialization laryngoplasty and arytenoid adduction), and reinnervation procedures.
8.4 Laryngeal Clefts
Laryngeal clefts are rare laryngeal defects that affect approximately 1 in 10,000 to 20,000 live births. It is more prevalent in boys, with a male-to-female ratio of 1.25:1 [27].
Most cases are sporadic; however, inheritance as an autosomal dominant trait has been observed in several families. Nonspecific maternal determinants of risk such as premature delivery, polyhydramnios, and alcohol or drug use during pregnancy have all been reported [28, 29].
As previously reported, during embryological development, the trachea and esophagus share a common lumen until they are separated by a tracheoesophageal septum by 35–40 days of gestation (fifth and sixth embryologic weeks). A fusion’s failure or an arrested development of the tracheoesophageal septum may result in congenital abnormalities such as isolated laryngeal clefts until tracheoesophageal fistulae or esophageal atresia. A posterior laryngeal cleft is believed to result from incomplete formation of the interarytenoid muscle with or without absence of the interarytenoid mucosa. The interarytenoid muscle (along with all intrinsic laryngeal muscles) and the cricoid cartilage are derivatives of the sixth branchial arch [30]. Other abnormalities of other systems, including gastrointestinal, genitourinary, and cardiovascular defects, often coexist with laryngeal clefts since up to 50 % of patients [28].
8.4.1 The Benjamin and Inglis’s Classification of Laryngeal Cleft
The most commonly used classification system is the one developed by Benjamin and Inglis (1989) [31], in which four types of laryngeal cleft had been described, based on their distal extent:
Type 1 laryngeal cleft (LC-I) is a supraglottic interarytenoid defect that extends no further caudally than the true vocal folds.
Type 2 laryngeal clefts extend below the level of the true vocal folds, and the cricoid lamina is partially involved.
Type 3 is a complete cleft of the cricoid lamina with or without extension into the cervical trachea.
It has been proposed that any newborn with feeding problems, persistent aspiration, and respiratory distress should be evaluated for a possible laryngeal cleft.
The most common symptoms include aspiration, stridor, recurrent pneumonia, chronic cough, wheezing, cyanotic episodes, choking, and difficulty feeding.
8.4.2 Diagnosis
The diagnosis of laryngeal cleft involves a high degree of suspicion, clinical evaluation of swallowing, chest radiography, and microlaryngoscopy that is the gold standard [27].
Other exams that may be useful are modified barium swallow (MBS) to see aspiration problem and functional endoscopic evaluation of swallowing (FEES) with flexible fiberoptic laryngoscopy.
Flexible fiberoptic laryngoscopy and functional endoscopic evaluation of swallowing (FEES) assist in diagnosis because they provide a dynamic view of laryngeal function [30].
8.4.3 Management
Despite a relative consensus regarding the diagnosis of laryngeal cleft type I (LC-I), a disagreement exists in medical literature regarding the management. Some studies advocate surgical repair, while others support medical management. The primary goal of treatment is to minimize respiratory complications related to aspiration. Initial conservative management includes antireflux therapy and modifications of feeding including thicker consistency and strict upright positioning [28]. The second strategy consists of an early repair using an endoscopic approach; this surgical approach entails the risk of complications such as laryngeal nerve injury, granulation tissue formation, and esophageal stricture formation [27].
The third treatment option, that can also be a diagnostic measure, is an injection augmentation of the posterior glottis. This injection laryngoplasty for the laryngeal cleft type I (LC-I) is a minimally invasive technique and can be used as a diagnostic and therapeutic tool, because it confirms the presence of a submucosal interarytenoid muscle dehiscence and resolves in most cases the chronic aspiration problem.
8.4.4 Cohen’s Algorithm for Management of Suspicion of LC-1
Cohen et al. propose a useful algorithm for the management of these children with aspiration problem, in which the injection laryngoplasty is the first step in a surgical resolution problem of the laryngeal cleft type I [28].
8.5 Injection Laryngoplasty in the Pediatric Population
Injection laryngoplasty in the pediatric population is an uncommon surgical treatment; in literature there are only twelve experiences described (Table 8.1). Vocal fold paralysis and type I laryngeal cleft are currently the only two diseases treated with injective laryngoplasty in children.
Table 8.1
Summary of injection laryngoplasty in the pediatric population in various studies
Experience of injection laryngoplasty | Year of public | N° of patient | Age | Cause | Indication | Material used | Result |
|---|---|---|---|---|---|---|---|
Emery et al. [32] | 1984 | 1 | – | VFP | Voice | Teflon | Good |
Levine et al. [33] | 1995 | 3 | – | VFP | Aspiration | All decannulated | |
Daya et al. [11] | 2000 | 3 | – | VFP | Voice | Teflon | |
Patel et al. [12] | 2003 | 4 | 5 years (21 days–18 years) | VFP | Cymetra | Good | |
Shah et al. [15] | 2006 | 7 | Not specified | UVFP | Voice | Not specified | Improvement in perceptual voice |
Sipp et al. [34] | 2007 | 12 | 10.6 (2.5–18) | VFP | Voice | Bovine collagen Cymetra Autologous fat Surgifoam Radiesse Voice Radiesse Voice Gel | Improvement in symptoms (>80 %) |
Cohen et al. [10] | 2011 | 13 | 8.0 (1.3–18.0) years | VFP | Voice (85 %); aspiration (40 %) | Gelfoam Radiesse Voice Radiesse Voice Gel | Improvement in symptoms (>80 %) |
Nakahara et al. [35] | 1995 | 1 | 16 months | Minor LC-1a (armitage) | Aspiration | Collagen | Good control; she needs another 2 IL |
Kennedy et al. [36] | 2000 | 8 | – | LC-I | Aspiration | Gelfoam | Clinical improvement |
Cohen et al. [28]
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