International Journal of Pediatric Otorhinolaryngology 78 (2014) 1866–1869
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Acquired bilateral adductor laryngeal paralysis in neonates and children: A case series and a systematic review Lavanya Bathini g, Mohammed Jomah d,b, Aleksandra Krajacic d,g, Caroline Jeffery b,d, Sandra Campbell f, Hamdy El-Hakim a,b,c,e,* a
Pediatric Otolaryngology Service, The Stollery Children’s Hospital & The University of Alberta Hospitals, Edmonton, Alberta, Canada Division of Otolaryngology – Head & Neck Surgery, The Stollery Children’s Hospital & The University of Alberta Hospitals, Edmonton, Alberta, Canada Division of Pediatric Surgery, The Stollery Children’s Hospital & The University of Alberta Hospitals, Edmonton, Alberta, Canada d Department of Surgery, The Stollery Children’s Hospital & The University of Alberta Hospitals, Edmonton, Alberta, Canada e Department of Pediatrics, The Stollery Children’s Hospital & The University of Alberta Hospitals, Edmonton, Alberta, Canada f John W. Scott Health Science Library, University of Alberta, Edmonton, Alberta, Canada g Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Alberta, Canada b c
A R T I C L E I N F O
A B S T R A C T
Article history: Received 1 March 2014 Received in revised form 23 June 2014 Accepted 11 August 2014 Available online 17 August 2014
Objectives: To present a series of acquired bilateral adductor laryngeal paralysis (BAdLP) and review the literature on clinical manifestations and management. Methods: A retrospective review of a single tertiary care practice of pediatric otolaryngology was conducted. Patients were identified from a surgical database spanning twelve years of practice (2002– 2013). The variables documented included gender, age at presentation, co-morbid conditions, documented laryngeal findings on endoscopy, management and outcome. A systematic review of the literature was conducted to identify reports on BAdLP in children and associated conditions. Results: Five cases (four girls and one boy) ranging from 3 months to 16 years of age were identified. All cases were documented using rigid and/or flexible laryngoscopy. In four cases, the onset was after major cardiac surgery complicated by cerebral vascular accidents, while one followed a thalamic stroke. Four were managed with tube feeding. Only three papers reported BAdLP in children. Conclusions: The cases identified were all acquired after a central neurological insult. The profile is distinct from the congenital adductor form of laryngeal paralysis previously described. However, the symptom complex is identical. We believe this is the largest case series from one center to be reported. ß 2014 Elsevier Ireland Ltd. All rights reserved.
Keywords: Laryngeal paralysis Adductor Abductor Aspiration Central nervous system Larynx
1. Introduction Laryngeal paralysis (LP) is not an uncommon cause of stridor in children and is a difficult clinical dilemma in the pediatric population. This condition usually presents with stridor, increased work of breathing, hoarseness, and/or an impaired cough reflex leading to aspiration, depending on laterality of the paralyzed vocal folds and laryngeal muscles affected. It is usually secondary to an impairment of the nerve supply to the larynx. This impairment can be congenital or acquired (e.g., iatrogenic, neurologic insults, or systemic muscular diseases etc.). However,
* Corresponding author at: 2C3.57 Walter MacKenzie Center, 8440–112 Street, Edmonton, AB T6G 2B7, Canada. Tel.: +1 780 407 8629; fax: +1 780 407 2004. E-mail addresses: [email protected], [email protected] (H. El-Hakim). http://dx.doi.org/10.1016/j.ijporl.2014.08.012 0165-5876/ß 2014 Elsevier Ireland Ltd. All rights reserved.
often times, laryngeal paralysis is labeled as idiopathic if an etiology cannot be elicited [1]. LP can be unilateral or bilateral, with the presentation varying accordingly. There are two distinct sets of muscle(s) that control the width of the glottis and both can be affected: laryngeal abductors and adductors. Most of the research in LP has focused on the commoner scenario affecting the abductor muscles. Rarely, laryngeal adductors can also be affected, causing dysphonia and aspiration, but there are only three publications that report this rare condition [2–4]. Mace was probably the first to describe the familial bilateral adductor laryngeal paralysis (BAdLP) in three generations of one family [2]. Afterwards, Berkowitz in 2003 reported three patients, coining for the first time the congenital non-familial variety of BAdLP [3]. There is only one other series that lists two patients with the diagnosis, but does not discuss them in detail [4]. In this paper, we report five cases of this rare form of laryngeal paralysis. We conducted a systematic review of the
L. Bathini et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 1866–1869
1867
literature looking for previously reported cases. Our results suggest that this is the first reported case series of acquired BAdLP of its kind.
3. Results
2. Methods
The search ‘‘vocal cord paralysis’’ yielded 4859 different papers, most of which were irrelevant to the topic at hand. When the search was narrowed using identifiers such as ‘‘bilateral adductor’’ and ‘‘congenital’’ the search yielded 5 studies, only 1 of which was applicable to this specific topic, by R.G. Berkowitz, after the titles and abstracts of the articles were reviewed [2]. The other 4 studies were selected on the basis of relevance to the general topic, however were not specific to the topic at hand. The reference lists for each of these studies and the citations quoting these studies were scanned to see if any other studies had been missed and this process was repeated for all 8 databases listed above. The authors of these articles were not contacted for additional information (Fig. 1; Table 1). On September 16, 2013 a citation chain was conducted to ensure that no studies that had cited the one applicable article written by R.G. Berkowitz were not relevant to the topic of BAdLP. Berkowitz’ article on congenital BAdLP had been cited in 25 different papers using the Web of Science database and 4 studies using the Scopus database. These studies were not of relevance as they did not meet the minimum inclusion or exclusion criteria and all of these papers looked at abductor paralysis and not adductor paralysis. Berkowitz cites two prior case reports on laryngeal adductor paralysis: Swift and Rogers listed two cases of BAdLP amongst a series of laryngeal paralysis but no other details were documented [3]. The only report of the familial disorder is by Mace in 1978 [4].
This is a retrospective observational controlled case series. Approval was sought and obtained from the Health Ethics Research Office at the University of Alberta. The patients were identified from a surgical database maintained by a tertiary-care pediatric otolaryngologist (HE) at the Stollery Children’s Hospital, Edmonton, Alberta, Canada. After verifying that each patient had fulfilled the inclusion and exclusion criteria, the following variables were collected: age, gender, date of procedure, co-morbid conditions, airway endoscopy findings, and management. 2.1. Inclusion criteria 1. Patients presenting with signs and symptoms of laryngeal incompetence (weak cry, hoarseness, poor feeding, aspiration). 2. Patients with poor or absent bilateral vocal fold adduction, documented by awake flexible laryngoscopy.
2.2. Exclusion criteria 1. Presence of stridor 2. Other features of upper airway obstruction present 3. Suspicion of bilateral recurrent nerve injury due to surgical/ obstetric injury 4. Endoscopic evidence of posterior glottic stenosis or cricoarytenoid ankylosis. Descriptive statistics were performed.
2.3. Systematic review A systematic review of the literature was conducted to identify reports on BAdLP in children and associated conditions. On June 17, 2013, a health sciences librarian assisted in conducting a literature search on the topic of congenital BAdLP. The databases searched included Cochrane, MEDLINE, EMBASE, Scopus, Web of Science, Proquest Dissertations, CINAHL and Pub Med. No limits such as language or dates were applied given the specificity and lack of literature on this topic. The keywords used in this search included vocal cord paralysis/paresis/palsy and laryngeal paralysis/paresis/palsy. The search was narrowed using unique identifiers such as congenital, adductor, and bilateral. The key words used in this search were modified depending on which database was used. Both reference lists and citations quoting the studies from the narrowed search were reviewed to ensure that no studies on this topic had been missed. RefWorks 2.0 online software was used to track all references for the relevant studies and all duplicate studies had been removed from the list of references. Methods for including studies in literature review:
3.1. Search results
3.2. Case reports Five cases were identified from the database (June 2002 till September 2013). All cases had confirmed diagnosis of acquired BAdLP. Of note, none of these patients had prior neck surgery. The findings for each of these patients are summarized in the table. The first case is a four-month-old female who presented to the otolaryngology service with new onset increased work of breathing in the setting of remote cardiac transplantation for dilated cardiomyopathy. She also has a history of previous CVA with neurologic deficits that was not temporally related to her cardiac transplantation. The second case is a three-month-old male referred for persistent dysphonia after atrial and ventricular septal defects closure. He also had a past medical history significant for bilateral subdural hematomas, multiple aortopulmonary collaterals and right diaphragmatic paralysis. He was
Types of studies: 1. Case series 2. Studies published in English. Types of participants: 1. Pediatric patients (
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Acquired bilateral adductor laryngeal paralysis in neonates and children: A case series and a systematic review Lavanya Bathini g, Mohammed Jomah d,b, Aleksandra Krajacic d,g, Caroline Jeffery b,d, Sandra Campbell f, Hamdy El-Hakim a,b,c,e,* a
Pediatric Otolaryngology Service, The Stollery Children’s Hospital & The University of Alberta Hospitals, Edmonton, Alberta, Canada Division of Otolaryngology – Head & Neck Surgery, The Stollery Children’s Hospital & The University of Alberta Hospitals, Edmonton, Alberta, Canada Division of Pediatric Surgery, The Stollery Children’s Hospital & The University of Alberta Hospitals, Edmonton, Alberta, Canada d Department of Surgery, The Stollery Children’s Hospital & The University of Alberta Hospitals, Edmonton, Alberta, Canada e Department of Pediatrics, The Stollery Children’s Hospital & The University of Alberta Hospitals, Edmonton, Alberta, Canada f John W. Scott Health Science Library, University of Alberta, Edmonton, Alberta, Canada g Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Alberta, Canada b c
A R T I C L E I N F O
A B S T R A C T
Article history: Received 1 March 2014 Received in revised form 23 June 2014 Accepted 11 August 2014 Available online 17 August 2014
Objectives: To present a series of acquired bilateral adductor laryngeal paralysis (BAdLP) and review the literature on clinical manifestations and management. Methods: A retrospective review of a single tertiary care practice of pediatric otolaryngology was conducted. Patients were identified from a surgical database spanning twelve years of practice (2002– 2013). The variables documented included gender, age at presentation, co-morbid conditions, documented laryngeal findings on endoscopy, management and outcome. A systematic review of the literature was conducted to identify reports on BAdLP in children and associated conditions. Results: Five cases (four girls and one boy) ranging from 3 months to 16 years of age were identified. All cases were documented using rigid and/or flexible laryngoscopy. In four cases, the onset was after major cardiac surgery complicated by cerebral vascular accidents, while one followed a thalamic stroke. Four were managed with tube feeding. Only three papers reported BAdLP in children. Conclusions: The cases identified were all acquired after a central neurological insult. The profile is distinct from the congenital adductor form of laryngeal paralysis previously described. However, the symptom complex is identical. We believe this is the largest case series from one center to be reported. ß 2014 Elsevier Ireland Ltd. All rights reserved.
Keywords: Laryngeal paralysis Adductor Abductor Aspiration Central nervous system Larynx
1. Introduction Laryngeal paralysis (LP) is not an uncommon cause of stridor in children and is a difficult clinical dilemma in the pediatric population. This condition usually presents with stridor, increased work of breathing, hoarseness, and/or an impaired cough reflex leading to aspiration, depending on laterality of the paralyzed vocal folds and laryngeal muscles affected. It is usually secondary to an impairment of the nerve supply to the larynx. This impairment can be congenital or acquired (e.g., iatrogenic, neurologic insults, or systemic muscular diseases etc.). However,
* Corresponding author at: 2C3.57 Walter MacKenzie Center, 8440–112 Street, Edmonton, AB T6G 2B7, Canada. Tel.: +1 780 407 8629; fax: +1 780 407 2004. E-mail addresses: [email protected], [email protected] (H. El-Hakim). http://dx.doi.org/10.1016/j.ijporl.2014.08.012 0165-5876/ß 2014 Elsevier Ireland Ltd. All rights reserved.
often times, laryngeal paralysis is labeled as idiopathic if an etiology cannot be elicited [1]. LP can be unilateral or bilateral, with the presentation varying accordingly. There are two distinct sets of muscle(s) that control the width of the glottis and both can be affected: laryngeal abductors and adductors. Most of the research in LP has focused on the commoner scenario affecting the abductor muscles. Rarely, laryngeal adductors can also be affected, causing dysphonia and aspiration, but there are only three publications that report this rare condition [2–4]. Mace was probably the first to describe the familial bilateral adductor laryngeal paralysis (BAdLP) in three generations of one family [2]. Afterwards, Berkowitz in 2003 reported three patients, coining for the first time the congenital non-familial variety of BAdLP [3]. There is only one other series that lists two patients with the diagnosis, but does not discuss them in detail [4]. In this paper, we report five cases of this rare form of laryngeal paralysis. We conducted a systematic review of the
L. Bathini et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 1866–1869
1867
literature looking for previously reported cases. Our results suggest that this is the first reported case series of acquired BAdLP of its kind.
3. Results
2. Methods
The search ‘‘vocal cord paralysis’’ yielded 4859 different papers, most of which were irrelevant to the topic at hand. When the search was narrowed using identifiers such as ‘‘bilateral adductor’’ and ‘‘congenital’’ the search yielded 5 studies, only 1 of which was applicable to this specific topic, by R.G. Berkowitz, after the titles and abstracts of the articles were reviewed [2]. The other 4 studies were selected on the basis of relevance to the general topic, however were not specific to the topic at hand. The reference lists for each of these studies and the citations quoting these studies were scanned to see if any other studies had been missed and this process was repeated for all 8 databases listed above. The authors of these articles were not contacted for additional information (Fig. 1; Table 1). On September 16, 2013 a citation chain was conducted to ensure that no studies that had cited the one applicable article written by R.G. Berkowitz were not relevant to the topic of BAdLP. Berkowitz’ article on congenital BAdLP had been cited in 25 different papers using the Web of Science database and 4 studies using the Scopus database. These studies were not of relevance as they did not meet the minimum inclusion or exclusion criteria and all of these papers looked at abductor paralysis and not adductor paralysis. Berkowitz cites two prior case reports on laryngeal adductor paralysis: Swift and Rogers listed two cases of BAdLP amongst a series of laryngeal paralysis but no other details were documented [3]. The only report of the familial disorder is by Mace in 1978 [4].
This is a retrospective observational controlled case series. Approval was sought and obtained from the Health Ethics Research Office at the University of Alberta. The patients were identified from a surgical database maintained by a tertiary-care pediatric otolaryngologist (HE) at the Stollery Children’s Hospital, Edmonton, Alberta, Canada. After verifying that each patient had fulfilled the inclusion and exclusion criteria, the following variables were collected: age, gender, date of procedure, co-morbid conditions, airway endoscopy findings, and management. 2.1. Inclusion criteria 1. Patients presenting with signs and symptoms of laryngeal incompetence (weak cry, hoarseness, poor feeding, aspiration). 2. Patients with poor or absent bilateral vocal fold adduction, documented by awake flexible laryngoscopy.
2.2. Exclusion criteria 1. Presence of stridor 2. Other features of upper airway obstruction present 3. Suspicion of bilateral recurrent nerve injury due to surgical/ obstetric injury 4. Endoscopic evidence of posterior glottic stenosis or cricoarytenoid ankylosis. Descriptive statistics were performed.
2.3. Systematic review A systematic review of the literature was conducted to identify reports on BAdLP in children and associated conditions. On June 17, 2013, a health sciences librarian assisted in conducting a literature search on the topic of congenital BAdLP. The databases searched included Cochrane, MEDLINE, EMBASE, Scopus, Web of Science, Proquest Dissertations, CINAHL and Pub Med. No limits such as language or dates were applied given the specificity and lack of literature on this topic. The keywords used in this search included vocal cord paralysis/paresis/palsy and laryngeal paralysis/paresis/palsy. The search was narrowed using unique identifiers such as congenital, adductor, and bilateral. The key words used in this search were modified depending on which database was used. Both reference lists and citations quoting the studies from the narrowed search were reviewed to ensure that no studies on this topic had been missed. RefWorks 2.0 online software was used to track all references for the relevant studies and all duplicate studies had been removed from the list of references. Methods for including studies in literature review:
3.1. Search results
3.2. Case reports Five cases were identified from the database (June 2002 till September 2013). All cases had confirmed diagnosis of acquired BAdLP. Of note, none of these patients had prior neck surgery. The findings for each of these patients are summarized in the table. The first case is a four-month-old female who presented to the otolaryngology service with new onset increased work of breathing in the setting of remote cardiac transplantation for dilated cardiomyopathy. She also has a history of previous CVA with neurologic deficits that was not temporally related to her cardiac transplantation. The second case is a three-month-old male referred for persistent dysphonia after atrial and ventricular septal defects closure. He also had a past medical history significant for bilateral subdural hematomas, multiple aortopulmonary collaterals and right diaphragmatic paralysis. He was
Types of studies: 1. Case series 2. Studies published in English. Types of participants: 1. Pediatric patients (
