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Intracordal cyst in a neonate
International Journal of Pediatric Otorhinolaryngology 52 (2000) 277 – 281 www.elsevier.com/locate/ijporl
Case report
Intracordal cyst in a neonate O. Daniel Smith * , Vincent Callanan, Jonathan Harcourt, David M. Albert Department of Paediatric Otolaryngology, Great Ormond Street Hospital for Children, London WC1N 3JH, UK Received 6 November 1999; accepted 22 December 1999
Abstract Vocal cord cysts are rare, and typically present with symptoms of dysphonia in older children and adults. There is often a background of voice abuse. We present the first recorded and photographed case of an intracordal cyst presenting in a neonate. The patient presented with symptoms of episodic stridor, respiratory distress, and feeding difficulties, which resolved rapidly after simple incision of the cyst. Theories for the aetiology of intracordal cysts are discussed. The authors believe that this case lends support to the theory that intracordal cysts are congenital in origin. © 2000 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Vocal cords; Cysts; Infant; Newborn; Respiratory sounds
1. Introduction Vocal cord cysts are rare, representing less than 1% of referrals to specialist voice clinics [1]. The typical presenting symptom is dysphonia, and they occur more frequently in adults than in children. In a series of 157 patients described by Bouchayer et al. [2] the youngest patient was 5 years old. Similarly in a series of 43 patients described by Kawasaki et al. [3] the youngest patient was 8 years old. In a series of 657 congen* Corresponding author. Fax: +44-171-8298644. E-mail address: [email protected] (O.D. Smith)
ital anomalies of the larynx, Holinger et al. [4] referred to three infants with ‘simple congenital glottic cysts’, although there is no discussion of the type, position, or presentation of these cysts. Several authors describe congenital cysts of the epiglottis and aryepiglottic folds [5,6]. De Santo et al. stated that all congenital cysts of the larynx occurred in the lateral supraglottic position [7]. Ferguson, however, stated that they may occur elsewhere in the larynx, including the true cords [8], but again there were no details of specific cases. We present, therefore, the first case in the world literature of an intracordal cyst occurring in a neonate.
0165-5876/00/$ - see front matter © 2000 Elsevier Science Ireland Ltd. All rights reserved. PII: S 0 1 6 5 - 5 8 7 6 ( 0 0 ) 0 0 2 7 7 - 9
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O.D. Smith et al. / Int. J. Pediatr. Otorhinolaryngol. 52 (2000) 277–281
2. Case report A female child was born at full term by spontaneous vaginal delivery. The child’s respirations were normal at birth and there was no history of endotracheal intubation. On the tenth day of life, the child developed episodes of coughing and choking and subsequently cyanosis during feeds. The parents had noted that the cry was weak, and reported ‘noisy’ breath sounds on inspiration. Examination revealed an afebrile child, who desaturated intermittently during feeding. Inspiratory stridor and recession were noted, but only when the child was agitated. Echocardiography revealed a small patent foramen ovale but otherwise normal anatomy, with no vascular ring. A contrast oesophagogram excluded the presence of a tracheosophageal fistula. Microlaryngoscopic examination was scheduled to be performed under general anaesthesia. Following induction of anaesthesia, the cords were sprayed with lignocaine 10%, and treated with a swab soaked in adrenaline 1 in 1000 for 5 min. Examination then revealed a well circumscribed
mass within the left vocal cord. This was located at the junction between the anterior and middle thirds of the vocal cord, on its superior aspect, slightly lateral to its free edge (Fig. 1). There was a smaller swelling in an equivalent position of the right vocal fold. The remainder of the examination was normal, with no evidence of laryngomalacia, tracheomalacia, or subglottic stenosis. Both vocal cords were observed to move normally. A superior cordotomy was performed on the left side, revealing the well circumscribed lesion to be cystic in nature. Clear, viscous fluid was released. After the procedure there were no further episodes of stridor, cyanosis, or respiratory distress. The episodes of choking during feeds persisted initially. Barium swallow and pH study excluded the presence of gastro-oesophageal reflux. Assessment by a specialist in paediatric speech and swallowing therapy noted a poorly co-ordinated suck–swallow–breathe pattern. This was easily corrected with training and supervision. Eight weeks later, the child had made a complete recovery, with no stridor or feeding
Fig. 1. Larynx at 3 weeks of age, illustrating cystic lesion on left cord, with smaller ‘contact lesion’ on right.
O.D. Smith et al. / Int. J. Pediatr. Otorhinolaryngol. 52 (2000) 277–281
279
Fig. 2. Larynx 8 weeks later, with normal vocal cords.
difficulties. A repeat microlaryngoscopy revealed normal vocal cords (Fig. 2), and no other potential cause for the earlier stridor.
3. Discussion Intracordal cysts, although rare and benign, are an important aspect of contemporary otolaryngological practice as they carry an excellent prognosis if diagnosed and correctly treated. The most frequent presenting symptom of an intracordal cyst is dysphonia, occurring in an older child or adult [2,3]. This may be intermittent or persistent. There may also be an associated history of sore throat or voice abuse. In contrast, congenital cysts of the supraglottis were described by Jacobi and Rascoff to present shortly after birth with laboured respiration, especially during feeding [9]. It is of interest to note that in the above case the cyst, although intracordal in position, presented with the classical symptoms of a supraglottic cyst. Both supraglottic and intracordal cysts may be diagnosed by laryngoscopy. An intracordal cyst may be seen as a discrete bulge of the affected
vocal fold. However the abnormality may only be detectable on stroboscopic examination, at which absent or diminished vibrations are noted. Although 11% are bilateral there is frequently a smaller bulge, referred to as a ‘contact lesion’, on the opposite cord [10]. A characteristic ‘opalescent’ appearance is described [11]. Histological examination of intracordal cysts reveals two distinct types [12]. Epidermoid cysts consist of a pultaceous cystic mass of squamous epithelium, containing keratin and embedded in the submucosa of the vocal cord. Mucus retention cysts are lined by respiratory or glandular epithelium. Incision of an epidermoid cyst tends to produce a cheesy material, similar to a cholesteatoma, whereas incision of a mucus retention cyst, as the name suggests, releases clear viscous mucus [12]. Management of intracordal cysts has been improved by contemporary microlaryngeal surgery. The most widespread surgical treatment involves superior cordotomy, with excision of the cyst ‘in toto’. Simple incision of the cyst, however, is considered to be an adequate treatment, with no greater incidence of recurrence [12].
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O.D. Smith et al. / Int. J. Pediatr. Otorhinolaryngol. 52 (2000) 277–281
The aetiology of intracordal cysts remains controversial. The principal theories are that such cysts are either congenital or acquired in origin. The congenital theory suggests that intracordal cysts and sulci represent remnants of the fourth and sixth branchial arches [2]. This is supported by the observations that these entities frequently occur together [10,13]; that the dysphonia often starts in childhood [10,14]; that there may be no history of voice abuse [14]; and that cases occurring in several members of one family have been described [15]. Most intracordal cysts that are believed to be of congenital origin are of epidermoid type on histological examination [1]. The acquired theory can be applied to both epidermoid type cysts and mucus retention cysts. For both of these histological subtypes, there is an initial injury to the vocal cord, from voice abuse, throat infection, external trauma, surgery, or chemical irritation [10]. In the case of a mucus retention cyst, this injurious agent initiates an inflammatory reaction, which obstructs the duct of a mucus gland [12]. In the case of an epidermoid cyst, the injurious agent produces a small fissure into the cordal epithelium, which grows after healing of the initial microwound, in a manner similar to acquired cholesteatoma pathogenesis [10]. The acquired theories are supported by the observations that the cysts are found most frequently in the most functionally active segment of the vocal fold, i.e. the junction between the anterior and middle third [1]; that there is frequent evidence of chronic inflammation on histological examination of the affected cord [2]; and that there is a frequent association with voice abuse [10], ‘vocal hyperfunction’ [2], or extrovert and anxious personality types [1]. Furthermore there is evidence of increased activity in the laryngeal muscles in cystic cases [1]. It is believed that this increased muscular activity is causative rather than secondary, as the presence of a cyst prevents sufficient glottic closure. The majority of authors believe that although some cases of intracordal cysts are congenital, the overwhelming majority are of acquired aetiology [1,12]. The few cases that are truly congenital are universally believed to be of epidermoid type [1].
We believe this case of an intracordal cysts occurring in a 10-day-old neonate could only have been congenital in aetiology. All of the proponents of the acquired theory emphasise the chronic nature of the condition. It is of interest to note that the fluid achieved on incision was the clear, viscous type associated with mucus retention cysts, rather than the ‘cheesy’ type associated with epidermoid cysts [12]. A case of congenital mucus retention cyst of the vocal cord is without precedent in the world literature.
4. Conclusion Congenital cysts of the larynx are rare. Whilst usually occurring in the supraglottic position, they may also be intracordal. There may be a tendency to under-diagnosis, as many acquired cysts are detectable only by stroboscopic examination. When correctly diagnosed they are simple to treat and carry an excellent prognosis. Laryngeal cysts should be considered in the differential diagnosis of any infant presenting with stridor, weak cry, or feeding difficulties.
References [1] Z. Milutinovic, J. Vasiljevic, Contribution to the understanding of the etiology of vocal fold cysts: a functional and histologic study, Laryngoscope 102 (1992) 568 – 571. [2] M. Bouchayer, G. Cornut, E. Witzig, R. Loire, J.B. Roch, R.W. Bastian, Epidermoid cysts, sulci, and mucosal bridges of the true vocal cord: a report of 157 cases, Laryngoscope 95 (1985) 1087 – 1094. [3] H. Kawasaki, K. Kuratomi, T. Mitsumasu, Cysts of the larynx. A 10-year review of 94 patients, Auris Nasus Larynx 10 (Suppl.) (1983) S47 – S52. [4] P.H. Holinger, W.T. Brown, C. Gables, Congenital webs, cysts, laryngoceles, and other anomalies of the larynx, Ann. Otol. 76 (1967) 744 – 752. [5] A.S. Bais, K. Uppal, K.B. Logani, Congenital cysts of the larynx, J. Laryngol. Otol. 103 (1989) 966 – 967. [6] P.C. Weber, M.A. Kenna, M.L. Casselbrant, Laryngeal cysts: a cause of neonatal airway obstruction, Otolaryngol. Head Neck Surg. 109 (1993) 129 – 134. [7] L.W. De Santo, K.D. Devine, L.H. Welland, Cysts of the larynx — Classification, Laryngoscope 80 (1970) 145 – 176.
O.D. Smith et al. / Int. J. Pediatr. Otorhinolaryngol. 52 (2000) 277–281 [8] C.F. Ferguson, Congenital abnormalities of the infant larynx, Otolaryngol. Clin. North Am. 3 (2) (1970) 185– 200. [9] M. Jacobi, H. Rascoff, Cysts of the larynx in infancy, Am. J. Dis. Child. 49 (1935) 448–459. [10] L.A. Monday, G. Cornut, M. Bouchayer, J.B. Roch, Epidermoid cysts of the vocal cords, Ann. Otol. Rhinol. Laryngol. 92 (1983) 124–127. [11] C.L. Jackson, Benign tumours of the larynx, in: C. Jackson, C.L. Jackson (Eds.), Diseases of the Nose, Throat and Ear, Saunders, Philadelphia, PA, 1959, pp. 681–689.
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[12] L.A. Monday, G. Cornut, M. Bouchayer, J.B. Roch, R. Loire, Diagnosis and treatment of intracordal cysts, J. Otolaryngol. 10 (1981) 363 – 370. [13] G.E. Arnold, Dysplastic dysphonia: minor anomalies of the vocal cords causing persistent hoarseness, Laryngoscope 68 (1958) 142 – 158. [14] J.B. Roch, M. Bouchayer, G. Cornut, Le sulcus glottidis, Rev. Laryngol. Otol. Rhinol. (Bord.) 102 (1981) 333 – 346. [15] J. Trevin, L. Cheesebeuf, G. Cornut, A propos de 3 cas familiaux de sulcus glottides, Bull. d’audiophonol. 7 (1977) 76 – 81.
Case report
Intracordal cyst in a neonate O. Daniel Smith * , Vincent Callanan, Jonathan Harcourt, David M. Albert Department of Paediatric Otolaryngology, Great Ormond Street Hospital for Children, London WC1N 3JH, UK Received 6 November 1999; accepted 22 December 1999
Abstract Vocal cord cysts are rare, and typically present with symptoms of dysphonia in older children and adults. There is often a background of voice abuse. We present the first recorded and photographed case of an intracordal cyst presenting in a neonate. The patient presented with symptoms of episodic stridor, respiratory distress, and feeding difficulties, which resolved rapidly after simple incision of the cyst. Theories for the aetiology of intracordal cysts are discussed. The authors believe that this case lends support to the theory that intracordal cysts are congenital in origin. © 2000 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Vocal cords; Cysts; Infant; Newborn; Respiratory sounds
1. Introduction Vocal cord cysts are rare, representing less than 1% of referrals to specialist voice clinics [1]. The typical presenting symptom is dysphonia, and they occur more frequently in adults than in children. In a series of 157 patients described by Bouchayer et al. [2] the youngest patient was 5 years old. Similarly in a series of 43 patients described by Kawasaki et al. [3] the youngest patient was 8 years old. In a series of 657 congen* Corresponding author. Fax: +44-171-8298644. E-mail address: [email protected] (O.D. Smith)
ital anomalies of the larynx, Holinger et al. [4] referred to three infants with ‘simple congenital glottic cysts’, although there is no discussion of the type, position, or presentation of these cysts. Several authors describe congenital cysts of the epiglottis and aryepiglottic folds [5,6]. De Santo et al. stated that all congenital cysts of the larynx occurred in the lateral supraglottic position [7]. Ferguson, however, stated that they may occur elsewhere in the larynx, including the true cords [8], but again there were no details of specific cases. We present, therefore, the first case in the world literature of an intracordal cyst occurring in a neonate.
0165-5876/00/$ - see front matter © 2000 Elsevier Science Ireland Ltd. All rights reserved. PII: S 0 1 6 5 - 5 8 7 6 ( 0 0 ) 0 0 2 7 7 - 9
278
O.D. Smith et al. / Int. J. Pediatr. Otorhinolaryngol. 52 (2000) 277–281
2. Case report A female child was born at full term by spontaneous vaginal delivery. The child’s respirations were normal at birth and there was no history of endotracheal intubation. On the tenth day of life, the child developed episodes of coughing and choking and subsequently cyanosis during feeds. The parents had noted that the cry was weak, and reported ‘noisy’ breath sounds on inspiration. Examination revealed an afebrile child, who desaturated intermittently during feeding. Inspiratory stridor and recession were noted, but only when the child was agitated. Echocardiography revealed a small patent foramen ovale but otherwise normal anatomy, with no vascular ring. A contrast oesophagogram excluded the presence of a tracheosophageal fistula. Microlaryngoscopic examination was scheduled to be performed under general anaesthesia. Following induction of anaesthesia, the cords were sprayed with lignocaine 10%, and treated with a swab soaked in adrenaline 1 in 1000 for 5 min. Examination then revealed a well circumscribed
mass within the left vocal cord. This was located at the junction between the anterior and middle thirds of the vocal cord, on its superior aspect, slightly lateral to its free edge (Fig. 1). There was a smaller swelling in an equivalent position of the right vocal fold. The remainder of the examination was normal, with no evidence of laryngomalacia, tracheomalacia, or subglottic stenosis. Both vocal cords were observed to move normally. A superior cordotomy was performed on the left side, revealing the well circumscribed lesion to be cystic in nature. Clear, viscous fluid was released. After the procedure there were no further episodes of stridor, cyanosis, or respiratory distress. The episodes of choking during feeds persisted initially. Barium swallow and pH study excluded the presence of gastro-oesophageal reflux. Assessment by a specialist in paediatric speech and swallowing therapy noted a poorly co-ordinated suck–swallow–breathe pattern. This was easily corrected with training and supervision. Eight weeks later, the child had made a complete recovery, with no stridor or feeding
Fig. 1. Larynx at 3 weeks of age, illustrating cystic lesion on left cord, with smaller ‘contact lesion’ on right.
O.D. Smith et al. / Int. J. Pediatr. Otorhinolaryngol. 52 (2000) 277–281
279
Fig. 2. Larynx 8 weeks later, with normal vocal cords.
difficulties. A repeat microlaryngoscopy revealed normal vocal cords (Fig. 2), and no other potential cause for the earlier stridor.
3. Discussion Intracordal cysts, although rare and benign, are an important aspect of contemporary otolaryngological practice as they carry an excellent prognosis if diagnosed and correctly treated. The most frequent presenting symptom of an intracordal cyst is dysphonia, occurring in an older child or adult [2,3]. This may be intermittent or persistent. There may also be an associated history of sore throat or voice abuse. In contrast, congenital cysts of the supraglottis were described by Jacobi and Rascoff to present shortly after birth with laboured respiration, especially during feeding [9]. It is of interest to note that in the above case the cyst, although intracordal in position, presented with the classical symptoms of a supraglottic cyst. Both supraglottic and intracordal cysts may be diagnosed by laryngoscopy. An intracordal cyst may be seen as a discrete bulge of the affected
vocal fold. However the abnormality may only be detectable on stroboscopic examination, at which absent or diminished vibrations are noted. Although 11% are bilateral there is frequently a smaller bulge, referred to as a ‘contact lesion’, on the opposite cord [10]. A characteristic ‘opalescent’ appearance is described [11]. Histological examination of intracordal cysts reveals two distinct types [12]. Epidermoid cysts consist of a pultaceous cystic mass of squamous epithelium, containing keratin and embedded in the submucosa of the vocal cord. Mucus retention cysts are lined by respiratory or glandular epithelium. Incision of an epidermoid cyst tends to produce a cheesy material, similar to a cholesteatoma, whereas incision of a mucus retention cyst, as the name suggests, releases clear viscous mucus [12]. Management of intracordal cysts has been improved by contemporary microlaryngeal surgery. The most widespread surgical treatment involves superior cordotomy, with excision of the cyst ‘in toto’. Simple incision of the cyst, however, is considered to be an adequate treatment, with no greater incidence of recurrence [12].
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O.D. Smith et al. / Int. J. Pediatr. Otorhinolaryngol. 52 (2000) 277–281
The aetiology of intracordal cysts remains controversial. The principal theories are that such cysts are either congenital or acquired in origin. The congenital theory suggests that intracordal cysts and sulci represent remnants of the fourth and sixth branchial arches [2]. This is supported by the observations that these entities frequently occur together [10,13]; that the dysphonia often starts in childhood [10,14]; that there may be no history of voice abuse [14]; and that cases occurring in several members of one family have been described [15]. Most intracordal cysts that are believed to be of congenital origin are of epidermoid type on histological examination [1]. The acquired theory can be applied to both epidermoid type cysts and mucus retention cysts. For both of these histological subtypes, there is an initial injury to the vocal cord, from voice abuse, throat infection, external trauma, surgery, or chemical irritation [10]. In the case of a mucus retention cyst, this injurious agent initiates an inflammatory reaction, which obstructs the duct of a mucus gland [12]. In the case of an epidermoid cyst, the injurious agent produces a small fissure into the cordal epithelium, which grows after healing of the initial microwound, in a manner similar to acquired cholesteatoma pathogenesis [10]. The acquired theories are supported by the observations that the cysts are found most frequently in the most functionally active segment of the vocal fold, i.e. the junction between the anterior and middle third [1]; that there is frequent evidence of chronic inflammation on histological examination of the affected cord [2]; and that there is a frequent association with voice abuse [10], ‘vocal hyperfunction’ [2], or extrovert and anxious personality types [1]. Furthermore there is evidence of increased activity in the laryngeal muscles in cystic cases [1]. It is believed that this increased muscular activity is causative rather than secondary, as the presence of a cyst prevents sufficient glottic closure. The majority of authors believe that although some cases of intracordal cysts are congenital, the overwhelming majority are of acquired aetiology [1,12]. The few cases that are truly congenital are universally believed to be of epidermoid type [1].
We believe this case of an intracordal cysts occurring in a 10-day-old neonate could only have been congenital in aetiology. All of the proponents of the acquired theory emphasise the chronic nature of the condition. It is of interest to note that the fluid achieved on incision was the clear, viscous type associated with mucus retention cysts, rather than the ‘cheesy’ type associated with epidermoid cysts [12]. A case of congenital mucus retention cyst of the vocal cord is without precedent in the world literature.
4. Conclusion Congenital cysts of the larynx are rare. Whilst usually occurring in the supraglottic position, they may also be intracordal. There may be a tendency to under-diagnosis, as many acquired cysts are detectable only by stroboscopic examination. When correctly diagnosed they are simple to treat and carry an excellent prognosis. Laryngeal cysts should be considered in the differential diagnosis of any infant presenting with stridor, weak cry, or feeding difficulties.
References [1] Z. Milutinovic, J. Vasiljevic, Contribution to the understanding of the etiology of vocal fold cysts: a functional and histologic study, Laryngoscope 102 (1992) 568 – 571. [2] M. Bouchayer, G. Cornut, E. Witzig, R. Loire, J.B. Roch, R.W. Bastian, Epidermoid cysts, sulci, and mucosal bridges of the true vocal cord: a report of 157 cases, Laryngoscope 95 (1985) 1087 – 1094. [3] H. Kawasaki, K. Kuratomi, T. Mitsumasu, Cysts of the larynx. A 10-year review of 94 patients, Auris Nasus Larynx 10 (Suppl.) (1983) S47 – S52. [4] P.H. Holinger, W.T. Brown, C. Gables, Congenital webs, cysts, laryngoceles, and other anomalies of the larynx, Ann. Otol. 76 (1967) 744 – 752. [5] A.S. Bais, K. Uppal, K.B. Logani, Congenital cysts of the larynx, J. Laryngol. Otol. 103 (1989) 966 – 967. [6] P.C. Weber, M.A. Kenna, M.L. Casselbrant, Laryngeal cysts: a cause of neonatal airway obstruction, Otolaryngol. Head Neck Surg. 109 (1993) 129 – 134. [7] L.W. De Santo, K.D. Devine, L.H. Welland, Cysts of the larynx — Classification, Laryngoscope 80 (1970) 145 – 176.
O.D. Smith et al. / Int. J. Pediatr. Otorhinolaryngol. 52 (2000) 277–281 [8] C.F. Ferguson, Congenital abnormalities of the infant larynx, Otolaryngol. Clin. North Am. 3 (2) (1970) 185– 200. [9] M. Jacobi, H. Rascoff, Cysts of the larynx in infancy, Am. J. Dis. Child. 49 (1935) 448–459. [10] L.A. Monday, G. Cornut, M. Bouchayer, J.B. Roch, Epidermoid cysts of the vocal cords, Ann. Otol. Rhinol. Laryngol. 92 (1983) 124–127. [11] C.L. Jackson, Benign tumours of the larynx, in: C. Jackson, C.L. Jackson (Eds.), Diseases of the Nose, Throat and Ear, Saunders, Philadelphia, PA, 1959, pp. 681–689.
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[12] L.A. Monday, G. Cornut, M. Bouchayer, J.B. Roch, R. Loire, Diagnosis and treatment of intracordal cysts, J. Otolaryngol. 10 (1981) 363 – 370. [13] G.E. Arnold, Dysplastic dysphonia: minor anomalies of the vocal cords causing persistent hoarseness, Laryngoscope 68 (1958) 142 – 158. [14] J.B. Roch, M. Bouchayer, G. Cornut, Le sulcus glottidis, Rev. Laryngol. Otol. Rhinol. (Bord.) 102 (1981) 333 – 346. [15] J. Trevin, L. Cheesebeuf, G. Cornut, A propos de 3 cas familiaux de sulcus glottides, Bull. d’audiophonol. 7 (1977) 76 – 81.