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Failure to thrive caused by the coexistence of vallecular cyst, laryngomalacia and gastroesophageal reflux in an infant
International Journal of Pediatric Otorhinolaryngology (2004) 68, 1459—1464
www.elsevier.com/locate/ijporl
CASE REPORT
Failure to thrive caused by the coexistence of vallecular cyst, laryngomalacia and gastroesophageal reflux in an infant Tsung-Chieh Yaoa,b, Chih-Yung Chiua, Kun-Chan Wua, Li-Jen Wua, Jing-Long Huanga,* a
Department of Pediatrics, Chang Gung Children’s Hospital, 5 Fu-Hsin Street, Kweishan, Taoyuan, Taiwan Graduate Institute of Clinical Medical Science, Chang Gung University, Taoyuan, Taiwan
b
Received 12 May 2004; accepted 26 June 2004
KEYWORDS Vallecular cyst; Stridor; Larynomalacia; Gastroesophageal reflux; Failure to thrive
Summary: Vallecular cyst is a rare but dangerous cause of stridor in neonates and young infants. Without recognition and proper therapy, the resulting airway obstruction can lead to serious morbidity and mortality. We herein report a young infant with a vallecular cyst synchronous with laryngomalacia and gastroesophageal reflux. The infant initially presented with signs and symptoms mimicking more benign laryngomalacia. Diagnosis was delayed with accompanying failure to thrive. The cyst was revealed by a barium esophagogram and confirmed by flexible bronchoscopy. Laryngomicrosurgery with CO2 laser not only removed the cyst, but resolved the coexisting laryngomalacia and gastroesophageal relfux as well. After surgery, the infant was symptom free and exhibited a steady weight gain. There was no recurrence during 1 year of follow-up. We have reviewed the literature for this rare and potentially devastating lesion. # 2004 Elsevier Ireland Ltd. All rights reserved.
1. Introduction Vallecular cyst is a rare but potentially devastating cause of stridor in neonates and young infants [1]. There appears to be two clinical entities in the presentation of vallecular cyst, either adult or infantile type. In adults, quite often they are inciAbbreviations: GER, gastroesophageal reflux * Corresponding author. Tel.: +886 3 3281200x8206; fax: +886 3 3274843. E-mail address: [email protected] (J.-L. Huang).
dental findings or only subtle symptoms are present [2]. In contrast, the cyst may leads to stridor and/or respiratory distress in neonates and young infants because of their relative small airway and there is a greater risk for life-threatening airway obstruction. In the past, a significant number of these cysts were identified only at autopsy [3]. We describe a young infant with a vallecular cyst synchronous with laryngomalacia and gastroesophageal reflux (GER), initially presenting with signs and symptoms mimicking more benign laryngomalacia. Diagnosis was delayed with accompanying failure to thrive.
0165-5876/$ — see front matter # 2004 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijporl.2004.06.004
1460
Vallecular cyst in combination with laryngomalacia and gastroesophageal reflux is an extremely rare clinical condition according to the literature. We propose a possible interaction of these disorders and emphasize that vallecular cyst should be included in the differential diagnosis of stridor in young infants, especially when feeding difficulties or failure to thrive are present.
2. Case report On 14 August 2002, a female infant aged 11 weeks was referred by a local pediatrician for evaluation of progressively worsening stridor and failure to thrive. The patient was born uneventfully at full term via normal spontaneous delivery and there were no perinatal problems; the birth weight was 2.8 kg. Her mother had noticed noisy breathing since birth and mild inspiratory stridor was noted in the nursery. The stridor worsened with agitation, supine position, or during feeding attempts and significantly decreased in the prone position or during sleep. These symptoms were attributed to laryngomalacia.
T.-C. Yao et al.
The infant was discharged from the hospital 3 days after birth but experienced progressive inspiratory stridor and respiratory difficulty at home. Feeding difficulty was present since 6 weeks of age and, although the infant sucked adequately initially, she refused to eat after 20—30 ml of formula feeding with exhaustion, and often vomited postprandially. Choking episodes occurred frequently during milk feeding. However, no fever, cough, hoarseness, or cyanosis was noted. The infant gained 1.5 kg during the first 6 weeks at home but failed to increase in weight during the subsequent 5 weeks. The infant was examined by different pediatricians, diagnosing as having laryngomalacia without laryngoscopic confirmation, and the parents were reassured. At 11 weeks of age, she was referred for further investigation. On admission, physical examination revealed an alert infant with the following results: body temperature, 37.2 8C; heart rate, 138 beats/min; respiratory rate, 42 breaths/min; blood pressure, 97/66 mmHg; body weight 4 kg (<3rd percentile); and body height 57 cm (50th percentile). The infant had low-pitched inspiratory stridor and expiratory
Fig. 1 (A) Barium esophagogram shows a large vallecular cyst (arrowheads); (B) gastroesophageal reflux up to the cervical level.
Vallecular cyst synchronous with laryngomalacia and gastroesophageal reflux
wheezing at rest, with nasal flaring, suprasternal and subcostal retractions. Her neck was supple without any palpable mass and her abdomen was distended. There were no cutaneous hemangiomas. The remainder of the physical examination was normal. Results of laboratory testing disclosed the following results: hemoglobin, 11.7 g/dl; white blood cell count, 8300/ml, with a normal differential cell count; and a platelet count, 378,000/ml. The admission chest roentgenogram demonstrated relatively high diaphragms with hypoaerated lungs and an obviously gaseous abdomen. A barium esophagogram (Fig. 1) clearly revealed a rounded, sharply defined opacity at the base of the tongue and GER up to the cervical level. A 24-h esophageal pH monitoring provided evidence of association for the choking spells with a fall of pH in the esophagus. Flexible bronchoscopy revealed a 2 cm cystic mass situated in the midline of the vallecular space and an omega-shaped, inferiorly and posteriorly displaced epiglottis that was flaccid and sucked into the laryngeal introitus during inspirations. The trachea and bronchi were normal. On 19 August, the cyst was removed by microlaryngoscopic excision with CO2 laser. Histology revealed a cyst lined by squamous epithelium with evidence of chronic inflammation. No thyroid tissue was found in the specimen. The vallecular cyst fluid was sterile. The post-operative course was uneventful. Two days after surgery respiratory distress resolved and no more stridor or wheezing was noted thereafter. The patient was discharged from the hospital in a good condition 9 days after
Fig. 2 Serial changes of weights relation to 10th, 50th and 90th centiles.
1461
admission. A 24-h esophageal pH monitoring performed 2 weeks after cyst removal revealed no more evidence of GER. From then the patient exhibit a steady weight gain. Fig. 2 shows her weight from birth, in relation to the 10th, 50th and 90th percentiles. The delay in weight gain until surgery is clear. There was no evidence of recurrence and she was in perfect health during 1 year of follow-up.
3. Discussion Laryngomalacia is a common disorder in clinical practice, which accounts for up to 75% of the stridor seen in neonates and young infants [4]. It is generally believed to be a benign and notneed-to-treat disorder [4]. Stridor and noisy breathing are commonly present shortly after birth. Feeding difficulties and respiratory distress occur occasionally. The prognosis is excellent and most patients are symptom free by 18—24 months of age. Many pediatricians do not request extensive airway evaluation in infants with clinical diagnosis of laryngomalacia based on the assumption that most of affected infants will resolve without therapy. Despite the frequent occurrence of laryngomalacia and its generally benign nature, the importance of considering other causes of stridor is demonstrated by this case. This infant had a congenital vallecular cyst which obstructed her airway and she might have had a acute airway obstruction or even sudden death if the lesion had not been noticed by the pediatrician. Despite that, her initial history and physical examination were compatible with more benign laryngomalacia. It has been demonstrated that synchronous airway lesions, one of which is a laryngeal cyst, are identified in 12—45% of infants with laryngomalacia [5] and frequently associated with complicated courses. Congenital laryngeal cyst is a rare disorder, first reported by Abercrombie in 1881, with an estimated annual incidence of 1 per 300,000 and 1.82 per 100,000 births in some selected populations [6,7]. A number of hypotheses have been used to explain the pathogenesis of laryngeal cysts. DeSanto et al. [8] have provided a comprehensive review of the etiologic mechanisms and classified laryngeal cysts into two major divisions, ductal and saccular types. The ductal type, mostly situated in the vallecula, appears to originate from obstruction of submucosal gland ducts; whereas the saccular type occurs in the plane of saccule as a result of a cystic distension of that laryngeal appendage. According to its location, the most common laryngeal cyst in infants is
1462
the aryepiglottic cyst, followed by the vallecular cyst [9]. There have very few reports of congenital vallecular cysts in the literature [1,9—16]. Vallecular cysts were described as containing clear, nonpurulent fluid, and malignant transformation has not been reported [2]. There is no gender predominance [1,10] and the bimodal age of presentation suggests that there may be two clinical forms of vallecular cyst, with adult and infantile vallecular cyst possibly explained by different pathogenesis [1]. In adults, quite often they are incidental findings [2]. Vallecular cyst is considered to be relatively rare in infants than in adults [1,8,10]. All affected infants developed symptoms during the first weeks of life. Inspiratory stridor and varying degrees of upper airway obstruction are the most frequently described presentations [1,10]. Other common findings included feeding difficulties and episodes of cyanosis. Additionally, failure to thrive is not uncommon if diagnosis is beyond the first week of life [1,10,12]. In accordance with previous studies, our patient presented with stridor and noisy breathing shortly after birth. The symptoms of our case were significantly associated with position changes that worsened with supine position and decreased with prone position, perhaps resulting from the backward or forward movement of the cyst. However, it is worthy noting that alarming symptoms such as poor feeding, cyanosis on feeding and failure to thrive are easily overlooked [9,12]. Therefore, the diagnosis is usually delayed with rare exception [7] and the duration of symptoms prior to diagnosis was 11 weeks in our case. In the past, vallecular cysts were frequently overlooked, and as many as 50% were discovered at autopsy [3]. Recognition of this rare entity requires a high index of suspicion. A lateral neck X-ray is helpful but can be difficult to interpret in our experience. The widely available barium esophagogram remains a valuable examination for showing structural and functional esophageal lesions and provides a first clue of vallecular cyst in our case. For confirmation direct visualization by either laryngoscopic or bronchoscopic examination is sufficient. Flexible bronchoscopy if available is superior to flexible laryngoscopy because about 12—45% of the patients with laryngomalacia have associated anomalies in the lower tracheobronchial tree [16]. If the mass proves to be cystic and localized specifically on the lingual surface of the epiglottis without any other distinguishing features, no further evaluation is necessary [1]. Otherwise, further investigation such as thyroid scan, computed tomography, or magnetic resonance imaging should be considered to rule out the possibility of other mass lesions in the vallecular region including hemangioma, lymphangioma, ter-
T.-C. Yao et al.
atoma, dermoid cyst, thyroglossal duct cyst, and lingular thyroid [1,10]. Treatment of vallecular cysts is by surgical removal. Most vallecular cysts improve symptomatically after operation. Simple aspiration of the cyst should be abandoned because of high recurrence rate [9] unless for decompression purpose for severe airway obstruction. Alternative treatment modalities, including endoscopic marsupialization, excision and de-roofing have been advocated. We recommend removing the cyst by CO2 laser because it could vaporize the lining epithelium after excision of the wall with minimal airway morbidity [16]. Our patient tolerated the surgery well without operative morbidity and there was no recurrence during 1 year of follow-up. Established or incipient airway obstruction is the most important complication of vallecular cysts. Airway obstruction is, in part, caused by the mass effect in the hypopharynx and, perhaps more importantly, by possible inferior and posterior displacement of the epiglottis causing supraglottic obstruction [17]. To the best of our knowledge, vallecular cyst associated with laryngomalacia and GER, as the infant we described, has been reported only once by Wong et al. [16]. However, they did not mention the role of GER in their case. Infants with vallecular cysts may manifest a secondary form of laryngomalacia. This can be explained by that altered airway dynamics caused by a progressively enlarging cyst may elevate inspiratory negative pressures, contributing to supraglottic prolapse and a secondary form of laryngomalacia. As shown in an animal model [18], it is reasonable to suggest that GER in our case is secondary to upper airway obstruction that induces wide swings in intrathoracic and abdominal pressures, which overcome the antireflux barrier. On the other hand, gastroesophageal reflux may be an additionally significant cofactor exacerbating the degree of preexisting airway compromise in our case. Many authors have documented the detrimental effects of acid and accompanying pepsin in the larynx and tracheobronchial tree. There are four mechanisms. First, acid itself can induce mucosal damage of the upper airway. Second, aspiration of gastric contents during an episode of GER may directly result in laryngospasm [19]. Third, upper airway or bronchial spasm may be induced by means of reflex pathways without direct involvement of acid on the upper airway mucosa [19]. Fourth, esophageal pain during GER may produce marked irritability resulting in more rapid or forceful breathing, thus producing a relative airway narrowing on an already compromised larynx [19]. In fact, GER was reported to be highly prevalent among more than half of infants
Vallecular cyst synchronous with laryngomalacia and gastroesophageal reflux
with large-airways malacia [19,20]. Coexistence of GER, although rarely reported in previous studies, may be overlooked and may contribute to the pathogenesis of complicated courses of congenital vallecular cysts. Furthermore, immaturity of the gastroesophageal junction in young infants makes them susceptible to GER and this may partly explaine why symptomatic vallecular cysts are usually seen in young infants. Furthermore, removal of the vallecular cyst in our case coincided with the rapid resolution of laryngomalacia and GER. This further strengthens the hypothesis that these two disorders are secondary to the vallecular cyst. A consequence of events (Fig. 3) would appear to explain the clinical course of the child in this report. There was initially no respiratory difficulty nor obvious feeding problem, but only mild stridor. Mucus production induced further expansion of the cyst, which produced progressive upper airway obstruction and secondary laryngomalacia. Subsequent altered respiratory mechanics and negative intrathoracic pressures caused by partial obstruction lead to GER, which results in feeding difficulties and further exacerbates the degree of airway compromise. A vicious cycle was thus set up with more obstruction, followed by more reflux and even more obstruction. Finally, failure to gain weight became apparent and there was a greater risk for life-threatening airway obstruction. This progressive interference with
Fig. 3 A vicious cycle describes the possible interaction of vallecular cyst, laryngomalacia, and gastroesophageal reflux.
1463
upper aerodigestive tract function correlates well with the postulated expansion of the vallecular cyst, which reached the considerable size of 2 cm in diameter. Cyst removal broke this vicious cycle and also improved the symptoms caused by coexisting laryngomalacia and GER. In summary, once an infant with congenital stridor has concomitant feeding difficulties and/or failure to thrive, evaluation should include either barium swallow or endoscopy. GER should be considered in any case of upper airway obstruction. Vallecular cysts may coexist with laryngomalacia and GER, making the clinical course much more complicated. Laryngomicrosurgery with CO2 laser not only removed the cyst, but resolved the coexisting laryngomalacia and GER as well.
References [1] J.P. Gutierrez, R.G. Berkowitz, C.F. Robertson, Vallecular cysts in newborns and young infants, Pediatr. Pulmonol. 27 (1999) 282—285. [2] R.J. Ruben, S.A. Kucinski, N. Greenstein, Cystic lymphangioma of the vallecula, Can J. Otolaryngol. 4 (1975) 180— 184. [3] A.L. Abramson, B. Zielinski, Congenital laryngeal saccular cyst of the newborn, Laryngoscope 94 (1984) 1580—1582. [4] D.R. Olney, J.H. Greinwald Jr., E.J.H. Smith, N.M. Bauman, Laryngomalacia and its treatment, Laryngoscope 109 (1999) 1170—1175. [5] R.F. Mancuso, S.S. Choi, G.H. Zalzal, K.M. Grundfast, Laryngomalacia. The search for the second lesion, Arch. Otolaryngol. Head Neck. Surg. 122 (1996) 302—306. [6] H. Suhonen, P.O. Kero, H. Puhakka, P. Vilkki, Saccular cyst of the larynx in infants, Int. J. Pediatr. Otorhinolaryngol. 8 (1984) 73—78. [7] M.W. Pak, J.K. Woo, C.A. van Hasselt, Congenital laryngeal cysts: current approach to management, J. Laryngol. Otol. 110 (1996) 854—856. [8] L.W. DeSanto, K.D. Devine, L.H. Weiland, Cysts of the larynx-classification, Laryngoscope 80 (1970) 145—176. [9] D.B. Mitchell, B.C. Irwin, C.M. Bailey, JN.G. Evans, Cysts of the infant larynx, J. Laryngol. Otol. 101 (1987) 833—838. [10] W.S. Hsieh, P.H. Yang, K.S. Wong, H.Y. Li, E.C.R. Wang, T.F. Yeh, Eur. J. Pediatr. 159 (2000) 79—81. [11] A.S. Ku, Vallecular cyst: report of four cases-one with coexisting laryngomalacia, J. Laryngol. Otol. 114 (2000) 224— 226. [12] P.G.C. Gluckman, T.W.F. Chu, C.A. van Hasselt, Neonatal vallecular cysts and failure to thrive, J. Laryngol. Otol. 106 (1992) 448—449. [13] C.M. Myer, Vallecular cyst in the newborn, Ear Nose Throat 67 (1988) 122—124. [14] M. Oluwell, Congenital vallecular cyst: a rare cause of failure to thrive, Br. J. Clin. Pract. 50 (1996) 170. [15] C.R. Wang, K.E. Lim, Vallecular cysts: report of two cases, Pediatr. Radiol. 25 (1995) S218—S219. [16] K.S. Wong, H.Y. Li, T.S. Huang, Vallecular cyst synchronous with laryngomalacia: presentation of two cases, Otolaryngol. Head Neck Surg. 113 (1995) 621—624.
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[17] J. LaBagnara Jr., Cysts of the base of the tongue in infants: an unusual cause of neonatal airway obstruction, Otolaryngol. Head Neck Surg. 101 (1989) 108—111. [18] W. Wang, J.A. Tovar, I. Eizaguirre, P. Aldazabal, Airway obstruction and gastroesophageal reflux: an experimental study on the pathogenesis of the association, J. Pediatr. Surg. 28 (1993) 995—998.
T.-C. Yao et al.
[19] R.F. Yellon, H. Goldberg, Update on gastroesophageal reflux disease in pediatric airway disorders, Am. J. Med. 111 (2001) 78S—84S. [20] H. Bibi, E. Khvolis, D. Shoseyov, M. Ohaly, D.B. Dor, D. London, et al. The prevalence of gastroesophageal reflux in children with tracheomalacia and laryngomalacia, Chest 119 (2001) 409—413.
www.elsevier.com/locate/ijporl
CASE REPORT
Failure to thrive caused by the coexistence of vallecular cyst, laryngomalacia and gastroesophageal reflux in an infant Tsung-Chieh Yaoa,b, Chih-Yung Chiua, Kun-Chan Wua, Li-Jen Wua, Jing-Long Huanga,* a
Department of Pediatrics, Chang Gung Children’s Hospital, 5 Fu-Hsin Street, Kweishan, Taoyuan, Taiwan Graduate Institute of Clinical Medical Science, Chang Gung University, Taoyuan, Taiwan
b
Received 12 May 2004; accepted 26 June 2004
KEYWORDS Vallecular cyst; Stridor; Larynomalacia; Gastroesophageal reflux; Failure to thrive
Summary: Vallecular cyst is a rare but dangerous cause of stridor in neonates and young infants. Without recognition and proper therapy, the resulting airway obstruction can lead to serious morbidity and mortality. We herein report a young infant with a vallecular cyst synchronous with laryngomalacia and gastroesophageal reflux. The infant initially presented with signs and symptoms mimicking more benign laryngomalacia. Diagnosis was delayed with accompanying failure to thrive. The cyst was revealed by a barium esophagogram and confirmed by flexible bronchoscopy. Laryngomicrosurgery with CO2 laser not only removed the cyst, but resolved the coexisting laryngomalacia and gastroesophageal relfux as well. After surgery, the infant was symptom free and exhibited a steady weight gain. There was no recurrence during 1 year of follow-up. We have reviewed the literature for this rare and potentially devastating lesion. # 2004 Elsevier Ireland Ltd. All rights reserved.
1. Introduction Vallecular cyst is a rare but potentially devastating cause of stridor in neonates and young infants [1]. There appears to be two clinical entities in the presentation of vallecular cyst, either adult or infantile type. In adults, quite often they are inciAbbreviations: GER, gastroesophageal reflux * Corresponding author. Tel.: +886 3 3281200x8206; fax: +886 3 3274843. E-mail address: [email protected] (J.-L. Huang).
dental findings or only subtle symptoms are present [2]. In contrast, the cyst may leads to stridor and/or respiratory distress in neonates and young infants because of their relative small airway and there is a greater risk for life-threatening airway obstruction. In the past, a significant number of these cysts were identified only at autopsy [3]. We describe a young infant with a vallecular cyst synchronous with laryngomalacia and gastroesophageal reflux (GER), initially presenting with signs and symptoms mimicking more benign laryngomalacia. Diagnosis was delayed with accompanying failure to thrive.
0165-5876/$ — see front matter # 2004 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijporl.2004.06.004
1460
Vallecular cyst in combination with laryngomalacia and gastroesophageal reflux is an extremely rare clinical condition according to the literature. We propose a possible interaction of these disorders and emphasize that vallecular cyst should be included in the differential diagnosis of stridor in young infants, especially when feeding difficulties or failure to thrive are present.
2. Case report On 14 August 2002, a female infant aged 11 weeks was referred by a local pediatrician for evaluation of progressively worsening stridor and failure to thrive. The patient was born uneventfully at full term via normal spontaneous delivery and there were no perinatal problems; the birth weight was 2.8 kg. Her mother had noticed noisy breathing since birth and mild inspiratory stridor was noted in the nursery. The stridor worsened with agitation, supine position, or during feeding attempts and significantly decreased in the prone position or during sleep. These symptoms were attributed to laryngomalacia.
T.-C. Yao et al.
The infant was discharged from the hospital 3 days after birth but experienced progressive inspiratory stridor and respiratory difficulty at home. Feeding difficulty was present since 6 weeks of age and, although the infant sucked adequately initially, she refused to eat after 20—30 ml of formula feeding with exhaustion, and often vomited postprandially. Choking episodes occurred frequently during milk feeding. However, no fever, cough, hoarseness, or cyanosis was noted. The infant gained 1.5 kg during the first 6 weeks at home but failed to increase in weight during the subsequent 5 weeks. The infant was examined by different pediatricians, diagnosing as having laryngomalacia without laryngoscopic confirmation, and the parents were reassured. At 11 weeks of age, she was referred for further investigation. On admission, physical examination revealed an alert infant with the following results: body temperature, 37.2 8C; heart rate, 138 beats/min; respiratory rate, 42 breaths/min; blood pressure, 97/66 mmHg; body weight 4 kg (<3rd percentile); and body height 57 cm (50th percentile). The infant had low-pitched inspiratory stridor and expiratory
Fig. 1 (A) Barium esophagogram shows a large vallecular cyst (arrowheads); (B) gastroesophageal reflux up to the cervical level.
Vallecular cyst synchronous with laryngomalacia and gastroesophageal reflux
wheezing at rest, with nasal flaring, suprasternal and subcostal retractions. Her neck was supple without any palpable mass and her abdomen was distended. There were no cutaneous hemangiomas. The remainder of the physical examination was normal. Results of laboratory testing disclosed the following results: hemoglobin, 11.7 g/dl; white blood cell count, 8300/ml, with a normal differential cell count; and a platelet count, 378,000/ml. The admission chest roentgenogram demonstrated relatively high diaphragms with hypoaerated lungs and an obviously gaseous abdomen. A barium esophagogram (Fig. 1) clearly revealed a rounded, sharply defined opacity at the base of the tongue and GER up to the cervical level. A 24-h esophageal pH monitoring provided evidence of association for the choking spells with a fall of pH in the esophagus. Flexible bronchoscopy revealed a 2 cm cystic mass situated in the midline of the vallecular space and an omega-shaped, inferiorly and posteriorly displaced epiglottis that was flaccid and sucked into the laryngeal introitus during inspirations. The trachea and bronchi were normal. On 19 August, the cyst was removed by microlaryngoscopic excision with CO2 laser. Histology revealed a cyst lined by squamous epithelium with evidence of chronic inflammation. No thyroid tissue was found in the specimen. The vallecular cyst fluid was sterile. The post-operative course was uneventful. Two days after surgery respiratory distress resolved and no more stridor or wheezing was noted thereafter. The patient was discharged from the hospital in a good condition 9 days after
Fig. 2 Serial changes of weights relation to 10th, 50th and 90th centiles.
1461
admission. A 24-h esophageal pH monitoring performed 2 weeks after cyst removal revealed no more evidence of GER. From then the patient exhibit a steady weight gain. Fig. 2 shows her weight from birth, in relation to the 10th, 50th and 90th percentiles. The delay in weight gain until surgery is clear. There was no evidence of recurrence and she was in perfect health during 1 year of follow-up.
3. Discussion Laryngomalacia is a common disorder in clinical practice, which accounts for up to 75% of the stridor seen in neonates and young infants [4]. It is generally believed to be a benign and notneed-to-treat disorder [4]. Stridor and noisy breathing are commonly present shortly after birth. Feeding difficulties and respiratory distress occur occasionally. The prognosis is excellent and most patients are symptom free by 18—24 months of age. Many pediatricians do not request extensive airway evaluation in infants with clinical diagnosis of laryngomalacia based on the assumption that most of affected infants will resolve without therapy. Despite the frequent occurrence of laryngomalacia and its generally benign nature, the importance of considering other causes of stridor is demonstrated by this case. This infant had a congenital vallecular cyst which obstructed her airway and she might have had a acute airway obstruction or even sudden death if the lesion had not been noticed by the pediatrician. Despite that, her initial history and physical examination were compatible with more benign laryngomalacia. It has been demonstrated that synchronous airway lesions, one of which is a laryngeal cyst, are identified in 12—45% of infants with laryngomalacia [5] and frequently associated with complicated courses. Congenital laryngeal cyst is a rare disorder, first reported by Abercrombie in 1881, with an estimated annual incidence of 1 per 300,000 and 1.82 per 100,000 births in some selected populations [6,7]. A number of hypotheses have been used to explain the pathogenesis of laryngeal cysts. DeSanto et al. [8] have provided a comprehensive review of the etiologic mechanisms and classified laryngeal cysts into two major divisions, ductal and saccular types. The ductal type, mostly situated in the vallecula, appears to originate from obstruction of submucosal gland ducts; whereas the saccular type occurs in the plane of saccule as a result of a cystic distension of that laryngeal appendage. According to its location, the most common laryngeal cyst in infants is
1462
the aryepiglottic cyst, followed by the vallecular cyst [9]. There have very few reports of congenital vallecular cysts in the literature [1,9—16]. Vallecular cysts were described as containing clear, nonpurulent fluid, and malignant transformation has not been reported [2]. There is no gender predominance [1,10] and the bimodal age of presentation suggests that there may be two clinical forms of vallecular cyst, with adult and infantile vallecular cyst possibly explained by different pathogenesis [1]. In adults, quite often they are incidental findings [2]. Vallecular cyst is considered to be relatively rare in infants than in adults [1,8,10]. All affected infants developed symptoms during the first weeks of life. Inspiratory stridor and varying degrees of upper airway obstruction are the most frequently described presentations [1,10]. Other common findings included feeding difficulties and episodes of cyanosis. Additionally, failure to thrive is not uncommon if diagnosis is beyond the first week of life [1,10,12]. In accordance with previous studies, our patient presented with stridor and noisy breathing shortly after birth. The symptoms of our case were significantly associated with position changes that worsened with supine position and decreased with prone position, perhaps resulting from the backward or forward movement of the cyst. However, it is worthy noting that alarming symptoms such as poor feeding, cyanosis on feeding and failure to thrive are easily overlooked [9,12]. Therefore, the diagnosis is usually delayed with rare exception [7] and the duration of symptoms prior to diagnosis was 11 weeks in our case. In the past, vallecular cysts were frequently overlooked, and as many as 50% were discovered at autopsy [3]. Recognition of this rare entity requires a high index of suspicion. A lateral neck X-ray is helpful but can be difficult to interpret in our experience. The widely available barium esophagogram remains a valuable examination for showing structural and functional esophageal lesions and provides a first clue of vallecular cyst in our case. For confirmation direct visualization by either laryngoscopic or bronchoscopic examination is sufficient. Flexible bronchoscopy if available is superior to flexible laryngoscopy because about 12—45% of the patients with laryngomalacia have associated anomalies in the lower tracheobronchial tree [16]. If the mass proves to be cystic and localized specifically on the lingual surface of the epiglottis without any other distinguishing features, no further evaluation is necessary [1]. Otherwise, further investigation such as thyroid scan, computed tomography, or magnetic resonance imaging should be considered to rule out the possibility of other mass lesions in the vallecular region including hemangioma, lymphangioma, ter-
T.-C. Yao et al.
atoma, dermoid cyst, thyroglossal duct cyst, and lingular thyroid [1,10]. Treatment of vallecular cysts is by surgical removal. Most vallecular cysts improve symptomatically after operation. Simple aspiration of the cyst should be abandoned because of high recurrence rate [9] unless for decompression purpose for severe airway obstruction. Alternative treatment modalities, including endoscopic marsupialization, excision and de-roofing have been advocated. We recommend removing the cyst by CO2 laser because it could vaporize the lining epithelium after excision of the wall with minimal airway morbidity [16]. Our patient tolerated the surgery well without operative morbidity and there was no recurrence during 1 year of follow-up. Established or incipient airway obstruction is the most important complication of vallecular cysts. Airway obstruction is, in part, caused by the mass effect in the hypopharynx and, perhaps more importantly, by possible inferior and posterior displacement of the epiglottis causing supraglottic obstruction [17]. To the best of our knowledge, vallecular cyst associated with laryngomalacia and GER, as the infant we described, has been reported only once by Wong et al. [16]. However, they did not mention the role of GER in their case. Infants with vallecular cysts may manifest a secondary form of laryngomalacia. This can be explained by that altered airway dynamics caused by a progressively enlarging cyst may elevate inspiratory negative pressures, contributing to supraglottic prolapse and a secondary form of laryngomalacia. As shown in an animal model [18], it is reasonable to suggest that GER in our case is secondary to upper airway obstruction that induces wide swings in intrathoracic and abdominal pressures, which overcome the antireflux barrier. On the other hand, gastroesophageal reflux may be an additionally significant cofactor exacerbating the degree of preexisting airway compromise in our case. Many authors have documented the detrimental effects of acid and accompanying pepsin in the larynx and tracheobronchial tree. There are four mechanisms. First, acid itself can induce mucosal damage of the upper airway. Second, aspiration of gastric contents during an episode of GER may directly result in laryngospasm [19]. Third, upper airway or bronchial spasm may be induced by means of reflex pathways without direct involvement of acid on the upper airway mucosa [19]. Fourth, esophageal pain during GER may produce marked irritability resulting in more rapid or forceful breathing, thus producing a relative airway narrowing on an already compromised larynx [19]. In fact, GER was reported to be highly prevalent among more than half of infants
Vallecular cyst synchronous with laryngomalacia and gastroesophageal reflux
with large-airways malacia [19,20]. Coexistence of GER, although rarely reported in previous studies, may be overlooked and may contribute to the pathogenesis of complicated courses of congenital vallecular cysts. Furthermore, immaturity of the gastroesophageal junction in young infants makes them susceptible to GER and this may partly explaine why symptomatic vallecular cysts are usually seen in young infants. Furthermore, removal of the vallecular cyst in our case coincided with the rapid resolution of laryngomalacia and GER. This further strengthens the hypothesis that these two disorders are secondary to the vallecular cyst. A consequence of events (Fig. 3) would appear to explain the clinical course of the child in this report. There was initially no respiratory difficulty nor obvious feeding problem, but only mild stridor. Mucus production induced further expansion of the cyst, which produced progressive upper airway obstruction and secondary laryngomalacia. Subsequent altered respiratory mechanics and negative intrathoracic pressures caused by partial obstruction lead to GER, which results in feeding difficulties and further exacerbates the degree of airway compromise. A vicious cycle was thus set up with more obstruction, followed by more reflux and even more obstruction. Finally, failure to gain weight became apparent and there was a greater risk for life-threatening airway obstruction. This progressive interference with
Fig. 3 A vicious cycle describes the possible interaction of vallecular cyst, laryngomalacia, and gastroesophageal reflux.
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upper aerodigestive tract function correlates well with the postulated expansion of the vallecular cyst, which reached the considerable size of 2 cm in diameter. Cyst removal broke this vicious cycle and also improved the symptoms caused by coexisting laryngomalacia and GER. In summary, once an infant with congenital stridor has concomitant feeding difficulties and/or failure to thrive, evaluation should include either barium swallow or endoscopy. GER should be considered in any case of upper airway obstruction. Vallecular cysts may coexist with laryngomalacia and GER, making the clinical course much more complicated. Laryngomicrosurgery with CO2 laser not only removed the cyst, but resolved the coexisting laryngomalacia and GER as well.
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