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Apnea and the elongated uvula
lnternationul Journal of Pediatric Otorhinolaryngology, 24 (1992) 1X3-1 89 A 1992 Elsevier Science Publishers B.V. All rights reserved 0165.5876/92/$05.00
PEDOT
1x3
00X07
Apnea and the elongated uvula
I
Sally R. Shott a and Michael J. Cunningham
b
” Department of Pediatric Otolaryngology and Muxillofac~al Surgery, Children S Hospital Medical (‘cwtc~. Bethesda and Elland Ac,enues, Cincinnati, OH and ’ Department of Otology and Laryn,gkJg?.. Massachusetts Eye and Eur Infirmuv. Boston, MA (USA)
(Revised
KKVwrds:
Apnea;
Respiratory
(Received 5 August 1991) version received 2 December (Accepted 7 December 1991)
Distress;
Uvula;
1991)
Soft palate
-
Abstract
Four cases of respiratory distress and apnea associated with an elongated uvula are presented. In all cases. the uvula was found to intermittently fall onto the epiglottis and vocal cords. In all four patients, resection of the uvula led to resolution of all airway symptoms. It is hypothesized that the uvula, touching the vocal cords, caused intermittent laryngospasm and subsequent symptoms of cough, airway obstruction, and cyanosis. The anatomic reasons for such phenomenon are discussed.
Introduction
Four children with respiratory distress and apnea associated with an elongated uvula are presented. We hypothesize that the uvula intermittently contacted the vocal cords and supraglottic structures, triggering laryngospasm with symptoms of cough, airway obstruction and cyanosis. Anatomic and physiologic mechanisms for such phenomenon are discussed. Correspondence to: S.R. Shott, M.D., Children’s Hospital Medical Center, Bethesda Avenues Cincinnati, OH 45229-2899, USA. ’ Presented at the 18th Annual Meeting of the Society of Ear. Nose and Throat Advances Washington. DC, December 7, 1990.
and
Eiland
in C’hildren
184
Fig. 1. Uvula touching epiglottis.
Case reports Case 1
B.L. presented at age 5 weeks with a history of intermittent apneic episodes. EEG and barium swallow done at age 3 weeks were both normal. At age 5 weeks she experienced two episodes of stridor and apnea associated with cyanosis. Symptoms occurred while she was sitting quietly and improved with stimulation and crying. There was no history of recent upper respiratory tract infections. She had been a full-term infant delivered without difficulty. She had no other medical problems. Her examination was most significant for her endoscopic finding. She demonstrated mild laryngomalacia with crying. At rest, her uvula was noted to rest on the superior surface of the vocal cords. She underwent a partial uvulectomy. Her postoperative course was without problems. Follow-up at 2 weeks and then 7 months later revealed no further apneic episodes. Case 2
A.S., a lo-month-old female, presented with a 3 week history of choking associated with cyanosis. Choking spells started spontaneously and were sometimes associated with coughing. She had two previous visits to the emergency room with these complaints.
185
Fig. 2. Elongated
uvula.
Airway films and pertussis cultures were both negative. Despite an admission chest x-ray revealing a mild left lower lobe infiltrate, she had clear lungs on auscultation and was afebrile throughout her hospital course. Otolaryngology consultation was obtained. Nasopharyngoscopy revealed an elongated uvula that came to rest on the superior surface of the true vocal cords (Fig. 11. She was taken to the operating room for microlaryngoscopy and bronchoscopy. Except for mild laryngomalacia, no other abnormalities were seen. The uvula was noted to be approximately 20 mm long, falling to the base of the epiglottis. A partial uvulectomy was performed (Fig. 2). Postoperatively, the patient had no further choking or cyanotic episodes and was discharged home 3 days later. 3 S.P. is a 16-day-old male who presented with a history of apnea and cyanosis occurring during sleep, with feeds and while sitting quietly. Review of systems was significant only for cough. He was the product of a 37 week gestation with normal birth. Otolaryngology consultation had been obtained while the patient was in the newborn nursery for high pitched stridor; laryngomalacia was diagnosed. In the emergency room, a 45 second episode of circumoral cyanosis was observed while he rested quietly. Nasopharyngoscopy revealed an elongated uvula that fell to the base of the epiglottis to the level of the true vocal cords. Case
186
Barium swallow and gastric reflux scan were normal. Operative microlaryngoscopy and bronchoscopy revealed mild laryngomalacia and mild tracheomalacia. A partial uvulectomy was performed. Postoperatively he experienced no further episodes of choking or cyanosis. Although he continued to have a mild high-pitched stridor consistent with his laryngomalacia, there were no further cyanotic episodes following surgery. Case 4 This 2-month-old male was hospitalized due to failure to thrive secondary to persistent feeding difficulties. During bottle feedings, he demonstrated ‘breath holding spells’ with associated nasal flaring and chest retractions. These episodes would occasionally be proceeded and/or followed by an inspiratory, stridorous sound. No cyanosis, cough or nasal regurgitation of formula were observed. He had multiple congenital problems including panhypopituitarism, left hydronephrosis and multiple cardiac anomalies. Neurologic developmental delay was observed; MRI demonstrated diffuse cortical atrophy with mild ventriculomegaly. Physical examination included malformed auricles and preauricular pits. His nose was short and broad in appearance. His hard palate was intact and higharched, and his soft palate was characterized by an extremely long uvula. Endoscopic examination revealed mild laryngomalacia. The uvula extended down to the level of the glottis. True vocal cord motion was normal bilaterally. When fed during his otolaryngologic evaluation, he was observed to become apneic with associated respiratory distress. Oxygen desaturation was documented by transcutaneous monitoring. A modified barium swallow study confirmed marked elongation of the velum. No active motion of the soft palate was documented. The uvula moved passively only with tongue motion, and essentially obstructed the passage of both thin and thickened barium from oral cavity to pharynx. No aspiration was present. A uvulectomy was performed. By the fifth postoperative day, he demonstrated improved bottle feedings and resolution of associated airway distress. Postoperative barium swallow revealed that the previously seen velar-oropharyngeal obstruction was absent with passage of barium from the oral cavity to the pharynx every two to three sucks with minimal oral residual. He again demonstrated no aspiration. His improvement was to the degree that gastrostomy tube placement was cancelled.
Discussion
These four case presentations suggest an interaction between the larynx and the soft palate, specifically the musculus uvulae. The musculus uvulae has longitudinal fibers which arise from the palatine aponeurosis and extend posteriorly, constituting the bulk of the uvula. These muscle fibers pull the uvula posteriorly, shortening it, and tensing the palate. This contraction of the uvula forms a bulge on the nasal aspect of the palate and is a major contributor to velopharyngeal closure. Although
187
numerous authors have studied the function and morphology of the paired muscle bundles that constitute the musculus uvulae, the length of the uvula has not specifically been addressed [l-3]. In an infant, the muscular components of the palate are complete and quite large relative to the size of the pharynx. In comparing the infant versus the adult pharynx, the critical difference in the infant pharynx compared to that of the adult is the spatial arrangement of the anterior wall of the pharynx [3]. The mouth is vertically shorter in the infant and therefore the uvula more closely approximates the epiglottis. The developmental growth of the larynx also contributes to this infantile uvula-epiglottis approximation. The larynx of the infant differs from the adult in regards to its anatomic location [4]. The newborn epiglottis rests at the level of the first cervical vertebra with the inferior border of the cricoid cartilage at the fourth cervical vertebra. During normal growth and associated pharyngeal elongation, the epiglottis and cricoid cartilage descend to the fourth and seventh cervical vertebrae respectively. The first three patients presented with essentially no other medical problems except for intermittent episodes of respiratory distress felt to be attributed to an elongated uvula. In these children airway compromise occurred principally at rest and interestingly was relieved by crying. This is explained by the fact that during crying the palate is retracted superiorly and posteriorly, pulling the uvula up and away from the laryngeal structures. Although the first three patients also were noted to have mild laryngomalacia, this was felt to be non-contributory because of its mild degree and because the relief of symptoms was so immediate after surgery on the uvula. Our fourth case demonstrates an adverse interaction between an elongated uvula and the larynx with subsequent feeding difficulties, as well as airway problems. In newborns a delicate balance is established between the respiratory and swallowing functions. Newborns can suckle while breathing and swallow in a rhythmic fashion between respirations. These abilities are lost with maturity. This normal infant suckle-swallow-respiration cycle appears to be sequenced under medullary control. During swallowing, respiration ceases and laryngeal closure occurs as a result of several coordinated actions. The infant’s ability to suckle and breathe simultaneously without aspiration is facilitated by the soft cartilagenous laryngeal framework at its high anatomic position in the neck. This elevated position of the larynx also promotes apposition of the epiglottis and the inferior aspect of the uvula, maintaining pharyngeal patency for nasal breathing between swallows and at rest. Although this last patient had multiple other congenital anomalies, his swallowing ability and associated respiratory distress improved immediately after performing a simple uvulectomy. In all four patients, we hypothesize that the elongated uvula triggered laryngospasm by contacting the supraglottic and glottic structures. Suzuki described laryngospasm as an exaggerated laryngeal response to stimulation of the superior laryngeal nerve [5]. Unilateral mucosal stimulation of the superior laryngeal nerve activates a sphincteric action of the larynx that is bilateral via the
188
nucleus ambiguous. A mass reflex contraction of the supraglottic structures and true vocal cords occurs. This reflex glottic closure continues long after the mucosal stimulation has ended. Laryngospasm is twice as common in the pediatric population as in the adult population [6]. Within the pediatric group, infants 1 to 3 months of age have the highest incidences of laryngospasm, being three times higher than in any other age group bl. Laryngospasm in children has many potential causes. The association between general anesthesia and laryngospasm is well documented, as is a protective reflex laryngospasm in cases of foreign body aspiration [6-91. Central deficits secondary to polio, rabies, demyelinating diseases, and rarely epilepsy can lead to laryngospasm [lo]. Though not seen often today, laryngismus stridulous is a clinical sign of tetany secondary to hypocalcemia, hypoparathyroidism and hypomagnesiumia. We wish to add to this list an abnormally elongated uvula as a cause of laryngospasm and airway distress in children.
Conclusion An elongated uvula in a child may be associated with intermittent episodes of airway distress and apnea by triggering laryngospasm when it touches the anatomically elevated laryngeal structures. Nasopharyngoscope exam is helpful in evaluating for this phenomenon, but it is important that the child be relaxed and not crying during the exam because the palate is elevated during such behavior pulling the uvula superiorly, away from the larynx. A simple uvulectomy may provide simple and immediate improvement.
References 1 Azzam, N.A. and Kuehn, D.P., The morphology of musculus uvulae. Cleft Palate J., 14 (1977) 78-87. 2 Lewin, M.L., Croft, C.B. and Shprintzen, R.J., Velopharyngeal insufficiency due to hypoplasia of the musculus uvulae and occult submucous cleft palate. Plastic Reconstr. Surg., 65 (1980) 585-591. 3 Dickson, D.R., Anatomy of the normal velopharyngeal mechanism. Clin. Plast. Surg., 2 (1975) 235-248. 4 Bosma, J.F., ‘Introduction to the symposium’ as in Symposium Development of Upper Respiratory Anatomy and Function U.S. Department of Health, Education and Welfare, 1975, No. 74-94. 5 Suzuki, M., Laryngeal spasm, a neurophysiologic redefinition. Ann. Otol., 86 (1977) 150-157. 6 Roy, W.L. and Lerman J., Laryngospasm in paediatric anesthesia. Can. J. Anaesth., 35 (1988) 93-98. 7 McGonagle, M. and Kennedy, T.L., Laryngospasm induced pulmonary edema. Larynaoscope, 94 (1984) 1583-1585. 8 Lee, K.W. and Downes, J.J., Pulmonary edema secondary to laryngospasm in children. Anesthesia, 59 (1983) 347-349.
189 9 Leicht, P., Wisborg, T. and Chraemmer-Jurgensen, B., Does intravenous lidocaine prevent laryngospasm after extubation in children? Anesth. Analg., 64 (1985) 1193-1196. 10 Amir, J., Ashkenazi, S., Schonfeld, T. et al. Latyngospasm as a single manifestation of epilepsy. Arch. Dis. Childhood., 58 (1983) 151-1.53.
PEDOT
1x3
00X07
Apnea and the elongated uvula
I
Sally R. Shott a and Michael J. Cunningham
b
” Department of Pediatric Otolaryngology and Muxillofac~al Surgery, Children S Hospital Medical (‘cwtc~. Bethesda and Elland Ac,enues, Cincinnati, OH and ’ Department of Otology and Laryn,gkJg?.. Massachusetts Eye and Eur Infirmuv. Boston, MA (USA)
(Revised
KKVwrds:
Apnea;
Respiratory
(Received 5 August 1991) version received 2 December (Accepted 7 December 1991)
Distress;
Uvula;
1991)
Soft palate
-
Abstract
Four cases of respiratory distress and apnea associated with an elongated uvula are presented. In all cases. the uvula was found to intermittently fall onto the epiglottis and vocal cords. In all four patients, resection of the uvula led to resolution of all airway symptoms. It is hypothesized that the uvula, touching the vocal cords, caused intermittent laryngospasm and subsequent symptoms of cough, airway obstruction, and cyanosis. The anatomic reasons for such phenomenon are discussed.
Introduction
Four children with respiratory distress and apnea associated with an elongated uvula are presented. We hypothesize that the uvula intermittently contacted the vocal cords and supraglottic structures, triggering laryngospasm with symptoms of cough, airway obstruction and cyanosis. Anatomic and physiologic mechanisms for such phenomenon are discussed. Correspondence to: S.R. Shott, M.D., Children’s Hospital Medical Center, Bethesda Avenues Cincinnati, OH 45229-2899, USA. ’ Presented at the 18th Annual Meeting of the Society of Ear. Nose and Throat Advances Washington. DC, December 7, 1990.
and
Eiland
in C’hildren
184
Fig. 1. Uvula touching epiglottis.
Case reports Case 1
B.L. presented at age 5 weeks with a history of intermittent apneic episodes. EEG and barium swallow done at age 3 weeks were both normal. At age 5 weeks she experienced two episodes of stridor and apnea associated with cyanosis. Symptoms occurred while she was sitting quietly and improved with stimulation and crying. There was no history of recent upper respiratory tract infections. She had been a full-term infant delivered without difficulty. She had no other medical problems. Her examination was most significant for her endoscopic finding. She demonstrated mild laryngomalacia with crying. At rest, her uvula was noted to rest on the superior surface of the vocal cords. She underwent a partial uvulectomy. Her postoperative course was without problems. Follow-up at 2 weeks and then 7 months later revealed no further apneic episodes. Case 2
A.S., a lo-month-old female, presented with a 3 week history of choking associated with cyanosis. Choking spells started spontaneously and were sometimes associated with coughing. She had two previous visits to the emergency room with these complaints.
185
Fig. 2. Elongated
uvula.
Airway films and pertussis cultures were both negative. Despite an admission chest x-ray revealing a mild left lower lobe infiltrate, she had clear lungs on auscultation and was afebrile throughout her hospital course. Otolaryngology consultation was obtained. Nasopharyngoscopy revealed an elongated uvula that came to rest on the superior surface of the true vocal cords (Fig. 11. She was taken to the operating room for microlaryngoscopy and bronchoscopy. Except for mild laryngomalacia, no other abnormalities were seen. The uvula was noted to be approximately 20 mm long, falling to the base of the epiglottis. A partial uvulectomy was performed (Fig. 2). Postoperatively, the patient had no further choking or cyanotic episodes and was discharged home 3 days later. 3 S.P. is a 16-day-old male who presented with a history of apnea and cyanosis occurring during sleep, with feeds and while sitting quietly. Review of systems was significant only for cough. He was the product of a 37 week gestation with normal birth. Otolaryngology consultation had been obtained while the patient was in the newborn nursery for high pitched stridor; laryngomalacia was diagnosed. In the emergency room, a 45 second episode of circumoral cyanosis was observed while he rested quietly. Nasopharyngoscopy revealed an elongated uvula that fell to the base of the epiglottis to the level of the true vocal cords. Case
186
Barium swallow and gastric reflux scan were normal. Operative microlaryngoscopy and bronchoscopy revealed mild laryngomalacia and mild tracheomalacia. A partial uvulectomy was performed. Postoperatively he experienced no further episodes of choking or cyanosis. Although he continued to have a mild high-pitched stridor consistent with his laryngomalacia, there were no further cyanotic episodes following surgery. Case 4 This 2-month-old male was hospitalized due to failure to thrive secondary to persistent feeding difficulties. During bottle feedings, he demonstrated ‘breath holding spells’ with associated nasal flaring and chest retractions. These episodes would occasionally be proceeded and/or followed by an inspiratory, stridorous sound. No cyanosis, cough or nasal regurgitation of formula were observed. He had multiple congenital problems including panhypopituitarism, left hydronephrosis and multiple cardiac anomalies. Neurologic developmental delay was observed; MRI demonstrated diffuse cortical atrophy with mild ventriculomegaly. Physical examination included malformed auricles and preauricular pits. His nose was short and broad in appearance. His hard palate was intact and higharched, and his soft palate was characterized by an extremely long uvula. Endoscopic examination revealed mild laryngomalacia. The uvula extended down to the level of the glottis. True vocal cord motion was normal bilaterally. When fed during his otolaryngologic evaluation, he was observed to become apneic with associated respiratory distress. Oxygen desaturation was documented by transcutaneous monitoring. A modified barium swallow study confirmed marked elongation of the velum. No active motion of the soft palate was documented. The uvula moved passively only with tongue motion, and essentially obstructed the passage of both thin and thickened barium from oral cavity to pharynx. No aspiration was present. A uvulectomy was performed. By the fifth postoperative day, he demonstrated improved bottle feedings and resolution of associated airway distress. Postoperative barium swallow revealed that the previously seen velar-oropharyngeal obstruction was absent with passage of barium from the oral cavity to the pharynx every two to three sucks with minimal oral residual. He again demonstrated no aspiration. His improvement was to the degree that gastrostomy tube placement was cancelled.
Discussion
These four case presentations suggest an interaction between the larynx and the soft palate, specifically the musculus uvulae. The musculus uvulae has longitudinal fibers which arise from the palatine aponeurosis and extend posteriorly, constituting the bulk of the uvula. These muscle fibers pull the uvula posteriorly, shortening it, and tensing the palate. This contraction of the uvula forms a bulge on the nasal aspect of the palate and is a major contributor to velopharyngeal closure. Although
187
numerous authors have studied the function and morphology of the paired muscle bundles that constitute the musculus uvulae, the length of the uvula has not specifically been addressed [l-3]. In an infant, the muscular components of the palate are complete and quite large relative to the size of the pharynx. In comparing the infant versus the adult pharynx, the critical difference in the infant pharynx compared to that of the adult is the spatial arrangement of the anterior wall of the pharynx [3]. The mouth is vertically shorter in the infant and therefore the uvula more closely approximates the epiglottis. The developmental growth of the larynx also contributes to this infantile uvula-epiglottis approximation. The larynx of the infant differs from the adult in regards to its anatomic location [4]. The newborn epiglottis rests at the level of the first cervical vertebra with the inferior border of the cricoid cartilage at the fourth cervical vertebra. During normal growth and associated pharyngeal elongation, the epiglottis and cricoid cartilage descend to the fourth and seventh cervical vertebrae respectively. The first three patients presented with essentially no other medical problems except for intermittent episodes of respiratory distress felt to be attributed to an elongated uvula. In these children airway compromise occurred principally at rest and interestingly was relieved by crying. This is explained by the fact that during crying the palate is retracted superiorly and posteriorly, pulling the uvula up and away from the laryngeal structures. Although the first three patients also were noted to have mild laryngomalacia, this was felt to be non-contributory because of its mild degree and because the relief of symptoms was so immediate after surgery on the uvula. Our fourth case demonstrates an adverse interaction between an elongated uvula and the larynx with subsequent feeding difficulties, as well as airway problems. In newborns a delicate balance is established between the respiratory and swallowing functions. Newborns can suckle while breathing and swallow in a rhythmic fashion between respirations. These abilities are lost with maturity. This normal infant suckle-swallow-respiration cycle appears to be sequenced under medullary control. During swallowing, respiration ceases and laryngeal closure occurs as a result of several coordinated actions. The infant’s ability to suckle and breathe simultaneously without aspiration is facilitated by the soft cartilagenous laryngeal framework at its high anatomic position in the neck. This elevated position of the larynx also promotes apposition of the epiglottis and the inferior aspect of the uvula, maintaining pharyngeal patency for nasal breathing between swallows and at rest. Although this last patient had multiple other congenital anomalies, his swallowing ability and associated respiratory distress improved immediately after performing a simple uvulectomy. In all four patients, we hypothesize that the elongated uvula triggered laryngospasm by contacting the supraglottic and glottic structures. Suzuki described laryngospasm as an exaggerated laryngeal response to stimulation of the superior laryngeal nerve [5]. Unilateral mucosal stimulation of the superior laryngeal nerve activates a sphincteric action of the larynx that is bilateral via the
188
nucleus ambiguous. A mass reflex contraction of the supraglottic structures and true vocal cords occurs. This reflex glottic closure continues long after the mucosal stimulation has ended. Laryngospasm is twice as common in the pediatric population as in the adult population [6]. Within the pediatric group, infants 1 to 3 months of age have the highest incidences of laryngospasm, being three times higher than in any other age group bl. Laryngospasm in children has many potential causes. The association between general anesthesia and laryngospasm is well documented, as is a protective reflex laryngospasm in cases of foreign body aspiration [6-91. Central deficits secondary to polio, rabies, demyelinating diseases, and rarely epilepsy can lead to laryngospasm [lo]. Though not seen often today, laryngismus stridulous is a clinical sign of tetany secondary to hypocalcemia, hypoparathyroidism and hypomagnesiumia. We wish to add to this list an abnormally elongated uvula as a cause of laryngospasm and airway distress in children.
Conclusion An elongated uvula in a child may be associated with intermittent episodes of airway distress and apnea by triggering laryngospasm when it touches the anatomically elevated laryngeal structures. Nasopharyngoscope exam is helpful in evaluating for this phenomenon, but it is important that the child be relaxed and not crying during the exam because the palate is elevated during such behavior pulling the uvula superiorly, away from the larynx. A simple uvulectomy may provide simple and immediate improvement.
References 1 Azzam, N.A. and Kuehn, D.P., The morphology of musculus uvulae. Cleft Palate J., 14 (1977) 78-87. 2 Lewin, M.L., Croft, C.B. and Shprintzen, R.J., Velopharyngeal insufficiency due to hypoplasia of the musculus uvulae and occult submucous cleft palate. Plastic Reconstr. Surg., 65 (1980) 585-591. 3 Dickson, D.R., Anatomy of the normal velopharyngeal mechanism. Clin. Plast. Surg., 2 (1975) 235-248. 4 Bosma, J.F., ‘Introduction to the symposium’ as in Symposium Development of Upper Respiratory Anatomy and Function U.S. Department of Health, Education and Welfare, 1975, No. 74-94. 5 Suzuki, M., Laryngeal spasm, a neurophysiologic redefinition. Ann. Otol., 86 (1977) 150-157. 6 Roy, W.L. and Lerman J., Laryngospasm in paediatric anesthesia. Can. J. Anaesth., 35 (1988) 93-98. 7 McGonagle, M. and Kennedy, T.L., Laryngospasm induced pulmonary edema. Larynaoscope, 94 (1984) 1583-1585. 8 Lee, K.W. and Downes, J.J., Pulmonary edema secondary to laryngospasm in children. Anesthesia, 59 (1983) 347-349.
189 9 Leicht, P., Wisborg, T. and Chraemmer-Jurgensen, B., Does intravenous lidocaine prevent laryngospasm after extubation in children? Anesth. Analg., 64 (1985) 1193-1196. 10 Amir, J., Ashkenazi, S., Schonfeld, T. et al. Latyngospasm as a single manifestation of epilepsy. Arch. Dis. Childhood., 58 (1983) 151-1.53.