Pediatric Radiology
Pediatr. Radiol. 8, 45-47 (1979)
9 by Springer-Verlag1979
Radiographic Studies of Upper Airway Obstruction with Cor Pulmonale in a Patient with Pycnodysostosis D. K. Y o u s e f z a d e h 1, A. S. A g h a 2, a n d J. R e i n e r t s o n 3 1Section of Pediatric Radiology, Department of Radiology, 2Department of Pediatrics, the University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA
Abstract.
T h e p u r p o s e of this article is to p r e s e n t r a d i o g r a p h i c aspects of o b s t r u c t i v e a n a t o m i c a l alt e r a t i o n s of the u p p e r airway b e f o r e a n d d u r i n g sleep in a 4 I h - y e a r - o l d b o y affected with pycnodysostosis. T h e striking l e n g t h of the u v u l a a n d the i n s p i r a t i o n e x s p i r a t i o n c h a n g e s in its p o s i t i o n was a n i m p o r t a n t factor in the o b s t r u c t i o n of the u p p e r airway. F o r b e t t e r a n d earlier u n d e r s t a n d i n g of the actual m e c h a n i s m of u p p e r airway o b s t r u c t i o n , the radiological d a t a s h o u l d b e o b t a i n e d d u r i n g the same c i r c u m s t a n ces that the p a t i e n t is e x p e r i e n c i n g his m a x i m u m discomfort.
Key words: U p p e r airway o b s t r u c t i o n - P u l m o n a r y hypertension - Pycnodysostosis - Radiology Children
inspiration. His mouth remained open and there was nasal flaring and intercostal and subcostal retraction. When awakened, these would disappear. 02 desaturation and CO 2 retention were pronounced during sleep. Subsequent cardiac catheterization revealed right ventricular and pulmonary artery pressure of 50 mm of mercury, and pulmonary vein and the left atrial oxygen saturation of 89% while breathing oxygen. The latter finding indicated intrapulmonary precapillary arteriovenous shunting.
Radiographic Findings The skeletal alterations were characteristic of pycnodysostosis. A representative recent chest roentgenogram (Figure 1) showed massive generalized cardiomegaly with slight increased pulmonary vascularity. While awake, fluoroscopic examination of the upper airway in hyperextended lateral view (Figure 2) showed that during inspiration, the markedly elongated uvula moved posteriorly narrowing the nasopharyngeal airway at the level of anterior arch of CI (arrowheads). The inspiratory airflow was, therefore, established via
Case Report J. B. is a 4~/2-year-oldwhite boy who was transferred to The University of Iowa Hospitals and Clinics for evaluation of lung infiltrate and cardiomegaly. He is a known case of pycnodysostosis. He was not acutely ill until one month prior to his present admission when he experienced cough and breathing difficulty diagnosed as "bronchitis". He showed no improvement after conventional therapy and became more tachypneic. During the year prior to admission, he had had ten episodes of "bronchitis". During sleep, he snores and sweats profusely. Upon his admission, he was dyspneic and cyanotic and had a heart rate of 150 per minute and a respiration rate of 80 per minute. Echocardiogram and electrocardiogram were consistent with pulmonary hypertension. While asleep, he snored, became dusky, and had marked substernal retraction and excessive sweating. Ausculation of the trachea revealed diminished airflow during
2 Pediatric Resident 3 Senior Medical Student
Fig. 1. There is generalized cardiomegaly with slightly increased pulmonary vascularity
0301-0449/79/0008/0045/$01.00
46
D.K. Yousefzadeh et al.: Upper Airway Obstruction with Cor Pulmonale
Fig. 2. Fluoroscopic spot films taken with the patient lying on his left side show posterior motion of the elongated uvula during inspiration so that inspiratory airflow is through the mouth. During expiration the uvula blocks off the oral route and the nasopharyngeal airways opens
Fig. 3. With the patient supine a horizontal beam cross table lateral shows that during inspiration the tongue moves posteriorly and, with uvular movement, markedly disrupts oropharyngeal airflow and diminishes nasopharyngeal airflow adequately opened oropharyngeal route. The base of the tongue did not obstruct this route. During expiration, the tip of the uvula moved anteriorly narrowing the oral route and opening the nasopharyngeal airway. The oral route became narrowed only at an area where the tip of the uvula came in contact with the inferior aspect of the base of the tongue (lower double arrowheads). There was no glossoptosis occuring while awake leaving an air column in between the anterior surface of elongated uvula and the base of the tongue (between two upper arrowheads). The motion of a feathery piece of cotton which was held against the mouth and nostrils was watched. This clearly swung toward the mouth during inspiration and away from the nostrils during expiration consistent with oropharyngeal inspiration and nasopharyngeal expiration observed fluoroscopically. Following intramuscular administration of a single dose of anti-
histamine, he went to sleep. While asleep in supine position during inspiration (Figure 3), the tongue moved posteriorly (note the distance between the tip of the tongue and lower lip shown between two larger arrowheads) and the base of the tongue and anterior border of the uvula silhouetted each other due to disappearance of the air column between them. Due to combined posterior motion of the tongue and uvula the oropharyngeal breathing became completely disrupted and nasopharyngeal airflow remarkably diminshed (between two smaller arrowheads). In addition, the structures adjacent to the base of the tongue became distorted. The expiration phase demonstrated inadequate opening of nasopharyngeal airway due to insufficient combined anterior motion of the palate and the tongue. The oropharyngeal airway however, remained closed due to persistence of glossoptosis. A left lateral view of the upper airway during inspiration while
D. K. Yousefzadeh et al.: Upper Airway Obstruction with Cor Pulmonale asleep, showed that although the base of the tongue and uvula still disrupted the oral breathing, the nasopharyngeal airway opened more than it did in supine position.
Discussion
The two-stage radiographic evaluation of the upper airway in this patient, before and during sleep, demonstrated two different anatomical alterations of the upper airway structures each of which with a different consequence. 1. Striking length of the uvula and its alternating obstructive effect of nasopharynx and oropharynx, resulted in oropharyngeal inspiration and nasopharyngeal expiration, although allowing relatively adequate airflow in both phases. Since the base of the tongue did not move posteriorly while awake, the oral route was not disrupted. The pendulous motion of the feathery piece of the cotton was another indicator of adequate airflow. Despite the demonstration of anatomical alterations observed in the first awake stage of the study, the mechanism of actual upper airway obstruction was observed only in the second sleep stage. 2. The second stage of the study during sleep showed that the patient could not sustain his oropharyngeal breathing due to significant glossoptosis. The combined posterior movement of the uvula and the base of the tongue in the other hand, compromised nasopharyngeal airway, resulting in poor air exchange in both respiratory phases. This explained the auscultatory findings of the trachea and more pronounced CO 2 retention and 02 desaturation during sleep. Pulmonary hypertension and cot pulmonale have been associated with many conditions causing alveolar hypoventilation, as noted in the following list. 1. Large adenoids and tonsils [2, 5] 2. Pierre-Robin syndrome [1] 3. Subglottic stenosis 4. Cleft palate surgery 5. Crouzon's disease 6. Laryngeal hamartoma 7. Laryngotracheomalacia 8. Laryngeal web Noonan [5] and Menashe and associates [3] reported cor pulmonale due to hypertrophied and obstructing adenoids and tonsils reversable after surgery. Luke and his associates [2] proposed that
47
chronic upper airway obstruction results in alveolar hypoventilation and CO2 retention and hypoxia. This in a "susceptible" individual may cause pulmonary vasoconstriction and increase pulmonary artery resistance and pressure which leads to right heart pressure overload and hypertrophy and failure. Although Nielsen has recently described long soft palate and respiratory difficulty in patients with pycnodysostosis [4], the association of pycnodysostosis, upper airway obstruction and pulmonary hypertension has not been previously reported. May individuals with nasopharyngeal obstruction continue to breathe through the mouth during sleep. A defect in the central nervous system may be responsible for the cessation of the mouth breathing during sleep in these patients who cannot establish an adequate airflow through the nasopharynx, therefore, resulting in alveolar hypoventilation. For better and earlier understanding of the actual mechanism of the upper airway obstruction, the radiological data should be obtained during the same circumstances that the patient is experiencing his maximum discomfort. Acknowledgement. We wish to thank Dr. Victor Ionasescu for his cooperation and patient management. References 1. Cogwell, J.J., Easton, D.M.: Cor pulmonale in Pierre-Robin syndrome. Arch. Child. 49, 905 (1974) 2. Luke, M. I., Dis. Mhrizi, A., Folger, J.M., Jr., et al.: Chronic nasopharyngeal obstruction as a cause of cardiomegaly, cor pulmonale and pulmonary edema. Pediatrics 37, 762 (1966) 3. Menashe, V.E., Farrehi, C., Miller, M.: Hypoventilation and cor pulmonale due to chronic upper airway obstruction. J. Pediatr. 67, 198 (1967) 4. Nielsen, E. L.: Pycnodysostosis. Acta Paediatr. Scand. 63, 437 (1974) 5. Noonan, J.A.: Reversible cor pulmonale due to hypertrophy tonsils and adenoids studied in two cases (Abstract). Circulation 32 [Suppl. II], 164 (1965) Date of final acceptance: June 26, 1978
David K. Yousefzadeh, M. D. Assistant Professor Section of Pediatric Radiology Department of Radiology The University of Iowa Hospitals and Clinics Iowa City, IA 52242 USA
Pediatr. Radiol. 8, 45-47 (1979)
9 by Springer-Verlag1979
Radiographic Studies of Upper Airway Obstruction with Cor Pulmonale in a Patient with Pycnodysostosis D. K. Y o u s e f z a d e h 1, A. S. A g h a 2, a n d J. R e i n e r t s o n 3 1Section of Pediatric Radiology, Department of Radiology, 2Department of Pediatrics, the University of Iowa Hospitals and Clinics, Iowa City, Iowa, USA
Abstract.
T h e p u r p o s e of this article is to p r e s e n t r a d i o g r a p h i c aspects of o b s t r u c t i v e a n a t o m i c a l alt e r a t i o n s of the u p p e r airway b e f o r e a n d d u r i n g sleep in a 4 I h - y e a r - o l d b o y affected with pycnodysostosis. T h e striking l e n g t h of the u v u l a a n d the i n s p i r a t i o n e x s p i r a t i o n c h a n g e s in its p o s i t i o n was a n i m p o r t a n t factor in the o b s t r u c t i o n of the u p p e r airway. F o r b e t t e r a n d earlier u n d e r s t a n d i n g of the actual m e c h a n i s m of u p p e r airway o b s t r u c t i o n , the radiological d a t a s h o u l d b e o b t a i n e d d u r i n g the same c i r c u m s t a n ces that the p a t i e n t is e x p e r i e n c i n g his m a x i m u m discomfort.
Key words: U p p e r airway o b s t r u c t i o n - P u l m o n a r y hypertension - Pycnodysostosis - Radiology Children
inspiration. His mouth remained open and there was nasal flaring and intercostal and subcostal retraction. When awakened, these would disappear. 02 desaturation and CO 2 retention were pronounced during sleep. Subsequent cardiac catheterization revealed right ventricular and pulmonary artery pressure of 50 mm of mercury, and pulmonary vein and the left atrial oxygen saturation of 89% while breathing oxygen. The latter finding indicated intrapulmonary precapillary arteriovenous shunting.
Radiographic Findings The skeletal alterations were characteristic of pycnodysostosis. A representative recent chest roentgenogram (Figure 1) showed massive generalized cardiomegaly with slight increased pulmonary vascularity. While awake, fluoroscopic examination of the upper airway in hyperextended lateral view (Figure 2) showed that during inspiration, the markedly elongated uvula moved posteriorly narrowing the nasopharyngeal airway at the level of anterior arch of CI (arrowheads). The inspiratory airflow was, therefore, established via
Case Report J. B. is a 4~/2-year-oldwhite boy who was transferred to The University of Iowa Hospitals and Clinics for evaluation of lung infiltrate and cardiomegaly. He is a known case of pycnodysostosis. He was not acutely ill until one month prior to his present admission when he experienced cough and breathing difficulty diagnosed as "bronchitis". He showed no improvement after conventional therapy and became more tachypneic. During the year prior to admission, he had had ten episodes of "bronchitis". During sleep, he snores and sweats profusely. Upon his admission, he was dyspneic and cyanotic and had a heart rate of 150 per minute and a respiration rate of 80 per minute. Echocardiogram and electrocardiogram were consistent with pulmonary hypertension. While asleep, he snored, became dusky, and had marked substernal retraction and excessive sweating. Ausculation of the trachea revealed diminished airflow during
2 Pediatric Resident 3 Senior Medical Student
Fig. 1. There is generalized cardiomegaly with slightly increased pulmonary vascularity
0301-0449/79/0008/0045/$01.00
46
D.K. Yousefzadeh et al.: Upper Airway Obstruction with Cor Pulmonale
Fig. 2. Fluoroscopic spot films taken with the patient lying on his left side show posterior motion of the elongated uvula during inspiration so that inspiratory airflow is through the mouth. During expiration the uvula blocks off the oral route and the nasopharyngeal airways opens
Fig. 3. With the patient supine a horizontal beam cross table lateral shows that during inspiration the tongue moves posteriorly and, with uvular movement, markedly disrupts oropharyngeal airflow and diminishes nasopharyngeal airflow adequately opened oropharyngeal route. The base of the tongue did not obstruct this route. During expiration, the tip of the uvula moved anteriorly narrowing the oral route and opening the nasopharyngeal airway. The oral route became narrowed only at an area where the tip of the uvula came in contact with the inferior aspect of the base of the tongue (lower double arrowheads). There was no glossoptosis occuring while awake leaving an air column in between the anterior surface of elongated uvula and the base of the tongue (between two upper arrowheads). The motion of a feathery piece of cotton which was held against the mouth and nostrils was watched. This clearly swung toward the mouth during inspiration and away from the nostrils during expiration consistent with oropharyngeal inspiration and nasopharyngeal expiration observed fluoroscopically. Following intramuscular administration of a single dose of anti-
histamine, he went to sleep. While asleep in supine position during inspiration (Figure 3), the tongue moved posteriorly (note the distance between the tip of the tongue and lower lip shown between two larger arrowheads) and the base of the tongue and anterior border of the uvula silhouetted each other due to disappearance of the air column between them. Due to combined posterior motion of the tongue and uvula the oropharyngeal breathing became completely disrupted and nasopharyngeal airflow remarkably diminshed (between two smaller arrowheads). In addition, the structures adjacent to the base of the tongue became distorted. The expiration phase demonstrated inadequate opening of nasopharyngeal airway due to insufficient combined anterior motion of the palate and the tongue. The oropharyngeal airway however, remained closed due to persistence of glossoptosis. A left lateral view of the upper airway during inspiration while
D. K. Yousefzadeh et al.: Upper Airway Obstruction with Cor Pulmonale asleep, showed that although the base of the tongue and uvula still disrupted the oral breathing, the nasopharyngeal airway opened more than it did in supine position.
Discussion
The two-stage radiographic evaluation of the upper airway in this patient, before and during sleep, demonstrated two different anatomical alterations of the upper airway structures each of which with a different consequence. 1. Striking length of the uvula and its alternating obstructive effect of nasopharynx and oropharynx, resulted in oropharyngeal inspiration and nasopharyngeal expiration, although allowing relatively adequate airflow in both phases. Since the base of the tongue did not move posteriorly while awake, the oral route was not disrupted. The pendulous motion of the feathery piece of the cotton was another indicator of adequate airflow. Despite the demonstration of anatomical alterations observed in the first awake stage of the study, the mechanism of actual upper airway obstruction was observed only in the second sleep stage. 2. The second stage of the study during sleep showed that the patient could not sustain his oropharyngeal breathing due to significant glossoptosis. The combined posterior movement of the uvula and the base of the tongue in the other hand, compromised nasopharyngeal airway, resulting in poor air exchange in both respiratory phases. This explained the auscultatory findings of the trachea and more pronounced CO 2 retention and 02 desaturation during sleep. Pulmonary hypertension and cot pulmonale have been associated with many conditions causing alveolar hypoventilation, as noted in the following list. 1. Large adenoids and tonsils [2, 5] 2. Pierre-Robin syndrome [1] 3. Subglottic stenosis 4. Cleft palate surgery 5. Crouzon's disease 6. Laryngeal hamartoma 7. Laryngotracheomalacia 8. Laryngeal web Noonan [5] and Menashe and associates [3] reported cor pulmonale due to hypertrophied and obstructing adenoids and tonsils reversable after surgery. Luke and his associates [2] proposed that
47
chronic upper airway obstruction results in alveolar hypoventilation and CO2 retention and hypoxia. This in a "susceptible" individual may cause pulmonary vasoconstriction and increase pulmonary artery resistance and pressure which leads to right heart pressure overload and hypertrophy and failure. Although Nielsen has recently described long soft palate and respiratory difficulty in patients with pycnodysostosis [4], the association of pycnodysostosis, upper airway obstruction and pulmonary hypertension has not been previously reported. May individuals with nasopharyngeal obstruction continue to breathe through the mouth during sleep. A defect in the central nervous system may be responsible for the cessation of the mouth breathing during sleep in these patients who cannot establish an adequate airflow through the nasopharynx, therefore, resulting in alveolar hypoventilation. For better and earlier understanding of the actual mechanism of the upper airway obstruction, the radiological data should be obtained during the same circumstances that the patient is experiencing his maximum discomfort. Acknowledgement. We wish to thank Dr. Victor Ionasescu for his cooperation and patient management. References 1. Cogwell, J.J., Easton, D.M.: Cor pulmonale in Pierre-Robin syndrome. Arch. Child. 49, 905 (1974) 2. Luke, M. I., Dis. Mhrizi, A., Folger, J.M., Jr., et al.: Chronic nasopharyngeal obstruction as a cause of cardiomegaly, cor pulmonale and pulmonary edema. Pediatrics 37, 762 (1966) 3. Menashe, V.E., Farrehi, C., Miller, M.: Hypoventilation and cor pulmonale due to chronic upper airway obstruction. J. Pediatr. 67, 198 (1967) 4. Nielsen, E. L.: Pycnodysostosis. Acta Paediatr. Scand. 63, 437 (1974) 5. Noonan, J.A.: Reversible cor pulmonale due to hypertrophy tonsils and adenoids studied in two cases (Abstract). Circulation 32 [Suppl. II], 164 (1965) Date of final acceptance: June 26, 1978
David K. Yousefzadeh, M. D. Assistant Professor Section of Pediatric Radiology Department of Radiology The University of Iowa Hospitals and Clinics Iowa City, IA 52242 USA
