Congenital Ankylosis of the Temporomandibular Joint* Resultant Upper Airway Obstruction and Cor Pulmonale Luis E. Alday, M.D.; Pedro/. Vega, M.D.; and Angel Heller, M.D.

Cor pulmonale and severe congestive heart faDure secondary to chronic upper airway obsfruction developed in a three-year-old girl with congenital ankylosis of the temporomandibular joint complicated by frequent respiratory infections. Nearly allsent mouth opening, micrognathia, and mandibular retropositlon with resultant gl0110ptosia obstructed the airway. Medical treatment followed by a tracheostomy and bDateral condylectomy relieved the obstruction allowing normal fnnction of the temporomandibular joint. The clinical, electrocardiopaphic, radiologic, and hemodynamic ftndings returned to normaL Congenital ankylosis of the temporomandibular joint has not been previously reported as a cause of cor pulmonale secondary to upper airway obstruction. and congestive heart failure occurring Corinpulmonale infancy and childhood secondary to chronic upper airway obstruction has been well defined. 1 • 7 Most frequently, the obstruction is caused by enlarged tonsils and adenoids, 1 •3 - 8 though uncommon etiologies such as laryngotracheomalacia, 2 the Pierre Robin syndrome,e,10 °From the Department of Cardiology, Hospital de Ninos and the Faculty of Dentistry, Universidad Nacional de eo;&;ba,

Cordoba, Argentina.



Reprint requests: Dr. Aldou, Hospital de NlfiDI de Cordoba, Corrientes 643, 5000 Cordoba, Argentina

Crouzon's disease,11 subglottic stenosis secondary to prolonged tracheal intubation,12 and airway obstruction following cleft palate repair13 have also been mentioned. In this communication, we add a heretofore unreported cause of upper airway obstruction and cor pulmonale in a child with congenital ankylosis of the temporomandibular jointa which resulted in underdevelopment of the jaw and glossoptosia. She was successfully treated with a bilateral condylectomy. CASE REPORT

The patient was a three-year-old girl born with micrognathia who had a history of respiratory and feeding difficulties since early infancy. Intercurrent upper respiratory infections frequently aggravated her symptoms. She was hospitalized several times, once requiring a tracheostomy. Noisy respiration and somnolence were frequently noticed and had been more prominent in the few weeks before referral to our hospital. Two weeks prior to this admission, there was increasing dyspnea and progressive generalized edema. At the time of physical examination, the patient was stuporous and moderately cyanosed. Anasarca was present and the patient weighed 12.5 kg. Her facies showed eyelid edema, the nose had normal external configuration, and the nasal mucosa was hyperemic. There was ankylosis of the temporomandibular joint with resultant micrognathia, retroposition of the mandible, glossoptosia, and it was nearly totally impossible to open her mouth (Fig I). The inferior incisors were absent, leaving a small hiatus which allowed protrusion of the tongue and made feeding possible. The restricted mouth opening prevented direct examination of the pharynx. The jugular veins were engorged. Laborious breathing with suprasternal and subcostal retractions was present with respirations of 50 per minute. Frequent rhonchi and subcrepitant rales were heard in both lung fields. The heart rate was 150 beats per minute and regular. Peripheral pulses

F'xcuBE 1. A (left), Side view of face of patient obtained a few days after admission. B (right), the lips have been retracted to show the restricted mouth opening. Feeding and protrusion of tongue were possible through hiatus left by missing inferior incisors.

384 ALDAY, VEGA, HELLER

CHEST, 75: 3, MARCH, 1979

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FIGURE 2. Admission ECG shows severe right atrial and right ventricular hypertrophy. were all present and had a smaller amplitude on inspiration. There was a right ventricular heave, gallop rhythm, and a loud and narrowly split second heart sound in the pulmonary area. A 3/6 regurgitant systolic murmur which increased on inspiration was heard at the lower left sternal border. There was also a 2/6 early diastolic murmur in the pulmonary area. The liver was pulsatile at 5 em below the right costal margin. The spleen was not palpable. Chest x-ray films showed cardiomegaly with a cardiothoracic ratio of 59 percent and signs of pulmonary venous hypertension. The ECG had an AQRS axis of +115• and signs of severe right atrial and ventricular hypertrophy ( Fig 2). The hematocrit value was 42 percent, the Po2 , 45 mm Hg, and the Pco 2 , 44 mm Hg. Table I --Cardiac Catheterisation Data Be/ore and After Condylectomy* Site Before condylectomy** Superior vena cava Right atrium Right ventricle Pulmonary artery Pulmonary artery wedge Left ventricle Aorta After condylectomyt Right atrium Right ventricle Pulmonary artery Pulmonary artery wedge Femoral artery

Oxygen Saturation, % 69 66 68 69

Pressure, mmHg

95

(1) 43/2 43/20 (28) (4) 98/3 98/63 (75)

71

26/1 25/6

(1) (11) (3)

95

*Figures in parentheses indicate mean pressures. **Cardiac index was 4.3 liters/min/sq m; pulmonary resistance, 6.4 units/sq m; and systemic resistance, 17.2 units/sq m. tCardiac index was 4.1 liters/min/sq m; pulmonary resistance, 2.7 units/sq m.

CHEST, 75: 3, MARCH, 1979

She was treated with antibiotics, digitalis, and diuretics with clearance of the respiratory infection and rapid improvement of the signs and symptoms of heart failure. The gallop rhythm and heart murmurs were no longer present one week after admission. Once her failure was controlled, cardiac catheterization was performed (Table 1). The pulmonary artery pressure was moderately elevated and showed wide respiratory variations. Congenital intracardiac lesions were ruled out by oximetry and selective pulmonary artery and left ventricular cineangiograms. Following a tracheostomy, a bilateral condylectomy was done. Continuous postoperative mandibular traction was applied. The postoperative course was uneventful. The child was discharged free of medication with normal temporomandibular joint function and a satisfactory mouth opening. Intraoral rehabilitation and physiotherapy were continued on an outpatient basis. Subsequent examinations showed normal physical findings. Six months after surgery, the chest x-ray film and ECG were normal. A repeat cardiac catheterization showed normal rightsided pressures (Table 1). · DISCUSSION

Alveolar hypoventilation resulting in hypoxia and hypercapnia is the proposed mechanism of the pulmonary hypertension in patients with cor pulmonale secondary to chronic upper airway obstruction.l-4 Right atrial and right ventricular hypertrophy develop and congestive heart failure may follow. The latter might be related to myocardial depression secondary to hypoxia and respiratory acidosis. 6 Pulmonary edema is often present though its actual mechanism is still controversial. It has been suggested that pulmonary venous constriction might be present as in high altitude pulmonary edema, 7 or that the wide intrathoracic pressure variations due to the respiratory efforts may alter the alveolocapillary gradient. • A loss of the normal respiratory hypoxic drive has also

CONGENITAL ANKYLOSIS OF TEMPOROMANDIBULAR JOINT 385

been described, and oxygen administration may be dangerous in these patients.1 •11-T Noisy respiration, somnolence, cyanosis, congestive heart failure, and signs of pulmonary artery hypertension were the presenting features in this child with congenital ankylosis of the temporomandibular joint. The congenital anomaly produced respiratory and feeding dif&culties aggravated during intercurrent respiratory infections and suggested the diagnosis of cor pulmonale secondary to upper airway obstruction. The cardiac catheterization data and the rapid improvement following tracheostomy and bilateral condylectomy confhmed the clinical impression. To the best of our knowledge, this is the first description of congenital ankylosis of the temporomandibular joint as a cause of chronic upper airway obstruction and cor pulmonale which can be success£ully treated. REFERENCES

1 Noonan J: Reversible cor pulmonale due to hyperb'Ophied tonsils and adenoids: Studies in two cases. Circulation 3132: Suppl IT: ll-164, 1965 2 Cox MA, Schiebler GL, Taylor WJ, et al: Revers~'ble pulmonary hypertension in a child with respiratory obstruction and cor pulmonale. J Pediatr 67:192-197, 1965 3 Menashe VD, Farrehi C, Miller M: Hypoventilation and cor pulmonale due to chronic upper airway obstruction. J Pediatr 67: 198-203, 1965 4 Luke MJ, Mehrizi A, Folger GM Jr, et al: Chronic nasopharyngeal obstruction as a cause of cardiomegaly, cor pulmonale, and pulmonary edema. Pediatrics 37:762768,1966 5 Levy AM, Tabalcin BS, Hanson JS, et al: Hypemophied adenoids causing pulmonary hypertension and severe congestive heart failure. N Engl J Med 277:506-511, 1967 6 Ainger LE: Large tonsils and adenoids in small children

with cor pulmonale. Br Heart J 30:356-362, 1968 7 Bland JW Jr, Edwards FK, Brinsfteld D: Pulmonary hypertension and congestive heart failure in children with chronic upper airway obstruction: New concepts of etiologic factors. Am J Cardiol23:830-837, 1969 8 Djalilian M, Kem EB, Brown HA, et al: Hypoventilation secondary to chronic upper airway obstruction in childhood. Mayo Clio Proc 50:11-14, 1975 9 Jeresaty RM, Huszalc RJ, Basu S: Pierre Robbin syndrome: Cause of respiratory obstruction, cor pulmonale and pulmonary edema. Am J Dis Child 117:710-116, 1969 10 Cogswell JJ, Gaston DM: Cor pulmonale in the Pierre Robin syndrome. Arch Dis Child 49:905-908, 1974 11 DonN, Siggers DC: Cor pulmonale in Crouzon's disease. Arch Dis Child 46:394-396, 1971 12 Paparo GP, Symchych PS: Postintubation subglottic stenosis and cor pulmonale. J Pediatr 90:97-98, 1977 13 Robson MC, Stankiewicz JA, Mendelsohn JS: Cor pulmonale secondary to cleft palate repair. Plast Reconstr Surg 59:754-757, 1977 14 Topazian RG: Etiology of anchylosis of the temporomandibular joint: Analysis of 44 cases. J Oral Surg 22: 227-233, 1964

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mRERA ET AL

Tuberculous Aneurysms of the Descending Thoracic Aorta* Report of a Case with Fatal Rupture Aaron S. Estrera, M.D.; Melvin R. Platt, M.D., F.C.C.P.; Lawrence]. Mills, M.D.; and HisaBhi Nilcaidoh, M.D. Tuberculous aortic aneurysm is a rare disease entity. The majority of allec:ted patients snccumb to perforation and exsanguination. The only chance for survival and cnre is by resection and proloqed antitnbemdosis chemotherapy. Oar case mustrates the high risk of rupture of tnbercnlous aortic anenrysms. Post-mortem examination revealed that the mechanism of aneurysm formation was by direct caseous involvement of the descending thoradc aol1a from a jutaposed left upper lobe parenchymal tnberculous process. Oar findings also favor the concept that miliary dissemination (in the presence of tnberculous aortic aneurysm) is the result rather than the cause of the tnbercnlous aortic process.

O

ver one hundred cases of tuberculosis involving the aorta have been reported in the literature. 1 Almost one half of these cases were associated with aneurysm formation, while the other one half were examples of tuberculous aortitis without aneurysm formation. The incidence of thoracic and abdominal aortic involvement is about equal. 2 Very rarely is the ascending aorta involved. 8 Up until the present time, only 13 cases of tuberculous aortic aneurysms have undergone resection with seven considered cured of the disease. 1 •' Recently, we have encountered our first case of tuberculous aortic aneurysm. However, it was unfortunate that rupture and death occurred about 48 hours prior to the planned resection.

CASE REPoRT A 49-year-old black female schoolteacher was admitted on Jan 17, 1978, with a 21 month history of weight loss, cough with minimal sputum production, and shortness of breath. These symptoms had progressively become worse to the point that she had been confined to bed for several days because of extreme weakness and shortness of breath. As a schoolteacher for the previous ten years, she had yearly chest roentgenograms which were reportedly normal. Tuberculin skin tests were periodically done and were negative. In May 1977, she was told her tuberculin skin test was positive, and her chest xray 6lm was interpreted as abnormal with an ill-de6ned infiltrate in her left lower lobe. She was advised to start on antituberculosis therapy, which she refused and she was subsequently lost to follow-up. She recalled that she was doing November 1977, when she began well during this time feeling ill and losing weight. By late December, she was having night sweats, nonproductive cough, generalized weakness, and shortness of breath. She consulted her family

until

°From the Department of Surgery, Division of CardioThoracic Surgery, University of Texas Health Science Center, Dallas. Reprint reqfl88fs: Dr. EstrBf'tl, Department of Surg81'1/, UrMoerritr/ of Te%118 Health Science Center, Dallaa 15235

CHEST, 75: 3, MARCH, 1979

Congenital ankylosis of the temporomandibular joint: resultant upper airway obstruction and cor pulmonale.

Congenital Ankylosis of the Temporomandibular Joint* Resultant Upper Airway Obstruction and Cor Pulmonale Luis E. Alday, M.D.; Pedro/. Vega, M.D.; and...
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