pulmonale. joint: resultant upper airway obstruction ...

Congenital ankylosis of the temporomandibular joint: resultant upper airway obstruction and cor pulmonale. L E Alday, P J Vega and A Heller Chest 1979;75;384-386 DOI 10.1378/chest.75.3.384 The online version of this article, along with updated information and services can be found online on the World Wide Web at: http://chestjournal.chestpubs.org/content/75/3/384

Chest is the official journal of the American College of Chest Physicians. It has been published monthly since 1935. Copyright1979by the American College of Chest Physicians, 3300 Dundee Road, Northbrook, IL 60062. All rights reserved. No part of this article or PDF may be reproduced or distributed without the prior written permission of the copyright holder. (http://chestjournal.chestpubs.org/site/misc/reprints.xhtml) ISSN:0012-3692

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Congenital

Ankylosis I#{149}

Joint

Resulfanf and Cor

Obsfrucfion

Luis E. Alday, M.D.; Angel Heller, M.D. Cor

pulmonale

and

ondary to chronic

severe

girl

poromandibular

joint

infections. obstructed tracheostomy

absent

has

been

C or

and

airway

and

has

obstruction

adenoids,1,3

Department

been

well

uncommon the

-

-

-

a

the

tempororn-

of

to

norjoint

cor

pul-

defined.’’

Most

by enlarged

tonsils

etiologies

Pierre

Robin

of Cardiology,

also

been

men-

with

a bilateral

condylectomy.

REPORT

such

as

restricted

syndrome,9bo

Hospital Nacional

de Ninos and

de Ninos

de Cordaba,

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

de Corcioba,

-----

Ficuax

1. A

the lips have tongue

384

by

relieved

heart failure occurring secondary to chronic

the Faculty of Dentistry, Universidad Cordoba, Argentina. Reprint requests: Dr. Alday, Hospital Corrientes 643, 5000 Cordoba, Argentina

-

glossoptosia

obstruction.

is caused

though

laryngotracheomalacia, #{176}From the

congestive childhood

have

to

obstruction

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 1). The inferior incisors were absent, leaving a small hiatus which allowed protrusion of the tongue and made feeding possible. The

tern-

followed

as a cause

airway

treated

CASE

micrognathia,

function of the electrocardiographic,

reported

repair’3

secondary

airway

in

respiratory

resultant

treatment

palate

sec-

of the

findings returned of the temporomandibular

obstruction

the

failure developed

condylectomy

to upper

infancy

frequently, and

Medical

previously

pulmonale in

heart

opening, with

hemodynamic ankylosis

successfully

ankylosis

mouth

bilateral

secondary

and

by frequent

allowing normal joint. The clinical,

not

upper

airway. and

diologic, and mal. Congenital monale

congenital

cleft

and

this communication, we add a heretofore unreported cause of upper airway obstruction and cor pulmonale in a child with congenital ankylosis of the temporomandibular joint’4 which resulted in underdevelopment of the jaw and glossoptosia. She was

obstruction

complicated

retroposition

the

obstruction mandibular

airway

with

Nearly

mandibular

M.D.;

stenosis

intubation,”

In

tioned.

congestive

upper

a three-year-old

and

J. Vega,

Pedro

tracheal

following

Temporomanaibuiar

subglottic

disease,’1

prolonged

*

#{149}

Upper Airway Pulmonale

Crouzon’s

the

of

t

were

(left), been possible

Side view of face of patient obtained retracted to show the restricted mouth through

hiatus

left

by

missing

a few days opening.

inferior

after admission. B (right), Feeding and protrusion of

incisors.

ALDAY, VEGA, HELLER

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CHEST, 75: 3, MARCH, 1979

HH

T1+---

f-TI

:.

ii fl DID

1

, 2

1

--4

t

LLt FT:jJ

vi Ficuax

V2 shows

all present and had a smaller amplitude was a right ventricular heave, gallop

were

There loud

and

narrowly

area.

A 3/6

split

second

regurgitant

heart

systolic

i_____I_

V3

ECG

sound

murmur

severe

Catheterization

on

which

atrial

and

and

a

pulmonary increased

on

After

Con

Before

with

Oxygen

Right

atrium

66

Right ventricle Pulmonary artery

68 69

artery

Following

a

done.

Continuous

plied.

The

mm

wedge

outpatient

98/63

(75)

*Figures **Cardiac sistance, units/sq fCardiac sistance,

in

71

6.4

25/6

wedge

(11)

A repeat

was

(3)

indicate 4.3

mean

liters/mm/sq

units/sq

m;

and

hypertension

2.7

units/sq

4.1

liters/mm/sq

m.

CHEST, 75: 3, MARCH, 1979

normal mouth

physiotherapy

were normal

the chest

x-ray

opening.

continued

physical

on

findings.

film and

catheterization

showed

ECC

were

normal

right-

1).

in

m;

pulmonary

re-

resistance, 17.2

systemic m;

pulmonary

re-

patients

airway

ventricular

in

resulting

proposed

upper

mechanism

with

cor

hy-

pulmonary

pulmonale

secondary

Right

obstruction.’

hypertrophy

and

hypoxia

of the

develop

and

may follow. The depression secondary

latter might to hypoxia

edema is often is still controversial.

present

atrial and congestive be related to and

respira-

acidosis.6

that pulmonary in high altitude intrathoracic

was

with

ap-

child temporo-

The

a satisfactory

showed

surgery, cardiac

is the

Pulmonary mechanism

pressures.

m. index

and

hypoventilation

Alveolar

tory

95

parentheses index

and

was

uneventful.

medication

examinations

after

heart failure myocardial

26/1

artery

was

was

traction

basis.

percapnia

right

artery

Femoral

im-

DISCUSSION

(28) (4)

artery

Pulmonary

of

function

rehabilitation

normal.

(1)

Pulmonary

rapid

condylectomy

mandibular course

free

Subsequent

condylectomyt atrium

diuretics

and

a bilateral

postoperative

joint

to chronic

ventricle

and

infection

(1)

95

Right

digitalis,

respiratory

postoperative

Six months

Hg

98/3

Right

antibiotics,

the

tracheostomy,

discharged

Pressure,

43/2 43/20

ventricle

Aorta After

with of

sided pressures (Table 69

Pulmonary

%

* *

Superior vena cava

Left

treated

*

Saturation,

condylectomy

V6

hypertrophy.

provement 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.

an

Before

was clearance

Intraoral

dylectomy

Site

ventricular

mandibular

and

rll

V5 right

She

inspiration.

rhythm, in the

Data

#{149}F

_____________

was 1-Cardiac

I

V4 right

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 cm below the right costa! 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 +1150 and signs of severe right atrial and ventricular hypertrophy (Fig 2). The hematocrit value was 42 percent, the Po,, 45 mm Hg, and the Pco.,, 44 mm Hg.

Table

k4

‘

if1

_.1____..____i

2. Admission

ftH

R

3

J

I

H-

efforts

A loss

CONGENITAL

venous constriction pulmonary edema,7 pressure

may

alter

of the

the normal

ANKYLOSIS

variations alveolocapilary respiratory

though

It has might

due

to

be the

actual

suggested present

or that

the

as

wide

respiratory

gradient. hypoxic

OF TEMPOROMANDIBULAR

Downloaded from chestjournal.chestpubs.org by guest on July 10, 2011 © 1979 American College of Chest Physicians

its

been

drive

has

JOINT

also

385

been

described,

gerous

and

in these

Noisy heart

oxygen

respiration,

failure,

somnolence,

and

signs

cyanosis,

of pulmonary

artery

were the presenting features in this ankylosis of the temporomandibular tal anomaly

produced

aggravated

during

respiratory intercurrent

To

description

condylectomy the of

mandibular

Tuberculous

best

joint.

our

congenital

joint

as

a

Report Aaron

difficulties infections

the

this

of

of chronic

which

clinical is the

the

majority unfirst

temporo-

upper

S.

airway

cor pulmonale

due to hypertrophied

Studies

cases.

in two

Tabakin

Melvin

J. Mills,

M.D.;

and

aortic

aneurysm

of affected

patients

exsanguination.

The

is by resection therapy. Our tuberculous revealed

that

Circulation

31-

1965

tonsils and adenoids in small Br Heart J 30:356-362, 1968

Platt,

Hisashi

O

Our

one

half

formation,

have of

for

Post-mortem of aneurysm

these while

equal.’

tuberculous

also

reported

the

is

Up

and cure

chemorupture of

examination formation was

descending thoracic lobe parenchymal favor

(in the presence result rather than

cases

aortitis of thoracic

about

findings

The

the

concept

of tuberculous the cause of the

cases of tuberculosis involving the

been

tuberculous incidence involved.’

M.D.

entity. to perforation survival and disease

process.

ver one hundred aorta

F.C.C.P.;

prolonged antituberculosis illustrates the high risk of

aortic aneurysms. the mechanism

aortic

M.D.,

Nikaidoh,

succumb chance

were other

Very

until

aortic

in the

literature.1

associated

with

one

half

were

Almost

aneurysm examples

of

without aneurysm formation. The and abdominal aortic involvement rarely

the

is

present

aneurysms

the

time, have

ascending

only

aorta

13 cases

undergone

of

resection

with seven considered cured of the disease.” Recently, we have encountered our first case of tuberculous aortic aneurysm. However, it was unfortunate that

rupture

the

planned

and

death

occurred

about

48

hours

prior

to

resection. CASE

8 Djalilian M, Kern EB, Brown HA, et al: Hypoventilation secondary to chronic upper airway obstruction in childhood. Mayo Clin Proc 50:11-14, 1975 9 Jeresaty RM, Huszak RJ, Basu 5: Pierre Robbin syndrome: Cause of respiratory obstruction, cor pulmonale and pulmonary edema. Am J Dis Child 117:710-716, 1969 10 Cogswell JJ, Caston DM: Cor pulinonale in the Pierre Robin syndrome. Arch Dis Child 49:905-908, 1974 11 Don N, 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

386

and

case

R.

children

7 Bland JW Jr, Edwards FK, Brinsfield D: Pulmonary hypertension and congestive heart failure in children with chronic upper airway obstruction: New concepts of etiologic factors. Am J Cardiol 23:830-837, 1969

Robson MC, Stankiewicz JA, Mendelsohn JS: monale secondary to cleft palate repair. Plast Surg 59:754-757, 1977 14 Topazian RG: Etiology of anchylosis of the mandibular joint: Analysis of 44 cases. J Oral 227-233, 1964

Rupture

is a rare

only

process.

tuberculosis

BS, Hanson JS, et a!: Hypertrophied pulmonary hypertension and severe failure. N EngI J Med 277:506-511,

13

Fatal

M.D.;

that miiary dissemination aortic aneurysm) is the

2 Cox MA, Schiebler CL, Taylor WJ, et al: Reversible 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 adenoids causing congestive heart 1967 6 Ainger LE: Large with cor pulmonale.

with

by direct caseous involvement of the aorta from a juxtaposed left upper

can be successfully

REFERENCES

AM,

the

Aorta*

Estrera,

tuberculous

1 Noonan J: Reversible tonsils and adenoids: 32: Suppl II: 11-164,

a Case

Tuberculous

following tracheostomy

ankylosis

Thoracic

of

Lawrence

secondary

treated.

Levy

of

congeni-

cardiac catheterization

knowledge,

cause

Descending

congenital

The

feeding

confirmed

of

obstruction and cor pulmonale

5

Aneurysms

hypertension with

respiratory

airway obstruction. The

bilateral

pression.

child and

data and the rapid improvement and

be dan-

congestive

the diagnosis of cor pulmonale

and suggested to upper

may

administration

patients.1’5-7

Cor pulReconstr temporoSurg

22:

REPORT

A 49-year-old black female schoolteacher was admitted on Jan 17, 1978, with a 23i 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 film was interpreted as abnormal with an ill-defined 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 well during this time until November 1977, when she began 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 #{176}Fromthe Department of Surgery, Division of CardioThoracic Surgery, University of Texas Health Science Center, Dallas. Reprint requests: Dr. Est,-era, Department of Surgery, finsversity of Texas Health Science Center, Dallas 75235

CHEST, 75: 3, MARCH, 1979

ESTRERA ET AL

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Congenital ankylosis of the temporomandibular joint: resultant upper airway obstruction and cor pulmonale. L E Alday, P J Vega and A Heller Chest 1979;75; 384-386 DOI 10.1378/chest.75.3.384 This information is current as of July 10, 2011 Updated Information & Services Updated Information and services can be found at: http://chestjournal.chestpubs.org/content/75/3/384 Permissions & Licensing Information about reproducing this article in parts (figures, tables) or in its entirety can be found online at: http://www.chestpubs.org/site/misc/reprints.xhtml Reprints Information about ordering reprints can be found online: http://www.chestpubs.org/site/misc/reprints.xhtml Citation Alerts Receive free e-mail alerts when new articles cite this article. To sign up, select the "Services" link to the right of the online article. Images in PowerPoint format Figures that appear in CHEST articles can be downloaded for teaching purposes in PowerPoint slide format. See any online figure for directions.

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