Respiration 27, Suppl.

: 67-70 (1970)

Respiratory and Cardiovascular Consequences of Scoliosis

S. G odfrey

Institute of Disease of the Chest, Brompton, London

Summary. Observations were made on a series now comprising 500 patients with
scoliosis studies at the Brompton Hospital by Dr. P. A. Z orab over a period of eight years.
Most of the patients are under the age of 20 years. Detailed studies of lung function were
performed on all patients, of these two-thirds had congenital or idiopathic scoliosis, and
one-third had paralytic scoliosis. The results on the first 200 subjects are illustrated in the
accompanying slides.
The chest X-ray can be very informative if one shoulder is rotated slightly forward to
move the curved spine posteriorally. The cardiac outline of lung markings are usually
normal but the extra and intrapulmonary arteries may be small in severely handicapped
patients. Lung function tests show a total lung capacity, vital capacity and maximum
breathing capacity which is reduced in proportion to the degree of curvature. The loss was
more marked in severe paralytic scoliosis compared with other types. Airways resistance
is altered in proportion to lung volume. The difficulty of predicting the expected normal
values in patients with curved spines will be discussed. Carbon dioxide retention, hypoxia
and respiratory failure are very common under the age of 20, but may occur suddenly in
older patients in their fourth or fifth decades.
Cardiac disorders are found with some frequency in patients with scoliosis. They may
occur in conditions such as Marfan’s Syndrome or Friedreich’s Ataxia in which scoliosis is
also part. Diagnostic procedures, including the electrocardiograph, can be interpreted in
the same fashion as in normal subjects. Right heart failure in the absence of congenital
heart disease in the young scoliotic is rare. Autopsies have been performed on patients
with scoliosis who have died from congenital heart disease, during surgery or from other
causes. The lungs were distorted and of unequal size due to the curvature. The injection
arteriogram is abnormal with failure of development of vessels of all sizes. The total number
of alveoli is reduced and the sizes of the alveoli are very irregular though the mean size
is normal for the age of the patient. These findings suggest failure of the normal post-natal
alveolar development in severe scoliosis beginning in infancy. In the case of late onset
scoliosis, evidence is found of atrophy of pulmonary parenchyma. The small blood vessels
are reduced in number and have thicker walls but there is no relationship between muscular
hypertrophy of the arteries and the right ventricle.

Zusammenfassung. Eine Gruppe von nun 500 Patienten mit Skoliose wurde im Bromp­
ton Hospital durch Dr. P. A. Z orab während 8 Jahren beobachtet. Die meisten waren
weniger als 20 Jahre alt. - ¡3 litten an kongenitaler oder idiopathischer Skoliose, 1/a an
paralytischer Skoliose. Bei allen Patienten wurden eingehende Untersuchungen der
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68 G odfrey

Lungenfunktion durchgeführt. Die Resultate der ersten 200 Patienten werden in den
folgenden Dias gezeigt.
Der Aussagewert der Thoraxaufnahme kann gross sein, wenn eine Schulter leicht
vorwärts gedreht wird, damit die gebogene Wirbelsäule nach hinten verschoben wird.
Die Herzkonfiguration ist meistens normal, doch können bei schwer behinderten Patienten
die mtra- und extrapulmonalen Arterien klein sein.
Bei der Lungenfunktion sind Total- und Vitalkapazität der Lungen sowie die M.B.C.
im Verhältnis zum Verkrümmungsgrad eingeschränkt, wobei der Verlust bei paralytischer
Skoliose am ausgeprägtesten ist. Der Bronchialwiderstand verändert sich im Verhältnis
zum Lungenvolumen. Es wird die Schwierigkeit diskutiert, die erwarteten Normalwerte bei
Patienten mit verkrümmter Wirbelsäule zu berechnen. CCh-Retention, Hypoxie und
respiratorische Insuffizienz sind sehr häufig im Alter unter 20 Jahren, können aber auch
ganz plötzlich bei älteren Patienten in ihrem 4. oder 5. Lebensjahrzehnt auftreten.
Herzstörungen werden bei Skoliose-Patienten relativ häufig gefunden. Sie treten vor
allem auf bei Zuständen wie das Marfan’sche Syndrom oder die Friedreich’sche Ataxie,
bei denen eine Skoliose zum Krankheitsbild hinzukommt. Diagnostische Verfahren, ein­
geschlossen das EKG, können in genau gleicher Art interpretiert werden wie beim nor­
malen Patienten. Versagen des rechten Herzens bei Fehlen einer kongenitalen Herzer­
krankung ist beim jungen Skoliotiker selten.
Es wurden Autopsien ausgeführt bei Patienten mit Skoliose, welche während der
Operation oder durch andere Ursachen an angeborenen Herzerkrankungen starben. Diese
zeigten, dass die Lungen verzogen und ungleich gross sind entsprechend der Beugung der
Wirbelsäule. Das Injektionsarteriogramm ist pathologisch mit mangelhafter Entwicklung
der Gefässe jeden Kalibers. Die Gesamtzahl der Alveolen ist verringert und ihre Grösse
sehr unregelmässig, obwohl die durchschnittliche Grösse normal ist entsprechend der
Altersstufe des Patienten. Diese Befunde lassen ein Versagen der normalen, postnatalen
Entwicklung der Alveolen bei schweren, frühkindlichen Skoliosen vermuten. In den Fällen
von später beginnender Skoliose sind Anhaltspunkte für eine Atrophie des Lungenparen­
chyms vorhanden. Die kleinen Blutgefässe sind in ihrer Zahl vermindert und zeigen dickere
Wandungen. Es ist aber keine Beziehung zwischen muskulärer Hypertrophie der Arterien
und des rechten Ventrikels nachweisbar.

Scoliosis is a crippling disease with many causes. It cripples the skeleton,

the lungs and sometimes the heart. A continuing study of this condition is
in progress at the Institute of Disease of the Chest, London by Dr. P. A.
Z orab with the cooperation of his colleagues in cardiology, pulmonary
function and pathology. Some of the results have recently been published
[Zorab, 1969] and this communication summarises these findings.
The series comprises some 500 patients mostly under the age of 20 years.
Of these patients 2/3 had congenital or idiopathic scoliosis and llz had
paralytic scoliosis.
Investigations have included clinical examination, chest X-ray electro­
cardiograph (ECG) and lung function tests by Dr. F. J. Prime. The pathology
of the lungs and heart has been studied in Professor L ynne R eid’s depart­
ment in a few patients who have died.
The chest X-ray taken in the conventional way is usually very difficult
to interpret due to the spinal curvature. However, if one shoulder is rotated
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 Respiratory and Cardiovascular Consequences of Scoliosis 69

Slight (< 60°) Severe« 60°) Slight« 60°) Severe« 60°)

(20 years) (19 years) 06.8 years) (18.3 years)
ml

Fig. 1. Total lung capacity. Idiopathic and congenital scoliosis.

Fig. 2. Total lung capacity paralytic scoliosis.

a few degrees forward, the heart and lungs can be seen clearly. The heart
shadow is usually normal unless congenital heart disease is present. The
lung fields are clear but the pulmonary vessels are often small.
Lung function tests have included simple spirometry, the measurement
of lung volumes and airway resistance in the whole body plethysmograph
and the steady state transfer factor for carbon monoxide. The results show
that the lungs are small with a reduction in both total lung capacity and
vital capacity and the reduction in size is proportional to the degree of spinal
curvature (grades I and II - less than 60°, grades III and IV - more than 60°).
The total lung capacities in idiopathic and congenital as compared with
paralytic scoliosis are shown in figures 1 and 2. There is little difference. The
loss of lung volume also means loss of ventilatory capacity, and the maximum
voluntary ventilation is reduced in proportion to the curvature and more
in paralytic than non-paralytic disease (figs. 3 and 4).
The transfer factor for carbon monoxide (‘diffusing capacity’) was a little
reduced in the severest disease. Airway resistance was generally higher than
normal but this difference was less marked if the small size of the lungs was
taken into consideration.
One problem with scoliosis has been to predict the expected result of a
test in a patient with a curved spine since most predictions have been based
on height. Recently we have studied a large population of normal children
[G odfrey et al., 1970] and found that we can predict lung function just as
well from arm span as from height. We are now using span to predict normal
values in scoliotics.
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70 G odfrey

Slight (<60°) Severe «60°) Slight (<60°) Severe(<60°)

(20years) ( 19years) (16.3 years) (16.8 years)
l/min 1/min

Fig. 3. Maximum voluntary ventilation. Idiopathic and congenital scoliosis.

Fig. 4. Maximum voluntary ventilation. Paralytic scoliosis.

Heart disease is relatively common in scoliosis since both may occur in

such conditions as Marfan’s Syndrome or Friedreich’s Ataxia. In the series
described Dr. M. K. T owers found that the ordinary rules of clinical and
electrocardiological diagnosis could be applied to these patients, just as if the
curvature was not present. If a murmur was heard it required just as much
care in evaluation as in the normal. There were few older patients in this
series and the incidence of cor pulmonale was low. Only 2 children developed
it so that breathlessness usually meant congenital heart disease in the
younger children or respiratory insufficiency in adolescents. Severe respira­
tory failure with cor pulmonale did sometimes develop quite suddenly in
middle age.
The pathological changes in the lungs at autopsy can be summarised as
failure in development and atrophy. The lobes underlying the curvature are
small and their alveoli are reduced in number. There is great variation in
alveolar size. If the scoliosis is present from infancy the alveoli do not
multiply normally, and if it develops later, true atrophy occurs. The pul­
monary vessels are more muscular than normal and this may be associated
with right ventricular hypertrophy.

References

G odfrey , S.; K amburoff , P. L., and N a irn , J. R.: Spirometry, lung volume and airway
resistance in normal children aged 5-18 years. Brit. J. Dis. Chest (1970).
Z orab , P. (ed.): Scoliosis (Heinemann, London 1969).
Author’s address: Dr. S. G odfrey , Senior Lecturer, Department of Pediatrics, Institute
of Disease of the Chest, Brompton Hospital, London, 5. IP. 3 (England).
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