Pulmonary

DENNIS

Agenesis
M.D., F.C.C.P*

M. L.
New

ROSENBERG, Orleans,

Louisiana

I
as able with

REFERENCE

TO

THE

DEVELOPMENT

OF

been cording no be in

evidence clinical expected the 19th

of

observation curiosity. could the early case reports

and

the

re-

the terms

lungs, age nesis,

indiscriminate aplasia, and has of in

usage hypoplasia of

of

the has in to well

of a medical information from century, although had In this year,

probably

Presumably reasonably reports. began Late to on the earlier is,

fre-

created a certain amount the literature. Agenesis denote to complete describe amount of a rudimentary a adopted their should development for incomplete be be absence conditions

confusion been used lungs which tissue To as

a variexists clarify only failand Potof the suggested degree or bronaffected outbut no of of

appear, condition texts. each

quency,

isolated remarks been included in pulmonary with

because of increasing improved

pulmonary bronchus. universal and precise the the terms meanings. designation of lung

century, reported

agenesis

communication, should to connote agenesis ure aplasia of

classification used for tissue Actually,

dian is pre-

agnostic
awareness

methods
of

the

and more possibility on for reports.

ORIGIN

widespread of such the subject in a

anomaly. far too sentation

The of case

literature

voluminous

survey

development.

ter1 classified all lung, but earlier a classification defect: Group chus side. and 1: no True

the malformations Schneider2 had according absence supply to the

EMBRYOLOGIC

The unknown; thought faulty and sumed, development

etiology however, to germ respiratory error since lobes the or occur

of

pulmonary the lesion with the because of

agenesis is commonly inherently result A to that vascular,

is

of lung to the

vascular

plasm,45 of the systems of lung

pulmonary, is defective. genetic are origin vessels usually

Group pocketing
no

2: A primordial of the trachea tissue. 3: Extreme

bud (a tiny as bronchus) hypoplasia with

dethe

velopmental missing and tem starts and then this anlage

is preabsent, coexist. systhat buds end later, the of the is lung

lung Group

pulmonary

true small without

tinum.

lung tissue in caliber ends lobes case may of such and histories

which in a lies

a bronchus fleshy structure the our to the mediaspersonal existing

other abnormalities Arey8 has described as beginning on grow of the of

of

frequently the respiratory an side evagination of the The posterior and, and In these the and From lung

within within

with ventral a groove.

the out the by

esophagus

Three experience knowledge

resembles laryngotracheal

contribute conditions.

from trachea

the groove,

HlsToluc Although frequent agenesis anomaly,

BACKGROUND

esophagus subsequent epithelial connective give

of it one. has but was is

the by

lung no

is an means

ina to the have

separated buds, stages

tissue

a constriction.

newly discovered of the condition

various

The first description been attributed the De earliest Pozzi can in only
Tulane

bronchi arise. development47 push the out into developing

evaginations to lobar rise, in

description

authors, observers instance,

Professor of

of

lungs

rise give This

or

reputed That

l673.

however,
of Surgery,

bronchi. turn, to
suggests

Each of segmental
that

the latter bronchi.

pulmonary

*Assistant sity School

Univer-

lobar about 68

agenesis the same

must time

occur, that the

respectively, lung buds

Medicine.

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July

Volume 42. No. 1 1962PULMONARY

AGENESIS

69
is definitely
series,

first after bronchi days

appear ovulation)48 first after

(Horizon or the arise ovulation) malformation was the tree,

XIII, time (Horizon

.#{176}

28 that XV, Ellis7

one the

day lobar 3 1-32

agenesis

not
agenesis

incompatible occurred and 22 the involved in one there condition females (32 was on

with in 49 on the side was desthe

survival!3 In Valles males (25 on

considered

the

left

side,

that the circulation with the

tigators

of primary of although

the pulmonary malformation, development not all invesof

right). In one patient, was not reported, and bilateral cribed left side, lateral). tioned. side in this cidence both in to has to be or vasphysicians tribution ing ciates of males. currence as frequent. the left agenesis found is, Katz,16 it to sides 62 dewas and group by agenesis. Valle in The 67

subsequent pulmonary
agree.

failure

Wilson ed In
also

and of

Warkany8 the the and left

have lungs vitamin

demonstratin rat arteries additional experimentchanges can are result known It similar fetuses were A deficiency.

and 24 on In 14 cases, Of the stated, the no existed were have to be however, Stowens,1 cases the right

the right, and one bithe sex was not menin which left in 54. lung the was involved absent in in Other inand

agenesis the fetuses,

subjected absent, also. that affect hereditary

to maternal there

pulmonary were has shown environmental

Apparently, difference the sexes, affected.

significant between equally also about by and of commonly Stowens was side of bobar to one Many absence

fects ally may be been

Warkany8 maternal the anomalies in

embryo human that a

and which defect

congenital

considered the sex disequal.101415 This findno means Lukas18 the lung invariable. and or that have more lung in 1909 often is rather is even more reported diagnosed twice also afa assoparts in oc-

subjects.

postulated

those from nutritional produced by diseases by cular infectious supply.

AGE AND SEX

deficiency may of the placenta, that affect of normal the

believe be Indeed,

more

agents by

suggested approximately be

development

modification

in males

investigators

DISTRIBUTION

fected.4505822 from the found rare While condition, agenesis

In literature, this

a survey distribution

of

57

cases

taken associates to age:

Brescia#{176} and according or fetus

uncommon.232#{176} 6 16 6 8 3 18

Berlinertm

stillborn newborn 0-1 2-10 year

an instance of agenesis of the right upper and middle lobes. From that time until 1954, according to Valle,1 only 14 cases were right absent reported, upper ones.
DIAGNOsIs

years

and and

in middle

nine

of lobes

them were

the the

11-20 years over 20 years In 53 44 were the baby two recorded, (seven hours, the were age larger were between was the and not group less one given. youngest months) the died the many oldest collected than and 72 years; Among was who was a lived one 30 and the by year

Valle, of age; 15 he only in eight, cases for Although cribed least, monary ation
for

patients between

the that

date for diagnosis

of

the

first

case

despul-

years;

is a matter clear agenesis of a living

than

disagreement, of unilateral

it is, at

31 and

premature a 7 2-year-

made by patient 100 have and years.

physical examinwas not feasible

Subsequent in-

more

old woman who rhage.12 Actually, unrecognized. postmortem during life. As

of cerebral hemorcondition often goes cases as unilateral are are found diagnosed or lobar at

vestigators in a live necropsy, Actually, nosis was (probably

might patient but this

suspected the later verified was antemortem in less cases.

state it at diagten that

sequence

unusual. than Since

examination Obviously,

before 1942,24 accomplished less than five)

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70

DENNIS

M.

L.

ROSENBERG

Diseases of the Chest

date,

however,

many

more

cases

have

been

tinum

to

be

shifted shadow

to the may

affected shadow represent

side411 on that fluid,

reported presumably bronchoscopy,

ographv

during th because in the

life of the of the wider

patient, use of

and side.1 fatty with helps al side ally, across

a dense The

homogeneous

bronchographv, study of obscure

and angipulmonary

tissue, or the shifted to occupy The is usually the the cavity.

persistent thymus, which, mediastinum, sometimes the elevated, otherwise on and, lung The or bony empty the is pleuraffected herniated may the of diaphragm

conditions. Clinically,
asymmetrical; the lag affected may also

the
side

chest
there
;3033

may
may

be be an

found
flattening

to be
of

characteristicthorax

and

inspiratory the are to be

contralateral midline. may scoliosis. study, may

pulmonary in

shape normal. deviated

pulmonary

and The

the

be noted. Usually, though, movements of the chest trachea is often the and side the found

be symmetrical, involved side concomitant On affected

respiratory

the interspaces on be narrowed because

towards
tissue,

of the missing heart and med-

fluoroscopic side On
excursions

the limited,
are

motion
in

of the although
some a tests,

jastinum If apex that On the will the the

comparably right be posterior be The and no with and impression lung noted chest

displaced.31435 is absent, on is that the wall, the right heart cardiac side, sounds so

be

normal function maximum

patients. significant

of dextrocardia.36

reduction

breathas a mod-

ing

capacity

is evident,

as

well

are audible, There may affected cussion.2 seem normal side,

the heart rotates also. breath sounds over the obvious the dullness lung physical to perto on is likely findings

erate to slight Near-normal and ported. In ommended accurate Formijne4 old female bronchoscopic 1937, means alveolar

diminution in vital capacity. values in oxygen consumption perfusion Hurwitz bronchoscopy for and diagnosis, diagnosis by that bronchi study are generally re-

remaining

Stephens4#{176} recas the only and in really 1938

that side will be the expected cal findings can be normal, the there upper portion
lung

ones. Physiparticularly in side existing affected, lung. if If

of of exist

the the

is herniation may
in patients

made the patient

in a 19-yearmethod. By the presence can be deterwill serve to tree and to in the existof herniaof to the the lung opposite

the remaining perresonance

however,

is emphysematous,

over

that lung has

hylung;3 agenstim-

examination

with existing

pulmonary

and character of the mined. Bronchographic outline show the ing lung, lion. the bronchial as well

esis,

the
so

state

of the

tracheobronchial distribution as the amount herniation

ulated no real
may

little agreement expected

investigation that there is as to whether that lung to be emphysematous recorded the bronchospirometric lungs in two suggested emphysematous usual of three residual the such Boyden3 remaining cases (right variants deviaanomalies. of the mediasintraadult hyperorlung or

be

Conspicuous

hypertrophic. Smart pleural pressures and tracings patients. trophied gans, findings also lung. lung), from tions, studied In the the extra each left usual of the The rather in contrast after the of lung pattern and existing findings than to the status his

through the mediastinum side is sometimes found.4 More came ary disorders. successfully examination
it

recently, valuable in the

angiocardiography diagnosis of pulmon-

bewas

A preoperative diagnosis made by angiocardiographic as early 1950 as

1942;14

pneumonectomy.

however, and

was

not

until

when

Ingram42

exhibited vascular

associates that this preciated. formation and sels.

published diagnostic The as to the

a paper on its value means was really apangiocardiogram position of gives the inheart

as segmental

lobes,

Roentgenographic chest usually shows

the

examination heart and

the distribution of the pulmonary vesAt the same time, associated congenital

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Voiume July 1962

42,

No.

PULMONARY

AGENESIS

71

malformations sels may graphic

be

of the heart and great vesdiscovered by angiocardio-

and, Almost histories

in such 50 are per

a patient, often cent recorded of

bronchopulmonary

complications

become
the died in

very patients the

serious. whose first five inthe comanomby asfault. was and, Thomas agenbody. Siamese surgicof right,

examination.tm
DIFFERENTIAL DIAGNOSIS

To pulmonary tions agenesis children,

establish must may be

a agenesis, resemble be

definitive a number

diagnosis of In In thymus confusion, is unilateral. of the cough may This difficulty Bronchiecsimulate agenresult to mediastinal medcondinewborns,

of

years of life (generally fection), and about perinatal in the mon high period.tm infant coexistence of In per rare

from pulmonary 20 per cent in Another obvious rate is the

factor

differentiated. atelectasis. of the of

mortality the

older may es-

cardiovascular

enlargement a source thymic after or secondary cystic may

presumption

occasionally pecially In young iastinal pneumonia, bronchial in

or

alies.124263394 Valle, 45 (37.5 sociated An reported since and esis In twins ally, the and then, Boydens died another were the left the from congenital and by by interesting

cases reviewed cent) had some cause and of death Boyden, pulmonary of a foreign in which separated hypoplasia of the was

if the nodes

shadow enlargement whooping

cardiovascular

children,

or or cir-

Thomas other patient aspiration unusual successfully

investigators. with case

development, collapse. diagnostic

of agenesis.

persistent the

pulmonary cause can Patients

cumstance tasis with

atelectasis

also

esis of the lung. gone pneumonectomy fuse complete the block

who have undercould, possibly, condiagnostician. of one of the main A

nonviable twin had lung, total absence right pleural space

obliterat-

inexperienced by tumor also

CASE A

1 three-pound, white, infant of seven months

bronchi could Differentiation sistent prime films tinal noted detection side does
hyperdistention

be mistaken for between agenesis however, chest since normal Furthermore, on the Routine misleading,

agenesis. and peris x-ray mediaslung affected as

across

massive importance. are shift often and of

atelectasis,

of

herniated conditions. sounds

and

are the the the

in both not

breath

gestation was delivered by Caesarean section on June 16, 1949. The mother was 42 years of age and was eclamptic. The infant did not breathe spontaneously at birth and was cyanotic. With passage of an intratracheal catheter, a small amount of watery fluid was aspirated. There was no vomiting, convulsions or twitching. In the heart sounds, no abnormalities were audible. A
few right scattered coarse rales were heard over both

disprove may produce the usually or of the side

agenesis,
herniation

of her

lung

fields,

but were
there was

more
also

notable
some

on the

mediastinum physical lung.tm the in diminution the lower cavity. signs On

nearly of be able the to

normal absent however, detect them of

side where

diminution

on careful will of areas sounds

examination, absence affected

examiner

thoracic

PROGNOSIS

Agenesis, anomalies, compatible creasing normal A patient withstand

unless is not with number existence with one respiratory

complicated of itself normal of patients after lung does infections

by necessarily living. An are

other inin-

of the breath sounds. About one week later there was only slight improvement in the infants condition. Respirations then became irregular. She became hard to arouse and had obvious cyanosis of the lips, hands, and feet. After an uneven course for two months she appeared for a time to respond well; however, on August 29, 1949, she died suddenly and unexpectedly. A small amount of bloody, frothy fluid extruded from the mouth and nose; the lips and feet were definitely cyanotic; and the cause of death was believed to be atelectasis.
At 1. of necropsy, Congenital these absence major of findings the by hemorrhagic right total were middle absence interstitial noted: lobe of

continuing
.

pneumonectom\ not, however, successfully,

the lung (as evidenced the middle lobe bronchi). 2. Acute bilateral
pneumonia.

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72

DENNIS 3. Pulmonary
4. The the left

M.

L. ROSENBERG
lung was the dull. apex N beat heart was murmurs were

Diseases of the Chest heard

edema.
common carotid artery. artery originated

and
area.

in

the

right

parasternal

from 5.
CASE

innominate

Prematurity. 2 28-year-old white physician had known

The impression at this time was that the child had (1) hydrocephalus, (2) atelectasis of the right lung with mediastinal shift, and (3) bilateral pneumonia. A series of chest x-ray films
were made that showed a narrowing of the right

agenesis
years, veyed
equipment

of the
at his which family
and

left lung since the age time his physician-father with some newly acquired
made the diagnosis. After

of

five surx-ray
this

rib spaces, the heart shadow in the opacity of the right upper lung field,
shift sema to of the the right, left lower marked lung with lobe. compensatory anterior

right chest, mediastinal

emphyherniation

discovery, the patient had a complete tion. Bronchoscopic and bronchographic

showed
pouch.

examinastudies in a blind

into
of

the
the

right
right

hemithorax,

and

normal

aeration
were

the

left

main

bronchus

to end

Hemivertebrae

noted

in the mid-thoracic

area.

On bronchograph-

In the history was included a questionable episode of acute respiratory tract infection when he was six months of age. In grade school, he had bronchitis of about two months duration. Later, he was active in track and other athletic activities in school. At the age of 18, the patient began to smoke. He smoked cigarettes for about six years, after which he began to smoke cigars.
At the present time, he smokes about five cigars

the left bronchial tree was norright there was dye in the lower was no dye in the upper and middle lobes. Indeed, there was no evidence that these lobes existed. The findings were consistent with agenesis of the right upper and middle lobes, Right axis deviation was demonstrable
on electrocardiographic disease and, study, probably, indicative of myocardial of left ventricular

ic examination, mal, but on the lobe only. There

a day. The never been

physician-patient incapacitated

by

states that his condition

he has and is

hypertrophy. He was treated with antibiotics and was discharged with instructions to remain on antibiotic therapy. He was readmitted on June 4, 1955, with a recurrent respiratory infection and was treated successfully. His next hospitalization was on December 1, 1955. Again, he had a respiratory infection and operative treatment was advised. Physical and laboratory findings were unchanged from those of the previous admission. Surgical intervention been seen was since. SUMMARY

well

able

to withstand
training

the and

physical

demands

of

an active surgical is now 160 pounds, On examination

left chest wall,

program. His weight his height is six feet. of the chest, flattening of the
expansion on the left

decreased

side, deviation of the trachea to the left, audible heart sounds in the left chest basally, and dullness to percussion in the lower half of the left chest are discernible. The upper half of the left chest and sternum are resonant to percussion. Roentgenograms of the chest confirm the clinical findings and show herniation of the right lung across the midline. CASE
3

refused

and

the

child

has

not

Congenital monary tissue

absence is a rare

or

deficiency condition,

of but

pulit is

A 13-month-old white boy was first hospitalized at the age of three weeks because of vomiting and possible hydrocephalus. At the age of two and one-half months, a right inguinal hydrocele had been found, and the baby was discharged to be readmitted in one month. He did
not return until he was readmitted at the age

not

as uncommon

as it was

once

presumed diagnostic of the with suffi-

to be. means condition,

Currently, with and augmented agenesis to

improved knowledge

is diagnosed

cient frequency the differentiation pulmonary The normal fects possible. piratory tence of tandy, findings is three with or life anomaly

justify consideration in of obscure or puzzling incommensurate unless make susceptibility or vascular The some lungs. hazard. illustrate be expected, of the associated survival to incompehistories of concomithe with deimres-

of five months for recurrent pneumonia with heavy breathing and cyanosis. On physical examination at this time the pharynx was found to be hyperemic. His head was large and symmetrical, with patent fontanella and widened sutures. The liver was palpable to three centimeters below the right costal margin. Moderately severe respiratory excursions were diminished on the right side. Breath sounds were distant on

conditions. is not expectation

malformations Heightened infections the that major may agenesis patients

the
ronchi

right,
were was

but

loud

on the
over to both

left.
lung

Moist
fields.

rales
The

and
left

heard tympanitic

lung

percussion

and

the

right

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Volume
July 1962

42, No.

PULMONARY

AGENESIS

73
ZIJ
SAM M EN FASSU oder
NC

RESUMEN

La
jido como

ausenci#{225} cong#{233}nita o la
pulmonar antes se es rara pero no presumia.

deficiencia

del

teso

Congenitales

Fehlen

Mangel

an

Lungen-

tan
de

poco

com#{252}n

gewebe Gegenwartig
schen

ist em

seltener
wie mit

Umstand,
vordem

ungew#{246}hnlich

aber er ist nicht unterstellt wurde.

Actualmente

con
y un

los

medios

diagn#{243}stico

und

den
sowie

verbesserten
groj3erer

diagnostiKenntnis des

mejorados

conocimiento

mayor

de esa posi-

Moglichkeiten

bilidad, Ia agenesia se diagnostica con suficiente frecuencia para justificar su consideraci#{243}n en el diagn#{243}stico diferencia en afecciones obscuras.
La esperanza anomalia normal no afecta de considerablemente Ia haya

Zustandbildes wird die Aplasie gen#{252}gend oft diagnostiziert und rechtfertigt ihre Anwendung bei der Differenzierung unklarer oder schwieriger pulmonaler Verh#{228}ltnisse. Die Mibildung ist nicht unvereinbar mit nor-

otros

defectos

vida a malformaciones

menos que

que

hagan

Ia suso las
hisde

maler
knupfte

Lebenserwartung,
Defekte oder

sofem
Fehlbildungen

nicht

damit
em

ver-

Weiter-

sobrevida imposible. El mayor peligro

ceptibilidad a
constituyen de tres enfermos

consiste
infecciones
el mayor

en

las

una mayor respiratorias

peligro. Las algunos

leben steht

unm#{246}glich macht. Die Hauptgefahr bein der erh#{246}hten Empfanglichkeit gegenuber

Infektionen Die oder mangeloder von 3 Krankengeschichten

respiratorischen Gefal3versorgung.

deficiencia
Estas torias

vasculares.

ejemplifican

los
de

hallazgos susceptibles Ia agenesia pulmonar.

de

esperarse

en

el caso

Fallen erl#{228}utern einige der Befunde, man gleichzeitig mit einer Lungenaplasie mul3. Complete reference
list will appear in

mit

denen
rechnen

the

reprints.

BALLISTOCARDIOGRAM A clinically adaptable, ation ballistocardiograph siderations of biophysical

to evaluate the overtly healthy cardiovascular men aged

AND
for the

AGING
development of an abnormal ballisto-

ultra-low frequency accelerthat met all theoretic condesign was used by Moss
18 to aging 54. process in 307

initial

The initial appearance of accelerated lar aging (grade I abnormality) was ballistocardlogram in 16 per cent of
by age

cardiovascupresent on the

cardiogram (grade I) during the 20 to 39 age period. and an accelerated attack rate after age 40. The rate of conversion to a more severe grade of ballistocardiographic abnormality increased significantly in the fifth decade. The relationship between an abnormai cardiogram, accelerated cardiovascular coronary artery disease is discussed.
Moss, A. J.: Bailistocardiographic Cardiovascular Aging Process,
Circsila,ion,

II) by

was age

the population 35. A more severe degree of aging (grade evident in 16.5 per cent of the individuals 50. There was a rather constant attack rate

baliistoaging and
of the 1961.

Evaluation 23:434,

ALTERED

HEMODYNAMICS REAERATION
have clarified a num-

IN

THE OF

PULMONARY AN with ATELECTATIC decrease in

CIRCULATION LUNG oxygen

the to

FOLLOWING
Experimental studies on dogs

uptake

ber

of

pertinent

points.

Reaeration

of

a its

chronically

atelectatic
partially. main.

lung
However, They include

in

the

dog

restores

function
reloss

by others. From tients, according

published available

has reports reports,

been

reported

in some pathe passage

specific physiologic a variable but

defects persistent

of compliance, a marked increase in pulmonary vascular resistance, a diminished pulmonary artery blood flow, and a concomitant decrease in oxygen uptake. Histopathologic changes have been noted in reaerated atelectatic animal lungs. A zone of subpleural hypoinflation was noted. Bronchiolar arteries exhibited formation of spurs or club-like processes. In human beings with a reaerated chronically atelectatic lung, a similar partial restoration of function

of time reaerated complete

reaerated erly include

has favorably influenced atelectatic lungs. It is functional evaluation of

lung after chronic bronchospirometry atelectasis and

the function suggested that a patient with

an might propinvestigation

of a
a

of vascular
LONG,

resistance Aeuis,
A.

and E.,

pulmonary
BENFIRLD,

compliance.

E. T.,

W.
F.,

T., REIMANN, namics in the

of an Atelectatic

AND N,cao,
Circulation

Pulmonary
Lung,

J. R., Mraoucu,, S.: Altered Hemodyfollowing Reseration

Cache,. Sari.,
40:

J.

Thor.

end

640,

1960.

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