“Acquired”

Congenital

By Nathan l

“Acquired”

hernia layed

or late

has

period

hernia of

presented. terized

defined

This

on the

as

de-

diagnosis

with new

Hernia

I. MacPherson

cases and

14 additional

literature.

A classification

ing of herniation

a documented

life

Three

basis

Roderick

of a congenital

after

postnatal

dence of herniation.

and

diaphragmatic

been

appearance

diaphragmatic time

E. Wiseman

congenital

(ACDH),

Diaphragmatic

development

and

patients

from

the

based

upon

tim-

state

of pulmonary

is presented.

no evicases

has been

of a review

are INDEX

charac-

hernia;

of these

WORDS:

Congenital

acquired

diaphragmatic

diaphragmatic

hernia.

C

ONGENITAL DIAPHRAGMATIC HERNIA occurs as the result of ;r developmental defect in the diaphragm. The common type is posterolateral in the region of the pleuro peritoneal canal (Foramen of Bochdalek). This lesion is an important cause of respiratory distress in the newborn period, and if untreated, usually proves fatal. The classification of infants with congenital diaphragmatic hernia is based upon the state of development of the lung.’ The presence and severity of pulmonary hypoplasia is the ultimate prognostic determinant of these infants.’ On this basis, three types of hernia have been described. Infants with Type I are born with severe bilateral pulmonary hypoplasia which usually proves to be lethal in the early neonatal period. In the past, these infants were largely undiagnosed in life and appeared in perinatal mortality statistics.j In recent years, these infants account for a significant part of the surgical mortality in this disease.‘,J Type 2 is characterized by unilateral (ipsilateral) pulmonary hypoplasia. Those who survive surgical treatment have been noted to develop emphysematous changes in the hypoplastic lung.’ This group of infants are the subject of a great deal of recent attention among pediatric surgeons with a view towards improving the survival.’ In Type 3 diaphragmatic hernia, pulmonary hypoplasia is insignificant and in these infants, survival with normal pulmonary function is the rule.‘.’ It is believed that this group of infants account for the good survival result which were reported following the early surgical experience with this disease.5m7 Although this classification is arbitrary and, the degree of pulmonary hypoplasia occurs as a continuum, there are distinct differences between these groups which lead to a clearer understanding of the pathophysiology of this anomaly. This report concerns a fourth group of patients with congenital diaphragmatic hernia who may appear well during the newborn period, but manifest the abnormality in later life. Three cases of “acquired” congenital diaphragmatic

From

the Departments

of Surgery

and The Clnrversit~ of Manitoba. Prrsenred January

at the annual

mrrrin~

of

RadioloRr.

Children’s

Ccwrre.

Healrh

Srienc~e~ (‘mire.

Canada.

The Canadian

Associatron

(I/ Pediatric

Surgeons.

7‘11ron1r~.

16. 1977.

Addres.~ for reprirrr Cmrre

and

Winnipeg.

Health

Sciences

requrvrr:

IL. E. It’i.\eman.

Cemre. 685 Bannarvne

cm1977 hr Grune & Slratron.

Journal of Pediatric Surgery, Vol.

Inc.. ISSN

12, No.

M.D.. Avenue,

Deparrmmr Winnipeg.

o/ Prdiatric, Maniioba.

Sur~rr>..

C‘hildreu’.\

R3E 0 WI. Canada.

Ml-72 3468

5 (October),

1977

657

WISEMAN

658

AND

MAC

PHERSON

hernia (ACDH) are presented, and by way of review, the characteristics of this diagnosis are examined. In the clinical spectrum of diaphragmatic hernia. this fourth type of the disease offers insight into the understanding of pathogenesis and underlines the significance of the timing of herniation. CASE

B.L.M.

This

of unsuccessful

STUDIES

3; yr old female was transferred treatment

for what was thought

the child had been well and had a normal

to the Children’\

Centre

chest radIograph

improvement,

the radmlogic admission.

On

absent breath sounds film (Fig.

I

shr

appearance of left lower lobe was

afebrllz

on the left with

slight

B) shuwcd the left hcmithorax

and in

with

shift

to this.

I A).

Her

there had been hymptomatlc

pneumonia

no distress.

mrdiastinal iillrd

one month Prior

at four years of age (Fig.

illness was characterized by cough. fever, and anorexia and although persisted.

follov,ing

to be a left lower lobe pnwmonia.

and pleural

Physical

to the right.

multiple

air

1.

1. (A)

effusion

examination The

admission

contalnlng

cystllkc

had

revealed chest Icsions

12.8.71

Fig. 4

yr

Case

demonstrating

diaphragm. hospital contrast

hernia

study in

remaining

the

radiograph

(age

41 yr)

demonstrated.

showing left

radiograph

normal

Chest

admission

phragmatic bowel

(6)

Chest

a

large

hemithorax

in the abdomen.

at age

appearance at the with (C)

of

time left

of

dia-

Barium

mass

of

with

stomach

small

“ACQUIRED”

displacing

DIAPHRAGMATIC

the heart and mediastinum

Intestinal (Fig.

CONGENITAL

tract demonstrated

I C). Surgery

poatrrolateral

to the right.

was performed

through

operatively

the lelt

months

jqunum

rapidly

reexpanded

later. the child required

tion, but there wa

exammation

small

bowel loops

of the upper gaslr~lin the left hemithor,l\

laparotomy.

A smooth-edged

7 cm in diameter w’as found. The to left tranaversr

rotated. The hernia wa

lung

A barium

3 left upper transverse

defect measuring

Included most of the midgut (proximal

Gshteen

6.59

the presence of multlple

diaphragmatlc

duodenum wab normally

HERNIA

and the child

further

colon)

reduced and a prlmarq surger)

no recurrence of the diaphragmatlc

herniated viscera

ah well as spleen. The repair carried out. Po\t-

wa\ discharged

one

for a mechanical small

ueeh

later.

bowjel obstrui--

hernia. Now 3 yr follovvlng

hernia rcpalr.

the child remains In good health.

C.S. Thib

5 mr, old Infant

male presented with :i 12 hr hIstory

lie had been under medlcal surveillance Fallot

way made and B Wnterston

teal chesl radIograph

smce birth

shunt performed.

Physical examination

revealed a qanotic

over the left hemithorax A chest radIograph

demonstrated

defect was encountered measuring of JeJunum

duodenum wah normally suggested that this and thereby

Infant in respiratory

defect.

distreqh with absent brelilh

the presence of small

bowel loop:,

3 cm In diameter with smooth

distal

to the ligament

in the left

this

hcrnla

from

of Trcltl

manifesting

and mid

rorller

3; mo female infant

of age.

day and at 4 yr

w;i\ admitted

transverse

In

life.

colon.

repair

with

well.

3 3 wh history

of Intermittent

She was tir\t seen at 4 wk of age when a diagnoslb of coarctation

aorta was established

by cardiac catheterization.

Physical

plain tilm of the chat At surgery

examination

and barium

a left pobterolateral

hat. The content

of the hernia

Bochdalek

tOth post oper;llivc

revealed mediastinat measuring

IS composed

of 14 reported

each case. there was radiographic apprarlng

no

colon. A prlm:iry

wa

discharged

on the

day. When last seen at the ape ofY yr, the child W;LS well. REVIEW

group

A

hernia.

1 add’s procedure was also c:ir-

and the palient

OF

LITERATURE

Seventeen C‘BSCSof delayed appearance of congenital diaphragmatlc This

to the right.

4 x 3 cm with

howel and right

and, because of matrotation,

the left lung expanded rapIdly

shift

of left dlaphragmatic

defect wa\ encountered small

of Ihe

at that time revealed an apparentI>

the dlagnoslh

conalrted of the entire

repatr at the hernia was performed ried out. Po~toperativrly,

on readmIssIon

enema contirmed

i)l‘ Ihe

of the left lung. The patlent H :I\

bile htalned komlting.

Chest tilms

lhe

ridge whlih was pressing

A primary

the patient rrmalns

to hospital

Bochdalck

edges and no sac. The herniated

rotated. .The convex surface of the spleen revealed a small

diachargcd on the 9th postoperatlbe

Intact diaphragm.

sound5

hrmithoral

a left posterolateral

to the rlgh~. At surgery.

may have been the point at which the defect In the diaphragm

preventing

This

of

HI\ last chest tilm made at 4 mo of age ahowcd

of a chaphragmatic

diaphragm was carried out and wab followed by rapld expansion

C.K.

of Tetrdlop!

defect was noted on his ,111

and shift of the apex beat to the right.

with a ahift of the heart and mrdiastinum viscera consisted

No dlaphragmatic

or the IY made subsequently.

n right upper lobe pneumonia but no suggestlon

of acute dyspnea and cyanov~

at which time 3 dqnosis

cases from

documentation

the literature’

hernia have been reviewed ”

of an early chest l-ray

plus

our

three

cazes. In

demonstrating

;I normal

diaphragm.

Timing of’ Herniatim The cars ;L normal

have been analyzed on a tlmc scale (FIR. appearing chest u-ray.

of the symptomatic tinally

period,

the duratmn

7) which includes the age at which there wah

of the latent asymptomatic

and the age at which

made In the patients reviewed, a normal

week of Ilfe In nine patients and beyond the tirst

the diagnosis

chebt radiograph

period,

of diaphragmatic

the duratLon hernia

had been taken within The

median .~ge

at which a normal x-ray had been documented was 3 wk of life. A latent asymptomatic

period wan

present In I

I

patients and ranged from

month of life in eight patients.

\+a\;

the tlrht

4 days to Xf months

with

a median duration

of Y v.h

WISEMAN

AND

12

MONTHS

MAC

PHERSON

34 YEARS

56

AGE

Fig.

2.

Time

course

in

17 patients

with

acquired

congenital

diaphragmatic

In six patients, the symptoms which led to the diagnosis of dlaphragmatic time of the initial normal from

chest x-ray.

The

I2 hr to as long as 5 yr. However,

relatively

short period

*ith

a median

period

during

which

in most patients, duration

of 2 wk.

diaphragmatic

hernia

is more often diagnosed

were present

ranged

led to a diagnoaia

The age at which

tinally made ranged between 8 days and 6 yr. with a median congenital

hernia dated hack to the

symptoms

symptoms

hernia.

the dlqnosis

age of 14 wk (Fig.

in the neonatal

after

;I

was

2, inset). Acquired

and infancy

period.

In

the cases reviewed. two thirds of the diagnoses were made within the tirst 6 mo of life.

Clinical and Anatomical The compiled diaphragm

was on the left side in nine patients

dence of a hernial

sac In 15 of the patienta

16 of the patients. complete of liver

Characreristics

group of 17 cases consisted of 12 male and 5 female patlents. and in two. a thin membranous

the defect was described

absence of the right diaphragm. in

the

of intestinal

right-sided

rotation

case and incomplete

Diagnosis

defecta

was infrequently fixation

as posterolateral The visceral

midgut

defect

in position

content

in the left-sided

described

(Table

sac was described.

with

of the hernia defects (Table

I. right).

in the

was no eviIn

one patient

having

consisted

mainly

I. left). The state

Malrotation

occurred

in one

In two.

and Trealment

In all of the cases revIewed. diaphragmatic

hernia. although

contrast examination was made

and

The

and on the right side in eight. There

was ultimately

considered

was often incorrect.

primary

and in two patients.

at postmortem

surgical repair was performed

successfully.

to be diagnostic

In ten patients,

was carried out to confirm the diagnosis. In one patient,

at thoracotomy

transabdominal

the chest x-ray

the initial impression

examination.

the correct In

I5

ol

barium

diagnosis patients.

a

“ACQUIRED”

CONGENITAL

Table

I.

DIAPHRAGMATIC

HERNIA

66 1

Content of Hernia and State of Intestinal

Rotation in Cases of A.C.D.H. Intestinalrotation

Herniacontent

Right

Left

Right Whole liver

3

Mid Gut

7

+ Colon

Left ~..__ 4

Normal

4

+ Spleen

2

Incomplete fixation

1

1

4

Right lobe of liver + Small bowel

1 1

Mid gut

Stomach and omentum

1

Molrotation

Colon

1

Not mentioned

-~

1

7

3 __.._.

RESULTS

Among the

this

death5

mortem,

group

of patients,

occurred

in

and the third

in a child was typical pansion

with

patients

were

not

having

death occurred

associated

in this

there

group

I4 survivors been

one month

anomalies.

An

of patients.

and 3 deaths.

diagnosed

following

uneventful

with

Two

prior

repair

to

of the hernia

postoperative

live patients

noted

ot

poht-

recovt:ry

to have rapid

1:x-

of lung. DISCUSSION

Pulmonary

hypoplasia

ment

of the lung

direct

result

space. The plastic

of the mass presence

of developmental the phase of rapid nary cantly with

with

This

bronchial arrest

peripheral

to the developing of experimental

lung

count.“‘This

can only discrepency

this

form

with

short

critical also

is accounted

for

timing which

week of of pulmc)-

most

observed

in the

hernia

has

hernia,

the deticienq

for by LI reduction by the reduced

of infants

of 3 later-timed

of hypoplasia

diaphragmatic

signili-

It bus been

in the lungs

is a result

;I late gestational

accounted

occurrence

3s being during

hypoplasia.’

occurs

of hypoplasia

;I

in the hypo-

the sixteenth

period

of pulmonary

congenital

be partly

before

is

the pleural

an early

by embryologists

occurs

and is the pattern in whom

within

generations

indicates

develop-

interference

and the consequent

hypoplasia

This

lambs

created.18 In inpants

in lung weight

which

expression

alveolar

insult gically

has been timed

within

hernia.”

lungs

hernia.

the normal

This viscera

of bronchial

of herniation

the v:triable

with

utero.“.lh

by abdominal

number

division

It is the timing

determines that

in

diaphragmatic

arrest.”

diaphragmatic

an interference

period

effect produced

developtnental

shown

from

3 critical

of a reduced

lung of infants

gestation.l”

results

during

been sur-

in radial

number

alveolar

of bronchial

generations. An

early

changes Type

variable

timed

seen in infants

3 diaphragmatic

herniation

with hernia

Type

appears

to account

I and 2 diaphragmatic

have been best

detined

for

the

hernia.

pulmonary Infants

by radiographic

with criteria

662

WISEMAN

AND MAC PHERSON

and are characterized by demonstrating rapid postoperative lung expansion with no evidence of overinflation.’ Among these infants, there appears to be no documented evidence of pulmonary hypoplastic change and it is possible that some of these infants have entirely normal lungs. It has been shown that among infants dying with congenital diaphragmatic hernia, as many as lo”,, have lungs which are normal by weight and by radial alveolar count and, thus are not hypoplastic.” Clearly there must be a significant number of surviving infants with congenital diaphragmatic hernia who also have normal lungs. Infants and children with acquired congenital diaphragmatic hernia are characterized as having a documented variable time period during postnatal life with no evidence of herniation. These infants have had no herniation during the fetal period to interfere with pulmonary development. They also have been noted to have rapid postoperative lung expansion with no evidence of subsequent overinflation. These patients should be expected to have normal lungs. The clinical features of acquired congenital diaphragmatic hernia are similar to those of other patients in whom congenital diaphragmatic hernia is diagnosed beyond the neonatal period.‘,“,” The only difference between these groups is the fortuitous chest radiograph demonstrating an apparently normal diaphragm in those patients labeled as having acquired congenital diaphrugmatic hernia. This would suggest that these two groups of patients are, in fact, one and the same, with visceral herniation through the congenital diaphragmatic defect having occurred at some time during postnatal life. The age incidence of these patients with ACDH indicates that herniation of abdominal viscera into the chest occurs with the greatest frequency during the early postnatal period, (Fig. 3). This distribution would suggest that there are similar cases in the early neonatal period. Thus, among neonates diagnosed as having diaphragmatic hernia, there may be some in whom herniation has occurred very soon after birth. In these infants, a previous normal chest x-ray is very unlikely.

Fig. 3. represents

Age

incidence

the proposed

of patients group

with

with neonatal

acquired ACDH.

congenital

diaphragmatic

hernia.

Dotted

bar

“ACQUIRED”

CONGENITAL

There tory is

DIAPHRAGMATIC

are neonates

distress

not

hernia

with

required.’

These from

pansion

relief

of lung.

At

pulmonary

infants

function

early

potentiate

hernia

well

to

atelectasis

of infants

studies

surgical

with

repaired

are normal.“-” infants.

respir;.lassistance

reduction

rapid

of

reei-

the neonatal

overinflation there

their

postoperative

during

Thus.

suggesting

in whom

and ventilatory

no evidence of pulmonary

in these

neonatal

pression

shift

defect.‘J canal.

a transient

the first

This

period.

and in whotn

is no evidence

that herniation

the tirst

hours

increase

in the volume

would

of life,

of pul-

was not present

apparent

absence

neonatal

period,

matic hernia hernia

which survival,

in the early

resultant which

with

time

hernia

at an early

bilateral

is timed

resultant

unilateral

of these cases is neonatal sequelae.

Type

Table

Type Early

as occurring

3: Cases

2.

in which

Classification

Hernwtlon

Hypoplas~o

of

patients

with

with

in the

diaphrag-

I:

diaphragmatic

Cases

(Table

in which

bronchial death.

the period with

is timed

Diaphragmatic

Four

herniation

division

Type

with

2: Cases

of rapid

The

survive

hypo2).

outcome

in some

detectable

pulmo-

as occurring

either

Hernia Pulmonary

Outcome

Bilateral

Death

(perinatal)

Unilateral

Death

(postnatal)

Sequelloe

bronchial

Late period rapid

of

bronchial

Survival

+

division 3

4

Late gestation

or

Neonatal

(ACDH)

tote

(ACDH)

Minimal

or

Survival

Absent

+ _

Absent Survival

in

bronchial

division 2

;I the

congenital

of pulmonary

sequelve

hypoplasia.

herniation

of Congenital

events with

herniation

congenital

of rapid

others

Pulmonary

period

neonates

presence

late during

Time of

rapid

for

Type

pulmonary

a significant These

in the infant

fsvouring

and perinatal

death. whereas,

is

period.

the period

hypoplasia

IS prc)During

of the age incidence,

of late pulmonary

are defined:

and

and thorax.

pressure

there

acquired

of herniation,

time during

pulmonary

herniation

division

neonatal

and the presence

of diaphragmatic

that

com-

traverses

and thorax. viscera.

in some

has been developed

is based upon

has occurred

consideration

the conclusion

classification

plasia, types

supports

abdomen

of herniation

events

a dia-

as 80 cm of H,O

tract,

occurrence hypoplasia

might

with

thoracic

intrapleural

the early

Thus

which

as the infant

abdomen

the intestinal

and the physiologic

can occur

A modified

negative between

by the intraabdominal

defect.

is initial

between

occupied

of pulmonary

hernia.

there

may be as high

a large

enters

events

in the infant

compression

gradient

gradient

as air

diaphragmatic

diaphragmatic

force

pressure

efrort,

all tend to favor

congenital

and delivery,

compressive

a pressure

by physiologic to thorax

by abdominal

positive

respiratory

causing

abdomen

labor

followed

produces duced also

is accompanied from

During

and recoil

the birth With

period

a visceral

phragmatic

1

diaphragmatic

gases are normal,

fetal life.

The

narl

663

respond

follow-up

hypoplasia

during

blood

compression

there are some who show monary

congenital

is not marked.

and

HERNIA

WISEMAN

10

0 10 20 30 40 E OI WEEKS OF GESTATION -

IGo.

1

IU

2v

3v

4v

5v s5u

POST NATAL PlGE -

AGE PITTIME OF HERNlPlTlON

AND

MAC

PHERSON

Fig. 4. This graph illustrates the time of herniation in all infants with diaphragmatic defects (curve). The four types of this disease are indicated (T) along with their expected mortality (TD).

during late gestation or during the early neonatal period. There is minimal or absent pulmonary hypoplasia and most of these patients survive with no detectable pulmonary sequelae. Type 4: Cases in which herniation is timed as occurring after the early neonatal period. There is no pulmonary hypoplasia and these patients survive with no pulmonary sequelae. The incidence of patients with a diaphragmatic defect falling into each of the four types has been estimated (Fig. 4). This estimation is based on the following information: I) Perinatal mortality statistics in all infants with diaphragmatic hernia.3 2) Survival of infants treated for diaphragmatic hernia.“,7~“,‘6 3) The incidence of pulmonary hypoplasia among neonatal deaths in infants with diaphragmatic hernia.’ 4) The incidence of pulmonary hypoplasia in survivors as evidenced by x-ray and pulmonary function studies.“,13 5) The incidence of late as compared to neonatal diagnosis of diaphragmatic hernia.‘,7.‘F From this data, it is possible to compile a composite graph presenting the timing of herniation, the classification of diaphragmatic hernia, and the estimated survival in each of the four types of this disease (Fig. 4). REFERENCES I.

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MhB.

M.

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