Massive Gastric Enlargement With Delayed Presentation of Congenital Diaphragmatic Hernia: Report of Three Cases and Review of the Literature By Paula l In three matic

In each entire

Mark

was

small

bowel

capacity

the

of the stomach,

of

H. Krasna

suggestive no site

of

obstruction

could

en-

strated

at

operation.

This

striking

stomach.

disappeared

of

the hernia.

thorax

sac. Despite

Irwin

the

herniation

into

of a hernial

and

after

of the intraabdominal was

E. Gershwind,

diaphrag-

long

there

case there

absence mous

presenting

period,

largement

Brill,

cases of congenital

hernia

newborn

W.

the

preoperatively,

following

be

demon-

enlargement

surgical

repair

of

and INDEX

the enorwhich

obstruction

WORDS:

hernia;

was

Congenital

massive

gastric

diaphragmatic

enlargement.

C

ONGENITAL DIAPHRAGMATIC HERNIA usually presents in the newborn period as an acute surgical emergency. Occasionally there are no symptoms early in life, and the diagnosis may be delayed until later childhood or even adulthood.’ There has been little attention in the literature to gastric enlargement in association with congenital diaphragmatic hernia. This report is prompted by our recent observation of striking gastric enlargement in three children and adolescents who had large congenital posterolateral diaphragmatic hernias without sac. The stomach was retained within the abdomen in each case. CASE

STUDIES

Case I M-0..

an 18 yr old asymptomatlc

to have intestinal

male was discovered

loops in the left hemithorax

on a preemployment

and displacement

chest radiograph

of the heart

to the right.

lie

had always been in good health wrth no previous history of trauma. An

upper

There

gastrointestinal

was no evidence

jqlunum

series revealed

of obstruction

a large

at any

and ileum and much of the colon were herniated

A diagnostic

pneumoperltoneum

free communication

between

the

was performed. peritoneal

absence of a hernial

sac could be anticipated

the spleen. and a small portion

were herniated

into the left hemithorax

Ladd‘s bands were present. repaired.

There

resultant

and

the

into tract.

left pneumothorax

left

At

pleural

surgery.

of the pancreas a posterolateral

was no hernial

extending

the

pelvis.

The

entlre

into the left hemithorax.

preoperatively.

through

stomach

in the gastrointestinal

The

cavity

intestine and colon,

phragm

atonic

level

sac. The

space. almost

and fundus defect

contents

indicated

Therefore. the entire

a the

small

of the stomach

in the diaphragm.

were reduced

Uo

and the dla-

The patient has done well since operation.

Case 2 U.G., a 4 yr

old male. was noted on routine physical examination

best In the right hemithorax. and there was no history

Journal of Pediatric

Surgery,

HIS growth and development

had

to have the heart sounds heard

been normal.

He was asymptomatlc.

of trauma.

Vol.

12, No.

5 (October),

1977

667

BRILL,

668

A chest radiograph series demonstrated thorax.

showed

loops of bowel

GERSHWIND,

in the left hemithorax.

the presence of almost the entire small intestine

The stomach was large, atonic.

and extended

An

AND

KRASNA

upper gastrointestinal

and colon

into the pelvis. There

in the left hemi-

was some retention

of

barium in the stomach but no evidence of obstruction. At surgery, demonstrated

a posterolateral

diaphragmatic

on the upper gastrointestinal

hernial sac. The postoperative Upper gastrointestinal

defect was found. series. No Ladd’s

course was uneventful.

The

hernial

contents

bands were present.

were

There

and the left lung expanded

as

was no

satisfactorily.

series showed return of the stomach to normal size and position.

Case 3 W.P.,

a I6 yr old male was admitted had

episodes of nausea and vomiting

with acute left upper occurred

quadrant

pain and vomiting.

in the year prior to admission.

Otherwise.

Two

he had

been well. There was no history of trauma. A chest radiograph

on admission showed multiple small air-fluid

vated left hemidiaphragm. gastrointestinal (Fig.

series. The stomach

1). In addition,

obstruction.

the duodenal

At operation, of the duodenal vomiting.

weeks

large

occupying

and

were demonstrated

atonic

and extended

ele-

on upper

into the pelvis

dilated. suggesting an element of duodenal

the splenic Rexure of the colon to be contained the right lower

quadrant

when distended

in the

and the left

when collapsed. the contents of the posterolateral

spleen, and most of the small Four

was extremely

bulb was inconstantly

A barium enema demonstrated

hernia. The cecum was mobile. lower quadrant

levels below an apparently

Small bowel loops in the left hemithorax

intestine.

There

bulb, and no other obstructing postoperatively,

the

patient

He did well on conservative

dtaphragmatic

hernia included

were no Ladd’s

transverse

bands to explam

colon,

the dilatation

lesion was found. There was no hernia sac. was readmitted

treatment.

An

with

epigastric

pain.

upper gastrointestinal

Fig. most

duodenal filled

loops

herniated

and time

Case

1. of the

markedly

nausea,

study at this

3:

At

barium dilated

bulb of

60

minutes,

remains stomach.

is dilated. small

in

a

The

Bariumbowel

into the thorax.

are

GASTRIC

ENLARGEMENT

AND

CONGENITAL

HERNIA

669

Fig. 2. Case 3: Following hernial repair, the stomach is in its usual position with no evidence of obstruction. The repaired left hemidiaphragm is in normal position, as is the small bowel.

revelrled

further

a

normal

stomach

with

no

evidence of obstruction

(Fig.

7). He

was

discharged

with

no

complaints. DISCUSSION

The newborn with a diaphragmatic hernia usually appears dyspnea. cyanosis, mediastinal shift, and a scaphoid abdomen. older child or adult is likely to have nonspecific abdominal or toms or to be asymptomatic (Table I).’ ” In the latter instance be detected on routine chest radiographs. Table

1. Summary

of Clinical Presentation Diaphragmatic Clhcal

Presentation

Abdominal

pain

Hernia

of 57 Previously Reported Cases of Posterolateral in Older Children or Adults* Number

of Cares

25

Vomiting

14

Chest

11

pain

10

Dyspnea

9

Asymptomatic Dysphagio

3

Obstruction

3

Heartburn Backache Obstructive Other *Age

12-72;

mean

age

40 years

jaundice

severely ill with In contrast, the thoracic sympthe hernia may

1 8

670

BRILL, GERSHWIND,

AND KRASNA

Two of our patients were asymptomatic, and the third presented with vomiting and abdominal pain. All had been asymptomatic in the newborn period. In all three patients the stomach was within the abdomen and was large and atonic, while the entire small bowel was in the left thoracic cavity. A hernia sac was lacking in each case. In Cases I and 2, most of the colon was also herniated into the thorax, while in Case 3 only a portion of the colon was herniated. A review of the literature revealed five cases of congenital diaphragmatic hernia in which enlargement of the intraabdominal stomach was reported. Two were infants, one year old and I8 months old. respectively.“,” The other three patients ranged in age from I3 to 36 yr.5,“,” In each of these cases, most or all of the small bowel and part of the colon were in the left hemithorax. In several of the cases, the duodenum lost its normal “C” configuration. Instead, the postapical portion of the duodenum turned upward and to the left with most of the duodenum contained in the hernia. Interestingly, the pancreas remained in its usual retroperitoneal position despite the abnormal location of the duodenum. In the two cases in which the pancreas was located in the chest, the duodenum was in the abdomen. In four of the five patients no sac was present. In the fifth there was no comment about the presence of a hernia sac. The three presently reported cases all lacked a hernia sac. The intraabdominal position of the stomach with congenital diaphragmatic position of hernia is common at all ages. A 40 9;) incidence of intraabdominal the stomach has been reported in cases presenting in the neonatal period.” We have found 35 cases in the literature with postneonatal presentation of congenital diaphragmatic hernia in which the position of the stomach was specifically noted.3~“,9~‘0~”Of these, the stomach was intraabdominal in I8 cases (5 I”,). The hernial contents of the 18 previous and three present cases with intraabdominal stomach are summarized in Table 2. It is of interest that in all eight cases with gastric enlargement most or all of the small bowel was specifically stated to be herniated. Unfortunately, descriptions of hernia contents are frequently imprecise, and in the four cases of small bowel herniation without gastric enlargement, the length of herniated small bowel is unspecified. The gastric enlargement in the three current cases does not appear to be Table

2.

Hernia

Contents

21 Cases of Diaphragmatic

in

( 18

Previously

Reported

and

Hernia Three

Number Hernia

Most

or entire

and (with

or

bowel

onlyt

llevm

and

case-cecum

t 1 case-transverse colon.

bowel

Stomach

of

Number With

of Cores

Large Stomach

8

8

spleen)

amount

Colon

lntraabdominal

Cases)

of colon

without

and

With

CClSES

only

Unspecified

descending

small

portion

Omentum

*l

Contents

New

part

of small

0 0

3

0

1

0

of the colon*

transverse only;

5 4

colon

3 cases-unspecified colon

only;

amount

1 case-transverse

of colon. colon

and

ascending

colon;

1 case-transverse

and

GASTRIC

ENLARGEMENT

secondary tomatic,

AND CONGENITAL

to mechanical each with

barium

except

puzz!ing

with

for

dilatation

obstruction

could

listed

90 causes

phragmatic Gastric

among

enlargement

capacity

allowing

in

the

in patients

ciated

Ladd’s

primary largement

bands

to

explain

soon

studied

case is somewhat

of gastric

No

found

retention

with

cause

signs

or

for

of

duodenal the

in

the

literature.

but

did

not

diaphragmatic from

to the pelvis

abdomen.

Although

the

is unlikely

after

hernia

gastric Rimcr

mention

intestine

gastric

size

in view

the successful

may

the normaily

to descend

of the small

etiology

resolved

two patients

empty

fixation

ncurogenic

however.

on the stomach

relatively

had abnormal

third

asymp-

of ingested

as radiographic

evidence

cases

gastric

the stomach

sented

The

as well

No

five

progression

dia-

them.”

to the absence of pressure nal viscera,

in the

of nonobstructive

hernia

retention.

at operation,

mentioned

two cases were entirely

and normal

of obstruction

of the duodenum.

be found

was

first

bulb

of gastric

support

671

The

duodenal

an element

intermittent enlargement

obstruction.

a normal

clinical

HERNIA

three

and colon,

prc-

had

an obstructive repair

:I large

patients

none

of the fact that

surgical

abdomi-

and achieve

all

on

bc related

adjacent

asso-

basis.

A

the gastric

of the hernia

en-

in the

postoperatively. REFERENCES

I. Grai\lrr Congenital

L,

Dorman

GW.

diaphragmatic

Surg Gyncwl

Obstct

13?:408, 197

2. Adams JT. McRrynolds ct al: Obstructive

Vottrlcr

hernia

jaundice

in

I

DC;. Hudnut

HB.

pleuro-

Am Surg 35:482.

3. Ahrcnd

TR. Thompson

l’oramcn of Bochdalck

BW: Hernia

in the adult.

of the

Am J Surg

9. Raichoudhury et al: Foramen

Hatnes JO. Collins

in an adult simulating olog! 95.277. 5, Kirkland

JA:

ctrusion.

Radi-

pusterolateral

in the adult.

Br J Surg

1960

7. Pccora Bochdslek-type

ccratrd

Abbott

through

AC.

congenital

Goodhand diaphrag-

111adults. Can J Surf 6:30l. DV:

Ventilatorq

diaphragmntic

changes hcrnix

1963 uith

Am Surf

in

1966

RC. Pstnnik SC, Sahw

Roper

Bochdnlck

hernia

M.

in adult\.

CL,

hernias

Carlasun in the

E: Inar-

adult.

Ann

Surg 160:X47. I964 children

uith

PE: Diaphragmatic a

report

of

hcrnla

thirteen

in