Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome: A Case Report

749

Megacystis-Microcolon-lntestinal Syndrome: Antenatal Ultrasound William and

C. Vezina,1

Fred

Fran#{231}ois R. Morin,

Winsberg

Diagnosis of fetal abnormalities now being made with increasing trates imaging

Hypoperistalsis Appearance

the to

combined use of clarify a congenital

information

available

investigations

were

from

by means frequency. ultrasonic problem.

these

undertaken

of ultrasound is This case illusand radiographic Because of the

examinations, after

gauge

needle

stomach

remained

A 25-year-old

woman

pregnant

at 31

weeks

menstrual

age

was

referred for ultrasound examination because she was large for dates. Sonography showed polyhydramnios, bilateral fetal hydro-

Fig.

displaces

1 -Sections

through

fetal

liver (L). Polyhydramnios

abdomen.

A, Transverse

was present.

P

=

scan.

amniotic

Dilated

renal

October

1 979

0361 -803x/79/

1 334-0749

a dilated

(fig.

2).

no

collecting

infant

colon

for

bilateral

and

pelvis was

and

1 B).

a 22

5 ml of

spontaneously showed

without

distal

gas.

barium

enema

that

the

A barium

examination

entirely on the left side. The barium several days. Excretory urognaphy showed hydronephrosis, and hydroureter. Cystog-

a huge

had

systems.

abdomen

dilated

(fig.

tract,

located

demonstrated

The

infant

infant’s

peristalsis

microcolon in the

were

renal

g female

of the

abdomen

poor excretion, raphy

a 2,300

bladder

two

5

=

bladder

laparotomies

spine; K

=

without because

kidney.

vesicoureteral of suspected

B, Distended

urinary

reflux. obstruc-

bladder

(B)

fluid.

Received February 9, 1 979; accepted after revision June 5, 1979. , All authors: Department of Diagnostic Radiology, Division of Diagnostic Canada H3G 1 A4. Address reprint requests to F. Winsberg. AJR 1 33:749-750,

into

injected

later

fetal

of the urinary

inserted

Radiography and

enlarged

of obstruction

was

showed

showed

Report

a markedly

was

weeks

delivered.

delivery.

1 A), and

the diagnosis

Renografin-6O

series

Case

(fig.

To confirm

Four

appropriate

immediately

nephrosis

$00.00

Ultrasound,

© American

Montreal

Roentgen

General

Ray Society

Hospital,

1 650

Cedar

Ave.,

Montreal,

Quebec,

750

CASE

REPORTS

AJR:133,

fibrosis

or infarction.

Ion-intestinal

The

final

diagnosis

hypopenistalsis

was

October

1979

megacystis-microco-

syndrome.

Discussion Berdon

et al. [1

microcolon-i had

]

described

ntestinal

five

abnormalities

of

fixation

improved

the

was

age Ours

graphic of

the

latter

with

required

was

The

and

diversion,

feeding

and

and

cells in produced

a

of the bladThe hydrone-

but

the

infants

location

dilatation systems.

urinary

for

megacystis-

mature ganglion hypoperistalsis

functional obstruction and there der, ureters, and renal collecting tation

with

syndrome.

intestinal

either normal or increased shortened intestine. Intestinal

phrosis

infants

hypoperistalsis

hyperalimen-

infants

died

before

of 3 years. Amoury et al. [2] reported a similar case. is the seventh reported case. Prenatal ultrasonofindings included polyhydramnios, marked dilatation urinary

and

bladder,

confirmed

bilateral

by a prenatal

hydronephrosis,

antegrade

the

pyelogram.

Dilatation of the bladder and hydronephrosis may also be seen with prune belly syndrome [3] and urethral valves. Since urinary obstruction is ordinarily associated with ohgohydramnios,

Fig. tion

of

2.-Oblique

radiograph

5 ml of Renognafin-60

contrast

has extravasated

of maternal into

around

fetal

adrenal

abdomen renal

pelvis.

gland

and

pelvis

Dilated

after

calyces.

injec-

and

intestinal

obstruction.

REFERENCES

Some 1

and kidney.

.

Berdon C:

WE, Baker

2. tion, but no organic obstruction was found and ganglion cells were identified on intestinal biopsies. The infant died at 5 weeks. Pathologic examination showed focal areas without ganghion cells and other areas with an apparent increase in mature ganglion cells. The abdominal musculature was normal and there was no urethral valve bladder

neck

obstruction.

There

was

no

evidence

of

cystic

period. Okulski

TV, Donovan

hypoperistalsis

126:957-964, 1976 RA, Fellows RA, Goodwin CD, KW: Megacystis-microcolon-intestinal

Amoury Ashcraft

a cause J Pediatr TA:

struction 270,

WA, Gay B, Santulhi

syn-

AJR

syndrome: 3.

DH, Blanc

Megacystis-microcolon-intestinal

drome.

or

finding of urinary tract should suggest coincident

the simultaneous polyhydramnios

obstruction

The

using 1977

of

Surg prenatal B scan

intestinal 1 2:1 063-i diagnosis ultrasound:

Hall

obstruction 065,

RT, Holder TM, hypoperistalsis in

the

newborn

1977

of lower a case

urinary

report.

tract

ob-

JCU 5:268-