Paper - Congenital hernia into the umbilical cord - two cases, one associated with persistent cloaca: Difference between revisions

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438
stances must be perfectly aseptic before their
use. Moreover they should be absolutely opaque
and insoluble. We can procure a powder of
choroidian pigment manufactured by Merck, of
Darmstadt. «
Knapp, of Basel, in 1925, obtained good results in tattooing after first scraping the leucoma,
and applying afterwards a 2 per cent gold
chloride solution. This solution can however be
increased in strength to 5 per cent.
Krautbauer, though adopting the same technique, advocated in 1928 the use of aplatinum
chloride solution of 2 per cent.
Finally certain authors employ a silver nitrate ,
solution after scarification of the leucoma.
In so far as I am concerned I have always
been faithful to China ink, and believe that in
experienced hands this ink, employed with
prudenee, can give excellent æsthetic results.
With regard to corneal infection, we know that
in a general manner this substance is sterile,
and when contamination occurs, we must blame
its preparation. Moreover, if the tattooing has
been well done, there is no elimination. Contrary to what Chevallereau and Polack say, I
have never observed, following my numerous
colourings of the cornea with China ink, small
zones of infiltration of the area of the tattooed
THE: GANADIAN Manto-U- Assoomrron JoUnNAn
[Nov. 1938
surfaces Holth advises to try the tattooing in
« one si«tting. I do not share this opinion, but I
rather believe it is better to intervene many
times. Thus the inflammatory reaction is of a
shorter duration, since it disappears two or
three days after the tattooing. · ,
All leucomas do not equally respond to intervention, which must be done only on those which
present old, solid and flat corneal cicatrices. As
to the adherent leucoma, we must practise the
tattooing with a great precaution. For this
small operation there is no need to use fixation
forceps, as advised by certain authors.
It is not necessary to use pilocarpine after the
tattooing, but we must especially beware of
atropine, because Tyson has observed a glaucoma
after having employed this mydriatic.
Placing a bandage over the tattooed eye is
not indicated; » ordinary coloured glasses are
amply sufficient
In conclusion I shall draw the attention a last
time to the importance of using for the tattooing
sterile, very opaque and insoluble substances,
and giving to the needle an oblique direction.
Thus the stable coloured matter penetrates more
deeply into the leucomatous tissue, and offers
for the same reason a better guarantee against
elimination.
CONGENITAL HBRNIA INTO THE UMBILICAL CORD; TWO CASES,
« ONE ASSOCIATED WITH PERSISTENT CLOACA
BY C. W. BURNs, M.D. AND M. A. 0eRYzLo, M.D.
Winmäpeg
»
Oksn 1
HIS was a male baby, about iifteen hours old when
admitted to the Winnipeg General IIospit-al. It was
normally delivered, born of healthy parents. The mother
was thirty—two years old, para II, gravida II, the previous child having been normal.
The baby was born on January 13, 1938, at three
« o’clock in the morning on a farm in rural Manitoba
Transportation to Winnipeg required a ten mile ride by
sleigh in below zero weather, one hundred miles by rai1,
and taxi conveyance to the hospitaL The local physiciam
who had skilfully conducted the accouchement, prepared
the tiny patient for its journey by wrapping it in cottonwool and blankets, and placing it in an ordinary parcel
This made a
comfortable and essentially private conveyance. A male
relative accepted the responsibility of the journey. At
the expected time a basket was calm1y deposited on the
admitting office counter, and from it was extracted the
little patient, apparently none the worse for its unique
experienca
Physical examination revealed a robust, full-term
infant, weighing eight pounds and thirteen ounces, whose
only abnormality was a large hernia into the umbilical
cord, about the size of its head. This hernia contained
practically the whole of the small bowel, and was
covered only by a thin transparent membrane, throu h
which the loops of intestine -and peristalsis could easi y
be seen. The colour of the sac was a pearly grey, while
the intestinal walls were a healthy red. The neck of
the sac was about three inches in diameter, and a small
cuff of skin about one inch wide was carried on to it
from the abdominal wall. The membrane, though intact,
showed local evidence of abrasions and peeling of its
outer layer. «
Since the child presented no other congenital anomaly an immediate operation was decided upon. A
normal passage of urine just before operation relieved
any amciety of the bladder being abnormal.
Bther was administered as a general sanæsthetic in
the usual manner, the period of anæsthesia being fifteen
minutes. Observing strict aseptic technique, the loops
of bowel were with diiiiculty reduced into the abdominal
cavity. The sac was then opened and the abdominal«
wall closed with one continuous chromic cat-gut suture,
catching the deep fascia along with the peritoneum. No
attempt was made to separate the various layers, as
this would only prolong the operation. The cuff of skin
was then trimmed so as to cover over the wound, and
this was closed with deep interrupted silkworm gut
sutures. A small binder was applied to support the
Nov. 19381
wound, and the infant was given a hypodermoclysis of
50 c.c. of 5 per cent glucose solution.
Recovery was uneventful except for a slight wound
infection, and the child Evas discharged after 23 days
of hospitalizatiom weighing 8 pounds and 9 ounces. It
has progressed normally since its discharge from hospital on February 5, 1938. The accompanying photographs taken before and after operation are typical of
the condition.
D1scUss1oN
In the early months of fetal life the greater
portion of the intestines lie in the proximal
part of the umbilical cord, which is called the
extra-coelomic cavity. Normally the intestines
withdraw into the abdominal cavity at about
the tenth to the twelfth week, the umbilical ring
closes, and the extracælomic cavity is thereby
obliterated In rare cases the umbilical ring
does not close, and in these instances variable
portions of the intestines remain in the extracoelomic cavity, which persists.
The size of the hernial mass varies, depending
on the amount of intestines or other abdominal
viscera it may contain. The cyst wall is composed of an outer layer of amnion lined by
peritoneum, being very thin and transparent,
so that its contents are easily seen. These are
usually coils of small intestine, but one may
find almost any of the movable intraperitoneal
Organs, depending on the size of the defect in
the abdominal wall. As a rule there is a cuff
of skin from one-half to one inch wide, which
extends from the abdominal wall on to the neck
of the sac.
The ineidence of this condition is low, being
given by TowI as 1 in 5,000 births for the true
congenital umbilical hernia, as differentiated
from post-natal hernia, which is common. It is
commoner in male infants and is associated with
prematurity. Hempelqlorgensen2 reported two
cases in one family, occurring in boys born a
year apart. The parents were normal.
Treatment.—ln the case of a small hernia
« careful reduction of the contents, with strict
aseptic handling of the sac, may succeed in producing a eure. A tight strapping may be applied
until the wound has completely cicatrizedg This
however may take weeks and is unreliable.
When the hernia is large and not reducible, as,
is frequently the case, surgical operation should
be undertaken as soon as possible after birth. If
the peritoneum is opened, one can more readily
detect other abnormalities, such as persistent
vitello-intestinal duct, or patent urachus, etc.
The cuff of skin facilitates closing of the wound.
Provided there are no other contraindications, a
BURNs AND Damm-o: CONGENITAL HERNIA
general ether anæsthetic, expertly administered,
is apparently quite safe.
Without operation the dangers are incarceration, with strangulation or volvulus, or infection of the sac wall, which has no blood supp1y,
with the development of a fatal peritonitis.
0ne of the most interesting cases reported is
that of Beedk Here the cord had ruptured at a
point about two inches from the abdominal wall.
The bed was filthy, and the intestines were
covered with fæcal matter, straw, and feathers.
Two hours after birth these were cleansed gently
but not thoroughly, and an appendicectomy performed. The loops of bowel were then replaced
and the abdominal wall closed. Recovery was
uneventful.
Cnsn 2
This baby was four hours old on admission to the
Winnipeg General IIospitaL It was a first-born, of
healthy parents, the delivery being normal in all respects.
The duration of the pregnancy was eight months.
Bxamination revealed a premature infant weighing
five pounds and nine ounces, with a gross defect of its
anterior abdominal wall extending from the umbilicus
down through the symphysis to where the anus should
be. In the region of the umbilicus there was a large
hernia into the umbilical cord, about three inches in
diameter. Below this the whole abdominal wall was
de1icient, and was occupied by a large everted sac covered
with mucous membrane, and consisting of three parts,
a pale central portion and two darker lateral masses.
No skin intervened between the hernia into the cord
and the everted mass. In the central portion near the
umbilical hernia was a conical projection with an aperture at its summit, from which meconium was expressed
This was the only opening that could be found, and it
was thought at first to be prolapsed sigmoid. Below
this was a smaller protrusion which could be invaginated
against the imperforate anus to form a small cul—de-sac
about 2 cm. deep. 0n each side, lying against the
thighs, was an everted mass covered with darker mucous
membrane, which was interpreted as representing two
halves of the bladder. An attempt was made to locate
the openings of the ureters but without success. No
external genitalia were present so that the sex was not
determined i
Postmtortesm jindi-w«o.5.—The head, chest and limbs
were normal. The lower half of the anterior abdominal
wall was deiicient of skin and muscles and was covered
only with the scab of the cord. The pubic bone was
absent. Projecting from the usual site of the pubes
and perineum was a red, rounded, everted pouch, three
and a half inches in diameter, covered with mucous
membrane and consisting of the three parts mentioned
above. At the upper end of the middle portion was a
conical projection with a small opening. No other
orilices were apparent, and no external genitalia.
The chest was negative except for a patent foramen
ovale. The Oesophagus and stomach were enormously
dilated, while the duodenum and small bowel appeared
normal, but the ileum ended by opening into the conical
projection in the middle of the large everted pouch
previously described. Thd appendix was seen arising
from the left side of the pouch inside the abdominal
cavity. The liver was enlarged and contained a few
small congenital cysts. The kidneys were large and
cystic, and the ureters dilated. They opened into the
everted lateral masses on each side near the thighs.
This ectopic pouch represented the latter half of the
mid-gut, the hind-gut, and the bladder, the latter in two
halves. Attached to the lateral masses on each side,«
439
440
THE CANADIAN MEDIUM« sAssocusrioN JOIJRNAL
[N0v. 1938
Fig. I. case 1.—showing hernia into umbilical cord. Fig. Z. Gase 1.——Appearance following repair.
Fig, s. Gase 2.—I«ateral view. (a)
»(c) Lateral mass, bladder.
Ilernia into umbilical cord. (b)
Fig. 4. case 2.—Anterior view·.
0pening of ssmall intestine.
(a) Hernia into umbilical cord. (b) Fæces
emerging from opening of small intestine. (c) Lateral masses, bladder. (d) 11nperforate anus.
near the openings of the ureters, was a short tortuous
cord of tissue, about 3 cm. long. A section near its
lower end showed this to be canalized and lined by
stratiiied epithelium two« to four cells thicla These
may have represented rudimentary vaginæ, but ovarian
tissue could not be demonstrated anywhere.
DIsctJssioN
y The above anomaly can be exp1ained on the
basis of an extroverted bladder, with persistence
of the cloaca, and a hernia into the umbilical
cord.
ln the early stages of development (4 weeks)
the cloaca forms a triangle-shaped cavity at
the tai1 end of the embryo, and receives the
openings of the· allantois and rectum, as well as
the Wolffian duct (F’ig. 5a). 1ts ventral wall
is largely formed by the cloacal membrane which
extends upwards on the ventral wall of the
allantois and on to the body stalk or umbilical
cord. This membrane consists only of the two
primitive layers, entoderm and ectoderm.
The cloacal orifice of the rectum then migrates
backwards, leaving the ventral part of the cloaca
to form the bladder and urogenital sinus, and
gives the appearance of these two being separated from the rectum by a septum, called the
urorectal septum (F’ig. 5b). When the recta1
orifice reaches the anal depressions it becomes
separated from the urogenital sinus (Fig. 5c).
In the meantime the cloacal membrane is invaded from each side by mesodermal tissue
growing between the two 1ayers, which then
fuses along the midline, and gives rise to the
infra-umbilical part of the abdominal wall as
well as the anterior wall of the bladder, etc.
This mesoderm arises from the primary mesen
ALAllantois. B.———Bladder. cl.—cloaca. Gm.——Oloacal
membrane. R.—Rectum. Ug.—llrogenital sinus.
Ur.———IJrorectal septum. P.—Penis.
Fig. s. Gase 2.—Ditkerentiation of cloaca.
. Pathology, University of
Nov. 1938]
chyme and as processes of secondary mesoderm
passing around the cloacal membrane from the
hind end of the primitive strealc The urorectal
septum in the male gives rise to» the greater part
of the floor of the penile urethra and perineum
(F’ig. 5d), while in the female the Uterus and
vagina develop in it.
Many theories have been put forward to account for extroversions of the bladder. Roughly
these fall into three groups: (a) Mechanical——where the reason has been given as rupture of
the cloacal membrane, or, persistence of the
membrane so that it is not comp1etely replaced
by mesoderm followed by rupture, or, short or
absent umbilical cord. (l)) Pathological———where
the cause has been given as due to ulceration of
the abdominal wall and necrosis of the symphysis
pubis with separation, or, fetal endometritis.
(c) Arrested development—where it is due to
arrest in development at an early stage of embryonal life, after the formation of the cloaca.
It would seem in this instance that the development had become arrested at a very early
stage, the urorectal septum having been incompletely developed. » The invasion of the cloacal
membrane by mesoderm has also not been completed, so that the whole infraumbilicalpart of
the abdominal wall has failed to form. In practically all of these cases paired appendices and
spina biiida are reported as being accompanying
features. Ours had only one appendix and no
spina bifida, the spinal canal being fused well
along its entire length.
Various degrees of ectopia vesicæ have been
treated surgically with good results. In this
OAMEEON ANDJ OTHER: Gar-I« BLADDER
441
case the malformation was so gross that no surgical procedure could be contemplated. The child
was put on a formula and treated along the
lines of premature infants, but it survived only
liftcen days. «
SUMMARY
Two congenital anomalies have been briefly
presented These are in no way alike, but both
are exceedingly interesting, the first from the
point of view of surgical treatment, the second
from a scientiiic standpoint. No attempt has
been made to discuss the theories of causation
in the second case, and those interested in this
aspect of the subject are referred to the
bibliography «cited.
BIBLIoGRAPHY
1. Tow: Diseases of the Newborn, Oxford Medical Publioation, New York, 1937, D. 224.« .
Z. HnMpnI«-Joncn1s1sDN, P.: Familial congenital umbilical
hernia. TIERE. I. Laeger» 1929, M: 273.
Z. Rand, E. N.: lnfant disemboweled at birthz appendectomy suocessfuh J. Am. M. Ase» 1913. Cl: 199.
4. FKDSHMALH B.: congenital umbilical hernia, Wie
Dei-need 1933. L: 701.
XVI-Hi, SIR A.: I—Iuman Bmbryology and Morphologzk
5th ed., Arnold F: Co» London, 1933, P. 431.
idem: Anomalies of the hind end of the body, Brit.
M. J» 1908, L: 1736. 1804, 1857.
idem: ilvlalformations of the body from a new point
of view, Brit. M. J» 1932, 1: 435, 489.
8. WooD-JoNk1s, R: Flxtroversion of the bladder and
some problems in eonnection with it, J. Anat. es
Physiol» 1912, 46: 193.
9. JoHNsToN, T. B.: Extroversion of the bladder complicated by the presence of intestinal openings on
the surface sof the extroverted area, J.
Find-s» 1914, 48: 89. .
10. VoN Gut-Dann, C. B.: The etiology of extrophy of the
bladder, Arcla Sarg» 1924, s: 61.
II. Verm, J. R. AND MCFDTRIDGEY B. M.: Dxtrophy of the
bladder, J. Pages» 1934, 4: 95.
12. Port-DR, A. B.: Ectopia vesicæ, imperforate rectum
and anus. true hermaphroditism and other ano
malies, Am. J. Sarg» 1936, s1: 172.
13. WYBURIV G. M.: Development of the infra-umbilical
portion of the abdominal wall, with remarlcs on
etiolzoogy of ectopia vesicæ, J. Anat. ck Physiol» 1937,
71: 1.
III-»F«
p
· CALCIUM OARBONATB DBPOSITS IN THE HUMAN GALL BLADDERV
BY A. T. CAMERoN, F. D. WHITE AND SARA MILDLTZER
Wiriiiipeg
FXJE present a chemical examination of two
unusual gall-bladder deposits. The analytical data are preceded by brief clinical notes, and
are followed by a discussion of these and similar
cases in the literature which exhibit this unusual
metabolic abnormality.
N
Gast: 1
Mrs. P.Gk., aged 31, was admitted to the Winnipeg
General I-Iospital on October 18, 1937, with a diagnosis
of cholelithiasis, and a history of gall-bladder trouble
dating back for more than a year, but with no other
« From the Departments of Biochemistry and
Manitoba and Winnipeg
General Eospitah s
unusual conditions pertinent to the present subject. A
gall-bladder visualization indicated that the bladder contained numerous small stones, while two shadows internal
to the bladder suggested stones in the common duct.
(Fi . l . ·
gThkz patient was operated on by Dr. P. II. T.
Thorlalcson on October 20th and recovery was uneventful. A cholecystectomy was done. The pathological
report by one of us (S.M.) reads, «A small thiclcened
and scarred gall bladder with tortuous duct. There
are many small calculi which are bound together in
one solid mass by a sticky yellowish material. The
whole mass entirely fills the gall bladder. Another
small stone bloclcs the cystic duct. Microscopic ex
- amination: Cholecystitis glandularis proliferans.« An
x-ray jilm of the gall-bladder area, made subsequent to
operation, showed no positive shadows. The small: dark
faeeted stone in" the cystic duct was not examined
chemically. «
Anat. ck


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Burns CW. and Ogryzlo MA. Congenital hernia into the umbilical cord; two cases, one associated with persistent cloaca. (1938) Can Med Assoc J. 39(5): 438-41. PMID 20321146

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This historic 1938 paper by Burns and Ogryzlo describes two cases of congenital hernia into the umbilical cord.


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basket equipped with hot water bottles.

438

stances must be perfectly aseptic before their use. Moreover they should be absolutely opaque and insoluble. We can procure a powder of choroidian pigment manufactured by Merck, of Darmstadt. «

Knapp, of Basel, in 1925, obtained good results in tattooing after first scraping the leucoma, and applying afterwards a 2 per cent gold chloride solution. This solution can however be increased in strength to 5 per cent.

Krautbauer, though adopting the same technique, advocated in 1928 the use of aplatinum chloride solution of 2 per cent.

Finally certain authors employ a silver nitrate ,

solution after scarification of the leucoma.

In so far as I am concerned I have always been faithful to China ink, and believe that in experienced hands this ink, employed with prudenee, can give excellent æsthetic results. With regard to corneal infection, we know that in a general manner this substance is sterile, and when contamination occurs, we must blame its preparation. Moreover, if the tattooing has been well done, there is no elimination. Contrary to what Chevallereau and Polack say, I have never observed, following my numerous colourings of the cornea with China ink, small zones of infiltration of the area of the tattooed

THE: GANADIAN Manto-U- Assoomrron JoUnNAn

[Nov. 1938

surfaces Holth advises to try the tattooing in

« one si«tting. I do not share this opinion, but I

rather believe it is better to intervene many times. Thus the inflammatory reaction is of a shorter duration, since it disappears two or three days after the tattooing. · ,

All leucomas do not equally respond to intervention, which must be done only on those which present old, solid and flat corneal cicatrices. As to the adherent leucoma, we must practise the tattooing with a great precaution. For this small operation there is no need to use fixation forceps, as advised by certain authors.

It is not necessary to use pilocarpine after the tattooing, but we must especially beware of atropine, because Tyson has observed a glaucoma after having employed this mydriatic.

Placing a bandage over the tattooed eye is not indicated; » ordinary coloured glasses are amply sufficient

In conclusion I shall draw the attention a last time to the importance of using for the tattooing sterile, very opaque and insoluble substances, and giving to the needle an oblique direction. Thus the stable coloured matter penetrates more deeply into the leucomatous tissue, and offers for the same reason a better guarantee against elimination.

CONGENITAL HBRNIA INTO THE UMBILICAL CORD; TWO CASES, « ONE ASSOCIATED WITH PERSISTENT CLOACA

BY C. W. BURNs, M.D. AND M. A. 0eRYzLo, M.D. Winmäpeg

» Oksn 1

HIS was a male baby, about iifteen hours old when admitted to the Winnipeg General IIospit-al. It was normally delivered, born of healthy parents. The mother was thirty—two years old, para II, gravida II, the previous child having been normal. The baby was born on January 13, 1938, at three

« o’clock in the morning on a farm in rural Manitoba

Transportation to Winnipeg required a ten mile ride by sleigh in below zero weather, one hundred miles by rai1, and taxi conveyance to the hospitaL The local physiciam who had skilfully conducted the accouchement, prepared the tiny patient for its journey by wrapping it in cottonwool and blankets, and placing it in an ordinary parcel This made a comfortable and essentially private conveyance. A male relative accepted the responsibility of the journey. At the expected time a basket was calm1y deposited on the admitting office counter, and from it was extracted the little patient, apparently none the worse for its unique experienca

Physical examination revealed a robust, full-term infant, weighing eight pounds and thirteen ounces, whose only abnormality was a large hernia into the umbilical cord, about the size of its head. This hernia contained practically the whole of the small bowel, and was

covered only by a thin transparent membrane, throu h which the loops of intestine -and peristalsis could easi y be seen. The colour of the sac was a pearly grey, while the intestinal walls were a healthy red. The neck of the sac was about three inches in diameter, and a small cuff of skin about one inch wide was carried on to it from the abdominal wall. The membrane, though intact, showed local evidence of abrasions and peeling of its outer layer. «

Since the child presented no other congenital anomaly an immediate operation was decided upon. A normal passage of urine just before operation relieved any amciety of the bladder being abnormal.

Bther was administered as a general sanæsthetic in the usual manner, the period of anæsthesia being fifteen minutes. Observing strict aseptic technique, the loops of bowel were with diiiiculty reduced into the abdominal

cavity. The sac was then opened and the abdominal«

wall closed with one continuous chromic cat-gut suture, catching the deep fascia along with the peritoneum. No attempt was made to separate the various layers, as this would only prolong the operation. The cuff of skin was then trimmed so as to cover over the wound, and this was closed with deep interrupted silkworm gut sutures. A small binder was applied to support the Nov. 19381



wound, and the infant was given a hypodermoclysis of 50 c.c. of 5 per cent glucose solution.

Recovery was uneventful except for a slight wound infection, and the child Evas discharged after 23 days of hospitalizatiom weighing 8 pounds and 9 ounces. It has progressed normally since its discharge from hospital on February 5, 1938. The accompanying photographs taken before and after operation are typical of the condition. D1scUss1oN

In the early months of fetal life the greater portion of the intestines lie in the proximal part of the umbilical cord, which is called the extra-coelomic cavity. Normally the intestines withdraw into the abdominal cavity at about the tenth to the twelfth week, the umbilical ring closes, and the extracælomic cavity is thereby obliterated In rare cases the umbilical ring does not close, and in these instances variable portions of the intestines remain in the extracoelomic cavity, which persists.

The size of the hernial mass varies, depending on the amount of intestines or other abdominal viscera it may contain. The cyst wall is composed of an outer layer of amnion lined by peritoneum, being very thin and transparent, so that its contents are easily seen. These are usually coils of small intestine, but one may find almost any of the movable intraperitoneal Organs, depending on the size of the defect in the abdominal wall. As a rule there is a cuff of skin from one-half to one inch wide, which extends from the abdominal wall on to the neck of the sac.

The ineidence of this condition is low, being given by TowI as 1 in 5,000 births for the true congenital umbilical hernia, as differentiated from post-natal hernia, which is common. It is commoner in male infants and is associated with prematurity. Hempelqlorgensen2 reported two cases in one family, occurring in boys born a year apart. The parents were normal.

Treatment.—ln the case of a small hernia

« careful reduction of the contents, with strict

aseptic handling of the sac, may succeed in producing a eure. A tight strapping may be applied until the wound has completely cicatrizedg This however may take weeks and is unreliable.

When the hernia is large and not reducible, as,

is frequently the case, surgical operation should be undertaken as soon as possible after birth. If the peritoneum is opened, one can more readily detect other abnormalities, such as persistent vitello-intestinal duct, or patent urachus, etc. The cuff of skin facilitates closing of the wound. Provided there are no other contraindications, a

BURNs AND Damm-o: CONGENITAL HERNIA

general ether anæsthetic, expertly administered, is apparently quite safe.

Without operation the dangers are incarceration, with strangulation or volvulus, or infection of the sac wall, which has no blood supp1y, with the development of a fatal peritonitis.

0ne of the most interesting cases reported is that of Beedk Here the cord had ruptured at a point about two inches from the abdominal wall. The bed was filthy, and the intestines were covered with fæcal matter, straw, and feathers. Two hours after birth these were cleansed gently but not thoroughly, and an appendicectomy performed. The loops of bowel were then replaced and the abdominal wall closed. Recovery was

uneventful.

Cnsn 2

This baby was four hours old on admission to the Winnipeg General IIospitaL It was a first-born, of healthy parents, the delivery being normal in all respects. The duration of the pregnancy was eight months.

Bxamination revealed a premature infant weighing five pounds and nine ounces, with a gross defect of its anterior abdominal wall extending from the umbilicus down through the symphysis to where the anus should be. In the region of the umbilicus there was a large hernia into the umbilical cord, about three inches in diameter. Below this the whole abdominal wall was de1icient, and was occupied by a large everted sac covered with mucous membrane, and consisting of three parts, a pale central portion and two darker lateral masses. No skin intervened between the hernia into the cord and the everted mass. In the central portion near the umbilical hernia was a conical projection with an aperture at its summit, from which meconium was expressed This was the only opening that could be found, and it was thought at first to be prolapsed sigmoid. Below this was a smaller protrusion which could be invaginated against the imperforate anus to form a small cul—de-sac about 2 cm. deep. 0n each side, lying against the thighs, was an everted mass covered with darker mucous membrane, which was interpreted as representing two halves of the bladder. An attempt was made to locate the openings of the ureters but without success. No external genitalia were present so that the sex was not determined i

Postmtortesm jindi-w«o.5.—The head, chest and limbs were normal. The lower half of the anterior abdominal wall was deiicient of skin and muscles and was covered only with the scab of the cord. The pubic bone was absent. Projecting from the usual site of the pubes and perineum was a red, rounded, everted pouch, three and a half inches in diameter, covered with mucous membrane and consisting of the three parts mentioned above. At the upper end of the middle portion was a conical projection with a small opening. No other orilices were apparent, and no external genitalia.

The chest was negative except for a patent foramen ovale. The Oesophagus and stomach were enormously dilated, while the duodenum and small bowel appeared normal, but the ileum ended by opening into the conical projection in the middle of the large everted pouch previously described. Thd appendix was seen arising from the left side of the pouch inside the abdominal cavity. The liver was enlarged and contained a few small congenital cysts. The kidneys were large and cystic, and the ureters dilated. They opened into the everted lateral masses on each side near the thighs. This ectopic pouch represented the latter half of the mid-gut, the hind-gut, and the bladder, the latter in two halves. Attached to the lateral masses on each side,«


439 440

THE CANADIAN MEDIUM« sAssocusrioN JOIJRNAL

[N0v. 1938

Fig. I. case 1.—showing hernia into umbilical cord. Fig. Z. Gase 1.——Appearance following repair.

Fig, s. Gase 2.—I«ateral view. (a) »(c) Lateral mass, bladder.

Ilernia into umbilical cord. (b) Fig. 4. case 2.—Anterior view·.

0pening of ssmall intestine.

(a) Hernia into umbilical cord. (b) Fæces

emerging from opening of small intestine. (c) Lateral masses, bladder. (d) 11nperforate anus.

near the openings of the ureters, was a short tortuous cord of tissue, about 3 cm. long. A section near its lower end showed this to be canalized and lined by stratiiied epithelium two« to four cells thicla These may have represented rudimentary vaginæ, but ovarian tissue could not be demonstrated anywhere.

DIsctJssioN

y The above anomaly can be exp1ained on the basis of an extroverted bladder, with persistence of the cloaca, and a hernia into the umbilical cord.

ln the early stages of development (4 weeks) the cloaca forms a triangle-shaped cavity at the tai1 end of the embryo, and receives the openings of the· allantois and rectum, as well as the Wolffian duct (F’ig. 5a). 1ts ventral wall is largely formed by the cloacal membrane which extends upwards on the ventral wall of the allantois and on to the body stalk or umbilical cord. This membrane consists only of the two primitive layers, entoderm and ectoderm.

The cloacal orifice of the rectum then migrates backwards, leaving the ventral part of the cloaca to form the bladder and urogenital sinus, and gives the appearance of these two being separated from the rectum by a septum, called the urorectal septum (F’ig. 5b). When the recta1 orifice reaches the anal depressions it becomes separated from the urogenital sinus (Fig. 5c).

In the meantime the cloacal membrane is invaded from each side by mesodermal tissue growing between the two 1ayers, which then

fuses along the midline, and gives rise to the infra-umbilical part of the abdominal wall as well as the anterior wall of the bladder, etc. This mesoderm arises from the primary mesen ALAllantois. B.———Bladder. cl.—cloaca. Gm.——Oloacal membrane. R.—Rectum. Ug.—llrogenital sinus. Ur.———IJrorectal septum. P.—Penis.

Fig. s. Gase 2.—Ditkerentiation of cloaca. . Pathology, University of

Nov. 1938]



chyme and as processes of secondary mesoderm passing around the cloacal membrane from the hind end of the primitive strealc The urorectal septum in the male gives rise to» the greater part of the floor of the penile urethra and perineum (F’ig. 5d), while in the female the Uterus and vagina develop in it.

Many theories have been put forward to account for extroversions of the bladder. Roughly these fall into three groups: (a) Mechanical——where the reason has been given as rupture of the cloacal membrane, or, persistence of the membrane so that it is not comp1etely replaced by mesoderm followed by rupture, or, short or absent umbilical cord. (l)) Pathological———where the cause has been given as due to ulceration of the abdominal wall and necrosis of the symphysis pubis with separation, or, fetal endometritis. (c) Arrested development—where it is due to arrest in development at an early stage of embryonal life, after the formation of the cloaca.

It would seem in this instance that the development had become arrested at a very early stage, the urorectal septum having been incompletely developed. » The invasion of the cloacal membrane by mesoderm has also not been completed, so that the whole infraumbilicalpart of the abdominal wall has failed to form. In practically all of these cases paired appendices and spina biiida are reported as being accompanying features. Ours had only one appendix and no spina bifida, the spinal canal being fused well along its entire length.

Various degrees of ectopia vesicæ have been treated surgically with good results. In this

OAMEEON ANDJ OTHER: Gar-I« BLADDER

441

case the malformation was so gross that no surgical procedure could be contemplated. The child was put on a formula and treated along the lines of premature infants, but it survived only liftcen days. «

SUMMARY

Two congenital anomalies have been briefly presented These are in no way alike, but both are exceedingly interesting, the first from the point of view of surgical treatment, the second from a scientiiic standpoint. No attempt has been made to discuss the theories of causation in the second case, and those interested in this

aspect of the subject are referred to the bibliography «cited.

BIBLIoGRAPHY

1. Tow: Diseases of the Newborn, Oxford Medical Publioation, New York, 1937, D. 224.« .

Z. HnMpnI«-Joncn1s1sDN, P.: Familial congenital umbilical hernia. TIERE. I. Laeger» 1929, M: 273.

Z. Rand, E. N.: lnfant disemboweled at birthz appendectomy suocessfuh J. Am. M. Ase» 1913. Cl: 199.

4. FKDSHMALH B.: congenital umbilical hernia, Wie Dei-need 1933. L: 701. XVI-Hi, SIR A.: I—Iuman Bmbryology and Morphologzk 5th ed., Arnold F: Co» London, 1933, P. 431.

idem: Anomalies of the hind end of the body, Brit. M. J» 1908, L: 1736. 1804, 1857.

idem: ilvlalformations of the body from a new point of view, Brit. M. J» 1932, 1: 435, 489.

8. WooD-JoNk1s, R: Flxtroversion of the bladder and some problems in eonnection with it, J. Anat. es Physiol» 1912, 46: 193.

9. JoHNsToN, T. B.: Extroversion of the bladder complicated by the presence of intestinal openings on the surface sof the extroverted area, J. Find-s» 1914, 48: 89. .

10. VoN Gut-Dann, C. B.: The etiology of extrophy of the bladder, Arcla Sarg» 1924, s: 61.

II. Verm, J. R. AND MCFDTRIDGEY B. M.: Dxtrophy of the bladder, J. Pages» 1934, 4: 95.

12. Port-DR, A. B.: Ectopia vesicæ, imperforate rectum and anus. true hermaphroditism and other ano malies, Am. J. Sarg» 1936, s1: 172.

13. WYBURIV G. M.: Development of the infra-umbilical portion of the abdominal wall, with remarlcs on etiolzoogy of ectopia vesicæ, J. Anat. ck Physiol» 1937, 71: 1.

III-»F«

p

· CALCIUM OARBONATB DBPOSITS IN THE HUMAN GALL BLADDERV

BY A. T. CAMERoN, F. D. WHITE AND SARA MILDLTZER

Wiriiiipeg

FXJE present a chemical examination of two

unusual gall-bladder deposits. The analytical data are preceded by brief clinical notes, and are followed by a discussion of these and similar cases in the literature which exhibit this unusual

metabolic abnormality.

N

Gast: 1

Mrs. P.Gk., aged 31, was admitted to the Winnipeg General I-Iospital on October 18, 1937, with a diagnosis of cholelithiasis, and a history of gall-bladder trouble dating back for more than a year, but with no other

« From the Departments of Biochemistry and Manitoba and Winnipeg General Eospitah s

unusual conditions pertinent to the present subject. A gall-bladder visualization indicated that the bladder contained numerous small stones, while two shadows internal to the bladder suggested stones in the common duct. (Fi . l . ·

gThkz patient was operated on by Dr. P. II. T. Thorlalcson on October 20th and recovery was uneventful. A cholecystectomy was done. The pathological report by one of us (S.M.) reads, «A small thiclcened and scarred gall bladder with tortuous duct. There are many small calculi which are bound together in one solid mass by a sticky yellowish material. The whole mass entirely fills the gall bladder. Another small stone bloclcs the cystic duct. Microscopic ex - amination: Cholecystitis glandularis proliferans.« An

x-ray jilm of the gall-bladder area, made subsequent to operation, showed no positive shadows. The small: dark faeeted stone in" the cystic duct was not examined chemically. «

Anat. ck


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