extending from the thorax to the lower part of the abdomen, and forms a abdominal viscera, the ...

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A CASE OF ECTOPIA VISCERUM, ASSOCIATED WITH SPINA
BIFIDA AND OTHER ABNORMALITIES.' By Dr E. EMRYS-
ROBERTS and Professor A. MELVILLE PATERSON, Liverpool.
THE subject of this memoir is a full-termn fetus, born dead, and we are
indebted to Dr E. T. Davies of Liverpool for the opportunity of describing
a somewhat rare condition.
The father of the child died,at the age of thirty-two, two months before
its birth, of acute phthisis,accelerated by alcoholism. The mother (iv. para)
is a healthy woman aged thirty years. Her three preceding children were
healthy and normal in every respect. There was no noticeable feature
about the pregnancy or labour. On the arrival of the accoucheur, the
membranes had already ruptured, and the viscera and a hand were pre-
senting. During strong expulsive pains delivery was accomplished, the
dead fetus being expelled with some force. The viscera and trunk caine
first, then the lower extremities, which were acutely flexed on the spine,
followed by the shoulders and head,and lastly,the membranes and placenta.
EXTERNAL APPEARANCES. (Fig. 1.)
The body of the child measures 9 inches from head to perineum. The
head and limbs are well formed, but the trunk is in a position of acute
opisthotonos, the legs embracing the scalp posteriorly. A spina bifida in
the lumbo-sacral region forms a fluctuating swelling the size of a hen's
egg. In the middle line of the perineum is a blind proctodceal depression;
on the left side of the thigh, on the level of this depression, is a small
pedunculated papilla, and on the right thigh there is a corresponding sessile
elevation (fig. 3). The amnion is attached to the skin alongan oval margin
extending from the thorax to the lower part of the abdomen, and forms a
large sac completed by its attachment to the placenta, and containing the
abdominal viscera, the pericardium and heart, and the left lung.
PLACENTA AND MEMBRANES. (Figs. 1 and 2.)
The placenta is discoidal and measures 6 x5 inches. Its foetal surface
iscovered bythe amnion, which at the margin of the placenta becomes con-
1 Read before the Anatomical Society, January 19, 1906.
Ectopia Viscerum, associated with Spina bifida, etc. 333
tinuous with the other fetal membranes and lines the amniotic sac. The
amnion is reflected offthe placenta near its centre, at the attachment of the
umbilical vessels, and becomes the wall of the ectopic sac, attached to the
body wall of the foetus along an oval line already referred to. The
Fie., 1.-Photograph ofthespecimenasreceived.
umbilical vessels (one artery and one vein) are carried from the foetus to
the placenta in the hinder part of the wall of this sac. The placental end
of the sac is adherent at several places to the amnion covering the feetal
surface of the placenta by means of delicate septal bands.
There are thus two distinct and separate cavities: (1) the ccelomic
cavity, the pleuro-peritoneal cavity of the feetus, and the ectopic sac, which
represents the persistence of the extra-embryonic ccelom; and (2) the
334 Dr E. Ernrys-Roberts and Professor A. Melville Paterson
FIG. 2.-Diagrammatic reproduction of the specimen representing asagittal section
throughthetrunkandectopic cavity. Thedotted line(* *)representsthelimit
oftheabdominalcavity.
A, spina bifida; B, pericardium; C, left lung; D, stomach; B, mesentery; F, small
intestine; G, cecal pouch andleftappendage; H,liver; J, placenta;K,umbilical
vessels; L,fibrous cord connectingtheguttothecoccyxandtotheposterior endof
the spinal cord; M., proctodwal recess; N, cloacal papilla; 0, termination of
intestine; P, bladder-like dilatation; Q, border of internal cloaca; R, external
cloacalopening; 8,cloacalcavityandopeningofbladder-likedilatation.
Ectopia Viscerum, associated with Spina bifida, etc. 335
cavity of the anmnion itself, in which the fetus and the ectopic sac are
contained.
External cloaca.-Between the two cavities is a natural opening or
external cloaca (fig. 3). This is a median longitudinal fissurefive-eighths of
an inch in length, which occurs chiefly in the amnion, but also encroaches
LEXTT4AL/
<s0 OF( /
\>
PAPSLM
/ . I PAPILA
FIG. 3.-Drawingoftheperinealregion, toshowtheproctodceurn,
externalcloaca, andgenital papille.
on to the skin for about a quarter of an inch. It lies three-quarters of an
inch in front of the proctodoeal depression. It not only forms a natural
communication between the amniotic cavity and the coelom, but, as will
be described later, it is also in relation to the opening of the internal
cloaca.
Abdominal wall-.The two halves of the abdominal wall, widely
separated from one another and forming on each side the fetal attachment
of the ectopic sac, consist of the following muscles and their aponeuroses:
336 Dr E Emrys-Roberts and Professor A. Melville Paterson
(1) obliquus externus, with an attachment above to the fifth, sixth, and
seventh ribs and below by a membranous band to the hip-bone: possessing
a well-developed aponeurosis, whichservesfortheattachment of theserratus
magnus, pectoralis major, pyramidalis and sternalis; (2) obliquus internus,
inserted byan aponeurosis into the border of the eighthcostalcartilage,and
into the skin-amnion junction; (3) transversalis, arising from the eighth,
ninth,and tenth costalcartilagesandinsertedintotheskin-amnionjunction;
-_
FIG. 4.-Drawingtoshowthedeepsurfaceoftheexternalcloaca
andthetermination ofthegut(withoutdissection).
(4) rectus abdominis, narrow and rounded, with an upper attachment to
the third costal cartilage, and a lower attachment by a long narrow
tendon into the pubis; (5) pyramidalis, a long narrow muscle lying
superficial to the rectus abdominis in its lower third; (6) sternalis, a well-
developed muscle on both sides in the same plane as the pyramidalis, and
superficial to the pectorals major and rectus abdominis.
THE ECTOPIC SAC AND ITS CONTENTS.
The ectopic sac was ruptured at the time of delivery, but its wall is
nevertheless complete. The deep surface of the external cloaca is seen
topossess a puckered margin (fig. 4), which is joined on each side by the
Ectopia Viscerum, associated with Spina bifida, etc. 337
edges of the internal cloaca. This is a narrow slit opening from the
cloaca into the ccalom. It is almost completely filled up by the projection
into it of a prominent papilla. A probe passed through the opening
enters in the middle line into the endof the intestinal canal, while on either
side of the papilla into the cavity of a thick-walled muscular sac which
on dissection resembles a bladder in structure. The connection of the
margins of the external cloaca and internal cloaca thus allows a probe,
passed through the external cloaca, to pass either into the celoon or
through the coelom into the internal cloacal opening.
The interiorof theectopic sacis linedbya serouscovering continuous and
identical with the peritoneum. In the wall of the sac, as already noted,
the right umbilical artery and vein course to the placenta. There is no
trace of left umbilical vessels. The right artery passes an eighth of an inch
from the right side of the external cloaca. The vein is half an inch further
out.
The interior of the sac includes the peritoneal cavity, and, on account
of a deficiency of the left half of the diaphragm, the rudimentary left
pleural sac. On the left side the pleuro-peritoneum is prolonged upwards
within the postal margin for only a short distance. It forms, in addition,
a small blind pouch or further prolongation at the root of the left lung.
The right pleural sac is completely isolated, and will be described in the
account of the thorax.
Tlhe contents of the ectopic sac and pleuro-peritoneal cavity are: the
lower two-thirds of the pericardium and heart, the left lung, stomach,
spleen, liver, and intestines. Attached to the posterior abdominal wall are
the kidneys, genital glands, pancreas, duodenum, blood-vessels, and the
structures involved in forming the cloacal cavity.
THE DIAPHRAGM.
The right half of the diaphragm forms a continuous, fibro-muscularpar-
tition separating the right pleural cavity from the abdominal cavity. It is
pierced by the inferior vena cava, and is covered by peritoneum on its
abdominal aspect. Its muscular fibres arise from three peripheral origins:
(1) anteriorly and on the right side, from the postal margin, (2) and
(3) posteriorly from the vertebral column by two bundles of muscular fibres
separated from one another by a plexiform mass of sympathetic nerves
and lymphatics which lie on the right side of the aorta. It is supplied by
the right phrenic nerve.
The lefthalf of thediaphragm is represented byamuscularbundlewhich
arises from the under surface of the left ninth postal cartilage, and passes
VOL. XL. (THIRD SER. VOL. I.)-JULY 1906. 27
338 Dr E. Enirys-Roberts and Professor A. Melville Paterson
between, first, the leftside of the (esophagusandleft lung, and then between
the liver and the pericardium on the one hand and the stomach oin the other
hand, to blend with the connective tissue of the small oinentuin and the
hilum of the liver. It is supplied by the left phrenic nerve.
PERITONEAL FOLDS.
The liver possesses a well-inarked falcifum ligament (in the free edge of
which the right umbilical vein courses), and distinct right and left lateral
ligaments connecting it to the diaphragm.
The stomach is connected to the liver by a small oinentumn. The great
omentuin extends from its greater curvature and blends below with the
root of the inesentery proper, which encloses the coils of the small intestine.
A short septal band of peritoneum attaches the border of the fundus of the
stomach to tile wall of the sac between the left lung and suprarenal
capsule.
The spleen is covered entirely by peritoneum, and is attached to the
fundus of the stomach by a gastro-splenic omentuin and to tile posterior
abdominal wall by a lieno-renal ligament.
The lesser sac is well developed, and communicates with the greater sac
by means of a large foramen of Winslow.
ABDOMINAL VISCERA.
The cesoplhagus enters the abdomen between the two halves of the
diaphragm.
The 8stowmch is well developed, and possesses a distinct pylorus. The
duodenum is normal in position and curvature and is of large calibre.
Thejejt)n?U aOid ileitm, together measure 26 inches in length. For 22
inches the calibre of the tube is uniform. It then dilates, ultimately
narrowing again at its termination. The interior of the tube in its whole
length isprovided with well-marked valvuloe conniventes.
Ter,)nuntwa of the sall intestive.-Half an inch from the internal
cloaca the small intestine ends in an enormously dilated caecal pouch,
measuring 2 by 11 inches (fig. 4). It is slightly sacculated on its exterior,
and is situated on the right side of the lower part of the abdominal cavity.
Behind it is a large ileo-caecal pouch of peritoneum, bounded by a fold of
peritoneum by which the cecal pouch is connected with the mesentery.
On each side of the jummction of tIle small intestine with the cuccal pouch
is a small, sacculated and curved, appendix. Thiscecalpouchcommunicates
by a narrow nieck with the upper end of the cavity of the cloaca.
Ectopia Viscerum, associated with Spina bifida, etc. 339
Attached to its dorsal wall is a thick fibrous cord partially enveloped in
muscular fibres, which is directed dorsally, narrowing as it goes, to be
attached chiefly to the tip of the coccyx. Behind the coccyx it is con-
tinuous with another cord-a prolongation from the sac of the spina
bifida.
The first-namned cord represents a post-anal gut. Along with the pro-
longation from the spina bifida the structure constitutes the remains of the
neurenteric canal. The interior of the coecal pouch is lined by a mucous
membrane, which presents numerous fine rugosities.
The liver is large and irregular. Its parietal surface, convex andkidney-
shaped, is wholly covered by peritoneum, and is in contact with the
abdominal wall, right half of the diaphragm,and pericardium. It possesses
no peritoneal folds.
The visceral surface of the liver presents numerous fissures. The
fissure for the gall-bladder extends from the anterior border of the liver
to the hilum. To the left of this is a superficial fissure, representing the
anterior portion of the longitudinal fissure and the position of the (absent)
left umbilical vein. The hilum and its vessels and ducts are normal. The
gall-bladder is small. The fissure for the ductus venosus is a deep fissure.
It attaches part of the small omentum, and receives the muscular fibres of
the rudimentary left half of the diaphragm. The fissure for the inferior
vena cava is also deep. It lies behind the spigelian lobe, and has three
peritoneal folds connected with it: the right and left lateral ligaments, and
the falcifum ligament. A deep notch occurs on the posterior border of the
liver for the entrance of the right umbilical vein. The several lobes of the
liver can be readily made out: the spigelian lobe is especially prominent,
projecting for over an inch into the lesser sac, behind the stomach and
duodenum.
The j)ortal veils is normal. On entering the hilum it forms a large
sinus, and divides into right and left branches. The left branch is in
continuity with the ductus venosus, which connects it to the inferior vena
cava in the usual way. As already said, there is no traceof a leftumbilical
vein. The right branch of the portal vein becomes embedded in the liver,
where it is joined by the right umbilical vein. This vein, after coursing
over the posterior abdominal wall, enters the free edge of the falcifum
ligament; passing over the right kidney, it enters the liver, and after a
course of half an inch opens into the right branch of the portal vein.
The spleeantnla pancreas are normal in position and relations.
The right kAidney (tand( satpralrenalcl capsidle lie onthe posterior abdominal
wall, covered over by peritoneum. Both are related to the liver, falcifum
ligament, duodenum, and the ceecum. The hilum of the kidney occupies its
340 Dr E. Emrys-Roberts and Professor A. Melville Paterson
anterior surface. The ureter descends vertically to terminate in amuscular
cul-de-sac, which communicates with the cavity of the cloaca.
The left kidney and s&pratrenal capsule are separated by peritoneum
from the spleen, left lung, and the lesser sac of the peritoneum. The lieno-
renal ligament is attached to the kidney. The ureter has an origin, course,
and termination just as on the right side. The renal vessels will be
described later.
The yeniital glands are similar on the two sides: each is attached by
a peritoneal fold to the lower end of the kidney. The gland is ovoid in
shape, laterally compressed, and about half an inch in length. It is divisible
into two parts: a basal part, soft and yellowish in colour and covered by
glistening peritoneum, and a smaller portion, separated by a marginal
fissure, brownish in colour, rough and wrinkled.
The genital duct has a different course andtermination on the two sides.
The right duct is represented by a fibrous cord which passes downwards
and outwards to the groin. It pierces the tissues of the abdominal wall,
and merges with a strong band of fascia attached to the pubis ( ? Poupart's
ligament). The left duct is pervious. It passes downwards over and
internal to the left ureter, and ends by joining a thick, glandular mass
( ? prostate) placed behind the cloacal chamber. Each genital gland is
supplied by branches from the renal vessels.
Cloaca.-Below the termination of the intestine, with its caecal
dilatation and bilateral appendices, a slit-like opening about half an inch in
length occurs in the ventral wall of the cloacal cavity, which allows of a
communication with the celom. This is the internal cloacal orifice. Its
margins are continuous above with the anterior margin of the opening of
the gut, and below with the sides of the external cloacal orifice (fig. 2).
The opening is almost completely filled up by the projection into it of a
cloacal papilla which projects from the dorsal wall of the cloacal chamber
and divides it into two lateral parts. The papilla possesses fraena, superior
and inferior. The superior fraenum passes upwards to the left side of the
opening of the gut: the inferior froenum descends in the middle line to the
orifice of the cloaca. The lateral walls of the cloacal chamber are thick
and muscular, and form on each side a small, almost spherical, bladder-likce
sac which opens into the cavity of the cloaca on each side of the papilla
by a wide,slit-like mouth. Each of these sacs is covered over anteriorly by
peritoneum, and each receives at its upper pole the termination of the
ureter.
Behind the dorsal wall of the cloacal chamber, above the side of the
cloacal papilla and below the attachment of the post-anal gut, and between
the urinary diverticula, is a solid median glandular mass, resembling the
Ectopia Viscerumn, associated with Spina bifida, etc. 341
prostate glanl. It intervenes betweenthe post-anal gut and the proctodeal
diverticulum, and, as already noted, receives the termination of the left
-genital duct.
The cloacal ))apdila attached to the dorsal wall of the cloacal chamber
has a clubbed and fissured extremity which projects through the internal
cloacal opening. On dissection the papilla is found to be composed of a
dense mass of fibrous tissue, and it contains in its centre a gradually
narrowing fibrous cord, easily detachable from its surroundings, and trace-
able into continuity with a narrow tube which finally opens on the surface
of the perineum as the proctodawal depression.
The perinean.-The pubic bones are widely separated from one an-
other. The two ischia are connected together by a strong, fibro-muscular
septum, which separates the spina bifida sac from the ischio-rectal fossee.
This sheet is pierced in the middle line by a fibrous cord which extends
from the spina bifida sac to the tip of the coccyx. The proctodceal depres-
sion lies between the two ischio-rectal fossee. It is half an inch in depth
and forms a cylindrical tube continued into the narrowing fibrous cord
already described as penetrating and ending in the substance of the
cloacal papilla. On each side is the levator ani muscle, behind which is the
coceygeus. The pudic vessels and nerves traverse the outer wall of the
fossa. In addition, a fibrous band comparable to the triangular ligament
and muscular bundles corresponding to the transversus perinei and com-
pressor urethra can be made out, in front of the proctodwceuin, between
the widely separated pubic bones.
ABDOMINAL VESSELS.
The al)bdontial aorta (fig. 5) is a short vessel. At the level of the
kidneys it gives off several visceral branches which divide to supply the
kidneys, suprarenal capsules, genital glands, and spleen. Other arteries
which were cut entered the mesentery to supply the gut. On the left side
a slender artery arises which soon divides into two: one branch was cut;
the other is the pudic artery, whichcourses alongtheleftside of the sacrum,
supplies the perineum, and terminates in the cloacal papilla. On the right
side also the pudic artery is a direct branch of the aorta.
The external ilac arteries arise differently on the two sides, The left
artery arises from the common trunk from which the renal, genital, supra-
renal, and splenic arteries arise; the right artery arises from the aorta,
just above the origin of the pudic artery. These two vessels supply sub-
ordinate branches to the buttocks, and proceed onwards to the lower limbs,
to form the femoral arteries. The internal iliac arteries arise directly from