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Saunders JB. and Lindner HH. [[Paper - Congenital anomalies of the duodenum (1940)|'''Congenital anomalies of the duodenum''']]. (1940) {{Amer. J Anat.}}, <noinclude>[[Category:Template]][[Category:Reference]][[Category:Historic Embryology]][[Category:1940's]][[Category:Duodenum]]</noinclude>
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Saunders JB. and Lindner HH. [[Paper - Congenital anomalies of the duodenum (1940)|'''Congenital anomalies of the duodenum''']]. (1940) Annals of Surgery 112{3}: 321-338.<noinclude>[[Category:Template]][[Category:Reference]][[Category:Historic Embryology]][[Category:1940's]][[Category:Duodenum]]</noinclude>
 
 
ANNALS OF SURGERY
 
 
 
VOL. 112 SEPTEMBER, 1940 No. 3
 
EE
 
es
 
 
 
   
 
 
 
congenital anomalies of the duodenum
 
 
 
Joux B. DEC. M. Saunpers, M.B., Cx.B., F.R.CS. (Ep),
 
AND
 
Harozp H. Linpner, MD.
 
San Francisco, Calif.
 
 
 
From The Department Of Applied Anatomy And Division Of Surgery, Univ Ersity Of California Medical School,
 
San Francisco, Calif.
 
 
 
Submitted for publication June 29, 1930.
 
 
 
CONGENITAL ANOMALIES of the duodenum are of sufficient rarity and
 
interest to warrant a report when encountered. We have had the fortune to
 
 
 
 
 
 
 
Fic. 1.—Case 1: Radiograph three hours after barium meal showing almost complete retention. À minute quantity of barium has passed into the
 
proximal jejunum.
 
 
 
examine and study, in some detail, three cases. These cases have offered the
 
opportunity of assessing the various theories as to their etiology and of
 
 
 
 
 
examining, in this respect, several details of duodenal development which
 
serve to clarify the subject. In addition, a short review of the classification,
 
incidence, diagnosis and treatment of such anomalies is considered.
 
 
 
The following is a short résumé of the three cases which prompted our
 
interest in this subject. The first of them illustrates an example of congenital
 
 
 
 
 
 
 
 
 
F1G. 2.—Appearances on opening abdomen in Case 1. The hepatoduodenal adhesions fix the coiled duodenum against the greater curvature of the stomach. The colon is not visible as owing to nonrotation
 
it lies behind and to the left of the small bowel.
 
 
 
duodenal stenosis associated with nonrotation of the intestine ; the second, of
 
congenital duodenal valve formation, and the third, a case exhibiting abnormalities of shape, position and fixation. All these cases were associated, as is
 
so common in congenital malformation, with other anomalies.
 
 
 
Case 1.—Baby H., male, birth weight 7 Ibs. 10 oz. The baby was spontaneously
 
delivered at term, markedly jaundiced. Meconium was passed at the end of the first 24
 
 
 
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Yolume 112 ANOMALIES OF DUODENUM
 
 
 
Number 3
 
 
 
hours. On routine formula, the infant nursed poorly. On the third day, the child regurgitated large quantities of sour-smelling food but passed a large brownish-yellow stool.
 
From the third to the seventh day, he vomited repeatedly. The intensity of the jaundice
 
increased and the child rapidly lost ground. There was a weight loss of 18 oz. by the
 
seventh day.
 
 
 
Physical Examination.—March 26, 1938: There was considerable loss of tissue turgor
 
and deep icterus. The abdomen was distended in both upper quadrants and reversed
 
gastric peristalsis was observed after feeding. No pyloric tumor was palpable but considerable gastric dilatation was determined. There was bilateral talipes equinovarus.
 
Radiographic examination revealed dilatation of both esophagus and stomach with con
 
 
 
 
F1G. 3.—Case 1: Following division of peritoneal adhesions.
 
 
 
siderable food retention. No barium passed the pylorus. At three hours, retention was
 
almost complete (Fig. 1.). However, a minute quantity of barium was seen in the
 
proximal jejunum. Preoperative Diagnosis: Pyloric stenosis or spasm.
 
 
 
Operation.—-March 26, 1938: Following preoperative supportive measures celiotomy
 
was carried out. The liver presented early and although of normal size, its unusually
 
mottled brownish color was noted. The transverse colon was absent from its usual position below the greater curvature of the stomach, being replaced by coils of small intestine.
 
The colon was found accumulated on the left side, indicating nonrotation. The pyloric
 
region was obscured by a persistent hepatoduodenal ligament (Fig. 2). On division of this
 
peritoneal ligament, the duodenum was found matted together by adhesions in the form
 
of an S-shaped loop and fused with the greater curvature of the stomach below the
 
pylorus (Fig. 3). On further dissection the curvature of the duodenum was restored, it
 
being found to be unfixed, suspended by a mesoduodenum.
 
 
 
À stenotic area, three-quarters of an inch long, reducing the bowel caliber to oneeighth of an inch, involved the proximal segment of the second and distal portion of the
 
first part of the duodenum (Fig. 4). On longitudinal incision of this area, a lumen the
 
size of a pencil lead was encountered. The openings of the pancreatic and biliary ducts
 
were not observed. The diameter of the lumen was increased by closure of the incision
 
 
 
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SAUNDERS AND LINDNER Annals of Surgers
 
 
 
transversely, as in the Heinecke-Mikulicz procedure, and the lumen now judged to be of
 
adequate size. After closure the child was returned to bed in fairly good condition.
 
 
 
The postoperative course was uneventful, except that the infant vomited once on the
 
second day. He was discharged on the seventh postoperative day.
 
 
 
S'ubsequent Course.—Fifteen days after operation the child died, following a convulsion associated with a respiratory infection. Autopsy was refused. Jaundice, though decreasing, persisted up to the time of death.
 
 
 
Case 2.—S. M. W., age 7%, white, was brought to the University of California Hospital, because of frequent and persistent vomiting since one month following birth. The
 
 
 
 
 
 
)
 
 
 
F1G. 4—Case 1: The duodenal loop has been freed by further
 
dissection of adhesions. The stenotic area and the unfixed mesoduodenum are clearly shown.
 
 
 
child had been apparently normal up to the age of one month, at which time she began to
 
have spells of projectile vomiting following each feeding. During the next two months
 
the child dropped from a birth weight of 736 Ibs. to about 4 1bs. At the age of three
 
months, the child was taken to a private hospital where she remained for the next nine
 
months. The vomiting continued and her weight increased to 9 1bs. At 15 months, vomiting
 
persisting, a preoperative diagnosis of congenital hypertrophic pyloric stenosis was made
 
and celiotomy performed. Examination revealed a large gaping pylorus, many veil-like
 
adhesions from the gallbladder to the duodenum, a large mesoduodenum and a uniform
 
collapse of the distal two-thirds of the duodenum and small intestine. Many enlarged
 
lymph nodes, thought to be tuberculous, were found in the mesentery. A long Meckel’s
 
diverticulum was present. There was no operative intervention and a postoperative diagnosis of “tuberculosis of the mesentery and intestines” was made.
 
 
 
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joume 122 ANOMALIES OF DUODENUM
 
 
 
S'ubsequent Course—The child rernained a hospital case until the age of three, continuing to vomit, and on discharge her weight was only 18 Ibs. For the following four
 
years, up to the age of seven, and her entry into the University of California Hospital, the
 
child continued to vomit after each meal, at times bringing up food eaten several days
 
previously. She gained weight slowly, and was constantly under medical care for seven
 
years. Physical examination on entry showed an underweight, pallid, dehydrated child.
 
No visible peristalsis or palpable organs. No other positive findings. Laboratory data
 
were essentially normal, including a negative tuberculin test. Radiographic examination,
 
made on entry, seemed to indicate a definite obstruction in the second or third portion of
 
 
 
 
 
 
Fic. 5.—Case 2: Radiograph three hours after barium meal showing dilated
 
stomach and megaduodenum with obstruction.
 
 
 
duodenum. (Fig. 5.) Preoperative Diagnosis: Congenital stenosis of the second or third
 
part of the duodenum.
 
 
 
Operation.—April 19, 1937 : The transverse colon and omentum appeared to be normal.
 
The mesentery of the small bowel and duodenum contained many hard, enlarged lymph
 
nodes of varying size. The entire small bowel distal to the ligament of Treitz was moderately collapsed and appeared quite normal. The stomach was markedly dilated and
 
flabby and extended well down into the pelvis. The stomach walls were very thick. The
 
pylorus admitted three fingers, and the first and a part of the second portion of the
 
duodenum were markedly dilated and hypertrophied. The common bile duct was moderately dilated and entered the duodenum at an abnormally high position. The cecum had
 
not descended from under the liver and was suspended by a mesentery.
 
 
 
In view of the foregoing findings it was decided that the procedure of choice was
 
mobilization of the duodenum to determine the cause and site of the obstruction rather
 
than a palliative gastro-enterostomy. Upon freeing and rolling up the duodenum, a
 
 
 
325
 
SAUNDERS AND LINDNER ‘ gun of Sureer
 
 
 
circular ring of constriction, four inches distal to the pylorus, was found. The third and
 
fourth portions of the duodenum distal to this constriction were of normal size. An incision was made directly over the constricted area and a diaphragm was encountered, completely occluding the lumen save for a small perforation anteriorly, which barely admitted
 
the tip of the little finger. The diaphragm was completely excised and the bowel was
 
closed in the Heinecke-Mikulicz manner to prevent stenosis. The child did extremely
 
well postoperatively ; taking food by mouth without vomiting and rapidly gained in weight.
 
When last seen, March 29, 1939, two years later, she had no gastro-intestinal symptoms
 
and was now up to normal weight for her age.
 
 
 
 
 
 
 
 
 
Fic. 6.—Appearances in Case 3 viewed from below and the right. The cecum is fixed to the under
 
surface of the liver and below it the highly constricted and obstructed duodenum may be seen.
 
 
 
Case 3.—A. C., female, age 3 weeks, was brought to the University of California
 
Hospital, August 6, 1934, because of a large, protruding umbilical mass and persistent
 
projectile vomiting for the two weeks previous to entry. The child was born at term and
 
delivered normally. The umbilical mass was observed at birth. It slowly increased in
 
size and became gangrenous. Foods were poorly taken, the child exhibiting projectile
 
vomiting at the end of the first week, which continued until the time of hospital entry.
 
At entry, the child was markedly emaciated and dehydrated. The umbilical mass was
 
large, dark brown, cylindric in shape, protruding some three inches beyond the abdominal
 
wall. It was covered by a thick, hard seal, from beneath which a foul-smelling, brownish,
 
serous fluid exuded. The mass was kept under considerable tension by intra-abdominal
 
pressure. There was a ring of unhealthy granulation tissue at the junction of the mass
 
with the abdominal wall. Methylene blue given orally did not appear in the mass or its
 
 
 
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xoume lt ANOMALIES OF DUODENUM
 
 
 
discharge. After routine preoperative care, the protruding gangrenous tissue was removed; the area left gradually granulating in, and the child was discharged, September
 
16, 1934. Pathologic examination of the material showed the presence of liver tissue.
 
 
 
S'ubsequent Course—During the next three months, while at home, the child did
 
poorly, vomiting at frequent intervals. On December 15, 1934 she was returned to hospital. The umbilical wound was well healed although marked divarication of the rectus
 
abdominus muscle was noted. Vomiting continued and on January 9, 1934 the child contracted a bronchopneumonia from which she expired, January 11, 1935.
 
 
 
 
 
 
Fic. 7.—Case 3: Semidiagrammatic illustration showing the pgsition and convolution of the abnormal duodenum.
 
 
 
Autopsy (Fig. 6).—The liver was extremely large and hard, and extended down to
 
the upper limits of the right iliac fossa. There was a scarred area on the anterior surface
 
of the liver in the region of the umbilicus, obviously the area of herniation. There was
 
an anomalous right lobe of the liver. A single umbilical artery extended from the hypogastric vessels to the liver where it entered an abnormal porta hepatis. The artery was
 
elevated from the posterior surface of the anterior abdominal wall, being enclosed in a
 
mesentery. It formed, with the posterior abdominal wall, a boundary which divided
 
the peritoneal cavity into two parts, a small right and a large left portion. The alimentary tract exhibited incomplete fixation. The stomach was of normal size and occupied
 
its approximate normal position. There was a marked duodenal anomaly present. The
 
first portion extended horizontally to the right and was then abruptly reflected upon
 
itself, in the form of a U, to the region behind the pyloric antrum. It then ascended
 
obliquely upward and to the right, making an abrupt flexure behind the liver to extend
 
in a sharp curve, downwards and to the right, making still another flexure behind
 
the first portion of the duodenum, and passed horizontally to the left where it terminated
 
in the duodenojejunal flexure (Fig. 7). It was interesting to note that in this case the
 
 
 
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SAUNDERS AND LINDNER Annals of Surgery
 
 
 
September, 1940
 
 
 
duodenum was so markedly convoluted as to mechanically offer obstruction despite the
 
complete lack of actual peritoneal fixation.
 
 
 
Congenital obstruction of the duodenum received much attention in the
 
latter half of the last and at the beginning of the present century. This was
 
to be expected as the outcome of the reconstructive methods introduced into
 
embryology by His, in 1868. Comprehensive articles were published on the
 
continent by Theremin‘! (1877), Kuliga®? (1903) and Kreuter?7:?8 (1905,
 
1909) ; in America by Cordes® (1901) ; and in England by Clogg*® (1904) and
 
Spriggs®® (1912). The foremost recent discussions are those of Davis and
 
Poynter® (1922) and of Ladd®° (1933).
 
 
 
It is difficult to determine with any accuracy the number of cases reported
 
as the literature is in some confusion. Spriggs,$® however, stated, in 1912,
 
that congenital duodenal obstruction “is not so very much rarer than imperforate anus as one might expect, the one affection being so obvious cannot be
 
missed, the other most certainly is not so constantly in the mind of the practitioner and not so obvious, hence it often is missed.” That the duodenum is
 
a common level of congenital obstruction is indicated by Davis and Poynter.?
 
These authors studied 392 cases of congenital intestinal obstruction, of which
 
134 occurred in the duodenum.
 
 
 
Attempts have been made to estimate the incidence of congenital obstruction in the general population at infancy. Again the figures are too unreliable to be of much service except to indicate the comparative rarity. Such
 
figures are given by Ernst!l (1916), two cases of intestinal atresia in 41,000
 
children in the Royal Lying-In Hospital, Copenhagen; and Theremin,“ nine
 
cases in 150,000 at Petrograd, and two in 111,451 born over an 11-year period
 
at Vienna.
 
 
 
Pathology.—Pathologically congenital duodenal obstruction has been found
 
to result from the effects of either intrinsic or extrinsic factors.
 
 
 
Extrinsic obstruction of the duodenal lumen has been described as the
 
result of either developmental error or prenatal pathologic processes. Among
 
developmental errors may be listed kinking of the bowel from lack of fixation
 
or abnormal fixation, massive volvulus, persistence of a hepatoduodenocolic
 
ligament, annular pancreas and vascular anomalies with aberrant vessels.
 
Among prenatal pathologic processes which have been encountered may be
 
mentioned abnormal adhesive bands, mesenteric cysts and neoplasms of related
 
organs such as the liver or pancreas.
 
 
 
Intrinsic obstruction is the outcome of atresia, amounting in some instances
 
to complete suppression of a segment of the intestine, stenosis, or valve formation. It has been stated (Cordes$ and Clogg”) that complete atresia is more
 
frequent than stenosis in all situations, and that both atresia and stenosis are
 
much more frequent than valve formation. These forms are sometimes associated with abnormalities of the biliary tract. The bile duct may have an
 
abnormal site of implantation, draining on occasion into the stomach or entering the duodenum at an unusually low level. In some instances doubling of
 
the bile duct has been reported, each duct emptying at a different level. In
 
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Yolume 112 ANOMALIES OF DUODENUM
 
 
 
Number 3
 
 
 
trinsic obstruction occurs either above, at, or below the ampulla of Vater. It
 
is impossible to give any very satisfactory opinion of its frequency at any
 
level. It would seem, however, that the great majority of obstructions occur
 
in the second portion of the duodenum immediately above or immediately
 
below the duodenal papilla. Davis and Poynter® cited 59 cases above and 75
 
below ; Cordes,$ 20 above and 13 below. The exact location is so frequently
 
not reported that statistical conclusions are impossible. A small percentage of
 
cases of duodenal atresia or stenosis are associated with stenosis or atresia of
 
one or more segments of the alimentary tract.
 
 
 
In extrinsic obstruction the etiologic factor is so readily demonstrable,
 
either at the operating or postmortem table, as to require no further comment.
 
 
 
For intrinsic obstructions, however, a wide variety of theories, many now
 
of only historic interest, have been propounded. Such views include fetal
 
intussusception, enteritis, localized spasm, Meckel’s discarded segmentation
 
theory, segmental atrophy, local vascular thrombosis or embolism, hypertrophy
 
of the valvulae conniventes, etc.
 
 
 
À theory widely held is based on the dictum of Bland-Sutton%® that
 
abnormalities tend to occur at the sites of embryologic events. As the biliary
 
and pancreatic systems arise from the second part of the duodenum, this view
 
has been accepted as the determining factor for the presence of the obstruction
 
at this level or in its immediate neighborhood. This theory has the attraction
 
of simplicity and is without doubt, for some regions of the body, such as the
 
branchial region, of the greatest significance. In this instance, however, we
 
find the theory highly questionable if not wholly unacceptable, as the greater
 
percentage of intrinsic obstructions of the duodenum occur relatively distant
 
from the entrance of the biliary duct. In addition, exactly similar pathologic
 
processes, occasionally concomitant, occur at other levels of the small intestine
 
where development is unassociated with any special embryologic event in the
 
Bland-Sutton sense. The pathologic processes being the same, it is reasonable
 
to suppose that the factors responsible for atresia or stenosis in the duodenum
 
and at other levels of the bowel are similar.
 
 
 
À hypothesis which requires more serious consideration because of its
 
almost universal acceptance is the outcome of observations made by Tandler,#°
 
in 1900, on the formation of the duodenal lumen. This author pointed out
 
that during development the duodenal lumen becomes completely obliterated
 
(fifth to sixth week) by an extraordinary proliferation of its epithelial lining.
 
This proliferation appears to completely block the lumen although this has
 
been questioned by Frazer.t# It has been assumed (Kreuter?7) that persistence of this state is responsible for atresia, stenosis and membrane formation. However, similar objections arise as in Bland-Sutton’s theory, for
 
atresias occur at levels which are stranger to such a process of proliferation.
 
 
 
Wyss has propounded a view that the disturbance is the outcome of interference with vascularization and often associated with changes in the vascular
 
pattern. He describes two cases with absent pancreaticoduodenal arteries.
 
This view is not to be confused with extrinsic obstructions caused by
 
 
 
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SAUNDERS AND LINDNER Annals of Surgery
 
 
 
September, 1940
 
 
 
anomalous vessels. It is well recognized that changes in vascular pattern are
 
common in congenital anomalies but this does not mean that the changes are
 
the primary cause of the anomaly and have not arisen secondary to the deficiency in the area supplied by the vessel.
 
 
 
Embryology.—It goes without saying that a necessary preliminary to an
 
understanding of anomalies of position, and to a discussion of the possible
 
mechanism of atresia or stenosis, is a review of the chief events in the developmental history of the duodenum. The outline of events herein described is
 
based upon a series of dissections of the embryo at various stages, carried out
 
 
 
 
 
 
F1G. 8.—Dissection of an embryo of 13.5 Mm. C.R. length viewed from the right anterior aspect. The liver has been excised exposing the opening of the omental bursa and
 
the severed portal vein. Note the formation of the primary duodenal curves at this stage.
 
The enterocolic segment occupies the umbilical cord. Inset shows the simplicity of the
 
primary curves.
 
 
 
under the microscope. It is felt that such a method gives a far more accurate
 
picture of morphologic details than the more usual technic of reconstruction.
 
 
 
We have found it convenient to divide the development of the duodenum
 
into four stages : .
 
 
 
Stage I.—Rudimentary Stage: At this stage the duodenum is recognized
 
as the segment of the primitive gut lying between the dilatation of the stomach
 
and the commencement of the enterocolic loop. We regard the duodenal segment as constituting, at an early stage, a distinctive part of the alimentary
 
tract. Supported by a thickened portion of the common dorsal mesentery, the
 
biliary system has already made its appearance, and at 5 Mm. the dorsal
 
pancreatic rudiment has budded into the mesoduodenum.
 
 
 
Stage II.—Formation of Primary Curves (Fig. 8) : As development proceeds, the stomach undergoes rotation to assume its permanent position, and
 
as it does so, the future omental bursa is defined. Meanwhile the enterocolic
 
 
 
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Volume 112 ANOMALIES OF DUODENUM
 
 
 
Number 3
 
 
 
loop has elongated and proceeded to occupy its position at the root of the
 
umbilical cord. The duodenum has likewise participated in the changes. These
 
involve predominantly its first portion. This part shows a far greater degree
 
of elongation than the rest. At 13.5 Mm. it is large and well developed and is
 
almost twice the diameter of the succeeding portion of the intestine. It extends
 
transversely across the abdomen with a very slight upward inclination and is
 
highly arched over the vitelline or future portal vein. Passing dorsally, it
 
 
 
f
 
 
 
 
 
 
Fi1c. 9.—Dissection of a 43 Mm. C.R. length embryo viewed
 
from the right anterior aspect. The liver has been removed. The
 
elongation and increase in size of the duodenum is extending into
 
its second portion in the establishment of the secondary duodenal
 
curves. The umbilical vein is indicated in dotted outline. The
 
prearterial segment of the enterocolic loop is about to return into
 
the abdominal cavity.
 
 
 
terminates by making a sudden flexure opposite the wolffan body (mesonephros). This flexure is the future duodenojejunal flexure. The duodenum as
 
a whole is shaped rather like the letter U placed horizontally with its convexity ventrally and with the vitelline vein lying in its concavity. The second
 
portion of the duodenum is extremely short and inclines a fraction to the
 
right to enter the third and fourth portions, which are represented by little
 
more than a slight curve at the future duodenojejunal junction. Under the
 
 
 
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SAUNDERS AND LINDNER Annals of Surgers
 
 
 
September. 1940
 
 
 
influence of the increase in size of the body cavity, the determination of the
 
position of the stomach and the enlargement of the future portal vein, the
 
essentials of the permanent curvature of the first portion of the duodenum have
 
been established.
 
 
 
Stage III.—The Duodenal Loop, (Fig. 9) : The stage initiating the establishment of the duodenal loop and the attainment of its adult form is characterized by the very rapid development and elongation of the second portion
 
of the duodenum and slightly later of the two succeeding parts. At this time,
 
various observers (Tandler,# Johnson?) have noted in the lower two-thirds
 
 
 
 
 
 
Fic. 10.—Dissection of a 4 Mm. CR. length embryo. The
 
enlargement and elongation of the duodenum has extended to the
 
third and fourth portions. The duodenal loop still exceeds that
 
of the jejunum in diameter. The duodenojejunal flexure has
 
crossed to the left of the midline.
 
 
 
of the duodenum, overgrowth of the epithelial lining amounting to an actual
 
occlusion of the duodenal lumen. Vacuolization follows, 22-24 Mm., leading
 
to reestablishment of the lumen and formation of the vill. At 30 Mm, the
 
vacuoles having coalesced, the lumen is pervious throughout. Differential
 
growth has resulted in the elongation of the ligament of Treitz and the transference of the duodenojejunal flexure to the left of the midline (Fig. 10).
 
Hunter,!7 in this connection, stresses the importance of the part played by
 
fixation bands in the formation of the duodenal curve. Frazer,!$ criticizing
 
this view, believes that the curve is produced under the influence of the
 
 
 
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xolume 112 ANOMALIES OF DUODENUM
 
 
 
growth of the head of the pancreas. We, however, do not subscribe to
 
either of these views and regard the curvature of the duodenal loop as due
 
to differential growth factors and time relationship rather than to mechanical
 
effects.
 
 
 
Stage IV.—Fixation: The final stage concerns the ultimate placement of
 
duodenojejunal flexure and fixation of the gut. The mesoduodenum has
 
closely approximated the dorsal parietal peritoneum by the development of
 
the pancreas between its layers, and fusion ensues. The adhesion is no doubt
 
influenced by the returning gut. The transverse colon during the rotation
 
of the extra-abdominal intestine is carried over the duodenum and adheres
 
to it at the point of crossing.
 
 
 
The Genesis of Duodenal Anomalies—We have thought it advisable in
 
view of the impossibility in the present state of our knowledge of making
 
any definite statement as to ultimate cause, to relate the various errors in
 
terms of the various stages of duodenal development previously discussed.
 
In addition, certain general principles need emphasis. It should be recognized that such fundamental processes as growth and differentiation proceed
 
independently of one another and may show varying velocities of change.
 
There is ample evidence to show that the original control of differentiation
 
appears to be exerted in relation to definite morphogenetic fields and is dependent, not upon any definite localization, but upon the position of any part
 
relative to the whole structure or on the levels which the various parts occupy
 
along an axis of development. It is perhaps safe to assert, judging from
 
experimental evidence, that there are, for the alimentary tract, critical periods
 
which precede differentiation and which differ in time in different parts of
 
the gut. The ultimate effects produced in the way of errors are influenced
 
by the relationship of the time of action of the noxious agent to the stage of
 
differentiation attained.
 
 
 
Anomalies of the first stage are those associated with the development of
 
the biliary and pancreatic rudiments resulting in such conditions as dichotomy
 
of the bile duct and annular pancreas. In the former it is probable that the
 
same influence that establishes the biliary anomaly is responsible for the
 
associated atresia or stenosis of the duodenum. In the latter deformity any
 
delay in the appearance of the ventral pancreatic rudiment from the duodenum is envisaged as forcing this portion of the pancreas to follow the
 
further development of the duodenum rather than the dorsal rudiment. Both
 
of these anomalies are regarded as having their origin in the earliest stages of
 
development.
 
 
 
Anomalies of the second stage associated with the development of the
 
first part of the duodenum are almost unknown. \Ve have been unable to
 
find records of a single instance of atresia or stenosis affecting this portion.
 
Such freedom from error is not unexpected by virtue of the early establishment of this portion of the duodenum. Its development, closely associated
 
with that of the stomach, shows an initial preponderance over the rest of the
 
duodenum and in the formation of its essential curves (Fig. 8).
 
 
 
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SAUNDERS AND LINDNER gnnals of Surgers
 
 
 
September, 1940
 
 
 
The third stage is of the greatest importance from the point of view of
 
errors affecting the lumen and shape of the duodenum. As already pointed
 
out, the formation of the second and third parts of the duodenum occurs
 
comparatively late in development, and is characterized by a period of rapid
 
elongation eventuating in the establishment of the adult form and shape
 
(Fig. 9). The majority of duodenal anomalies involve this portion of the
 
gut. Any factor which interferes with this critical period of growth would
 
lead to complete or partial obliteration of the lumen.
 
 
 
It would seem to us that the mechanism producing atresia, stenosis or
 
valve formation is essentially the same, varying only in degree and in time
 
 
 
 
 
 
F1G. 11.—Illustrating the successive stages in the formation of a duodenal valve. Differential growth above and below the retarded area has produced the valve which therefore contains muscle between the two layers of
 
epithelium.
 
 
 
of genesis. In addition, such retardation, particularly if extensive, must
 
secondarily bring about distortion of the duodenal loop, as is so commonly
 
found. Atresia is regarded as the result of early retardation. The same
 
factor acting a little later would result in stenosis rather than atresia with
 
less disturbance of the duodenal loop (Case 1). If the disturbance affects
 
a very limited segment of the gut, differential growth above and below the
 
point of interference would result in the formation of a valve (Fig. 11).
 
Such a mechanism would account for the otherwise unexplained presence of
 
muscular tissues in such septa as in Case 2. The presence or absence of
 
septal perforation and its size may, apart from the possibility of secondary
 
perforation, be related to the time of retardation: during, before or after the
 
establishment of a lumen.
 
 
 
We regard the rapid elongation of the second and third portions as
 
being responsible for the opening out of the duodenal loop.  Anomalies
 
of shape or retention of the primitive curve as in the duodenum in “M”? may
 
result from premature elongation before the requisite amount of space is
 
 
 
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Volume 112 ANOMALIES OF DUODENUM
 
 
 
Number 3
 
 
 
available for its reception or from the influence of neighboring organs such
 
as an excessively large liver (Case 3) or premature return of the umbilical
 
loop.
 
 
 
Errors of the fourth stage are the result of failure of peritoneal fixation.
 
In themselves they are of little importance excepting insofar as they may
 
allow of kinking of the bowel or be associated with abnormalities of rotation
 
affecting the remaining intestine. Errors of fixation are commonly associated
 
with obstruction of the lumen. In Case 1 a free duodenum was associated
 
with stenosis, nonrotation of the gut, together with persistence of a hepatoduodenocolic ligament.
 
 
 
Diagnosis.—The past decade has added little to our store of knowledge
 
with regard to the early diagnosis of congenital duodenal obstruction, whether
 
due to stenosis or atresia. Ladd,%° in a recent paper, again stresses the fact
 
that duodenal obstruction will give the signs common to high intestinal obstruction. He emphasizes the importance of upper abdominal distention,
 
visible reverse gastric peristalsis, the presence of dehydration and shock and
 
the laboratory findings of ketosis. The afore-mentioned signs are common to
 
all high bowel obstructions. It should be emphasized at this point that children with true hypertrophic pyloric stenosis do not vomit until the seventh
 
or ninth day at the earliest, whereas those infants with duodenal obstructions
 
vomit as early as the first 24 hours. Farber,l? some five years ago, emphasized the fact that the absence of cornified epithelial cells in the meconium
 
was proof that an atresia existed somewhere along the length of the gastrointestinal tract. He has developed a specific stain for these cells and both
 
he and Ladd° have found that this test is of value in diagnosis. Most writers
 
agree that hematemesis and the presence of coffee-ground vomitus in the
 
absence of a blood dyscrasia, is a pathognomonic sign of duodenal atresia,
 
most authors giving figures as high as from 65 to 85 per cent of all cases
 
having this finding. Absolute constipation is the rule, but some cases pass
 
what appears to be a true stool. Icterus of moderate degree is present in a
 
good number of cases. One should not be misled as to the site of the atresia by
 
the presence of bile pigments in the stool, as authors have stressed the fact
 
that the presence of accessory biliary ducts emptying below the obstruction
 
are not at all uncommon. The roentgenogram is, of course, of considerable
 
value, the presence of a large gas bubble in the stomach and its site of termination often helping to confirm the diagnosis. Most surgeons attempt to
 
corroborate their diagnosis by the giving of a small amount of barium by
 
mouth. We feel that a warning should be posted with regard to this procedure if a later short circuiting operation is to be carried out. This warning
 
is necessary because it has been found that the barium will often plug the
 
anastomotic stoma, with -subsequent obstruction. If barium is used it should
 
be followed by a thorough washing out of the stomach and upper duodenum
 
to prevent this catastrophe.
 
 
 
Treatment.—The treatment of congenital duodenal obstruction is essentially surgical and should be instituted at the earliest possible time compatible
 
 
 
335
 
SAUNDERS AND LINDNER ganalsof Surgers
 
 
 
with the physical condition of the infant. It is unnecessary to discuss the
 
actual surgical procedures to be employed, except to point out that because
 
of the smallness of the bowel in infancy, they are often attended by considerable technical difficulty. To overcome these difficulties two points with
 
regard.to surgical treatment are of special interest and deserve emphasis.
 
The extreme contraction of the distal segment for anastomosis is one of the
 
prime causes of trouble in performing the operative procedure and is responsible for the large percentage of postoperative complications. Clogg,"
 
as early as 1904, and Wangensteen,* in 1931, have emphasized the importance of ballooning up the distal segment by means of hydrostatic pressure.
 
This maneuver has the double advantage of increasing the size of the distal
 
segment, thereby making it more amenable for anastomosis, and also testing
 
the patency of the bowel lumen distal to the area of obstruction, often indicating other constrictions at lower levels which necessarily would alter the
 
plan of procedure. Multiple constrictions are relatively common. The final
 
point of interest, from a technical standpoint, regards the use of extremely
 
fine silk or linen as a suture material, and the performance of the anastomosis
 
with a single anterior and posterior layer to obviate narrowing of the lumen.
 
It has been found that this technic rewards its user with a higher percentage
 
of successful results. We feel that the early diagnosis of these conditions
 
combined with prompt surgical procedure will bear fruit in giving a higher
 
percentage of cures. A thorough knowledge of the developmental anatomy
 
of the duodenum is essential to the diagnostic and therapeutic problems involved in the treatment of these conditions. It is perhaps the rarity of congenital obstructions which is responsible, more than anything else, for the
 
poor prognosis in the majority of cases reported and for the relative therapeutic inertia.
 
 
 
SUMMARY
 
 
 
(1) Three cases of congenital duodenal malformation are reported.
 
 
 
(2) The literature and etiologic hypotheses are briefly discussed.
 
 
 
(3) The development of the duodenum, as observed from the microscopic
 
dissections, is given as a basis for the classification and opinions on the genesis
 
of these anomalies.
 
 
 
(4) A brief outline of diagnosis and treatment is given.
 
 
 
We wish to acknowledge with thanks the courtesy of the Division of Surgery, Dr.
 
Jacob Smith for Case 2; for Case 3, Dr. Francis S. Smyth of the Division of Pediatrics;
 
and Dr. Parry Douglas for referring Case 1.
 
 
 
BIBLIOGRAPHY
 
 
 
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2 Anderson, W. W.: Congenital Atresia of the Colon in a Newborn. Jour. Med. Assoc.
 
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336
 
Volume 113 ANOMALIES OF DUODENUM
 
 
 
Number à
 
 
 
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$ Cautley, E.: Duodenal Stenosis. Brit. Jour. Children's Dis., 16, 65-73, 1919.
 
 
 
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SAUNDERS AND LINDNER gnnals o Burger
 
 
 
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Latest revision as of 12:31, 10 December 2019

Saunders JB. and Lindner HH. Congenital anomalies of the duodenum. (1940) Annals of Surgery 112{3}: 321-338.