Paper - Congenital anomalies of the duodenum (1940)

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

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This historic 1940 paper by Saunders and Lindner describes duodenum anomalies.

Modern Notes: duodenum | gastrointestinal abnormalities

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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 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 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.

Fig. 1.— Case 1: Radiograph three hours after barium meal showing almost complete retention. À minute quantity of barium has passed into the proximal jejunum.

Fig. 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.

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 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 considerable 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.

F1G. 3. — Case 1: Following division of peritoneal adhesions.

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 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.

Subsequent 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 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.

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.

Subsequent 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 duodenum. (Fig. 5.) Preoperative Diagnosis: Congenital stenosis of the second or third part of the duodenum.

Fig. 5. — Case 2: Radiograph three hours after barium meal showing dilated stomach and megaduodenum with obstruction.

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 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.

Fig. 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 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.

Subsequent 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.

Fig. 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 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. Intrinsic 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 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 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 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 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.

Fig. 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.

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 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 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.

Fig. 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.

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).

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 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.

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.

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 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 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 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.


  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.


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