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Duodenal diaphragm associated with superior mesenteric artery syndrome
Duodenal Diaphragm Associated With Superior Mesenteric Artery Syndrome Nicholas G. Economides, MD, Memphis, Tennessee Thomas M. Fortner, MD, Memphis, Tennessee W. David Dunavant, MD, FACS, Memphis, Tennessee
The most common cause of gastric outlet obstruction in the adult is peptic ulceration, usually duodenal. The second most common cause is carcinoma of the gastric antrum. Other obstructive lesions are numerous, but rare; they include pyloric hypertrophy, benign tumors, adjacent carcinoma, ectopic pancreatic tissue, prepyloric mucosal diaphragm and congenital lesions. One of the least common is a persistent congenital diaphragm in the first or second portion of the duodenum. We were able to find only 120 cases reported in the English literature, less than 40 of which were in adults. According to Richardson and Martin [I], about 50 percent of the cases are associated with other anomalies like mongolism, malrotation, hiatal hernia, congenital heart disease, situs inversus, Meckel’s diverticulum, double obstruction and annular pancreas. To our knowledge, no case of duodenal diaphragm associated with superior mesenteric artery syndrome in an adult has been reported before.
Case Report A 24 year old black woman was admitted to Baptist Memorial Hospital Medicine Service on August 28,197s with a chief complaint of postprandial vomiting for 3 weeks. The vomitus consisted of undigested food and liquids without any blood. She reported a 25 pound weight loss during the saine period. Associated with her symptoms had been intermittent, cramping abdominal pain with radiation through to the back. Relief was obtained after vomiting and by lying on her left side with her legs drawn up towards her chest. However, a few days before admission, the abdominal pain had become persistent and the vomiting projectile. She had a history of rheumatoid arthritis which had been treated with salicylateswith codeine since 1974. Physical examination revealed a lean patient with a palpable mass in the left upper quadrant of the abdomen. Bowel sounds were of normal character and frequency. Admission laboratory data revealed a sodium of 124, potassium 2.3, chloride 57, and bicarbonate 48 mEq/liter. The amylase creatinine clearance ratio was slightly increased at 6.1 percent. Findings on barium enema and chest roentgenography were normal. Two saline load tests indicated obstruction. A rheumatoid arthritis test gave normal results. Two lupus erythematosus cell preparations gave From the Department of Surgery, Baptist Memorial Hospital, Memphis, Tennessee. Requests for reprints should be addressed to Nicholas G. Economides. MO, Departmant of Svgery. Baptist Memorial Hospital, Memphii. Tennessee 36146.
274
positive results, An initial gastrointestinal series revealed an obstruction of the duodenum between the third and fourth portions, compatibie with the superior mesenteric artery syndrome (Figure 1). Hypotonic duodenography demonstrated, in addition to obstruction of the third portion of the duodenum, a questionable intraduodenal lesion between the first and second portions (Figure 2). Intraoperative findings: Abdominal exploration was performed on September 11,1978 through a right paramedian incision with a preoperative diagnosis of obstruction in the second and third portions of the duodenum. Upon entering the peritoneal cavity, the first and third portions of the duodenum were found markedly distended, with the second portion normal in diameter (Figure 3). The distal duodenum was digitally examined after making an anterior duodenotomy over the third portion. It was found to be,obstructed by extrinsic pressure from the superior mesenteric artery. The distance between the take-off of the superior mesenteric artery from the aorta and ita crossing with the mid-circumference of the duodenum was 5 mm. The angle of the take-off was 20°. Further digital examination of the duodenum revealed another obstruction proximal to the duodenotomy. A second duodenotomy was performed over the first portion of the duodenum at the level of this obstruction. A complete duodenal diaphragm was found which had an obliterated aperture 1 mm in diameter, secondary to an inflammatory reaction as evidenced by scarring. The ampulla was 1.5 cm distal to the orifice of the diaphragm. Circumferential total excision of the diaphragm was undertaken (Figure 4). Next, the mucosal defect was closed transversely with a running absorbable suture. The proximal duodenotomy was closed ih two layers transversely. Duodenojejunostomy was then performed in two layers by utilizing the initial duodenotomy in order to bypass the inferior mesenteric artery obstruction of the third portion of the duodenum. The postoperative course was uneventful. The patient was discharged on the seventh postoperative day. She was seen on two occasions at 3 and 6 month intervals. She has remained asymptomatic and has gained 25 pounds.
Comments Duodenal diaphragms or congenital webs are rare; they occur in 1 of every 9,000 to 40,000 births [2]. This anomaly occurs early during the embryo’s life. Tandler [3], in his classic study of duodenal development, noted that when the embryo measured 8.5 mm, the cavity between the outlets of the pancreatic and common bile ducts were completely obliterated. In the 15 mm embryo, the pancreatic and bile ducts ended in closed cavities of the duodenum; the duoTheAmerican
Journal of Surgery
Duodenal Diaphragm
denal lumen was also obliterated by mesodermal investment. Between the 15 and 20 mm stages, vacuoles began to coalesce, reestablishing the lumen. Boyden et al [4] referred to Tandler’s work as the first step of duodenal development. Studying their own models, they discovered that during the same period, the pancreatic and bile ducts acquired separate orifices, which they considered the second step of duodenal development. Step three was reported by Streeter [5], who observed that the proliferating activity of the duodenal epithelium results in two fusiform enlargements of the embryonic duodenum,
one proximal to the entrance of the ducts and one distal. The fourth step was described by Boyden, who pointed out that a wave of mesenchymal overgrowth and differentiation passes over the entire duodenal loop. In the 23 to 38 mm stages, the process of recanalization of the duodenum has been completed. Interruption of these normal events at any point might result in a variety of congenital duodenal defects: diaphragms, stenosis, atresia or any combination of these. What triggers the abnormality is not known, but the possibility of an in utero vascular accident has been implicated. Louw and Bernard [6] were able to produce ileal atresia by ligating the superior mesenteric artery in the near-term puppy. The diaphragm is composed of duodenal mucosa on both sides. About 80 percent of the reported cases
Ftgure Upper 1. gastrolntestinai series demonstratingobstruction of b&urn at the duodenum’s third portion.
Ffgure 3. Diagram of the duodenum showfng
Ffgure 2. Hypotonk duodenogram demonstrating the same obsbuctlon of the thkd portion of the duodenum in additlon to an lntraduodenal lesion between the first and second portlons. Volume 141, February 1991
Flgure 4. Diagram of clrcumferentlal diaphragm.
the two lesions
excision of
the duodenal
275
Economides et al
have been within an inch from the ampulla. They can either be complete (with one or multiple apertures), incomplete or elongated (diverticulum). The size of the aperture varies from 1 mm to 3 cm. The onset of symptoms may be delayed for years, if the diaphragm is incomplete. Krieg [7] reported that the onset of symptoms in 30 percent of the cases occurred after age 24; symptoms consist of epigastric fullness, postprandial nausea, vomiting and weight loss [2]. The size of the aperture does not appear to affect the early or late development of symptoms as long as it remains patent. If it fuses for some reason (such as medications or inflammation), the onset of symptoms is inevitable. This may have been the precipitating factor in the onset of our patient’s symptoms, since microscopic examination of the specimen showed nonspecific inflammatory changes not compatible with rheumatoid disease. Complications such as complete duodenal obstruction, gastrointestinal bleeding, pancreatitis, jaundice and intraluminal perforation of the diaphragm have been reported [8]. Diagnosis can be made preoperatively 95 percent of the time with hypotonic duodenography and approximately 75 percent of the time with upper gastrointestinal series. Endoscopy under skillful hands also can give a high diagnostic yield. Cineradiography and intravenous cholangiography, reported in isolated instances, have been successful. In our patient, hypotonic duodenography demonstrated an intraduodenal lesion. The surgical treatment is anterior duodenotomy; the diaphragm is excised circumferentially, assuring protection of the ampulla. The mucosal defect of the posterior duodenal wall is then repaired with a continuous suture and the duodenotomy is closed transversely. The biggest problem that the surgeon faces is operation for duodenal obstruction without a preoperative diagnosis. Under these circumstanc&, Ferguson and Rothfield [9] pointed out indications for duodenotomy: (1) extreme dilation of the stomach and duodenum, (2) abrupt transition from hypertrophied duodenum to a wall of normal thickness, (3) inability to feel the lesion from the outside, (4) distortion of the anatomic rotation of the duodenum (malrotation), (5) a history of jaundice with a normal intravenous cholangiogram, and (6) mongolism. The first operative treatment for this disease was outlined by Terry and Kilgore in 1916 [IO]. They performed gastroenterostomy in a 24 year old man, but he died 5 days postoperatively. Krieg [7] performed the first successful operation on a duodenal diaphragm using a posterior gastrojejunostomy. Morton in 1922 was the first to suggest direct surgical excision for treatment [II]. In cases such as ours, with double duodenal obstruction with a superimposed superior mesenteric artery syndrome, duodenojejunostomy should be added, either by using the original duodenotomy or by anastomosing the distal duo-
276
denum to the jejunum. Concomitant abnormalities should be looked for and corrected if feasible. The diagnosis of superior mesenteric artery syndrome and its correlation to clinical situations is extremely difficult. According to the reports of Mansberger et al [12], Hearne [13] and Wayne et al [14], patients with a narrow aorto- or vertebromesenteric artery space at the site of duodenal crossing could be classified as having vascular compression of the duodenum. Most of these measurements were done arteriographically. We measured the aortosuperior mesenteric artery angle and its length to the duodenum intraoperatively, and the measurements were compatible with the superior mesenteric artery syndrome. Our patient had lost a lot of weight, which itself has been implicated as a cause of the syndrome. Summary An unusual case of duodenal diaphragm in a 24 year old patient who presented with symptoms of the superior mesenteric artery syndrome is presented. The patient had rheumatoid arthritis and had been treated with salicylates, which were thought to have produced inflammation and occlusion of the aperture to the point of complete obstruction. Complete excision of the diaphragm through anterior duoden&my and distal du&denojejunostomy provided an excellent result. References 1. Richardson WR, Martin LW. Pitfalls in the surgical management of the incomplete duodenaphragm. J Pediatr Surg 1969; 21:303. 2. Cooperman AM, Adachi M, Rankin GB, Sivak M. Congenital duodenal diaphragm in adults: a delayed course of intestinal obstruction. Ann Surg 1975; 182:739. 3. Tandler J. Zur Entwicklungyeschidhte des manchichen duodenums in frechen embryonal stodium. Morphol Gahub 1900;29:187. 4. Boyden EA, Cope JG, Hill AH. Anatomy and embryology of congenital intrinsic obstruction of the duodenum. Am J Surg 1967;114:190. 5. Streeter GL. Developmental horizons in human embryos. Description of age groups XV, SVI, SVII, SVIII. Publication 575. Contributions to embryology, no. 211.32, 133. Washington: Carnegie Institute, 1948. 6. Louw JH, Bernard CN. Congenital intestinalatresia, observations on its origin. Lancet 1955;2:1005. 7. Krieg EG. Duodenal diaphragm. Ann Surg 1937;106:33. 8. Economides NG, McBurney RP, Hamilton FH 111.lntraluminal duodenal diverticulum in the adult. Ann Surg 1977;185: 147. 9. Ferguson AF. Rothfield NJ. Intestinal obstruction by duodenal diaphragm. Great Ormond S! J 1954;8: 105. 10. Nelson WI. Congenital diaphragm of the duodenum. Minn Med 1947;30:745. 11. Knutrud 0, Eek S. Combined intrinsic duodenal obstruction and malrotation. Acta Chir Stand 1960; 119:506. 12. Mansberger AR Jr, l-learn JB, Byers RM, Fleisig N, Buxton RW. Vascular compression of the duodenum, emphasis on accurate diagnosis. Am J Surg 1968;115:84. 13. Hearne JB. Duodenal ileus with special reference to superior mesenteric artefy compression. Radiology 1966;86:305. 14. Wayne E, Miller RE, Eiseman B. Duodenal obstruction by the superior mesenteric artery in bedriien combat casualties. Am J Surg 1971;174:339.
The American Journal of Surgery
The most common cause of gastric outlet obstruction in the adult is peptic ulceration, usually duodenal. The second most common cause is carcinoma of the gastric antrum. Other obstructive lesions are numerous, but rare; they include pyloric hypertrophy, benign tumors, adjacent carcinoma, ectopic pancreatic tissue, prepyloric mucosal diaphragm and congenital lesions. One of the least common is a persistent congenital diaphragm in the first or second portion of the duodenum. We were able to find only 120 cases reported in the English literature, less than 40 of which were in adults. According to Richardson and Martin [I], about 50 percent of the cases are associated with other anomalies like mongolism, malrotation, hiatal hernia, congenital heart disease, situs inversus, Meckel’s diverticulum, double obstruction and annular pancreas. To our knowledge, no case of duodenal diaphragm associated with superior mesenteric artery syndrome in an adult has been reported before.
Case Report A 24 year old black woman was admitted to Baptist Memorial Hospital Medicine Service on August 28,197s with a chief complaint of postprandial vomiting for 3 weeks. The vomitus consisted of undigested food and liquids without any blood. She reported a 25 pound weight loss during the saine period. Associated with her symptoms had been intermittent, cramping abdominal pain with radiation through to the back. Relief was obtained after vomiting and by lying on her left side with her legs drawn up towards her chest. However, a few days before admission, the abdominal pain had become persistent and the vomiting projectile. She had a history of rheumatoid arthritis which had been treated with salicylateswith codeine since 1974. Physical examination revealed a lean patient with a palpable mass in the left upper quadrant of the abdomen. Bowel sounds were of normal character and frequency. Admission laboratory data revealed a sodium of 124, potassium 2.3, chloride 57, and bicarbonate 48 mEq/liter. The amylase creatinine clearance ratio was slightly increased at 6.1 percent. Findings on barium enema and chest roentgenography were normal. Two saline load tests indicated obstruction. A rheumatoid arthritis test gave normal results. Two lupus erythematosus cell preparations gave From the Department of Surgery, Baptist Memorial Hospital, Memphis, Tennessee. Requests for reprints should be addressed to Nicholas G. Economides. MO, Departmant of Svgery. Baptist Memorial Hospital, Memphii. Tennessee 36146.
274
positive results, An initial gastrointestinal series revealed an obstruction of the duodenum between the third and fourth portions, compatibie with the superior mesenteric artery syndrome (Figure 1). Hypotonic duodenography demonstrated, in addition to obstruction of the third portion of the duodenum, a questionable intraduodenal lesion between the first and second portions (Figure 2). Intraoperative findings: Abdominal exploration was performed on September 11,1978 through a right paramedian incision with a preoperative diagnosis of obstruction in the second and third portions of the duodenum. Upon entering the peritoneal cavity, the first and third portions of the duodenum were found markedly distended, with the second portion normal in diameter (Figure 3). The distal duodenum was digitally examined after making an anterior duodenotomy over the third portion. It was found to be,obstructed by extrinsic pressure from the superior mesenteric artery. The distance between the take-off of the superior mesenteric artery from the aorta and ita crossing with the mid-circumference of the duodenum was 5 mm. The angle of the take-off was 20°. Further digital examination of the duodenum revealed another obstruction proximal to the duodenotomy. A second duodenotomy was performed over the first portion of the duodenum at the level of this obstruction. A complete duodenal diaphragm was found which had an obliterated aperture 1 mm in diameter, secondary to an inflammatory reaction as evidenced by scarring. The ampulla was 1.5 cm distal to the orifice of the diaphragm. Circumferential total excision of the diaphragm was undertaken (Figure 4). Next, the mucosal defect was closed transversely with a running absorbable suture. The proximal duodenotomy was closed ih two layers transversely. Duodenojejunostomy was then performed in two layers by utilizing the initial duodenotomy in order to bypass the inferior mesenteric artery obstruction of the third portion of the duodenum. The postoperative course was uneventful. The patient was discharged on the seventh postoperative day. She was seen on two occasions at 3 and 6 month intervals. She has remained asymptomatic and has gained 25 pounds.
Comments Duodenal diaphragms or congenital webs are rare; they occur in 1 of every 9,000 to 40,000 births [2]. This anomaly occurs early during the embryo’s life. Tandler [3], in his classic study of duodenal development, noted that when the embryo measured 8.5 mm, the cavity between the outlets of the pancreatic and common bile ducts were completely obliterated. In the 15 mm embryo, the pancreatic and bile ducts ended in closed cavities of the duodenum; the duoTheAmerican
Journal of Surgery
Duodenal Diaphragm
denal lumen was also obliterated by mesodermal investment. Between the 15 and 20 mm stages, vacuoles began to coalesce, reestablishing the lumen. Boyden et al [4] referred to Tandler’s work as the first step of duodenal development. Studying their own models, they discovered that during the same period, the pancreatic and bile ducts acquired separate orifices, which they considered the second step of duodenal development. Step three was reported by Streeter [5], who observed that the proliferating activity of the duodenal epithelium results in two fusiform enlargements of the embryonic duodenum,
one proximal to the entrance of the ducts and one distal. The fourth step was described by Boyden, who pointed out that a wave of mesenchymal overgrowth and differentiation passes over the entire duodenal loop. In the 23 to 38 mm stages, the process of recanalization of the duodenum has been completed. Interruption of these normal events at any point might result in a variety of congenital duodenal defects: diaphragms, stenosis, atresia or any combination of these. What triggers the abnormality is not known, but the possibility of an in utero vascular accident has been implicated. Louw and Bernard [6] were able to produce ileal atresia by ligating the superior mesenteric artery in the near-term puppy. The diaphragm is composed of duodenal mucosa on both sides. About 80 percent of the reported cases
Ftgure Upper 1. gastrolntestinai series demonstratingobstruction of b&urn at the duodenum’s third portion.
Ffgure 3. Diagram of the duodenum showfng
Ffgure 2. Hypotonk duodenogram demonstrating the same obsbuctlon of the thkd portion of the duodenum in additlon to an lntraduodenal lesion between the first and second portlons. Volume 141, February 1991
Flgure 4. Diagram of clrcumferentlal diaphragm.
the two lesions
excision of
the duodenal
275
Economides et al
have been within an inch from the ampulla. They can either be complete (with one or multiple apertures), incomplete or elongated (diverticulum). The size of the aperture varies from 1 mm to 3 cm. The onset of symptoms may be delayed for years, if the diaphragm is incomplete. Krieg [7] reported that the onset of symptoms in 30 percent of the cases occurred after age 24; symptoms consist of epigastric fullness, postprandial nausea, vomiting and weight loss [2]. The size of the aperture does not appear to affect the early or late development of symptoms as long as it remains patent. If it fuses for some reason (such as medications or inflammation), the onset of symptoms is inevitable. This may have been the precipitating factor in the onset of our patient’s symptoms, since microscopic examination of the specimen showed nonspecific inflammatory changes not compatible with rheumatoid disease. Complications such as complete duodenal obstruction, gastrointestinal bleeding, pancreatitis, jaundice and intraluminal perforation of the diaphragm have been reported [8]. Diagnosis can be made preoperatively 95 percent of the time with hypotonic duodenography and approximately 75 percent of the time with upper gastrointestinal series. Endoscopy under skillful hands also can give a high diagnostic yield. Cineradiography and intravenous cholangiography, reported in isolated instances, have been successful. In our patient, hypotonic duodenography demonstrated an intraduodenal lesion. The surgical treatment is anterior duodenotomy; the diaphragm is excised circumferentially, assuring protection of the ampulla. The mucosal defect of the posterior duodenal wall is then repaired with a continuous suture and the duodenotomy is closed transversely. The biggest problem that the surgeon faces is operation for duodenal obstruction without a preoperative diagnosis. Under these circumstanc&, Ferguson and Rothfield [9] pointed out indications for duodenotomy: (1) extreme dilation of the stomach and duodenum, (2) abrupt transition from hypertrophied duodenum to a wall of normal thickness, (3) inability to feel the lesion from the outside, (4) distortion of the anatomic rotation of the duodenum (malrotation), (5) a history of jaundice with a normal intravenous cholangiogram, and (6) mongolism. The first operative treatment for this disease was outlined by Terry and Kilgore in 1916 [IO]. They performed gastroenterostomy in a 24 year old man, but he died 5 days postoperatively. Krieg [7] performed the first successful operation on a duodenal diaphragm using a posterior gastrojejunostomy. Morton in 1922 was the first to suggest direct surgical excision for treatment [II]. In cases such as ours, with double duodenal obstruction with a superimposed superior mesenteric artery syndrome, duodenojejunostomy should be added, either by using the original duodenotomy or by anastomosing the distal duo-
276
denum to the jejunum. Concomitant abnormalities should be looked for and corrected if feasible. The diagnosis of superior mesenteric artery syndrome and its correlation to clinical situations is extremely difficult. According to the reports of Mansberger et al [12], Hearne [13] and Wayne et al [14], patients with a narrow aorto- or vertebromesenteric artery space at the site of duodenal crossing could be classified as having vascular compression of the duodenum. Most of these measurements were done arteriographically. We measured the aortosuperior mesenteric artery angle and its length to the duodenum intraoperatively, and the measurements were compatible with the superior mesenteric artery syndrome. Our patient had lost a lot of weight, which itself has been implicated as a cause of the syndrome. Summary An unusual case of duodenal diaphragm in a 24 year old patient who presented with symptoms of the superior mesenteric artery syndrome is presented. The patient had rheumatoid arthritis and had been treated with salicylates, which were thought to have produced inflammation and occlusion of the aperture to the point of complete obstruction. Complete excision of the diaphragm through anterior duoden&my and distal du&denojejunostomy provided an excellent result. References 1. Richardson WR, Martin LW. Pitfalls in the surgical management of the incomplete duodenaphragm. J Pediatr Surg 1969; 21:303. 2. Cooperman AM, Adachi M, Rankin GB, Sivak M. Congenital duodenal diaphragm in adults: a delayed course of intestinal obstruction. Ann Surg 1975; 182:739. 3. Tandler J. Zur Entwicklungyeschidhte des manchichen duodenums in frechen embryonal stodium. Morphol Gahub 1900;29:187. 4. Boyden EA, Cope JG, Hill AH. Anatomy and embryology of congenital intrinsic obstruction of the duodenum. Am J Surg 1967;114:190. 5. Streeter GL. Developmental horizons in human embryos. Description of age groups XV, SVI, SVII, SVIII. Publication 575. Contributions to embryology, no. 211.32, 133. Washington: Carnegie Institute, 1948. 6. Louw JH, Bernard CN. Congenital intestinalatresia, observations on its origin. Lancet 1955;2:1005. 7. Krieg EG. Duodenal diaphragm. Ann Surg 1937;106:33. 8. Economides NG, McBurney RP, Hamilton FH 111.lntraluminal duodenal diverticulum in the adult. Ann Surg 1977;185: 147. 9. Ferguson AF. Rothfield NJ. Intestinal obstruction by duodenal diaphragm. Great Ormond S! J 1954;8: 105. 10. Nelson WI. Congenital diaphragm of the duodenum. Minn Med 1947;30:745. 11. Knutrud 0, Eek S. Combined intrinsic duodenal obstruction and malrotation. Acta Chir Stand 1960; 119:506. 12. Mansberger AR Jr, l-learn JB, Byers RM, Fleisig N, Buxton RW. Vascular compression of the duodenum, emphasis on accurate diagnosis. Am J Surg 1968;115:84. 13. Hearne JB. Duodenal ileus with special reference to superior mesenteric artefy compression. Radiology 1966;86:305. 14. Wayne E, Miller RE, Eiseman B. Duodenal obstruction by the superior mesenteric artery in bedriien combat casualties. Am J Surg 1971;174:339.
The American Journal of Surgery