Superior mesenteric artery syndrome in burned children

Superior mesenteric artery syndrome in burned children

Superior Mesenteric Artery Syndrome in Burned Children Chukwuma Godwin Ogbuokiri, MD, Cincinnati, Ohio Edward J. Law, MD, Cincinnati, Ohio Bruce G. Ma...

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Superior Mesenteric Artery Syndrome in Burned Children Chukwuma Godwin Ogbuokiri, MD, Cincinnati, Ohio Edward J. Law, MD, Cincinnati, Ohio Bruce G. MacMillan,

MD, Cincinnati, Ohio

Rokitansky in 1842 described a syndrome characterized by emesis of copious, intermittent, bile-stained material in asthenic persons which resulted from compression of the duodenum between the aorta and superior mesenteric vessels. This entity has been designated the superior mesenteric artery syndrome. It has been associated with a variety of pathologic states. Some clinicians continue to question whether the superior mesenteric artery syndrome is a distinct entity [I]. It is described as a complication of appendicitis [2], usually in very ill patients, and as a complication of scoliosis [3]. The term, cast syndrome, is sometimes used because compression of the third portion of the duodenum is common in patients with total body casts [3-51. The condition has also been related to the biped posture [5], prolonged immobilization [6], neurotic states [7j, aganglionosis of Auerbath’s plexus of the duodenum [8], acute pancreatitis [9], and regional enteritis [9]. It may occur in previously robust persons whose disease process entails prolonged immobilization or emaciation. Since the severely burned child tends to lose a great deal of weight within a short time, this symptom complex may develop. Three such cases in adults with burns treated surgically with success have been reported

denum from becoming occluded. In the emaciated patient, however, with the loss of this fatty tissue, the duodenum may become partially or completely obstructed. Clinical Material

Of 390 children with acute burns, four had symptomatic partial obstruction of the duodenum.

As seen in Figure 1, the duodenum passes through the acute angle formed by the aorta posteriorly and the superior mesenteric vessels anteriorly. These vessels compress the transverse portion of the duodenum in a pair-of-scissors fashion with resultant duodenal obstruction which causes megaduodenum and hyperemesis. In the normal person the surrounding retroperitoneal fat is thought to keep the duo-

Case I. The patient was a thirteen year old white boy who was admitted three days after sustaining a 45 per cent total body surface burn, 32 per cent of which was third degree. On admission he weighed 133 pounds and appeared well nourished. Physical examination was otherwise noncontributory. He was treated topically with silver sulfadiazine and placed on a Stryker frame. Vomiting began on the seventh postburn day and recurred after subsequent attempts to feed the patient, especially when he was in the prone position. An upper gastrointestinal series, obtained on the eleventh postburn day (Figure 2), confirmed the diagnosis of vascular compression of the duodenum. Cinefluoroscopy demonstrated megaduodenum, marked retroperistalsis, and reflux of barium into the stomach. Intravenous hyperalimentation was begun and continued for thirty-three days. The patient was transferred from the Stryker frame to a regular hospital bed, the head of which was elevated 40 degrees. With this therapy, his weight stabilized, vomiting subsided, and oral feeding was gradually resumed. The patient had successful autografting on the forty-second and fiftieth postburn days, and he was discharged on the seventy-fourth postburn day. Three months later he had gained 20 pounds. An upper gastrointestinal series nine months post burn did not demonstrate any abnormality.

From the Department of Surgery, Unwerslty of Cincinnati College of Medicine, and Shrwws Burns Institute, Cincinnati, Ohio. Reprint requests should be addressed to Dr Law, Department of Surgery, Cincinnati General Hospital, Cincinnati, Ohio 45229.

Case II. The patient was a five year old black girl transferred to this hospital five days after receiving a 70 per cent total body surface burn, 60 per cent of which was third degree.

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After admission her condition appeared stable except for a positive blood culture for Candida species obtained on admission. Because of the extent of the burn, intravenous hyperalimentation in addition to oral feeding was begun on the eighth postburn day. On the sixteenth postburn day she vomited a large amount of greenish material. Emesis persisted and she required nasogastric drainage. Since guaiac-positive material was recovered from

the nasogastric tube, an antacid ulcer regimen was begun. Oral feeding was tolerated only intermittently. After the thirtieth postburn day, blood cultures were consistently positive for multiple organisms including yeast species, enterococcus, and Staphylococcus aureus. The patient was therefore treated with intravenous penicillin, Staphcillin, and amphotericin B, all of which were continued throughout the remainder of the hospital course. Serial debridements were carried out and the burn wound was dressed with porcine heterografts which were changed every three days. An autograft on the fortieth postburn day did not result in a satisfactory take. Weight loss was severe. Treatment was also complicated by thrombocytobleeding, and pulmonary penia, mild gastrointestinal edema. On the forty-ninth postburn day, because of persistent vomiting of guaiac-positive material, an upper gastrointestinal series was obtained. (Figure 3.) This showed a large ulcer crater on the anterior wall of the stomach which had eroded into the liver. There was marked gastric and duodenal dilatation and a sharp “cut-off” sign in the transverse portion of the duodenum. Positional changes did not reduce the obstruction. Her septic course continued and the patient died on the fifty-second postburn day. Autopsy revealed a moderately dilated stomach containing bloody fluid. Two ulcers were identified, one on the anterior wall of the stomach penetrating the liver substance and the other in the first portion of the duodenum. The proximal half of the duodenum was enlarged. Additional autopsy findings included septic thrombosis of the superior vena cava, bilateral pneumonia, endocarditis, and lower nephron nephrosis. Septicemia due to multiple

Figure 2. Case I. An upper gastrointestinal series obtained on the eleventh postburn day showing obstruction of the third portion of the duodenum. A Levin tube extends into the duodenum.

Case II. An upper gastrointestinal series obFigure 3. tained on the forty-ninth postburn day showing partial obstruction of the third portion of the duodenum. Deformity of the duodenal bulb and a large gastric ulcer penetrating the liver are present.

Figure 1. Schematic representation showing duodenal dilatation and obstruction of the third portion of the duodenum by the superior mesenteric vessels.

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organisms including yeast species and Staph aureus complicating a 70 per cent total body surface burn was judged to be the cause of death. Case III. The patient was a fifteen year old white girl transferred from another hospital thirteen days after receiving a 68 per cent total body surface burn, 40 per cent of which was third degree. She weighed 138 pounds on admission. She was treated with intravenous penicillin for pneumonia of the lower lobe of the left lung. The topical administration of silver sulfadiazine was begun, and she was placed on a rotating circle bed. Oral feeding was well tolerated. On the seventeenth postburn day the patient vomited a large amount of bilious material. An upper gastrointestinal series showed partial duodenal obstruction. Intravenous hyperalimentation was begun and continued until the completion of autografting. As the emesis, which was usually postprandial, occurred with both the supine and prone position, the head of the bed was elevated 40 degrees. The patient’s weight stabilized and then slowly began to increase. Successful autografting was carried out on the thirty-third and forty-third postburn days. A small open area of exposed bone on one hand was slow to heal and a prolonged course of physical therapy was required. The patient was discharged on the one hundred and second postburn day. She has been asymptomatic since discharge. An upper gastrointestinal series performed on postburn day 386 revealed nothing abnormal. Case IV. The patient was a fourteen year old white boy admitted to this hospital four hours after sustaining an 87 per cent total body surface burn, 80 per cent of which was third degree. The patient weighed 174 pounds on admission. He was treated with silver sulfadiazine and placed on a Stryker frame. On the third postburn day, he vomited a large amount of greenish material despite the fact that a functioning Levin tube was in place. An ulcer regimen of antacids was begun via the tube since the aspirate was guaiac-positive. Emesis persisted each time oral feeding was attempted. On the seventh postburn day an abdominal roentgenogram showed distention of the stomach. An upper gastrointestinal series confirmed the diagnosis of superior mesenteric artery compression of the duodenum. The patient was therefore placed in the head-elevated position after which the frequency and volume of the emesis diminished; however, oral feeding was never well tolerated. Intravenous hyperalimentation was begun and continued throughout the patient’s hospital course. In the third week, after he had lost over 40 per cent of his weight on admission, emesis recurred intermittently and a Levin tube was required. From the seventeenth to the fortieth postburn day, he underwent debridement and dressing changes on alternate days. On the twenty-eighth postburn day, an above the elbow amputation of the left arm was required. On the forty-second postburn day a left supracondylar amputation was performed because of nonclostridial gas gangrene.

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Blood cultures were intermittently positive for multiple organisms. The patient received systemic gentamicin and penicillin. On the fifty-second postburn day the chest was autografted. Bleeding developed postoperatively, with oozing from the wounds and hemorrhaging from the gastrointestinal tract. Blood and fresh frozen plasma were required for control. Thereafter, the patient’s condition deteriorated rapidly and he died on the fifty-fifth postburn day. Autopsy revealed superficial giant ulcers of the esophagus and stomach. The pylorus was widely patent without ulceration, and the duodenum was distended. There was evidence of congestion and fatty degeneration of the liver. Death was thought to be due to an 80 per cent third degree burn and septicemia. Comments

These four patients with superior mesenteric artery syndrome were noted to have symptomatic partial obstruction of the duodenum in the area of the vascular pedicle of the small intestine. All patients were of slender or average build. Vomiting was first noted and was most profound with the patient in the prone position. In two patients the vomiting developed in the first postburn week whereas in the others the symptom was noted in the second week. The burns ranged from 45 to 86 per cent of the total body surface and were mostly third degree. Two patients with extremely extensive burns died. The cases are summarized in Table I. All patients with extensive burns tend to lose weight. In two patients, repeated episodes of septicemia increased the caloric demands. The feeding difficulties created by the superior mesenteric artery syndrome further complicate the problem of maintaining adequate nutrition. By the time the diagnosis was confirmed, all patients had lost between 8 and 14 per cent of their weight at admission. Diuresis of edematous fluid may have accounted for some of this early weight loss. The weight loss created by poor nutrition results in partial bowel obstruction. This tends to aggravate the syndrome in a vicious TABLE I

Case

I II Ill IV

Summary of Cases

Per cent of Burn

Per cent of Third Degree Burn

45 70 66

32 60 40

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80

Postburn Day of Onset of Emesis 7 16 17 3

Postburn Day of Upper Gastrointestinal Series 11 49 18 7

Result Recovered Died Recovered Died __-__

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TABLE II

Case

I

Weight Loss

Postburn Day of Admission

Admission Weight (pounds)

TABLE Weight at Onset of Emesis (pounds)

Per cent of Loss

II III

3 5 12

133 46 138

122 42 118

a.5 8.0 14.0

IV

0

174

160

8.0

III

Results of Hyperalimentation

Patient

Days of Hyperalimentation

I II* III Iv*

33 34 26 26

Weight at Start of Hyperalimentation (pounds) 93 40 114 126

Weight at End of Hyperalimentation (pounds) 105 37 108 94

*Died.

cycle since the loss of retroperitoneal fat may increase the degree of obstruction. Weight changes at the onset of emesis are summarized in Table II. Recognition of the syndrome depends on maintaining a high index of suspicion, particularly since there are many possible causes of vomiting in acutely burned patients. Moreover, adynamic ileus can result from septicemia, burn wound sepsis, or electrolyte imbalance. In the presence of active bowel sounds, however, the diagnosis of duodenal obstruction due to compression of the superior mesenteric artery should be considered. This syndrome must be distinguished from other causes of mechanical bowel obstruction which may occur in burned patients such as fecal impaction, which is rare in children, and channel ulcers, which may cause pyloric obstruction. Clinical differentiation between the superior mesenteric artery syndrome and obstruction of the pylorus due to an ulcer is impossible without an upper gastrointestinal series. Burned patients can safely undergo an upper gastrointestinal series if certain precautions are taken. Patients must be transported to the x-ray department by trained personnel, such as a nurse or physician, depending on the condition of the patient. The radiologist and his staff must be aware of the severity of the patient’s illness. A suction unit must be available. If obstruction or ileus is present, most of the barium must be removed from the patient’s stomach and a large-bore Levin tube must be left in place at the termination of the procedure to avoid aspiration of gastric contents. To date, ninety-eight upper gastrointestinal series have been performed in seventy-four patients without complications. The roentgenographic appearance is distinctive. The stomach and duodenum may be dilated, the dilatation of the duodenum being most marked up to the third portion. A cutoff across the third portion is readily demonstrable. There is usually some passage of barium through the partially obstructed area. Cinefluoroscopy, which has been carried out in two

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patients, reveals duodenal dilatation and reflux of barium within the duodenum and into the stomach. Once the diagnosis is established, conservative management should be begun. Nasogastric drainage should be used to decompress the dilated stomach. Thereafter, various positional changes should be employed for feeding since it is believed that the angle between the superior mesenteric vessels and the aorta can be opened by appropriate positioning. The association of vomiting with burned patients in the prone position on the Stryker frame was noted in all four patients, although this posture has been advised for treatment by some authors. The knee-chest position cannot be used in burned patients. The Trendelenberg position has been recommended by Kettenbath and Palumbo [II], but was not employed in our patients because of the risk of aspiration. A semisitting position with the head elevated 45 degrees has been found to give symptomatic relief. The most effective method of preventing this problem is to control the initial severe weight loss. Patients with extensive burns should be given a supplementary high calorie-high protein diet throughout their hospital stay. Intravenous hyperalimentation should be used as required and is invaluable after the syndrome has fully developed. Hyperalimentation is not without risk, however, and septicemia, venous thrombosis, and electrolyte imbalance have been encountered. In patients with extensive burns who cannot tolerate oral feeding, hyperalimentation may be life-saving. This technic was employed in all four patients. The weight changes during the period of hyperalimentation are summarized in Table III. It can be seen that weight loss was slight during the period of hyperalimentation in cases I, II, and III despite the presence of extensive burns. However, severe weight loss did occur during.the period of hyperalimentation in the fourth patient (case IV) who died of uncontrolled sepsis after having undergone amputation of one arm and one leg.

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Two patients (cases II and IV) received almost all of their caloric intake in this way throughout the hospital course. It is believed that their prolonged survival, unusual with burns of this magnitude, was in large measure due to intravenous hyperalimentation. Theoretically, a high caloric regimen, either orally or intravenously, begun soon after initial resuscitation is complete can prevent the usual course of progressive weight loss. This in turn can prevent the development of superior mesenteric artery compression of the duodenum. The association of this syndrome with ulcers has been reported [12] and was encountered in the two patients in our series who died, It is difficult to prove a cause and effect relationship between the two problems since ulcerative disease is commonly associated with both extensive burns and sepsis, as demonstrated by Pruitt, Foley, and Moncrief [12]. One patient (case II) had both a duodenal and a gastric ulcer with penetration of the liver by the gastric ulcer. The gastric ulcer was demonstrated on an upper gastrointestinal series prior to death. (Figure 3.) A second patient (case IV) had both gastric and esophageal ulcerations at autopsy. Since both patients had extensive burns with episodes of sepsis, the incidence of ulcer would be expected to be high. It is possible that the presence of duodenal obstruction added to these other predisposing factors. Abnormalities of ganglion cells in Auerbach’s plexus have been reported by Barnett and Wall [8] as a cause of megaduodenum. In case II a review of a limited number of sections from the duodenum indicated that the Auerbach plexus was intact. In case IV extensive biopsy specimens of the duodenum were taken. Auerbach and Meissner’s plexus were normal. The recommended operative treatment of superior mesenteric artery syndrome has varied over the years. A formidable procedure involving gastroenterostomy, duodenojejunostomy, and gastropylorojejunostomy was described by Delbet [7]. Gastrojejunostomy has been performed [13]. Currently, side to side retrocolic duodenojejunostomy is considered to be the operative procedure of choice [7,10,14] and has successfully been performed in burned patients [lo]. In two cases in our experience, conservative therapy proved adequate. In case II the diagnosis was made in a terminal patient too ill to undergo surgery. In case IV the size of the burn was thought to represent a sufficient contraindication. It is of interest that in the two patients who recovered, weight gain progressed normally and they were

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asymptomatic after discharge. Follow-up upper gastrointestinal series obtained nine and twelve months post burn indicate that no abnormalities remain. Summary

Superior mesenteric artery compression of the duodenum has been diagnosed in 4 of 390 acutely burned children, two of whom survived. Severe weight loss occurred in all patients, Postprandial vomiting of bilious material in the presence of active bowel sounds should suggest the presence of this syndrome. An upper gastrointestinal series, which can be performed safely in patients with extensive burns, is necessary to confirm the diagnosis. Intravenous hyperalimentation may be valuable in providing nutritional support. A semi-upright sitting position after meals may relieve the symptoms. Duodenojejunostomy should be considered if conservative measures fail. As patients with extensive burns survive for progressively longer periods, the incidence of this syndrome can be expected to increase.

References 1. Cimmino CV: Arteriomesenteric occlusion of the duodenum: an entity? Radiology 76: 626, 1961. 2. Bloodgood JC: Acute dilatation of the stomach-gastromesenteric ileus. Ann Surg 46: 736, 1907. 3. Bunch W, Delaney J: Scoliosis and acute vascular compression of the duodenum. Surgery 67: 901, 1970. 4. Dorph MD: The cast syndrome: review of the literature and report of a case. New Eng J Med 243: 440,195O. 5. Kaiser GC, McKain JM, Shumacker HB: The superior mesenteric artery syndrome. Surg Gynec Obstet 110: 133, 1960. 6. Mindell HJ, Holm JL: Acute superior mesenteric artery syndrome. Radiology 94: 299, 1970. 7. Rosenburg SA, Sampson A: The syndrome of mesenteric vascular compression of the duodenum. Arch Surg 73: 296,1956. 6. Barnett WO, Wall L: Megaduodenum resulting from absence of parasympathetic ganglion cells in Auerbach’s plexus. Ann Surg 141: 527, 1955. 9. Simon M, Lerner MA: Duodenal compression by the mesenteric root in acute pancreatitis and inflammatory conditions of the bowel. Radiology 79: 75, 1962. 10. Wallace RG, Howard WB: Acute superior mesenteric artery syndrome in the severely burned patient. Radiology 94: 307,197o. 11. Kettenbach EL, Palumbo LT: Arteriomesenteric duodenal compression: surgical treatment. Arch Surg 62: 145, 1951. 12. Pruitt BA, Foley FD, Moncrief JA: Curling’s ulcer: a clinical pathology study of 323 cases. Ann Surg 172: 523, 1970. 13. Stavely A: Chronic gastromesenteric ileus. Surg Gynec Obstet 11: 266, 1910. 14. Pool EH, Niles WL, Martin KA: Duodenal stasis: duodenojejunostomy. Ann Surg 96: 567, 1933.

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