WAIN INJURY,
1992, VOL. 6, NO. 4,351-358
Superior mesenteric artery syndrome: an unusual cause of intestinal obstruction in brain-injured children
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PULIYODIL A . PHILIP Department of Physical Medicine and Rehabilitation, Northwestern University Medical School and Rehabilitation Institute of Chicago, 345 East Superior Street, Chicago, Illinois 6061 1, USA
(Received 27Jdy 1991; accepted 20 September 1991) Superior mesenteric artery syndrome is a rare cause of upper intestinal obstruction in both adults and children. Sixteen children with severe traumatic brain injury and spastic quadriparesis developed small intestinal obstruction while undergoing a rehabilitation programme between 1981 and 1990. Five patients met the roentgenographic diagnostic criteria. The presenting symptom was post-prandial bilious vomiting. The mean age was 13 (10-16) years. The mean time elapsed from injury to diagnosis was 53 days and from rehabilitation admission to diagnosis 22 days. The mean delay in diagnosis after onset of symptoms was 4 days. All patients were of disproportionately lower body weight in relation to height, with a mean weight loss of 7 kg. The mean percentile for weight was 18 and height 58, with a difference of 30 between height and weight percentiles. The patients were receiving nasogastric or gastrostomy tube fecdings at the onset of the symptoms. All patients were treated non-surgically with gastric aspiration, nasojejunal or gastrojejunal feeding by passing a feeding tube distal to obstruction. No patient required intravenous hyperalimentation. There was no recurrence in any patient during the follow-up period of 1-5 years. Though rare, superior mesentcric artery syndrome can develop in brain-injured children with spastic quadriparesis, prolonged recumbency and recent weight loss. Increased awareness of occurrence of this condition and timely management will decrease morbidity and complications that may interfere with recovery.
Introduction Superior mesenteric artery (SMA) syndrome is an unusual form of high intestinal obstruction due to compression at the third part of duodenum by SMA against the aorta [l, 21. This entity was first described by Rolutansky in 1842 [3]. Subsequently Wilkie reported the first comprehensive series of 75 cases in 1927 [4].This condtion is known by a variety of names such as vascular compression of the duodenum, superior mesenteric artery syndrome, chronic intermittent arteriomesenteric occlusion of the duodenum, arteriomesenteric duodenal obstruction, chronic duodenal ileus and Wilkie’s syndrome [5-91. Some investigators suggested that this condition occurs only very infrequently [lo]. The classical symptoms are post-prandial epigastric pain, fullness, nausea, vomiting and weight loss with partial or complete relief when the patient adopts the knee-elbow o r left lateral positions [ l l , 121. Upper gastrointestinal (UGI) contrast study is the standard for making the diagnosis. Hines and associates used the following roentgenographic criteria for the diagnosis [13]: (1) dilatation of the first and second portions of the duodenum, with or without gastric dilatation; (2) abrupt vertical and oblique compression of mucosal folds; (3) antiperistaltic flow of barium proximal to the obstruction producing to-and-fro 0269-9052/92 $3.00 0 1992 Taylor & Francis Ltd.
P . A . Philip
352
Brain Inj Downloaded from informahealthcare.com by V U L Periodicals Rec on 12/27/14 For personal use only.
niovements; (1)delay in transit of 4-6 h through the gastroduodenal regon; (5) relief of obstruction when the patient is placed in a position that diminishes the drag of the small bowel mesentery (the left lateral decubitus, prone or knee-chest position) [ 141. SMA syndrome, also known as cast syndrome, can occur in adults and children. Only three separate cases of SMA syndrome have been reported in the literature concerning brain-injured children during the immediate post-acute period [ 13, 151. There has been no report of occurrence of this clinical entity in children with traumatic brain injury while undergoing a rehabilitation programme. The purpose of this paper is to emphasize the occurrence of this rare syndrome in children after traumatic brain injury, and to discuss the management options.
Methods Medical records of all children under the age of 18 years with severe traumatic brain injuries and the diagnosis of SMA syndrome or upper gastrointestinal obstruction from 1981 to 1990 were reviewed. Patient demographics including age, sex, cause of brain in-jury, Glasgow Coma Scale (GCS), length of stay in acute care and rehabilitation hospitals, and time elapsed from brain injury and from rehabilitation admission to diagnosis of SMA syndrome were recorded. The UGI series were used to confirni the clinical diagnosis of SMA syndrome. The radiographic criteria used for diagnosis were the following: (1) abrupt vertical and oblique extrinsic compression of the third part of the duodenum and dilatation of the first and second portion, and (2) marked antiperistaltic flow of barium proximal to the obstruction producing to-and-fro movements [13]. Level of cognitive function (LCF) at diagnosis and discharge was recorded using the measure developed at Rancho Los Amigos Hospital [16]. This included the following: I-no response; 11-generalized response; 111-localized response; IV-confused and agitated; V-onfused, inappropriate and non-agitated; VI-appropriate and confused; VIII-automatic and appropriate; VIII-purposeful.
Results There were 321 children under the age of 18 years admitted to the paediatric rehabilitation programme with the diagnosis of severe traumatic brain injury during the 10-year period. Of these children, 31% were 0-5, 33% were 6-12 and 35% were 13-18 years old respectively. There were 16 cases with the diagnosis of small intestinal obstruction and of these five patients met the UGI roentgenographic criteria for the diagnosis of SMA syndrome (Figure 1). Patient demographics, management and outcome are shown in Table 1. Among the five patients, three were males and two were females, with the mean age of 13 (10-16) years. All patients suffered severe brain injuries due to pedestrian-motor vehicle accidents. GCS during the first 24 h ranged from 3 to 5 with a mean of 4. The length of hospitalization at the acute-care hospital ranged from 21 to 44 days with a mean of 38 days, and the mean rehabilitation stay was 67 (41-82) days. The mean time elapsed from injury to diagnosis of SMA syndrome was 53 (38-72) days. Three patients were diagnosed during the third week of rehabilitation stay, the fourth patient during the fourth week and the fifth in the fifth week. The patients were of disproportionately lower body weight in relation to height. The mean percentile for weight was 18 and height 58. The difference between height and weight percentiles ranged from 30 to 55 with a mean of 38. There was a mean loss of 7 kg of body weight since the injury. All patients had been receiving non-oral feeding before the onset of
Brain Inj Downloaded from informahealthcare.com by V U L Periodicals Rec on 12/27/14 For personal use only.
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353
Figure 1 . Film from UGI series of an 1 I-year-old male patient showing abrupt vertical termination of barium column in the third portion ofthe duodenum. Note thegastrortomyfeeding tube in place.
symptoms, three patients through gastrostomy tube and two through nasogastric tube. In one patient the symptoms began soon after insertion of a gastrostomy tube percutaneously. Post-prandial bilious vomiting was the main clinical presentation. Vomiting was intermittent initially and it became regular, usually within the first 24 h. Three patients developed moderate abdominal distension, with normal bowel sounds. Flat-plate roentgenograms of abdomen revealed non-specific gas patterns, and in two patients moderate gastric dilatation. Definitive diagnosis was made in five patients based on diagnostic criteria in UGI series. All patients in this study received non-surgical treatment. The gastric contents were aspirated and the feeding tubes were inserted past the obstruction into jejunum, and the patients continued to receive nasojejunal or gastrojejunal tube feedings. The patients were also placed right side up during, and for about 1 h after, each feeding. No intravenous hyperalimentation was used. Four patients became oral feeders before discharge. One patient who continued to require tube feeding at discharge received jejunostomy tube placement before discharge. All five patients in this series developed hip flexion contractures ranging from 15 to 45" with associated increase in lumbar lordosis before the diagnosis of SMA syndrome. The LCF of the patients at the time of diagnosis of Sh4A syndrome ranged from 11 to IV. The LCF of oral feeders a t discharge ranged from VI to VIII, and that of the remaining patient was IV. Length of follow-up ranged from 1 to 5 years and there was no recurrence.
IV/VII III/VII
5
5
3
3 / 14/F
4/15/M
5/16/F 60/30
60125
55/20
65/10
50/15
6
8
5
7
5
0%)
Weight loss
and outcome.
+
+ + +
+
Prolonged recumbency
Patient demopphics, management
Percentile Ht/Wt
1.
Non-surgcal/ gastroj ejunal Non-surgcal/ gastrojejunal Non-surgical/ nasojcjunal Non-surgcal/ nasojejunal Non-surgical/ gastrojejunal
Treatment/ feeding
GCS = Glasgow Coma Scale; LCF = level of cognitive function; A/D = admission/dischargc; Ht/Wt = heightlweight
IV/VI
III/VII
4
2/11/M
II/IV
LCF AID
3
GCS
l/lO/M
Patient No./age (ycars)/wx
..table
Brain Inj Downloaded from informahealthcare.com by V U L Periodicals Rec on 12/27/14 For personal use only.
Asymptomatic/ oral Asymptomatic/ oral
O d
Jejunostomy/ non-oral Asymptomatic/ oral Asymptomatic/
Outcome/ feeding
355
SMA syndrome in brain-injured children
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Discussion SMA syndrome is a rare form of duodenal obstruction first described by Rokitansky in 1842 [3]. Since that time the entity has remained as a subject of controversy [17]. Many later studies concluded that SMA syndrome is a real, although overdiagnosed, disorder [13, 18, 191. The advent of modern roentgenographic techniques provides workers with the evidence needed to support the existence of the syndrome [2]. The SMA is the second branch of the abdominal aorta at the level of the first lumbar vertebra, and supplies the entire small bowel except the duodenal bulb. In its course the SMA crosses the duodenum and may cause compression of the relatively immobile third portion against the aorta producing a 'nutcracker' effect (Figure 2) [19]. The mean angle formed by the SMA and aorta has been described by various authors as 38", 41" and 56" respectively [20-221. Complete obstruction is related to a number of factors that can diminish the arteriomesenteric angle to approximately 6-16" [22, 231. Factors include prolonged supine position, visceroptosis, exaggerated lumbar lordosis, abnormally high fixation of the dudenal fixture of the ligament of Treitz, an unusually low origin of the SMA, loss of muscle tone in the duodenum or marked weight loss with subsequent loss of mesenteric and retroperitoneal fat [13]. The disorders that predispose to the SMA syndrome have been divided into five categories: (1) severe wasting diseases such as cancer
Duodenum Figure 2. Diagram showing the anatomical relationship among the duodenum, aorta, and superior mesenteric artery in SMA syndrome. The lateral view demonstrates nutcracker effect with compression of the duodenum between the vascular structures. (Reproduced with pemissionfrom r$ 19.)
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3.56
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and burns; (2) severe injuries; (3) disease, deformity or trauma of the spine; (4) dietary disorders such as anorexia nervosa or malabsorption; and (5) spinal instrumentation (24-271. Predisposing events such as cast application, insertion of feeding gastrostomy, abdominal procedures and endoscopy have also been identified. SMA syndrome in children after brain injury has not been described in the rehabilitation setting. It is more commonly seen in children following spinal instrumentation or cart application, especially with the prior existence of spinal deformities 11, 251. The age ofpediatric patients with this condition ranges from 12 to 18 years and is fairly constant. In this series the mean age was 13 years. This age group corresponds to the adolescent growth spurt in which height increases a t a faster rate, causing a temporary decrease in SMA angle. Thus a tall, thin person would tend to have a smaller angle and a greater chance for development of SMA syndrome. Many patients who develop this entity also have significant difference between the age-related height and weight percentiles [18]. The contributing factors in this series include prolonged recumbency, spasticity, hip flexion contractures producing increased lumbar lordosis and recent weight loss. The acute form of the disorder is reversible if the initiating factor can be corrected 1191. A chronic form of the syndrome is suggested by a long history of intermittent recurrent upper gastrointestinal obstruction which may culminate in an acute episode of duodenal obstruction unresponsive to medical therapy [ 191. Most patients with SMA syndrome present with nausea and bilious vomiting of variable dcgreer. As in this series, vomiting tends to be intermittent and most often post-prandial (19, 281. Epigastric pain and abdominal distension are reported in 50 - 200% of cases 12, 291. Post-prandial bilious vomiting was the cardinal feature in the patients in this series, and epigastric pain could not be demonstrated due to the low LFC. The UGI series using barium is the standard in making the diagnosis based on specific radiological criteria 12, 13). Flouroscopic examination provides most information, which may not be revealed in static roentgenograms. In chronic cases angiography can be used to measure the arteriomesenteric angle. There are a number of therapeutic approaches to SMA syndrome. A trial of non-surgical management is indicated in all patients. Griffith and Whitchouse reported 10 paediatnc patients who were successfully treated with nasogastric drainage and intravenous fluid support [29]. Therapy includes gastric decompression, parenteral alimentation, feeding through a tube passed distal to the obstruction, prone or left lateral position, and correction of fluid and electrolyte imbalance. Total parenteral nutrition, by producing a rapid increase in weight, has been an effective adjunct if weight loss is considered as a precipitating factor. Care should be taken to identify and correct any precipitating factor. In children with brain injuries and low LCF, prolonged recumbency should be avoided and measures should be taken to decrease or avoid abnormal posturing, spasticity and soft tissue contractures of joints, especially hip joints. In this series the gastric decompression was achieved by aspirating through the existing tube. The nasogastric tube was then passed distal to the obstruction into the jejunum. In cases of patients with a gastrostomy tube, a second feeding tube was passed through the gastrostomy stoma into the jejunum while the gastrostomy tube was used for gastric decompression. All patients in this study were successfillly treated with nasojejunal or gastrojejunal feedings and none required intravenous hyperalimentation or surgcal intervention. S u r g d management of the SMA syndrome has been attempted for almost a century. Certain criteria must be niet before operative intervention: (1) the patient meets all radiographic and clinical criteria for the diagnosis; (2) a thorough radiographic, endoscopic and psychological evaluation rules out other causes; ( 3 ) the symptoms do not disappear
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S M A syndrome in brain-injured children
357
spontaneously after a prolonged period of observation and medical therapy; and (4) in thin and cachectic individuals an attempt at weight gain is unsuccessful [18]. The surgical procedures include duodenojejunostomy, gastrojejunostomy, lysis of the ligament of Trietz and derotation of small bowel and right colon [18]. The results of gastrojejunostomy were inferior to duodenojejunostomy, as in the former the duodenal obstruction cannot be completely relieved and the biliary and pancreatic secretions will still descend the duodenum causing recurrent symptoms, blind loop and marginal ulceration. Lee and Mangla reported that statistically the results of duodenojejunostomy were significantly better than the other procedures [l]. Marchant and associates reported a 90% success rate with the derotation procedure [18]. This procedure does not require enterostomy, and a bypass procedure can still be easily performed to alleviate obstruction if symptoms persist.
Conclusions In this series of 321 children with severe traumatic brain injuries five (1.5%) developed SMA syndrome during the post-acute period. O f 16 children with severe brain injuries and upper gastrointestinal obstruction five (31%) were diagnosed with SMA syndrome. While the incidence in the severely brain-injured children is difficult to determine, the reported incidence in other conditions ranged from 0.10 to 0.53% [2, 71. The UGI series is the standard in making the diagnosis. If the patients are unable to swallow, barium can be introduced into the stomach through nasogastric or gastric tube. The principles of non-surgical treatment include gastric decompression, jejunal feeding, correction of electrolyte imbalance, proper positioning and management of spasticity and contractures. Increased awareness and timely management of SMA syndrome will effectively reduce the sequelae such as progressive dehydration, weight loss, oliguria and hypokalaemia, and decrease additional morbidty that may interfere with the functional recovery of these brain-injured children.
References 1. LEE, C. and MANGLA, J. C.: Superior mesenteric artery compression syndrome. American Jourrial of Gastroenterology, 70: 141-150, 1978. 2. ANDERSON, J. R., EARNSHAW, P. M. and FKASER,G. M.: Extrinsic compression of the third part of the duodenum. Clinical Radiology, 33: 75-81, 1982. 3. ROKITANSKY, C.: Lehrbuch der pathologische Anatomie (Branmuller and Seidcl, Vienna), vol. 3, p. 187, 1842. 4. WILKIE, D. P. D.: Chronic duodenal ileus. American Journal of Medical Science, 173: 643-649, 1927. H. B. and SHERMAN,C. D.: Vascular cornpression of the duodenum. International 5. BARNEK, Abstracts OfSurgery, 117: 102-118, 1963. 6. MCKINNON, D. A. and SPENCEK, J. R.: Superior mesenteric artery syndrome. AmericanJournal OfSurgery, 106: 552-557, 1963. 7. GOIN,L. S. and WILK,S. P.: Intermittent arterio-mesentcric occlusion of the duodenum. Radiology, 67: 729-737, 1956. 8. STRONG,E. K.: Mechanics of arterio-mcscnteric duodenal obstruction. Annals of Surgery, 148: 725-730, 1958. 9. WILKIE, D. P. D.: Chronic duodenal ilcus. Britishjournal ofSurgery, 9: 204214, 1921. 10. CIMMINO, C. V.: Superior mesenteric artery syndrome. New York State Journal of Medicine, 76: 986-988, 1976. 11. MARTINEZ, N. S., KHAN, A. H., PACIS,A. and LEKAS, C.: Arterio-mescnteric duodenal compression syndrome: a study of 24 cases. Vascular Surgery, 13: 1-10, 1979.
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12. ANDEKSON, W . C., VIVIT,I
1992, VOL. 6, NO. 4,351-358
Superior mesenteric artery syndrome: an unusual cause of intestinal obstruction in brain-injured children
Brain Inj Downloaded from informahealthcare.com by V U L Periodicals Rec on 12/27/14 For personal use only.
PULIYODIL A . PHILIP Department of Physical Medicine and Rehabilitation, Northwestern University Medical School and Rehabilitation Institute of Chicago, 345 East Superior Street, Chicago, Illinois 6061 1, USA
(Received 27Jdy 1991; accepted 20 September 1991) Superior mesenteric artery syndrome is a rare cause of upper intestinal obstruction in both adults and children. Sixteen children with severe traumatic brain injury and spastic quadriparesis developed small intestinal obstruction while undergoing a rehabilitation programme between 1981 and 1990. Five patients met the roentgenographic diagnostic criteria. The presenting symptom was post-prandial bilious vomiting. The mean age was 13 (10-16) years. The mean time elapsed from injury to diagnosis was 53 days and from rehabilitation admission to diagnosis 22 days. The mean delay in diagnosis after onset of symptoms was 4 days. All patients were of disproportionately lower body weight in relation to height, with a mean weight loss of 7 kg. The mean percentile for weight was 18 and height 58, with a difference of 30 between height and weight percentiles. The patients were receiving nasogastric or gastrostomy tube fecdings at the onset of the symptoms. All patients were treated non-surgically with gastric aspiration, nasojejunal or gastrojejunal feeding by passing a feeding tube distal to obstruction. No patient required intravenous hyperalimentation. There was no recurrence in any patient during the follow-up period of 1-5 years. Though rare, superior mesentcric artery syndrome can develop in brain-injured children with spastic quadriparesis, prolonged recumbency and recent weight loss. Increased awareness of occurrence of this condition and timely management will decrease morbidity and complications that may interfere with recovery.
Introduction Superior mesenteric artery (SMA) syndrome is an unusual form of high intestinal obstruction due to compression at the third part of duodenum by SMA against the aorta [l, 21. This entity was first described by Rolutansky in 1842 [3]. Subsequently Wilkie reported the first comprehensive series of 75 cases in 1927 [4].This condtion is known by a variety of names such as vascular compression of the duodenum, superior mesenteric artery syndrome, chronic intermittent arteriomesenteric occlusion of the duodenum, arteriomesenteric duodenal obstruction, chronic duodenal ileus and Wilkie’s syndrome [5-91. Some investigators suggested that this condition occurs only very infrequently [lo]. The classical symptoms are post-prandial epigastric pain, fullness, nausea, vomiting and weight loss with partial or complete relief when the patient adopts the knee-elbow o r left lateral positions [ l l , 121. Upper gastrointestinal (UGI) contrast study is the standard for making the diagnosis. Hines and associates used the following roentgenographic criteria for the diagnosis [13]: (1) dilatation of the first and second portions of the duodenum, with or without gastric dilatation; (2) abrupt vertical and oblique compression of mucosal folds; (3) antiperistaltic flow of barium proximal to the obstruction producing to-and-fro 0269-9052/92 $3.00 0 1992 Taylor & Francis Ltd.
P . A . Philip
352
Brain Inj Downloaded from informahealthcare.com by V U L Periodicals Rec on 12/27/14 For personal use only.
niovements; (1)delay in transit of 4-6 h through the gastroduodenal regon; (5) relief of obstruction when the patient is placed in a position that diminishes the drag of the small bowel mesentery (the left lateral decubitus, prone or knee-chest position) [ 141. SMA syndrome, also known as cast syndrome, can occur in adults and children. Only three separate cases of SMA syndrome have been reported in the literature concerning brain-injured children during the immediate post-acute period [ 13, 151. There has been no report of occurrence of this clinical entity in children with traumatic brain injury while undergoing a rehabilitation programme. The purpose of this paper is to emphasize the occurrence of this rare syndrome in children after traumatic brain injury, and to discuss the management options.
Methods Medical records of all children under the age of 18 years with severe traumatic brain injuries and the diagnosis of SMA syndrome or upper gastrointestinal obstruction from 1981 to 1990 were reviewed. Patient demographics including age, sex, cause of brain in-jury, Glasgow Coma Scale (GCS), length of stay in acute care and rehabilitation hospitals, and time elapsed from brain injury and from rehabilitation admission to diagnosis of SMA syndrome were recorded. The UGI series were used to confirni the clinical diagnosis of SMA syndrome. The radiographic criteria used for diagnosis were the following: (1) abrupt vertical and oblique extrinsic compression of the third part of the duodenum and dilatation of the first and second portion, and (2) marked antiperistaltic flow of barium proximal to the obstruction producing to-and-fro movements [13]. Level of cognitive function (LCF) at diagnosis and discharge was recorded using the measure developed at Rancho Los Amigos Hospital [16]. This included the following: I-no response; 11-generalized response; 111-localized response; IV-confused and agitated; V-onfused, inappropriate and non-agitated; VI-appropriate and confused; VIII-automatic and appropriate; VIII-purposeful.
Results There were 321 children under the age of 18 years admitted to the paediatric rehabilitation programme with the diagnosis of severe traumatic brain injury during the 10-year period. Of these children, 31% were 0-5, 33% were 6-12 and 35% were 13-18 years old respectively. There were 16 cases with the diagnosis of small intestinal obstruction and of these five patients met the UGI roentgenographic criteria for the diagnosis of SMA syndrome (Figure 1). Patient demographics, management and outcome are shown in Table 1. Among the five patients, three were males and two were females, with the mean age of 13 (10-16) years. All patients suffered severe brain injuries due to pedestrian-motor vehicle accidents. GCS during the first 24 h ranged from 3 to 5 with a mean of 4. The length of hospitalization at the acute-care hospital ranged from 21 to 44 days with a mean of 38 days, and the mean rehabilitation stay was 67 (41-82) days. The mean time elapsed from injury to diagnosis of SMA syndrome was 53 (38-72) days. Three patients were diagnosed during the third week of rehabilitation stay, the fourth patient during the fourth week and the fifth in the fifth week. The patients were of disproportionately lower body weight in relation to height. The mean percentile for weight was 18 and height 58. The difference between height and weight percentiles ranged from 30 to 55 with a mean of 38. There was a mean loss of 7 kg of body weight since the injury. All patients had been receiving non-oral feeding before the onset of
Brain Inj Downloaded from informahealthcare.com by V U L Periodicals Rec on 12/27/14 For personal use only.
SMA syndrome in brain-injured
children
353
Figure 1 . Film from UGI series of an 1 I-year-old male patient showing abrupt vertical termination of barium column in the third portion ofthe duodenum. Note thegastrortomyfeeding tube in place.
symptoms, three patients through gastrostomy tube and two through nasogastric tube. In one patient the symptoms began soon after insertion of a gastrostomy tube percutaneously. Post-prandial bilious vomiting was the main clinical presentation. Vomiting was intermittent initially and it became regular, usually within the first 24 h. Three patients developed moderate abdominal distension, with normal bowel sounds. Flat-plate roentgenograms of abdomen revealed non-specific gas patterns, and in two patients moderate gastric dilatation. Definitive diagnosis was made in five patients based on diagnostic criteria in UGI series. All patients in this study received non-surgical treatment. The gastric contents were aspirated and the feeding tubes were inserted past the obstruction into jejunum, and the patients continued to receive nasojejunal or gastrojejunal tube feedings. The patients were also placed right side up during, and for about 1 h after, each feeding. No intravenous hyperalimentation was used. Four patients became oral feeders before discharge. One patient who continued to require tube feeding at discharge received jejunostomy tube placement before discharge. All five patients in this series developed hip flexion contractures ranging from 15 to 45" with associated increase in lumbar lordosis before the diagnosis of SMA syndrome. The LCF of the patients at the time of diagnosis of Sh4A syndrome ranged from 11 to IV. The LCF of oral feeders a t discharge ranged from VI to VIII, and that of the remaining patient was IV. Length of follow-up ranged from 1 to 5 years and there was no recurrence.
IV/VII III/VII
5
5
3
3 / 14/F
4/15/M
5/16/F 60/30
60125
55/20
65/10
50/15
6
8
5
7
5
0%)
Weight loss
and outcome.
+
+ + +
+
Prolonged recumbency
Patient demopphics, management
Percentile Ht/Wt
1.
Non-surgcal/ gastroj ejunal Non-surgcal/ gastrojejunal Non-surgical/ nasojcjunal Non-surgcal/ nasojejunal Non-surgical/ gastrojejunal
Treatment/ feeding
GCS = Glasgow Coma Scale; LCF = level of cognitive function; A/D = admission/dischargc; Ht/Wt = heightlweight
IV/VI
III/VII
4
2/11/M
II/IV
LCF AID
3
GCS
l/lO/M
Patient No./age (ycars)/wx
..table
Brain Inj Downloaded from informahealthcare.com by V U L Periodicals Rec on 12/27/14 For personal use only.
Asymptomatic/ oral Asymptomatic/ oral
O d
Jejunostomy/ non-oral Asymptomatic/ oral Asymptomatic/
Outcome/ feeding
355
SMA syndrome in brain-injured children
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Discussion SMA syndrome is a rare form of duodenal obstruction first described by Rokitansky in 1842 [3]. Since that time the entity has remained as a subject of controversy [17]. Many later studies concluded that SMA syndrome is a real, although overdiagnosed, disorder [13, 18, 191. The advent of modern roentgenographic techniques provides workers with the evidence needed to support the existence of the syndrome [2]. The SMA is the second branch of the abdominal aorta at the level of the first lumbar vertebra, and supplies the entire small bowel except the duodenal bulb. In its course the SMA crosses the duodenum and may cause compression of the relatively immobile third portion against the aorta producing a 'nutcracker' effect (Figure 2) [19]. The mean angle formed by the SMA and aorta has been described by various authors as 38", 41" and 56" respectively [20-221. Complete obstruction is related to a number of factors that can diminish the arteriomesenteric angle to approximately 6-16" [22, 231. Factors include prolonged supine position, visceroptosis, exaggerated lumbar lordosis, abnormally high fixation of the dudenal fixture of the ligament of Treitz, an unusually low origin of the SMA, loss of muscle tone in the duodenum or marked weight loss with subsequent loss of mesenteric and retroperitoneal fat [13]. The disorders that predispose to the SMA syndrome have been divided into five categories: (1) severe wasting diseases such as cancer
Duodenum Figure 2. Diagram showing the anatomical relationship among the duodenum, aorta, and superior mesenteric artery in SMA syndrome. The lateral view demonstrates nutcracker effect with compression of the duodenum between the vascular structures. (Reproduced with pemissionfrom r$ 19.)
Brain Inj Downloaded from informahealthcare.com by V U L Periodicals Rec on 12/27/14 For personal use only.
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and burns; (2) severe injuries; (3) disease, deformity or trauma of the spine; (4) dietary disorders such as anorexia nervosa or malabsorption; and (5) spinal instrumentation (24-271. Predisposing events such as cast application, insertion of feeding gastrostomy, abdominal procedures and endoscopy have also been identified. SMA syndrome in children after brain injury has not been described in the rehabilitation setting. It is more commonly seen in children following spinal instrumentation or cart application, especially with the prior existence of spinal deformities 11, 251. The age ofpediatric patients with this condition ranges from 12 to 18 years and is fairly constant. In this series the mean age was 13 years. This age group corresponds to the adolescent growth spurt in which height increases a t a faster rate, causing a temporary decrease in SMA angle. Thus a tall, thin person would tend to have a smaller angle and a greater chance for development of SMA syndrome. Many patients who develop this entity also have significant difference between the age-related height and weight percentiles [18]. The contributing factors in this series include prolonged recumbency, spasticity, hip flexion contractures producing increased lumbar lordosis and recent weight loss. The acute form of the disorder is reversible if the initiating factor can be corrected 1191. A chronic form of the syndrome is suggested by a long history of intermittent recurrent upper gastrointestinal obstruction which may culminate in an acute episode of duodenal obstruction unresponsive to medical therapy [ 191. Most patients with SMA syndrome present with nausea and bilious vomiting of variable dcgreer. As in this series, vomiting tends to be intermittent and most often post-prandial (19, 281. Epigastric pain and abdominal distension are reported in 50 - 200% of cases 12, 291. Post-prandial bilious vomiting was the cardinal feature in the patients in this series, and epigastric pain could not be demonstrated due to the low LFC. The UGI series using barium is the standard in making the diagnosis based on specific radiological criteria 12, 13). Flouroscopic examination provides most information, which may not be revealed in static roentgenograms. In chronic cases angiography can be used to measure the arteriomesenteric angle. There are a number of therapeutic approaches to SMA syndrome. A trial of non-surgical management is indicated in all patients. Griffith and Whitchouse reported 10 paediatnc patients who were successfully treated with nasogastric drainage and intravenous fluid support [29]. Therapy includes gastric decompression, parenteral alimentation, feeding through a tube passed distal to the obstruction, prone or left lateral position, and correction of fluid and electrolyte imbalance. Total parenteral nutrition, by producing a rapid increase in weight, has been an effective adjunct if weight loss is considered as a precipitating factor. Care should be taken to identify and correct any precipitating factor. In children with brain injuries and low LCF, prolonged recumbency should be avoided and measures should be taken to decrease or avoid abnormal posturing, spasticity and soft tissue contractures of joints, especially hip joints. In this series the gastric decompression was achieved by aspirating through the existing tube. The nasogastric tube was then passed distal to the obstruction into the jejunum. In cases of patients with a gastrostomy tube, a second feeding tube was passed through the gastrostomy stoma into the jejunum while the gastrostomy tube was used for gastric decompression. All patients in this study were successfillly treated with nasojejunal or gastrojejunal feedings and none required intravenous hyperalimentation or surgcal intervention. S u r g d management of the SMA syndrome has been attempted for almost a century. Certain criteria must be niet before operative intervention: (1) the patient meets all radiographic and clinical criteria for the diagnosis; (2) a thorough radiographic, endoscopic and psychological evaluation rules out other causes; ( 3 ) the symptoms do not disappear
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spontaneously after a prolonged period of observation and medical therapy; and (4) in thin and cachectic individuals an attempt at weight gain is unsuccessful [18]. The surgical procedures include duodenojejunostomy, gastrojejunostomy, lysis of the ligament of Trietz and derotation of small bowel and right colon [18]. The results of gastrojejunostomy were inferior to duodenojejunostomy, as in the former the duodenal obstruction cannot be completely relieved and the biliary and pancreatic secretions will still descend the duodenum causing recurrent symptoms, blind loop and marginal ulceration. Lee and Mangla reported that statistically the results of duodenojejunostomy were significantly better than the other procedures [l]. Marchant and associates reported a 90% success rate with the derotation procedure [18]. This procedure does not require enterostomy, and a bypass procedure can still be easily performed to alleviate obstruction if symptoms persist.
Conclusions In this series of 321 children with severe traumatic brain injuries five (1.5%) developed SMA syndrome during the post-acute period. O f 16 children with severe brain injuries and upper gastrointestinal obstruction five (31%) were diagnosed with SMA syndrome. While the incidence in the severely brain-injured children is difficult to determine, the reported incidence in other conditions ranged from 0.10 to 0.53% [2, 71. The UGI series is the standard in making the diagnosis. If the patients are unable to swallow, barium can be introduced into the stomach through nasogastric or gastric tube. The principles of non-surgical treatment include gastric decompression, jejunal feeding, correction of electrolyte imbalance, proper positioning and management of spasticity and contractures. Increased awareness and timely management of SMA syndrome will effectively reduce the sequelae such as progressive dehydration, weight loss, oliguria and hypokalaemia, and decrease additional morbidty that may interfere with the functional recovery of these brain-injured children.
References 1. LEE, C. and MANGLA, J. C.: Superior mesenteric artery compression syndrome. American Jourrial of Gastroenterology, 70: 141-150, 1978. 2. ANDERSON, J. R., EARNSHAW, P. M. and FKASER,G. M.: Extrinsic compression of the third part of the duodenum. Clinical Radiology, 33: 75-81, 1982. 3. ROKITANSKY, C.: Lehrbuch der pathologische Anatomie (Branmuller and Seidcl, Vienna), vol. 3, p. 187, 1842. 4. WILKIE, D. P. D.: Chronic duodenal ileus. American Journal of Medical Science, 173: 643-649, 1927. H. B. and SHERMAN,C. D.: Vascular cornpression of the duodenum. International 5. BARNEK, Abstracts OfSurgery, 117: 102-118, 1963. 6. MCKINNON, D. A. and SPENCEK, J. R.: Superior mesenteric artery syndrome. AmericanJournal OfSurgery, 106: 552-557, 1963. 7. GOIN,L. S. and WILK,S. P.: Intermittent arterio-mesentcric occlusion of the duodenum. Radiology, 67: 729-737, 1956. 8. STRONG,E. K.: Mechanics of arterio-mcscnteric duodenal obstruction. Annals of Surgery, 148: 725-730, 1958. 9. WILKIE, D. P. D.: Chronic duodenal ilcus. Britishjournal ofSurgery, 9: 204214, 1921. 10. CIMMINO, C. V.: Superior mesenteric artery syndrome. New York State Journal of Medicine, 76: 986-988, 1976. 11. MARTINEZ, N. S., KHAN, A. H., PACIS,A. and LEKAS, C.: Arterio-mescnteric duodenal compression syndrome: a study of 24 cases. Vascular Surgery, 13: 1-10, 1979.
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12. ANDEKSON, W . C., VIVIT,I
