Pediatr Radio1 (1990) 20:58~589
Pediatric Radiology 9 Springer-Verlag 1990
Familial superior mesenteric artery syndrome C. O r t i z 1, R. H. C l e v e l a n d I , J. G. B l i c k m a n 1, D. J a r a m i l l o 1 a n d S..H. K i m 2 Divisions of i Pediatric Radiology and 2 Pediatric Surgery, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts, USA Received: 14 November 1989; accepted: 17 January 1990
Abstract. Five m e m b e r s of a family of eight, including the father and four daughters, p r e s e n t e d with s y m p t o m s previously attributed to the superior mesenteric artery syndrome. T h e four daughters also had radiographic studies supporting that diagnosis. This familial clustering raises the possibility of a genetic predisposition to this s y m p t o m and radiographic complex.
T h e superior mesenteric artery ( S M A ) s y n d r o m e is a controversial disorder which m a y occasionally occur in isolation, or be identified in individuals with constricting b o d y casts or w h o have u n d e r g o n e recent significant weight loss. T h e i m p o r t a n c e of the spine against which the d u o d e n u m is compressed in these circumstances, rather than a p r i m a r y abnormality of the S M A has b e e n recognized [1-5]. Idiopathic cases are r e p o r t e d [1-2]. We wish to report, to our knowledge, the first familial clustering of this disorder. Cases and methods The propositus of this study is a 17-year-old girl who presented with proximal small bowel obstruction with vomiting and abdominal pain. A review of the family history revealed similar symptoms in
three of her sisters and her father. One daughter experienced early satiety as her only sympton. The four sisters had radiographic imaging consistent with that described in the SMA syndrome [1-4]. The father was deceased and all his medical records were destroyed. The girls' mother reports a history in the father similar to that in her daughters and states that her husband had been treated for an obstruction in the duodenum non operatively. A listing of the family's symptoms and radiographic findings is presented in Table 1. The family members were of average height and otherwise healthy. The 17-year-old daughter had more severe, persistent symptoms and underwent laparotomy with duodenotomy and mobilization of the ligament of Treitz for relief of her obstruction. During surgery, the superior mesenteric artery and vein were noted to impinge upon the duodenum at the site of obstruction. The other three daughters responded adequately to medical therapy (positioning with the left side down or prone). Results T h e radiographic record of the father was not available for our review. U p p e r gastrointestinal ( U G I ) studies in the four affected daughters all revealed a partial obstruction at the junction of the third and fourth portions of the d u o d e n u m with dilatation of the proximal d u o d e n u m (Figs. 1, 2). T h r e e of the daughters were shown to have partial or intermittent d e c o m p r e s s i o n of the dilated
Table 1. Patients symptoms and radiographic findings Family member
Age (years)
Symptoms
Duration of symptoms
Radiographic findings
Treatment
Father
45 (at demise)
Abdominal pain, distention and vomiting (reported by wife)
Many years (reported by wife)
(None available)
Medical (reported by wife)
Daughter
24
Abdominal distention and vomiting
17 years
Medical
Daughter
22
Intermitent abdominal pain, distention, vomiting
Since early childhood
Dilatation of the duodenum to the level of the 2nd. and 3rd. portions with moderate distention (Fig. 1) Partial obstruction at the 3rd. portion of duodenum
Daughter
20
Early satiety
"A few years"
Medical
Daughter
17
Abdominal distention, vomiting
11 years
Mother Son Daughter
48 26 18
Asymptomatic Asymptomatic Asymptomatic
Obstruction of the 3rd. portion of the duodenum Partial obstruction of the 3rd. portion of the duodenum with moderate dilatation (Fig. 2) Normal UGI Normal UGI Normal UGI
Medical
Surgery
C. Ortiz et al.: Familial SMA syndrome
589 Fig.l. Left posterior oblique (LPO) radiograph of the abdomen in the 24-year old daughter following an upper gastrointestinal examination (UGI). There is a vertical extrinsic compression on the third portion of the duodenum causing a partial obstruction. Passage of barium into the jejunum was moderately, but only intermittently, enhanced by positioning in the steep LPO or left lateral decubitus position
Fig.2. Anterior-posterior radiograph of the abdomen in the 17-year-old daughter following an UGI. An obstructive pattern similar to her sister's (Fig. 1) is demonstrated. At fluoroscopy, there was vigorous to and fro peristalsis proximal to the obstruction. Passage of barium into the jejunum was delayed but augmented by positioning on the left side d u o d e n u m in the left lateral decubitus position during fluoroscopy. The ligament of Treitz was in normal position in all four daughters; the remainder of the small bowel was also normal (Figs. 1, 2).
Occasionally operative intervention will be necessary to relieve the obstruction [1, 3].
Discussion
The S M A syndrome is a rare entity when seen outside of certain clinical settings such as significant rapid weight loss or body cast [1-2, 4-5]. Idiopathic cases have been documented [1-2]. We present a family of eight, five of w h o m presumably suffered from the S M A syndrome suggesting the possibility of a genetic predisposition.
In the S M A syndrome, obstruction of the d u o d e n u m is reported to be secondary to compression of the d u o d e n u m as it passes between the superior mesenteric artery and vein anteriorly and the spine posteriorly [1-5]. The radiographic findings are: (1) dilatation of the first, second and third portions of the duodenum; (2) an abrupt vertical filling defect at the level of the superior mesenteric artery; (3) vigorous to and fro peristalsis proximal to the obstruction; (4) delayed passage of small amounts of contrast into the jejunum; and (5) frequently relief of obstruction in the left lateral decubitus or prone positions
[1].
O p e r a t e d cases have demonstrated that the angle between the aorta and the superior mesenteric artery normally ranges f r o m 25 ~to 60 ~but is decreased to 6 ~to 15 ~in the S M A syndrome [3]. Some investigators have suggested that the condition is very infrequent and m a y not exist at all [1, 4]. Others feel that the syndrome exists only in specific situations such as rapid weight loss, exaggerated lumbar lordosis, or in individuals in body casts [4]. In idiopathic cases, non-operative treatment m a y frequently be successful. This consists of gravitational maneuvers such as positioning in the left lateral decubitus position after eating or in the prone knee-chest position.
Conclusion
References 1. Hines JR, Gore RM, Ballentyne GH (1984) Superior mesenteric artery syndrome. Diagnostic criteria and therapeutic approaches. AmJ Surg 148:630 2. Akin JT, Gray SW, Skandalakis JE (1976) Vascular compression of the duodenum: presentation of ten cases and review of the literature. Surgery 79:515 3. Balmaseda MT, Gordon C, Culmingham ML, Clairmont AC (1987) Superior mesenteric artery syndrome after resection of an arteriovenous malformation in the cervical cord. Am J Gastroentero182: 896 4. Gondos B (1977) Duodenal compression defect and the "superior mesentery artery syndrome". Radiology 123:575 5. Vaisman N, Stringer DA, Pencharz P (1989) Functional duodenal obstruction (superior mesenteric artery or cast syndrome) in cerebral palsy. J Parent Enteral Nutrit 13:326 R. H. Cleveland, M. D. Division of Pediatric Radiology Massachusetts General Hospital Boston, MA 02114 USA
Pediatric Radiology 9 Springer-Verlag 1990
Familial superior mesenteric artery syndrome C. O r t i z 1, R. H. C l e v e l a n d I , J. G. B l i c k m a n 1, D. J a r a m i l l o 1 a n d S..H. K i m 2 Divisions of i Pediatric Radiology and 2 Pediatric Surgery, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts, USA Received: 14 November 1989; accepted: 17 January 1990
Abstract. Five m e m b e r s of a family of eight, including the father and four daughters, p r e s e n t e d with s y m p t o m s previously attributed to the superior mesenteric artery syndrome. T h e four daughters also had radiographic studies supporting that diagnosis. This familial clustering raises the possibility of a genetic predisposition to this s y m p t o m and radiographic complex.
T h e superior mesenteric artery ( S M A ) s y n d r o m e is a controversial disorder which m a y occasionally occur in isolation, or be identified in individuals with constricting b o d y casts or w h o have u n d e r g o n e recent significant weight loss. T h e i m p o r t a n c e of the spine against which the d u o d e n u m is compressed in these circumstances, rather than a p r i m a r y abnormality of the S M A has b e e n recognized [1-5]. Idiopathic cases are r e p o r t e d [1-2]. We wish to report, to our knowledge, the first familial clustering of this disorder. Cases and methods The propositus of this study is a 17-year-old girl who presented with proximal small bowel obstruction with vomiting and abdominal pain. A review of the family history revealed similar symptoms in
three of her sisters and her father. One daughter experienced early satiety as her only sympton. The four sisters had radiographic imaging consistent with that described in the SMA syndrome [1-4]. The father was deceased and all his medical records were destroyed. The girls' mother reports a history in the father similar to that in her daughters and states that her husband had been treated for an obstruction in the duodenum non operatively. A listing of the family's symptoms and radiographic findings is presented in Table 1. The family members were of average height and otherwise healthy. The 17-year-old daughter had more severe, persistent symptoms and underwent laparotomy with duodenotomy and mobilization of the ligament of Treitz for relief of her obstruction. During surgery, the superior mesenteric artery and vein were noted to impinge upon the duodenum at the site of obstruction. The other three daughters responded adequately to medical therapy (positioning with the left side down or prone). Results T h e radiographic record of the father was not available for our review. U p p e r gastrointestinal ( U G I ) studies in the four affected daughters all revealed a partial obstruction at the junction of the third and fourth portions of the d u o d e n u m with dilatation of the proximal d u o d e n u m (Figs. 1, 2). T h r e e of the daughters were shown to have partial or intermittent d e c o m p r e s s i o n of the dilated
Table 1. Patients symptoms and radiographic findings Family member
Age (years)
Symptoms
Duration of symptoms
Radiographic findings
Treatment
Father
45 (at demise)
Abdominal pain, distention and vomiting (reported by wife)
Many years (reported by wife)
(None available)
Medical (reported by wife)
Daughter
24
Abdominal distention and vomiting
17 years
Medical
Daughter
22
Intermitent abdominal pain, distention, vomiting
Since early childhood
Dilatation of the duodenum to the level of the 2nd. and 3rd. portions with moderate distention (Fig. 1) Partial obstruction at the 3rd. portion of duodenum
Daughter
20
Early satiety
"A few years"
Medical
Daughter
17
Abdominal distention, vomiting
11 years
Mother Son Daughter
48 26 18
Asymptomatic Asymptomatic Asymptomatic
Obstruction of the 3rd. portion of the duodenum Partial obstruction of the 3rd. portion of the duodenum with moderate dilatation (Fig. 2) Normal UGI Normal UGI Normal UGI
Medical
Surgery
C. Ortiz et al.: Familial SMA syndrome
589 Fig.l. Left posterior oblique (LPO) radiograph of the abdomen in the 24-year old daughter following an upper gastrointestinal examination (UGI). There is a vertical extrinsic compression on the third portion of the duodenum causing a partial obstruction. Passage of barium into the jejunum was moderately, but only intermittently, enhanced by positioning in the steep LPO or left lateral decubitus position
Fig.2. Anterior-posterior radiograph of the abdomen in the 17-year-old daughter following an UGI. An obstructive pattern similar to her sister's (Fig. 1) is demonstrated. At fluoroscopy, there was vigorous to and fro peristalsis proximal to the obstruction. Passage of barium into the jejunum was delayed but augmented by positioning on the left side d u o d e n u m in the left lateral decubitus position during fluoroscopy. The ligament of Treitz was in normal position in all four daughters; the remainder of the small bowel was also normal (Figs. 1, 2).
Occasionally operative intervention will be necessary to relieve the obstruction [1, 3].
Discussion
The S M A syndrome is a rare entity when seen outside of certain clinical settings such as significant rapid weight loss or body cast [1-2, 4-5]. Idiopathic cases have been documented [1-2]. We present a family of eight, five of w h o m presumably suffered from the S M A syndrome suggesting the possibility of a genetic predisposition.
In the S M A syndrome, obstruction of the d u o d e n u m is reported to be secondary to compression of the d u o d e n u m as it passes between the superior mesenteric artery and vein anteriorly and the spine posteriorly [1-5]. The radiographic findings are: (1) dilatation of the first, second and third portions of the duodenum; (2) an abrupt vertical filling defect at the level of the superior mesenteric artery; (3) vigorous to and fro peristalsis proximal to the obstruction; (4) delayed passage of small amounts of contrast into the jejunum; and (5) frequently relief of obstruction in the left lateral decubitus or prone positions
[1].
O p e r a t e d cases have demonstrated that the angle between the aorta and the superior mesenteric artery normally ranges f r o m 25 ~to 60 ~but is decreased to 6 ~to 15 ~in the S M A syndrome [3]. Some investigators have suggested that the condition is very infrequent and m a y not exist at all [1, 4]. Others feel that the syndrome exists only in specific situations such as rapid weight loss, exaggerated lumbar lordosis, or in individuals in body casts [4]. In idiopathic cases, non-operative treatment m a y frequently be successful. This consists of gravitational maneuvers such as positioning in the left lateral decubitus position after eating or in the prone knee-chest position.
Conclusion
References 1. Hines JR, Gore RM, Ballentyne GH (1984) Superior mesenteric artery syndrome. Diagnostic criteria and therapeutic approaches. AmJ Surg 148:630 2. Akin JT, Gray SW, Skandalakis JE (1976) Vascular compression of the duodenum: presentation of ten cases and review of the literature. Surgery 79:515 3. Balmaseda MT, Gordon C, Culmingham ML, Clairmont AC (1987) Superior mesenteric artery syndrome after resection of an arteriovenous malformation in the cervical cord. Am J Gastroentero182: 896 4. Gondos B (1977) Duodenal compression defect and the "superior mesentery artery syndrome". Radiology 123:575 5. Vaisman N, Stringer DA, Pencharz P (1989) Functional duodenal obstruction (superior mesenteric artery or cast syndrome) in cerebral palsy. J Parent Enteral Nutrit 13:326 R. H. Cleveland, M. D. Division of Pediatric Radiology Massachusetts General Hospital Boston, MA 02114 USA
