Wilkie’s syndrome: a case report of favourable minimally invasive surgery

Wilkie’s syndrome: a case report of favourable minimally invasive surgery Wilkie’s syndrome, or superior mesenteric artery syndrome (SMAS), is a rare clinical entity caused by compression of the third portion of the duodenum between the abdominal aorta and superior mesenteric artery, leading to duodenal obstruc- tion and severe malnutrition. The authors report a case of a female patient with years of chronic intestinal obstruction with abdominal pain, vomits and weight loss. Contrast intestinal series showed dilation of stomach and duodenum. Abdominal computed tomography study revealed findings compatible with SMAS. After initial nutritional support, she was successfully treated by laparoscopic duodenojejunostomy. Surgical treatment of SMAS may be necessary in most cases with chronic symptoms or conservative treatment failure. A minimally invasive approach can be considered a safe surgical option with favourable outcomes. Clinical details, diagnostic studies and treatment are discussed. or refractory symptoms [3, 7]. A minimally invasive approach can INTRODUCTION be considered a good approach with favourable outcomes [3, 8]. Wilkie’s syndrome, or superior mesenteric artery syndrome (SMAS), is a rare clinical condition characterized by partial or complete duodenal obstruction [1, 2]. It is caused by a narrowed CASE REPORT aortomesenteric angle that leads to compression of the third portion of duodenum between the abdominal aorta (AA) and A 39-year-old Caucasian female patient presented to a General surgery outpatient clinic with complaints of epigastric pain, nau- superior mesenteric artery (SMA) [2]. The syndrome was first reported in 1861 by Von Rokitansky and was studied afterwards sea, vomiting and weight loss for more than 5 years. The pain was worse after eating and with supine position. Vomiting was by Wilkie who published the largest series of cases in 1927 [2–4]. The main symptoms of this condition are postprandial usually initiated 3–4 h after meals and consisted of undigested food. She had no significant prior medical history. On examin- abdominal pain, early satiety, vomits, weight loss and malnutri- tion [3–5]. Diagnosis of SMAS is challenging but may be suspected ation, the patient was extremely emaciated, with normal vital signs but with a distended abdomen and fullness over the epi- based on clinical presentation and then supported by imaging studies [1, 3, 6]. SMAS may be treated medically and/or surgically gastrium. She had already done an upper endoscopy that only showed gastric stasis. Abdominal ultrasound revealed a reduced [4]. Conservative management consists mainly of nutritional sup- port [1, 6]. Surgical options are indicated for patients with chronic angle between the AA and SMA (Fig. 1). An upper GI contrast Received: January 18, 2018. Accepted: January 30, 2018 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2018. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com Downloaded from https://academic.oup.com/jscr/article-abstract/2018/2/rjy027/4859682 by Ed 'DeepDyve' Gillespie user on 16 March 2018 2 S. Catarino Santos et al. Figure 1: Abdominal ultrasound—reduced angle between abdominal aorta and superior mesenteric artery. Figure 3: CT scan—sagittal CT image of reduced angle between AAA and SMA. Figure 4: CT scan—axial CT image of obstruction of the third portion of duode- num between AAA and SMA. (4–5 mm) between these two arteries (Figs 3 and 4). These findings were suggestive of an aortomesenteric clamp. Hence, based on known findings, the diagnosis of Wilkie’s syndrome was established. Initially, conservative management was adopted with some Figure 2: Contrasted intestinal series—distended stomach with delayed gastric improvement of patient’s nutrition condition. However, as emptying and reduced passage of contrast at the third portion of duodenum. symptoms persisted, surgical treatment was recommended. Our surgical approach was a laparoscopic duodenojejunostomy study was requested, which revealed a distended stomach with with a latero-lateral stapled anastomosis between jejunum delayed gastric emptying and lagging of contrast at the third por- (30 cm from Treitz’s angle) and the second portion of the duo- tion of the duodenum (Fig. 2). Abdominal computed tomography denum (Figs 5–9). The patient recovered with no pain but with (CT) scan was then performed. It demonstrated a severe disten- a delay in diet acceptance. An upper GI contrast study was per- sion of the stomach and proximal portions of the duodenum with formed on the fifth post-operative day revealing a distended constriction of the third part of the duodenum between the AA stomach with gastric emptying delay, but with unobstructed and SMA, with a reduced angle (11°) and shortened distance anastomosis, therefore without stenosis or leaks (Fig. 10). She Downloaded from https://academic.oup.com/jscr/article-abstract/2018/2/rjy027/4859682 by Ed 'DeepDyve' Gillespie user on 16 March 2018 Wilkie’s syndrome: a case report 3 Figure 5: Surgery—initial laparoscopic view with distended stomach and prox- imal portions of duodenum. Figure 8: Surgery—laparoscopic hand-sew closure of the anastomosis. Figure 6: Surgery—laparoscopic visualization of the second and third portion of duodenum. Figure 9: Surgery—final laparoscopic surgical view of laparoscopic duodenojejunostomy. [2, 8], is a rare entity defined as a compression of the third por- tion of the duodenum between the SMA and AA leading to par- tial or complete duodenum obstruction [5, 6, 9]. The normal aortomesenteric angle and distance are 25–60° and 10–28 mm, respectively [6]. However, in SMAS this angle is narrowed to <25° and the distance is shortened to < 8mm [7, 10]. Several factors are associated with SMAS, mainly marked weight loss as a consequence of other diseases (cancer, bariatric surgery, chronic infections, severe burns) but may also be congenital such as shorten Treitz’s ligament or abnormal origin of the SMA, or associated with surgical interventions that distorts the anatomy as scoliosis correction or esophagectomy [3, 4, 6]. SMAS has an estimated prevalence in the general popula- Figure 7: Surgery—laparoscopic stapled anastomosis between jejunum and tion that varies between 0.013 and 0.3% [4, 6], and it most com- second portion of duodenum. monly affects females between 10 and 40 years of age [1, 10]. Its manifestation is complex, including postprandial epigastric was discharged home after 8 days with liquid diet and digestive pain, nausea, vomiting, early satiety, weight loss and malnutri- transit restored. After 3 months of follow-up, the patient gained tion [3–5]. SMAS may present as an acute obstruction or it may some weight and remained asymptomatic. have an insidious onset with chronic symptoms [1, 6, 9]. The diagnosis requires a high degree of suspicion [3, 8]. The low index of suspicious can lead to delays in diagnosis resulting in DISCUSSION a chronic course of symptoms [1, 6]. Several imaging studies SMAS, also known as Wilkie’s syndrome, mesenteric duodenal such as ultrasonography, CT scan, contrast digestive series and compression syndrome, chronic duodenal ileus or cast syndrome endoscopy may be necessary to confirm the diagnosis [2, 3, 8]. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/2/rjy027/4859682 by Ed 'DeepDyve' Gillespie user on 16 March 2018 4 S. Catarino Santos et al. Starley in 1910, and it is the current preferred treatment of SMAS with a success rate that reaches 90% [3, 8]. All these proce- dures can be done by a minimally invasive approach although experience is limited [8]. In 1998, Gersin and Heniford first described a laparoscopic duodenojejunostomy and this proced- ure spread as a viable surgical alternative, without the risks and morbidity associated with an open surgery [3]. The present case report emphasizes that a minimally invasive surgery may be a relatively easy approach, safe and effective in the definitive treatment of SMAS. CONFLICT OF INTEREST STATEMENT None declared. FUNDING None. REFERENCES 1. Bohanon FJ, Nunez Lopez O, Graham BM, Griffin LW, Radhakrishnan RS. A case series of laparoscopic duodenoje- juostomy for the treatment of pediatric superior mesenteric artery syndrome. Int J Surg Res 2016;2016:1–5. 2. Kumar N, Rehmani B. Wilkie’s syndrome: effect or cause of severe malnutrition—an unusual case report. Int J Surg 2013;29:1. 3. Barchi LC. Favorable minimal invasive surgery in the treat- Figure 10: Post-operative contrasted intestinal series—delayed gastric emptying ment of superior mesenteric artery syndrome: case report. with without stenosis or leaks of the anastomosis. Int J Surg Case Rep 2016;29:223–6. 4. Fisahn C. Unusual manifestation of superior mesenteric CT scan and magnetic resonance angiography enable visualiza- artery syndrome in a female patient: a case presentation. tion of the vascular compression of the duodenum and precise Cureus 2016;8:e800. measurement of aortomesenteric angle and distance [1, 6]. 5. Felton BM, White JM, Racine MA. An uncommon case of Current therapy of SMAS consists of medical and/or surgical abdominal pain: superior mesenteric artery syndrome. West treatment [2, 4]. Conservative therapy includes nutritional sup- J Emerg Med 2012;13:501–2. port, left-lateral decubitus positioning, prokinetic and anti-reflux 6. Salem A, Al Ozaibi L, Nassif SMM, Osman RAGS, Al Abed medications and fluid resuscitation [1, 6]. It is conviction of most NM, Badri FM. Superior mesenteric artery syndrome: a diag- of the authors that a trial of medical treatment should be nosis to be kept in mind (Case report and literature review). attempted before any surgical intervention [3]. However, there is Int J Surg Case Rep 2017;34:84–6. neither clear time limit nor the long-term outcome of conserva- 7. Oliva-Fonte C, Fernández-Rey CL, Pereda-Rodrígyez J, tive therapy [1]. Failure of conservative medical therapy or recur- González-Fernández AM. Wilkie’s syndrome. Rev Esp Enferm rent episodes of thedisease areindications forsurgical Dig 2017;109:62–3. treatment [2, 3, 7]. Surgical options include duodenojejunost- 8. Lorentziadis ML. Wilke’s syndrome. A rare cause of duo- omy, gastrojejunostomy and Strong’s procedure (mobilization of denal obstruction. Ann Gastrenterol 2011;24:59–61. the duodenum by division of the Treitz’s ligament) [1, 8]. 9. Reece K, Day R, Welch J. Superior mesenteric artery syn- Strong’s procedure is mostly indicated in infants, but it has a drome with abdominal compartment syndrome. Case Rep high failure rate [8, 9]. Gastrojejunostomy also presents a high Emerg Med 2016;2016:Article ID 7809281, 4 pages. risk of failure because it does not relieve the proximal duodenal 10. Cardarelli-Leite L, Velloni FG, Salvadori PS, Lemos MD, obstruction and there is increased risk of peptic stomal ulcer- D’Ippolio G. Abdominal vascular syndromes: characteristic ation [1, 3, 8]. Duodenojejunostomy was first introduced by imaging findings. Radiol Bras 2016;49:257–63. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/2/rjy027/4859682 by Ed 'DeepDyve' Gillespie user on 16 March 2018 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Surgical Case Reports Oxford University Press

Wilkie’s syndrome: a case report of favourable minimally invasive surgery

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Abstract

Wilkie’s syndrome, or superior mesenteric artery syndrome (SMAS), is a rare clinical entity caused by compression of the third portion of the duodenum between the abdominal aorta and superior mesenteric artery, leading to duodenal obstruc- tion and severe malnutrition. The authors report a case of a female patient with years of chronic intestinal obstruction with abdominal pain, vomits and weight loss. Contrast intestinal series showed dilation of stomach and duodenum. Abdominal computed tomography study revealed findings compatible with SMAS. After initial nutritional support, she was successfully treated by laparoscopic duodenojejunostomy. Surgical treatment of SMAS may be necessary in most cases with chronic symptoms or conservative treatment failure. A minimally invasive approach can be considered a safe surgical option with favourable outcomes. Clinical details, diagnostic studies and treatment are discussed. or refractory symptoms [3, 7]. A minimally invasive approach can INTRODUCTION be considered a good approach with favourable outcomes [3, 8]. Wilkie’s syndrome, or superior mesenteric artery syndrome (SMAS), is a rare clinical condition characterized by partial or complete duodenal obstruction [1, 2]. It is caused by a narrowed CASE REPORT aortomesenteric angle that leads to compression of the third portion of duodenum between the abdominal aorta (AA) and A 39-year-old Caucasian female patient presented to a General surgery outpatient clinic with complaints of epigastric pain, nau- superior mesenteric artery (SMA) [2]. The syndrome was first reported in 1861 by Von Rokitansky and was studied afterwards sea, vomiting and weight loss for more than 5 years. The pain was worse after eating and with supine position. Vomiting was by Wilkie who published the largest series of cases in 1927 [2–4]. The main symptoms of this condition are postprandial usually initiated 3–4 h after meals and consisted of undigested food. She had no significant prior medical history. On examin- abdominal pain, early satiety, vomits, weight loss and malnutri- tion [3–5]. Diagnosis of SMAS is challenging but may be suspected ation, the patient was extremely emaciated, with normal vital signs but with a distended abdomen and fullness over the epi- based on clinical presentation and then supported by imaging studies [1, 3, 6]. SMAS may be treated medically and/or surgically gastrium. She had already done an upper endoscopy that only showed gastric stasis. Abdominal ultrasound revealed a reduced [4]. Conservative management consists mainly of nutritional sup- port [1, 6]. Surgical options are indicated for patients with chronic angle between the AA and SMA (Fig. 1). An upper GI contrast Received: January 18, 2018. Accepted: January 30, 2018 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2018. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com Downloaded from https://academic.oup.com/jscr/article-abstract/2018/2/rjy027/4859682 by Ed 'DeepDyve' Gillespie user on 16 March 2018 2 S. Catarino Santos et al. Figure 1: Abdominal ultrasound—reduced angle between abdominal aorta and superior mesenteric artery. Figure 3: CT scan—sagittal CT image of reduced angle between AAA and SMA. Figure 4: CT scan—axial CT image of obstruction of the third portion of duode- num between AAA and SMA. (4–5 mm) between these two arteries (Figs 3 and 4). These findings were suggestive of an aortomesenteric clamp. Hence, based on known findings, the diagnosis of Wilkie’s syndrome was established. Initially, conservative management was adopted with some Figure 2: Contrasted intestinal series—distended stomach with delayed gastric improvement of patient’s nutrition condition. However, as emptying and reduced passage of contrast at the third portion of duodenum. symptoms persisted, surgical treatment was recommended. Our surgical approach was a laparoscopic duodenojejunostomy study was requested, which revealed a distended stomach with with a latero-lateral stapled anastomosis between jejunum delayed gastric emptying and lagging of contrast at the third por- (30 cm from Treitz’s angle) and the second portion of the duo- tion of the duodenum (Fig. 2). Abdominal computed tomography denum (Figs 5–9). The patient recovered with no pain but with (CT) scan was then performed. It demonstrated a severe disten- a delay in diet acceptance. An upper GI contrast study was per- sion of the stomach and proximal portions of the duodenum with formed on the fifth post-operative day revealing a distended constriction of the third part of the duodenum between the AA stomach with gastric emptying delay, but with unobstructed and SMA, with a reduced angle (11°) and shortened distance anastomosis, therefore without stenosis or leaks (Fig. 10). She Downloaded from https://academic.oup.com/jscr/article-abstract/2018/2/rjy027/4859682 by Ed 'DeepDyve' Gillespie user on 16 March 2018 Wilkie’s syndrome: a case report 3 Figure 5: Surgery—initial laparoscopic view with distended stomach and prox- imal portions of duodenum. Figure 8: Surgery—laparoscopic hand-sew closure of the anastomosis. Figure 6: Surgery—laparoscopic visualization of the second and third portion of duodenum. Figure 9: Surgery—final laparoscopic surgical view of laparoscopic duodenojejunostomy. [2, 8], is a rare entity defined as a compression of the third por- tion of the duodenum between the SMA and AA leading to par- tial or complete duodenum obstruction [5, 6, 9]. The normal aortomesenteric angle and distance are 25–60° and 10–28 mm, respectively [6]. However, in SMAS this angle is narrowed to <25° and the distance is shortened to < 8mm [7, 10]. Several factors are associated with SMAS, mainly marked weight loss as a consequence of other diseases (cancer, bariatric surgery, chronic infections, severe burns) but may also be congenital such as shorten Treitz’s ligament or abnormal origin of the SMA, or associated with surgical interventions that distorts the anatomy as scoliosis correction or esophagectomy [3, 4, 6]. SMAS has an estimated prevalence in the general popula- Figure 7: Surgery—laparoscopic stapled anastomosis between jejunum and tion that varies between 0.013 and 0.3% [4, 6], and it most com- second portion of duodenum. monly affects females between 10 and 40 years of age [1, 10]. Its manifestation is complex, including postprandial epigastric was discharged home after 8 days with liquid diet and digestive pain, nausea, vomiting, early satiety, weight loss and malnutri- transit restored. After 3 months of follow-up, the patient gained tion [3–5]. SMAS may present as an acute obstruction or it may some weight and remained asymptomatic. have an insidious onset with chronic symptoms [1, 6, 9]. The diagnosis requires a high degree of suspicion [3, 8]. The low index of suspicious can lead to delays in diagnosis resulting in DISCUSSION a chronic course of symptoms [1, 6]. Several imaging studies SMAS, also known as Wilkie’s syndrome, mesenteric duodenal such as ultrasonography, CT scan, contrast digestive series and compression syndrome, chronic duodenal ileus or cast syndrome endoscopy may be necessary to confirm the diagnosis [2, 3, 8]. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/2/rjy027/4859682 by Ed 'DeepDyve' Gillespie user on 16 March 2018 4 S. Catarino Santos et al. Starley in 1910, and it is the current preferred treatment of SMAS with a success rate that reaches 90% [3, 8]. All these proce- dures can be done by a minimally invasive approach although experience is limited [8]. In 1998, Gersin and Heniford first described a laparoscopic duodenojejunostomy and this proced- ure spread as a viable surgical alternative, without the risks and morbidity associated with an open surgery [3]. The present case report emphasizes that a minimally invasive surgery may be a relatively easy approach, safe and effective in the definitive treatment of SMAS. CONFLICT OF INTEREST STATEMENT None declared. FUNDING None. REFERENCES 1. Bohanon FJ, Nunez Lopez O, Graham BM, Griffin LW, Radhakrishnan RS. A case series of laparoscopic duodenoje- juostomy for the treatment of pediatric superior mesenteric artery syndrome. Int J Surg Res 2016;2016:1–5. 2. Kumar N, Rehmani B. Wilkie’s syndrome: effect or cause of severe malnutrition—an unusual case report. Int J Surg 2013;29:1. 3. Barchi LC. Favorable minimal invasive surgery in the treat- Figure 10: Post-operative contrasted intestinal series—delayed gastric emptying ment of superior mesenteric artery syndrome: case report. with without stenosis or leaks of the anastomosis. Int J Surg Case Rep 2016;29:223–6. 4. Fisahn C. Unusual manifestation of superior mesenteric CT scan and magnetic resonance angiography enable visualiza- artery syndrome in a female patient: a case presentation. tion of the vascular compression of the duodenum and precise Cureus 2016;8:e800. measurement of aortomesenteric angle and distance [1, 6]. 5. Felton BM, White JM, Racine MA. An uncommon case of Current therapy of SMAS consists of medical and/or surgical abdominal pain: superior mesenteric artery syndrome. West treatment [2, 4]. Conservative therapy includes nutritional sup- J Emerg Med 2012;13:501–2. port, left-lateral decubitus positioning, prokinetic and anti-reflux 6. Salem A, Al Ozaibi L, Nassif SMM, Osman RAGS, Al Abed medications and fluid resuscitation [1, 6]. It is conviction of most NM, Badri FM. Superior mesenteric artery syndrome: a diag- of the authors that a trial of medical treatment should be nosis to be kept in mind (Case report and literature review). attempted before any surgical intervention [3]. However, there is Int J Surg Case Rep 2017;34:84–6. neither clear time limit nor the long-term outcome of conserva- 7. Oliva-Fonte C, Fernández-Rey CL, Pereda-Rodrígyez J, tive therapy [1]. Failure of conservative medical therapy or recur- González-Fernández AM. Wilkie’s syndrome. Rev Esp Enferm rent episodes of thedisease areindications forsurgical Dig 2017;109:62–3. treatment [2, 3, 7]. Surgical options include duodenojejunost- 8. Lorentziadis ML. Wilke’s syndrome. A rare cause of duo- omy, gastrojejunostomy and Strong’s procedure (mobilization of denal obstruction. Ann Gastrenterol 2011;24:59–61. the duodenum by division of the Treitz’s ligament) [1, 8]. 9. Reece K, Day R, Welch J. Superior mesenteric artery syn- Strong’s procedure is mostly indicated in infants, but it has a drome with abdominal compartment syndrome. Case Rep high failure rate [8, 9]. Gastrojejunostomy also presents a high Emerg Med 2016;2016:Article ID 7809281, 4 pages. risk of failure because it does not relieve the proximal duodenal 10. Cardarelli-Leite L, Velloni FG, Salvadori PS, Lemos MD, obstruction and there is increased risk of peptic stomal ulcer- D’Ippolio G. Abdominal vascular syndromes: characteristic ation [1, 3, 8]. Duodenojejunostomy was first introduced by imaging findings. Radiol Bras 2016;49:257–63. Downloaded from https://academic.oup.com/jscr/article-abstract/2018/2/rjy027/4859682 by Ed 'DeepDyve' Gillespie user on 16 March 2018

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Journal of Surgical Case ReportsOxford University Press

Published: Feb 1, 2018

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