World Journal of Pharmaceutical Research SJIF Impact Factor 7.523

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1 SJIF Impact Factor Volume 7, Issue 1, Case Report ISSN INTESTINAL OBSTRUCTION IN AN 18-YEAR-OLD FEMALE WITH SUPERIOR MESENTERIC ARTERY SYNDROME: CASE REPORT AND LITERATURE REVIEW 1* Hibah A. Almahdi, 2 Nada H. Mahmoud, 3 Sultan Tawfiq and 4 Fatema K. Al-Madani 1,2,4 Medical Intern, Taibah University. 3 Senior Registrar General Surgery, King Fahad Hospital. Article Received on 16 Nov. 2017, Revised on 07 Dec. 2017, Accepted on 28 Dec. 2017, DOI: /wjpr *Corresponding Author Dr. Hibah A. Almahdi Medical Intern, Taibah University. ABSTRACT Introduction: Superior mesenteric artery (SMA) syndrome is characterized by compressed third part of duodenum between SMA and the abdominal aorta. According to literature review, the incidence of SMA syndrome was estimated to be %. Case report: An 18-year-old female patient was presented to King Fahad Hospital with 15 days history of abdominal pain associated with recurrent postprandial vomiting of gastric content and absolute constipation, preceded by rapid weight loss of 12 kilograms. Her vitals were stable. Laboratory investigations were normal except for Lactate dehydrogenase and alanine aminotransferase which were slightly high. Esophagogastroduodenoscopy showed stenosis. Enhanced CT abdomen and pelvis showed distended stomach and duodenum proximal to the narrowed 3 rd part of duodenum. Patient was treated conservatively for 2 weeks and discharged in a good condition. Discussion: SMA syndrome is a rare vascular cause of small bowel obstruction. Several factors play a rule in narrowing the aorto-mesentric angle. Significant rapid loss of weight is the commonest cause. Conservative approach should be attempted first in managing SMA syndrome. If conservative management fails, surgical management is indicated. Conclusion: SMA syndrome is a diagnosis that should be kept in mind in cases of high intestinal obstruction especially with young patients who have history of recent weight loss. Enhanced CT is good to establish the diagnosis of SMA syndrome. Sometimes mechanical obstruction causes should be ruled out by Esophagogastroduodenoscopy. KEYWORDS: Superior mesenteric artery syndrome, Intestinal obstruction, rapid weight Vol 7, Issue 1,

2 loss, absolute constipation, conservative. INTRODUCTION Superior mesenteric artery (SMA) syndrome is a rare life threatening vascular cause of small bowel obstruction. It is also known as Cast syndrome, Wilkie syndrome, arterio-mesenteric duodenal obstruction, and chronic duodenal ileus. SMA syndrome is characterized by compressed third part of duodenum between the SMA anteriorly and the abdominal aorta posteriorly [1]. Rotikansky was the first to describe SMA syndrome in 1842, while the first case series of 75 patients with SMA syndrome was published by Wilkie in 1927 [1]. However, the incidence of SMA syndrome has been estimated to be % of the general population [2]. 75% of reported SMA syndrome cases were between years of age, predominantly females in a ratio of 3:2 with no racial differences [3]. In this study, we are presenting a case of SMA syndrome that was successfully treated in King Fahad Hospital in order to increase awareness of SMA syndrome and its presentation. CASE REPORT A 18-year-old female patient was transferred to emergency department of King Fahad Hospital due to 15 days history of progressive epigastric pain associated with recurrent postprandial vomiting of copious gastric content occasionally bilious, absolute constipation and episodes of carpo-pedal spasm. Patient initially received IV fluids and antibiotic at a local hospital, but showed no improvement. However, these symptoms were preceded by intentional rapid weight loss of 12 kilograms. Based on her medical records, patient is medically free, had no previous surgeries and no similar attack. On examination, patient looked ill and dehydrated. Her vitals were within the normal range. Weight, 52.5 kg, height, 1.67 m, and body mass index (BMI), 18.8 kg/m 2. The abdominal examination revealed a soft distended abdomen with epigastric tenderness. Laboratory investigations (complete blood count, urinalysis, renal and liver function) were normal; Lactate dehydrogenase and alanine aminotransferase were slightly high (354 U/L, 59 U/L, respectively). Esophagogastroduodenoscopy (EGD) showed stenosis due to extra- luminal compression. Thus, enhanced CT abdomen and pelvis was performed as soon as possible. The CT scan showed distended stomach and duodenum proximal to the narrowed segment of the 3 rd part of duodenum which is seen compressed between the SMA and the aorta (Fig.1-2-3). Both clinical picture and radiological findings strongly suggested the diagnosis of SMA syndrome and the patient was managed accordingly. A nasogastric tube was placed for Vol 7, Issue 1,

3 gastric decompression, patient kept NPO, received I.V fluids and electrolyte replacement. Later on she started oral intake of nutritious liquids gradually and advised to take left lateral decubitus, prone, or the knee-chest position after meals. Patient slowly improved and symptoms were relived. Two weeks later, after gaining some weight she was discharged on multi-vitamins tablets and dietary advice. The patient did not come back for follow up. DISCUSSION At the level of L1, the SMA arise from the abdominal aorta anteriorly. It goes downward ventrally and laterally, wrapped by mesenteric fat pad, crossing the third part of duodenum [4]. Normally, the angle between SMA and abdominal aorta is almost 45 (range of ). Several factors play a rule in narrowing the aorto-mesentric angle. Congenital anomalies like short ligament of Treitz, duodenal malrotation or unusually low origin of the SMA. Acquired factors like rapid loss of weight due to debilitating conditions, dietary disorders or post bariatric surgeries, peritoneal adhesions, abdominal aortic aneurysm repair, proctocolectomy, ileoanal pouch anastomosis, Nissen fundoplication, vertebral fractures, spinal traction, increased lordosis and corrective scoliosis surgery [5,6]. In this case, the patient reported an intentional rapid weight loss of 12 kilograms by excessive dieting. Diagnosis of SMA syndrome requires a high degree of suspicion. Although most of SMA syndrome patients present with chronic postprandial abdominal pain, distension, vomiting, nausea and weight loss due to avoiding food, some of them may present in acute form of high intestinal obstruction [3]. As in our patient, even though SMA syndrome develops gradually, the presenting symptoms may develop abruptly. Due to nonspecific presentation, the diagnosis of SMA syndrome can be delayed. Sever symptoms usually lead to anorexia and weight loss, worsening the condition of SMA syndrome, creating a vicious cycle. The diagnosis of SMA syndrome is usually established by radiographic studies in 95% of cases [7]. Uppergastrointestinal radiography, computed tomography (CT) or CT angiography, magnetic resonance (MR) angiography, conventional angiography, ultrasonography (US), and endoscopy have all been used in diagnosing SMA syndrome [8]. In our patient, after performing EGD that showed third part duodenal stenosis due to external compression, the diagnosis was established by enhanced CT scan. However, it has been reported that CT angiography is the gold standard for diagnosis of SMA syndrome since it shows the obstruction accurately, measures the aorto-mesentric angel precisely and rule out other causes of duodenal obstruction [4,9]. In 1984, Hines et al established radiographic criteria for diagnosing SMA syndrome: (1) dilatation of the 1st and 2nd portions of the duodenum, with Vol 7, Issue 1,

4 or without gastric dilatation. (2) abrupt vertical and oblique compression of the mucosal folds. (3) anti-peristaltic flow of barium proximal to the obstruction producing to-and-fro movements. (4) delay in transit of 4 to 6 hours through the gastroduodenal region. (5) relief of obstruction when the patient is placed in a position that diminish the drag of the small bowel mesentery (the left lateral decubitus, prone, or the kneechest position) [10]. Once the diagnosis is established, management can be either by conservative or surgical approach. Starting with the least invasive, therefore, conservative approach should be attempted first unless there was local pathology requiring urgent surgical exploration. Acute management usually focus on gastric decompression through nasogastric tube, adequate flued resuscitation, nutritional support by enteral or post pyloric feeding when handled followed by oral feeding when possible. The key for treating SMA syndrome is by regaining the fat pad in the aorto-mesentric angle that reverses the whole pathology. 83% of SMA syndrome patients improve slowly by conservative management, as happened in our patient. Some studies report that SMA syndrome patients do well with high caloric nutritional support through both enteral and parenteral routes [11,12]. Anyhow, when conservative management fails or chronic refractory gastroparesis persists, surgical management is indicated. Surgically, patients can be treated either by bypass procedure or mobilization of the duodenum. Multiple surgical options that have been reported such as duodenojejunostomy, duodenoduodenostomy, gastrojejunostomy and Strong s operation (duodenal mobilization and division of the ligament of Treitz). However, researchers suggest that when surgical treatment is unavoidable, duodenojejunostomy is the operation of choice with a success rate of 90%. Gastrojejunostomy is not used anymore due to associated complications like blind loop syndrome, gastric bile reflux and gastric ulceration. Much uncertainty still exists about the timing of surgical treatment. Choosing the surgical procedure depends on the clinical situation and the surgeon himself [4,13,14]. CONCLUSION SMA syndrome is a rare condition with non-specific presentation that requires a high degree of suspicion to diagnose it. Therefore, SMA syndrome is a diagnosis that should be kept in mind in cases of high intestinal obstruction especially with patients presenting with history of postprandial symptoms or recent weight loss. Enhanced CT is good to establish the diagnosis of SMA syndrome. Sometimes intra-luminal obstruction causes should be ruled out by (EGD). Vol 7, Issue 1,

5 More broadly, research is needed to evaluate the long term outcomes of both conservative and surgical approaches in managing SMA syndrome. Figures Figure1: Axial enhanced CT scans of the abdomen shows duodenal compression between the superior mesenteric artery (SMA) and abdominal aorta. Figure 2: Coronal enhanced computed tomography scan of the abdomen shows dilated stomach and proximal duodenum. Vol 7, Issue 1,

6 Figure 3: Sagittal enhanced computed tomography scan of the abdomen shows decreased Aorto- mesenteric angel. Funding: None Conflict of interest: None declared REFERENCES 1. Motility G, Disorders FB. Superior Mesenteric Artery Syndrome Ylinen P, Kinnunen J, Hockerstedt K. Superior mesenteric artery syndrome. A follow-up study of 16 operated patients. J Clin Gastroenterol. 1989; 11(4): Welsch T, Büchler MW, Kienle P. Recalling Superior Mesenteric Artery Syndrome. 2007; doi: / Mandarry MT, Zhao L, Zhang C, Wei ZQ. A comprehensive review of superior mesenteric artery syndrome. 2010; doi: /s y. 5. Ahmed AR, Taylor I. Superior mesenteric artery syndrome. 1997; 778(February): Jain R. Superior Mesenteric Artery Syndrome Hines JR, Gore RM, Ballantyne GH. Superior mesenteric artery syndrome. Am J Surg. 2017; 148(5): doi: / (84) Yakan S, Cal C, Kaplan H. Superior Mesenteric Artery Syndrome : A Rare Cause of Intestinal Obstruction. Diagnosis and Surgical Management. 2013; 75(April): doi: /s x. Vol 7, Issue 1,

7 9. Madhab G. Superior Mesenteric Artery Syndrome : A Rare Mimic of Common Causes of Upper Gastrointestinal Obstruction Hlnas JR. Diagnostic Criteria and Therapeutic Approaches. 1861: L F, H C, W W, A H, C M. Superior mesenteric artery syndrome : diagnosis and treatment from the gastroenterologist s view. 2002; Biank V, Werlin S. Superior Mesenteric Artery Syndrome in Children : A 20-Year Experience. 2006; 53226(May): Kumar R, Jaiswal G, Bhargava A, Kundu J. Superior Mesenteric Artery Syndrome : Diagnosis and Management. 2016; 14(3): Johanna E, Rodríguez O De, Hernández-villegas AC, Serralde-zúñiga AE, Luz A. Nutrición Hospitalaria Nota clínica The two sides of superior mesenteric artery syndrome treatment : conservative or surgical. 2017; 34(4): Vol 7, Issue 1,