Journal of Pediatric Surgery 49 (2014) 1533–1535

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Laparoscopic Ladd's procedure for superior mesenteric artery syndrome☆ Mohammad Alsulaimy, Jun Tashiro, Eduardo A. Perez, Juan E. Sola ⁎ Division of Pediatric Surgery, DeWitt Daughtry Family Department of Surgery, Leonard M. Miller School of Medicine, University of Miami, Miami, FL, USA

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Article history: Received 22 May 2014 Received in revised form 15 July 2014 Accepted 16 July 2014 Key words: Superior mesenteric artery syndrome Laparoscopy Adolescent

a b s t r a c t Superior mesenteric artery (SMA) syndrome is an uncommon condition where the third portion of the duodenum is compressed and obstructed between the SMA and the aorta. An otherwise healthy 17-year-old female presented with a 2-month history of postprandial abdominal pain and weight loss. Upper gastrointestinal (UGI) series demonstrated SMA obstruction of the third portion of the duodenum. Despite nasojejunal tube feedings over 4 months with appropriate weight gain, symptoms continued and repeat UGI demonstrated persistent SMA syndrome. A laparoscopic Ladd's procedure served as definitive treatment. The steps of the procedure include mobilization of the Ligament of Treitz, mobilization of the right colon, complete derotation of the duodenum, delivery of the small bowel to the right upper quadrant, and appendectomy. Following the procedure, a postoperative UGI showed complete resolution of SMA compression of the duodenum. The patient had an uneventful postoperative course with immediate resolution of symptoms. She was discharged home tolerating a regular diet. © 2014 Elsevier Inc. All rights reserved.

Superior mesenteric artery (SMA) syndrome is a rare cause of proximal intestinal obstruction. It is also referred to as Wilkie syndrome, Cast syndrome, arteriomesenteric duodenal obstruction, and chronic duodenal ileus [1,2]. In these patients, the third portion of the duodenum is compressed and obstructed as a result of narrowing of the space between the SMA and aorta. There are a number of factors that result in narrowing of the space between the SMA and aorta. The most common cause is profound weight loss, thus leading to loss of the intervening mesenteric fat pad between the SMA and aorta. Significant weight loss can be attributable to several medical conditions (e.g. severe burns, HIV), psychological ailments (e.g. anorexia nervosa), or bariatric surgical procedures. Other causes include correction of scoliosis, especially in younger individuals [3]. Treatment modalities for scoliosis include bracing, casting, and corrective surgery, all of which result in lengthening of the vertebral column. The SMA is thereby cranially displaced from its origin which results in restriction of its lateral mobility and reduction in the acuity of the angle between the SMA and aorta. SMA syndrome is most commonly seen in young, underweight, and female individuals [4]. Symptoms include postprandial epigastric abdominal pain, and obstructive symptoms in more advanced cases including profound nausea and bilious vomiting. This condition can lead to further weight loss, as well as in nutritional and electrolyte abnormalities. Rare, yet severe, complications include gastric perforation and peritonitis, which can be fatal. ☆ Financial disclosures: No financial affiliations to disclose. ⁎ Corresponding author at: Division of Pediatric Surgery, DeWitt-Daughtry Family Department of Surgery, University of Miami Miller School of Medicine, Miami, FL, USA. Tel.: +1 305 243 5072; fax: +1 305 243 5731. E-mail address: [email protected] (J.E. Sola). http://dx.doi.org/10.1016/j.jpedsurg.2014.07.008 0022-3468/© 2014 Elsevier Inc. All rights reserved.

The treatment of SMA syndrome commonly entails medical and rarely surgical therapy. The goal of medical therapy is to relieve obstructive symptoms and potentially reverse underlying causes. In the acute phase, this can be achieved via nasogastric decompression, fluid resuscitation, and correction of electrolyte abnormalities. As mentioned above, weight loss is the common cause, thus nutritional support via parenteral or enteral means is paramount. Enteral means, via placement of nasojejunal feeding access, is the preferred route. Once sufficient weight gain is achieved, oral feedings can be initiated. Surgery is required only if conservative medical therapy fails. Surgical options in adults include Strong's procedure, surgical bypass via gastrojejunostomy, or duodenojejunostomy, or Ladd's procedure in children. Open and laparoscopic approaches have been described. We present a case of a 17 year-old previously healthy female, with an established diagnosis of SMA syndrome. She was successfully treated at our institution via a laparoscopic Ladd' procedure. 1. Case report A previously healthy 17-year-old female presented to our outpatient clinic with a 2-month history of postprandial abdominal pain, and 22-pound weight loss. She was already diagnosed with SMA syndrome after evaluation at an outside institution, which included an upper gastrointestinal (UGI) series. This study demonstrated obstruction of the third portion of the duodenum. She was treated conservatively with nasojejunal feedings and total parental nutrition with appropriate weight gain and resultant normalization of her body mass index (BMI) over the next month. However, she had persistent abdominal pain and obstructive symptoms with oral feedings. Her physical examination was positive only for mild epigastric tenderness on the abdominal examination. Her BMI was 22.2 kg/m [2]. Laboratory

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data were unremarkable. A repeat UGI series showed persistent evidence of SMA syndrome (Fig. 1). Surgical options were discussed with the patient and family, including Ladd's procedure, and surgical bypass with gastrojejunostomy or duodenojejunostomy. A laparoscopic Ladd's procedure and appendectomy was chosen as the primary option and the patient was thus scheduled for elective surgery. A total of four trocars were utilized with placement in subcostal, umbilical, and left flank regions. The patient's small bowel was normally rotated, with a well-formed Ligament of Treitz. The Ligament of Treitz was completely taken down using a Harmonic scalpel device (Ethicon Endo-Surgery Inc., Blue Ash, OH; Fig. 2) allowing mobilization of the fourth part of the duodenum. Next, the right colon was mobilized by dividing the lateral attachments up to the hepatic flexure. The hepatic flexure and proximal transverse colon were then separated from the duodenum, and all of the retroperitoneal attachments on the second and third portions of the duodenum were subsequently lysed, leading to full duodenal derotation. This step achieved the release of the third portion of the duodenum from its narrowed space between the SMA and aorta. The small bowel was then sequentially delivered to the right side of the abdomen. The procedure was concluded with an appendectomy. The patient was started on a clear liquid diet on postoperative day 1 and quickly advanced to a regular diet. Her symptoms immediately resolved. She was discharged home tolerating a regular diet after an uneventful hospital stay. A postoperative UGI series showed complete resolution of SMA compression on the duodenum (Fig. 3).

Fig. 2. Transection of the Ligament of Treitz using a Harmonic scalpel device (Ethicon Endo-Surgery Inc., Blue Ash, OH).

“angular torque” on the third portion of the duodenum [5]. A short Ligament of Treitz may cause an unnatural shift of the duodenum into the aortomesenteric angle, whereas angular torque refers to a disturbance in the relationship of the SMA and the duodenum caused by an elongation of the mesentery. As previously discussed, surgical management of SMA syndrome is necessary only in rare cases where

2. Discussion Several factors have been implicated in the development of SMA syndrome. These include a shortened Ligament of Treitz or an

Fig. 1. Preoperative upper gastrointestinal (UGI) series demonstrating persistent superior mesenteric artery (SMA) syndrome after 4 months of enteral tube feedings.

Fig. 3. (A and B) Postoperative UGI demonstrated complete resolution of SMA compression on the duodenum.

M. Alsulaimy et al. / Journal of Pediatric Surgery 49 (2014) 1533–1535

medical management fails. Three common surgical options for SMA syndrome are: duodenojejunostomy, gastrojejunostomy, and Strong's operation. According to the current literature, duodenojejunostomy is the most commonly performed procedure [6]. It was first described by Stavely [7], in 1908. In 1998, Gersin and Heniford [8] reported the first laparoscopic duodenojejunostomy. The feasibility of the laparoscopic approach has been confirmed with many case reports from various institutions. As a result, most centers are now performing this procedure laparoscopically. By using either approach, the affected portion of the duodenum is bypassed by anastomosing the proximal duodenum to a loop of jejunum. As with any anastomotic procedure however, leakage and stricture of the site may present as major complications requiring reoperation. In addition, a non-physiologic loop is created. Gastrojejunostomy is another surgical option, which has been largely abandoned. First, it only achieves adequate gastric decompression, without addressing duodenal obstruction, thus leading to possible need for reoperation requiring duodenojejunostomy. Second, it is associated with several significant complications including blind loop syndrome, dumping syndrome, and marginal ulceration [9]. This procedure is indicated if severe dilation is found in the stomach and duodenum, or duodenal anastomosis is deemed unsafe. Finally, a third surgical option for the management of SMA syndrome is Strong's operation. Strong [10] first described his operation in 1958. It involves lysis of the Ligament of Treitz, with mobilization of the fourth portion of the duodenum. This leads to mobilization of the duodenojejunal junction and its release from the aortomesenteric junction, which relieves the obstruction. Martorell et al. [11] confirmed the success of this procedure in 1961, when the Strong's procedure was performed on a 35 year-old male with SMA syndrome. Massoud [12] described his laparoscopic approach to Strong's original open procedure in 1995. He reported successful relief of symptoms in 3 out of 4 patients. The advantage of Strong's procedure includes the avoidance of a gastrointestinal anastomosis, thus eliminating risk of anastomotic leakage. Furthermore, it has been associated with earlier postoperative recovery. One theoretical disadvantage is that it creates a narrow mesenteric attachment of nonrotation or incomplete rotation with its attendant lifetime risk of volvulus which maybe significant in pediatric cases. In our case, we demonstrated the utility of a laparoscopic Ladd's procedure in achieving relief of SMA syndrome. This is a commonly performed procedure by pediatric surgeons for treatment of intestinal

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malrotation, and associated duodenal obstruction. It is very similar to Strong's procedure in many respects in that it achieves derotation of the duodenum, and its subsequent release of the duodenojejunal junction from the aortomesenteric junction. However, a Ladd's procedure mobilizes the right colon off the right gutter and the hepatic flexure facilitating access and lysis of the retroperitoneal attachments of the third portion of the duodenum and widening the mesentery of the midgut, thereby minimizing the risk of volvulus. Finally, a laparoscopic Ladd's procedure is technically easier to perform than a laparoscopic duodenojejunostomy.

3. Conclusion A laparoscopic Ladd's procedure is a feasible option for the treatment of SMA syndrome in pediatric patients. It achieves full derotation of the duodenum even in patients with a normally located Ligament of Treitz and relieves SMA compression of the duodenum. In addition, a laparoscopic Ladd's procedure avoids the need for a duodenal anastomosis and its attendant risk of leak or stricture formation.

References [1] Dorph MH. The cast syndrome; review of the literature and report of a case. N Engl J Med 1950;243(12):440–2. [2] WIlkie D. Chronic duodenal ileus. Br J Surg 1921;9:204–14. [3] Schwartz A. Scoliosis, superior mesenteric artery syndrome, and adolescents. Orthop Nurs 2007;26(1):19–24 [quiz 25–16]. [4] Welsch T, Buchler MW, Kienle P. Recalling superior mesenteric artery syndrome. Dig Surg 2007;24(3):149–56. [5] Ha CD, Alvear DT, Leber DC. Duodenal derotation as an effective treatment of superior mesenteric artery syndrome: a thirty-three year experience. Am Surg 2008;74(7):644–53. [6] Mathenge N, Osiro S, Rodriguez II Salib C, et al. Superior mesenteric artery syndrome and its associated gastrointestinal implications. Clin Anat 2013 [Epub ahead of print]. [7] Stavely A. Acute and chronic gastro-mesenteric ileus, with cure in a chronic case by duodeno-jejunostomy. Bull Johns Hopkins Hosp 1908;19:252–5. [8] Gersin KS, Heniford BT. Laparoscopic duodenojejunostomy for treatment of superior mesenteric artery syndrome. JSLS 1998;2(3):281–4. [9] Geer DA. Superior mesenteric artery syndrome. Mil Med 1990;155(7):321–3. [10] Strong EK. Mechanics of arteriomesenteric duodenal obstruction and direct surgical attack upon etiology. Ann Surg 1958;148(5):725–30. [11] Martorell R. Operative treatment of the superior mesenteric artery syndrome. Am Surg 1961;27:681–5. [12] Massoud WZ. Laparoscopic management of superior mesenteric artery syndrome. Int Surg 1995;80(4):322–7.

Laparoscopic Ladd's procedure for superior mesenteric artery syndrome.

Superior mesenteric artery (SMA) syndrome is an uncommon condition where the third portion of the duodenum is compressed and obstructed between the SM...
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