Successful treatment of a 14-year-old patient with intestinal malrotation with laparoscopic Ladd procedure: case report and literature review

  • Yuka Nakajima1,

    Affiliated with

    • Hiroyuki Sakata1,

      Affiliated with

      • Tomohiro Yamaguchi1,

        Affiliated with

        • Norichika Yoshie1,

          Affiliated with

          • Taihei Yamada1,

            Affiliated with

            • Takaaki Osako1,

              Affiliated with

              • Mariko Terashima1,

                Affiliated with

                • Naomi Mambo1,

                  Affiliated with

                  • Ryuta Saka2,

                    Affiliated with

                    • Satoko Nose2,

                      Affiliated with

                      • Takashi Sasaki2,

                        Affiliated with

                        • Hiroomi Okuyama2,

                          Affiliated with

                          • Atsunori Nakao1Email author and

                            Affiliated with

                            • Joji Kotani1

                              Affiliated with

                              World Journal of Emergency Surgery20138:19

                              DOI: 10.1186/1749-7922-8-19

                              Received: 22 February 2013

                              Accepted: 12 May 2013

                              Published: 17 May 2013

                              Abstract

                              Midgut malrotation is an anomaly of intestinal rotation that occurs during fetal development and usually presents in the neonatal period. We present a rare case of malrotation in a 14-year-old patient who presented with cramping, generalized right abdominal pain, and vomiting for a duration of one day. A computed tomography abdominal scan and upper gastrointestinal contrast studies showed malrotation of the small bowel without volvulus. Laparoscopy revealed typical Ladd’s bands and a distended flabby third and fourth duodenal portion extrinsically obstructing the misplaced duodeno-jejunal junction. The Ladd procedure, including widening of the mesenteric base and appendectomy, was performed. Symptoms completely resolved in a half-year follow up period. Patients with midgut malrotation may present with vague abdominal pain, intestinal obstruction, or intestinal ischemia. The laparoscopic Ladd procedure is feasible and safe, and it appears to be as effective as the standard open Ladd procedure in the diagnosis and treatment of teenage or adult patients with intestinal malrotation.

                              Keywords

                              Malrotation Laparoscopic surgery Ladd procedure Acute abdomen Teenager

                              Introduction

                              Midgut malrotation is a congenital anomaly of intestinal rotation presenting mainly in childhood, usually within the first month of life. Midgut malrotation refers to a failure in the counter-clockwise rotation of the midgut, which results in the misplacement of the duodeno-jejunal junction to the right midline, comprising non-rotation and incomplete rotation of the superior mesenteric artery. Malrotation is typically diagnosed in the first few months of life, and 90% of cases are diagnosed during the first year. However, older children and adolescents are likely to present with recurrent abdominal pain, intermittent obstructive symptoms, or failure to thrive due to intestinal obstruction or intestinal ischemia [14].

                              We present the case of a symptomatic 14-year-old patient complaining of abdominal pain found to have intestinal malrotation that was successfully treated with a laparoscopic Ladd procedure. In adults or older children, the diagnosis is mostly incidental, based on investigation carried out for unrelated symptoms. Indeed, most adult patients are asymptomatic and incidentally, malrotation is often discovered later in life during surgery for other conditions. We diagnosed congenital intestinal malrotation, which rarely occurs in adults or older children, by using several modalities such as barium studies, computed tomography, and laparoscopy. We describe the clinical and radiological data of this patient followed by a brief review of the literature. This case report serves to demonstrate the benefits of laparoscopic surgery for malrotation. Also, the present case reminds us that intestinal malrotation should be considered in the differential diagnosis of a wide variety of symptoms and should be treated promptly once the diagnosis has been confirmed.

                              Presentation of case

                              A 14-year-old man presented to our emergency center with cramping and generalized abdominal pain. His abdominal pain began the previous night shortly after eating and recurred intermittently. Multiple presentations with similar symptoms during his teenage years had failed to identify the cause of his pain. He had no history of previous abdominal surgeries. He was on no medication at the time and denied alcohol or tobacco use. The patient also vomited on the day of presentation with vomitus containing biliary contents. On physical examination, the patient’s vital signs were: pulse, 67 beats/minute; blood pressure, 121/61 mmHg; body temperature, 36.9°C; and respiration rate, 15 breaths/minute. He was well-nourished and alert without cyanosis. His abdomen was not distended, but his bowel sounds were weak. He exhibited no peritoneal signs; however mild diffuse tenderness to deep palpation was noted. His white blood cell count was 10160 /mm3. Serum biochemistry and liver function test results were within normal limits, except a C-reactive protein level of 4.2 mg/dl.

                              Chest radiography did not reveal any signs of perforation of a hollow viscus. Ultrasonography demonstrated a fluid-filled, distended, small gut loop. No free liquid was visible between the intestinal segments or in the pelvis. Axial contrast-enhanced computed tomography (CT) obtained through the mid-abdomen showed an inverted relationship between the superior mesenteric artery (SMA) and superior mesenteric vein (SMV). The SMV was positioned to the anterior of the SMA (Figure 1A). Opacified small bowel presented almost entirely on the right side (Figure 1B). Upper gastrointestinal tract barium studies revealed that the duodenum ran caudally in a straight line from the first part onwards. The fourth duodenal segment and the normal duodeno-jejunal junction (Treitz ligament) were not developed (Figure 2A). Barium enema revealed that all colon segments with the cecum were found to the left of the spine. The cecum lay on the left side of the abdomen and the ileum entered it from the right (Figure 2B).
                              http://static-content.springer.com/image/art%3A10.1186%2F1749-7922-8-19/MediaObjects/13017_2013_297_Fig1_HTML.jpg
                              Figure 1

                              Contrast enhanced CT of the abdomen. A: Contrast enhanced CT can show the abnormal anatomic location of a right sided small bowel, a left-sided colon, and an abnormal relationship of the superior mesenteric vein (white arrow) situated to the anterior of the superior mesenteric artery (black arrow) instead of to the right. B: Opacified small bowel present almost entirely on the right side.

                              http://static-content.springer.com/image/art%3A10.1186%2F1749-7922-8-19/MediaObjects/13017_2013_297_Fig2_HTML.jpg
                              Figure 2

                              Gastrointestinal contrast studies. A: Upper gastrointestinal contrast studies showed malrotation of the small bowel without evidence of the duodenum crossing the lumbar spine. B: All small bowel was noted to be sequestered on the right side of the abdomen. The cecum lay on the left side of the abdomen and the ileum entered it from the right.

                              Based on the diagnosis of malrotation, the patient consented to exploratory laparoscopy. No segmented gangrene of the small intestine was present. Adhesions surrounding the SMA and cecal bands attaching the duodenum and right colon were noted. The Ladd’s procedure was performed. In detail, the cecum and right colon were rotated medially to expose the duodenum. The base of the mesentery was widened by incising the peritoneum. Then, the duodenum was moved until it was oriented inferiorly toward the right lower quadrant. The entire length of bowel was examined to assure that no other obstructive bands or kinks were present. The small bowel was then placed on the right side of the abdomen, and the colon was placed on the left side of the abdomen. Finally, the appendix was removed.

                              Operative time was 195 minutes with negligible bleeding. Postoperative course was uneventful. The patient was discharged two days later and has remained asymptomatic without recurrence of abdominal pain three months postoperatively.

                              Discussion

                              Malrotation of the intestinal tract is a congenital anomaly referring to either lack of or incomplete rotation of the fetal intestines around the axis of the superior mesenteric artery during fetal development. The malrotaion of the gut and abnormal location of the cecum produces a narrow superior mesenteric vascular pedicle, as opposed to the normally broadbased small bowel mesentery. This narrow superior mesenteric artery takeoff and lack of posterior peritoneal fusion predispose the patient to subsequent midgut volvulus and obstruction with potential vascular catastrophe.

                              Approximately 85% of malrotation cases present in the first two weeks of life [5, 6]. However, presentation of intestinal malrotation is very rare and its incidence has been reported to be between 0.2% and 0.5% [7]. True incidence of malrotation in older children or adults is unclear, because a number of patients may be asymptomatic. Not all patients with malrotation present with symptoms. Even once the anomaly is discovered, many live without complaint.

                              In adults or older children, the difficulty of diagnosis results from both the absence of specific physical findings and the low frequency in adults [8, 9]. Midgut malrotation in adults presents in numerous ways and the symptoms are non-specific. There are no typical sets of symptoms that are diagnostic of clinical problems. Symptoms in the adult patient are often mistaken for irritable bowel syndrome, peptic ulcer disease, biliary and pancreatic disease, and psychiatric disorders [8]. The location of the pain may vary from the epigastric region to the left upper abdominal quadrant, and the pain may be described as either intermittent cramping or persistent aching. It most often occurs postprandially and may last several minutes to an hour. Our patient had experienced abdominal distension, nausea, vomiting, and vague abdominal pain several times before, but the symptoms had always disappeared spontaneously.

                              Frequently, the plain radiograph is normal or may show an incomplete bowel obstruction. Specific findings that are diagnostic of malrotation can be detected through the use of both upper and lower gastrointestinal tract barium studies, angiography of the superior mesenteric artery, CT scan, and often emergency laparotomy. Occasionally, an abdominal radiograph will show dilated bowel loops with the orientation of a spiral nebula in the midabdomen. Barium studies may reveal a dilated duodenal loop caused by bowel obstruction with a spiral configuration of the proximal jejunal loops. CT is also used to investigate small-bowel volvulus and various signs have been described. Characteristic findings include the positioning of the superior mesenteric vein lying to the left or anterior to the artery because of torsion of the mesentery around its attachment, the presence of a right-sided duodeno-jejunal junction, the absence of a cecal gas shadow on the patient’s right side, or third and fourth duodenal junction that does not cross the patient’s spine [10, 11].

                              Management of intestinal rotation without midgut volvulus is controversial. In general, symptomatic patients with malrotation should be treated with surgical intervention. The classic treatment for incomplete intestinal rotation is the Ladd procedure, which requires mobilization of the right colon and cecum by division of Ladd bands, mobilization of the duodenum, division of adhesions around the superior mesenteric artery to broaden the mesenteric base, and an appendectomy [1214]. Spigland et al. recommended that all patients with malrotation are candidates for laparotomy, even if they are asymptomatic [15]. Mozziotti et al. recently reported a series of malrotation patients managed successfully with laparoscopic intervention [16]. Laparoscopy can be used to determine the position of the Treitz ligament and whether the cecum is fixed in the right lower quadrant. If the patient is decided to be at risk for volvulus (i.e. a shortened mesenteric pedicle), a Ladd's procedure can be accomplished laparoscopically with good long-term results [16, 17]. Due to the abnormal cecal position inflicted by malrotation, patients with associated appendicitis will demonstrate atypical symptoms with pain projected to the left of the middle line since the appendix will not be located in the normal area in the abdomen. This could lead to confusion and delay in diagnosing appendicitis in the future. Therefore, appendectomy is usually performed during surgical intervention.

                              Although most of the literature consists of occasional case reports or small case series, we searched for literature published between 1983 and 2012 using PubMed and Web Japan Medical Abstracts Society and found 37 reported cases of teenage patients (ages 13 through 19) with intestinal malrotation (Table 1). Twenty patients were male and seventeen were female. The diagnosis could be made by radiographic studies in all these patients. Patients presented with a variety of gastrointestinal disorders. Abdominal pain was the most frequent symptom (30/37). Other symptoms were nausea, feeding intolerance, reflux, and respiratory problems. The Ladd procedure was performed on 27 patients; on 12 patients the procedure was conducted laparoscopically.
                              Table 1

                              Reported cases of intestinal malrotaion (13–19 years old)

                              Year

                              Author

                              Journal

                              Age

                              Gender

                              Symptoms

                              Surgery

                              1991

                              Ko, et al.

                              Jpn J Surg (in Japanese)

                              19

                              F

                              abdominal distention

                              Ladd procedure

                              1992

                              Lal, et al.

                              Indian J Gastroenterol

                              17

                              F

                              abdominal pain, vomiting

                              gastrojejunostomy, vagotomy

                              1994

                              Pelucio, et al.

                              Am J Emerg Med

                              15

                              M

                              abdominal pain

                              Ladd procedure

                              1997

                              Kimura, et al.

                              Jpn J Clin Surg (in Japanese)

                              16

                              M

                              vomiting

                              Ladd procedure

                              1997

                              Ishida, et al.

                              J Jpn Soc Pediatr Surg (in Japanese)

                              13

                              F

                              abdominal pain, vomiting

                              Ladd procedure

                              1997

                              Yahata, et al.

                              Surg Laparosc Endosc

                              17

                              F

                              abdominal pain

                              laparoscopic Ladd procedure

                              1998

                              Yokota, et al.

                              Kesennuma Hosp Medical J (in Japanese)

                              15

                              F

                              abdominal pain

                              Ladd procedure

                              1999

                              Kang, et al.

                              J Jpn Soc Pediatr Surg (in Japanese)

                              16

                              M

                              abdominal pain, vomiting

                              Ladd procedure

                              1999

                              Yamashita, et al.

                              Surg Endosc

                              13

                              F

                              vomiting

                              laparoscopic Ladd procedure

                              2000

                              Walsh, et al.

                              J Pediatr Surg

                              13

                              F

                              abdominal pain

                              laparoscopic Ladd procedure

                              2001

                              Horiba, et al.

                              J Jpn Clin Surg (in Japanese)

                              17

                              M

                              vomiting

                              Ladd procedure

                              2003

                              Tsumura, et al.

                              Surg Endosc

                              15

                              F

                              abdominal pain

                              laparoscopic Ladd procedure

                              2003

                              Singer, et al.

                              J Am Coll Surg

                              19

                              M

                              abdominal pain, vomiting

                              Ladd procedure

                              2004

                              Tseng, et al.

                              JBR-BTR

                              14

                              F

                              abdominal pain

                              Ladd procedure

                              2005

                              Sato, et al.

                              Hokkaido Surg J (in Japanese)

                              18

                              M

                              abdominal pain

                              release of ileus

                              2005

                              Kamiyama, et al.

                              Radiat Med

                              14

                              M

                              abdominal pain

                              Ladd procedure

                              2007

                              Vechvitvarakul, et al.

                              J Pediatr Surg

                              13

                              M

                              abdominal pain, nausea, vomiting

                              Ladd procedure, appendectomy

                              2007

                              Kusuda, et al.

                              J Abdominal Emergency Medicine (in Japanese)

                              17

                              M

                              abdominal pain

                              Ladd procedure

                              2007

                              Draus, et al.

                              Am Surg

                              17

                              F

                              abdominal pain, nausea

                              laparoscopic Ladd procedure

                              2008

                              Duran, et al.

                              Turk J Gastroenterol

                              17

                              F

                              abdominal pain

                              division of adhesions

                              2008

                              Uchida, et al.

                              J Pediatr Surg

                              13

                              F

                              vomiting

                              Bypass

                              2009

                              Fukushima, et al.

                              Jpn J Endosc Surg (in Japanese)

                              15

                              F

                              abdominal pain, distention

                              laparoscopic Ladd procedure

                              2009

                              Tazaki, et al.

                              J Abdominal Emergency Medicine (in Japanese)

                              14

                              M

                              abdominal pain, vomiting

                              release of ileus

                              2009

                              Shimodaira, et al.

                              J of Jpn Soc Psychosomatic Med (in Japanese)

                              17

                              M

                              vomiting

                              laparoscopic Ladd procedure

                              2009

                              Fujii, et al.

                              J Jpn Clin Surg (in Japanese)

                              14

                              M

                              vomiting

                              Ladd procedure

                              2009

                              Mano, et al.

                              J Jpn Soc Pediatr Surg (in Japanese)

                              18

                              M

                              abdominal pain

                              laparoscopic Ladd procedure

                              2010

                              Watanabe, et al.

                              J Jpn Soc Gastrointestinal Dis (in Japanese)

                              19

                              F

                              abdominal pain

                              release of ileus

                              2010

                              Takazawa, et al.

                              Jpn J Pediatr Surg Nutr (in Japanese)

                              14

                              M

                              vomiting, distention

                              resection of necrotic intestine

                              2011

                              Kokado, et al.

                              J Jpn Soc Pediatr Surg (in Japanese)

                              13

                              F

                              abdominal pain, vomiting

                              fixation of colon

                              2011

                              Lam, et al.

                              J Pediatr Surg

                              14

                              M

                              abdominal pain, vomiting

                              resection of necrotic intestine

                              2012

                              Nath, et al.

                              Ann R Coll Engl

                              16

                              M

                              abdominal pain

                              laparoscopic Ladd procedure

                              2012

                              Jain, et al.

                              Case Rep Radiol

                              15

                              M

                              abdominal pain

                              Ladd procedure

                              2012

                              Wanjari, et al.

                              N Am J Med Sci

                              17

                              M

                              abdominal pain, vomiting

                              laparoscopic Ladd procedure

                              2012

                              Macedo, et al.

                              Einstein

                              13

                              F

                              abdominal pain

                              laparoscopic Ladd procedure

                              2012

                              Tran, et al.

                              J Pediatr Surg

                              18

                              M

                              abdominal pain

                              Ladd procedure

                              2012

                              Katsura, et al.

                              J Jpn Clin Surg (in Japanese)

                              19

                              F

                              abdominal pain

                              resection of necrotic intestine

                              2013

                              Nakajima, et al.

                              present case

                              17

                              M

                              abdominal pain, vomiting

                              laparoscopic Ladd procedure

                              An important point is that since many patients with intestinal malrotation are asymptomatic, everyone in the medical community should be made aware of the problem. Also, patients with acute volvulus should be treated promptly. Some asymptomatic adults may not need surgery. Of note, there is always the possibility that laparoscopic surgery will not entirely rule out the chance of acute volvulus; it could introduce problems such as band adhesion and future adhesive small bowel obstruction.

                              In conclusion, a number of teenage patients with intestinal malrotation present with symptoms. Increased awareness of this condition and an understanding of its varied presentation at different ages may reduce the time needed to diagnose the problem and improve patient outcome. Laparoscopy is an excellent technique for the evaluation and definitive management of patients without midgut volvulus with intestinal rotation abnormalities.

                              Consent

                              Written informed consent was obtained from the patient’s guardian/parent/next in keen for publication of this report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

                              Declarations

                              Authors’ Affiliations

                              (1)
                              Department of Emergency, Disaster and Critical Care Medicine, Hyogo College of Medicine
                              (2)
                              Department of Pediatric Surgery, Hyogo College of Medicine, Nishinomiya

                              References

                              1. Maxson RT, Franklin PA, Wagner CW: Malrotation in the older child: surgical management, treatment, and outcome. Am Surg 1995, 61:135–138.PubMed
                              2. Yanez R, Spitz L: Intestinal malrotation presenting outside the neonatal period. Arch Dis Child 1986, 61:682–685.PubMedView Article
                              3. Hsu SD, Yu JC, Chou SJ, Hsieh HF, Chang TH, Liu YC: Midgut volvulus in an adult with congenital malrotation. Am J Surg 2008, 195:705–707.PubMedView Article
                              4. Wanjari AK, Deshmukh AJ, Tayde PS, Lonkar Y: Midgut malrotation with chronic abdominal pain. N Am J Med Sci 2012, 4:196–198.PubMedView Article
                              5. Gamblin TC, Stephens RE Jr, Johnson RK, Rothwell M: Adult malrotation: a case report and review of the literature. Curr Surg 2003, 60:517–520.PubMedView Article
                              6. Ford EG, Senac MO Jr, Srikanth MS, Weitzman JJ: Malrotation of the intestine in children. Ann Surg 1992, 215:172–178.PubMedView Article
                              7. Wang CA, Welch CE: Anomalies of intestinal rotation in adolescents and adults. Surgery 1963, 54:839–855.PubMed
                              8. Fukuya T, Brown BP, Lu CC: Midgut volvulus as a complication of intestinal malrotation in adults. Dig Dis Sci 1993, 38:438–444.PubMedView Article
                              9. Nehra D, Goldstein AM: Intestinal malrotation: varied clinical presentation from infancy through adulthood. Surgery 2011, 149:386–393.PubMedView Article
                              10. Nichols DM, Li DK: Superior mesenteric vein rotation: a CT sign of midgut malrotation. AJR Am J Roentgenol 1983, 141:707–708.PubMedView Article
                              11. Singh S, Das A, Chawla AS, Arya SV, Chaggar J: A rare presentation of midgut malrotation as an acute intestinal obstruction in an adult: Two case reports and literature review. Int J Surg Case Rep 2013, 4:72–75.PubMedView Article
                              12. Schultz LR, Lasher EP, Bill AH Jr: Abnormalities of rotation of the bowel. Am J Surg 1961, 101:128–133.PubMedView Article
                              13. Matzke GM, Moir CR, Dozois EJ: Laparoscopic ladd procedure for adult malrotation of the midgut with cocoon deformity: report of a case. J Laparoendosc Adv Surg Tech A 2003, 13:327–329.PubMedView Article
                              14. Badea R, Al Hajjar N, Andreica V, Procopet B, Caraiani C, Tamas-Szora A: Appendicitis associated with intestinal malrotation: imaging diagnosis features. Case report. Med Ultrason 2012, 14:164–167.PubMed
                              15. Spigland N, Brandt ML, Yazbeck S: Malrotation presenting beyond the neonatal period. J Pediatr Surg 1990, 25:1139–1142.PubMedView Article
                              16. Mazziotti MV, Strasberg SM, Langer JC: Intestinal rotation abnormalities without volvulus: the role of laparoscopy. J Am Coll Surg 1997, 185:172–176.PubMed
                              17. Waldhausen JH, Sawin RS: Laparoscopic Ladd’s procedure and assessment of malrotation. J Laparoendosc Surg 1996,6(Suppl 1):S103-S105.PubMed

                              Copyright

                              © Nakajima et al.; licensee BioMed Central Ltd. 2013

                              This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://​creativecommons.​org/​licenses/​by/​2.​0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                              Advertisement