Intestinal malrotation in adults
- Terry Buchmiller, MD
Terry Buchmiller, MD
- Assistant Professor
- Harvard Medical School
Intestinal malrotation occurs when the normal rotation of the embryonic gut is arrested or disturbed during in utero development. While most patients present during the neonatal period or the first year of life , some present as older children or adults [1-3]. (See "Intestinal malrotation in children", section on 'Epidemiology'.)
Because of its rarity, the diagnosis of intestinal malrotation in adult patients is often delayed and therefore associated with increased morbidity [4,5]. The clinical manifestations, diagnosis and treatment of intestinal malrotation in adults will be discussed in this topic. Intestinal malrotation in children is discussed separately. (See "Intestinal malrotation in children".)
Intestinal malrotation occurs as a result of arrested normal rotation of the embryonic gut. As a result, two anatomic variations develop (Ladd bands (picture 1) and narrow mesenteric base (figure 1)), which predispose to symptoms of gastrointestinal obstruction. The pathogenesis is discussed in detail separately. (See "Intestinal malrotation in children", section on 'Embryology and pathogenesis'.)
In neonates, intestinal malrotation almost always presents with acute obstruction or volvulus. However, the clinical presentation of intestinal malrotation in adults is more variable [4,6,7], and some adults are asymptomatic. In asymptomatic patients, malrotation is incidentally discovered by imaging studies obtained for other purposes.
Chronic clinical presentation — Most adults with symptomatic intestinal malrotation present insidiously with one or more of the following features that usually occur during the postprandial period :
- Bass KD, Rothenberg SS, Chang JH. Laparoscopic Ladd's procedure in infants with malrotation. J Pediatr Surg 1998; 33:279.
- Stewart DR, Colodny AL, Daggett WC. Malrotation of the bowel in infants and children: a 15 year review. Surgery 1976; 79:716.
- Warner, B. Malrotation. In: Surgery of Infants and Children: Scientific Principles and Practice, Oldham, KT, Colombani, PM, Foglia, RP (Eds), Lippincott Williams & Wilkins, Philadelphia 1997. p.1229.
- Durkin ET, Lund DP, Shaaban AF, et al. Age-related differences in diagnosis and morbidity of intestinal malrotation. J Am Coll Surg 2008; 206:658.
- Coe TM, Chang DC, Sicklick JK. Small bowel volvulus in the adult populace of the United States: results from a population-based study. Am J Surg 2015; 210:201.
- Pickhardt PJ, Bhalla S. Intestinal malrotation in adolescents and adults: spectrum of clinical and imaging features. AJR Am J Roentgenol 2002; 179:1429.
- von Flüe M, Herzog U, Ackermann C, et al. Acute and chronic presentation of intestinal nonrotation in adults. Dis Colon Rectum 1994; 37:192.
- Yanez R, Spitz L. Intestinal malrotation presenting outside the neonatal period. Arch Dis Child 1986; 61:682.
- Devlin HB, Williams RS, Pierce JW. Presentation of midgut malrotation in adults. Br Med J 1968; 1:803.
- Bachur RG. Abdominal emergencies. In: Textbook of Pediatric Emergency Medicine, 5th ed, Fleisher GR, Ludwig S, Henretig FM (Eds), Lippincott, Williams & Wilkins, Philadelphia 2006. p.1605.
- Morrison SC. Controversies in abdominal imaging. Pediatr Clin North Am 1997; 44:555.
- Yang B, Chen WH, Zhang XF, Luo ZR. Adult midgut malrotation: multi-detector computed tomography (MDCT) findings of 14 cases. Jpn J Radiol 2013; 31:328.
- Ashley LM, Allen S, Teele RL. A normal sonogram does not exclude malrotation. Pediatr Radiol 2001; 31:354.
- Zerin JM, DiPietro MA. Superior mesenteric vascular anatomy at US in patients with surgically proved malrotation of the midgut. Radiology 1992; 183:693.
- Pracros JP, Sann L, Genin G, et al. Ultrasound diagnosis of midgut volvulus: the "whirlpool" sign. Pediatr Radiol 1992; 22:18.
- Dilley AV, Pereira J, Shi EC, et al. The radiologist says malrotation: does the surgeon operate? Pediatr Surg Int 2000; 16:45.
- Frasier LL, Leverson G, Gosain A, Greenberg J. Laparoscopic versus open Ladd's procedure for intestinal malrotation in adults. Surg Endosc 2015; 29:1598.
- Moldrem AW, Papaconstantinou H, Broker H, et al. Late presentation of intestinal malrotation: an argument for elective repair. World J Surg 2008; 32:1426.
- Seymour NE, Andersen DK. Laparoscopic treatment of intestinal malrotation in adults. JSLS 2005; 9:298.
- Ben Ely A, Gorelik N, Cohen-Sivan Y, et al. Appendicitis in adults with incidental midgut malrotation: CT findings. Clin Radiol 2013; 68:1212.
- Malek MM, Burd RS. The optimal management of malrotation diagnosed after infancy: a decision analysis. Am J Surg 2006; 191:45.
- McVay MR, Kokoska ER, Jackson RJ, Smith SD. Jack Barney Award. The changing spectrum of intestinal malrotation: diagnosis and management. Am J Surg 2007; 194:712.
- Matzke GM, Dozois EJ, Larson DW, Moir CR. Surgical management of intestinal malrotation in adults: comparative results for open and laparoscopic Ladd procedures. Surg Endosc 2005; 19:1416.
- Lessin MS, Luks FI. Laparoscopic appendectomy and duodenocolonic dissociation (LADD) procedure for malrotation. Pediatr Surg Int 1998; 13:184.
- Mazziotti MV, Strasberg SM, Langer JC. Intestinal rotation abnormalities without volvulus: the role of laparoscopy. J Am Coll Surg 1997; 185:172.
- Waldhausen JH, Sawin RS. Laparoscopic Ladd's procedure and assessment of malrotation. J Laparoendosc Surg 1996; 6 Suppl 1:S103.
- Hsu SD, Yu JC, Chou SJ, et al. Midgut volvulus in an adult with congenital malrotation. Am J Surg 2008; 195:705.
- Dietz DW, Walsh RM, Grundfest-Broniatowski S, et al. Intestinal malrotation: a rare but important cause of bowel obstruction in adults. Dis Colon Rectum 2002; 45:1381.
- Lin JN, Lou CC, Wang KL. Intestinal malrotation and midgut volvulus: a 15-year review. J Formos Med Assoc 1995; 94:178.
- Fonkalsrud E. Rotational anomalies and volvulus. In: Principles of Pediatric Surgery, O'Neill JA, et al (Eds), Mosby, St. Louis 2003. p.477.
- CLINICAL PRESENTATION
- Chronic clinical presentation
- Acute clinical presentation
- DIAGNOSTIC EVALUATION
- Suggestive imaging studies
- - Plain films
- - Barium enema
- - Abdominal CT
- - Abdominal ultrasound
- Confirmatory imaging study
- - Upper gastrointestinal series
- DIFFERENTIAL DIAGNOSIS
- Differential diagnosis of chronic presentation
- Differential diagnosis of acute presentation
- Treatment of patients with chronic presentation
- Treatment of patients with acute presentation
- Treatment of incidental or asymptomatic malrotation
- SURGICAL OPTIONS
- Open Ladd procedure
- Laparoscopic Ladd procedure
- SUMMARY AND RECOMMENDATIONS