Malrotation

F9 20 187 2 10.0 2 1.1 1 Dilated kidney, malpositioned kidney, gastroschisis, acaudia, malrotated hindlimbs, malformed caudal vertebrae 2 Dilated aortic arch... 

Midgut volvulus is the most dramatic consequence of intestinal malrotation. When present at birth, the classic finding on a plain film is partial obstruction of the duodenum, but evidence of complete obstruction may also be present. In such cases, it is impossible to distinguish midgut volvulus from... 

Malrotation is a general term that includes a wide spectrum of anomalies that occur when intestinal rotation and fixation happens in an abnormal way. Failure of completion of the normal intestinal rotation leads to a continuum of anatomic abnormalities with a common clinical denominator consisting of obstruction with the potential for midgut volvulus. Only its presentation as an abdominal emergency in the neonatal period is discussed here. 

A patient with malrotation may also develop dense peritoneal bands, termed Ladd s bands, that originate in an attempt to fix the bowel. These bands extend from the cecum to the hilum of the liver, posterior peritoneum, or abdominal wall across the duodenum and can cause extrinsic duodenal obstruction. 

Obstruction due to Ladd s bands produces a Z-shaped configuration, outlining the lack of normal rotation and fixation of the duodenum. The Z-configuration may appear similar to the corkscrew of volvulus, but it does not indicate volvulus itself (Fig. 1.12). It must be stressed, however, that in most children with malrotation obstruction is caused by the volvulus with the band playing a lesser role or no roll at all (Ford et al. 1992 Torres and Ziegler 1993). 

Fig. 1.12a,b. Malrotation with z-shape of the duodenum and jejunum. Upper gastrointestinal series. Anteroposterior (a) and lateral (b) projections show a central, downward, Z course of the duodenum. No volvulus was found at surgery. Surgery demonstrated the partial obstruction to be produced by Ladd s bands... 

Currently, a contrast enema has fallen out of favor for the diagnosis of malrotation and its main complication is that the cecum can he normal in up to 20%-30% of infants with malrotation and, therefore, a normal cecum does not exclude malrotation (Slovis et al. 1980). On the other hand, approximately 15% of patients with normal rotation have a mobile cecum... 

Bonadio WA, Clarkson T, Naus J (1991) The clinical features of children with malrotation of the intestine. Pediatr Emerg Care 7 348-349... 

Carty HM (2002) Paediatric emergencies non-traumatic abdominal emergencies. Eur Radiol 12 2835-2848 Ceres L, Alonso I, Lopez P et al (1990) Ultrasound study of acute appendicitis in children with emphasis upon the diagnosis of retrocecal appendicitis. Pediatr Radiol 20 258-261 Chao HC, Kong MS, Chen JY et al (2000) Sonographic features related to volvulus in neonatal intestinal malrotation. J Ultrasound Med 19 371-376 Chinn DH, Millar El, Piper N (1987) Hemorrhagic cholecystitis. Sonographic and clinical presentation. J Ultrasound Med 6 313-317... 

Kong MS, Wong HF, Lin SL et al (1997) Factors related to detection of blood flow by color Doppler ultrasonography in intussusception. J Ultrasound Med 16 141-144 Koplewitz BZ, Daneman A (1999) The lateral view a useful adjunct in the diagnosis of malrotation. Pediatr Radiol 29 144-145... 

Long FR, Kramer SS, Markowitz RI et al (1996) Radiographic patterns of intestinal malrotation in children. Radiographics 16 547-556... 

Maxson RT, Eranklin PA, Wagner CW (1995). Malrotation in the older child surgical management, treatment, and outcome. Am Surg 61 135-138... 

Millar AJ, Rode H, Cywes S (2003) Malrotation and volvulus in infancy and childhood. Semin Pediatr Surg 12 229-236... 

Pickhardt PJ, Bhalla S (2002) Intestinal malrotation in adolescents and adults spectrum of clinical and imaging features. AJR Am J Roentgenol 179 1429-143 Pierro A, Hall N (2003) Surgical treatments of infants with necrotizing enterocolitis. Semin Neonatol 8 223-232 Ping AJ, Blane CE, Carver KA (1998) Current prognosis in necrotizing enterocolitis with portal vein gas. Can Assoc Radiol J 49 237-240... 

Spear R, Kimmey MB, Wang KY et al (1992) Appendiceal US scans histologic correlation. Radiology 183 831-834 Spigland N, Brandt ML, Yazbeck S (1990) Malrotation presenting beyond the neonatal period. J Pediatr Surg 25 1139-1142... 

Stringer MD, Pablot SM, Brereton RJ (1992) Paediatric intussusception. Br J Surg 79 867-876 Strouse PJ (2004) Disorders of intestinal rotation and fixation ( malrotation ). Pediatr Radiol 34 837-851 Strouse PJ, DiPietro MA, Saez F (2003) Transient small-bowel intussusception in children on CT. Pediatr Radiol 33 316-320... 

Toma P, Mengozzi E, DelPAcqua A et al (2002) Pyloric atresia report of two cases (one associated with epidermolysis bullosa and one associated with multiple intestinal atresias). Pediatr Radiol. 32 552-555 Torres AM, Ziegler MM (1993) Malrotation of the intestine. World J Surg 17 326-331... 

Fig. 3.2. a Radiograph with air as contrast agent malrotation. b Radiograph with high density contrast agent confirmation of malrotation. (Courtesy of Dr. Carlo Buonomo, Boston, MA, USA)... 

Duodenal Obstruction 170 Jejunal and Ileal Obstruction 172 Meconium Peritonitis 173 Meconium Ileus 173 Megacystis-Microcolon-Malrotation-Intestinal-Hypoperistalsis Syndrome (MMMIHS) 174... 

Some of the pathologies of the small intestine are discussed in Chapter 1. In the current chapter, therefore, little or no attention will be paid to (midgut volvulus) malrotation, the atresias, meconium ileus, meconium peritonitis, duplication cysts, necrotizing enterocolitis (NEC) and intussusception. 

Most important in these cases is to exclude conditions that are life-threatening for the patient. As intestinal malrotations may lead to bowel ischemia, which may subsequently lead to bowel necrosis, the patient s life is at risk when less than 3 in. of small bowel remain (Andrassy and Mahour 1981). 

In the case of duodenal obstruction with similar clinical signs as atresia, but with air distal to the duodenum, intestinal malrotation should be ruled out. 

The sign of an intestinal malrotation is sometimes recognizable on an abdominal plain film in which the small bowel will be located in the right upper abdomen. When this is observed, color Doppler sonography shows in 85% of the cases an abnormal position and course of the superior mesenteric artery with dextroposition to the superior mesen-... 

Fig. 5.4. An infant with intestinal malrotation on US. Abnormal position of the superior mesenteric arteria dextropositioned to the mesenteric vein...

Fig. 5.6. a UGI determines normal rotation by identifying the duodenojejunal junction (ligament of Treitz). b Intestinal malrotation demonstrated with a barium enema. Localization of jejunum in right upper quadrant. The duodenum does not cross the spine... 

To avoid the risk of aspiration, the contrast medium is injected in very small proportions (Devos and Meradji 2003). A contrast enema cannot reliably exclude malrotation as in 15%-20% of normal neonates the caecum has not yet descended, which will occur in the subsequent 6-12 months (Berrocal et al. 1999). 

Megacystis-Microcolon-Malrotation-Intestinal-Hypoperistalsis Syndrome (MMA/llHS)... 

Andrassy RJ, Mahour GH (1981) Malrotation of the midgut in infants and children a 25-year review. Arch Surg 116 158-160... 

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