Malrotation

Teenager with
chronic abdominal pain. AP image from a small bowel follow through exam (below) shows the small bowel on the right side of the abdomen and the colon on the left side of the abdomen. Close examination of the upper GI portion of the exam (above) beyond the abnormal position of the ligament of Treitz shows a thickened appearance of the folds throughout the duodenum (above left) and proximal jejunum (above right) but there was no evidence of spiraling of the bowel or obstruction.The diagnosis on the upper GI was malrotation without midgut volvulus. In the operating room the patient was found to have malrotation with chronic midgut volvulus with chronically dilated lymphatic and mesenteric venous systems which were the cause of the thickened appearance of the small bowel folds.
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Newborn with
bilious vomiting who continued to have bilious vomiting after a Ladd procedure. AP (above left) and lateral (above right) images from an upper GI exam on day of life 1 show dilation of the first part of the duodenum and a spiral or corkscrew appearance of the second and third parts of the duodenum with the ligament of Trietz projecting over the midline of the spine and lower than the first part of the duodenum. AP (below left) and lateral (below right) images from an upper GI obtained several days after a Ladd procedure show the first part of the duodenum now to be normal in caliber while the second and third parts of the duodenum continue to have a spiral or corkscrew appearance with the ligament of Trietz continuing to project over the midline of the spine and lower than the first part of the duodenum.The diagnosis was malrotation with midgut volvulus on the initial upper GI exam and malrotation without midgut volvulus after a Ladd procedure on the repeat upper GI exam.
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Infant with
heterotaxy syndrome and complex congenital heart disease and bilious vomiting. AP image from an upper GI shows the ligament of Treitz to be in the right upper quadrant. There is no evidence of duodenal obstruction. The proximal jejunum is also in the right upper quadrant. There is also gastroesophageal reflux.The diagnosis was malrotation without midgut volvulus in a patient with heterotaxy syndrome.
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Midgut
volvulus in an infant with intestinal malrotation. Upper gastrointestinal imaging at delayed phase revealing dilated stomach and proximal duodenum with stagnation of the contrast.
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Midgut
volvulus in an infant with intestinal malrotation. Abdominal radiograph revealing paucity of bowel gas distal to the duodenum.
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Midgut
volvulus in an infant with intestinal malrotation. Ultrasonography with colour Doppler showing an inversion of the position of the SMA and SMV.
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School ager
who fell out of a tree and has abdominal pain. Axial CT with contrast of the abdomen shows the unopacified small bowel on the right side of the abdomen and the colon on the left side of the abdomen.The diagnosis was malrotation without midgut volvulus.
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Teenager with
chronic vomiting. AP image from an UGI exam (above) shows a redundant second portion of the duodenum to the right of the spine. AP image from later in the exam (below) shows the duodenal-jejunal junction to project over the middle of the spine and to be much lower in position than the duodenal bulb.The diagnosis was malrotation without midgut vovulus.
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Newborn with
non-bilious vomiting. Delayed AP image from an upper GI shows the ligament of Treitz to be over the right pedicle of the L2 verteral body and all of the jejunum to be on the right side of the abdomen.The diagnosis was malrotation without midgut volvulus.
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Infant with
bilious vomiting. AP image from an upper GI (left) shows the ligament of Treitz projecting over the right pedicle of the L2 vertebral body and to be lower in position than the duodenal bulb that projects over the right pedicle of the L1 vertebral body. The spiral appearance of the proximal jejunum is best appreciated on the lateral image (right).The diagnosis was malrotation with midgut volvulus.
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Newborn with
a family history of malrotation. AP image from an UGI exam performed through a feeding tube whose tip is in the duodenal bulb shows the entire duodenum, the ligament of Treitz and the proximal jejunum to lie in the right side of the abdomen. There is no evidence of obstruction or midgut volvulus.The diagnosis was malrotation without midgut volvulus.
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Newborn with
bilious vomitingTwo AP images from an upper GI exam show the duodenal-jejunal junction to be to the left of the spine but to be low lying. The proximal jejunum has a spiral appearance.The diagnosis was malrotation with midgut volvulus.
pediatricimaging.org • CC-by-nc-sa 4.0
Newborn with
bilious vomiting. AP image from an UGI exam shows dilation of the first and second parts of the duodenum. The ligament of Treitz was located in the middle of the spine and was in a lower position than the duodenal bulb. The proximal jejunum was in the right upper quadrant.The diagnosis was malrotation with midgut volvulus.
pediatricimaging.org • CC-by-nc-sa 4.0
Corkscrew
sign (midgut volvulus) • Intestinal malrotation and midgut volvulus - Ganzer Fall bei Radiopaedia
Gaillard, F. Intestinal malrotation and midgut volvulus. Case study, Radiopaedia.org. (accessed on 02 Nov 2022) https://doi.org/10.53347/rID-7909 • CC by-nc-sa 3.0 (modified)
Corkscrew
sign (disambiguation) • Corkscrew sign of malrotation with midgut volvulus - Ganzer Fall bei Radiopaedia
Amini, B. Corkscrew sign of malrotation with midgut volvulus. Case study, Radiopaedia.org. (accessed on 02 Nov 2022) https://doi.org/10.53347/rID-35920 • CC by-nc-sa 3.0 (modified)
Delayed
gastric emptying in the setting of intestinal malrotation. 99m-Tc sulfur colloid gastric emptying study. Dynamic images demonstrate delayed passage of radiotracer into abnormally positioned small bowel, located in the right abdomen (arrow).
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Delayed
gastric emptying in the setting of intestinal malrotation. CT shows the abnormal course of the duodenum (yellow arrow) which wraps around the SMA (red arrow) before continuing into the right abdomen. Note the presence of proximal small bowel in the right abdomen.
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Delayed
gastric emptying in the setting of intestinal malrotation. Same CT abdomen showing left-to-right inversion of the superior mesenteric artery (red) and superior mesenteric vein (yellow).
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Infant with
bilious vomiting and abdominal distension. AXR upright (left) shows multiple dilated loops of small bowel with air fluid levels. Sagittal US of the abdomen (above right) shows multiple dilated loops of peristalsing small bowel, however an intussusception was not seen. AP image from an upper GI exam (below right) shows the duodenal-jejunal junction to be low in position and to be to the right of the spine.The diagnosis was Meckel diverticulum causing a small bowel obstruction and a false positive diagnosis of malrotation with midgut volvulus due to displacement of the duodenal-jejunal junction by dilated loops of small bowel.
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Infant with
bilious vomiting for several days and a tender abdomen. AXR supine shows multiple dilated loops of bowel with thickened walls.The diagnosis was small bowel obstruction. In the operating room the patient was found to have malrotation with midgut volvulus. The small bowel was non-viable.
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Newborn with
bilious vomiting. AP image from an upper GI exam shows a normal appearing duodenal C loop with the duodenal-jejunal junction projecting to the left of the spine at the same level as the duodenal bulb. The jejunum is on the left side of the abdomen.The diagnosis was normal rotation of the bowel and thus no evidence of malrotation with midgut volvulus.
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Newborn with
bilious residuals from the nasogastric tube. AXR AP shows the tip of the nasogastric tube to be transpyloric in position, in the first part of the duodenum.The diagnosis was malposition of the nasogastric tube tip in the duodenum, clinically mimicking malrotation with midgut volvulus.
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Newborn with
bilious emesis. AP image from an upper GI exam (left) shows the duodenal jejunal junction to be located to the right of the spine. Lateral image (right) shows the duodenum to have a corkscrew appearance.The diagnosis was malrotation with midgut volvulus.
pediatricimaging.org • CC-by-nc-sa 4.0
Infant with
bilious vomiting. AP image from an UGI exam (above) shows the duodenal jejunal junction to project to the left of the spine in its normal position. Later image from the exam (below) shows the jejunum to be on the right side, rather than the left side, of the abdomen.The diagnosis was normal rotation of the bowel with no evidence of malrotation with midgut volvulus.
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Infant with
projectile vomiting. Transverse US of the pylorus (left) showed a normal appearing pylorus and showed the superior mesenteric vein to be directly above the superior mesenteric artery (the round structure with an echogenic rim in the center of the image), raising suspicion for malrotation. AP image from an UGI exam (right) shows the duodenal jejunal junction to be over the right pedicle of the spine and to be below the level of the duodenal bulb.The diagnosis was malrotation with midgut volvulus.
pediatricimaging.org • CC-by-nc-sa 4.0
Newborn with
3 days of bilious vomiting. Serial AP images from an UGI exam show dilation of the first and second parts of the duodenum, the ligament of Treitz projects over the midline of the spine and lower than the duodenal bulb, and the third portion of the duodenum has a spiral or corkscrew appearance.The diagnosis was malrotation with midgut volvulus.
pediatricimaging.org • CC-by-nc-sa 4.0
Newborn with
bilious vomiting. AP image from an UGI exam (left) shows dilation of the stomach and first and second parts of the duodenum. There is an abrupt transition in bowel counter at the ligament of Treitz which projects low in position and over the middle of the spine. The jejunum then appears to spiral away downstream. AP image from later in the exam (right) better shows the spiraling / corkscrew appearance of the proximal jejunum.The diagnosis was malrotation with midgut volvulus.
pediatricimaging.org • CC-by-nc-sa 4.0
Infant with
bilious vomiting. Early AP image from an UGI exam (above) showed the ligament of Treitz to project over the midline of the spine and at the same level as the duodenal bulb. Later image (below left) showed the proximal jejunum in the right upper quadrant. Final image (below right) after following contrast through to the colon showed the cecum to be on the right side of the abdomen but to be high in position. The patient was surgically explored and in the operating room the ligament of Treitz was seen in the left upper quadrant and there was normal rotation of the bowel.The diagnosis was false positive diagnosis of malrotation with midgut volvulus.
pediatricimaging.org • CC-by-nc-sa 4.0
Newborn with
bilious vomiting. AP image from an upper GI shows complete obstruction of the duodenum at the junction of the second and third parts of the duodenum and associated massive gastroesophageal reflux. A final image taken 5 minutes later was unchanged.The diagnosis was gastroesophageal reflux due to malrotation with midgut volvulus causing duodenal obstruction.
pediatricimaging.org • CC-by-nc-sa 4.0
Infant with
projectile vomiting. Transverse color US of the upper abdomen in the midline (above) shows the mesenteric vessels swirling around themselves (Whirlpool sign). The superior mesenteric vein was above the superior mesenteric artery. Upper GI exam (below) shows the duodenal jejunal junction to be to the left of the spine but to be lower in position than the duodenal bulb and the proximal jejunum appears to spiral downwards away from it.The diagnosis was malrotation with midgut volvulus.
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Midgut
volvulus in an infant with intestinal malrotation. Ultrasonography showing an epigastric mass with a typical whirlpool pattern (arrow).
www.eurorad.org • CC by-nc-sa-4.0
Midgut
volvulus in an infant with intestinal malrotation. Ultrasonography without colour Doppler showing an inversion of the position of the SMA and the SMV.
www.eurorad.org • CC by-nc-sa-4.0
Radiopaedia • CC-by-nc-sa 3.0 • de

Intestinal malrotation is a congenital anatomical anomaly that results from an abnormal rotation of the gut as it returns to the abdominal cavity during embryogenesis.

Although some individuals live their entire life with malrotated bowel without symptoms, the abnormality does predispose to midgut volvulus and internal hernias, with the potential for life-threatening complications.

Epidemiology

Intestinal malrotation is a congenital abnormality seen in up to 1:6000 live births .

Associations

It is frequently (~50%) associated with other abdominal anomalies, some of which are causative and others merely associated :

Clinical presentation

The clinical presentation of malrotation often correlates with the age of onset .

In the infant, the most common presentation is with midgut volvulus. Patients with intestinal nonrotation have a lower incidence of midgut volvulus than other types of malrotation.

In the older child or even adult presentation is more frequently intermittent with episodes of spontaneously resolving duodenal obstruction. This is thought to be due to kinking of the duodenum by Ladd bands rather than a volvulus . Internal hernias are also encountered.

In some individuals, the presentation is very non-specific with episodes of abdominal pain, weight loss, melena, or even chronic pancreatitis .

Pathology

During normal embryogenesis, the bowel herniates into the base of the umbilical cord and rapidly elongates. As it returns to the abdominal cavity, it undergoes complex ~270 degrees counter-clockwise rotation resulting in the duodenojejunal (DJ) flexure typically located to the left of the midline, at the level of the L1 vertebral body and the terminal ileum located in the right iliac fossa. This results in a broad mesentery running obliquely down from the DJ flexure to the cecum and prevents rotation around the superior mesenteric artery (SMA) .

In malrotation, this does not occur and, as a result, the mesentery often has a short root, which allows it to act as a pedicle (through which the SMA and SMV pass) around which volvulus can occur.

The rotation of the duodenojejunal loop has been described as beginning around the 5th gestational week and being complete around the 8th.  Rotation of the cecocolic loop occurs at a later time, around the 10th week of gestational age. This might explain forms of partial intestinal malrotation .

Intestinal nonrotation is a subtype of malrotation in which the small bowel is mainly located in the right hemiabdomen and the cecum in the left hemiabdomen. The risk of volvulus is much lower in complete nonrotation because patients have the effective anatomy of those who have undergone a Ladd procedure.

Radiographic features

Plain radiograph

Abdominal radiographs, in the absence of midgut volvulus, are neither specific nor sensitive . They may show:

  • right sided jejunal markings
  • absence of stool filled colon in the right lower quadrant
Ultrasound 

May show an inversion in the SMA/SMV relationship with the SMA on the right and the SMV on the left. Although this has classically been advocated as a useful sign for suspecting or excluding intestinal malrotation, a normal SMA/SMV relationship can be seen in up to 29% of patients with surgically proven malrotation, whereas an inverted relationship is seen in up to 11% of patients .

A more useful sign on ultrasound is demonstrating the retro-mesenteric D3 segment of the duodenum, where the horizontal (D3) segment of the duodenum is seen in a transverse plane between the superior mesenteric vessels and the aorta .

Although demonstrating a retro-mesenteric duodenum is extremely sensitive and specific for excluding malrotation, it is not perfect and such a normal anatomic relationship has been described in at least one case of surgically treated intestinal malrotation with midgut volvulus . This case was likely secondary to partial intestinal malrotation, based on the embryological delay described above between the rotation of the duodenojejunal loop and the cecocolic loop.

CT

Depending on the degree of malrotation, CT may show:

  • absence of a retro-mesenteric (retro-peritoneal) D3 segment of the duodenum
  • may again show abnormal SMA (smaller and more circular)/SMV relationship
  • large bowel predominantly on the left and small bowel predominantly on the right
Fluoroscopy 

A pediatric upper gastrointestinal contrast study is the examination of choice when the diagnosis is suspected. The key findings of malrotation is an abnormal duodenojejunal (DJ) junction location:

  • frontal view
    • DJ junction fails to cross the midline to the left of the left-sided vertebral body pedicle
    • DJ junction lies inferior to the duodenal bulb
  • lateral view
    • D2 and D3 segments of the duodenum not located posteriorly in a retroperitoneal position

Although not a specific criteria of malrotation, the jejunum is commonly located to the left of the spine.

Contrast enema has historically also been used, the theory being that in malrotation the large bowel will also be malrotated. Unfortunately, in ~25% (range 20-30%) of cases with malrotation, the cecum is normally located. The converse is also true, with the position of the cecum in normal individuals being variable . Very rarely, the cecum may be malrotated and the small bowel in a normal position.

Treatment and prognosis

Due to the potential for life-threatening midgut volvulus and ischemic bowel, once discovered malrotation is corrected surgically. The general principles of treatment are:

  • mobilization of the bowel including untwisting any volvulus present
  • division of abnormal peritoneal bands (Ladd bands) 
  • widening of the mesenteric base
  • fixation of the duodenum and cecum to the retroperitoneum (pexy) is not universally performed
  • prophylactic appendectomy is often considered, since in later life a delayed/missed diagnosis of appendicitis may occur, due to lack of awareness that the patient has a left sided cecum/appendix causing left sided abdominal pain

It should be noted that normal anatomical positioning is not achieved; the duodenum and small bowel remain on the right, and the cecum and colon are on the left side of the abdomen .

Differential diagnosis

A rate of 15% false positive has been reported in the diagnosis of malrotation using barium meal . Hence, differential diagnoses must be kept in mind, including:

  • normal duodenum: located inferiorly because of gastric distension or abnormally because of a feeding tube, renal agenesis, splenomegaly, etc.
  • duodenum inversum
  • wandering duodenum
Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu intestinale Malrotation:
Dünndarmileus
 
Wikipedia • CC BY-SA 4.0
intestinale
Malrotation ohne Volvulus
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intestinale
Malrotation mit Volvulus
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