Intestinal malrotation

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Intestinal malrotation
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Intestinal malrotation is a congenital anomaly of rotation of the midgut. It occurs during the first trimester as the fetal gut undergoes a complex series of growth and development. Malrotation can lead to a dangerous complication called volvulus, in which cases emergency surgery is indicated. [1] Malrotation can refer to a spectrum of abnormal intestinal positioning, often including:[ citation needed ]

Contents

The position of the intestines, narrow mesentery and Ladd's bands can contribute to several severe gastrointestinal conditions. The narrow mesentery predisposes some cases of malrotation to midgut volvulus, a twisting of the entire small bowel that can obstruct the mesenteric blood vessels leading to intestinal ischemia, necrosis, and death if not promptly treated. The fibrous Ladd's bands can constrict the duodenum, leading to intestinal obstruction.

Signs and symptoms

Signs and symptoms of malrotation vary depending on age and whether the patient is suffering from an acute volvulus or experiencing chronic symptoms.[ citation needed ]

Complications

Intestinal malrotation can lead to a number of disease manifestations and complications such as:[ citation needed ]

Causes

Diagram showing the process by which the intestine rotates and herniates during normal development. From panel A to B (left-sided views), the midgut loop rotates 90deg in a counterclockwise direction, so that its position changes from midsagittal (A) to transverse (B1). The small intestine forms loops (B2) and slides back into the abdomen (B3) during resolution of the hernia. Meanwhile, the cecum moves from the left to the right side, which represents the additional 180deg counterclockwise rotation of the intestine (C, central view). Intestinal rotation and herniation.png
Diagram showing the process by which the intestine rotates and herniates during normal development. From panel A to B (left-sided views), the midgut loop rotates 90° in a counterclockwise direction, so that its position changes from midsagittal (A) to transverse (B1). The small intestine forms loops (B2) and slides back into the abdomen (B3) during resolution of the hernia. Meanwhile, the cecum moves from the left to the right side, which represents the additional 180° counterclockwise rotation of the intestine (C, central view).

The exact cause of intestinal malrotation is unknown. It is not definitively associated with a particular gene, but there is some evidence of recurrence in families. [5]

Diagnosis

Malrotation is most often diagnosed during infancy, however, some cases are not discovered until later in childhood or even adulthood. [6] [2]

With acutely ill patients, consider emergency surgery laparotomy if there is a high index of suspicion.[ citation needed ]

In cases of volvulus, plain radiography may demonstrate signs of duodenal obstruction with dilatation of the proximal duodenum and stomach but it is often non-specific. Ultrasonography may be useful in some cases of volvulus, depicting a "whirlpool sign" where the superior mesenteric artery and superior mesenteric vein have twisted. [7]

Upper gastrointestinal series is the modality of choice for the evaluation of malrotation, as it will often show an abnormal position of the duodenum and duodeno-jejunal flexure (ligament of Treitz). In cases of malrotation complicated with volvulus, upper GI demonstrates a corkscrew appearance of the distal duodenum and jejunum. In cases of obstructing Ladd's bands, upper GI may reveal a duodenal obstruction. Although upper GI series is regarded as the most reliable diagnostic test for intestinal malrotation, false negatives may occur in 5% of cases. [7] False negatives are most frequently attributed to radiographer error, uncooperative pediatric patients, or variations in intestinal positioning. In equivocal cases physicians may wish to repeat the upper GI or consider additional diagnostic modalities. Lower gastrointestinal series, may be helpful in some patients by showing the caecum at an abnormal location. CT scan and magnetic resonance imaging may also aide in the diagnosis of equivocal cases.[ citation needed ]

The incidence of intestinal malrotation in infants with omphalocoele is low. Therefore, there is little evidence to support the screening for intestinal malrotation in infants with omphalocoele. [8]

Treatment

Prompt surgical treatment is necessary for intestinal malrotation when volvulus has occurred:[ citation needed ]

With this condition the appendix is often on the wrong side of the body and therefore removed as a precautionary measure during the surgical procedure.

This surgical technique is known as the "Ladd's procedure", after Dr. William Ladd. [9] [10] Long-term research on the Ladd's procedure indicates that even after surgery, some patients are susceptible to GI issues and may need further surgery. [11]

See also

Related Research Articles

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<span class="mw-page-title-main">Mesentery</span> Contiguous fold of tissues that supports the intestines

The mesentery is an organ that attaches the intestines to the posterior abdominal wall and is formed by the double fold of peritoneum. It helps in storing fat and allowing blood vessels, lymphatics, and nerves to supply the intestines, among other functions.

<span class="mw-page-title-main">Meckel's diverticulum</span> Medical condition

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<span class="mw-page-title-main">Omphalocele</span> Rare abdominal wall defect in which internal organs remain outside of the abdomen in a sac

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<span class="mw-page-title-main">Suspensory muscle of duodenum</span> Muscle between the duodenum and jejunum

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<span class="mw-page-title-main">Volvulus</span> Twisting of part of the intestine, causing a bowel obstruction

A volvulus is when a loop of intestine twists around itself and the mesentery that supports it, resulting in a bowel obstruction. Symptoms include abdominal pain, abdominal bloating, vomiting, constipation, and bloody stool. Onset of symptoms may be rapid or more gradual. The mesentery may become so tightly twisted that blood flow to part of the intestine is cut off, resulting in ischemic bowel. In this situation there may be fever or significant pain when the abdomen is touched.

<span class="mw-page-title-main">Gastrointestinal perforation</span> Medical condition

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<span class="mw-page-title-main">Annular pancreas</span> Medical condition

Annular pancreas is a rare condition in which the second part of the duodenum is surrounded by a ring of pancreatic tissue continuous with the head of the pancreas. This portion of the pancreas can constrict the duodenum and block or impair the flow of food to the rest of the intestines. It is estimated to occur in 1 out of 12,000 to 15,000 newborns. The ambiguity arises from the fact that not all cases are symptomatic.

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<span class="mw-page-title-main">Intestinal atresia</span> Medical condition

Intestinal atresia is any congenital malformation of the structure of the intestine that causes bowel obstruction. The malformation can be a narrowing (stenosis), absence or malrotation of a portion of the intestine. These defects can either occur in the small or large intestine.

<span class="mw-page-title-main">Bowel resection</span> Surgical procedure in which a part of an intestine is removed

A bowel resection or enterectomy is a surgical procedure in which a part of an intestine (bowel) is removed, from either the small intestine or large intestine. Often the word enterectomy is reserved for the sense of small bowel resection, in distinction from colectomy, which covers the sense of large bowel resection. Bowel resection may be performed to treat gastrointestinal cancer, bowel ischemia, necrosis, or obstruction due to scar tissue, volvulus, and hernias. Some patients require ileostomy or colostomy after this procedure as alternative means of excretion. Complications of the procedure may include anastomotic leak or dehiscence, hernias, or adhesions causing partial or complete bowel obstruction. Depending on which part and how much of the intestines are removed, there may be digestive and metabolic challenges afterward, such as short bowel syndrome.

<span class="mw-page-title-main">Bowel infarction</span> Injury to the intestine resulting from insufficient blood flow

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<span class="mw-page-title-main">Duodenal atresia</span> Medical condition

Duodenal atresia is the congenital absence or complete closure of a portion of the lumen of the duodenum. It causes increased levels of amniotic fluid during pregnancy (polyhydramnios) and intestinal obstruction in newborn babies. Newborns present with bilious or non-bilous vomiting within the first 24 to 48 hours after birth, typically after their first oral feeding. Radiography shows a distended stomach and distended duodenum, which are separated by the pyloric valve, a finding described as the double-bubble sign.

<span class="mw-page-title-main">Intestinal ischemia</span> Restriction of blood flow to the small intestine resulting in injury

Intestinal ischemia is a medical condition in which injury to the large or small intestine occurs due to not enough blood supply. It can come on suddenly, known as acute intestinal ischemia, or gradually, known as chronic intestinal ischemia. The acute form of the disease often presents with sudden severe abdominal pain and is associated with a high risk of death. The chronic form typically presents more gradually with abdominal pain after eating, unintentional weight loss, vomiting, and fear of eating.

<span class="mw-page-title-main">Ladd's bands</span>

Ladd's bands, sometimes called bands of Ladd, are fibrous stalks of peritoneal tissue that attach the cecum to the retroperitoneum in the right lower quadrant (RLQ). Obstructing Ladd's Bands are associated with malrotation of the intestine, a developmental disorder in which the cecum is found in the right upper quadrant (RUQ), instead of its normal anatomical position in the RLQ. Ladd's bands then pass over the second part of the duodenum, causing extrinsic compression and obstruction. This clinically manifests as poor feeding and bilious vomiting in neonates. Screening can be performed with an upper GI series. The most severe complication of malrotation is midgut volvulus, in which the mesenteric base twists around the superior mesenteric artery, compromising intestinal perfusion, leading to bowel necrosis.

<span class="mw-page-title-main">Superior mesenteric artery syndrome</span> Medical condition

Superior mesenteric artery (SMA) syndrome is a gastro-vascular disorder in which the third and final portion of the duodenum is compressed between the abdominal aorta (AA) and the overlying superior mesenteric artery. This rare, potentially life-threatening syndrome is typically caused by an angle of 6–25° between the AA and the SMA, in comparison to the normal range of 38–56°, due to a lack of retroperitoneal and visceral fat. In addition, the aortomesenteric distance is 2–8 millimeters, as opposed to the typical 10–20. However, a narrow SMA angle alone is not enough to make a diagnosis, because patients with a low BMI, most notably children, have been known to have a narrow SMA angle with no symptoms of SMA syndrome.

<span class="mw-page-title-main">William E. Ladd</span> American physician

William Edwards Ladd was an American surgeon, and is commonly regarded as one of the founders of pediatric surgery.

Neonatal bowel obstruction (NBO) or neonatal intestinal obstruction is the most common surgical emergency in the neonatal period. It may occur due to a variety of conditions and has an excellent outcome based on timely diagnosis and appropriate intervention.

<span class="mw-page-title-main">Double bubble (radiology)</span>

In radiology, the double bubble sign is a feature of pediatric imaging seen on radiographs or prenatal ultrasound in which two air filled bubbles are seen in the abdomen, representing two discontiguous loops of bowel in a proximal, or 'high,' small bowel obstruction. The finding is typically pathologic, and implies either duodenal atresia, duodenal web, annular pancreas, or on occasion midgut volvulus, a distinction that requires close clinical correlation and, in most cases, surgical intervention.

References

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  3. Yan, Bing; Zhang, Kun (2023-12-01). "Midgut volvulus due to congenital intestinal malrotation with an ileal duplication cyst in an adult: An unusual case report". Asian Journal of Surgery. 46 (12): 5815–5816. doi: 10.1016/j.asjsur.2023.08.160 . ISSN   1015-9584.
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