AUTHOR AND EDITOR INFORMATION

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
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INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Background

The study and treatment of malrotation and volvulus have a long and intriguing history. In 1832, Reid described a malpositioned cecum, found in the left upper quadrant, in 2 autopsies.¹ However, the landmark work of Mall in 1898 first contributed to the understanding of normal embryological GI rotation and development, resulting from his study of 41 cadavers.²

Over the next 35 years, studies continued on intestinal rotation, and case reports of malrotation began appearing in medical literature, along with attempts at treatment and correction. In 1932, Ladd published his landmark paper on congenital duodenal obstruction and presented a surgical correction of malrotation.³ This technique has survived the past century with only minor modifications to form the basis of surgical treatment today. The understanding of GI embryology heavily contributed to the development of a successful treatment and has remained vital to the ability to recognize clinical presentations and implications of this abnormality.

Pathophysiology

A good comprehension of the embryological development of the GI tract is central to the understanding of malrotation and volvulus. At 4 weeks' gestation, the GI system is a straight tube centrally located in the abdomen. During the fifth week, the first stage of rotation begins and lasts until the tenth week. The tube lengthens and herniates into the umbilical cord carrying its blood supply, the superior mesenteric artery (SMA), with it as an axis; it is in line with the omphalomesenteric duct, which is located at the apex of the herniation.

The prearterial segment includes the developing duodenum, jejunum, and much of the ileum, while the postarterial segment becomes the terminal ileum, cecum, ascending colon, and part of the transverse colon. While in this position, the developing midgut begins to rotate. The duodenojejunal (DJ) loop is first pushed inferiorly and to the right, possibly by the developing liver. Rotation is in a counterclockwise direction and continues in this fashion. This completes the first 90° of rotation. The cecocolic loop follows, moving to the left of the artery. The DJ loop then completes a second 90° of rotation, coming to rest inferior to the SMA.

The second stage of rotation occurs at 10-12 weeks' gestation and involves the return of the intestine to the abdominal cavity. Upon return of the prearterial segment, the DJ loop completes another 90° of rotation for a total of 270°, coming to rest to the left of the SMA. The cecocolic loop also completes another 90° of rotation, coming to rest superior and anterior to the SMA.

The third and final phase of rotation occurs after 12 weeks' gestation. The cecum completes its final descent to the right lower quadrant, accomplishing another 90° of rotation or 270° total. Once this is completed, the intestine becomes fixed to the posterior abdominal wall. The DJ junction is fixed to the left of the aorta, anterior to the left renal vein at the ligament of Treitz, and the cecum forms attachments to the right iliac fossa. These 2 points of attachment leave the mesentery and its blood supply broad-based and fixed. Arrest of development at any stage not only changes anatomical placement of organs but, perhaps most importantly, narrows the mesenteric base and impairs fixation, leaving the bowel at high risk for volvulus.

The common rotational abnormalities can be divided into stage of occurrence. Arrest of the first phase leaves the bowel herniated into the umbilical cord, creating omphalocele. This is often accompanied by other congenital abnormalities. Developmental arrest during the second phase may result in nonrotation, incomplete rotation, hyperrotation, or reversed rotation. In addition, gastroschisis and diaphragmatic hernias occur during this period. Mesocolic paraduodenal hernias also occur during the late second phase and early third phase. Failure of the third phase results in a mobile cecum, unattached duodenum, or unattached small bowel mesentery, which allows cecal volvulus and internal hernias.

Nonrotation is the most common abnormality and results from failure of normal counterclockwise rotation around the SMA. Rotation may be completely absent or arrested at 90°. The small intestine remains entirely to the right of the artery, with the cecum at or near the midline and the colon in the left abdomen. The proximal jejunum and colon pass very closely to the SMA, leaving a narrow pedicle as the base of the mesentery. This is sometimes called the "bell-clapper" or the omega configuration and leaves the midgut highly susceptible to volvulus and infarction. Also, the peritoneal attachments of the cecum pass anterior and lateral to the duodenum, leading to future extrinsic partial obstruction.

Incomplete rotation is the next most common abnormality and involves arrest of rotation at or near 180°. This arrest leaves the cecum in the upper abdomen and the DJ junction short of its left abdomen destination. In this case, the SMA pedicle is also narrow and susceptible to volvulus. Cecal peritoneal bands once again cross over the duodenum to the right upper quadrant. These abnormal cecal attachments are called Ladd bands. In both nonrotation and incomplete rotation, cecal bands may cause obstruction and ischemia before birth, leading to congenital duodenal atresia or stenosis. Therefore, patency of the duodenum should be checked routinely during corrective surgery. In addition, intermittent antenatal volvulus may cause varying degrees of ischemia, leading to atresia or duplication of parts of the jejunum and ileum.

Mixed rotational abnormalities are more uncommon and varied. Hyperrotation may narrow the SMA pedicle or lead to a confusing clinical picture in appendicitis. Failure of descent of the cecum may be inconsequential or lead to cecal volvulus. Reversed rotation results in a 90° clockwise rotation, leaving the transverse colon behind the SMA and the duodenum anterior to the artery. In this case, the transverse colon may herniate into a mesocolic pocket or become obstructed by the SMA.

Mesocolic paraduodenal hernias occur with failure of fixation to the posterior abdominal wall. Spaces remain between the mesentery and the wall, allowing possible sequestration and strangulation of bowel segments. This occurs more often on the left and is associated with nonrotation. Right-sided hernia is associated with congenital short bowel.

Volvulus of the midgut may result in several manifestations. Venous and lymphatic obstructions occur first because of lower intravascular pressures. Vascular congestion leads to bowel edema and possible oozing of blood, causing melena. Lymphatic congestion causes the formation of a mesenteric cyst and chylous ascites. If volvulus is intermittent, children may have chronic malabsorption from congestion and edema or intermittent bouts of symptoms, usually vomiting and alternating diarrhea and constipation. Arterial compromise requires higher obstructive pressures but is more acutely dangerous. Sequelae include ischemia, mucosal necrosis, intramural air formation, gram-negative sepsis, perforation, peritonitis, and death.

Malrotation is often associated with other congenital abnormalities, and, in some hospitals, as many as 62% of cases are associated with these abnormalities. This is important for several reasons. First, some abnormalities are highly associated with malrotation, such as polysplenia, asplenia, and congenital abdominal wall defects. The presence of these abnormalities should automatically lead to investigation for malrotation. Patients with congenital wall defects by nature have some aspect of malrotation, which should be examined and fixed during correction of the defect. Rotational anomalies in conjunction with dextro-isomerism or levo-isomerism should point to polysplenia or asplenia syndrome. These syndromes have devastating effects on outcomes, especially asplenia.

Perhaps most importantly, mortality related to malrotation and its correction highly correlates with the presence or absence of other congenital abnormalities. Serious anomalies may raise the risk of mortality 22 times. The pediatrician and surgeon must be conscientious in exploring the possibilities of associated congenital abnormalities.

Frequency

United States

Malrotation is estimated to occur in 1 per 500 live births. Autopsy studies have shown incidence of malrotation in 0.2-1% in the total population; however, clinical discovery is achieved in only 1 per 600 live births. Increasingly, many cases are diagnosed at laparotomy for other congenital abnormalities as physician awareness of associated anomalies improves. A mobile cecum or a high cecum (found in the right upper quadrant) may be considered a minor rotational abnormality but is considered a normal finding in the absence of symptoms and has been recorded in 6-16% of the total population. Its incidence is slightly higher in the immature infant abdomen and may confuse the diagnosis of true malrotation.

International

Incidence internationally is similar to incidence in the United States.

Mortality/Morbidity

Morbidity and mortality of malrotation and volvulus has significantly decreased since the advent of surgical correction. Early figures on the mortality rate ranged from 23-33%, mostly resulting from bowel dysfunction and malnutrition. With the development of total parenteral nutrition and improved nutritional support, survival has increased. In addition, improved knowledge of pathophysiology and the correlation of associated anomalies have allowed for more rapid diagnosis. Clinical awareness of the association of malrotation with other anomalies and increased sensitivity to the importance of more minor symptoms has led to improved diagnosis, allowing for correction of malrotation before life-threatening volvulus occurs. The significance of rapid diagnosis is evident in the high mortality rate (£33%) still shown with diagnostic delay, especially in very young persons. Present mortality figures range from around 3-9% overall.

• In 1992, Messineo et al determined that the most important factors influencing mortality are the presence of necrotic bowel, associated abnormalities, and younger age.⁴ Risk of mortality increases 25 times in the presence of bowel necrosis and is influenced heavily by length of viable bowel. They determined that the survival rate is maintained above 93% with 10%, 25%, and 50% of the bowel necrosed; however, the survival rate drops to 35% with the loss of 75% of the length of the small bowel. The presence of other serious abnormalities also increases the risk of mortality by a factor of 22. Given the high association of malrotation with other congenital anomalies, this risk factor heavily affects mortality figures. In addition, Messineo et al found that patients who died after surgery had a significantly lower median age (4 d versus 30 d).
• The most common cause of death in these patients is sepsis. Other noted causes are pneumonia, intracranial hemorrhage, short-bowel syndrome, malnutrition, renal failure or hepatic failure, continued small bowel obstruction, and other life-threatening anomalies.
• Causes of postoperative morbidity are adhesive obstruction, short-bowel syndrome, and recurrent volvulus. Adhesive obstruction has been noted in 1-10% of these patients postoperatively and can contribute to renewed symptoms of partial to complete obstruction. Short-bowel syndrome may occur after resection of a significant length of necrotic bowel. Children with malrotation and volvulus comprise 18% of the population of patients with short-bowel syndrome, which can result in chronic learning and psychomotor disabilities as well as the obvious risk of malnutrition. Midgut volvulus recurs after the Ladd procedure in 7% of cases. This has resulted in several attempts over the past century to augment the Ladd procedure with fixation of the cecum and duodenum; however, overall statistics have not shown improvement in outcome. Therefore, patient education on the signs and symptoms of obstruction and volvulus has proved the only effective prevention of the consequences of recurrent volvulus.
• In general, in the absence of other significant abnormalities or bowel necrosis secondary to midgut volvulus, morbidity and mortality of malrotation and its correction are low, and these children can be expected to do well, with an excellent chance of total resolution of related symptoms. Volvulus has been reported in 45-80% of infants with malrotation and is much more life threatening. Surgical correction is highly recommended as long as other systems are stable enough to undergo the procedure. In addition, malrotation places the infant at increased risk for intussusception. Waugh syndrome, the presence of intussusception and malrotation, was first described in 1911. Poor fixation of the cecum allowing easier ileal advancement is noted in almost all infants with intussusception and is central to the pathophysiology of malrotation.
• Patients who are asymptomatic and whose condition is undiagnosed before age 2 years may never become symptomatic; however, without operation, malrotation results in volvulus in 1 out of 3 throughout a lifetime.

Race

No known racial difference has been reported.

Sex

During the first week of life, the ratio of male-to-female presentation is 2:1. In the 25% of patients diagnosed with this condition after age 1 year, the male-to-female ratio equalizes, creating an overall ratio of 3 males to 2 females. Reasons for the slight male predominance are largely unknown.

Age

The presentation of symptomatic malrotation is much more common in younger children. Children younger than 1 year account for 75-90% of cases, with 50-64% of cases occurring in infants younger than 1 month and 25-40% of cases occurring in the first week of life.

CLINICAL

Section 3 of 11  

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History

No unique signs or symptoms pathognomonic for intestinal malrotation and volvulus are recognized; however, the most common presenting symptom is bilious vomiting (ie, vomiting yellow or green). Physicians must recognize that bilious vomiting can occur in various other medical conditions, but, given the significant morbidity and mortality of midgut volvulus, any patient with bilious vomiting must be considered at risk for having a midgut volvulus until proven otherwise. See Media files 5-6.

• Malrotation
• • The typical history of a patient with intestinal malrotation depends on age at presentation and degree of obstruction.
  • Infants who present in the first 24 hours after birth through the first week of life tend to have more severe obstruction. They present with bilious vomiting and feeding intolerance, as expected of a high postampullary obstruction. They may also have upper abdominal distension.
  • Slightly older infants often also present with bilious vomiting and have a history of tolerating at least some feeds. After age 2 months, bilious and nonbilious vomiting occurs with similar frequency.
  • Other symptoms include anorexia, intermittent apnea, and failure to thrive. Parents may report constipation, which results from obstruction as well as dehydration due to decreased fluid intake. Bloody stools may signify volvulus.
• Malrotation in older children
• • Older children present more insidiously. Symptoms include cyclic vomiting, immunodeficiency, and protein-calorie malnutrition.
  • Malrotation may cause secondary illness such as peptic ulcer disease due to gastroduodenal stasis or intussusception caused by lack of ileal fixation.
  • Altered anatomy may also cause a delay in the diagnosis of appendicitis.
  • In general, younger children are more likely to present with acute symptoms lasting less than 48 hours.
  • An abrupt change from feed tolerance to vomiting and irritability at any age is suspicious for volvulus in the presence of known malrotation.
• Volvulus
• • Malrotation and volvulus are 2 distinct entities. Malrotation may cause intermittent and incomplete signs and symptoms of proximal intestinal obstruction with mesenteric congestion. If volvulus has developed as a consequence of intestinal malrotation, the obstruction is typically complete, and compromise to the blood supply of the midgut has started (because of the twisting of the mesentery at the narrow pedicle of the superior mesenteric artery). Media files 5-6 illustrate the operative appearance of a midgut volvulus. Thus, the signs and symptoms depend on the degree of ischemia. It can range from lymphatic and venous congestion with simple edema to full intestinal necrosis secondary to arterial and venous thrombosis. See Media file 8.
  • Once intestinal ischemia develops, pain becomes a more pronounced symptom, and the patient may have signs of an acute abdomen with rigidity and tenderness to palpation.
  • Because superior mesenteric artery (SMA) territory includes the segment from the proximal duodenum to the mid transverse colon, the entire midgut may become necrotic and nonviable if the volvulus is not corrected in time. Necrosis of the entire midgut is incompatible with life.

Physical

• Physical findings in malrotation are predictable because of the anatomy.
• • Abdominal distension may be present in the epigastric area, especially in infants younger than 1 year. Distension may be absent even in this age group if vomiting has cleared gastric contents.
  • Peristaltic waves may also be visible in the epigastrium moving obliquely down from right to left.
  • Bilious or nonbilious emesis may be present.
  • Infants may also show evidence of dehydration and weight loss.
  • Signs of shock combined with bloody diarrhea and abdominal distension usually signify volvulus and possible gangrene. Such patients need urgent laparotomy as soon as possible.
• Physical examination findings in older children may be more subtle and nonspecific.
• • Evidence of weight loss and malnutrition may be present, and stools may contain occult blood in the case of intermittent volvulus.
  • A secondary disease process, such as peptic ulcer disease, may be the only presenting sign.
  • Inconclusive findings on examination of an older child with a chronic history of vomiting and nonspecific symptoms warrant radiologic studies to rule out malrotation or other obstruction.
• Physical findings associated with volvulus are as follows:
• • As previously mentioned, in the event of malrotation with midgut volvulus, vascular compromise develops and this determines the severity of the clinical presentation.
  • The physical signs may vary from mild abdominal pain to severe pain with an acute abdomen or even a shocklike picture with dehydration, lethargy, and respiratory distress.

Causes

Specific causes of malrotation are unknown, although repeated associations have been made with congenital syndromes such as Down syndrome and VACTERL (vertebral, anal, cardiac, tracheal, esophageal, renal, limb) association of anomalies. Because malrotation is an embryological development abnormality, the assumption can be made that any interference during critical periods may lead to malrotation.

Associated anomalies include the following:

• Trisomy 21
• Congenital heart disease
• Imperforate anus
• Ileal and jejunal atresia or duplication
• Omphalocele
• Duodenal atresia and stenosis
• Diaphragmatic hernia
• Meckel diverticulum
• VACTERL (vertebral, anal, cardiac, tracheal, esophageal, renal, limb) association of anomalies
• Trisomy 13, trisomy 18
• Esophageal atresia
• Situs inversus and asplenia (may be associated with biliary atresia)
• Kidney abnormalities
• Agenesis of corpus callosum
• Pyloric stenosis
• Annular pancreas
• Erythroblastosis
• Intussusception
• Cystic fibrosis
• Meconium ileus
• Hirschsprung disease
• Duodenal web
• Biliary atresia

DIFFERENTIALS

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Other Problems to be Considered

The differential diagnosis of malrotation is that of high obstruction. If volvulus has occurred causing necrosis in the first week of life, necrotizing enterocolitis may be included in the differential. In older children, symptoms are often more subtle and chronic. Vomiting may be accompanied by alternating diarrhea and constipation. The differential diagnosis can then be expanded to include inflammatory bowel disease, gastroesophageal reflux disease (GERD), celiac disease, Crohn disease, and ulcerative colitis.

In the past, many children with malrotation were diagnosed with psychogenic vomiting. Upon diagnosis and surgical correction, all symptoms disappeared. This diagnostic omission is less likely today, but the example emphasizes the insidiousness of symptoms in older children and reminds physicians to consider the diagnosis in the differential for a child presenting with intermittent abdominal symptoms.

In addition to the differential diagnosis listed above, consider the following:

• Duodenal stenosis
• Duodenal web
• Annular pancreas
• Increased intracranial pressure (intracranial hemorrhage, hydrocephalus, intracranial mass)
• Primary gastric atony
• Chronic renal failure

WORKUP

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• Follow-up
• Miscellaneous
• Multimedia
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Lab Studies

A few laboratory studies may be helpful in the diagnosis and support of a patient with malrotation.

• Stool heme test
• • Findings ranging from occult to frank blood may be present in the stools of a child with malrotation; therefore, heme testing of the stools of a child suspected to have malrotation may be useful.
  • The presence of blood suggests volvulus, at least intermittently, and should encourage expeditious transfer to the operating room once diagnosis is confirmed. However, the presence of melena or currant jelly stools is not necessarily predictive of bowel necrosis. Viable bowel may ooze blood secondary to venous congestion without arterial obstruction.
• Hematologic studies
• • CBC count monitoring helps the physician assess the severity of illness.
  • Ischemia may cause an elevation in the WBC count. A very high WBC count may ominously suggest gangrene and sepsis.
  • A decrease in hemoglobin and hematocrit may occur with venous oozing.
• Chemistry studies
• • Regular electrolyte monitoring also assists the physician in assessing the severity of disease as well as directing management and support.
  • Abnormal electrolytes are a result of dehydration, sepsis, and acidosis.
  • Bowel edema caused by volvulus and obstruction can lead to shifts of large amounts of fluid and electrolytes into the interstitial space and bowel lumen, leaving the patient intravascularly depleted even without the vomiting and diarrhea that often accompany this condition.
  • Common electrolyte abnormalities in such cases include hyponatremia, hyperkalemia, metabolic acidosis, increased BUN and creatinine levels, hypochloremia, and lactic acidosis.
  • Careful monitoring and aggressive support of fluid and electrolyte status can treat or prevent dehydration and subsequent worsening of vascular compromise due to hypotension.

Imaging Studies

• Imaging studies are an integral part of the diagnostic process for a patient suspected of having malrotation or other GI obstruction.
• First, obtain abdominal radiographs, preferably in 4 views (ie, supine, prone, upright, and lateral recumbent). These views assist in determining the level of obstruction.
• • The classic sign on abdominal radiography is the double-bubble. Duodenal obstruction causes gastric and duodenal distension with a prominent duodenal bulb and air-fluid levels in the proximal duodenum and stomach on upright radiographs. The double-bubble may not be evident if the patient's vomiting has cleared the proximal GI contents. In an older child, the sign may be absent during an asymptomatic period.
  • Accompanying the double-bubble is often a relative paucity of lower GI gas. This is highly suggestive of volvulus or duodenal stenosis and atresia.
  • A double-bubble sign with paucity of lower GI gas combined with clinical signs and symptoms of distress, such as fever, lower abdominal distension, melena, and/or hemodynamic instability, suggest volvulus and possibly gangrene and should lead directly to laparotomy. In this situation, pneumatosis coli may be observed on plain radiographs and is an ominous sign. Nonspecific radiographic findings are more common in older children. The chronicity of their condition usually allows some adaptation. Their obstruction, by necessity, must be more partial and intermittent, allowing GI contents and gas passage into lower GI areas.
  • Normal findings on plain radiographs in a child of any age with clinical signs and symptoms of malrotation can lend a false sense of security to the physician, creating a dangerous situation for the patient. In the presence of clinical suspicion, contrasted studies are warranted for confirmation.
• Contrast studies
• • Confirmation and definitive diagnosis are accomplished more easily with an upper GI contrast series. If volvulus is present, signs include incomplete duodenal obstruction, usually at the third portion of the duodenum (see Media file 1). The proximal duodenum is often dilated with a "bird-beak" obstruction (see Media file 2) and a spiral or corkscrew duodenal configuration (see Media file 1). This is a result of the abnormal positioning and the adhesive obstruction of Ladd bands.
  • The duodenojejunal (DJ) junction in malrotation is misplaced, either at or to the right of the midline. The fourth part of the duodenum and the ligament of Treitz are not visible to the left of the midline between the lesser and greater curvatures of the stomach.
  • Although most malrotations may be diagnosed by following the passage of contrast to the ligament of Treitz, continuing the study through the small bowel is important. Here the proximal jejunal loops are observed in an abnormal position, usually leading down to the right lower quadrant. The walls of the small bowel may also be thickened.
  • If the findings on the upper GI study are nonspecific and suggestion of malrotation continues, a contrast enema may be administered, preferably of barium. The sign sought with this study is an abnormally placed cecum, high in the right upper quadrant, midline, or on the left side. Barium enema (BE) results can be obscured. A high or mobile cecum can be considered normal in the absence of other abnormalities and is present in as much as 16% of the population. Additionally, barium often refluxes into the terminal ileum, confusing the identification of the cecum, especially in the newborn.
• Upper GI series versus lower GI series
• • Over the past 7 decades, controversy has emerged over the use of the upper GI versus the contrasted enema. Physicians have been reluctant to administer a barium meal to infants suspected of obstruction, hence the popularity of the contrasted enema. The upper GI series provides more information regarding the degree of obstruction and actual location of proximal GI anatomy.
  • BE findings are more likely to be inconclusive, and, furthermore, BE impedes reading of any subsequent upper GI study.
  • In 1992, Ford et al found that a limited upper GI study using 5 mL of barium was well tolerated and allowed visualization of anatomy and subsequent BE if needed, such as in the case of complete obstruction with necessary observation of lower anatomy.⁵ Media file 3 illustrates the appearance of a BE in a patient with malrotation.
• Nuclear imaging: Rarely, lymphatic obstruction leads to mesenteric cyst formation and chylous ascites. In this case, a dense liver can be visualized pushed medially by chylous ascites (less dense) in the paracolic gutters.
• Ultrasonography and CT scanning
• • The use of ultrasonography and CT scanning in the diagnosis of malrotation has also been investigated.
  • In 1992, Dufour et al examined more than 300 patients with ultrasonography.⁶ Finding the superior mesenteric vein (SMV) to the left of the superior mesenteric artery (SMA) was highly suggestive of malrotation, whereas an anterior SMV was questionable. Normal positioning of the mesenteric vessels was found in 326 patients, 9 of whom had malrotation. Based on these results, Dufour et al recommended upper GI studies for any suspected malrotation; however, if ultrasonography is used to evaluate abdominal symptoms, useful information may be obtained from the placement of the mesenteric vessels.
  • Reminding physicians that patients with malrotation always have a left-sided SMV postoperatively is helpful.
  • If volvulus has occurred, CT scanning reveals a whirllike pattern to the SMA (see Media file 4).
  • Ultrasonography may reveal a midline abdominal mass with dilated collateral mesenteric vessels and whirling small intestinal loops, shown as sonolucent layers.
  • These studies may be useful as adjuncts or to encourage evaluation for malrotation and volvulus, but definitive diagnosis is made based on findings from contrast upper GI series, BE, or laparotomy.

Histologic Findings

The histology of the affected bowel is related to the degree of vascular compromise. The venous obstruction caused by volvulus may cause venous engorgement and edema. Arterial obstruction can result in various stages of ischemia from a mild degree to frank necrosis and gangrene, complete with neutrophilic infiltrate.

Lymphatic obstruction, which results in chylous ascites, may show enlargement of the lacteals, which are found within the intestinal villi. In the case of malrotation without volvulus, bowel histology may be normal.

Associated abnormalities cause their own histologic changes. For instance, peptic ulcer disease has a high correlation with malrotation in older children and adults; therefore, gastric and duodenal erosions may be present.

TREATMENT

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Medical Care

The definitive treatment of malrotation and intestinal volvulus is surgical correction. Medical care revolves around support of the patient preoperatively and postoperatively, treatment and/or stabilization of any coexisting conditions, and expedition of transport to the operating room.

Of note, nonoperative treatment might be appropriate for older patients that have intestinal malrotation and are truly asymptomatic. However, the patient and the family must be made aware of the fact that intestinal volvulus may occur at any time and that they need to seek immediate medical attention if any GI symptoms develop.

• Insertion of a nasogastric or orogastric tube begins GI decompression.
• Support the respiratory system with intubation and ventilation as needed.
• Prophylactic broad-spectrum antibiotics are administered and continued postoperatively if the bowel vascular supply is compromised, causing necrosis.
• Closely monitor fluid and electrolyte status, with intravenous fluids administered at least at maintenance levels and increased for any signs of dehydration and/or fluid shifts.
• Electrolytes are aggressively replaced.
• Foley catheter placement may be appropriate, especially in older children.
• In addition, if volvulus has not yet occurred, closely monitor for acute changes in symptoms (eg, vomiting, distension, fever, hemodynamic instability), which may show that volvulus has occurred.

Surgical Care

The basis of surgical correction of malrotation is to free obstruction and to widen the base of mesenteric attachment. Several approaches were attempted with some success before Ladd first described a reliable technique in 1932. His approach placed the intestines in a prior embryological state but accomplished the greatest stability to that point. His procedure survives, relatively unchanged, as the most widely used technique.

• Ladd procedure
• • The Ladd procedure uses a transverse incision through the right rectus muscle in the right upper quadrant. This incision allows the greatest visualization and access to the anatomy.
  • The surgeon must quickly explore the anatomical defect and then completely eviscerate the midgut.
  • Chylous ascites is occasionally present. This turbid fluid may appear infected but is actually sterile.
  • In most patients, volvulus is corrected by rotating the small intestine in a counterclockwise direction (see Media file 7).
  • Thoroughly examine the bowel for signs of necrosis and cover the bowel with warm sponges while correcting malrotation to allow restoration of blood flow.
  • The surgeon should dissect the SMA pedicle of any constricting peritoneal attachment all the way to the pancreas to facilitate the widening of the pedicle. Ladd bands, which constrict the duodenum, are thoroughly divided, using a Kocher maneuver to ensure resolution of restriction.
  • The cecum is placed in the left abdomen, and the duodenum is directed down the right paravertebral gutter.
  • Closing or widening any defect to prevent future strangulation can repair mesocolic hernias.
  • Appendectomy is performed in the absence of cecal necrosis and/or the placement of prosthetic material, preventing later confusion in diagnosing appendicitis. At this point, conservatively resect the necrotic bowel, leaving questionable bowel in situ, because edematous and ischemic bowel may regain function over the next few days.
  • A second-look laparotomy may be used 36 hours later to ensure viability of the remaining bowel.
  • Focus all efforts on leaving most of the bowel intact if possible because necrosis and resection of large amounts of bowel cause long-term nutritional problems and may lead to death.
  • In the tragic case of total midgut infarction, close the abdomen with the entire bowel intact, and provide terminal care. Assure duodenal patency before closure.
  • Many techniques have been used, such as the injection of air and saline, but the most successful approach is the use of a large Fogarty embolectomy catheter or other appropriately sized balloon-tipped catheter. The catheter is placed orogastrically or nasogastrically and passed through the duodenum. Then, the balloon is inflated and retracted through the duodenum. Atresia prevents the complete passage of the catheter, while stenosis or a duodenal web or diaphragm causes tension on the intestinal wall upon retraction of the catheter.
  • Transluminal repair of any obstruction is preferred if possible, or resection with end-to-end reanastomosis is performed.
• Abdominal closure
• • If the abdominal wall cannot be closed without causing abdominal compartment syndrome, a silo may be placed for temporary closure. This technique has been used with some significant success.
  • Edematous bowel may place excessive pressure on the diaphragm, great vessels, and kidneys, causing hemodynamic, respiratory, and renal compromise.
  • A silo optimizes cardiorespiratory status while optimizing blood flow through the SMA. Additionally, the silo may allow a bedside second-look, thus decreasing expenses and transport, which is especially pertinent in the care of premature neonates.
  • Surgeons must use clinical judgment in the assessment of abdominal pressures for the purpose of silo application.
• Role of laparoscopy
• • A laparoscopic variation of the Ladd procedure has been used in some centers, with the general advantage of decreased scarring and adhesions; however, good visualization of the entire bowel is vital to the accurate resection or preservation of ischemic bowel, and a laparoscopic approach impairs full examination of the intestine.
  • Although adhesions may create obstructive problems in 1-10% of patients, they may also provide stability to the new intestinal placement.
• Role of surgical fixation during the Ladd procedure
• • The 7% recurrence rate of volvulus after the Ladd procedure has encouraged surgeons to attempt fixation of the cecum and/or duodenum. In 1966, Bill reported successful results with fixation of the cecum in the left lower quadrant with and without duodenal fixation.⁷
  • Other authors have argued that volvulus recurrence rates remain unchanged, and that continued abdominal symptoms are more common after fixation; therefore, in general, cecal and duodenal fixation are not widely used today. Applications may be noted; in 1992, Ford et al used colopexy and duodenopexy only in rare cases of persistent narrow SMA pedicle despite dissection of peritoneal attachments with good results.⁵
  • In 1975, Gohl and DeMeester used the less well-known Fitzgerald technique with good results in adults.⁸ This approach places the bowel in the mature anatomic position. The abdomen is entered through a midline incision. The surgeon then lyses bands and peritoneal attachments to mobilize the bowel. A new retroperitoneal bed is created in the right paravertebral gutter for the placement of the ascending colon, which is then secured laterally. The small bowel is pulled under the base of the colonic mesentery, with fixation of the duodenum medially and a new ligament of Treitz at the duodenal exit beneath the transverse mesocolon. Historically, most authors have reported difficulty with the creation of a new ligament of Treitz of the proper tension. Fixation may also contribute to continued symptoms.
• Management of other associated anomalies
• • Coexisting conditions may complicate clinical decisions. Stabilize congenital heart defects before the Ladd procedure in the absence of ischemic volvulus.
  • In the case of malrotation and Hirschsprung disease, surgery is also delayed, if possible, until a simultaneous pull-through can be accomplished. In these cases, educate patients and parents on obstructive symptoms.
  • In Waugh syndrome, intussusception can often be reduced with contrast enema. The Ladd procedure actually adds to hospital stay and expenses, and it appears inefficient. However, intussusception is likely to recur if the anatomical defect remains.
  • Abdominal wall defects may contribute to thickening and shortening of the bowel, making anatomical identification and correction difficult.
• Postoperative care
• • This depends on the presence of other abnormalities, which should be treated accordingly, and the presence of necrotic bowel.
  • If necrosis and resection have occurred, nutritional support becomes an integral part of medical care. Total parenteral nutrition is needed acutely and may be continued long term.
  • The patient needs close monitoring for several days after surgery because questionable bowel may recover or become necrotic.
  • In addition, the occurrence of volvulus, although low in incidence, remains a possibility.
  • Any signs and symptoms of obstruction should be noted by physicians and family members and attended to quickly.

Consultations

• Involve a pediatric surgeon early in the care of a patient suspected to have malrotation or intestinal volvulus.
• The surgeon, pediatrician, and an experienced radiologist should be directly involved in the performance of imaging studies.
• In the case of volvulus, rapid procession to the operating room may be necessary, and all facilitating measures should be taken.

Diet

• The determination of diet postoperatively depends on the degree of bowel distress. Markedly edematous bowel may take longer to recuperate, delaying the tolerance of oral feeds.
• Total parenteral feeds may be necessary in the interim period.
• In addition, necrosis of the bowel necessitates removal. If a significant portion of the bowel is removed, the child may develop short-bowel syndrome. This condition requires a lifetime of dietary modifications and possibly long-term hyperalimentation supplementation.
• However, in the absence of complicating factors, feeding can resume as soon as the bowel recovers and toleration begins.
• No special diet is required.

Activity

No specific restrictions regarding postoperative activity are indicated. Age-appropriate activity is always encouraged as soon as tolerated after surgery, barring other restricting abnormalities.

MEDICATION

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• Workup
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• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

No medical therapy is indicated in the management of malrotation and intestinal volvulus. Treatment is surgical correction. Observation may be used in the patient for whom surgery is not appropriate (eg, awaiting the stabilization of congenital heart defects). Broad-spectrum antibiotics are used preoperatively (postoperatively, if needed) and should be chosen to cover both skin and enteric flora.

FOLLOW-UP

Section 8 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Further Inpatient Care

• Postoperatively, transfer the patient to an intensive care unit (ICU) and observe for signs of deterioration or recurring volvulus.
• The patient should remain in the ICU at least until the second-look laparotomy and longer if indicated.
• Patients continue to need aggressive fluid and electrolyte support.
• Consider total parenteral nutrition early because edematous bowel may recuperate slowly.
• Use antibiotics in the presence of necrotic bowel.

Transfer

• The child with malrotation should have access to a hospital system with critical care facilities appropriate for the child's age and an experienced staff, including a pediatrician, pediatric surgeon, and radiologist. If these resources are not available, transfer the patient to a facility that can provide them.

Complications

• The most common postoperative complications are adhesive obstruction, short-bowel syndrome, and recurrent volvulus.
• Patients who have large portions of necrotic intestine that require resection have chronic difficulties with short-bowel syndrome.
• Make resources available early to prevent any difficulties with long-term care.
• Educate the parents of these patients, as well as the patients themselves, if appropriate, to prevent difficulties resulting from complications.

Prognosis

• In the absence of serious associated abnormalities or major bowel resection, these children can be expected to do well.
• Adhesive obstruction and recurrent volvulus are the most common complications, and, with education, they can be recognized and easily treated. The occurrence of each is recorded at less than 10%; however, patients who develop short-bowel syndrome face nutritional challenges and, possibly, difficulties with learning capacity and psychomotor development. These children also have higher mortality rates.
• Patients with other congenital defects have a poorer prognosis, depending on the severity of the abnormality.
• Younger children are at higher risk and require more aggressive support and treatment. In general, rapid diagnosis and facilitation of correction improve overall prognosis in all age groups.

Patient Education

• Focus patient education on recurrence of symptoms after surgery or before surgery, if delayed.
• Patients and their family members should understand and be sensitive to any obstructive symptoms, especially signs and symptoms of volvulus.
• Any intolerance of food or continued abdominal symptoms may warrant reexploration of the abdomen.
• Also instruct patients to inform any physician they visit of this medical history, especially if the appendix is left in situ.

MISCELLANEOUS

Section 9 of 11  

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• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical/Legal Pitfalls

• The most significant problem is failure to recognize midgut volvulus in the differential diagnosis of a child with a history of bilious vomiting. One must remember that the signs and symptoms of an acute abdomen may be absent if intestinal ischemia and necrosis have not yet developed.

MULTIMEDIA

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• Follow-up
• Miscellaneous
• Multimedia
• References

Media file 1:  Upper GI contrast study of a 10-year-old patient with intestinal malrotation. Note the normal appearance of the stomach but the abnormally shaped duodenum. The duodenum does not have the C-loop appearance, it does not cross back over the midline (spine), and the proximal jejunum is located on the right side of the abdomen. In addition, this patient had a partial volvulus illustrated by the corkscrew appearance of the duodenum.
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Media type:  Radiograph

Media file 2:  Upper GI contrast study of a full-term newborn baby with bilious vomiting. Note the obstruction at the third and fourth portion of the duodenum with the "bird beak" appearance, which is compatible with a midgut volvulus and malrotation.
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Media type:  Radiograph

Media file 3:  Barium enema of a 1-year-old infant with a history of intermittent bilious vomiting. The colon is positioned abnormally, with most of the colon on the left side of the abdomen. Note the cecum and terminal ileum in the left upper quadrant of the abdomen.
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Media type:  Radiograph

Media file 4:  CT scan appearance of a 4-year-old patient with intermittent episodes of bilious vomiting. The CT scan was performed at the time of volvulation of the midgut. Note the "hurricane" or swirl appearance in the mid abdomen at the site of the narrow pedicle of the superior mesenteric artery, which is compatible with the acute twist observed during a midgut volvulus.
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Media type:  CT

Media file 5:  Operative photograph illustrating the midgut volvulus of the baby whose upper GI series was shown in Image 2. Note the complete twist (>360°) of the entire small bowel over the narrow pedicle of its mesentery. Note the appearance of the small bowel and congestion and cyanosis due to vascular compromise from the volvulus. Fortunately, early operative intervention prevented the development of necrosis, and emergent untwisting combined with a Ladd procedure was successful.
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Media type:  Photo

Media file 6:  Operative photograph of a midgut volvulus due to intestinal malrotation in a 10-year-old patient. Note the twisting at the base of the mesentery with evidence of intestinal congestion and ischemia but no necrosis.
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Media type:  Photo

Media file 7:  Diagram illustrating the operative maneuver to untwist the volvulized midgut. Note that the untwisting is performed in a counterclockwise fashion by the operating surgeon. Once this is accomplished, the Ladd procedure is completed by dividing any obstructing bands and by broadening the base of the mesentery.
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Media type:  Illustration

Media file 8:  Operative photograph of a patient with a midgut volvulus in which the diagnosis was made late. Note that the entire small bowel is necrotic and nonviable. This infant did not survive.
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Media type:  Photo

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

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Article Last Updated: Sep 11, 2008