Intestinal volvulus is defined as a complete twisting of a loop of intestine around its mesenteric attachment site. It is related to but not precisely synonymous with malrotation, a more general term used when the normal process of rotation and fixation of the midgut goes awry. Malrotation may lead to 2 critical complications: mechanical obstruction of the proximal intestine and twisting of the intestines with subsequent ischemia (with or without necrosis) of part or all of the midgut.
Volvulus can occur at various sites in the gastrointestinal (GI) tract, including the stomach, small intestine, cecum, transverse colon,[1] and sigmoid colon. Midgut volvulus refers to twisting of the entire midgut around the axis of the superior mesenteric artery (SMA). Patients with intestinal malrotation are at risk for developing a midgut volvulus. This article primarily focuses on midgut volvulus because it is the most common type of volvulus and it can have devastating consequences if not promptly recognized. Midgut volvulus secondary to intestinal malrotation is more common in infants and children, but it can occur in persons of all ages. Sigmoid volvulus, more common in elderly persons, is also briefly discussed.
Volvulus involving the GI tract can cause severe clinical problems; the most feared complication is ischemia and necrosis of the entire midgut, which can be fatal.
In 1832, Reid described a malpositioned cecum, found in the left upper quadrant, in 2 autopsies.[2] Mall first described the process of normal intestinal rotation during the embryologic period in 1898.[3] The first clear description of anomalies of intestinal rotation and fixation (the clinical manifestations of which correlated with the observations of the embryologist His) was published by Dott in 1923.[4]
In 1932, Ladd, the father of pediatric surgery in North America, published his landmark paper on congenital duodenal obstruction and presented a surgical correction for the anomaly described as congenital intestinal malrotation.[5] This technique has been adopted as the standard therapy worldwide and is still the basis of surgical treatment today. Minor modifications have been reported, including the use of minimally invasive surgical techniques. The understanding of GI embryology heavily contributed to the development of a successful surgical treatment for this condition. The anatomical understanding of malrotation and volvulus is considered vital for the early recognition of the clinical presentations and implications of this abnormality.
Laboratory findings are nonspecific. Imaging studies are an integral part of the diagnostic process for a patient suspected of having malrotation or other GI obstruction. Confirmation and definitive diagnosis are accomplished more easily with an upper GI contrast series, but other imaging modalities can also be used (as will be discussed here).
The management of abnormalities of rotation and volvulus is well established. Treatment of midgut volvulus secondary to congenital intestinal malrotation is surgical; no other effective treatment is available. High priority must be placed on early recognition of midgut volvulus before serious complications develop. The surgical techniques used to treat this condition are discussed in this article.
The midgut is that portion of the intestine supplied by the SMA. It includes the distal duodenum, all of the jejunum and ileum, and the colon as far as the mid transverse. Normally, the duodenum courses posterior to the SMA and the superior mesenteric vein (SMV), and the transverse colon lies anterior to these vessels.
The bowel is relatively fixed at several points: the duodenum (including the duodenojejunal [DJ] junction), the ascending colon, the splenic flexure, and part of the descending colon. If the normal process of rotation and fixation fail to occur, the midgut may not be normally arranged and lacks physiologic points of fixation, and thus may be subject to twisting or volvulus.
A good understanding of the embryologic development of the GI tract is central to the understanding of malrotation and volvulus. At 4 weeks’ gestation, the GI system is a short, straight tube centrally located in the abdomen. During the fifth week, the first stage of rotation begins and lasts until the tenth week. As this tube grows and lengthens, it must leave the domains of the fetal abdominal cavity and thus herniates into the area of the umbilical cord, carrying its mesentery with its blood supply (ie, the SMA) as a central 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, the jejunum, and much of the ileum, whereas the postarterial segment becomes the terminal ileum, the cecum, the ascending colon, and part of the transverse colon.
While in this position (partially outside the fetal abdomen), the developing midgut begins to rotate. The 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 fetal abdominal cavity. Upon the 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 for a total of 270°. Once this descent 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. At this point, the GI tract has returned to the peritoneal cavity and the abdominal wall of the fetus has completely formed and closed at the level of the umbilicus.
Arrest of development at any stage not only changes the anatomic placement of organs but, perhaps most important, narrows the mesenteric base and impairs fixation, leaving the bowel at high risk for volvulus.
Rotational abnormalities
The common rotational abnormalities may be broadly grouped according to the stage at which they occur, as follows:
Arrest in the first phase leaves the bowel herniated into the umbilical cord region, covered by a membrane, creating the so-called omphalocele; this is often accompanied by other congenital abnormalities (eg, cardiac and urogenital defects)
Arrest in the second phase may result in nonrotation, incomplete rotation, hyperrotation, or reversed rotation; gastroschisis and diaphragmatic hernias seem to potentially also occur during this period. mesocolic paraduodenal hernias also occur during the late second phase and early third phase
Arrest in the third phase results in a mobile cecum, an unattached duodenum, or an unattached small bowel mesentery, which allows cecal volvulus and internal hernias
It is important to note that intestinal malrotation may occur as an isolated event or in association with other types of congenital anomalies as listed above.
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. This variant of malrotation is usually considered benign, given the fact that it may not cause any symptoms unless a volvulus develops.
In nonrotation, 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. In addition, the peritoneal attachments of the cecum pass anterior and lateral to the duodenum, which may lead to some degree of extrinsic duodenal obstruction (a common cause of intermittent bilious vomiting).
Incomplete rotation is the next most common rotational abnormality, involving arrest of rotation at or near 180°. It is the most important form of malrotation. In this condition, midgut rotation is incomplete, the DJ loop remains to the right of the SMA, and the ileocecal loop comes to lie in the right upper quadrant, anterior to the SMA and closely related to the duodenum.
With incomplete rotation, the entire midgut is attached by a very narrow pedicle, consisting mostly of the SMA and the SMV. Twisting or volvulus of this pedicle may occur, leading to ischemia and mechanical obstruction of the intestine, which may lead to intestinal necrosis (see the image below). The ischemia begins with venous congestion, which progresses to arterial occlusion, and, finally, bowel necrosis.
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Malrotation and midgut volvulus with intestinal ischemia. Note narrow pedicle at base of mesentery. No resection was required since ischemic necrosis ....
As previously described, abnormal cecal peritoneal bands that cross over the duodenum in the right upper quadrant may be found in cases of malrotation. 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, the 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. In many cases, the abnormal Ladd bands cause only partial and intermittent obstruction, which can manifest as intermittent episodes of bilious vomiting and/or feeding intolerance.
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 may 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 (also described as an internal hernia).
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-side hernia is associated with congenital short bowel.
Volvulus
Volvulus of the midgut may result in several manifestations, depending on the degree of twisting. Venous and lymphatic obstructions occur first because of lower intravascular pressures. Vascular congestion leads to bowel edema and possible oozing of blood, potentially causing GI bleeding. Lymphatic congestion causes the formation of a mesenteric cyst and/or chylous ascites. It is not uncommon to find chylous fluid in the peritoneal cavity and enlarged lymph nodes at the time of corrective surgery for malrotation and volvulus.
If volvulus is intermittent, children may have chronic malabsorption from congestion and edema or intermittent bouts of symptoms, usually vomiting and possibly alternating diarrhea and constipation. Arterial compromise is seen when the twisting is significant enough to occlude venous and arterial vessels. This represents an acute and dangerous event. Sequelae include (in order of less to more severe) ischemia, mucosal necrosis, intramural air formation, bacterial translocation, gram-negative sepsis, full-thickness intestinal wall necrosis, perforation, peritonitis, and death.
Other congenital abnormalities
Malrotation is often associated with other congenital abnormalities, and, in some regions, as many as 62% of cases are associated with these abnormalities. This is important for several reasons.
First, some abnormalities (eg, polysplenia, asplenia, and congenital abdominal wall defects) are commonly associated with malrotation, and their presence should automatically lead to an investigation for malrotation. Patients with congenital wall defects, by definition, have some degree of malrotation, which should be evaluated during correction of the defect. Rotational anomalies in conjunction with dextroisomerism or levoisomerism should point to possible polysplenia or an asplenia syndrome. These syndromes have devastating effects on outcomes, especially asplenia.
Second, and perhaps most important, mortality related to malrotation and its correction is strongly correlated with the presence or absence of other congenital abnormalities. Serious anomalies may raise the risk of mortality 22 times. Physicians treating patients with malrotation must be aware of the possibility of other associated congenital abnormalities.
Developmental abnormalities such as gastroschisis, omphalocele, and congenital diaphragmatic hernia are associated with displacement of the abdominal contents outside the domain of the abdominal cavity. Since such developmental abnormalities occur at the time of fetal intestinal growth and rotation, they typically result in intestinal malrotation. Malrotation has also been described in also association with duodenal atresia, Meckel diverticulum, intussusception, small bowel atresia, prune belly syndrome, gastric volvulus, persistent cloaca, Hirschsprung disease, and extrahepatic biliary anomalies.
Specific causes of malrotation are unknown, although repeated associations have been made with congenital syndromes such as Down syndrome and the VACTERL (vertebral, anal, cardiac, tracheal, esophageal, renal, limb) association of anomalies. Because malrotation is an embryologic development abnormality, it may be assumed that any interference during critical periods of fetal development can 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 association
Trisomy 13, trisomy 18
Esophageal atresia
Situs inversus and asplenia (may be associated with biliary atresia)
Malrotation is estimated to occur in 1 per 500 live births. Autopsy studies have reported a 0.2-1% incidence of malrotation in the total population; however, clinical discovery is achieved in only 1 per 600 live births. Increasingly, many cases are diagnosed at the time of laparotomy for other GI anomalies.
A mobile cecum or a high cecum (found in the right upper quadrant) may be considered a minor rotational abnormality, and it should not be the cause of any major medical problems. It 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 statistics
Overall, the incidence of volvulus internationally is similar to the incidence in the United States. Sigmoid volvulus in adult patients is more common in developing nations than in the United States, probably because of dietary differences in terms of fiber. Chagas disease in Brazil may account for a significant proportion of cases in that country in children and adults.
Age-related demographics
Of those in whom midgut volvulus occurs, 68-71% are neonates. Most cases occur by age 2 months, but as many as 41% of cases occur at an older age. Again, as previously mentioned, sigmoid volvulus is more common in elderly persons.
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.
Sex-related demographics
Overall, no significant sex predilection has been established. However, midgut volvulus predominates in male infants. 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 3:2 male-to-female ratio. Reasons for the slight male predominance are largely unknown.
The prognosis of midgut volvulus depends on prompt recognition before bowel necrosis occurs. In general, rapid diagnosis and immediate intervention for correction improve overall prognosis in all age groups. Younger children are at higher risk and require more aggressive support and treatment. Patients with other congenital defects have a poorer prognosis, depending on the severity of the abnormality.
The morbidity and mortality of malrotation and volvulus have decreased significantly since the advent of surgical correction. Early mortality figures ranged from 23% to 33%, mostly resulting from the sequelae of major bowel resection with bowel dysfunction and malnutrition. With the development of total parenteral nutrition and improved nutritional support, survival has increased. Current mortality figures range from about 3% to 9% overall.
In addition, improved understanding of the pathophysiology and the associated anomalies has allowed more prompt diagnosis. Clinical awareness of the association of malrotation with other anomalies and increased sensitivity to the importance of minor symptoms (eg, bilious vomiting) 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 (≤33%) still associated with diagnostic delay, especially in very young persons.
Messineo et al determined that the most important factors influencing mortality were the presence of necrotic bowel, associated abnormalities, and younger age.[6] Risk of mortality increases 25 times in the presence of bowel necrosis and is influenced heavily by the length of viable bowel. The investigators found that the survival rate was maintained above 93% with 10%, 25%, and 50% of the bowel necrosed but that it fell to 35% with the loss of 75% of the length of the small bowel.
Messineo et al also found that the presence of other serious abnormalities increased 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, the authors determined that patients who died after surgery had a significantly lower median age (4 days versus 30 days).
The most common cause of death in patients who develop a volvulus 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 long-term postoperative morbidity include adhesive bowel 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.
Recurrent volvulus can occur in patients with intestinal malrotation that was successfully treated with a Ladd procedure (see Treatment). The operation for malrotation decreases the risk of intestinal volvulus, but several cases have been reported in which volvulus developed weeks and even months and years after Ladd procedure.[7]
The significant recurrence rate (7%) for midgut volvulus after the Ladd procedure has resulted in several attempts over the past century to augment this operation with fixation of the cecum and duodenum; however, overall results of fixation procedures have not shown improvement in outcomes. The fixation itself can be a source of volvulus, internal hernias, and intestinal obstruction. Therefore, patient and family education on the signs and symptoms of obstruction and volvulus has proved to be 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 mandatory as long as the patient is stable enough to undergo the procedure and general anesthesia.
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 is estimated to result in volvulus in 1 out of 3 patients.
It is important to provide 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, particularly if associated with bilious vomiting. In addition, instruct patients to inform any physician they visit of their medical history of volvulus, especially if the appendix is left in situ.
The clinical presentation of patients with volvulus varies. No unique signs or symptoms pathognomonic for intestinal malrotation and volvulus are recognized; however, certain findings are commonly observed. In the first month of life, the most typical presentation includes feeding intolerance with bilious (ie, yellow or green) vomiting and sudden onset of abdominal pain.
Bilious vomiting is the hallmark presentation and is observed 77-100% of the time. Although bilious vomiting can occur in various other medical conditions, such a presentation in young infants, should be considered diagnostic of malrotation with midgut volvulus until proved otherwise.
In older children, symptoms can be vague and may include chronic intermittent vomiting and abdominal cramping, failure to thrive, constipation, bloody diarrhea, and hematemesis. Children with vague clinical features are sometimes incorrectly diagnosed as having irritable bowel syndrome, peptic ulcer disease, kidney stones, or even psychogenic or emotional disorders. However, even in older children, intermittent bilious vomiting is commonly seen with malrotation.
Sigmoid volvulus typically presents with abdominal pain, distention, and inability to pass stool or flatus (obstipation). It is usually associated with a history of constipation and/or megasigmoid. Vomiting may be a late presenting feature, and cases may progress to peritonitis, sepsis, and death.
Malrotation
The typical history of a patient with intestinal malrotation depends on the age at presentation and the 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, due to the presence of a duodenal postampullary obstruction. They may also have upper abdominal distention. Young patients usually present with bilious vomiting and have a history of intermittent feeding intolerance.
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.
In general, younger children are more likely to present with acute symptoms lasting less than 48 hours. Older children may present more insidiously, with symptoms that include cyclic vomiting (bilious), 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.
An abrupt change from feed tolerance to vomiting and irritability at any age is suggestive of 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 of the blood supply of the midgut has started as a consequence of the twisting of the mesentery (see the images below) at the narrow pedicle of the superior mesenteric artery (SMA). This results in ischemia and possibly necrosis.
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Operative photograph illustrating midgut volvulus of full-term newborn who underwent upper GI contrast study. Note complete twist (> 360°) of entire s....
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Operative photograph of midgut volvulus due to intestinal malrotation in 10-year-old patient. Note twisting at base of mesentery with evidence of inte....
Thus, the signs and symptoms depend on the degree of ischemia. Manifestations can range from lymphatic and venous congestion with simple edema to full intestinal necrosis secondary to arterial and venous thrombosis. 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 the vascular territory of the SMA includes the distal duodenum to the midtransverse 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 (see the image below).
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Operative photograph of patient with midgut volvulus in which diagnosis was made late. Note that entire small bowel is necrotic and nonviable. This in....
Malrotation and volvulus should be suspected in all cases of proximal small-bowel obstruction, especially in infants. Besides the cardinal clinical manifestation of bilious vomiting,[8] clinical features may include pain (colicky at first, then steady), anorexia, blood and mucus in the stool,[9] abdominal tenderness, and, eventually, shock.
In early cases, patients may appear well, and abdominal examination findings may be normal. In fact, normal findings on abdominal examination have been reported in as many as 50% of patients. Because the obstruction is very proximal, abdominal distention is not usually present. In one series, 32% of patients had abdominal distention but no tenderness. Patients who present acutely usually have pain out of proportion to the degree of abdominal tenderness.
The initial physical examination may be almost normal. As ischemia develops, examination may reveal a palpable abdominal mass in some patients. Signs of intraluminal blood loss, such as hematochezia or stool guaiac testing, are usually positive.
A high index of suspicion is required to establish the diagnosis at an early stage. If the diagnosis is missed, intestinal ischemia progresses to gangrene, and bowel distention from gas-producing organisms within the intestine occurs.
Once ischemia occurs, almost all patients develop diffuse and severe abdominal pain and signs of peritonitis. Patients with gangrene are usually tachycardic and hypovolemic. Patients may appear lethargic. Passage of blood or sloughed mucosal tissue may be noted as vascular compromise progresses. As ischemia progresses to infarction and necrosis, fever, peritonitis, abdominal distention, profound hypovolemia, and septic shock develop.
Malrotation
Physical findings in malrotation are subtle. Abdominal distention may be present in the epigastric area, especially in infants younger than 1 year. Distention may be absent even in this age group if vomiting has cleared gastric contents. Peristaltic waves may occasionally 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 distention 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.
Volvulus
In the event of malrotation with midgut volvulus, vascular compromise develops, and this determines the severity of the clinical presentation. The physical signs may range from mild abdominal pain to severe pain with an acute abdomen or even a shocklike picture with dehydration, lethargy, and respiratory distress.
In early infancy, the clinical picture of volvulus is one of proximal intestinal obstruction that may progress to shock and peritonitis if unrecognized. In older children, the main clinical feature may be recurrent midabdominal colic, with or without vomiting. A rare manifestation of nonrotation is left-side appendicitis, which occurs when the ileocecal loop remains on the left side of the abdomen.[10]
If malrotation is suspected (eg, in an infant with bilious vomiting), a diagnostic workup is mandatory. A few laboratory studies may be helpful in the management of a patient with malrotation. However, laboratory tests should not delay immediate surgical consultation and operation when volvulus is suspected; no laboratory tests are specific for this problem.
Imaging studies are an integral part of the diagnostic process for a patient suspected of having malrotation or other gastrointestinal (GI) obstruction. The most expeditious and definitive test is usually an upper GI contrast study, which is performed in real time to document the position of the ligament of Treitz and the possible presence of proximal intestinal obstruction.
Although various other studies may be useful as adjuncts or to encourage evaluation for malrotation and volvulus, the definitive diagnosis is made on the basis of findings from a contrast upper GI series, barium enema (BE), or laparotomy.
A complete blood count (CBC), clotting studies, electrolyte level tests, and blood glucose level tests are usually sufficient for preoperative evaluation. In the early stages, all blood test findings may be normal; however, as the disease progresses from mechanical obstruction to intestinal ischemia and necrosis, it produces electrolyte disturbances, elevated blood urea nitrogen (BUN) and creatinine levels, hypocarbia, and lactic acidosis.
CBC monitoring helps the physician assess the severity of illness. Ischemia may cause an elevation in the white blood cell (WBC) count. A very high WBC count may ominously suggest gangrene and sepsis. A decrease in hemoglobin and hematocrit may occur with venous oozing.
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.
Findings ranging from occult to frank blood may be present in the stools of a child with malrotation, if mucosal ischemia has developed; 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.
Plain abdominal radiography is useful, but findings are not definitive. This study may reveal the following:
Normal gas pattern
Gasless abdomen
Dilated intestine suggestive of a paralytic ileus or mechanical obstruction
Duodenal obstruction with a double bubble
In early cases, plain abdominal radiography may not be helpful, but oral contrast studies (see Contrast Radiography) may reveal malrotation and intestinal obstruction.
Ideally, abdominal radiographs should be obtained in 2 views (ie, supine, upright, or lateral recumbent). These views may reveal evidence of small bowel obstruction, including dilated small-bowel loops; marked gastric or proximal duodenal dilatation, with or without intestinal gas; and air-fluid levels.
The classic sign on abdominal radiography is the double bubble, indicative of gastric and duodenal dilatation (see the image below). Duodenal obstruction causes gastric and duodenal distention with a prominent duodenal bulb and air-fluid levels in the proximal duodenum and stomach on upright radiographs. The double-bubble sign 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.
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Plain abdominal radiograph shows dilated stomach and proximal bowel with some air distally (ie, double-bubble sign).
The double-bubble is often accompanied by 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 distention, melena, or hemodynamic instability, suggests 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 give the physician a false sense of security, creating a dangerous situation for the patient. In the presence of clinical suspicion, contrasted studies (eg, an upper GI) are warranted for confirmation.
In sigmoid volvulus, as contrasted to midgut volvulus, plain radiography may reveal a characteristically distended sigmoid colon.
The diagnosis of malrotation can be confirmed with upper or lower GI contrast study findings. However, over the past 7 decades, controversy has emerged over the use of the upper GI series versus the use of the lower GI series (contrast enema). Physicians have been reluctant to administer a barium meal to infants suspected of obstruction; hence the popularity of BE.
The upper GI series provides more information regarding the degree of obstruction and the actual location of proximal GI anatomy. BE findings are more likely to be inconclusive; furthermore, BE impedes reading of any subsequent upper GI study, owing to the presence of contrast in the colon.
In 1992, Ford et al found that a limited upper GI study using only 5 mL of barium was well tolerated and allowed visualization of anatomy and subsequent BE if needed, as in the case of complete obstruction with necessary observation of lower anatomy.[11]
Overall, most centers prefer upper GI imaging for the radiologic evaluation of malrotation and midgut volvulus. In neonates, a contrast enema is useful in ruling out intestinal atresia, meconium ileus, Hirschsprung disease, and other rare causes of intestinal obstruction.
Upper GI series
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 the image below).
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Upper GI series of child with malrotation and midgut volvulus that reveals abnormal position and obstruction in third part of duodenum.
The proximal duodenum is often dilated with a “bird’s-beak” obstruction (see the first image below) and a spiral or corkscrew duodenal configuration (see the second and third images below). This is a result of the abnormal positioning and the adhesive obstruction of Ladd bands.
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Upper GI contrast study of full-term newborn baby with bilious vomiting. Note obstruction at third and fourth portions of duodenum with "bird's-beak" ....
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Upper GI contrast study in a 10-year-old patient with intestinal malrotation. Note normal appearance of stomach but abnormal shape of duodenum. Duoden....
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Lateral view from upper GI series reveals duodenum with corkscrew appearance.
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 appear to be thickened.
Specificity of upper GI studies in detecting malrotation is almost 100%, but their sensitivity for detecting midgut volvulus is only 54%, reflecting the importance of clinical judgment in diagnosis.
Contrast enema
If the findings on the upper GI study are nonspecific and suggestion of malrotation continues, a contrast enema may be administered, preferably of barium. However, the results are not reliable if a midgut volvulus is present. In patients with bilious vomiting and a low clinical suspicion for midgut volvulus, lower GI imaging may be used to rule out colonic obstruction due to conditions such as atresia, Hirschsprung disease, and meconium ileus or meconium plug; it may actually prove to be therapeutic.
The sign sought with this study is an abnormally placed cecum, high in the right upper quadrant, midline, or on the left side (see the image below). 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.
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Barium enema of 1-year-old infant with history of intermittent bilious vomiting. Colon is positioned abnormally, with most of it on left side of abdom....
In malrotation, the contrast enema usually reveals the cecum in the right upper quadrant (see the image below). Outside the newborn period, an upper GI contrast study is more commonly performed in children who develop symptoms and signs suggestive of volvulus after their bowels have “opened up.” Malrotation should be suspected if the ligament of Treitz is not positioned to the left of the midline at or above the level of the duodenal bulb. The upper GI study may also reveal a swirling or corkscrew pattern within the bowel, leading to an obstruction.
View Image
Barium enema in child with malrotation and midgut volvulus. Note cecum in right upper quadrant and dilated loops of small bowel.
Failure to recognize malrotation has been observed with lower GI imaging in 5-20% of patients with a normally located cecum.
In patients with sigmoid volvulus, lower GI imaging reveals dilated rectosigmoid loops with an abrupt inability to pass contrast beyond obstruction.
The use of ultrasonography in the diagnosis of malrotation has been investigated, although it usually is not very helpful and is not recommended in evaluating such patients.
Ultrasonography may reveal a midline abdominal mass with dilated collateral mesenteric vessels and whirling small intestinal loops, shown as sonolucent layers. In some cases, it may reveal intraluminal fluid or edema in the bowel wall. If performed for other reasons, it may reveal persistent distention of the duodenum as it courses around the head of the pancreas.
Although not usually necessary, ultrasonography may reveal abnormalities of intestinal rotation based on vascular anatomy. In many patients with malrotation, the normal anatomic relationship between the superior mesenteric artery (SMA) and the superior mesenteric vein (SMV) is altered, with the artery lying anterior or even to the right of the vein (see the image below). On the other hand, a normal anatomic relation between SMA and SMV does not rule out malrotation. Reminding physicians that patients with malrotation always have a left-sided SMV postoperatively is helpful.
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Ultrasound image with Doppler flow revealing twisted superior mesenteric artery and vein in child with midgut volvulus.
Dufour et al, in a study examining more than 300 patients with ultrasonography, found that an SMV to the left of the SMA was highly suggestive of malrotation, whereas an anterior SMV was questionable.[12] Positioning of the mesenteric vessels was normal in 326 patients, 9 of whom had malrotation. On the basis of 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.
Computed tomography
The use of computed tomography (CT) scanning in the diagnosis of malrotation has also been investigated. Abdominal contrast CT has a high sensitivity for demonstrating small bowel obstruction.[13] The finding of multiple and posterior location of transition points within the small bowel (segments where dilated small bowel is immediately followed by collapsed bowel) is predictive of volvulus (see the image below).
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CT scan of abdomen in child with midgut volvulus. Note twisted mesentery and bowel anterior to right kidney.
The “whirl sign” (clockwise or counterclockwise twisting of the bowel that extends for at least 180°) can also be seen on CT scans in the mesentery of the midgut when a frank volvulus is present.
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CT scan of 4-year-old patient with intermittent episodes of bilious vomiting. CT scan was performed at time of volvulation of the midgut. Note whirl o....
An article published by Hsiao and Langer reviewed the utility of laparoscopy for evaluating rotation and fixation anomalies in children with diagnostic or equivocal imaging results. In 41 % of children with diagnostic imaging, a discrepancy was found by laparoscopy. Similarly, in 32% of children with equivocal imaging, laparoscopy demonstrated a rotational anomaly that placed them at risk for midgut volvulus.[14]
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.
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, ranging from a mild degree to frank necrosis and gangrene, complete with neutrophilic infiltration.
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.
The management of abnormalities of rotation and volvulus is well established. Surgical correction is indicated; no other treatment is available. The most important point is to recognize and address the diagnosis early before complications develop. Some authors have reported using laparoscopy to treat these conditions, but this has not become standard practice and is still being evaluated.
A surgeon should be immediately consulted when malrotation or volvulus is suspected. Any symptomatic child with peritonitis should be taken immediately for laparotomy. If peritonitis is not present, the diagnosis should be confirmed with an upper gastrointestinal (GI) contrast study, and the child should be resuscitated with intravenous (IV) fluids before surgery. However, any compromise of blood flow to the involved bowel constitutes a true surgical emergency.
Consider unexplained bilious vomiting in an otherwise healthy infant a surgical emergency; likewise, assume malrotation with midgut volvulus until proven otherwise.
Some would consider the use of prokinetics in infants inadvisable until imaging has ruled out intestinal malrotation.
In neonates who have not established oral feeding and bowel function, ruling out duodenal atresia, stenosis, or web at the time of surgery is important.
Although definitive treatment of malrotation and intestinal volvulus consists of surgical correction, there is a role for nonoperative measures. Medical care revolves around support of the patient preoperatively and postoperatively, treatment or stabilization of any coexisting conditions, and expedition of transport to the operating room (OR).
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 that intestinal volvulus may occur at any time and that they must seek immediate medical attention if any GI symptoms develop.
Insertion of a nasogastric or orogastric tube begins GI decompression. This may be successful in alleviating the vomiting and discomfort associated with obstruction. Rectal tube decompression of a sigmoid volvulus can be achieved. This may be aided by endoscopic placement.
The respiratory system is supported with intubation and ventilation as needed. Prophylactic broad-spectrum antibiotics are administered and continued postoperatively if the bowel vascular supply is compromised, causing necrosis.
Fluid and electrolyte status is closely monitored, with IV fluids administered at least at maintenance levels and increased for any signs of dehydration 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, distention, fever, hemodynamic instability), which may show that volvulus has occurred.
Definitive treatment for sigmoid volvulus remains surgical with resection and primary anastomosis. As with most instances of bowel resection, an open approach is usually used. Most patients undergo the Ladd procedure. A laparoscopic Ladd procedure has been described with good success rates.[15]
Once the diagnosis of volvulus has been established, urgently proceeding to operative intervention is important. Do not delay operation in a patient who is not stable. A brief period spent on supportive measures (see above) may be appropriate; however, the main goal is to reduce the volvulus as quickly as possible by means of surgical exploration.
During the operation, the midgut volvulus is reduced by untwisting the bowel in a counterclockwise fashion. The viability of the small bowel loops can then be assessed. Doppler probe or fluorescein with a Wood light may be helpful in documenting bowel viability. Necrotic bowel is resected if it is encountered.
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 embryologic state but accomplished the greatest stability to that point. His procedure survives, relatively unchanged, as the most widely used technique.
Incision and exposure
A transverse incision is made through the right rectus muscle in the right upper quadrant. This incision allows the greatest visualization and access to the anatomy. Upon exploration, 1 of 2 arrangements of the intestine is commonly seen.
In one arrangement, the cecum is visible in the right upper quadrant and is attached to the retroperitoneum by Ladd bands (see Pathophysiology), which cross in front of the duodenum. This is usually associated with duodenal obstruction but not with volvulus or ischemia. The peritoneal bands should be divided, and the cecum and ascending colon should be moved to the left side of the abdomen as far from the duodenum as possible.
In the other, more common arrangement, the colon is obscured by loops of small bowel. This is the pattern in patients with volvulus, with or without bowel ischemia. The intestines should be delivered from the abdomen. The surgeon is then able to observe that the entire midgut is attached by a very narrow pedicle containing the superior mesenteric artery (SMA) and superior mesenteric vein (SMV) and little else, which may or may not be twisted at its base. Loops of small bowel may be seen coiled around the base of the mesentery.
Chylous ascites is occasionally present. This turbid fluid may appear infected but is actually sterile.
Untwisting of bowel
The intestine is rotated counterclockwise until the twist is completely relieved (see the images below). This may require up to 3 complete turns of the bowel. During correction of malrotation, thoroughly examine the bowel for signs of necrosis and cover the bowel with warm sponges to allow restoration of blood flow.
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Operative findings of malrotation of gut with volvulus.
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Diagram illustrating operative maneuver to untwist volvulized midgut. Note that untwisting is performed in counterclockwise fashion by operating surge....
The SMA pedicle is dissected free 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 with the help of a Kocher maneuver to ensure resolution of restriction. Closing or widening any defect to prevent future strangulation can repair mesocolic hernias.
Appendectomy
In the absence of cecal necrosis, most surgeons also perform an appendectomy; this prevents any subsequent confusion in diagnosing appendicitis that may arise from the abnormal position of the cecum and appendix on the left side of the abdomen.
After the previously described steps have been accomplished, the bowel is returned to the abdomen in the nonrotated position, with the proximal small bowel on the far right, the colon on the far left, and the mesentery spread out like an apron between them. The appendix is then removed. As a rule, no attempt should be made to stitch (or fixate) the bowel down to prevent it from twisting again.
Excision of necrotic bowel
Any bowel that is frankly necrotic should be conservatively excised (see the image below). A primary reanastomosis or enterostomy and mucous fistula can be performed, depending on the state of the bowel at the resection margin and the extent of the resection. If the bowel is congested and ischemic but not frankly necrotic, it may be left in situ after the volvulus is reduced. Edematous and ischemic bowel may regain function over the next few days. This may necessitate a second-look laparotomy within 24-48 hours from the initial operation.
View Image
Malrotation and midgut volvulus with intestinal necrosis. Massive resection of small bowel was required, but child survived and was eventually weaned ....
The length of remaining jejunum and ileum should be carefully measured and documented. 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. When a massive resection that will leave less than 100 cm of healthy small bowel is necessary, a gastrostomy may be inserted.
If a significant length of unresected bowel is of questionable viability after the initial operation, it may be reinspected and its viability reassessed at a planned second-look operation within 24-48 hours.
In the tragic case of total midgut infarction, close the abdomen with the entire bowel intact, and provide terminal care.
Confirmation of duodenal patency
Duodenal patency must be ensured before closure. Many techniques have been used (eg, injection of air or saline), but the most successful is to use a large Fogarty embolectomy catheter or another appropriately sized balloon-tipped catheter. The catheter is inserted orogastrically or nasogastrically and advanced through the duodenum. The balloon is then inflated and retracted through the duodenum. Atresia prevents the complete passage of the catheter; 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.
Closure of abdomen
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, following the same principles as the open procedure, has been used in many pediatric surgical centers. Laparoscopic surgery provides the general advantage of decreased scarring and adhesions; although adhesions may create obstructive problems in 1-10% of patients, they may also provide stability to the new intestinal placement. 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. For that reason, if necrosis is seen at the time of laparoscopy, the procedure should be converted to open laparotomy.
Successful laparoscopic management of malrotation has been described in a number of case reports and small series.[16] It remains unclear, however, whether laparoscopy for the treatment of malrotation has a success rate equal to that of open surgery. Preliminary data suggest that it is equally effective.
Role of cecal or duodenal fixation
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.[17]
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.[11]
In 1975, Gohl and DeMeester used the less well-known Fitzgerald technique with good results in adults.[18] 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. For that reason, fixation is no longer recommended in the management of malrotation and volvulus.
Management of 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 anatomic defect remains.
Abdominal wall defects may contribute to thickening and shortening of the bowel, making anatomic identification and correction difficult.
Complications of surgery
The most common postoperative complications are adhesive obstruction, short-bowel syndrome (occurring in 20% of cases), and recurrent volvulus (occurring in as many as 8%). Patients who have large portions of necrotic intestine that require resection have chronic difficulties with short-bowel syndrome. Motility disturbances develop in some children. A prolonged paralytic ileus may complicate the postoperative recovery.
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.
Postoperative care depends on the presence of other abnormalities, which should be treated accordingly, and the presence of necrotic bowel.
After the procedure, 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. Questionable bowel may either recover (sometimes slowly) or become necrotic. Use antibiotics in the presence of necrotic bowel.
Postoperatively, patients still require aggressive fluid resuscitation and IV antibiotics. IV parenteral nutrition is begun in patients who have undergone resection of a significant length of necrotic bowel. When the entire bowel appears necrotic, massive resection typically results in short-bowel syndrome and a lifetime of parenteral nutrition with its associated morbidities, most notably progressive cholestatic liver disease.
Small bowel transplant for short-bowel syndrome continues to be associated with high morbidity and mortality, although increasing experience and advances in immunosuppressive therapy continue to increase the survival rates in children. Early listing with a small bowel transplant service before the development of end-stage liver disease may result in improved outcome after transplant.
Any signs and symptoms of obstruction should be noted by physicians and family members and attended to quickly. It is important to remember that recurrent volvulus may occur even after a successful Ladd procedure (open or laparoscopic). For that reason, recurrent bilious vomiting in a patient with a history of corrected malrotation should be investigated promptly.[7]
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, if a significant portion of the bowel is removed, and short-bowel syndrome develops, a lifetime of dietary modifications, and possibly long-term hyperalimentation supplementation, will be necessary.
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 restriction
No specific restrictions regarding postoperative activity are indicated. Age-appropriate activity is always encouraged as soon as tolerated after surgery, barring other restricting abnormalities.
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.
A pediatric surgeon should be involved early in the care of any patient suspected of having 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 OR may be necessary, and all facilitating measures should be taken.
No specific medical therapy is indicated in the management of malrotation and intestinal volvulus. Treatment consists of surgical correction. Observation may be used in the patient for whom surgery is not appropriate (eg, those awaiting stabilization of congenital heart defects).
Broad-spectrum antibiotics, chosen to cover both skin and enteric flora, should be administered preoperatively (and postoperatively, if needed) to patients in whom vascular compromise, bowel necrosis, perforation, or sepsis is suspected. These agents have proven effective in decreasing the rate of postoperative wound infection and improving outcome.
Clinical Context:
Ampicillin interferes with bacterial cell wall synthesis during active replication, causing bactericidal activity against susceptible organisms.
Clinical Context:
Clindamycin is useful as a treatment against serious skin and soft tissue infections caused by most staphylococcal strains. It is also effective against aerobic and anaerobic streptococci, except for enterococci. It inhibits bacterial protein synthesis by inhibiting peptide chain initiation at the bacterial ribosome, where it preferentially binds to the 50S ribosomal subunit, causing bacterial replication inhibition.
Clinical Context:
Cefotetan is a second-generation cephalosporin used as single-drug therapy to provide broad gram-negative coverage and anaerobic coverage. The half-life is 3.5 hours.
Andre Hebra, MD, Chief, Division of Pediatric Surgery, Professor of Surgery and Pediatrics, Medical University of South Carolina College of Medicine; Surgeon-in-Chief, Medical University of South Carolina Children's Hospital
Disclosure: Nothing to disclose.
Chief Editor
Carmen Cuffari, MD, Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine
Disclosure: Nothing to disclose.
Additional Contributors
Liz D Dancel, MD Resident, Department of Pediatrics, Greenville Hospital System University Medical Center
Disclosure: Nothing to disclose.
Jeffrey J DuBois, MD Chief of Children's Surgical Services, Division of Pediatric Surgery, Kaiser Permanente, Women and Children's Center, Roseville Medical Center
Jeffrey J DuBois, MD, is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, and California Medical Association
Disclosure: Nothing to disclose.
Andre Hebra, MD Chief, Division of Pediatric Surgery, Professor of Surgery and Pediatrics, Medical University of South Carolina College of Medicine; Surgeon-in-Chief, Medical University of South Carolina Children's Hospital
Andre Hebra, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, Children's Oncology Group, Florida Medical Association, International Pediatric Endosurgery Group, Society of American Gastrointestinal and Endoscopic Surgeons, Society of Laparoendoscopic Surgeons,South Carolina Medical Association, Southeastern Surgical Congress, and S outhern Medical Association
Disclosure: Nothing to disclose.
B UK Li, MD Professor of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, Director, Pediatric Fellowships and Gastroenterology Fellowship, Medical Director, Functional Gastrointestinal Disorders and Cyclic Vomiting Program, Medical College of Wisconsin; Attending Gastroenterologist, Children's Hospital of Wisconsin
B UK Li, MD is a member of the following medical societies: Alpha Omega Alpha, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.
Jonathan E Markowitz, MD Associate Professor of Clinical Pediatrics, University of South Carolina School of Medicine; Attending Pediatric Gastroenterologist, Associate Director of Pediatric Residency Program, Greenville Hospital System
Jonathan E Markowitz, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, Crohns and Colitis Foundation of America, and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.
Melissa Miller, MD Department of Surgery, Medical University of South Carolina
Melissa Miller, MD is a member of the following medical societies: American Medical Association and American Medical Student Association/Foundation
Disclosure: Nothing to disclose.
Robert K Minkes, MD, PhD Professor of Surgery, University of Texas Southwestern Medical Center at Dallas, Southwestern Medical School; Medical Director and Chief of Surgical Services, Children's Medical Center of Dallas-Legacy Campus
Robert K Minkes, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, and Phi Beta Kappa
Disclosure: Nothing to disclose.
David A Piccoli, MD Chief of Pediatric Gastroenterology, Hepatology and Nutrition, The Children's Hospital of Philadelphia; Professor, University of Pennsylvania School of Medicine
David A Piccoli, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.
Marleta Reynolds, MD Professor of Surgery, Northwestern University, The Feinberg School of Medicine; Head, Department of Surgery and Surgeon in Chief, Head, Division of Pediatric Surgery, Children's Memorial Hospital of Chicago
Marleta Reynolds, MD is a member of the following medical societies: American Pediatric Surgical Association
Disclosure: Nothing to disclose.
Prem C Shukla, MD Associate Chairman, Associate Professor, Department of Emergency Medicine, University of Arkansas for Medical Sciences
Prem C Shukla, MD, is a member of the following medical societies: American Academy of Emergency Medicine
Disclosure: Nothing to disclose.
David E Wesson, MD Professor of Surgery, Professor of Pediatrics, Chief of Division of Pediatric Surgery, Michael E DeBakey Department of Surgery, Baylor College of Medicine; Chief of Pediatric Surgery Service, Texas Children's Hospital
David E Wesson, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Association for the Surgery of Trauma, American College of Physicians, American Pediatric Surgical Association, American Surgical Association, Canadian Association of Pediatric Surgeons, Children's Oncology Group, Eastern Association for the Surgery of Trauma, Society for Surgery of the Alimentary Tract,Society of University Surgeons, and Trauma Association of Canada
Disclosure: Nothing to disclose.
Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.
Additional Contributors
Liz D Dancel, MD Resident, Department of Pediatrics, Greenville Hospital System University Medical Center
Disclosure: Nothing to disclose.
Jeffrey J DuBois, MD Chief of Children's Surgical Services, Division of Pediatric Surgery, Kaiser Permanente, Women and Children's Center, Roseville Medical Center
Jeffrey J DuBois, MD, is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, and California Medical Association
Disclosure: Nothing to disclose.
Andre Hebra, MD Chief, Division of Pediatric Surgery, Professor of Surgery and Pediatrics, Medical University of South Carolina College of Medicine; Surgeon-in-Chief, Medical University of South Carolina Children's Hospital
Andre Hebra, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, Children's Oncology Group, Florida Medical Association, International Pediatric Endosurgery Group, Society of American Gastrointestinal and Endoscopic Surgeons, Society of Laparoendoscopic Surgeons,South Carolina Medical Association, Southeastern Surgical Congress, and S outhern Medical Association
Disclosure: Nothing to disclose.
B UK Li, MD Professor of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, Director, Pediatric Fellowships and Gastroenterology Fellowship, Medical Director, Functional Gastrointestinal Disorders and Cyclic Vomiting Program, Medical College of Wisconsin; Attending Gastroenterologist, Children's Hospital of Wisconsin
B UK Li, MD is a member of the following medical societies: Alpha Omega Alpha, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.
Jonathan E Markowitz, MD Associate Professor of Clinical Pediatrics, University of South Carolina School of Medicine; Attending Pediatric Gastroenterologist, Associate Director of Pediatric Residency Program, Greenville Hospital System
Jonathan E Markowitz, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, Crohns and Colitis Foundation of America, and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.
Melissa Miller, MD Department of Surgery, Medical University of South Carolina
Melissa Miller, MD is a member of the following medical societies: American Medical Association and American Medical Student Association/Foundation
Disclosure: Nothing to disclose.
Robert K Minkes, MD, PhD Professor of Surgery, University of Texas Southwestern Medical Center at Dallas, Southwestern Medical School; Medical Director and Chief of Surgical Services, Children's Medical Center of Dallas-Legacy Campus
Robert K Minkes, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, and Phi Beta Kappa
Disclosure: Nothing to disclose.
David A Piccoli, MD Chief of Pediatric Gastroenterology, Hepatology and Nutrition, The Children's Hospital of Philadelphia; Professor, University of Pennsylvania School of Medicine
David A Piccoli, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.
Marleta Reynolds, MD Professor of Surgery, Northwestern University, The Feinberg School of Medicine; Head, Department of Surgery and Surgeon in Chief, Head, Division of Pediatric Surgery, Children's Memorial Hospital of Chicago
Marleta Reynolds, MD is a member of the following medical societies: American Pediatric Surgical Association
Disclosure: Nothing to disclose.
Prem C Shukla, MD Associate Chairman, Associate Professor, Department of Emergency Medicine, University of Arkansas for Medical Sciences
Prem C Shukla, MD, is a member of the following medical societies: American Academy of Emergency Medicine
Disclosure: Nothing to disclose.
David E Wesson, MD Professor of Surgery, Professor of Pediatrics, Chief of Division of Pediatric Surgery, Michael E DeBakey Department of Surgery, Baylor College of Medicine; Chief of Pediatric Surgery Service, Texas Children's Hospital
David E Wesson, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Association for the Surgery of Trauma, American College of Physicians, American Pediatric Surgical Association, American Surgical Association, Canadian Association of Pediatric Surgeons, Children's Oncology Group, Eastern Association for the Surgery of Trauma, Society for Surgery of the Alimentary Tract,Society of University Surgeons, and Trauma Association of Canada
Disclosure: Nothing to disclose.
Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine
Malrotation and midgut volvulus with intestinal ischemia. Note narrow pedicle at base of mesentery. No resection was required since ischemic necrosis had not yet developed.
Operative photograph illustrating midgut volvulus of full-term newborn who underwent upper GI contrast study. Note complete twist (> 360°) of entire small bowel over narrow pedicle of its mesentery. Note appearance of small bowel and congestion and cyanosis due to vascular compromise from volvulus. Fortunately, early operative intervention prevented development of necrosis, and emergent untwisting combined with Ladd procedure was successful.
Operative photograph of midgut volvulus due to intestinal malrotation in 10-year-old patient. Note twisting at base of mesentery with evidence of intestinal congestion and ischemia but no necrosis.
Operative photograph of patient with midgut volvulus in which diagnosis was made late. Note that entire small bowel is necrotic and nonviable. This infant did not survive.
Plain abdominal radiograph shows dilated stomach and proximal bowel with some air distally (ie, double-bubble sign).
Upper GI series of child with malrotation and midgut volvulus that reveals abnormal position and obstruction in third part of duodenum.
Upper GI contrast study of full-term newborn baby with bilious vomiting. Note obstruction at third and fourth portions of duodenum with "bird's-beak" appearance, which is compatible with midgut volvulus and malrotation.
Upper GI contrast study in a 10-year-old patient with intestinal malrotation. Note normal appearance of stomach but abnormal shape of duodenum. Duodenum does not have C-loop appearance, it does not cross back over midline (spine), and proximal jejunum is located on right side of abdomen. In addition, this patient had partial volvulus illustrated by corkscrew appearance of duodenum.
Lateral view from upper GI series reveals duodenum with corkscrew appearance.
Barium enema of 1-year-old infant with history of intermittent bilious vomiting. Colon is positioned abnormally, with most of it on left side of abdomen. Note cecum and terminal ileum in left upper quadrant of abdomen.
Barium enema in child with malrotation and midgut volvulus. Note cecum in right upper quadrant and dilated loops of small bowel.
Ultrasound image with Doppler flow revealing twisted superior mesenteric artery and vein in child with midgut volvulus.
CT scan of abdomen in child with midgut volvulus. Note twisted mesentery and bowel anterior to right kidney.
CT scan of 4-year-old patient with intermittent episodes of bilious vomiting. CT scan was performed at time of volvulation of the midgut. Note whirl or swirl appearance in midabdomen at site of narrow pedicle of superior mesenteric artery, which is compatible with acute twist observed during midgut volvulus.
Operative findings of malrotation of gut with volvulus.
Diagram illustrating operative maneuver to untwist volvulized midgut. Note that untwisting is performed in counterclockwise fashion by operating surgeon. Once this is accomplished, Ladd procedure is completed by dividing any obstructing bands and by broadening base of mesentery.
Malrotation and midgut volvulus with intestinal necrosis. Massive resection of small bowel was required, but child survived and was eventually weaned off total parenteral nutrition.
Upper GI contrast study in a 10-year-old patient with intestinal malrotation. Note normal appearance of stomach but abnormal shape of duodenum. Duodenum does not have C-loop appearance, it does not cross back over midline (spine), and proximal jejunum is located on right side of abdomen. In addition, this patient had partial volvulus illustrated by corkscrew appearance of duodenum.
Upper GI contrast study of full-term newborn baby with bilious vomiting. Note obstruction at third and fourth portions of duodenum with "bird's-beak" appearance, which is compatible with midgut volvulus and malrotation.
Barium enema of 1-year-old infant with history of intermittent bilious vomiting. Colon is positioned abnormally, with most of it on left side of abdomen. Note cecum and terminal ileum in left upper quadrant of abdomen.
CT scan of 4-year-old patient with intermittent episodes of bilious vomiting. CT scan was performed at time of volvulation of the midgut. Note whirl or swirl appearance in midabdomen at site of narrow pedicle of superior mesenteric artery, which is compatible with acute twist observed during midgut volvulus.
Operative photograph illustrating midgut volvulus of full-term newborn who underwent upper GI contrast study. Note complete twist (> 360°) of entire small bowel over narrow pedicle of its mesentery. Note appearance of small bowel and congestion and cyanosis due to vascular compromise from volvulus. Fortunately, early operative intervention prevented development of necrosis, and emergent untwisting combined with Ladd procedure was successful.
Operative photograph of midgut volvulus due to intestinal malrotation in 10-year-old patient. Note twisting at base of mesentery with evidence of intestinal congestion and ischemia but no necrosis.
Diagram illustrating operative maneuver to untwist volvulized midgut. Note that untwisting is performed in counterclockwise fashion by operating surgeon. Once this is accomplished, Ladd procedure is completed by dividing any obstructing bands and by broadening base of mesentery.
Operative photograph of patient with midgut volvulus in which diagnosis was made late. Note that entire small bowel is necrotic and nonviable. This infant did not survive.
Operative findings of malrotation of gut with volvulus.
Plain abdominal radiograph shows dilated stomach and proximal bowel with some air distally (ie, double-bubble sign).
Lateral view from upper GI series reveals duodenum with corkscrew appearance.
Upper GI series of child with malrotation and midgut volvulus that reveals abnormal position and obstruction in third part of duodenum.
Barium enema in child with malrotation and midgut volvulus. Note cecum in right upper quadrant and dilated loops of small bowel.
Ultrasound image with Doppler flow revealing twisted superior mesenteric artery and vein in child with midgut volvulus.
CT scan of abdomen in child with midgut volvulus. Note twisted mesentery and bowel anterior to right kidney.
Malrotation and midgut volvulus with intestinal ischemia. Note narrow pedicle at base of mesentery. No resection was required since ischemic necrosis had not yet developed.
Malrotation and midgut volvulus with intestinal necrosis. Massive resection of small bowel was required, but child survived and was eventually weaned off total parenteral nutrition.
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