The clinical manifestations, diagnosis, and treatment of intestinal malrotation in adults will be discussed in this topic. Midgut malrotation refers to twisting of the entire midgut about the axis of the superior mesenteric artery sma. Embryology, signs and symptoms, diagnosis and treatment for. Primary midgut volvulus in a patient with marfan syndrome.
Successful outcome of this clinical condition depends on prompt diagnosis and timely surgical intervention. The opinions and assertions contained herein are the private views of the authors and are not to be constructed as official or as reflecting the views of the department of the navy or the department of defense. Neonatal malrotation with midgut volvulus mimicking duodenal. A fourdayold boy presented with persistent bilious vomiting, bloody stained stool, and mild abdominal distension. Intestinal rotation abnormalities and midgut volvulus. Malrotation with midgut volvulus can be a catastrophic event resulting in intestinal ischemia and shock, and it represents a surgical emergency in the neonate. It occurs during the first trimester as the fetal gut undergoes a complex series of growth and development. Often volvulus occurs about the superior mesenteric artery axis, which results in ischemia of regions supplied by the artery, from duodenum to the splenic flexure. The gastric volvulus and midgut volvulus resulted in both proximal and distal obstruction of the duodenum, reproducing clinically a previously reported experimental model designed to produce pancreatitis. Gut malrotation, nonrotation, and volvulus is arrest of the normal gut as it rotates around the super mesenteric artery. Nevertheless, the presentation may vary from lifethreat ening acute ischemic midgut volvulus to.
The study includes all cases in which midgut volvulus was prenatally suspected after ultrasound. Using the bowel grasper, the volvulus was derotated in an anticlockwise direction and restored to its normal anatomical position. It happens when your babys intestinal tract doesnt form as it should during pregnancy. The midgut volvulus was then identified and approximated to be around 1. Malposition of the intestine malposition malrotation volvulus midgut volvulus. All of the small bowel was examined and determined to be completely viable. Midgut volvulus is a lifethreatening complication of intestinal malrotation that should be diagnosed early and.
Any child with bilious vomiting must be assumed to have midgut volvulus until proven otherwise. Midgut volvulus in an infant with intestinal malrotation. Because of its rarity, the diagnosis of intestinal. However, this symptom is not synonymous with the diagnosis of malrotation. Intestinal malrotation an overview sciencedirect topics. Volvulus of the cecum, transverse colon, or sigmoid colon occurs, usually in adults, with only minor predisposing factors such. It can give clue about abnormal position of sma and smv.
Malrotation with gastric volvulus, midgut volvulus, and. Intestinal malrotation radiology reference article. Case report adult congenital intestinal malrotation. Intestinal malrotation is more commonly diagnosed in the neonatal period, while adult presentations are reported with an incidence of 0. Duodenum with corkscrew appearance in intestinal malrotation contrast study in duodenal atresia barium enema intestinal malrotation barium enema image of transverse colon cutoff due to midgut vol ileus plain radiograph of an infant with duodenal atresia ct whirlpool sign of midgut volvulus. An evaluation for malrotation is part of every upper gastrointestinal gi tract examination in pediatric patients, particularly neonates and infants. As noted by ladd in 1932, malrotation is a condition rare enough that it is likely to escape the mind, and it is common enough to be important. Midgut volvulus is a complication of malrotated bowel. Epidemiology a midgut volvulus of malrotated bowel can potentially occur at any ag. Vomiting was the sympton of paramount importance in 97% but was not initially bilestained in 20%. All patients were men with an average age of 33 years. Neonatal malrotation with midgut volvulus mimicking. Malrotation and volvulus due to the risk for loss of bowel, any infant with bilious emesis must be evaluated for malrotation and volvulus. Disorders of intestinal rotation and fixation are of paramount impor.
History and clinical examination a 10 yr old female patient comes to casualty with complaints of pain in epigastric region which was acute in onset since 24 days bilious vomiting since 24 days a lump was felt in the epigastrium with localised tenderness temperature was slightly raised rest parameters were within normal limits. Kenney introduction malrotation is a spectrum of anatomic abnormalities of incomplete. The classic presentation of malrotation with acute midgut volvulus is a neonate with bilious emesis. Spectrum of clinical and imaging features original research.
Pdf the surgery of malrotation and midgut volvulus. Malrotation american pediatric surgical association. This problem can be managed surgically through a laparoscopic ladd procedure. Malrotation may result from predisposing genetic mutations and are often found in conjunction with other congenital malformations 1, 2, 5, 10. Operative reduction of the midgut volvulus and the gastric volvulus and repair of the gastric perforation was accomplished. Ct angiographic demonstration of a mesenteric vessel. Intestinal malrotation presenting with midgut volvulus in an. Midgut volvulus occurs in people usually babies that are predisposed because of congenital intestinal malrotation. Infants with malrotation and midgut volvulus often are described as having bilestained emesis, abdominal distention, and peritoneal signs and may appear ill. The midgut can at any time twist around its narrow mesenteric pedicle resulting in a midgut volvulus.
Intestinal malrotation is a congenital anomaly of rotation of the midgut. Malrotation pediatrics clerkship the university of chicago. Intestinal malrotation is a pathological condition which results due to an underdeveloped intestine. Intestinal malrotation is congenital abnormal positioning of the bowel loops within the peritoneal cavity resulting in abnormal shortening of mesenteric root that is predisposed to midgut volvulus.
Symptomatic malrotation occurs in 16000 live births. Intestinal malrotation and midgut volvulus johanna r. Intestinal malrotation and volvulus in infants and. Detorsion and ladd s procedure were completed laparoscopically. Diagnosis and surgical management of congenital intestinal. We present a case of bowel malrotation complicated by midgut volvulus in an adult patient. Intestinal malrotation is most commenly complicated by volvulus in the first weeks of life. However, as the use of ct in emergency departments increases, midgut volvulus is increasingly being recognized in adults. Midgut volvulus is an extremely rare anomaly in adults.
It is particularly important to recognize intestinal malrotation and midgut volvulus as mimics of the doublebubble sign. Intestinal malrotation and midgut volvulus oxford medicine. The clinical presentation of malrotation often correlates with the age of onset 5. Ct features of the usual interstitial pneumonia pattern. Chapter 65 intestinal malrotation and midgut volvulus. A nine year experience in neonates article pdf available in annals of the royal college of surgeons of england 654. Seven adults with midgut volvulus as a complication of malrotation are presented. We present our experience to emphasize the importance of suspecting midgut volvulus as a cause of abdominal pain also beyond infancy, particularly in relation to malrotation, and the relevance of ultrasonographic us signs in. Intestinal malrotation with midgut volvulus presenting as. Midgut volvulus is a lifethreatening complication of intestinal malrotation that should be diagnosed early and treated properly to avoid sepsis and mortality. In the infant, the most common presentation is with a midgut volvulus. Intestinal malrotation leading to midgut volvulus is a paediatric emergency.
The authors have declared that no competing interests exist. Computed tomography showed an intestinal malrotation, without the duodenum crossing the midline and with the colic frame arranged in the left hemiabdomen figure 1, complicated with a midgut volvulus as demonstrated by the whirlpool sign figure 2, arrow. A 2yearold child with intestinal malrotation had simultaneous gastric volvulus, midgut volvulus, acute pancreatitis, and gastric perforation, followed postoperatively by a temporary period of both lactosuria and sucrosuria. Chapter 65 intestinal malrotation and midgut volvulus johanna r. By this method, the midgut volvulus is corrected by rotating the small intestine in anticlockwise manner with the cecum located on the left of abdomen and the duodenum down the right paravertebral gutter.
A diagnosis of malrotation was established in only 1 patient, whereas the other 6 were diagnosed on laparoscopy. Gut malrotation, nonrotation and volvulus for usmle youtube. Intestinal malrotation and volvulus childrens hospital. Intestinal malrotation with midgut volvulus is an important surgical emergency in pediatric practice. Intestinal malrotation and volvulus in infants and children. The second complication is duodenal obstruction due to ladds band. Malrotation results in narrow and long base of mesentery which predisposes bowel to twist around it. While most patients present during the neonatal period or the first year of life, some present as older children or adults.
With color doppler ultrasound, the whirlpool sign was observed. In conclusion, we present a rare case of malrotation with midgut volvulus in an adult. Differentiating connective tissue diseaseassociated interstitial lung disease from idiopathic pulmonary fibrosis. Congenital intestinal malrotation in adolescent and adult patients. Malrotation in adults may present in an acute way due to midgut volvulus or may have a chronic indolent course with recurrent vomiting and abdominal pain. Midgut volvulus midgut volvulus is a different clinical entity and is most common in children. We present our experience to emphasize the importance of suspecting midgut volvulus as a cause of abdominal pain also beyond infancy, particularly in relation to malrotation, and the relevance of ultrasonographic us signs in its diagnosis.
Corkscrew duodenum in malrotation with a midgut volvulus. Transabdominal ultrasound demonstrated a round softtissue masslike structure in the right upper quadrant. A volvulus is a problem that can occur after birth as a result of intestinal malrotation. The developmental basis for malrotation and malfixation of the midgut was first described by mall in 1898. Kenney introduction malrotation is a spectrum of anatomic abnormalities of incomplete rotation and fixation of the intestinal tract during foetal development. It can be an incidental finding or present with mild symptoms, but it can also lead to an intestinal obstruction or acute ischemia due to a midgut torsion requiring emergency surgery 1. The main presentation of intestinal malrotation is bilious vomiting and abdominal distension. Primary midgut volvulus is a term used when there is no underlying cause for the volvulus. Thirty four neonates presented with acute duodenal obstruction due to malrotation during a 9 year period between 1973 and the end of 1981.
In cases midgut volvulus, ultrasound can be the first noninvasive, radiation free, mostly available, and inexpensive imaging modality to start with. Midgut volvulus with malrotation is typical in newborns. Jun 05, 2015 midgut malrotation refers to twisting of the entire midgut about the axis of the superior mesenteric artery sma. Midgut volvulus becomes symptomatic within 1 month after birth in 80% of cases. Pdf intestinal malrotation and midgut volvulus researchgate. In patients with acute obstruction, this differential should be kept in mind, especially if the patient has no previous abdominal surgery or evidence of tuberculosis. It may result in proximal bowel obstruction with resultant ischemia if prompt treatment is not instigated. Characteristics of the patients, the pregnancy, and the delivery are presented in table 1, case by case. Congenital intestinal malrotation is a gastrointestinal anomaly whose most serious complication is midgut volvulus.
The point of obstruction is typically beyond the ampulla of vater, as demonstrated by the bilious emesis. Case report adult congenital intestinal malrotation accompanied by midgut volvulus. Segmental volvulus occurs in people of any age, usually with a predisposition because of abnormal intestinal contents e. In 1923 dott 4 applied this knowledge to the clinical problem of volvulus and intestinal obstruction.
Intestinal malrotation is a very common cause of midgut volvulus in the pediatric population. In three patients, the twisted, corkscrew appearance of the barium column in the duodenum and proximal jejunum in an upper gastrointestinal series made the diagnosis. However, more boys have symptoms by the first month of life than girls. Variants include incomplete, nonrotation, or reversed rotation.
Congenital intestinal malrotation, midgut volvulus, computed tomography introduction congenital intestinal malrotation is a complex gastrointestinal anomaly, which could easily lead to midgut volvulus and gastrointestinal obstruction. Rotation abnormalities represent a spectrum from nonrotation to normal rotation. Midgut volvulus was diagnosed most often during the third trimester, with peaks at 27 n 5 and 32 weeks of gestation n 6. Intestinal malrotation occurs when the normal rotation of the embryonic gut is arrested or disturbed during in utero development. Chapter 28 covers intestinal malrotation and midgut volvulus, and includes information on definition, demographics, clinical features, anatomy, imaging modalities and features, imaging algorithm, applied physics, differential diagnoses and pitfalls, and a bulleted summary of key points. Nov 26, 20 up to 80% of patients present in the first month of life,2 and in this age group the cardinal symptom is bile green vomiting due to duodenal obstruction through midgut volvulus. Ct angiographic demonstration of mesenteric vessel whirlpool in intestinal malrotation and midgut volvulus korean j radiol 95, october 2008 467 ab fig. Malrotation is in itself harmless, and an infant can have malrotation without a volvulus. The surgery done for midgut volvulus is called as a ladds procedure and is the preferred choice of treatment for midgut volvulus. Intestinal malrotation develops as a result of the intestines not being able to develop in.
Progressive bowel strangulation then results in an ischemic loss of extensive bowel. Four presented with long histories of intermittent abdominal pain. Intestinal malrotation presenting with midgut volvulus in. Ugi duodenal bulb volvulus corkscrew duodenum with corkscrew appearance in intestinal malrotation contrast study in duodenal atresia barium enema intestinal malrotation barium enema image of transverse colon cutoff due to midgut vol ileus plain radiograph of an infant with duodenal atresia ct whirlpool sign of midgut volvulus plain radiographs necrotizing. Congenital abdominopelvic cysts or masses can cause both high and low usually partial. Portal venous gas in intestinal malrotation with mild.
The differential diagnoses for low intestinal obstruction include ileal and colonic atresia, anorectal malformation, meconium ileus, colonic dysmotility syndromes, and hirschsprungs disease. Pdf thirty four neonates presented with acute duodenal obstruction due to malrotation during a 9 year period between 1973 and the end of 1981. Although 62% presented as neonates, 20% were over 1 year of age. During the open surgery procedure, the entire intestine was seen free in abdominal cavity. Intestinal malrotation develops as a result of a deficient rotation and fixation of the midgut segment after its passage through the umbilicus and return to coelomic space. Because of its rarity, the diagnosis of intestinal malrotation in adult patients is often delayed and therefore associated with increased morbidity. At the fourth week of gestation, the gi system is a straight tube centrally located in the abdomen. The most common clinical presentation of midgut volvulus is bilious vomiting, which is a nonspecific sign of intestinal obstruction. The prevalence of volvulus is difficult to quantify, but any patient with malrotation is considered at a risk of midgut volvulus despite the critical nature of this condition and the need for prompt surgical correction, many reports show delay between the onset of symptoms and surgical intervention 2 3 4. Midgut volvulus most patients with malrotation develop midgut volvulus within the first weeks of life. Oblique digital subtraction angiogram a of patient depicting barbers pole sign arrows due to rotations of superior. A knowledge of embryology is necessary to understand the mechanisms that lead to the development of volvulus and malrotation.
Such patients pose a unique challenge for the surgeon acutely both from a diagnostic and treatment standpoint. Red dotted lines represent the path of sma and its branches. Operative findings showed a mild volvulus with neither congestion nor ischemic change of the twisted bowel. Malrotation and volvulus in infancy and childhood by a. Intestinal malrotation and volvulus in children what is intestinal malrotation in children. However, because having malrotation places the infant at an increased risk for a midgut volvulus. Learningradiology malrotation with a midgut volvulus. Given his acute presentation, operative intervention was the natural trajectory in his management. Sonographic findings of midgut malrotation with volvulus. Midgut volvulus occurs most often in the context of congenital intestinal malrotation, in which the small intestine is not normally fastened in place. Pdf malposition of the intestine malposition malrotation. Patients with intestinal nonrotation have a lower incidence of midgut volvulus than other types of malrotation.
This condition usually begins in the first trimester around the 10th week in pregnancy. This may only be clinically apparent on upper gi fluoroscopy when the duodenum and distal small bowel is seen to fill with contrast right of midline without crossing to the left upper quadrant. Early diagnosis and surgical intervention are needed to prevent gangrene of the midgut and peritonitis. They all presented with features of intestinal obstruction. Ultrasonographic diagnosis of midgut volvulus with. The clinical course is acute with rapid progression to midgut ischemia, unstable hemodynamics, intractable metabolic acidosis. The study included cases of fetal midgut volvulus during the study period. Pdf a fourdayold boy presented with persistent bilious vomiting, bloody stained stool, and mild abdominal distension. Embryology, signs and symptoms, diagnosis and treatment for students taking. Malrotation is defined as an abnormal rotation of the duodenum andor caecum around the sma axis, with variable fixation of the midgut to the retroperitoneum 1. See intestinal malrotation in children, section on epidemiology. Aiken malrotation is a term used to describe a spectrum of anatomic abnormalities resulting from incomplete rotation and fixation of the intestine during early fetal development. Jun 22, 2016 the diagnosis of intestinal malrotation with midgut volvulus was established and the treated surgically.
Case 4 a boy born at term in no distress was placed in the wellbaby nursery. The gastric volvulus and midgut volvulus resulted in both proximal and. This video shows a stepwise laparoscopic ladd procedure done in a day old female infant who presented with. This frequent association is stressed as bowel necrosis occurs very rapidly. Intestinal malrotation and volvulus childrens hospital of. Midgut volvulus was diagnosed most often during the third trimester, with peaks at 27 n. Malrotation occurs when the intestine does not make the turns as it should. Malrotation can lead to a dangerous complication called volvulus malrotation can refer to a spectrum of abnormal intestinal positioning, often including. Characteristics of the patients, the pregnancy, and the delivery are presented intable 1, case by case. This case describes an 80yearold female with marfan syndrome presenting with primary midgut volvulus, which preoperatively was suspected based on imaging, and. Sep 19, 2019 a nasogastric tube was placed and fluid therapy started.
1341 1258 330 119 69 1393 420 397 267 541 153 393 421 844 861 702 861 560 1593 994 38 387 1297 1345 1527 222 1129 1398 563 818 615 1486 889 149 1318 139