Malrotation is a possible cause of volvulus of the midgut around its vascular axis. Such patients pose a unique challenge for the surgeon acutely both from a diagnostic and treatment standpoint. Malrotation is in itself harmless, and an infant can have malrotation without a volvulus. Contrastenhanced ct, performed 1 h later from echography, revealed a whirlpool sign suggesting an intestinal malrotation with midgut volvulus, but with no signs of residual intrahepatic gas. The frontal ugis view a shows mildly inferiorly displaced djj. Ct abdomen showing intestinal malrotation with suspected midgut volvulus. The most feared complication of intestinal malrotation is midgut volvulus with ischemia and eventual necrosis due to the narrowbased mesentery that is associated with malrotation. There are swirling proximal jejunal loops arrowhead. First described in 1941, the traditional surgical repair for malrotation is division and lysis of ladds bands, straightening. Bilious vomiting with abdominal distension i n a neonate infant. Malrotation is a possible cause of volvulus of the midgut.
Asymptomatic malrotation is present in 1 in 500 births. Appropriate management of the child with malrotation andor midgut volvulus requires a multidisciplinary approach with early clinical suspicion, rapid confirmation of diagnosis, and expedient operative intervention in order to obtain the most effective outcomes. In regard to accuracy, it does no good to make comparisons between publications that negatively distort the accuracy of upper gi series for malrotation and malrotation with volvulus versus publications that positively distort the accuracy of us for malrotation and malrotation with volvulus. Recurrent acute pancreatitis in bowel malrotation european. Congenital intestinal malrotation with midgut volvulus. Case reports colonic volvulus in an adult after childhood ladds procedure for malrotation megan addink, md, aziz m. Midgut volvulus as a complication of intestinal malrotation. Intestinal rotation abnormalities and midgut volvulus. Laveran, marseille armees, france address for correspondence.
Intestinal malrotation is a birth defect involving a malformation of the intestinal tract that occurs while a fetus is forming in its mothers uterus. Upon exploration it was clear that congenital malrotation with midgut volvulus was present the cecum was free floating in the right upper quadrant figure 3, the right ureter, inferior vena cava, and aorta were readily apparent to the right of the root of the mesentery figure 4, and significant fibrofatty bands contiguous with the. Bowel obstruction, ladds procedure, left side appendicitis, midgut malrotation, volvulus departments of visceral and thoracic surgery, 1radiology, hopital dinstruction des armees a. The mortalityin this group of patientswas 4% and represents a significantimprove. Midgut volvulus is the most feared complication of intestinal malrotation, far more common among the pediatric than the adult population. Plain abdominal and chest films were unremarkable, with no free air. The propositus, his 2 sons and 3 daughters, and his 2 grandchildren demonstrated this midgut malrotation syndrome. Midgut volvulus is a serious anomaly that develops as a result of narrow attachment of midgut mesentery due to a rotation anomaly, resulting in clockwise twisting of intestinal segments on the superior mesenteric artery axis. Portal venous gas in intestinal malrotation with mild midgut. Presentation in adulthood with a midgut volvulus accounts.
Figure intermittent distended proximal duodenum in a 6monthold boy with malrotation and midgut volvulus who presented with bilious emesis. Midgut volvulus is an extremely rare anomaly in adults. The subject of malrotation and midgut volvulus in infancy. Case report, intestinal malrotation, midgut volvulus, ladds. Malrotation of the midgut is generally regarded as paediatric pathology with the majority of. This frequent association is stressed as bowel necrosis occurs very rapidly.
Pdf malposition of the intestine malposition malrotation. 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. Intestinal malrotation due to its lifethreatening complication. Introduction intestinal malrotation is a congenital anomaly that is usually diagnosed in neonates or infants. The ladd procedure can be done laparoscopically or as an open procedure. However, each person may person symptoms differently. Midgut volvulus in an infant with intestinal malrotation.
Intestinal malrotation and volvulus childrens hospital of. What is the surgical treatment for midgut volvulus. Intestinal malrotation and volvulus childrens hospital. Midgut volvulus becomes symptomatic within 1 month after birth in 80% of cases. The diagnosis of intestinal malrotation with midgut volvulus was established and. Patients with malrotation of gut may develop midgut volvulus due to their narrow mesenteric base on which it twists resulting in ischaemia of bowel requiring urgent laparotomy and a ladds procedure. Sonographic examination displayed an amount of free fluid in the. Intestinal malrotation and midgut volvulus topic of. Intestinal malrotation is a failure of the gi tract to undergo normal rotation around mesenteric vessels during embryogenesis. When symptomatic, emergency ladds procedure is required to prevent a sequence of catastrophic intestinal events starting from midgut volvulus. Symptomatic malrotation midgut volvulus occurs in 1.
Chronic midgut volvulus a challenging diagnosis sciencedirect. Jan 04, 2021 abdominal us is emerging as an alternative to the upper gastrointestinal gi series as the preferred diagnostic imaging test for midgut malrotation complicated by volvulus. Jan 18, 2017 one week later, although the patient presented with normal vital signs, the abdominal pain and vomiting worsened and there was an increase in creactive protein to 102 mgl. This work is reported in line with the scare criteria. The most common symptoms are vomiting bile, stomach pain, diarrhea or constipation, and bloody stools. Intestinal malrotation and midgut volvulus ncbi nih. Apr 03, 2021 kotobi h, tan v, lefevre j, durame f, audry g, parc y.
We present the case of an 8 year old girl, with a longstanding history of atypical gastrointestinal symptoms. Feb 15, 2019 surgical treatment for midgut volvulus. Intestinal malrotation and midgut volvulus with intussusception. Midgut volvulus complicating congenital malrotation is a rare but lifethreatening condition that can occur in pregnancy. Left side appendicitis with midgut malrotation in an adult. Pdf laparoscopic treatment of intestinal malrotation in. Midgut volvulus as presentation of intestinal malrotation in. Untwisting the complexity of midgut malrotation and. Malrotation of gut with midgut volvulus is one of the common causes of neonatal intestinal obstruction presenting as bilious vomiting. Untwisting the complexity of midgut malrotation and volvulus. 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.
Asymptomatic intestinal malrotation progressing to midgut. Key points about malrotation and volvulus in children. However, more boys have symptoms by the first month of life than girls. Midgut volvulus secondary to congenital malrotation in pregnancy. In asymptomatic patients, malrotation is incidentally discovered by imaging studies obtained for other purposes. Pdf intestinal malrotation and midgut volvulus researchgate. Bariatric and pediatric emergencies for the nonpediatric, nonbariatric surgeon. Multiple articles in the literature have documented the presence of intestinal malrotation in adults. Intestinal malrotation leading to midgut volvulus is a paediatric emergency. In this age group, malrotation with midgut volvulus is one of the differential possibilities. Due to the bilious emesis, the patient underwent an upper gi flu.
Normal rotation and fixation events in an embryo occur between the 5th and 12th weeks of gestation. A cluster of dilated small bowel loops is noted in the left hemiabdomen along with airfluid levels seen on the lateral decubitus view, suggestive of an obstructive process. We present a case of intestinal infarction resulting from midgut volvulus in a healthy 32week pregnant woman who underwent emergency laparotomy and small bowel resection in the setting of fetal death in utero. Figure from imaging of malrotation in the neonate. Malrotation is rarely seen in older children, and when it does occur, symptoms may be absent or intermittent.
Midgut malrotation is a congenital condition resulting from incomplete normal intestinal rotation after physiologic gut herniation. Duplication cyst with midgut volvulus in a neonate. Congenital intestinal malrotation is an incomplete rotation and fixation of the. Midgut volvulus is a condition or disorder in which the intestine of the fetus has become twisted as a result of malrotation of the intestine during fetal development. Malrotation with midgut volvulus can be a catastrophic event resulting in intestinal ischemia and shock, and it represents a surgical emergency in the neonate. Dec 01, 2020 chronic midgut volvulus is a rare condition and the diagnosis can pose a serious challenge.
Pdf colonic volvulus in an adult after childhood ladds. Laparoscopic ladds procedure for intestinal malrotation. Unlike the upper gi series, us is free from ionizing radiation, does not require oral contrast agent, and can be performed portably and at times remotely from the interpreting radiologist, expediting diagnosis. Midgut volvulus is a rare cause of intestinal obstruction in. Sonographic findings of midgut malrotation with volvulus. Midgut volvulus symptoms, causes, corkscrew sign, treatment.
The presence of malrotation and midgut volvulus is an emergency requiring immediate surgery, which is a ladd procedure with lysis of the retroperitoneal bands and relief of the midgut volvulus. Midgut volvulus occurs as a complication of intestinal malrotation. 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. Request pdf on jan 1, 2012, nancy laurence and others published malrotation with midgut volvulus find, read and cite all the research you need on researchgate. Intestinal malrotation is more commonly diagnosed in the neonatal period, while adult presentations are reported with an incidence of 0. Neonates and infants with persistent bilious vomiting should undergo diagnostic workup and preferably ultrasound as the first step. Adult midgut malrotation presented with acute bowel. Presented by diana leigh diesen, md at the sages 20 annual meeting. Malrotation and volvulus due to the risk for loss of bowel, any infant with bilious emesis must be evaluated for malrotation and volvulus. Pdf the surgery of malrotation and midgut volvulus.
Intestinal malrotation is a congenital anomaly due to partial or complete failure of the 270 degrees counterclockwise rotation of the midgut around the superior mesenteric vessels in fetal life 1. Although the findings are often classic on images obtained in an upper gastrointestinal tract series, malrotation. This is the case of a 2 year old female who presents with abdominal pain and bilious emesis. Normally, the midgut undergoes a 270 counterclockwise rotation around the superior mesenteric artery, leading to retroperitoneal fixation of the. Portal venous gas in intestinal malrotation with mild. Cecopexy was also performed for the free cecum lying. Abdominal us is emerging as an alternative to the upper gastrointestinal gi series as the preferred diagnostic imaging test for midgut malrotation complicated by volvulus. Pdf a fourdayold boy presented with persistent bilious vomiting, bloody stained. Radiographic patterns of intestinal malrotation in children. Figure 5 a simple xray of a newborn babys abdomen with prenatal suspicion of high intestinal occlusion. Midgut volvulus occurs in 4565% of children with malrotation and still carries a mortality rate of 715%. 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. Stalker the department of pediatrics, division of medical genetics, and the center for human genetics, the montreal childrens hospital and mcgill university, montreal, quebec, canada. Reversed bowel rotation, midgut volvulus, foramen of winslow internal hernia, first reported case introduction.
Merchant, md, and peter bistolarides, md department of surgery, synergy medical education alliance, saginaw, michigan malrotation and volvulus are encountered early in infancy and case report sometimes later in childhood, but rarely in adulthood. B during the study, the proximal duodenum was intermittently distended arrow. Incidence of midgut malrotation among pediatrics is 1 in 500 livebirths whereas incidence of adult midgut malrotation has been reported between 0. Surgical concerns in malrotation and midgut volvulus. In here, we report a young male case with bowel malrotation complicated by midgut volvulus. Ultrasonographic diagnosis of midgut volvulus with. Intestinal malrotation presenting with midgut volvulus in. The fetal intestine is rotated as a result of a deviation of normal rotation of the embryonic gut.
Barber pole sign in ct angiography, adult presentation of. Chapter 65 intestinal malrotation and midgut volvulus. Some people who have malrotation go through their entire life without having any symptoms and are never diagnosed. Operative findings showed a mild volvulus with neither congestion nor ischemic change of the twisted bowel. However, the clinical presentation of intestinal malrotation in adults is more variable, and some adults are asymptomatic. Midgut volvulus with malrotation is typical in newborns. Intestinal malrotation occurs when there is arrest or disruption of the normal rotation of the embryonic gut during the fourth to twelfth week of embryonic development. Familial intestinal malrotation with midgut volvulus and. The surgery done for midgut volvulus is called as a ladds procedure and is the preferred choice of treatment for midgut volvulus.
An adult presentation of midgut volvulus secondary to intestinal. Pdf thirty four neonates presented with acute duodenal obstruction due to. Neonates and infants with persistent bilious vomiting should undergo diagnostic workup and preferably ultrasound as. The patient accepted surgery, which confirmed the midgut malrotation associated with small bowel volvulus, and a ladds procedure was done. Although most cases of intestinal malrotation in infancy can be treated successfully, in some circumstances, patients symptoms may not be detected early enough for effective treatment, and therefore may result in catastrophic midgut volvulus and death. Malrotation and volvulus in infancy and childhood by a. 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. However, because having malrotation places the infant at an increased risk for a midgut volvulus. The classical volvulus is one of the few truly acute situations in paediatric surgery.
Malrotation nonrotation heterotaxia intestinal obstruction bilious vomiting. Total midgut volvulus in adults with intestinal malrotation. Reversed bowel rotation with midgut volvulus and internal. Intestinal malrotation and catastrophic volvulus in infancy. Malrotation occurs when the intestine does not make the turns as it should. Double bubble sign with gastric and proximal duodenum distension and no distal air.
Malrotation is an abnormal turn and fixation of small bowel that can result into inconveniences. A disorder involving a gene controlling the normal gut rotation. Familial intestinal malrotation with midgut volvulus and facial anomalies. 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. In neonates, intestinal malrotation almost always presents with acute obstruction or volvulus. We describe a rare case of intestinal malrotation with mild volvulus who was diagnosed with air bubbles floating in the intrahepatic portal vein detected by bedside emergency ultrasonography. Definition table 1 malposition or malrotation of the intestine is a deviation from the known normal anatomy the duodenum to the right of the spine, the duodenojejunal flexure to the left of the spine, and the coecum in the right aspect of the lower abdomen.
An upper gi series, though not perfect, is a good test. Laparoscopic ladds procedure for intestinal malrotation in. Midgut malrotation is a congenital anomaly that occurs during embryonic development. Six of the affected had undergone a total of 24 operative procedures to alleviate problems caused by the malrotation of the midgut. A volvulus happens when the intestine becomes twisted. B ultrasound transverse cut showing the image equivalent to the sign of the double bubble with two 2 liquid filleddistended cavities. Intestinal malrotation with midgut volvulus is an important surgical emergency in pediatric practice. Any information contained in this pdf file is automatically generated from. The most common clinical presentation of midgut volvulus is bilious vomiting, which is a nonspecific sign of intestinal obstruction. Malrotation happens when the intestine doesnt turn like it should. With malfixation, the distance between these two points of attachment may become shortened, leaving the midgut loop hanging on a narrow and unstable pedicle that.
1422 710 1445 91 616 1329 195 773 437 1426 342 1543 1091 337 1354 202 1099 908 45 1301 1201 1264 439 471 191 1343 1159 755