Sep 19, 2019 a nasogastric tube was placed and fluid therapy started. Pdf intestinal malrotation and volvulus in adults researchgate. Delay in diagnosis can lead to an increase in mortality and morbidity. Intestinal malrotation and midgut volvulus johanna r. During this stage of development, the intestines normally migrate back into the abdominal cavity following a brief period where they are temporarily located at the base of the umbilical cord. Intestinal rotational abnormalities pediatric surgery nat. Malrotation with gastric volvulus, midgut volvulus, and. This paper documents the difficulties in reaching an early diagnosis. Volvulus occurs when a loop of intestine twists around itself and the. Malrotation can lead to a dangerous complication called volvulus.
Midgut volvulus due to intestinal malrotation is a rare cause of intestinal obstruction when occurring in adult life. Ad the dotted line marks the border between the intra and extraembryonic coelom. Computed tomographic diagnosis of volvulus in intestinal. At the fourth week of gestation, the gi system is a straight tube centrally located in the abdomen. Access to the right lower quadrant was achieved by a modified hasson technique with a step trocar autosuture. Midgut volvulus occurs in people usually babies that are predisposed because of congenital intestinal malrotation. The main diagnosis is based on radiological features seen. Reversed bowel rotation with midgut volvulus and internal. Intestinal malrotation is the result of abnormal rotation of the intestinal tract and resultant abnormal fixation of the intestine. The recurrent midgut volvulus was successfully treated by a fixation procedure cecopexy and duodenopexy.
Midgut malrotation first presenting as acute bowel obstruction in. Malrotation is a possible cause of volvulus of the midgut around its vascular axis. 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. Midgut malrotation is an anomaly of the fetal intestinal rotation occurring in 1 of 6000 live births.
Acute or chronic midgut volvulus or duodenal obstruction. Surgical management of intestinal malrotation in adults. Malrotation and volvulus in infancy and childhood by a. Contrastenhanced computerized tomography ct revealed malrotation of the gut. Note the corkscrew appearance of the contrast in the. Malrotation of the midgut is generally regarded as paediatric pathology with the majority of patients presenting in childhood. Cecal volvulus associated with intestinal malrotation presenting as. Malrotation american pediatric surgical association. Fetal midgut volvulus with meconium peritonitis detected. A spiral appearance of the distal duodenum and proximal jejunum giving a corkscrew configuration on barium would indicate midgut volvulus.
Discrimination of 3 midgut segments at ed 15 at ed 15 figure 4, the 3 distinct parts of the midgut are clearly discernable. Seven patterns of duodenal malrotation were observed. Midgut malrotation with volvulus has classically affected males more than females but has also been reported as having the same predominance. 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. Malrotation of the small bowel is the major predisposing factor for midgut volvulus. Laparoscopic rouxeny gastric bypass lrygb is 1 of the most common procedures performed for severe obesity. Intestinal malrotation is a major diagnostic challenge in the newborn period. Kenney introduction malrotation is a spectrum of anatomic abnormalities of incomplete rotation and fixation of the intestinal tract during foetal development. An adult case of midgut volvulus in familial visceral. Clinically signs of fetal midgut volvulus are not pathognomonic, such as intestinal dilatation, abdominal mass, ascites, peritoneal calcifications, or polyhydramnios. We report a case of small bowel obstruction secondary to a.
An eighteenmonthold boy who had undergone a ladds procedure for malrotation in the newborn period presented with acute onset of nausea, vomiting, rectal bleeding, and confusion. 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. 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. Intestinal malrotation and volvulus in infants and children. Sonographic findings of midgut malrotation with volvulus. Neonatal malrotation with midgut volvulus mimicking duodenal. It is believed to occur in approximately 1 in 500 live births. Intestinal malrotation presenting with midgut volvulus in. Malrotation with midgut volvulus can be a catastrophic event resulting in intestinal ischemia and shock, and it represents a surgical emergency in the neonate. Jun 22, 2016 the diagnosis of intestinal malrotation with midgut volvulus was established and the treated surgically. Lateral view of an upper gastrointestinal study shows intestinal malrotation with midgut volvulus. Malrotation can lead to a dangerous complication called volvulus malrotation can refer to a spectrum of abnormal intestinal positioning, often including. Laparoscopic steering wheel derotation technique for midgut.
Malrotation can refer to a spectrum of abnormal intestinal positioning, often including. Almost all children in the series had abnormalities of rotation or fixation of both the duodenum and colon, resulting in narrowing of the mesenteric base with potential for midgut volvulus. Intestinal malrotation leading to midgut volvulus is a paediatric emergency. When volvulus involves the entire small bowel, it is referred to as midgut volvulus. Malrotationan unexpected finding at laparoscopic rouxen. Intestinal malrotation is a congenital anomaly of rotation of the midgut. 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.
Novel variant of reversed midgut rotation retroarterial. This video shows a stepwise laparoscopic ladd procedure done in a day old female infant who presented with worsening bilious emesis and ultrasound findings suspicious of malrotation and midgut volvulus. Chapter 65 intestinal malrotation and midgut volvulus. This can result in the loss of most of the intestine. Neonatal malrotation with midgut volvulus mimicking.
We present a video case report of a patient incidentally found to have midgut congenital malrotation at lrygb. Malrotation of the intestine results when the normal embryologic sequence of bowel development and. The diagnosis of intestinal malrotation with midgut volvulus was established and the treated surgically. It usually presents in the first month of life with bilious vomiting resulting from duodenal obstruction or volvulus. Symptomatic intestinal malrotation first presenting in the adults is rare. Case of midgut malrotation it is a congenital anomaly. 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. Contrastenhanced ct can readily help the radiologist recognize perfusion abnormalities of the bowel, which is vital for reducing morbidity and. Pdf midgut volvulus due to intestinal malrotation is a rare cause of intestinal obstruction when occurring in adult life. Superior mesenteric vein smv was turning around sma in a clockwise fashion, and then following its course toward portal vein figs 1a and c. Midgut volvulus is a complication of malrotated bowel. No evidence of volvulus is noted on barium study or clinically.
Intestinal malrotation in neonates with nonbilious emesis. It occurs during the first trimester as the fetal gut undergoes a complex series of growth and development. Most children who have symptoms of malrotation and volvulus are less than 1 year old and most babies. Congenital midgut malrotation is rarely en countered outside the pediatric. Ct scan showed abnormal location of the midgut and abnormal. The most common clinical presentation of midgut volvulus is bilious vomiting, which is a nonspecific sign of intestinal obstruction. The propositus, his 2 sons and 3 daughters, and his 2 grandchildren demonstrated this midgut malrotation syndrome. The patient was operated and the operation confirmed the diagnosis. 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. Radiographic patterns of intestinal malrotation in children. The gastric volvulus and midgut volvulus resulted in both proximal and. The diagnosis of intestinal malrotation with midgut volvulus was. Midgut malrotation is a congenital anomaly that occurs during embryonic development.
Incidental anatomic abnormalities found at surgery are uncommon and can require an alternative operative approach. Consequently, the diagnosis is usually disclosed with imaging or surgery. Preoperative computerised tomography scan showed small bowel obstruction and features in keeping with midgut malrotation. Variants include incomplete, nonrotation, or reversed rotation. A nasogastric tube was placed and fluid therapy started. Infants with malrotation and midgut volvulus often are described as having bilestained emesis, abdominal distention, and peritoneal signs and may appear ill. Successful outcome of this clinical condition depends on prompt diagnosis and timely surgical intervention. Intestinal rotational abnormalities is a topic covered in the pediatric surgery nat. Volvulus of the cecum, transverse colon, or sigmoid colon occurs, usually in adults, with only minor predisposing factors such. 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 is a feared pediatric surgical emergency as the loss of small intestine and the ensuing risk of short gut syndrome and intestinal failure can cause significant morbidity and mortality. Portal venous gas in intestinal malrotation with mild midgut. A diagnosis of malrotation was established in only 1 patient, whereas the other 6 were diagnosed on laparoscopy.
Delayed presentation of familial intestinal malrotation with volvulus in two adult siblings. Intestinal malrotation and volvulus in adult life sciencedirect. The whirl pattern around the superior mesenteric artery found on ct scan in patients with. We report an adult case of midgut volvulus in familial visceral myopathy fvm that had affected family members over three generations. Intestinal malrotation an overview sciencedirect topics.
Malrotation may result from predisposing genetic mutations and are often found in conjunction with other congenital malformations 1, 2, 5, 10. They become apparent when the gut is filled with air in the first. We present a rare case of malrotation in a 14yearold patient who presented with cramping, generalized right abdominal pain, and vomiting for a duration of one day. The surgery done for midgut volvulus is called as a ladds procedure and is the preferred choice of treatment for midgut volvulus.
A rare but serious complication of ladds procedure. Disorders of intestinal rotation and fixation are of paramount impor. Intestinal malrotation is a congenital condition that can have disastrous, potentially fatal sequelae. It has been estimated that it affects approximately 1 in 500 live births. In conclusion, we present a rare case of malrotation with midgut volvulus in an adult. Midgut malrotation commonly presents in the neonatal period, and rarely manifests its symptoms in adulthood with an estimated incidence of 0. Since both cecal volvulus and intestinal malrotation are rare, diagnostic delay is common and hence complications. A number of other acute and chronic signs of disease are related to intestinal malrotation and lack of fixation. A knowledge of embryology is necessary to understand the mechanisms that lead to the development of volvulus and malrotation. In addition, there were small bowel volvulus and a segment of terminal ileal. 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. Volvulus is associated with intestinal malrotation, an enlarged colon. Pdf surgically managed isolated midgut malrotation a. However, another similar rare entity known as neonatal primary segmental volvulus without malrotation has been reported before.
The most common clinical manifestation of intestinal malrotation in neonates is an acute duodenal obstruction or midgut volvulus, a lifethreatening condition in which the bowel and its accompanying mesenteric vessels twist around the short mesenteric pedicle resulting in bowel obstruction and ischemia. Midgut volvulus as a complication of intestinal malrotation in adults. Nevertheless, the symptoms are nonspecific with no strong pointers towards the clinical diagnosis. Although 62% presented as neonates, 20% were over 1 year of age. However, the true incidence is difficult to determine as a substantial number of. Given his acute presentation, operative intervention was the natural trajectory in his management. Ct is a quick and comprehensive examination in the evaluation of complex acute abdominal pathology in children. Six of the affected had undergone a total of 24 operative procedures to alleviate problems caused by the malrotation of the midgut. Midgut malrotation is a congenital anomaly in the embryological development of the foetal intestinal rotation. Neonatal terminal ileum volvulus in the absence of malrotation has never been reported before in english literature. Portal venous gas in intestinal malrotation with mild. Pdf intestinal malrotation and midgut volvulus researchgate. The most feared complication of intestinal malrotation is midgut volvulus, vascular compromise, and eventual bowel necrosis due to the narrow mesenteric base.
Malrotation is considered any deviation of the normal rotation of the midgut in embryological. Presentation of intestinal malrotation syndromes in older children. The principal complication of malrotation is midgut volvulus, which can be a lifethreatening condition requiring immediate surgical intervention 1. Laparoscopic steering wheel derotation technique for midgut volvulus in children with intestinal malrotation. Jan 20, 2014 presented by diana leigh diesen, md at the sages 20 annual meeting. Red dotted lines represent the path of sma and its branches. Intestinal malrotation presenting as midgut volvulus after. Midgut volvulus is the most common complication of malrotation in the adults. What is the difference between malrotation and volvulus. Midgut volvulus, the most common serious complication of malrotation, can be diagnosed using conventional contrast fluoroscopy, us or ct. Primary midgut volvulus in a patient with marfan syndrome. Intestinal malrotation and midgut volvulus in adulthood are rare events.
Intestinal malrotation with midgut volvulus is an important surgical emergency in pediatric practice. Midgut volvulus often occurs early in life, and in such cases surgery is performed to repair. 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. Recurrent intestinal volvulus in midgut malrotation. Midgut malrotation refers to lack or incomplete rotation of the midgut around the axis of the superior mesenteric artery during embryonal development.
The smallbowel loops encircling the superior mesenteric artery create a whirllike pattern that may be distinctive for this diagnosis. The patient was a japanese woman in her fifties, who had chronic intestinal pseudo. Corkscrew duodenum in malrotation with a midgut volvulus. Radiographic patterns of intestinal malrotation in. Intestinal ischemia introduction twisting of the small bowel around its mesenteric artery axis is termed midgut volvulus, frequently causing luminal obstruction and most importantly compromising blood flow to and from the bowel wall threatening intestinal viability. Reversed bowel rotation, midgut volvulus, foramen of winslow internal hernia, first reported case introduction. Laparoscopic treatment of intestinal malrotation in. We present the case of a 28yearold woman who presented with vomiting and mild acute pain in the abdomen.
The fetal intestine is rotated as a result of a deviation of normal rotation of the embryonic gut. These abnormalities lead to increased risks of bowel obstruction and midgut volvulus. 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. The computed tomographic appearance of volvulus in intestinal malrotation in an adult is presented. Intestinal malrotation and midgut volvulus sciencedirect. Most patients present with bilious vomiting and signs of obstruction in the neonatal period. Epidemiology a midgut volvulus of malrotated bowel can potentially occur at any ag. Laparotomy revealed midgut volvulus, mesenteric lymphadenopathy and massive. Segmental volvulus occurs in people of any age, usually with a predisposition because of abnormal intestinal contents e. The clinical diagnosis of intestinal malrotation in the older child is not always easy because of its nonspecific presentations. Intestinal malrotation and volvulus intestinal malrotation is a defect that occurs at the 10th week of gestation.
They all presented with features of intestinal obstruction. Because of more differential diagnosis, computed tomography ct scan can play an important role in the evaluation of patients with this abnormality. A segment of small bowel volvulus was resected and an endtoend anastomosis was performed with uneventful recovery. Vomiting was the sympton of paramount importance in 97% but was not initially bilestained in 20%. The most reported cases concerning pvg with intestinal malrotation are associated with bowel necrosis induced by midgut volvulus 1, 3,4,5,6,7. Intestinal malrotation and volvulus in infants and. Midgut malrotation with congenital peritoneal band. With malrotation, the intestines do not stick in the right spot and get twisted. Intrauterine volvulus of terminal ileum without malrotation. Emergency laparotomy findings confirmed midgut malrotation with absent appendix, abnormal location of caecum, ascending colon and small bowel. Jun 05, 2015 midgut malrotation refers to twisting of the entire midgut about the axis of the superior mesenteric artery sma. It is particularly important to recognize intestinal malrotation and midgut volvulus as mimics of the doublebubble sign.
Seven adults with midgut volvulus as a complication of malrotation are presented. Surgical technique, christine wang, tyrone rogers, katherine su, eugene. Intestinal malrotation and volvulus cincinnati childrens. 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. Utility of the ct scan in diagnosing midgut volvulus in.
Learningradiology malrotation with a midgut volvulus. The patient underwent an emergency laparotomy with an uneventful postoperative recovery. Recurrent midgut volvulus in an adult patient the case for pexy. The various imaging appearances of volvulus of the gastrointestinal tract are. Unusual presentation of midgut malrotation with incidental. The diagnosis of malrotation and volvulus in the older child and adult. Malrotation with volvulus is a true lifethreatening emergency. The diagnosis is rare in adults, which sometimes results in delayed diagnosis and treatment. Midgut malrotation is an anomaly of intestinal rotation that occurs during fetal development and usually presents in the neonatal period. Pdf the surgery of malrotation and midgut volvulus.
Intestinal malrotation presenting with midgut volvulus in an. Midgut volvulus in an infant with intestinal malrotation. It may result in proximal bowel obstruction with resultant ischemia if prompt treatment is not instigated. Malrotation of midgut was made with these ct findings. Intestinal malrotation is a very common cause of midgut volvulus in the pediatric population. Intestinal malrotation with midgut volvulus presenting as.