A volvulus is a medical condition where the intestines twist upon themselves. This condition can occur at any age. However, it is more frequent in children and infants. A volvulus can cause a blockage that may cut off blood flow usually due to a congenital disability called intestinal malrotation; this can occur in any part of the intestine without this condition present. Intestinal malrotation can make an infant more likely to develop a midgut volvulus and occurs in the first few weeks of life. A midgut volvulus is usually part of a vascular compromise in the intestinal mesentery in intestinal malrotation.
During fetal development, the intestines develop as three portions. These are foregut, midgut, and hindgut. The midgut portion of the intestines undergoes normal rotation during the four weeks and six weeks of gestation. The fetal midgut intestines develop so rapidly that the abdominal cavity cannot house the all the intestines. Therefore, a portion of intestines herniates into the umbilical cord. The intestines herniate back into the abdominal cavity with a 270-degree counter-clockwise rotation around the bloody supply to midgut, the superior mesenteric artery. The fourth portion of the duodenum and proximal jejunum of the intestines acquires a C-loop, with the development of the ligament of Treitz, and is superior to the superior mesenteric artery. The cecal portion of the intestine becomes fixed to the retroperitoneum, superior to the superior mesenteric artery, in a diagonal axis at approximately the level of the T12 vertebra to the level of the right of the L5 vertebra.
Anomalies of rotation and fixation are twice as common in males as in females. They are classified as non-rotation, incomplete rotation, reverse rotation, and anomalous fixation of the mesentery.
Genetic mutations likely disrupt the signaling critical for normal intestinal rotation. For instance, mutations in the gene BCL6 leading to the absence of left-sided expression of its transcript result in malrotation. In an incomplete rotation, the cecum remains in the epigastrium, and the fibrotic band forms from the duodenum to the retroperitoneum as the cecum continues to form. The band, also knowns as Ladd’s bands, crosses over the duodenum, connecting the cecum to the lateral abdominal wall. The Ladd's band creates a potential point of obstruction.
The mesenteric takeoff is then confined to the epigastrium leading to a narrow pedicle, constricting all the branches of the superior mesenteric artery that supplies the midgut. A volvulus can occur around the mesentery which then obstructs the proximal jejunum and causes ischemia to the midgut. Obstruction and bowel ischemia can then ensue if the problem is not promptly corrected surgically.
Midgut volvulus can happen at any age, but most commonly occurs during the first few weeks of life. Bilious emesis is usually the first sign of volvulus. Infants that present with bilious vomiting, acute duodenal obstruction, or abdominal tenderness associated with hemodynamic deterioration must be rapidly evaluated to ensure that they do not have intestinal malrotation along with volvulus, as this should raise particular suspicions for this diagnosis. If left untreated, vascular compromise of the midgut initially causes bloody stools but eventually results in circulatory collapse. Physical exam findings for the advanced disease will show signs of peritonitis suggestive of intestinal ischemia with erythema and edema of the abdominal wall which will lead to shock and death if left untreated. An index of suspicion for this condition must be high, as the abdominal signs are minimal early in the disease process. Plain abdominal x-rays show a paucity of gas throughout the intestine with a few scattered air-fluid levels. When these findings are present, the patient should undergo immediate fluid resuscitation to ensure adequate perfusion and urine output followed by prompt exploratory laparotomy. In cases where the child is stable, consider laparoscopy.
In older children and adults, abdominal pain is the most common symptom and may present with abrupt onset over hours or days or as chronic intermittent pain over weeks, months, or years. Intermittent vomiting, chronic diarrhea, malabsorption, or failure to thrive comprises other potential presenting symptoms.
The patient may not always appear ill, and the plain films may suggest partial duodenal obstruction. Upper gastrointestinal series that shows incomplete rotation with the duodenojejunal junction displaced to the right is the best indication of obstruction. The duodenum may show a corkscrew effect diagnosing volvulus or complete duodenal obstruction, with the small bowel looping entirely on the right side of the abdomen. A barium enema may show a displaced cecum, but this sign is unreliable, especially in a small infant in whom the cecum is normally in a somewhat higher position than in an older child .
When volvulus is suspected, early surgical intervention is mandatory if the ischemic process is to be prevented or reversed. Volvulus occurs clockwise and is therefore untwisted counterclockwise; which one can remember with the phrase "turning back the hands of time."
Follow by a Ladd procedure, named after William Edward Ladd, the pediatrician who first performed the procedure in 1936. This operation does not correct the malrotation but instead helps to open the narrow mesenteric pedicle to prevent volvulus from recurring. The Ladd procedure is performed by lysing the band formed between the cecum and the lateral abdominal wall as well as duodenum and terminal ileum, which allows the superior mesenteric artery to relax. This maneuver allows the duodenum to relax into the right lower quadrant and the cecum into the left lower quadrant; these structures do not need to be secured with a suture. An appendectomy (removal of the appendix) is also done to circumvent errors in the event patient has diagnostic imaging.
The gold standard approach of the this procedure has been an open Ladd's operation; however, there are an increasing number of centers that are performing this procedure laparoscopically due to equivalent operative times compared to standard open techniques along with earlier feeds and decreased hospital stays.
When a patient presents with advanced ischemia, a simple reduction of the volvulus without the Ladd procedure should be performed followed by a "second-look" laparotomy which is performed 24 to 36 hours later to evaluate the vascular integrity of the small intestines.
A transparent plastic silo may be placed to facilitate constant evaluation of the intestine and to plan for the timing of re-exploration. If a necrotic bowel is present, then the surgeon can conservatively resect it to ensure adequate length for feeding and prevention of short-gut syndrome. With early diagnosis and correction, the prognosis is excellent. Delay can lead to mortality or short-gut syndrome requiring intestinal transplantation.
A small portion patients that present with malrotation will demonstrate chronic obstructive symptoms. These symptoms may result from Ladd bands across the duodenum or, occasionally, from intermittent volvulus. Symptoms include intermittent abdominal pain and intermittent vomiting, which may occasionally be bilious. Infants with malrotation may demonstrate a failure to thrive, and they may be diagnosed initially as having gastroesophageal reflux disease (GERD). Surgical correction using the Ladd procedure, as described earlier, can prevent volvulus from occurring and improve symptoms in these instances.
A team approach coordinating the care with surgeons, clinicians, and nursing staff will lead to the best outcomes. [Level 5]
|||Millar AJ,Rode H,Cywes S, Malrotation and volvulus in infancy and childhood. Seminars in pediatric surgery. 2003 Nov; [PubMed PMID: 14655161]|
|||Birajdar S,Rao SC,Bettenay F, Role of upper gastrointestinal contrast studies for suspected malrotation in neonatal population. Journal of paediatrics and child health. 2017 Jul; [PubMed PMID: 28425590]|
|||Intestinal Rotation Anomalies., Pelayo JC,Lo A,, Pediatric annals, 2016 Jul 1 [PubMed PMID: 27403672]|
|||Sivakumar A,Mahadevan A,Lauer ME,Narvaez RJ,Ramesh S,Demler CM,Souchet NR,Hascall VC,Midura RJ,Garantziotis S,Frank DB,Kimata K,Kurpios NA, Midgut Laterality Is Driven by Hyaluronan on the Right. Developmental cell. 2018 Sep 10; [PubMed PMID: 30174180]|
|||Strouse PJ, Disorders of intestinal rotation and fixation ( [PubMed PMID: 15378215]|
|||Adams SD,Stanton MP, Malrotation and intestinal atresias. Early human development. 2014 Dec; [PubMed PMID: 25448782]|
|||Nagdeve NG,Qureshi AM,Bhingare PD,Shinde SK, Malrotation beyond infancy. Journal of pediatric surgery. 2012 Nov; [PubMed PMID: 23163993]|
|||Applegate KE,Anderson JM,Klatte EC, Intestinal malrotation in children: a problem-solving approach to the upper gastrointestinal series. Radiographics : a review publication of the Radiological Society of North America, Inc. 2006 Sep-Oct; [PubMed PMID: 16973777]|
|||Shah MR,Levin TL,Blumer SL,Berdon WE,Jan DM,Yousefzadeh DK, Volvulus of the entire small bowel with normal bowel fixation simulating malrotation and midgut volvulus. Pediatric radiology. 2015 Dec; [PubMed PMID: 26209961]|
|||Reddy AS,Shah RS,Kulkarni DR, Laparoscopic Ladd'S Procedure in Children: Challenges, Results, and Problems. Journal of Indian Association of Pediatric Surgeons. 2018 Apr-Jun; [PubMed PMID: 29681694]|
|||Tsumura H,Ichikawa T,Kagawa T,Nishihara M, Successful laparoscopic Ladd's procedure and appendectomy for intestinal malrotation with appendicitis. Surgical endoscopy. 2003 Apr; [PubMed PMID: 12574927]|
|||Isani MA,Schlieve C,Jackson J,Elizee M,Asuelime G,Rosenberg D,Kim ES, Is less more? Laparoscopic versus open Ladd's procedure in children with malrotation. The Journal of surgical research. 2018 Sep; [PubMed PMID: 29937013]|
|||Bass KD,Rothenberg SS,Chang JH, Laparoscopic Ladd's procedure in infants with malrotation. Journal of pediatric surgery. 1998 Feb; [PubMed PMID: 9498402]|
|||Arnaud AP,Suply E,Eaton S,Blackburn SC,Giuliani S,Curry JI,Cross KM,De Coppi P, Laparoscopic Ladd's procedure for malrotation in infants and children is still a controversial approach. Journal of pediatric surgery. 2018 Oct 28; [PubMed PMID: 30442460]|
|||Catania VD,Lauriti G,Pierro A,Zani A, Open versus laparoscopic approach for intestinal malrotation in infants and children: a systematic review and meta-analysis. Pediatric surgery international. 2016 Dec; [PubMed PMID: 27709290]|
|||Huntington JT,Lopez JJ,Mahida JB,Ambeba EJ,Asti L,Deans KJ,Minneci PC, Comparing laparoscopic versus open Ladd's procedure in pediatric patients. Journal of pediatric surgery. 2017 Jul; [PubMed PMID: 27856011]|
|||Kulaylat AN,Hollenbeak CS,Engbrecht BW,Dillon PW,Safford SD, The impact of children's hospital designation on outcomes in children with malrotation. Journal of pediatric surgery. 2015 Mar; [PubMed PMID: 25746700]|
|||Marseglia L,Manti S,D'Angelo G,Gitto E,Salpietro C,Centorrino A,Scalfari G,Santoro G,Impellizzeri P,Romeo C, Gastroesophageal reflux and congenital gastrointestinal malformations. World journal of gastroenterology. 2015 Jul 28; [PubMed PMID: 26229394]|