Intestinal Volvulus Clinical Presentation

  • Author: Andre Hebra, MD; Chief Editor: Carmen Cuffari, MD   more...
 
Updated: Jan 20, 2012
 

History

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.

Operative photograph illustrating 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 intOperative 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.

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).

Operative photograph of patient with midgut volvulOperative 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.
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Physical Examination

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]

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Contributor Information and Disclosures
Author

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 Southern Medical Association

Disclosure: Nothing to disclose.

Chief Editor

Carmen Cuffari, MD  Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, and Royal College of Physicians and Surgeons of Canada

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: 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 Southern 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: 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 Southern 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.

References

  1. Houshian S, Sørensen JS, Jensen KE. Volvulus of the transverse colon in children. J Pediatr Surg. Sep 1998;33(9):1399-401. [Medline].

  2. Reid J. Anatomical observations. Edinburgh M. & S. J. 1836;46:70.

  3. Mall FP. Development of the human intestine and its position in the adult. Bulletin of Johns Hopkins Hospital. 1898;9:197.

  4. Dott NM. Anomalies of intestinal rotation: Their embryology and surgical aspects with report of five cases. Brit J Surg. 1923;11:251.

  5. Ladd WE. Congenital obstruction of the duodenum in children. NEJM. 1932;206:277-83.

  6. Messineo A, MacMillan JH, Palder SB. Clinical factors affecting mortality in children with malrotation of the intestine. J Pediatr Surg. Oct 1992;27(10):1343-5. [Medline].

  7. Lesher AP, Dixon JA, Barbour JR, Hebra A. Recurrence of midgut volvulus after a Ladd procedure. Am Surg. Jan 2010;76(1):120-2. [Medline].

  8. Walker GM, Neilson A, Young D, Raine PA. Colour of bile vomiting in intestinal obstruction in the newborn: questionnaire study. BMJ. Jun 10 2006;332(7554):1363. [Medline]. [Full Text].

  9. Murphy MS. Management of bloody diarrhoea in children in primary care. BMJ. May 3 2008;336(7651):1010-5. [Medline]. [Full Text].

  10. Welte FJ, Grosso M. Left-sided appendicitis in a patient with congenital gastrointestinal malrotation: a case report. J Med Case Reports. Sep 19 2007;1:92. [Medline]. [Full Text].

  11. Ford EG, Senac MO Jr, Srikanth MS. Malrotation of the intestine in children. Ann Surg. Feb 1992;215(2):172-8. [Medline].

  12. Dufour D, Delaet MH, Dassonville M. Midgut malrotation, the reliability of sonographic diagnosis. Pediatr Radiol. 1992;22(1):21-3. [Medline].

  13. Jabra AA, Fishman EK. Small bowel obstruction in the pediatric patient: CT evaluation. Abdom Imaging. Sep-Oct 1997;22(5):466-70. [Medline].

  14. Hsiao M, Langer JC. Value of laparoscopy in children with a suspected rotation abnormality on imaging. J Pediatr Surg. Jul 2011;46(7):1347-52. [Medline].

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  17. Bill A, Grauman D. Rationale and technic for stabilization of the mesentery in cases of nonrotation of the midgut. J Pediatr Surg. 1966;1:127-36.

  18. Gohl ML, DeMeester TR. Midgut nonrotation in adults. An aggressive approach. Am J Surg. Mar 1975;129(3):319-23. [Medline].

 
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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. 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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