Basics
Description
Volvulus represents an abnormal rotation (torsion) of the viscera that results in ischemia. Gastric, cecal, and midgut volvulus can occur. Midgut volvulus is the most common form in infants and children.
Epidemiology
- Malrotation with midgut volvulus occurs in 1 in 6,000 live births.
- Slightly more common in boys
- Most children with midgut volvulus present in the 1st month of life.
Risk Factors
- Children with malrotation have a narrow mesenteric vascular pedicle and are predisposed to volvulus.
- Familial associations can occur.
- The risk of volvulus in patients with malrotation does not decrease with age.
Etiology
Volvulus occurs due to failure of the fetal gut to undergo normal in utero rotation and fixation (malrotation), resulting in a narrow mesenteric vascular pedicle.
Commonly Associated Conditions
Up to 30% of children with malrotation have congenital heart disease. Additionally, 30 " 60% of patients with other congenital gastrointestinal (GI) malformations (gastroschisis, omphalocele, intestinal atresia, Hirschsprung disease) have malrotation.
Diagnosis
History
- The primary presenting sign is the sudden onset of bilious vomiting.
- Recurrent colicky abdominal pain
- Feeding intolerance
- Chylous ascites and/or protein-losing enteropathy due to lymphatic congestion and bacterial overgrowth
- In older children, recurrent abdominal pain and emesis and constipation
- Bloody stools or blood-tinged mucus per rectum can occur and can be late manifestations of ischemic bowel.
Alert
Infants and children with bilious emesis require evaluation to rule out malrotation and volvulus.
Physical Exam
- Infants with volvulus may manifest complaints of severe pain without significant physical exam findings (out of proportion to physical exam).
- Abdominal tenderness (mild to severe)
- Irritability, lethargy
- Palpable abdominal mass
- Edema of abdominal wall (late finding)
- Flexion of legs
- Tachypnea and tachycardia
- Hypotension (late finding)
Alert
Pitfalls in management include the following:
- Delay in diagnosis
- Failure to recognize the key sign of bilious emesis
- Failure to recognize colicky pain (in infants) or cyclic bilious emesis (in older children) as possible manifestations of malrotation
- Failure to order the diagnostic study (a limited upper gastrointestinal series [ "upper GI " ]) to evaluate the duodenal sweep
Diagnostic Tests & Interpretation
Lab
Laboratory analysis is unpredictable. May see elevated acute phase reactants, leukocytosis, metabolic acidosis, and thrombocytopenia
Imaging
- No imaging modality is 100% sensitive.
- The goal is to delineate whether the duodenum follows a normal sweep to the right of the vertebral bodies before transition to the jejunum.
- The 3rd portion of the duodenum crosses behind the superior mesenteric artery to the left of the vertebral body at L1 and rises to the level of the pyloric bulb before transition into the jejunum.
- Plain radiographs
- Can be normal or show a paucity of bowel gas
- A dilated stomach and duodenum (double bubble) can be present.
- Abdominal ultrasound
- May show inversion of normal position of superior mesenteric artery (SMA) and superior mesenteric vein (SMV)
- If SMA is to the right of the SMV, malrotation may be present.
- The ultrasound has a sensitivity of 80%.
- Upper GI tract (contrast) radiography
- May show abnormal position of the ligament of Treitz and, if volvulus is present, a corkscrew appearance of the midgut
- The upper GI is approximately 95% sensitive for identifying malrotation.
- False positives may occur in children with a distended stomach or ileus. In these cases, the ligament of Treitz is then pushed into an abnormal position.
- Barium enema (BE)
- Can be useful in evaluating the position of the colon and cecum
- The sensitivity of BE for diagnosing malrotation and volvulus is 75%.
- In cases of cecal, sigmoid, or transverse colonic volvulus, contrast enema shows a beak deformity at site of volvulus.
Differential Diagnosis
- Ileus
- Intestinal atresia
- Perforated viscus
- Necrotizing enterocolitis
- Meconium ileus or meconium plug syndrome
- Hirschsprung enterocolitis
- Appendicitis
- Intussusception
- Pyelonephritis
Treatment
General Measures
- Any child who presents with bilious emesis and an acute abdomen may require emergent surgical exploration. The concern for midgut volvulus is paramount, and delays for further workup may not be warranted.
- Intravenous fluid resuscitation is indicated, until normal urine output is established.
- Nasogastric decompression and intravenous antibiotics are indicated.
Surgery/Other Procedures
- Laparotomy with reduction of the torsion and inspection of the bowel for necrosis or ischemia
- The Ladd procedure consists of 4 components:
- Reduction and untwisting of the volvulus by a 360-degree counterclockwise rotation of the midgut
- Division of abnormal adhesions extending over the duodenum (Ladd bands) that are transfixing the cecum in the right upper quadrant
- Division and opening of the mesenteric attachments to provide a wider vascular base to the midgut and appendectomy
- Removal of the appendix: The appendix is removed because it is in a highly unusual place and would result in a diagnostic dilemma should the child develop appendicitis.
- Many surgeons can perform the Ladd procedure laparoscopically.
- Second-look operations may be indicated if a large portion of the midgut is ischemic; the volvulus may be reduced with reexploration in 12 " 24 hours.
- On rare occasions, bowel resection and ostomy may be necessary.
Ongoing Care
Follow-up Recommendations
Patient Monitoring
- Prognosis depends on extent of involvement and degree of bowel ischemia.
- A majority of these children develop a profound ileus and require TPN for days or potentially weeks postoperatively.
- Third-space volume loss is common postoperatively. Monitoring of intake/output is critical to maintain euvolemia.
- Postoperative small bowel obstruction and even recurrent volvulus can occur.
Complications
- Recurrent volvulus (occurs in 2% of patients)
- Short gut syndrome
- Adhesive bowel obstruction
- Wound infection and abdominal abscess
Additional Reading
- Draus JM Jr, Foley DS, Bond SJ. Laparoscopic Ladd procedure: a minimally invasive approach to malrotation without midgut volvulus. Am Surg. 2007;73(7):693 " 696. [View Abstract]
- El-Gohary Y, Alagtal M, Gillick J. Long-term complications following operative intervention for intestinal malrotation: a 10-year review. Pediatr Surg Int. 2010;26(2):203 " 206. [View Abstract]
- Fonio P, Coppolino F, Russo A. Ultrasonography (US) in the assessment of pediatric non traumatic gastrointestinal emergencies. Crit Ultrasound J. 2013;5(Suppl 1):S12. [View Abstract]
- Ladd WE. Surgical diseases of the alimentary tract in infants. N Engl J Med. 1936;215:705 " 708.
- Malek MM, Burd RS. Surgical treatment of malrotation after infancy: a population-based study. J Pediatr Surg. 2005;40(1):285 " 289. [View Abstract]
- Nagdeve NG, Qureshi AM, Bhingare PD, et al. Malrotation beyond infancy. J Pediatr Surg. 2012;47(11):2026 " 2032. [View Abstract]
- Sizemore A, Rabbani K, Ladd A, et al. Diagnostic performance of the upper gastrointestinal series in the evaluation of children with clinically suspected malrotation. Pediatr Radiol. 2008;38(5):518 " 528. [View Abstract]
- Stephens LR, Donoghue V, Gillick J. Radiological versus clinical evidence of malrotation, a tortuous tale " 10-year review. Eur J Pediatr Surg. 2012;22(3):238 " 242.
Codes
ICD09
- 560.2 Volvulus
- 537.89 Other specified disorders of stomach and duodenum
- 751.5 Other anomalies of intestine
ICD10
- K56.2 Volvulus
- K31.89 Other diseases of stomach and duodenum
- Q43.8 Other specified congenital malformations of intestine
SNOMED
- 9707006 intestinal volvulus (disorder)
- 71851009 Gastric volvulus (disorder)
- 253786009 Congenital volvulus (disorder)
- 235811005 Cecal volvulus (disorder)
FAQ
- Q: When should workup for volvulus be initiated?
- A: In any child with bilious vomiting.
- Q: What is the best diagnostic study to confirm the diagnosis?
- A: Limited upper GI to evaluate the duodenal sweep and position of the ligament of Treitz.
- Q: What is the most common age of presentation of volvulus?
- A: In the 1st month of life; however, it can occur in any age.
- Q: What are the other types of volvulus that have been reported in children?
- A: Gastric, small bowel, and colonic volvulus can occur in children. Gastric volvulus presents with abdominal pain and retching. Two types occur: meso-axial (rotation about the lesser and greater curvature) and organo-axial (rotation around the longitudinal axis of the stomach). A majority of these children have structural abnormality of the stomach such as asplenia or abnormal fixation to the esophagus. Both small bowel and colonic volvulus present similarly to midgut volvulus, with high-grade bowel obstruction, abdominal pain, and bilious vomiting.
- Q: Can volvulus occur in teenagers and young adults?
- A: Yes. Age is not a determinant of presentation of volvulus, which may occur in older children and adults. As in infants, catastrophic consequences can occur, including entire midgut loss.
- Q: Who developed the Ladd procedure?
- A: William Edwards Ladd, MD (1880 " 1967) first described the procedure in 1936. He was a pioneer in the field of pediatric surgery. He was the first surgeon-in-chief at Boston Children 's Hospital and coauthored the first pediatric surgical textbook.