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Condition: Malrotation (rotational anomalies, malrotation with volvulus)
Overview (“What is it?”)
- Malrotation is the failure of normal intestinal rotation and fixation during the development of the fetus. In the first trimester of pregnancy, the lungs develop very quickly and the fetus’ (baby’s) intestines go outside of the belly for a certain time then returns back to the belly. Malrotation happens when the intestine does not follow the normal way that they are supposed to be inside the belly. When malrotation is present, there are two things that can give a child problems: (1) volvulus which is when the intestine twists upon itself, cutting off its own blood supply and (2) abnormal adhesions or bands that can partially block the passage of contents within the intestines. Volvulus (Figure 1) is dangerous because it can cut off the blood supply to most of the intestines causing the intestines, and sometimes the child, to die.
Figure 1. Volvulus
- Malrotation occurs in 1 in 200 to 500 of live births. Most patients are asymptomatic. Symptomatic malrotation occurs in approximately 1 in 6,000 of live births. Most patients have problems in the first months of life.
Signs and Symptoms (“What symptoms will my child have?”)
- Early signs of malrotation depend of the problems it is causing. If there are abnormal bands that are partially blocking the intestines, the baby may have intermittent vomiting that may interfere with weight gain. If there is volvulus, the classic symptom is bilious (green or bright yellow) vomiting.
- Later signs/symptoms of malrotation with volvulus include firm, distended and painful belly. There may also be blood in the stool and shock. These symptoms may signal dead intestine.
Diagnosis (“What tests are done to find out what my child has?”)
- Labs and tests: If the baby has lots of vomiting, blood tests will be sent to check levels of electrolytes (minerals) in the blood.
- Imaging studies:
- Abdominal X-rays may be obtained in a child with vomiting, initially to look for causes of vomiting.
- An upper gastrointestinal contrast study is the test of choice if malrotation is suspected. In this study, the child is given contrast to drink. Several X-rays are taken to follow the course of the contrast through the stomach and the intestine. This study would show whether the positioning of the intestines is normal or not.
- An ultrasound may be helpful to look for other causes of vomiting and belly pain such as intussusception.
- Conditions that mimic this condition include intestinal atresia, annular pancreas, meconium ileus, intussusception, Hirschsprung disease, gastroesophageal reflux, gastroenteritis.
Treatment (“What will be done to make my child better?”)
- Treatment for malrotation depends on the symptoms that the child has, the overall health, and the degree of malrotation. For example, in children with complex cardiac problems and malrotation but with NO symptoms related to malrotation, surgical intervention is not recommended. In these children, there is high risks for surgery but potentially little benefit. In children who has symptoms of vomiting and inability to gain good weight or to advice volume of feeding, doing surgical repair is done on a scheduled basis. In patients with volvulus where the blood supply to the intestine is potentially cut off, the repair is done on an emergency basis. In these cases, the longer the intestines don’t have blood flow, the higher risk it is for the intestines to die.
- Malrotation without volvulus
- Medicine: No medicine can make this condition better.
- Surgery: This is usually a scheduled operation. The name of the procedure for malrotation is called “Ladd’s procedure”—named after the surgeon who invented it. The abnormal bands causing partial obstruction are released, the integrity of the blood supply to the intestines is reassured and the appendix is removed. In malrotation, the appendix is not in the normal position, and this is why it is removed. In this set of patients, Ladd’s procedure can be done the traditional way (“open” or larger incision) or laparoscopic.
- Open: Ladd’s procedure is done through a transverse cut in the right upper part of the belly or through a vertical cut in the middle of the belly.
- Laparoscopy: Several small cuts (incisions) are made. Through one of the cuts, a video camera is placed. The surgery itself is done using small instruments placed through the other incisions. The usual number of incisions (cuts) for laparoscopic surgery vary from one (single port umbilical) to multiple.
- Preoperative preparation: Your child should shower or bathe the night before or the morning of surgery. Prior to surgery, antibiotics may be given through the vein.
- Postoperative care:
- Activity: Typically, the child is encouraged to resume regular activity as soon as possible.
- Diet: Oral feeding will resume once there is evidence that the intestines have recovered from surgery. This is different from patient to patient.
- Medicines: Your child may need any of the following:
- Antibiotics: To help prevent or treat an infection caused by bacteria.
- Anti-nausea medicine: To control vomiting (throwing up).
- Pain medicine: Pain medicine can include acetaminophen (Tylenol®), ibuprofen (Motrin®), or narcotics. These medicines can be given by vein or by mouth.
- Malrotation with volvulus
- Medicine: Patients with malrotation with midgut volvulus will need an IV for fluids and medicines to fight infection (antibiotics). If the patient is in pain, medications to help relieve discomfort may be needed.
- Surgery: Volvulus is a surgical emergency! The procedure is usually done through a horizontal or vertical cut in the belly. First, the intestines are untwisted to restore blood flow. The intestines are assessed to see how much damage is there. Sometimes, untwisting and restoring blood flow revives intestine. If the intestines are damaged beyond repair, then they will need to be removed. After the damage to the intestines are assessed and taken care of, then a Ladd’s procedure is performed.
- Preoperative preparation: Patients need to be given fluids and antibiotics through the vein. Transfusions of blood products may be needed.
- Postoperative care: Patients will remain on antibiotics after surgery. Patients will not be fed for a period of time after surgery until their intestine begins working again. If there is a question on whether segments of intestine can recover, repeated examinations under anesthesia may be required prior to final closure.
- Risks of abdominal surgery include bleeding, infection, injury to abdominal structures.
- Benefits: Surgical intervention is required to deal with complications of malrotation with or without volvulus. In cases where there is suspected volvulus, surgery may be lifesaving.
Home Care (“What do I need to do once my child goes home?”)
- Diet: Will be based on how much intestine is remaining.
- Activity: Normal infant/pediatric activity
- Wound care: None
- Medicines: Usually none
- What to call the doctor for: Green vomit, not tolerating feedings, fever or signs of a wound infection (redness or drainage).
- Follow-up care: Surgery follow-up in 1-2 weeks. Routine pediatrician follow-up.
Long-Term Outcomes (“Are there future conditions to worry about?”)
- For malrotation without volvulus, long-term outcomes are excellent. There is a 10-15% risk of intestinal obstruction in the child’s lifetime. This complication is usually suspected if green or bright yellow vomiting, belly pain happens.
- Long-term outcomes in patients with volvulus where intestines needed to be removed are dependent upon the length of intestines remaining after surgery. Children with no bowel removed or very little removed will have normal intestinal function. Those with a lot of small bowel removed may be unable to sustain their nutrition by intestinal feeding alone and require nutrition given through the veins. Very close follow up with surgery and gastroenterology (specialists of intestinal function) is needed.
Authors: Steven L. Lee, MD; Nhan Huynh
Editors: Patricia Lange, MD; Marjorie J. Arca, MD