Malrotation

Malrotation is a developmental lack of fixation of the intestine that can lead to twisting of the intestines or bowel. This lack of fixation occurs in utero but the twisting usually occurs later. Eighty percent of the patients who have a twist (mid gut volvulus) have this in the first year of life. If the twisting occurs it can lead to severe infection and loss of up to 95% of the intestines. The long-term consequences of this “short gut syndrome” are severe.

May be associated with:

• Diaphragmatic hernia
• Abdominal wall defects such as gastroschisis and omphalocele
• Other abnormalities of the intestine

Problems that occur from malrotation:

• Bands of tissue, Ladd’s bands, may form, causing an obstruction of the duodenum, the first part of the small intestine.
• Volvulus occurs when the bowel twists upon itself, cutting off the blood supply to the tissue.
• Both conditions can be potentially life-threatening.

Symptoms:

• Pain, cramping
• Green or bilious vomiting, especially in an infant
• Tender abdomen
• Diarrhea or bloody stools
• Rapid heart rate and breathing
• Little or no urine output
• Fever

Treatment:

• Almost always requires surgery
• If elective may be done laparoscopically, but in cases of emergency require open surgery

Surgery:

Ladd’s procedure: The intestine is straightened out, the bands are divided, the mesentery which holds the bowel in place is widened to provide a more stable base for the bowel. The small intestine is placed on the right side of the abdomen and the colon on the left. Usually, the appendix is removed, as well.

In cases where the intestine has lost its blood supply due to twisting, a portion of the intestine is removed. Results are directly related to how much intestine has died and require removal. In these cases the patients may be severely sick with sepsis. This will occasionally require an ileostomy or colostomy. This is usually temporary and can be reversed several months later with another operation.