The Ladd's procedure for correction of intestinal malrotation with volvulus in children

AORN Journal,  Feb, 2007  by Renee Ingoe,  Patricia Lange

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Intestinal malrotation with volvulus is an emergent and possibly life-threatening condition that can occur in infants and children and that requires immediate surgical intervention. The term malrotation is defined as an abnormality of the anatomic position of the bowel that occurs in utero during embryonic development. The term volvulus describes a situation in which a portion of the bowel becomes twisted upon itself. This can cause bowel necrosis from lack of a blood supply.

Symptoms of intestinal malrotation with or without a volvulus can be varied, but when a volvulus is suspected or diagnosed, emergency surgery is performed during which the intestines are untwisted to allow restoration of blood flow, and the mesentery (ie, tissue containing blood and lymphatic vessels) is widened to prevent recurrence of the volvulus. The Ladd's procedure is the gold standard for treatment of intestinal malrotation. (1)

EPIDEMIOLOGY

Intestinal malrotation occurs in approximately one in 500 births and occurs equally in males and females. (2) Approximately 60% of intestinal malrotation cases present in the first month of life, about 20% present between one month and one year of age, and the remainder present after the first year. (3) Malrotation may occur as an isolated condition, but it usually is found in combination with other congenital anomalies. As many as 70% of children with intestinal malrotation also have other congenital malformations (eg, any combination of digestive system, cardiac, or spleen and liver abnormalities). (3) When intestinal malrotation is associated with volvulus, however, the anomaly usually is the patient's only disorder. (4) Intestinal malrotation is considered a life-threatening situation when it occurs in conjunction with a volvulus that is causing a bowel obstruction.

All children diagnosed with or suspected of having intestinal malrotation should be referred to a pediatric surgeon who should look immediately for any signs of obstruction or sepsis related to the condition. It is not clear, however, whether intestinal malrotation should be treated when the condition is discovered inadvertently and no symptoms are present. Some surgeons advise that the abnormality be surgically corrected only in patients younger than two years of age. Others believe that treatment should be more aggressive and that it should be corrected whenever it is discovered to minimize the chance of an emergent situation later. (5)

ANATOMY AND PHYSIOLOGY

During embryonic development, the colon and small bowel grow very rapidly. The bowel starts out as a straight tube from the end of the stomach to the rectum. (6) As the intestines develop further, they move into the umbilical cord for a short time where they receive nutrients. Between the seventh and 10th week of gestation, the bowel begins to gravitate back toward the abdominal cavity, during which time the intestines undergo a two-part rotation before assuming their normal position in the abdominal cavity.

In the first phase of rotation, the duodenojejunal junction passes behind the superior mesenteric artery and becomes attached to the upper left retroperitoneum. In the second phase, the cecum passes from the left side of the abdomen, anterior to the superior mesenteric artery, and assumes its normal position right of the midline. At completion of the rotation, the mesentery becomes attached to the retroperitoneum by a broad band from the upper left at the duodenojejunal junction (ie, the ligament of Trietz) to the lower right abdomen at the ileocecal junction, which prevents the intestines from twisting on themselves. (4) This process usually is complete by the 12th week of gestation.

ABNORMAL BOWEL DEVELOPMENT

Intestinal malrotation occurs when the two-part process does not proceed normally and the intestines do not make complete turns on re-entry into the abdominal cavity from the umbilicus. This abnormal rotation can have variable results. The cecum and the attached appendix may be positioned in the right upper quadrant, midline, or to the left of midline. With malrotation, the intestines are not secured to the abdominal cavity by the mesentery; instead, the intestines are suspended on a narrow stem of tissue (ie, mesenteric stalk) containing the supplying blood vessels (Figure 1).

[FIGURE 1 OMITTED]

Lack of fixation and a narrow, mesenteric stalk allow the intestines to twist on themselves, a condition known as volvulus. A volvulus cuts off the intestinal blood supply (ie, from branches of the superior mesenteric artery) causing vascular compromise that ultimately leads to catastrophic bowel infarction (ie, a massive loss of bowel as a result of the lack of blood supply) that could result in death. When a volvulus Involves the entire small bowel, it is referred to as a mid-gut volvulus (Figure 2). This can result in the loss of most of the intestine and, in some cases, may result in death. (4)

[FIGURE 2 OMITTED]

Additionally, bands that normally fix the cecum to the sidewall may be abnormally situated within the abdominal cavity in such a position that they compress underlying bowel, causing partial or complete obstruction. These abnormally positioned bands, referred to as Ladd's bands, are named after William Ladd, MD, a pioneer in pediatric surgery. During a Ladd's procedure, the mesentery is widened, the bands are ligated, and the appendix is removed to prevent future confusion because the cecum will be on the left side of the abdomen after the procedure. Obstructions caused by a volvulus or Ladd's bands are life threatening and indicate the need for an emergency surgical procedure. (6)