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2.1 The Ladd’s Procedure for Correction of Intestinal Malrotation With Volvulus in Children indicates that continuing education contact hours are available for this activity. Earn 2.1 continuing education contact hours by reading this article and taking the examination on pages 309310 and then completing the answer sheet and learner evaluation on pages 311-312. You also may access this article online at http://www.aorn journal.org. Renee Ingoe, RN; Patricia Lange, MD I ntestinal malrotation with volvulus is an emergent and possibly lifethreatening 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 ABSTRACT • MALROTATION that occurs when the embryonic, two-part, intestinal rotation does not proceed normally results in an abnormal anatomical position of the intestines. • VOLVULUS OCCURS when the intestines are not fixated to the intestinal wall but are suspended on a narrow stalk of mesenteric tissue containing the supplying blood vessels. This formation allows the intestines to twist on themselves and cut off the blood supply to the bowel. • WHEN MALROTATION WITH VOLVULUS is diagnosed, emergency surgery must be performed to untwist the intestines, restore blood flow, and widen the mesentery. AORN J 85 (February 2007) 300-312. © AORN, Inc, 2007. 300 • AORN JOURNAL • FEBRUARY 2007, VOL 85, NO 2 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 © AORN, Inc, 2007 Ingoe — Lange ANATOMY FEBRUARY 2007, VOL 85, NO 2 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). 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 reThe intestines sult in the loss of most of the intestine and, in gravitate from some cases, may result in death.4 Additionally, bands the umbilical cord that normally fix the ceback to the cum to the sidewall may be abnormally situated abdominal cavity within the abdominal cavity in such a position and undergo a that they compress underlying bowel, causing two-part rotation partial or complete obstruction. These abnorbefore assuming mally positioned bands, referred to as Ladd’s their normal bands, are named after William Ladd, MD, a piposition in the oneer in pediatric surgery. During a Ladd’s abdomen. 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 SIGNS AND SYMPTOMS The presentation of intestinal malrotation can be extremely varied, from lifethreatening sepsis as a result of bowel AORN JOURNAL • 301 Ingoe — Lange FEBRUARY 2007, VOL 85, NO 2 Figure 1 • Part of the small and large intestine are unattached so they can twist and cut off blood supply, which will kill that part of the intestines. failure to pass flatus or stool. The child may become very dehydrated, which may manifest as decreased urination; dry mouth, lethargy; and possibly a depressed fontanel. When the intestines are compromised because of lack of blood flow, the infant’s vomit can become bloody.5 Rectal bleeding is an ominous sign of bowel compromise and may indicate that the infant has a gangrenous bowel. A person can live with intestinal malrotation for life, however, and never experience any symptoms or present with signs of malrotation. In fact, malrotation in an adult usually is an incidental sign on a computed tomography (CT) scan diagnosed by the anatomic location of a right-sided small bowel, left-sided colon, and an abnormal relationship of the superior mesenteric artery.7 This malrotation would have occurred in utero but not have been found until much later in life. Intestinal malrotation in an adult also may be discovered during abdominal surgery that is being performed for another reason (eg, cholecystectomy, trauma). DIAGNOSIS Figure 2 • In a midgut volvulus, the colon is tightly coiled around the base of the mesentery of the small intestine. necrosis to no symptoms at all. Bilious vomiting is the most common sign of intestinal malrotation with volvulus and should be considered a volvulus in infants until proven otherwise. Abdominal pain and lethargy also are common. Other symptoms may include sudden bouts of crying and drawing up of the legs because of abdominal cramps; rapid heart rate; rapid breathing; and 302 • AORN JOURNAL AND FINDINGS The most common and accurate way to diagnose intestinal malrotation with or without volvulus is an upper gastrointestinal (UGI) series. A barium enema (BE) also may be performed but yields less information. Both diagnostic procedures are quick and relatively safe, and they identify malrotation by demonstrating an abnormally positioned intestine. A UGI series is valuable in demonstrating the position of the ligament of Treitz, a band of tissue that anchors the last part of the duodenum to the retroperitoneum. This portion of bowel should be positioned to the left of midline and approximately at the level of the gastroduodenal junction. Any abnormal positioning of this portion of intestine should be considered diagnostic or at Ingoe — Lange least suspicious for intestinal malrotation. A volvulus may be seen as a thin line of contrast in a portion of intestine that is abnormally positioned. Either finding should generate an immediate referral to a pediatric surgeon. Plain x-rays, ultrasounds, and CT scans are not as reliable as a UGI series because the UGI uses contrast media that lights up on the scan. The contrast either stops flowing or only moves through a thin opening. A contrast BE may be performed for evaluation of cecal position if the cause of the obstruction still cannot be determined after a UGI series is performed.4 A BE is more reliable than x-rays, ultrasounds, and CT scans but is not as reliable as a UGI series because the barium only flows up a certain distance, whereas the contrast in a UGI follows the intestines all the way down. The area above the obstruction would not be visualized during a BE. PREOPERATIVE PREPARATION When the OR is being prepared for a pediatric procedure, the circulating nurse turns up the room temperature to between 75° F and 85º F (23.9º C and 46º C) depending on the surgeon’s preference. The circulating nurse places a pediatric, underbody, forced-air, temperatureregulating blanket on the bed8 and obtains an IV-solution warmer and irrigation fluid warmer. The circulating nurse ensures that the pediatric emergency anesthesia cart is in the room. PREOPERATIVE CARE OF THE PATIENT. After the infant has been admitted to the preoperative holding unit, the circulating nurse greets the patient and his or her parents and confirms the patient’s identity using at least two identifiers (eg, the identification band, medical record, parent’s confirmation). After reviewing the patient’s medical record, the nurse ensures that the patient’s laboratory, radiology, and gastrointestinal test results are on the FEBRUARY 2007, VOL 85, NO 2 chart and verifies that the surgical consent is accurate according to the patient’s records, OR schedule, and parents. The nurse verifies the patient’s allergies and NPO status with the parents and medical record. The nurse then performs an assessment that includes the patient’s vital signs, nutritional status, skin turgor, and skin pallor. The nurse then develops a care plan specific for this patient undergoing the Ladd’s procedure (Table 1). CARING FOR THE PARENTS BEThe circulating FORE SURGERY. This surgery usually is performed on an nurse can take emergency basis, so the infant’s parents may be emonumerous actions tionally distraught. The circulating nurse can take to help alleviate numerous actions to help alleviate some of the parthe parents’ fear, ents’ fear. If possible, the circulating nurse should such as speaking sit down with the parents and introduce himself or in a reassuring herself. The nurse should explain exactly what the voice and role of the circulating nurse is in taking care of explaining the their child and explain the role of each perioperative role of the team member, including ancillary personnel. circulating nurse The nurse should speak in a soft, calm, and reassurin taking care of ing tone and should not speak rapidly. He or she their child. should give the parents time to ask questions and to contemplate what he or she is telling them. To instill trust, the nurse should make eye contact with each parent and use touch when appropriate. The nurse should ensure that all of the parents’ questions have been answered and that they understand the procedure completely. The nurse should reassure the parents that he or she will call the waiting room periodically to give AORN JOURNAL • 303 Ingoe — Lange FEBRUARY 2007, VOL 85, NO 2 TABLE 1 Nursing Care Plan for Pediatric Patients Undergoing the Ladd’s Procedure Diagnosis Risk for compromised family coping related to the stress of surgery • • • • • • Risk of hypothermia related to perioperative environment, patient age, and exposed body surfaces • • • Risk for infection related to immature immune system and poor nutritional status Interim outcome criteria Outcome statement Identifies communication barriers and knowledge level. Assesses readiness to learn. Elicits family members’ perception of anesthesia and surgery. Uses appropriate communication skills to ease fears and improve comprehension of patient and family members, such as • sitting with family member when performing patient education, • making eye contact with each parent, • speaking slowly and clearly and observing for indications of confusion versus comprehension, and • allowing time for questions and answers. Ensures parental presence during anesthesia induction, if desired and permitted by facility policy. Evaluates family members’ response to instruction. Patient’s family members • verbalize understanding of the procedure, sequence of events, and expected outcomes and • demonstrate the ability to cope throughout the perioperative period. Patient’s family members demonstrate knowledge of the physiological and psychological responses to the procedure. Monitors body temperature throughout the perioperative period. Implements thermoregulation measures, including • heating the OR to between 75° F and 85º F (23.9º C and 46º C); • placing a pediatric, underbody, forcedair, temperature-regulating blanket on the OR bed under the patient; • placing a stocking cap on the infant’s head; • using IV and irrigation solution warmers according to manufacturer instructions; and • wrapping the infant’s extremities with cotton cast padding to help minimize skin exposure as much as possible. Evaluates response to thermoregulation measures. Patient’s core body temperature remains in expected range throughout the perioperative experience. Patient is at or returning to normothermia at the conclusion of the immediate postoperative period. Assesses skin integrity, sensory impairments, and gastrointestinal status. Observes sterile field and perioperative team members to ensure that asepsis is maintained. Validates that preoperative antibiotic was administered according to facility policy. Allows sufficient time for surgical prep solution to dry before the patient is draped. Patient’s wound is dry, nonreddened, and non-tender. Patient is free of signs and symptoms of infection through the 30 days after the perioperative procedure. Nursing interventions • • • • 304 • AORN JOURNAL Parents and patient demonstrate appropriate bonding. Patient does not demonstrate symptoms of infection (eg, wound induration, foul odor, purulent drainage, fever) Ingoe — Lange them updates on how the procedure is progressing. INTRAOPERATIVE NURSING CARE The circulating nurse and anesthesia care provider transport the patient to the OR. The circulating nurse ensures that all surgical team members are present and then initiates the surgical time out. INTRAOPERATIVE PATIENT WARMING. Infants and children—particularly those who are younger than one month old as is the case for many patients with intestinal malrotation—can lose body heat very rapidly and will lose even more body heat when the abdomen is opened. Infants • have a large body surface area in comparison to their size, • have only a thin layer of subcutaneous fat, and • are unable to shiver. All of these factors contribute to the possibility of rapid hypothermia for infants during surgery. Before transporting the patient from the preoperative area, the circulating nurse ensures that the patient is wearing a hospital stocking cap to prevent heat loss from the head. As soon as the patient is placed on the OR bed, the circulating nurse and anesthesia care provider institute active warming techniques. The anesthesia care provider implements temperature monitoring that he or she will maintain throughout the surgical procedure; a nasopharyngeal temperature probe is a reliable method to approximate the infant’s core temperature. The circulating nurse ensures that the pediatric, underbody, forced-air, temperatureregulating blanket is turned on.8 The circulating nurse remains with the patient throughout induction of anesthesia and offers assistance to the anesthesia care provider as necessary. The anesthesia care provider inserts peripheral IV lines and uses a solution warmer according to the manufactur- FEBRUARY 2007, VOL 85, NO 2 er’s instructions to warm the IV solutions. When the peripheral IV lines have been inserted, the circulating nurse wraps the patient’s extremities with cotton cast padding, making sure it is not too tight. The scrub person uses irrigation fluids warmed by an irrigation warmer to approximately 98.6º F (37º C). The irrigation warmer keeps the fluids warm throughout the procedure and prevents the fluids from getting too hot, which can occur if irrigation fluids are taken directly out of a fluidInfants are warming unit. ESSENTIALS OF POSITIONING. vulnerable to The circulating nurse, anesthesia care provider, hypothermia and surgeon place the patient in the supine posiduring surgery tion. The circulating nurse places rolled-up sheets lengthwise alongside the because they have patient according to sura large body geon preference to keep the patient from shifting to surface area in the side. The surgeon may place a rolled towel under comparison to the patient’s buttocks to gently lift the pelvis into their size, have a modified Trendelenburg position to displace abonly a thin layer dominal contents upward. The circulating nurse or of subcutaneous surgeon may place plastic adhesive drapes around fat, and are the abdomen before the surgical skin prep is perunable to shiver. formed to keep the patient warm and dry and to prevent pooling of fluids during the prep and the procedure. THE SURGICAL SKIN PREP. Patients of this age have immature immune responses. Furthermore, they may have poor nutritional status as a result of their condition, so they are less capable of fighting infection. It is of great importance, therefore, to ensure that all team AORN JOURNAL • 305 Ingoe — Lange FEBRUARY 2007, VOL 85, NO 2 Figure 3 • The surgeon excises Ladd’s bands so that the mesentery can be released, allowing the intestines to relax unrestrained. members strictly adhere to aseptic technique. The circulating nurse performs a surgical skin prep of the patient’s entire abdomen from nipple line to pubis with a solution of the surgeon’s preference. The nurse ensures that the prep solution has enough time to dry sufficiently and that no solution pools under the patient. THE PROCEDURE After the surgeon makes a midline laparotomy incision to ensure adequate visualization of the intestines and related structures, he or she takes the intestines out of the abdominal cavity and untwists them if a twist is present. The surgeon then removes any Ladd’s bands overlying the cecum or duodenum (Figure 3) with a goal of spreading out the mesentery, separating the small and large intestines as much as possible. Eventually, the surgeon positions the small bowel primarily to the right of the patient’s midline and positions the large intestine on the left. Although these bowel positions are the opposite of where they normally would lie if the complete turn had oc- 306 • AORN JOURNAL curred during fetal development, the bowel will remain in this backwards position for the rest of the patient’s life. The cecum and appendix, therefore, are positioned on the left, and appendicitis could very easily be misdiagnosed later in life. To prevent this kind of misdiagnosis from occurring, the surgeon also will perform an appendectomy.4 When a volvulus is present, the surgeon’s first step is to immediately untwist the intestine by rotating the bowel counterclockwise. After the surgeon has reduced the volvulus, the intestines usually are edematous and congested, and some areas may even appear necrotic.4 The surgeon observes the bowel closely for several minutes to verify viability of the bowel. If the bowel does not regain some normality before the end of the procedure (ie, color change to indicate improved blood flow), the surgeon will resect the necrotic portions. If the surgeon has doubts as to the viability of some areas, the patient will be brought back to surgery within 24 to 36 hours for reevaluation. When the surgery is underway, the circulating nurse calls the waiting room to tell the parents that the procedure has started. The circulating nurse then calls periodically during the procedure to update the parents on the progress of surgery. POSTOPERATIVE NURSING CARE Where the patient is cared for and recovers after surgery depends on the surgical findings. Patients with uncomplicated malrotation with or without volvulus are managed on the pediatric surgical unit. Patients with severe volvulus or those needing extensive bowel resection often are acutely ill with shock or sepsis after the procedure, and require monitoring in the pediatric intensive care unit (PICU). The circulating nurse communicates closely with the anesthesia care provider and surgeon to plan for the Ingoe — Lange postoperative needs of the patient. The circulating nurse calls the pediatric surgical unit nurse or PICU nurse during wound closure to communicate these needs. This gives the receiving nurse time to prepare the room for the patient. The circulating nurse’s report includes • the type and length of procedure that was performed, • the amount and type of narcotics administered during the procedure, • the antibiotics received before and during the procedure, • the total amount of IV fluids administered intraoperatively, • the blood or blood products administered during surgery, • whether the patient will be extubated, and • the patient’s temperature during the procedure and a current end-ofprocedure temperature. More information may be provided depending on specific unit policies and patient circumstances. The nurse also ensures that extra support staff members are available to assist with transporting the patient. ENSURING POSTOPERATIVE NORMOTHERMIA. A child’s body temperature can drop very quickly, particularly during extubation or transport to the postoperative unit. The nurse ensures that the patient is kept warm during this time. If the child will be transported in a crib, the circulating nurse lines the bed of the crib with warm blankets immediately before placing the patient in the crib and keeps the patient securely wrapped in a warm blanket, papoosestyle, as much as possible. The circulating nurse also places a rolled blanket behind the patient’s back to keep the patient on his or her side to prevent aspiration if vomiting occurs. AVOIDING POSTOPERATIVE HYPOXIA. The circulating nurse ensures that a full oxygen tank is on the transport vehicle (ie, crib, FEBRUARY 2007, VOL 85, NO 2 bed) and easily accessible. After moving the patient from the OR bed to the transport vehicle, the circulating nurse attaches one end of the oxygen tubing to the tank and holds or tapes the other end close to the patient’s mouth and nose so that the oxygen is blowing by the patient. This is much like using a nasal cannula for an older patient. This method of administering supplemental oxygen is continued until the patient’s oxygen saturation level conSupplemental sistently remains within normal limits. oxygen is If the anesthesia care provider does not extuadministered bate the patient, the circulating nurse ensures until the the availability of a selfinflating, bag-valve-mask patient’s oxygen resuscitator for transport. The circulating nurse obsaturation level tains a portable cardiac monitor to monitor the consistently patient’s condition during transport to the postremains within operative recovery unit. PROVIDING POSTOPERATIVE normal limits. NUTRITION. Infants who undergo extensive intestinal resections often are not able to tolerate enteral nutrition for quite some time. If that is the situation, total parenteral nutrition (TPN) will be started and continued long term. Children on long-term TPN will be monitored for chronic liver damage, a risk of long-term TPN.2 PROGNOSIS If intestinal malrotation with volvulus is recognized and treated quickly, the patient should recover well and not have any long-lasting effects, providing there is enough bowel left to sustain life. Children who have undergone this procedure most often progress through life with little or no AORN JOURNAL • 307 Ingoe — Lange FEBRUARY 2007, VOL 85, NO 2 additional consequences. Very rarely, volvulus can recur after surgical correction. Small bowel transplantation sometimes is indicated in children who have lost a significant amount of intestine. Individuals with intestinal malrotation but without associated volvulus may remain asymptomatic, and the condition may go undetected throughout life. Although some surgeons believe it should not be treated when discovered after infancy if the patient is asymptomatic, other surgeons believe it always should be treated surgically even if asymptomatic because of the lifetime risk of volvulus.5 Some surgeons now are performing the Ladd’s procedure laparoscopically if there is no evidence of volvulus. This approach is especially useful if a diagnosis of intestinal malrotation is in question. Identifying structures appropriately and obtaining adequate separation of the mesentery, however, can be technically difficult during a laparoscopic approach. In this case, the surgeon may need to abandon the laparoscopic approach midprocedure and convert to an open procedure. This is especially true for younger, smaller patients. ❖ Renee Ingoe, RN, CNOR, is a perioperative staff nurse at Wake Med Health and Hospitals, Raleigh, NC. Patricia Lange, MD, is an assistant professor of pediatric surgery at the University of North Carolina at Chapel Hill, Chapel Hill, NC. REFERENCES 1. Parish A, Hatley R. Intestinal malrotation. Available at: http://www.emedicine.com/ped /topic1200.htm. Accessed December 7, 2006. 2. Kamal, IM. Defusing the intra-abdominal ticking bomb: intestinal malrotation in children. CMAJ [online serial]. 2000;162. Available at: http://www.cmaj.ca/cgi/content /full/162/9/1315. Accessed December 7, 2006. 3. Cincinnati Children’s Hospital Medical Center. Intestinal malrotation. February 2004. Available at: http://www.cincinnati childrens.org/health/info/abdomen/diag nose/intestinal-malrotation.htm. Accessed December 19, 2006. 4. Ford EG, Senac MO Jr, Srikanth MS, Weitzman, JJ. Malrotation of the intestine in children. Annuals of Surgery. 1992;215:172-178. 5. The American Pediatric Surgical Association. Malrotation and volvulus. Available at: http://www.eapsa.org/parents/rota tional.htm. Accessed December 7, 2006. 6. KidsHealth. Intestinal malrotation. Available at: http://kidshealth.org/parent/med ical/digestive/malrotation.html. Accessed December 7, 2006. 7. MedPix. Incidental malrotation and acute appendicitis. Available at: http://rad.usuhs .mil/medpix/medpix.html?mode=+search2. Accessed December 7, 2006. 8. Bair Hugger® temperature management: model 555—pediatric underbody blanket. Arizant Healthcare. Available at: http:// www.bairhugger.com/arizanthealthcare /faw_b_pediatric_555.shtml. Accessed January 3, 2006. Foundation Offers Ways to Support Perioperative Nursing B y supporting the AORN Foundation, you also support the advancement of perioperative nursing. There are many ways to extend your personal legacy to the AORN Foundation, including • naming the AORN Foundation in your last will and testament; • allocating gift annuities; • committing charitable remainder/annuity trusts; or • naming the AORN Foundation as a beneficiary 308 • AORN JOURNAL in a life insurance policy. You can double or triple your donation to the Foundation if your employer matches your donation. To find out if your employer will match your gift to the AORN Foundation, please check with your human resources department. For more information on Foundation activities, call Nancy Harbin in the AORN Foundation office at (800) 755-2676 x 366, or visit http://www.aorn.org/foundation.