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Treatment of Cholelithiasis in Children with Sickle Cell Disease
JUNE 2003, VOL 77, NO 6
Home Study Program TREATMENT OF CHOLELCCnlASlS IN CHILDREN WITH SICKLE CELL DISEASE
he article “Treatment of cholelithiasis in children with sickle cell disease,” is the basis for this AORN Journal independent study. The behavioral objectives and examination for this program were prepared by Rebecca Holm, RN, MSN, CNOR, clinical editor, with consultation from Susan Bakewell, RN, MS, education program professional, Center for Perioperative Education. A minimum score of 70% on the multiple-choice examination is necessary to earn 2.3 contact hours for this independent study. Participants receive feedback on incorrect answers. Each applicant who successhlly completes this study will receive a certificate of completion. The deadline for submitting this study is June 30,2006. Send the completed application form, multiple-choice examination, learner evaluation, and appropriate fee to AORN Customer Service c/o Home Study Program 2170 S Parker Rd, Suite 300 Denver, CO 8023 1-571 1 or fax the information with a credit card number to (303) 750-3212.
BEHAVIORAL OBJECTIVES
After reading and studying the article on treatment of cholelithiasis in children with sickle cell disease (SCD), the nurse will be able to explain how a child with SCD may suffer from cholelithiasis, describe the gallbladder according to SCD, identify treatment options for cholelithiasis, define developmental theory in regard to prominent child psychologists, explain the nursing care provided during the preoperative phase of a cholecystectomy procedure performed on a child with SCD, discuss the intraoperative phase of the procedure, and elaborate on the postoperative experience of a child with SCD undergoing cholecystectomy. This program meets criteria for CNOR and CRNFA recertification, as well as other continuing education requirements.
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Treatment of Cholelithiasis in Children with Sickle Cell Disease Cholecystectomy (ie, removal of the gallbladder) is the standard surgical intervention for the diagnosis of cholelithiasis in patients without a chronic illness. In contrast, cholecystectomy has been considered a controversial surgical intervention for children with SCD because these children usually display asymptomatic cholelithiasis. Currently, most physicians advocate elective cholecystectomy for children with SCD who display asymptomatic cholelithiasis to prevent symptoms of acute cholecystitisand the risk of requiring emergency cholecystectomythat may place them at risk of complications.’ Typically, the indication for undergoing a cholecystectomy is the presence of symptomatic gallstones in patients, regardless of age. Prophylactic cholecystectomy also is appropriate for asymptomatic individuals who have a chronic illness (eg, SCD, diabetes) and those who have an increased risk of developing symptomatic gallstones? Cholelithiasis typically is observed less frequently in healthy children who do not have a chronic illness. Gallstones are observed with greater frequency among children with SCD. Age and chronic hemolysis are identified as the major factors that increase the risk of formation of black pigment type gallstones in children with SCD.9Additionally, development of pigment gallstones in children with SCD appears to be aee deDendent and occurs in A B S T R A C T Medical and surgical advances have Improved the treatment of approximately 15% of children cholelithiasis (ie, gallstones) In children with sickle cell disease younger than 10 years of agei0and (SCD). Children with SCD have an increased risk of developing pig- 50% of children by 20 years of ment gallstones that initially may be asymptomatic but that can lead age.” The incidence of gallstones to acute symptoms of cholellthlasls. An elective laparoscoplc chole- varies with age across the life span cystectomy procedure Is recommended for pediatric patlents with for patients with SCD. For SCD to prevent the risk of requiring an emergency cholecystectomy instance, the incidence is 12% in the two- to fow-yearprocedure. The primary benefits of this approach Include a shorter old age group,‘* hospital stay after surgery, decreased postoperative discomfort, 9% in the five- to seven-yeardecreased risk of complications, and a quicker return to normal activold age group,” ities. AORN J 77 (June 2003) 1 170-1182.
ickle cell disease (SCD) is one of the most common genetic disorders in the United States.’ Primary symptoms of this chronic disease include chronic hemolytic anemia and tissue damage secondary to vaso-occlusion by sickle-shaped red blood cells (Figure 1). Patients with SCD have an increased risk of developing cholelithiasis (ie, gallstones)? Cholelithiasis should be considered as a potential diagnosis when a child with SCD experiences recurrent abdominal pain? Evaluation criteria used to c o n f m the diagnosis of cholelithiasis include radiological evaluation using biliary scintigram and ultrasound and laboratory results that indicate elevations in serum bilirubin, alkaline phosphatase, hepatic enzyme levels, and white blood cell An estimated 50% of patients in sickle cell crisis (ie, pain crisis) experience acute abdominal pain, which often is difficult to differentiate from acute chole~ystitis.~ One group of researchers noted that acute abdominal pain symptoms in children with SCD often were diagnosed as vaso-occlusive crisis rather than biliary colic associated with cholelithiasis.“
Y
VERNA HENDRICKS-FERGUSON, RN; MARTHA A. NELSON, RN
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43% in the 15- to 18-year-old age group,l4 83% in people older than 30 years of age,I5 and 60% to 85% in people age 33 or older.16 GALLBLADDER ANATOMY
The gallbladder’s shape has been compared to a pear (Figure 2). It is approximately 7 cm long and holds 30 mL to 50 mL of bile.” Branches of the cystic artery originating from the right hepatic artery provide the gallbladder’s blood supply. The common and right hepatic arteries provide the common bile duct with its blood supply. During cholecystectomy, the triangle of Calot serves as a surgical landmark to identify important structures.ls These structures include the cystic duct, the common hepatic duct, and the inferior border of the liver. The common hepatic duct, gallbladder, and common bile duct finction as a storage site for bile and a conduit for bile flow fiom the liver to the duodenum. PIGMENT GALLSTONES
Children with chronic hemolytic disorders generally have an increased risk Of pigment gallstone^.'^ Pigment gallstones have been observed in 15% of children with SCD who are younger than 10 years of ageZoand 50% of children with SCD by 20 years of age. The frequency of pigment gallstones is higher in this population because of a sustained increase in heme catabolism among children with SCD.” Approximately 10% to 20% of gallstones are referred to as pigment stones. These are composed of poorly soluble calcium salts, especially calcium bilirubin, and are dark in color. Pigment stones are categorized as black, brown, or mixed pigment gallstones. Black pigment gallstones are small, hard, and spiculated (ie, sharp body with needle-like points) and are made of calcium bilirubinate, inorganic salts (eg, calcium phosphate), and calcium carbonate.= Black pigment stones typically develop in patients who have a history of excessive secretion of unconjugated bilirubin into bile?’ Black pigment stones are observed in approximately 14% of children with SCD who are younger than 10 years of age. The percentage increases to 36% among school-age children and adolescents (ie. 10 to 20 years of age) and to 50% by age Z2‘ Brown pigment stones are soft, crumbly aggregates that often form in the bile ducts of patients with a history of chronically infected bile above biliary strictures or cholesterol cysts. They are made of calcium bilirubinate and calcium ~alrnitate.~~ Mixed
Figure 1 illustration of normal red blood cells in relation to sickled cells.
Figure 2 Normal anatomy of the gallbladder and related Structures.
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operations stage of cognitive development.” Characteristicsof this stage include interest in acquiring more detailed knowledge, improved concept of time, and increased self-control. Psychoanalyst Erik TREATMENT OF CHOLELlTHlASlS Erikson characterized children ages six to 12 as being The current recommended treatment for children in the industry versus inferiority stage of psychosoCharacteristics of this stage with SCD who are diagnosed with cholelithiasis is cia1 de~elopment.~~ elective laparoscopic cholecystectomy.27An essential include interest in participating in various school-related activities, component of the preoperative preparation for elective cholecystectomy among children with SCD is such as development of peer relationships; attaining skills in competition and cooperation; erythrocyte transfusion therapy. The purpose of this therapy is to reduce the percentage of hemoglobin S and developing a sense of competence. and increase the hemoglobin concentrationto greater than 10 g per dL. This reduces the risk of complicaSchool-age children may experience anxiety tions associated with vaso-occIusion.28 before an unfamiliar or familiar medical procedure Risks associated with erythrocyte transfusion because of their increased cognitiveability. They may therapy among patients with SCD may include an have many questions related to medical procedures increased risk of exposure to infectious agents and that require health care providers to give age-approallosensitizationto erythrocyte antigens. Undertaking priate explanations using correct scientific and medthese risks may be reasonable because of the ical terminology before all procedures. Explanation decreased risk of postoperative morbidity with elec- of these procedures can be enhanced by the use of tive laparoscopic cholecystectomy compared to the simple anatomy and physiology diagrams and concrete terms explaining why and how equipment knctraditional cholecystectomyprocedure. The primary goal of elective cholecystectomyis tions. According to developmental theory, school-age to prevent complications associated with emergency children are focused on gaining a sense of control and surgical intervention. Potential complications after competence during various activities; therefore, emergency cholecystectomy in this patient population health care providers should strive to keep children are associated with general anesthesia and the inabili- informed of what to expect before all medical procety to implement recommended preoperative interven- dures. Allowing time before procedures for patient tions, such as erythrocyte transfusion therapy? In questions and additional explanations is recommendaddition, children with SCD who undergo emergency ed. Health care providers also should educate schoolcholecystectomy are at high risk of developing age children about self-control strategies, such as intraoperative complications, such as vaso-occlu- helpful postoperative strategies (eg, deep breathing sion secondary to transient hypoxia, hypothermia, exercises, relaxation techniques, counting during dressing changes), before scheduled surgical procedehydration, or acidosis; postoperative complications, such as pneumonia, dures to prevent postoperative complications and acute chest syndrome, or painful vaso-occlusive minimize feelings of anxiety. events; and other complications,such as ascending cholangitis PREOPERATIVE PHASE (ie, inflammatory disease of the bile ducts), choleIn the preoperative holding area, the circulating docholithiasis (ie, gallstones in the common bile nurse and anesthesia care provider complete all duct), or acute cholecystitis (ie, gallstones or required preoperative assessments for a pediatric sludge obstructingthe cystic duct).’O patient before transferring the patient to the OR. Both participate in preoperative teaching for the parents to DEVELOPMENTALTHEORY inform them about what their child will experience. FOR SCHOOL-AGE PATIENTS The circulatingnurse confirms the child’s identity and Knowledge of the specific developmental char- the scheduled laparoscopic cholecystectomy proceacteristics of school-age children is important for dure with the child’s parents. The circulating nurse nurses and other pediatric surgical team members. also verifies that all surgical informed consents are Child psychologist Jean Piaget, PhD, characterized present and complete. In addition, the nurse docuchildren ages seven to 11 as being in the concrete ments the patient’s allergies, NPO status, presences of gallstones are made of both cholesterol and calcium bilirubinate, and bacterial colonization or inflammation of the gallbladder may develop later.26
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prostheses, loose teeth, rashes, bruises, and any other pertinent information that facilitates safe patient care. The perioperative nurse ensures that the following documents are present in the patient’s chart: medical history, physical examination, preoperative records, and appropriate laboratory test results. He or she then performs an assessment and documents the patient’s physical condition, including vital signs, height, weight, and skin integrity. The nurse also can review age-appropriate (ie, concrete operational thinking) concepts with school-age patients, such as information related to what the patient can expect during the surgical process (eg, approximate time in surgery, recovery, return to the unit), regarding the location of the surgical incision, and regarding the appearance of postoperative surgical dressings. The nurse should review the rationale and importance of implementing postoperative strategies, such as deep breathing exercises with the child. The perioperative nurse offers the child the opportunity to select a scented oxygen mask (eg, cherry, grape, strawberry) for use during anesthesia induction in the OR. Encouraging the patient’s participation in selecting a scented mask and providing concrete information related to the surgical process and postoperative strategies are examples of appropriate developmental strategies nurses can implement to aid in meeting the developmental needs of schoolage patients (eg, gaining self-control, developing self-competence). The perioperative nurse then develops a care plan for the pediatric patient with SCD undergoing elective laparoscopic cholecystectomy (Table 1). INTRAOPERATWE PHASE
Perioperative team members transport the pediatric patient on a child-size stretcher to the OR suite and place the patient on a locked OR bed. The circulating nurse reassures the patient and provides simple explanations of the intraoperative equipment using concrete terms, such as explaining that the purpose of the OR bed strap is to ensure safety and prevent accidental falls rather than to tie the child down. The circulating nurse incorporates distraction techniques appropriate for the school-age patient (eg, discusses hobbies, school activities, clubs, team sports) to minimize the emotional distress often expe-
rienced by children of this age group. The circulating nurse encourages the parents to accompany their child to the OR until after anesthesia induction to reduce the child’s fears. Anesthesia The circulating nurse assists the anesthesia care provider during induction of general anesthesia and intubation of the child. The nurse either places the patient’s IV line preoperatively in the pediatric unit or in the OR. The anesthesia care provider induces general anesthesia and places the endotracheal tube. After induction of anesthesia, the anesthesia care provider inserts an orogastric tube for decompression. After the child is intubated, a staff member escorts the parents to the parent surgical waiting room where they typically wait until their child’s surgical procedure is complete. Positioning and prepping. The circulating nurse initially positions the pediatric patient in the supine position on the OR bed and positions two video cameras at the head of the OR bed. The nurse evacuates the patient’s urinary bladder via the Credt’s maneuver (ie, massaging the bladder by pressing down on the anterior, superior surface of the abdomen). The circulating nurse cleanses the patient’s abdomen with an antiseptic solution. The pediatric surgeon, with the assistance of the scrub person, drapes the patient’s surgical site. Depending on the patient’s age, a four-cannula technique generally is used for the laparoscopic cholecystectomy procedure. During the initial steps of the procedure, the circulating nurse and anesthesia care provider rotate the patient and OR bed for improved visualization. The patient and bed are rotated into the reverse Trendelenburg’s and left-dependent positions to aid in allowing the adjacent abdominal viscera to fall away from the surgical area. The surgeon performs lateral retraction of the junction of the inhndibulum (ie, h n nel-shaped passage or structure) of the gallbladder and cystic d u ~ t . 3The ~ primary benefit of using lateral retraction is the ability to reposition the cystic duct into a 90% orientation to the common bile duct versus an oblique or even parallel orientation. This minimizes the need for surgical manipulation by retraction.” Risks. Surgery and general anesthesia are potential stressors for all children with SCD. Intraoperative complications may include transient hypoxia; hypothermia; dehydration;
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Table 1 NURSING CARE PLAN FOR PEDlATRlC M T I E N T S Wrm SICKLE C E U DISEASE ( X D ) UNDERGOING ELECTWE -0ScOPlC CHOLECYSlEClO MY
Nursing diagnosis
Interventions
Knowledge deficit regarding SCD and cholelithiasis and undergoing cholecystectomy
Includes family members in preoperative teaching and discharge planning.
Fear of the unknown related to undergoing cholecystectomy
Explains the purpose of intraoperative equipment in age-appropriate terms, including use of a safeiy belt to prevent falls, anesthesia equipment to monitor heart rate, pulse oximetty, and blood pressure. Incorporates distraction techniques appropriate for the school-age patient by discussing interests, such as , hobbies, school activities, clubs, or team sports.
Describes how SCD can cause cholelithiasis. Explains how cholecystectomy iS 0 definitive trC?ahel nt cholelithiasis. Evaluates responses to learning.
for
Q
Q
Interim outcome criteria
Outcome statement
Patient’s fear and anxiety are minimized related to his or her sense of uncertainty of events to occur in the surgical area.
Patient participates in discussions related to the surgical procedure.
Patient does not demonstrate emotional distress oflen experienced by pediatric
Patient demonstrates control over his or her environment appropriate for developmental level.
Patient does not demonstrate nonverbal signs and symptoms of pain throughout the pefioperative period.
Patient demonstrates adequate pain management and verbalizes relief of pain.
men’.
Q
Q Q Q
Encourages the patient to a select scented oxygen mask for use during anesthesia induction in the OR. Risk for acute pain related to surgical intervention and potential for sickle cell crisis
Assesses pain using ageappropriate assessment tools. Identifies cultural and value components related to pain and provides pain management instructions accordingly. hlpkmentS pain guidelines and eVahateS response to interventions.
increased risk of reduced respiratory effort due to postoperative incisional pain, resulting in poor pulmonary toilet and hypoxia symptoms;36and bleeding diathesis. Pediatric patients with SCD can experience bleeding diathesis and a sudden hemoglobin drop during the postoperative period even after an uneventful laparoscopic cholecystectomy procedure.” Erythrocyte transfusion therapy during the preoperative period, therefore, is recommended to increase the percentage of hemoglobin A to more than 65% of the total hemoglobin and a hematocrit of 35% to 45%.’8 Physiological factors that influence the development of complications include
patient immobility, hypothermia as a result of exposure to the cool surgical environment, hypoventilation, and pain. Supportive perioperative interventions for the patient with SCD, therefore, include careful attention to ensuring adequate oxygenation, hydration, and acidbase balance. In addition, patients with SCD receive IV hydration therapy at a rate of one and one-half times the calculated maintenance amounts for 12 hours before elective surgery. Hydration therapy is continued into the postoperative period until the patient displays adequate oral intake, which usually occurs by the third postoperative day.J’ Supportive
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postoperative recommendations include careful attention to assessment of the patient's abdomen, and oxygen saturation and monitoring of the hematocrit and hemoglobin values at six hours after surgery and each morning for two consecutive days." Other recommended precautions are related to the risk of hypoxia and hyp~thermia.~' The anesthesia care provider ensures that the patient receives 100% oxygenation before and after intubation and extubation and monitors the patient's body temperature to avoid hypothermia. The circulating nurse maintains the room temperature at 80" F to 85" F (27" C to 29" C) during the procedure, ensures the patient is positioned properly, and is careful to determine that IV stability devices and dressings have not caused circulatory stasis. LAPAROSCOPIC CHOLECYSTECTOMY PROCEDURE
The surgeon uses electrosurgeryto make a 1-cm umbilical incision and dissect through the abdomen until the fascia1 tissue is entered. He or she then inserts the Veress insumation needle into the abdomen and then places a 10-mm epigastric port. Using a 10-mm laparoscope, the surgeon visually inspects the patient's gallbladder.The anesthesia care provider rotates the bed into reverse Trendelenburg's and left-dependent position to help the adjacent viscera fall away from the surgical area." The surgeon looks for adhesions and other abnormalities. He or she infiltrates the additional port sites with bupivicaine and uses electrosurgery to make incisions for three 5-mm ports at the anterior axillary line in the right costal margin, at the midclavicular line along the right costal margin, and under the epigastrium. The surgeon places trocars and instruments into the 5-mm ports to grasp and elevate the gallbladder and then bluntly takes down any adhesions and grasps the infundibulum. The surgeon uses electrosurgery to remove the peritoneum from the gallbladder and then clear and reverse the triangle of Calot. The surgeon also inspects the cystic duct from its origin down to the gallbladder. He or she M e r divides the patient's peritoneum along the gallbladder to mobilize it before removing the cystic duct. The surgeon places two clips distal to the intended line of division and one on the cystic duct approximately 5 mm from its insertion into the common bile ducts. He or she transects the cystic duct and divides the
cystic artery with electrosurgery before removing the gallbladder. The surgeon creates a small hole in the gallbladder to drain it with suction, which prevents bile leakage into the peritoneal cavity, before removing the gallbladder from the gallbladder bed. He or she pulls the gallbladder out through the umbilical port in an endoscopic specimen bag. The surgeon inspects the gallbladder and irrigates and aspirates the area until it is clear. The surgeon removes all surgical instruments from the patient's abdomen and deflates the patient's abdomen by allowing the carbon dioxide to escape via the ports, after which he or she removes the ports. The surgeon closes the umbilical fascia with interrupted 2-0 braided absorbable polyglactin suture. The surgeon then infiltrates all surgical wounds with bupivicaine and closes the skin with subcuticular stitches of monofilament absorbable poliglecaprone. The surgeon uses tincture of benzoin and self-adhesive wound approximating strips to close the 5-mm port sites and applies antibiotic ointment and a sterile adhesive bandage strip on the patient's umbilicus. POSTOPERATIVE CARE
The circulating nurse and anesthesia care provider transport the patient to the postanesthesia care unit (PACU) after the surgical procedure is completed. The circulating nurse gives report to the PACU nurse. After receiving report, the PACU nurse assesses the patient's vital signs, oxygen saturation level, level of consciousness, circulation, pain, IV site, fluid rate, and hydration status, as well as the status of the surgical site and dressing and all related monitoring equipment. The nurse also places warming blankets on the patient to enhance comfort and preserve the patient's body temperature. DkchargR Pediatric patients with SCD usually are discharged four to six days after elective laparoscopic cholecystectomy.The pediatric surgical nurse provides in-depth discharge teaching to the parents about the child's recommended activity level and restrictions, pain management, and diet. He or she also discusses recommended follow-up management with the physician and the surgeon (Table 2). POTENTIAL COMPLICATIONS
The major benefits of elective laparoscopic cholecystectomy include shorter hospitalization, decreased postoperative discomfort, a shorter interval before returning to normal activities," and increased
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respiratory efforts.” Patients who have undergone this procedure, however, have a slightly increased risk of major bile duct injury (ie, 0.3% versus 0.2% for those who undergo traditional open chole~ystectomy).4~ The primary complication identified is the risk of bile duct injury among adults6 and bleeding diathesjs among children with SCD.4’ It is recommended, therefore, that all children with SCD be monitored closely postoperatively, including having their hemoglobin and hematocrit assessed for 48 hours to 72 hours. Laparoscopic cholecystectomy also carries a small risk of complications for all patients related to required surgical treatments, such as anesthesia,blood transfusion, hemorrhage, and insufflation of the peritoneal cavity with carbon CASE STUW
Sarah, a 10-year-old African-American female with a history of SCD, was admitted to the pediatric unit at St Louis Children’s Hospital with a suspected diagnosis of acute cholecystitis. After undergoing numerous diagnostic examinations, the physician confirmed diagnoses of acute cholecystitis, prolonged prothrombrin time, and multiple pigmented stones in the gallbladder.Hematology-oncologyteam members evaluated Sarah and initiated medical interventions, including pain management and vitamin K administration. Sarah recovered from the acute painhl episode and was scheduled for elective laparoscopic cholecystectomy.
Table 2 DISCHARGE ORDERS
Activities No swimming or submersion of incision in water for 1 week No running or contact sports for 2 weeks Diet
Regular
Pain management Acetaminophen with codeine, 1 tablet every 4 hrs to 6 hrs as needed Wound care Shower 1 time per day and do not submerge incision Allow self-adhesive wound approximating strips to fall off with time
Sarah’s pediatric nurse started an IV in Sarah’s left anticubital fossa and began administering IV fluids (ie, 5% dextrose in 0.45%normal saline with 20 mEq potassium chloride) at 80 mL per hour in preparation for the scheduled surgical procedure. The nurse also administered prophylactic antibiotic therapy (ie, 1 g IV cefoxitin sodium) every eight hours for three doses in preparation for the surgical procedure. The elective laparoscopic cholecystectomy procedure proceeded uneventfully,and although the surgeon identified some adhesions from the omentum to the gallbladder, she detected no other abnormalities except for mild hepatomegaly. The surgeon bluntly dissected all detected adhesions from the omentum on the duodenum into the gallbladder and dissected the gallbladder free. She was unable to locate Sarah’s dominant artery; however, small branches of this artery were identified. While mobilizing and transecting the cystic duct, the surgeon determined that one of the distally placed clips appeared not to have approximated appropriately; therefore, the surgeon placed an endoloop proximal to the clips on the cystic duct stump. She removed the gallbladder without difficulty and closed the incisions. Sarah was transferred via pediatric stretcher to the PACU, and after she recovered for 60 minutes, PACU nurses transferred Sarah to the pediatric surgical unit. Sarah’s pediatric surgical unit nurse completed an admission assessment and then monitored Sarah’s vital signs every four hours until discharge. During the surgical procedure and the postoperative phase, the nurse managed Sarah’s pain with a continuous IV infusion via a computerized patientcontrolled anesthesia (PCA) pump of 1 mg per mL of morphine sulfate (MS) at a rate if 0.5 mg per hour. In the event that Sarah experienced MS-associated side effects, such as pruritus, the nurse had orders to administer 25 mg diphenhydramine hydrochloride IV or by mouth (PO) every six hours as needed. If the pruritus was unresponsive to diphenhydramine, Sarah’s nurse could administer 2 mg of IV nalbuphine every one to two hours as needed. Additionally, the n m e had orders to treat other side effects associated with MS therapy, such as nausea and vomiting, with 4 mg lV ondansetron every six hours or 6 mg IV metaclopramide hydrochloride every six hours as needed. Fortunately, Sarah did not experience side effects typically associated with MS. On the first postoperative day, Sarah was not allowed any oral intake for 24 hours. On her second postoperative day, the nurse slowly advanced Sarah’s
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diet beginning with clear liquids, increasing to a normal diet as tolerated. The nurse assisted Sarah out of bed on the second postoperative day and encouraged her to increase her activity as tolerated, offering assistance as needed. The nurse discontinued Sarah’s PCA, IV fluids, and IV medications during the second day and managed Sarah’s pain with PO oxycodone 5 mg or acetaminophen with codeine every four to six hours as needed. The nurse instructed Sarah and her parents about discharge instructions. He also instructed Sarah’s parents to make Sarah’s follow-up appointment in approximately four to six weeks. Overall, this patient experienced an uneventful recovery period after the surgical procedure. SUMMARY
Elective laparoscopic cholecystectomy in children has been described as a safe and effective alternative to the traditional cholecystectomy surgical procedure because of a shortened hospital stay and improved postoperative recovery.” One of the significant benefits of laparoscopic cholecystectomy for children with SCD is reduced postoperative pain, which improves pulmonary toilet and decreases risk of pneumonia and vaso-occlusive events.5o Other identified postoperative benefits include reduced risk NOTES 1. V L Hendricks-Ferguson, M A
Nelson, “Laparoscopic splenectomy for splenic sequestration crisis,” AORN Journal 7 1 (April 2000) 820834. 2. R E Ware et al, “Diagnosis and
of postoperative ileus, smaller incision, and decreased keloid formation, resulting in an improved body image for young patients with SCD.5’ Current guidelines for children with SCD experiencing symptoms of cholelithiasis recommend elective laparoscopic cholecystectomy to prevent complications such as acute cholecystitis and the risk of emergency surgery for an unprepared patient.s2The cholecystectomy procedure, whether traditional or laparoscopic, does not cure SCD. The elective laparoscopic cholecystectomy procedure, however, offers a planned surgical intervention, reducing the risk of complications associated with emergency cholecystectomy. For school-age patients with SCD, these benefits minimize feelings of fear and anxiety associated with surgery and hospitalization and foster a sense of self-control. A
Vema Hendricks-Ferguson,RN, DNSc, CS, is an associate professor at Jewish Hospital, College of Nursing and Allied Health, Edwarriville, Ill. Martha A. Nelson, RN,BS, CPNR is the pediatric nurse practitioner and sickle eel/ patient coordinator at St Louis Children k Hospital.
laparoscopic cholecystectomy on the management of cholelithiasis in children with sickle cell disease,” Journal of Pediatric Surgery 29 (February 1994) 209-213;A J Jawad et al, “Laparoscopic cholecystectomy for cholelithiasis during infancy and childhood: Cost analysis and review of current indications,” World Journal of Surgery 22 (January
management of common bile duct stones in patients with sickle hemoglobinopathies,”Journal of Pediatric Surgery 27 (May 1992) 572-575. 3. D De Caluwe, U Akl,M Corbally, “Cholecystectomy versus cholecystolithotomy for cholelithiasis in childhood: Long-term outcome,” Journal of Pediatric Surgery 36 (October 2001) 1518-1521. 4. R Rudolph, J S Williams, “Cholecystectomy in patients with sickle cell disease: Experience at a regional hospital in southeast Georgia,” Journal of the National Medical Association 84 (August
“Cholecystectomy in patients with sickle cell disease: Experience at a regional hospital in southeast Georgia,” 692-696. 8. E A Pomfret, J J Pomposelli, R L Jenkins, “Surgical approaches to diseases of the biliary system,” in Schiff k Diseases of the Livel: eighth ed, E R Schiff, M F Sorrell, W C Maddrey, eds (Philadelphia: Lippincott-Raven Publishers, 1999)
1992) 692-696. 5 . Ibid 6. E P Tagge et al, “Impact of
693-717. 9. E A Shaffer, “Gallbladder disease,” in Pediatric Gastrointestinal
1998) 69-74. 7. Rudolph, Williams,
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Disease: Pathophysiology, Diagnosis, Management, third ed, W A
Walker et al, eds (Hamilton, Ontario: B C Decker, 2000) 1291-1311. 10. C F Gholson et al, “Sequential
endoscopicflaparoscopic management of sickle hemoglobinopathyassociated cholelithiasis and suspected choledocholithiasis,” Southern Medical Journal 88 (November 1995) 113 1- 1 135; Jawad et al, “Laparoscopic cholecystectomy for cholelithiasis during infancy and childhood: Cost analysis and review of current indications,” 69-74. 11. G W Holcomb, J B Pietsch, “Gallbladder disease and hepatic infections,” in Pediatric Surgery, vol two, fifth ed,J A O’Neill et al, eds (St Louis: Mosby, 1998) 1495-1511. 12. Rudolph, Williams, “Cholecystectomy in patients with sickle cell disease: Experience at a regional hospital in southeast Georgia,” 692-696. 13. N D Duncan, A H McDonald,
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D I G Mitchell, “Cholecystectomy in Jamaican children with homozygous sickle-cell disease,” Tropical Doctor 30 (October 2000) 214-216. 14. Rudolph, Williams, “Cholecystectomy in patients with sickle cell disease: Experience at a regional hospital in southeast Georgia,” 692-696. 15. Duncan, McDonald, Mitchell, “Cholecystectomy in Jamaican children with homozygous sickle-cell disease,” 2 14-216. 16. Shaffer, “Gallbladder disease,” 1291-1311. 17. D L Nahrwold, “The b i l i q system,” in Textbook of Surgery: The Biological Basb of Modern Surgical Practice, 15th ed, D C Sabiston, H K Lyerly, eds (Philadelphia: W B Saunders Co, 1997) 1117-1125. 18. Ibid. 19. Shaffer, “Gallbladder disease,” 1291-1311. 20. Gholson et al, “Sequential endoscopicflaparoscopic management of sickle hemoglobinopathyassociated cholelithiasisand suspected choledocholithiasis,” 1131-1135. 2 1. Holcomb, Pietsch, “Gallbladder disease and hepatic infections,”1495-1511;TMWalker,IR Hambleton, G R Sejeant, “Gallstones in sickle cell disease: Observations from the Jamaican cohort study,” The Journal of Pediatrics 136 (January 2000) 80-85. 22. Shaffer, “Gallbladder disease,” 1291-1311. 23. D M Heuman, W Y Wassef, Z R Vlahcevic, “Gallstones,” in Schiff 5 Diseases of the Liver: eighth ed, E R Schiff, M F Sorrell,W C Maddrey, eds (Philadelphia: Lippincott-Raven Publishers, 1999)63 1647. 24. Ibid. 25. Ibid. 26. Shaffer, “Gallbladder disease,” 1291-13 11. 27. Heuman, Wassef, Vlahcevic, “Gallstones,” 63 1-647; Jawad et al,
“Laparoscopiccholecystectomyfor cholelithiasisduring infancy and childhood: Cost analysis and review of current indications,” 69-74. 28. Ibid. 29. A H Al-Salem et al, “Laparoscopic versus open cholecystectomy in children,” Pediatric Surgery International 12 (October1997) 587-590; A H Al-Salem, S Qaisruddin, “The significanceof biliary sludge in children with sickle cell disease,” Pediatric Surgery International 13 (January 1998) 14-16; Duncan, McDonald, Mitchell, “Cholecystectomy in Jamaican children with homozygous sickle-cell disease,” 2 14-216; R M Hatley et al, “Laparoscopiccholecystectomy in children with sickle cell disease,” The American Surgeon 61 (February 1995) 169-171; Holcomb, Pietsch, “Gallbladder disease and hepatic infections,” 1495-1511; Tagge et al, “Impact of laparoscopiccholecystectomy on the management of cholelithiasisin children with sickle cell disease,” 209-2 13, 30. R Ware et al, “Laparoscopic cholecystectomyin young patients with sickle hemoglobinopathies,” The Journal of Pediatrics 120 (January 1992) 58-6 1. 3 1. J Piaget, The Theory of Stages of Cognitive Development (New York McGraw-Hill, 1969). 32. E Erikson, Childhood and Socie& second ed (New York: Norton Press, 1963). 33. Nahrwold, ‘The biliary system,” 1117. 34. Holcomb, Pietsch, ‘‘Gallbladder disease and hepatic infections,” 1495-1511. 35. Ware et al, “Laparoscopic cholecystectomy in young patients with sickle hemoglobinopathies,” 58-61. 36. Ibid. 37. Hatley et al, “Laparoscopic cholecystectomyin children with sickle cell disease,” 169-17 1.
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38. Ware et al, “Elective cholecystectomy in children with sickle hemoglobinopathies: Successfid outcome using a preoperativetransfusion regimen,” 17-22. 39. Ibid. 40. Hatley et al, “Laparoscopic cholecystectomyin children with sickle cell disease,” 169-17 1. 4 1. Ware et al, “Laparoscopic cholecystectomy in young patients with sickle hemoglobinopathies,” 58-61. 42. Holcomb, Pietsch, “Gallbladder disease and hepatic infections,” 1495-151 1. 43. G W Holcomb, D 0 Olsen, K W Sharp, “Laparoscopiccholecystectomy in the pediatric patient,” Journal of Pediatric Surgery 26 (October 1991) 1186-1190; E Leandros et al, “Laparoscopic or open cholecystectomyin patients with sickle cell disease: Which approach is superior?“ The European Journal of Surgery 166 (November 2000) 859-861. 44.Hatley et al, “Laparoscopic cholecystectomyin children with sickle cell disease,” 169-17 1. 45. Pomfret, Pomposelli, Jenkins, “Surgical approaches to diseases of the biliary system,” 693-717. 46. Ibid. 47. Hatley et al, “Laparoscopic cholecystectomyin children with sickle cell disease,” 169-17 1. 48. Walker, Hambleton, Sejeant, “Gallstones in sickle cell disease: Observations from the Jamaican Cohort study,” (January 2000) 8085. 49. Hatley et al, “Laparoscopic cholecystectomyin children with sickle cell disease,’’ 169-171. 50. Ibid. 5 1. Ware et al, “Laparoscopic cholecystectomy in young patients with sickle hemoglobinopathies,” 58-61. 52. Ibid.
Home Study Program TREATMENT OF CHOLELCCnlASlS IN CHILDREN WITH SICKLE CELL DISEASE
he article “Treatment of cholelithiasis in children with sickle cell disease,” is the basis for this AORN Journal independent study. The behavioral objectives and examination for this program were prepared by Rebecca Holm, RN, MSN, CNOR, clinical editor, with consultation from Susan Bakewell, RN, MS, education program professional, Center for Perioperative Education. A minimum score of 70% on the multiple-choice examination is necessary to earn 2.3 contact hours for this independent study. Participants receive feedback on incorrect answers. Each applicant who successhlly completes this study will receive a certificate of completion. The deadline for submitting this study is June 30,2006. Send the completed application form, multiple-choice examination, learner evaluation, and appropriate fee to AORN Customer Service c/o Home Study Program 2170 S Parker Rd, Suite 300 Denver, CO 8023 1-571 1 or fax the information with a credit card number to (303) 750-3212.
BEHAVIORAL OBJECTIVES
After reading and studying the article on treatment of cholelithiasis in children with sickle cell disease (SCD), the nurse will be able to explain how a child with SCD may suffer from cholelithiasis, describe the gallbladder according to SCD, identify treatment options for cholelithiasis, define developmental theory in regard to prominent child psychologists, explain the nursing care provided during the preoperative phase of a cholecystectomy procedure performed on a child with SCD, discuss the intraoperative phase of the procedure, and elaborate on the postoperative experience of a child with SCD undergoing cholecystectomy. This program meets criteria for CNOR and CRNFA recertification, as well as other continuing education requirements.
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Treatment of Cholelithiasis in Children with Sickle Cell Disease Cholecystectomy (ie, removal of the gallbladder) is the standard surgical intervention for the diagnosis of cholelithiasis in patients without a chronic illness. In contrast, cholecystectomy has been considered a controversial surgical intervention for children with SCD because these children usually display asymptomatic cholelithiasis. Currently, most physicians advocate elective cholecystectomy for children with SCD who display asymptomatic cholelithiasis to prevent symptoms of acute cholecystitisand the risk of requiring emergency cholecystectomythat may place them at risk of complications.’ Typically, the indication for undergoing a cholecystectomy is the presence of symptomatic gallstones in patients, regardless of age. Prophylactic cholecystectomy also is appropriate for asymptomatic individuals who have a chronic illness (eg, SCD, diabetes) and those who have an increased risk of developing symptomatic gallstones? Cholelithiasis typically is observed less frequently in healthy children who do not have a chronic illness. Gallstones are observed with greater frequency among children with SCD. Age and chronic hemolysis are identified as the major factors that increase the risk of formation of black pigment type gallstones in children with SCD.9Additionally, development of pigment gallstones in children with SCD appears to be aee deDendent and occurs in A B S T R A C T Medical and surgical advances have Improved the treatment of approximately 15% of children cholelithiasis (ie, gallstones) In children with sickle cell disease younger than 10 years of agei0and (SCD). Children with SCD have an increased risk of developing pig- 50% of children by 20 years of ment gallstones that initially may be asymptomatic but that can lead age.” The incidence of gallstones to acute symptoms of cholellthlasls. An elective laparoscoplc chole- varies with age across the life span cystectomy procedure Is recommended for pediatric patlents with for patients with SCD. For SCD to prevent the risk of requiring an emergency cholecystectomy instance, the incidence is 12% in the two- to fow-yearprocedure. The primary benefits of this approach Include a shorter old age group,‘* hospital stay after surgery, decreased postoperative discomfort, 9% in the five- to seven-yeardecreased risk of complications, and a quicker return to normal activold age group,” ities. AORN J 77 (June 2003) 1 170-1182.
ickle cell disease (SCD) is one of the most common genetic disorders in the United States.’ Primary symptoms of this chronic disease include chronic hemolytic anemia and tissue damage secondary to vaso-occlusion by sickle-shaped red blood cells (Figure 1). Patients with SCD have an increased risk of developing cholelithiasis (ie, gallstones)? Cholelithiasis should be considered as a potential diagnosis when a child with SCD experiences recurrent abdominal pain? Evaluation criteria used to c o n f m the diagnosis of cholelithiasis include radiological evaluation using biliary scintigram and ultrasound and laboratory results that indicate elevations in serum bilirubin, alkaline phosphatase, hepatic enzyme levels, and white blood cell An estimated 50% of patients in sickle cell crisis (ie, pain crisis) experience acute abdominal pain, which often is difficult to differentiate from acute chole~ystitis.~ One group of researchers noted that acute abdominal pain symptoms in children with SCD often were diagnosed as vaso-occlusive crisis rather than biliary colic associated with cholelithiasis.“
Y
VERNA HENDRICKS-FERGUSON, RN; MARTHA A. NELSON, RN
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43% in the 15- to 18-year-old age group,l4 83% in people older than 30 years of age,I5 and 60% to 85% in people age 33 or older.16 GALLBLADDER ANATOMY
The gallbladder’s shape has been compared to a pear (Figure 2). It is approximately 7 cm long and holds 30 mL to 50 mL of bile.” Branches of the cystic artery originating from the right hepatic artery provide the gallbladder’s blood supply. The common and right hepatic arteries provide the common bile duct with its blood supply. During cholecystectomy, the triangle of Calot serves as a surgical landmark to identify important structures.ls These structures include the cystic duct, the common hepatic duct, and the inferior border of the liver. The common hepatic duct, gallbladder, and common bile duct finction as a storage site for bile and a conduit for bile flow fiom the liver to the duodenum. PIGMENT GALLSTONES
Children with chronic hemolytic disorders generally have an increased risk Of pigment gallstone^.'^ Pigment gallstones have been observed in 15% of children with SCD who are younger than 10 years of ageZoand 50% of children with SCD by 20 years of age. The frequency of pigment gallstones is higher in this population because of a sustained increase in heme catabolism among children with SCD.” Approximately 10% to 20% of gallstones are referred to as pigment stones. These are composed of poorly soluble calcium salts, especially calcium bilirubin, and are dark in color. Pigment stones are categorized as black, brown, or mixed pigment gallstones. Black pigment gallstones are small, hard, and spiculated (ie, sharp body with needle-like points) and are made of calcium bilirubinate, inorganic salts (eg, calcium phosphate), and calcium carbonate.= Black pigment stones typically develop in patients who have a history of excessive secretion of unconjugated bilirubin into bile?’ Black pigment stones are observed in approximately 14% of children with SCD who are younger than 10 years of age. The percentage increases to 36% among school-age children and adolescents (ie. 10 to 20 years of age) and to 50% by age Z2‘ Brown pigment stones are soft, crumbly aggregates that often form in the bile ducts of patients with a history of chronically infected bile above biliary strictures or cholesterol cysts. They are made of calcium bilirubinate and calcium ~alrnitate.~~ Mixed
Figure 1 illustration of normal red blood cells in relation to sickled cells.
Figure 2 Normal anatomy of the gallbladder and related Structures.
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operations stage of cognitive development.” Characteristicsof this stage include interest in acquiring more detailed knowledge, improved concept of time, and increased self-control. Psychoanalyst Erik TREATMENT OF CHOLELlTHlASlS Erikson characterized children ages six to 12 as being The current recommended treatment for children in the industry versus inferiority stage of psychosoCharacteristics of this stage with SCD who are diagnosed with cholelithiasis is cia1 de~elopment.~~ elective laparoscopic cholecystectomy.27An essential include interest in participating in various school-related activities, component of the preoperative preparation for elective cholecystectomy among children with SCD is such as development of peer relationships; attaining skills in competition and cooperation; erythrocyte transfusion therapy. The purpose of this therapy is to reduce the percentage of hemoglobin S and developing a sense of competence. and increase the hemoglobin concentrationto greater than 10 g per dL. This reduces the risk of complicaSchool-age children may experience anxiety tions associated with vaso-occIusion.28 before an unfamiliar or familiar medical procedure Risks associated with erythrocyte transfusion because of their increased cognitiveability. They may therapy among patients with SCD may include an have many questions related to medical procedures increased risk of exposure to infectious agents and that require health care providers to give age-approallosensitizationto erythrocyte antigens. Undertaking priate explanations using correct scientific and medthese risks may be reasonable because of the ical terminology before all procedures. Explanation decreased risk of postoperative morbidity with elec- of these procedures can be enhanced by the use of tive laparoscopic cholecystectomy compared to the simple anatomy and physiology diagrams and concrete terms explaining why and how equipment knctraditional cholecystectomyprocedure. The primary goal of elective cholecystectomyis tions. According to developmental theory, school-age to prevent complications associated with emergency children are focused on gaining a sense of control and surgical intervention. Potential complications after competence during various activities; therefore, emergency cholecystectomy in this patient population health care providers should strive to keep children are associated with general anesthesia and the inabili- informed of what to expect before all medical procety to implement recommended preoperative interven- dures. Allowing time before procedures for patient tions, such as erythrocyte transfusion therapy? In questions and additional explanations is recommendaddition, children with SCD who undergo emergency ed. Health care providers also should educate schoolcholecystectomy are at high risk of developing age children about self-control strategies, such as intraoperative complications, such as vaso-occlu- helpful postoperative strategies (eg, deep breathing sion secondary to transient hypoxia, hypothermia, exercises, relaxation techniques, counting during dressing changes), before scheduled surgical procedehydration, or acidosis; postoperative complications, such as pneumonia, dures to prevent postoperative complications and acute chest syndrome, or painful vaso-occlusive minimize feelings of anxiety. events; and other complications,such as ascending cholangitis PREOPERATIVE PHASE (ie, inflammatory disease of the bile ducts), choleIn the preoperative holding area, the circulating docholithiasis (ie, gallstones in the common bile nurse and anesthesia care provider complete all duct), or acute cholecystitis (ie, gallstones or required preoperative assessments for a pediatric sludge obstructingthe cystic duct).’O patient before transferring the patient to the OR. Both participate in preoperative teaching for the parents to DEVELOPMENTALTHEORY inform them about what their child will experience. FOR SCHOOL-AGE PATIENTS The circulatingnurse confirms the child’s identity and Knowledge of the specific developmental char- the scheduled laparoscopic cholecystectomy proceacteristics of school-age children is important for dure with the child’s parents. The circulating nurse nurses and other pediatric surgical team members. also verifies that all surgical informed consents are Child psychologist Jean Piaget, PhD, characterized present and complete. In addition, the nurse docuchildren ages seven to 11 as being in the concrete ments the patient’s allergies, NPO status, presences of gallstones are made of both cholesterol and calcium bilirubinate, and bacterial colonization or inflammation of the gallbladder may develop later.26
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prostheses, loose teeth, rashes, bruises, and any other pertinent information that facilitates safe patient care. The perioperative nurse ensures that the following documents are present in the patient’s chart: medical history, physical examination, preoperative records, and appropriate laboratory test results. He or she then performs an assessment and documents the patient’s physical condition, including vital signs, height, weight, and skin integrity. The nurse also can review age-appropriate (ie, concrete operational thinking) concepts with school-age patients, such as information related to what the patient can expect during the surgical process (eg, approximate time in surgery, recovery, return to the unit), regarding the location of the surgical incision, and regarding the appearance of postoperative surgical dressings. The nurse should review the rationale and importance of implementing postoperative strategies, such as deep breathing exercises with the child. The perioperative nurse offers the child the opportunity to select a scented oxygen mask (eg, cherry, grape, strawberry) for use during anesthesia induction in the OR. Encouraging the patient’s participation in selecting a scented mask and providing concrete information related to the surgical process and postoperative strategies are examples of appropriate developmental strategies nurses can implement to aid in meeting the developmental needs of schoolage patients (eg, gaining self-control, developing self-competence). The perioperative nurse then develops a care plan for the pediatric patient with SCD undergoing elective laparoscopic cholecystectomy (Table 1). INTRAOPERATWE PHASE
Perioperative team members transport the pediatric patient on a child-size stretcher to the OR suite and place the patient on a locked OR bed. The circulating nurse reassures the patient and provides simple explanations of the intraoperative equipment using concrete terms, such as explaining that the purpose of the OR bed strap is to ensure safety and prevent accidental falls rather than to tie the child down. The circulating nurse incorporates distraction techniques appropriate for the school-age patient (eg, discusses hobbies, school activities, clubs, team sports) to minimize the emotional distress often expe-
rienced by children of this age group. The circulating nurse encourages the parents to accompany their child to the OR until after anesthesia induction to reduce the child’s fears. Anesthesia The circulating nurse assists the anesthesia care provider during induction of general anesthesia and intubation of the child. The nurse either places the patient’s IV line preoperatively in the pediatric unit or in the OR. The anesthesia care provider induces general anesthesia and places the endotracheal tube. After induction of anesthesia, the anesthesia care provider inserts an orogastric tube for decompression. After the child is intubated, a staff member escorts the parents to the parent surgical waiting room where they typically wait until their child’s surgical procedure is complete. Positioning and prepping. The circulating nurse initially positions the pediatric patient in the supine position on the OR bed and positions two video cameras at the head of the OR bed. The nurse evacuates the patient’s urinary bladder via the Credt’s maneuver (ie, massaging the bladder by pressing down on the anterior, superior surface of the abdomen). The circulating nurse cleanses the patient’s abdomen with an antiseptic solution. The pediatric surgeon, with the assistance of the scrub person, drapes the patient’s surgical site. Depending on the patient’s age, a four-cannula technique generally is used for the laparoscopic cholecystectomy procedure. During the initial steps of the procedure, the circulating nurse and anesthesia care provider rotate the patient and OR bed for improved visualization. The patient and bed are rotated into the reverse Trendelenburg’s and left-dependent positions to aid in allowing the adjacent abdominal viscera to fall away from the surgical area. The surgeon performs lateral retraction of the junction of the inhndibulum (ie, h n nel-shaped passage or structure) of the gallbladder and cystic d u ~ t . 3The ~ primary benefit of using lateral retraction is the ability to reposition the cystic duct into a 90% orientation to the common bile duct versus an oblique or even parallel orientation. This minimizes the need for surgical manipulation by retraction.” Risks. Surgery and general anesthesia are potential stressors for all children with SCD. Intraoperative complications may include transient hypoxia; hypothermia; dehydration;
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Table 1 NURSING CARE PLAN FOR PEDlATRlC M T I E N T S Wrm SICKLE C E U DISEASE ( X D ) UNDERGOING ELECTWE -0ScOPlC CHOLECYSlEClO MY
Nursing diagnosis
Interventions
Knowledge deficit regarding SCD and cholelithiasis and undergoing cholecystectomy
Includes family members in preoperative teaching and discharge planning.
Fear of the unknown related to undergoing cholecystectomy
Explains the purpose of intraoperative equipment in age-appropriate terms, including use of a safeiy belt to prevent falls, anesthesia equipment to monitor heart rate, pulse oximetty, and blood pressure. Incorporates distraction techniques appropriate for the school-age patient by discussing interests, such as , hobbies, school activities, clubs, or team sports.
Describes how SCD can cause cholelithiasis. Explains how cholecystectomy iS 0 definitive trC?ahel nt cholelithiasis. Evaluates responses to learning.
for
Q
Q
Interim outcome criteria
Outcome statement
Patient’s fear and anxiety are minimized related to his or her sense of uncertainty of events to occur in the surgical area.
Patient participates in discussions related to the surgical procedure.
Patient does not demonstrate emotional distress oflen experienced by pediatric
Patient demonstrates control over his or her environment appropriate for developmental level.
Patient does not demonstrate nonverbal signs and symptoms of pain throughout the pefioperative period.
Patient demonstrates adequate pain management and verbalizes relief of pain.
men’.
Q
Q Q Q
Encourages the patient to a select scented oxygen mask for use during anesthesia induction in the OR. Risk for acute pain related to surgical intervention and potential for sickle cell crisis
Assesses pain using ageappropriate assessment tools. Identifies cultural and value components related to pain and provides pain management instructions accordingly. hlpkmentS pain guidelines and eVahateS response to interventions.
increased risk of reduced respiratory effort due to postoperative incisional pain, resulting in poor pulmonary toilet and hypoxia symptoms;36and bleeding diathesis. Pediatric patients with SCD can experience bleeding diathesis and a sudden hemoglobin drop during the postoperative period even after an uneventful laparoscopic cholecystectomy procedure.” Erythrocyte transfusion therapy during the preoperative period, therefore, is recommended to increase the percentage of hemoglobin A to more than 65% of the total hemoglobin and a hematocrit of 35% to 45%.’8 Physiological factors that influence the development of complications include
patient immobility, hypothermia as a result of exposure to the cool surgical environment, hypoventilation, and pain. Supportive perioperative interventions for the patient with SCD, therefore, include careful attention to ensuring adequate oxygenation, hydration, and acidbase balance. In addition, patients with SCD receive IV hydration therapy at a rate of one and one-half times the calculated maintenance amounts for 12 hours before elective surgery. Hydration therapy is continued into the postoperative period until the patient displays adequate oral intake, which usually occurs by the third postoperative day.J’ Supportive
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postoperative recommendations include careful attention to assessment of the patient's abdomen, and oxygen saturation and monitoring of the hematocrit and hemoglobin values at six hours after surgery and each morning for two consecutive days." Other recommended precautions are related to the risk of hypoxia and hyp~thermia.~' The anesthesia care provider ensures that the patient receives 100% oxygenation before and after intubation and extubation and monitors the patient's body temperature to avoid hypothermia. The circulating nurse maintains the room temperature at 80" F to 85" F (27" C to 29" C) during the procedure, ensures the patient is positioned properly, and is careful to determine that IV stability devices and dressings have not caused circulatory stasis. LAPAROSCOPIC CHOLECYSTECTOMY PROCEDURE
The surgeon uses electrosurgeryto make a 1-cm umbilical incision and dissect through the abdomen until the fascia1 tissue is entered. He or she then inserts the Veress insumation needle into the abdomen and then places a 10-mm epigastric port. Using a 10-mm laparoscope, the surgeon visually inspects the patient's gallbladder.The anesthesia care provider rotates the bed into reverse Trendelenburg's and left-dependent position to help the adjacent viscera fall away from the surgical area." The surgeon looks for adhesions and other abnormalities. He or she infiltrates the additional port sites with bupivicaine and uses electrosurgery to make incisions for three 5-mm ports at the anterior axillary line in the right costal margin, at the midclavicular line along the right costal margin, and under the epigastrium. The surgeon places trocars and instruments into the 5-mm ports to grasp and elevate the gallbladder and then bluntly takes down any adhesions and grasps the infundibulum. The surgeon uses electrosurgery to remove the peritoneum from the gallbladder and then clear and reverse the triangle of Calot. The surgeon also inspects the cystic duct from its origin down to the gallbladder. He or she M e r divides the patient's peritoneum along the gallbladder to mobilize it before removing the cystic duct. The surgeon places two clips distal to the intended line of division and one on the cystic duct approximately 5 mm from its insertion into the common bile ducts. He or she transects the cystic duct and divides the
cystic artery with electrosurgery before removing the gallbladder. The surgeon creates a small hole in the gallbladder to drain it with suction, which prevents bile leakage into the peritoneal cavity, before removing the gallbladder from the gallbladder bed. He or she pulls the gallbladder out through the umbilical port in an endoscopic specimen bag. The surgeon inspects the gallbladder and irrigates and aspirates the area until it is clear. The surgeon removes all surgical instruments from the patient's abdomen and deflates the patient's abdomen by allowing the carbon dioxide to escape via the ports, after which he or she removes the ports. The surgeon closes the umbilical fascia with interrupted 2-0 braided absorbable polyglactin suture. The surgeon then infiltrates all surgical wounds with bupivicaine and closes the skin with subcuticular stitches of monofilament absorbable poliglecaprone. The surgeon uses tincture of benzoin and self-adhesive wound approximating strips to close the 5-mm port sites and applies antibiotic ointment and a sterile adhesive bandage strip on the patient's umbilicus. POSTOPERATIVE CARE
The circulating nurse and anesthesia care provider transport the patient to the postanesthesia care unit (PACU) after the surgical procedure is completed. The circulating nurse gives report to the PACU nurse. After receiving report, the PACU nurse assesses the patient's vital signs, oxygen saturation level, level of consciousness, circulation, pain, IV site, fluid rate, and hydration status, as well as the status of the surgical site and dressing and all related monitoring equipment. The nurse also places warming blankets on the patient to enhance comfort and preserve the patient's body temperature. DkchargR Pediatric patients with SCD usually are discharged four to six days after elective laparoscopic cholecystectomy.The pediatric surgical nurse provides in-depth discharge teaching to the parents about the child's recommended activity level and restrictions, pain management, and diet. He or she also discusses recommended follow-up management with the physician and the surgeon (Table 2). POTENTIAL COMPLICATIONS
The major benefits of elective laparoscopic cholecystectomy include shorter hospitalization, decreased postoperative discomfort, a shorter interval before returning to normal activities," and increased
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respiratory efforts.” Patients who have undergone this procedure, however, have a slightly increased risk of major bile duct injury (ie, 0.3% versus 0.2% for those who undergo traditional open chole~ystectomy).4~ The primary complication identified is the risk of bile duct injury among adults6 and bleeding diathesjs among children with SCD.4’ It is recommended, therefore, that all children with SCD be monitored closely postoperatively, including having their hemoglobin and hematocrit assessed for 48 hours to 72 hours. Laparoscopic cholecystectomy also carries a small risk of complications for all patients related to required surgical treatments, such as anesthesia,blood transfusion, hemorrhage, and insufflation of the peritoneal cavity with carbon CASE STUW
Sarah, a 10-year-old African-American female with a history of SCD, was admitted to the pediatric unit at St Louis Children’s Hospital with a suspected diagnosis of acute cholecystitis. After undergoing numerous diagnostic examinations, the physician confirmed diagnoses of acute cholecystitis, prolonged prothrombrin time, and multiple pigmented stones in the gallbladder.Hematology-oncologyteam members evaluated Sarah and initiated medical interventions, including pain management and vitamin K administration. Sarah recovered from the acute painhl episode and was scheduled for elective laparoscopic cholecystectomy.
Table 2 DISCHARGE ORDERS
Activities No swimming or submersion of incision in water for 1 week No running or contact sports for 2 weeks Diet
Regular
Pain management Acetaminophen with codeine, 1 tablet every 4 hrs to 6 hrs as needed Wound care Shower 1 time per day and do not submerge incision Allow self-adhesive wound approximating strips to fall off with time
Sarah’s pediatric nurse started an IV in Sarah’s left anticubital fossa and began administering IV fluids (ie, 5% dextrose in 0.45%normal saline with 20 mEq potassium chloride) at 80 mL per hour in preparation for the scheduled surgical procedure. The nurse also administered prophylactic antibiotic therapy (ie, 1 g IV cefoxitin sodium) every eight hours for three doses in preparation for the surgical procedure. The elective laparoscopic cholecystectomy procedure proceeded uneventfully,and although the surgeon identified some adhesions from the omentum to the gallbladder, she detected no other abnormalities except for mild hepatomegaly. The surgeon bluntly dissected all detected adhesions from the omentum on the duodenum into the gallbladder and dissected the gallbladder free. She was unable to locate Sarah’s dominant artery; however, small branches of this artery were identified. While mobilizing and transecting the cystic duct, the surgeon determined that one of the distally placed clips appeared not to have approximated appropriately; therefore, the surgeon placed an endoloop proximal to the clips on the cystic duct stump. She removed the gallbladder without difficulty and closed the incisions. Sarah was transferred via pediatric stretcher to the PACU, and after she recovered for 60 minutes, PACU nurses transferred Sarah to the pediatric surgical unit. Sarah’s pediatric surgical unit nurse completed an admission assessment and then monitored Sarah’s vital signs every four hours until discharge. During the surgical procedure and the postoperative phase, the nurse managed Sarah’s pain with a continuous IV infusion via a computerized patientcontrolled anesthesia (PCA) pump of 1 mg per mL of morphine sulfate (MS) at a rate if 0.5 mg per hour. In the event that Sarah experienced MS-associated side effects, such as pruritus, the nurse had orders to administer 25 mg diphenhydramine hydrochloride IV or by mouth (PO) every six hours as needed. If the pruritus was unresponsive to diphenhydramine, Sarah’s nurse could administer 2 mg of IV nalbuphine every one to two hours as needed. Additionally, the n m e had orders to treat other side effects associated with MS therapy, such as nausea and vomiting, with 4 mg lV ondansetron every six hours or 6 mg IV metaclopramide hydrochloride every six hours as needed. Fortunately, Sarah did not experience side effects typically associated with MS. On the first postoperative day, Sarah was not allowed any oral intake for 24 hours. On her second postoperative day, the nurse slowly advanced Sarah’s
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diet beginning with clear liquids, increasing to a normal diet as tolerated. The nurse assisted Sarah out of bed on the second postoperative day and encouraged her to increase her activity as tolerated, offering assistance as needed. The nurse discontinued Sarah’s PCA, IV fluids, and IV medications during the second day and managed Sarah’s pain with PO oxycodone 5 mg or acetaminophen with codeine every four to six hours as needed. The nurse instructed Sarah and her parents about discharge instructions. He also instructed Sarah’s parents to make Sarah’s follow-up appointment in approximately four to six weeks. Overall, this patient experienced an uneventful recovery period after the surgical procedure. SUMMARY
Elective laparoscopic cholecystectomy in children has been described as a safe and effective alternative to the traditional cholecystectomy surgical procedure because of a shortened hospital stay and improved postoperative recovery.” One of the significant benefits of laparoscopic cholecystectomy for children with SCD is reduced postoperative pain, which improves pulmonary toilet and decreases risk of pneumonia and vaso-occlusive events.5o Other identified postoperative benefits include reduced risk NOTES 1. V L Hendricks-Ferguson, M A
Nelson, “Laparoscopic splenectomy for splenic sequestration crisis,” AORN Journal 7 1 (April 2000) 820834. 2. R E Ware et al, “Diagnosis and
of postoperative ileus, smaller incision, and decreased keloid formation, resulting in an improved body image for young patients with SCD.5’ Current guidelines for children with SCD experiencing symptoms of cholelithiasis recommend elective laparoscopic cholecystectomy to prevent complications such as acute cholecystitis and the risk of emergency surgery for an unprepared patient.s2The cholecystectomy procedure, whether traditional or laparoscopic, does not cure SCD. The elective laparoscopic cholecystectomy procedure, however, offers a planned surgical intervention, reducing the risk of complications associated with emergency cholecystectomy. For school-age patients with SCD, these benefits minimize feelings of fear and anxiety associated with surgery and hospitalization and foster a sense of self-control. A
Vema Hendricks-Ferguson,RN, DNSc, CS, is an associate professor at Jewish Hospital, College of Nursing and Allied Health, Edwarriville, Ill. Martha A. Nelson, RN,BS, CPNR is the pediatric nurse practitioner and sickle eel/ patient coordinator at St Louis Children k Hospital.
laparoscopic cholecystectomy on the management of cholelithiasis in children with sickle cell disease,” Journal of Pediatric Surgery 29 (February 1994) 209-213;A J Jawad et al, “Laparoscopic cholecystectomy for cholelithiasis during infancy and childhood: Cost analysis and review of current indications,” World Journal of Surgery 22 (January
management of common bile duct stones in patients with sickle hemoglobinopathies,”Journal of Pediatric Surgery 27 (May 1992) 572-575. 3. D De Caluwe, U Akl,M Corbally, “Cholecystectomy versus cholecystolithotomy for cholelithiasis in childhood: Long-term outcome,” Journal of Pediatric Surgery 36 (October 2001) 1518-1521. 4. R Rudolph, J S Williams, “Cholecystectomy in patients with sickle cell disease: Experience at a regional hospital in southeast Georgia,” Journal of the National Medical Association 84 (August
“Cholecystectomy in patients with sickle cell disease: Experience at a regional hospital in southeast Georgia,” 692-696. 8. E A Pomfret, J J Pomposelli, R L Jenkins, “Surgical approaches to diseases of the biliary system,” in Schiff k Diseases of the Livel: eighth ed, E R Schiff, M F Sorrell, W C Maddrey, eds (Philadelphia: Lippincott-Raven Publishers, 1999)
1992) 692-696. 5 . Ibid 6. E P Tagge et al, “Impact of
693-717. 9. E A Shaffer, “Gallbladder disease,” in Pediatric Gastrointestinal
1998) 69-74. 7. Rudolph, Williams,
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Disease: Pathophysiology, Diagnosis, Management, third ed, W A
Walker et al, eds (Hamilton, Ontario: B C Decker, 2000) 1291-1311. 10. C F Gholson et al, “Sequential
endoscopicflaparoscopic management of sickle hemoglobinopathyassociated cholelithiasis and suspected choledocholithiasis,” Southern Medical Journal 88 (November 1995) 113 1- 1 135; Jawad et al, “Laparoscopic cholecystectomy for cholelithiasis during infancy and childhood: Cost analysis and review of current indications,” 69-74. 11. G W Holcomb, J B Pietsch, “Gallbladder disease and hepatic infections,” in Pediatric Surgery, vol two, fifth ed,J A O’Neill et al, eds (St Louis: Mosby, 1998) 1495-1511. 12. Rudolph, Williams, “Cholecystectomy in patients with sickle cell disease: Experience at a regional hospital in southeast Georgia,” 692-696. 13. N D Duncan, A H McDonald,
JUNE 2003, VOL 77, NO 6 Hendricks-Ferguson Nelson
D I G Mitchell, “Cholecystectomy in Jamaican children with homozygous sickle-cell disease,” Tropical Doctor 30 (October 2000) 214-216. 14. Rudolph, Williams, “Cholecystectomy in patients with sickle cell disease: Experience at a regional hospital in southeast Georgia,” 692-696. 15. Duncan, McDonald, Mitchell, “Cholecystectomy in Jamaican children with homozygous sickle-cell disease,” 2 14-216. 16. Shaffer, “Gallbladder disease,” 1291-1311. 17. D L Nahrwold, “The b i l i q system,” in Textbook of Surgery: The Biological Basb of Modern Surgical Practice, 15th ed, D C Sabiston, H K Lyerly, eds (Philadelphia: W B Saunders Co, 1997) 1117-1125. 18. Ibid. 19. Shaffer, “Gallbladder disease,” 1291-1311. 20. Gholson et al, “Sequential endoscopicflaparoscopic management of sickle hemoglobinopathyassociated cholelithiasisand suspected choledocholithiasis,” 1131-1135. 2 1. Holcomb, Pietsch, “Gallbladder disease and hepatic infections,”1495-1511;TMWalker,IR Hambleton, G R Sejeant, “Gallstones in sickle cell disease: Observations from the Jamaican cohort study,” The Journal of Pediatrics 136 (January 2000) 80-85. 22. Shaffer, “Gallbladder disease,” 1291-1311. 23. D M Heuman, W Y Wassef, Z R Vlahcevic, “Gallstones,” in Schiff 5 Diseases of the Liver: eighth ed, E R Schiff, M F Sorrell,W C Maddrey, eds (Philadelphia: Lippincott-Raven Publishers, 1999)63 1647. 24. Ibid. 25. Ibid. 26. Shaffer, “Gallbladder disease,” 1291-13 11. 27. Heuman, Wassef, Vlahcevic, “Gallstones,” 63 1-647; Jawad et al,
“Laparoscopiccholecystectomyfor cholelithiasisduring infancy and childhood: Cost analysis and review of current indications,” 69-74. 28. Ibid. 29. A H Al-Salem et al, “Laparoscopic versus open cholecystectomy in children,” Pediatric Surgery International 12 (October1997) 587-590; A H Al-Salem, S Qaisruddin, “The significanceof biliary sludge in children with sickle cell disease,” Pediatric Surgery International 13 (January 1998) 14-16; Duncan, McDonald, Mitchell, “Cholecystectomy in Jamaican children with homozygous sickle-cell disease,” 2 14-216; R M Hatley et al, “Laparoscopiccholecystectomy in children with sickle cell disease,” The American Surgeon 61 (February 1995) 169-171; Holcomb, Pietsch, “Gallbladder disease and hepatic infections,” 1495-1511; Tagge et al, “Impact of laparoscopiccholecystectomy on the management of cholelithiasisin children with sickle cell disease,” 209-2 13, 30. R Ware et al, “Laparoscopic cholecystectomyin young patients with sickle hemoglobinopathies,” The Journal of Pediatrics 120 (January 1992) 58-6 1. 3 1. J Piaget, The Theory of Stages of Cognitive Development (New York McGraw-Hill, 1969). 32. E Erikson, Childhood and Socie& second ed (New York: Norton Press, 1963). 33. Nahrwold, ‘The biliary system,” 1117. 34. Holcomb, Pietsch, ‘‘Gallbladder disease and hepatic infections,” 1495-1511. 35. Ware et al, “Laparoscopic cholecystectomy in young patients with sickle hemoglobinopathies,” 58-61. 36. Ibid. 37. Hatley et al, “Laparoscopic cholecystectomyin children with sickle cell disease,” 169-17 1.
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38. Ware et al, “Elective cholecystectomy in children with sickle hemoglobinopathies: Successfid outcome using a preoperativetransfusion regimen,” 17-22. 39. Ibid. 40. Hatley et al, “Laparoscopic cholecystectomyin children with sickle cell disease,” 169-17 1. 4 1. Ware et al, “Laparoscopic cholecystectomy in young patients with sickle hemoglobinopathies,” 58-61. 42. Holcomb, Pietsch, “Gallbladder disease and hepatic infections,” 1495-151 1. 43. G W Holcomb, D 0 Olsen, K W Sharp, “Laparoscopiccholecystectomy in the pediatric patient,” Journal of Pediatric Surgery 26 (October 1991) 1186-1190; E Leandros et al, “Laparoscopic or open cholecystectomyin patients with sickle cell disease: Which approach is superior?“ The European Journal of Surgery 166 (November 2000) 859-861. 44.Hatley et al, “Laparoscopic cholecystectomyin children with sickle cell disease,” 169-17 1. 45. Pomfret, Pomposelli, Jenkins, “Surgical approaches to diseases of the biliary system,” 693-717. 46. Ibid. 47. Hatley et al, “Laparoscopic cholecystectomyin children with sickle cell disease,” 169-17 1. 48. Walker, Hambleton, Sejeant, “Gallstones in sickle cell disease: Observations from the Jamaican Cohort study,” (January 2000) 8085. 49. Hatley et al, “Laparoscopic cholecystectomyin children with sickle cell disease,’’ 169-171. 50. Ibid. 5 1. Ware et al, “Laparoscopic cholecystectomy in young patients with sickle hemoglobinopathies,” 58-61. 52. Ibid.