The Ex Utero Intrapartum Treatment (EXIT) Procedure for Fetal Head and Neck Masses

Article Outline

• ABSTRACT
• The EXIT Procedure
• Our Case
• Planning for the Procedure
• Algorithm for Care
• The Procedure
• Complications
• Conclusion
• Examination
  • The Ex Utero Intrapartum Treatment (EXIT) Procedure for Fetal Head and Neck Masses
    • Purpose/Goal
    • Behavioral Objectives
    • Questions
• Answer Sheet
  • The Ex Utero Intrapartum Treatment (EXIT) Procedure for Fetal Head and Neck Masses
• Learner Evaluation
  • The Ex Utero Intrapartum Treatment (EXIT) Procedure for Fetal Head and Neck Masses
    • Purpose/Goal
    • Objectives
    • Content
    • Test Questions/Answers
    • Learner Input
• Acknowledgement
• References
• Copyright

ABSTRACT 

A congenital head or neck mass increases the risk of airway obstruction and imminent respiratory failure at birth. The ex utero intrapartum treatment (EXIT) procedure is an option to secure the infant's airway in some clinical situations when an antenatal diagnosis of a head or neck mass or potential airway obstruction has been established.

This article discusses an EXIT procedure performed on a maternal patient whose 38-week gestational age fetus was diagnosed with a lymphatic malformation. Planning and coordination between surgical teams at two clinical sites allowed the multidisciplinary teams to achieve a safe, successful outcome for the mother and child. AORN J 90 (November 2009) 661–672. © AORN, Inc, 2009.

Key words:  ex utero intrapartum treatment , EXIT procedure , fetal airway obstruction , lymphatic malformation , placental support , pediatric airway emergencies , maternal complications

Antenatal diagnosis of fetal abnormalities allows pediatric specialists to anticipate potentially fatal neonatal airway compromise and manage it in a safe, controlled manner by using the ex utero intrapartum treatment (EXIT) procedure. Although the EXIT procedure was first developed in 1989 for the treatment of severe congenital diaphragmatic hernias, it has been adapted for other fetal abnormalities, such as giant neck masses; congenital, high-airway obstruction syndrome; lung or mediastinal tumors; separation of conjoined twins; and extracorporeal membrane oxygenation procedures for fetal cardiac and pulmonary defects.¹ Currently, the most common indication for the procedure is fetal airway obstruction.², ³ With the development of the EXIT procedure, certain fetal abnormalities causing airway obstruction can now be managed in a planned, sequential manner.

Successful completion of an EXIT procedure often involves a team from a pediatric hospital working at the obstetric hospital. This requires a significant amount of planning, communication, cooperation, and respect. An established policy and a collegial relationship between team members at the pediatric and maternal facilities can determine the success of the EXIT procedure and help the multidisciplinary teams achieve a safe, successful outcome for the mother and baby.

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The EXIT Procedure 

The EXIT procedure has replaced the operation on placental support (OOPS) procedure as the procedure of choice for fetal airway management. In the OOPS procedure, the surgeon delivers the fetus by cesarean delivery and maintains the uteroplacental circulation by leaving the umbilical cord intact. There is no attempt during an OOPS procedure to compensate for the loss of uterine volume, and uteroplacental circulation may not be adequately maintained, which can potentially lead to neonatal complications.⁴

In contrast, the EXIT procedure allows a controlled and timely approach to securing the fetal airway while maintaining uteroplacental circulation. During an EXIT procedure, the surgeon initially delivers only the fetal head and thorax via cesarean incision, thus the uteroplacental circulation is optimized. Goals of the EXIT procedure include optimizing uteroplacental circulation and maintaining the uterus in a state of hypotonia (ie, relaxation) via anesthesia, which preserves uterine volume and prevents placental abruption that can cause maternal hemorrhage.² The EXIT procedure allows for controlled evaluation of the fetal airway, endotracheal intubation, or performance of tracheostomy or neck dissection while avoiding fetal hypoxia, ischemic brain injury, or death, which could occur if the fetus is separated from the maternal circulation during the cesarean delivery.⁵ The deep maternal anesthesia used for an EXIT procedure also allows the fetal surgery to be performed with optimal perfusion to the fetus and optimal uterine blood flow.² It has been reported that the uteroplacental circulation can be maintained for up to one hour; however, the average time is 30.3 minutes, plus or minus approximately 14.7 minutes.², ⁶ A team approach is absolutely crucial to the planning and coordination of this procedure.

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Our Case 

Mrs M was diagnosed at 30 weeks of gestation as carrying a female fetus with a lymphatic malformation of the right, submandibular area of the neck. Lymphatic malformations are cystic lesions caused by abnormal prenatal development of the lymphatic system.⁷ These malformations are composed of spongy cysts filled with lymphatic fluid and are classified as microcystic (ie, small cysts) or macrocystic (eg, large cysts) or may be a combination of both types.⁸ The lesions may vary in size from several millimeters to more than 30 cm in diameter.⁹

Lymphatic malformations occur most commonly in the head and neck regions, and more than 50% are present and recognizable at birth.⁹ If a fetus presents with a large neck mass during diagnostic study, there may be significant distortion of the anatomy and a risk of airway obstruction that can make the airway difficult to secure at birth.⁵ Lymphatic malformations may also be seen with other fetal chromosomal abnormalities and genetic conditions, such as Down, Fryns, Klinefelter, Noonan, and Turner syndromes.⁹, ¹⁰ Exposure to teratogens (eg, alcohol) during pregnancy is linked to lymphatic malformation development.⁹ Both genders are affected equally by this birth defect.⁹

A lymphatic malformation may be diagnosed via prenatal ultrasound as early as 10 weeks of gestation.⁹, ¹¹ Although an ultrasound can identify the relationship of the lymphatic malformation to adjacent structures, magnetic resonance imaging (MRI) is the diagnostic imaging method of choice. An MRI can accurately define the fetal anatomy and the proximity of the lesion to other structures, such as the trachea and great vessels, as well as determine whether the airway is patent or compressed.¹¹

Mrs M's prenatal ultrasound at 30 weeks of gestation demonstrated a fetal neck cyst that was 3.2 cm by 3 cm by 2.7 cm. Five weeks later, an MRI of Mrs M's pelvis showed a right-sided, submandibular mass consistent with a lymphatic malformation that measured 4.3 cm by 4.6 cm by 3.4 cm (Figure 1). At 38 weeks of gestation, the MRI showed that the fetal neck cyst had increased in size and was causing tracheal displacement. It had also extended into the prevertebral space. If left untreated, this mass would pose significant potential for life-threatening respiratory compromise at birth.

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• Figure 1. 

  Magnetic resonance imaging scan showing the fetus with a right-sided, submandibular mass at 35 weeks of gestation.

The EXIT procedure described in this article was performed at the Royal Alexandra Hospital's Women's Health Centre, Edmonton, Alberta, Canada, which specializes in high-risk maternal care. The pediatric team was from Stollery Children's Hospital, Edmonton. A series of conferences and meetings between health care professionals from the tertiary maternity and pediatric centers who would care for Mrs M and her child took place to plan and coordinate

The perinatologist from Stollery Children's Hospital arranged a case conference that included the neonatologist, obstetrician, and pediatric otolaryngologist to discuss

The collaborating team members later held a meeting with the parents to provide preoperative teaching, obtain a surgical consent, and give psychosocial support to the family. The team demonstrated a caring attitude and provided time for the patient and family members to ask questions to alleviate their fear of the unknown.

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Planning for the Procedure 

Preoperative planning and communication between the coordinators and the two teams ultimately determines the success of any EXIT procedure. A contingency plan must be developed that covers all possible outcomes. Meticulous attention to detail is required when planning the timing of the delivery, the delivery site, the members of the mother's and the baby's teams, transportation of supplies and staff members, and the technical aspects of the procedure. While planning for an elective EXIT procedure, team members also should develop a plan to care for the mother and fetus if labor begins before the planned procedure is scheduled. The members of both teams for this procedure were on call until the planned EXIT procedure had taken place.

When two teams from different sites are required, as in our case, a site visit for the visiting team should be arranged well in advance of the procedure. Contacts can be established and the necessary equipment and OR space can be evaluated. The site visit also provides the visiting pediatric team with the opportunity to become familiar with the locations of on-site facilities, such as parking and emergency entrances.

In the planning process, decisions about the members of the two separate multidisciplinary teams should be made as soon as possible. The maternal team should consist of a high-risk obstetrician, an anesthesia care provider, a scrub person, and two circulating nurses. The pediatric team requires a pediatric otolaryngologist, a pediatric anesthesia care provider, a neonatologist, a scrub person, and a circulating nurse, as well as a neonatal intensive care unit (NICU) team. Each team member must respect the other team members' expertise, and the team members must have the ability to work well together and communicate effectively.

The nursing staff members of both the maternal and pediatric teams are chosen based on their experience, knowledge, and decision-making abilities. Nursing expertise is crucial to the ability of both teams to safely perform the procedure and care for the mother and baby.¹² All team members must be able to act appropriately in stressful situations and handle complications successfully during the surgical procedure. It is through astute observation, attention to the procedure, and active participation that the scrub persons are able to anticipate what will occur during the surgical procedure and prepare instruments and equipment as necessary. The circulating nurses use their clinical expertise to assess the patients and plan for their care, prepare and maintain the OR environment, provide necessary resources for team members, and direct patient care throughout the perioperative period.

Two adjacent ORs are required for an EXIT procedure. The first OR is set up for the cesarean delivery (Figure 2) and also has a sterile respiratory instrument table (Figure 3) that contains a variety of pediatric respiratory instruments and supplies (Table 1). In addition to the basic sterile setup, the maternal OR should have a radiant warming isolette and monitoring and resuscitation equipment for the baby. A neck dissection set should be available.

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• Figure 2. 

  Maternal and neonatal OR teams work together to perform the cesarean delivery and the EXIT procedure.

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• Figure 3. 

  The respiratory instrument table for the maternal OR.

Table 1. Maternal OR Setup

* Have an abdominal hysterectomy set available

The second OR should be set up for the neonate if an intubation, tracheostomy, or neck dissection cannot be performed while the fetus is on uteroplacental support (Table 2). This OR should be set up and staffed so that the pediatric team can simply move from one OR to the other without any disruption in the care of the neonate. The anesthesia machine should be set up with pediatric anesthetic tubing, bags, and monitoring equipment. This OR also should have an electrosurgery unit, positioning equipment, and sterile supplies in place.

Table 2. Neonatal OR Setup

An endoscope tower with a light source and camera equipment should be available in both ORs. The team should formulate a plan for how equipment will be transported between sites, if necessary. We were able to borrow some equipment from the obstetric hospital; however, most of our equipment and supplies were transported with the pediatric team to the obstetric hospital.

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Algorithm for Care 

An airway algorithm for head and neck masses should be developed for any hospital where EXIT procedures are performed (Figure 4). In our airway algorithm, direct laryngoscopy and endotracheal intubation are first performed to secure the fetal airway. If this fails, the next step is to perform a flexible or rigid bronchoscopy. A tracheostomy, a neck dissection with tracheostomy, and a tracheostomy with retrograde intubation are other techniques that may be used to secure the fetal airway if endotracheal intubation is unsuccessful. Our objective was to secure the fetal airway within the first 10 minutes after gaining access to it. Both of the teams and the sterile tables, supplies, and equipment for both ORs must be in place before the start of the cesarean delivery to accommodate this plan of care. It is very important to be mindful of space requirements as the OR can quickly become very crowded with additional equipment and personnel, making it more difficult to provide care.

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• Figure 4. 

  Algorithm for securing the neonatal airway during an EXIT procedure.

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The Procedure 

Before transporting the patient to the OR, a perioperative RN performed a preoperative assessment of the maternal patient. There was no preoperative monitoring of the fetus because the mother was not in labor, her amniotic membranes were intact, and the fetus was not in distress. The assessment included a review of surgical consent, medical history, physical examination, diagnostic test results, presence of allergies, and a review of the patient's baseline vital signs.¹³ The perioperative nurse performed a preoperative interview during which she confirmed the patient's identity, the planned surgical procedure, the presence of allergies, the removal of all personal belongings, and NPO status. The nurse also assessed the maternal patient's physical condition and anxiety level.¹³ These assessments provided valuable information about the patient; initiated a trusting relationship with the patient; and allowed the RN to formulate an individualized, intraoperative maternal (Table 3) and fetal (Table 4) plans of care.¹⁴

Table 3. Nursing Care Plan for the Maternal Patient Undergoing the Ex Utero Intrapartum Treatment (EXIT) Procedure for Fetal Head and Neck Masses

Table 4. Nursing Care Plan for the Fetal Patient Undergoing the Ex Utero Intrapartum Treatment (EXIT) Procedure for Fetal Head and Neck Masses

The circulating nurse transported the patient to the OR when the assessment was complete and all pertinent information had been communicated to the team members. The nurse provided the patient with warm blankets, positioned her supine with left uterine displacement to maximize uterine artery perfusion and prevent fetal hypoxia,¹ and remained at her side to provide support and assist the anesthesia care provider until the patient was anesthetized. The anesthesia care provider induced the patient using a rapid-sequence induction. Rapid-sequence inductions are used for pregnant women to reduce their risk of aspiration from inadequate stomach emptying, which results from abdominal crowding caused by the gravid uterus. After the team members positioned the patient and the nurse performed the abdominal skin prep, the team members completed a time out to ensure that the patient present in the OR was the correct patient and that the correct procedure was about to occur. When that information was verified, the surgical team finished draping the patient.

The surgeon then made a low, transverse abdominal incision to begin the cesarean delivery. The use of a deep inhalation general anesthetic decreased uterine tone, optimized maternal blood pressure, and decreased myometrial vasoconstriction while maximizing the fetal placental circulation.² The anesthesia care provider used a high concentration of inhalation agents that cross the placental barrier to provide fetal anesthesia,³ which facilitates intubation of the fetus and provides sedation and surgical analgesia for procedures performed before the fetus is separated from maternal circulation.¹⁵

The surgeon used chromic sutures to secure the edges of the uterus to decrease the incidence of uterine bleeding before delivering the fetus. The surgeon delivered the fetal head, shoulders, and thorax, leaving the fetus' body and limbs undelivered to maintain uterine volume and prevent uterine contraction and expulsion of the placenta. The surgeon placed a sterile pulse oximeter on the fetus' hand to provide continuous fetal monitoring. Normal fetal arterial saturation is 60% to 70%; however, values of greater than 40%, while not optimal, indicate adequate fetal oxygenation.¹

The pediatric otolaryngologist then performed a direct laryngoscopy with a 2.7-mm, zero-degree rigid laryngoscope by using a size zero Wisconsin blade. Finally, the otolaryngologist secured the baby's airway with an uncuffed, 2.0 endotracheal tube (Figure 5). The pediatric anesthesia care provider ventilated the fetus by hand and verified endotracheal tube placement. At this point, the surgeon completed the fetus' delivery, clamped and divided the umbilical cord, and passed the neonate to the neonatal resuscitation team. The pediatric team immediately sent cord blood gases to the laboratory and rated the baby's status according to the Apgar scale. The neonate's Apgar ratings were 3 at one minute, 7 at five minutes, and 9 at 10 minutes. Immediately after resuscitation, the pediatric team transported the neonate to the NICU.

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• Figure 5. 

  The pediatric otolaryngologist secures the airway via laryngoscopy during the EXIT procedure.

In the maternal OR, the surgeon removed the chromic sutures from the mother's uterus and completed uterine closure followed by surgical wound closure as the scrub person and circulating nurse completed the surgical counts. After completion of the procedure, the team transported the mother, awake and extubated, to the postanesthesia care unit. Several days later, the obstetrician discharged the mother home after an uneventful postoperative period.

The neonatologist extubated the neonate within 48 hours after the EXIT procedure and after an MRI confirmed that her airway would not be compromised. The pediatric otolaryngologist performed multiple bronchoscopies and injected OK-432, a sclerotherapy agent currently used in clinical research trials for the treatment of lymphatic malformations, into the lesion to reduce its size.¹⁶, ¹⁷ The OK-432 is not a one-time therapy; it must be injected over a period of time to sclerose lymphatic malformations. The time required to effectively treat a lymphatic malformation may differ depending on whether the malformation is macrocystic or microcystic. The pediatric otolaryngologist discharged the infant after three weeks in the NICU. The otolaryngologist is currently observing the baby on a monthly basis and continuing to inject OK-432 into the malformation.

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Complications 

Contraindications to performing an EXIT procedure include a disabling or lethal structural or genetic abnormality in the fetus or a serious medical condition in the mother.¹¹ There are also several potential complications that may occur as a result of performing an EXIT procedure.² The mother may experience an increase in bleeding because of the uterine hypotonia or lack of uterine contraction after this procedure, and, in rare situations, a hysterectomy may need to be performed.⁵ In addition, the inhalation agents used during the cesarean delivery can lead not only to a decrease in myometrial tone but also may cause maternal hypotension and a decrease in placental blood flow.¹ If uterine volume is lost from delivering too much of the fetus, then placental abruption may occur.¹ Any compromise to the uteroplacental circulation that results in fetal or maternal distress may necessitate the immediate termination of the EXIT procedure.¹ In this event, the surgeon would deliver the fetus and clamp and cut the umbilical cord. The pediatric team would resuscitate the neonate in the adjacent OR.

There is an increased risk of postpartum maternal wound complications as well as an increased risk of infection after an EXIT procedure.¹⁸ Infection risks increase because of the number of personnel in the OR as well as the length of time required to perform the procedure.

The fetus' body temperature may drop significantly during an EXIT procedure because of exposed skin surface areas. The fetus may be gestationally immature and have little body fat to provide insulation and thus be unable to regulate its temperature. The fetus also may be at risk of hypoxia and acidosis because of increased oxygen demand caused by hypothermia.¹⁵, ¹⁹ For these reasons, it may be advantageous in some cases for the surgeon to deliver only the fetus' head and leave the rest of the body in utero until the airway is secured.³ Lack of preparation and a lack of contingency plans for all potential airway challenges may contribute to fetal complications or death if the baby's airway cannot be secured.²⁰

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Conclusion 

Early diagnosis of fetal abnormalities has allowed potentially fatal neonatal airway compromise to be anticipated and managed in a safe, controlled manner with the EXIT procedure. It is important that each patient identified for an EXIT procedure be evaluated individually. It is necessary, however, for the staff members of a hospital performing EXIT procedures to develop a protocol for the management of head and neck masses, and a standard operating procedure should be in place. The importance of meticulous planning and communication for an EXIT procedure cannot be overemphasized and must occur when the procedure is performed at a site other than the team's home base. Two multidisciplinary teams working together can achieve a safe, successful outcome for the mother and baby.

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Examination 

The Ex Utero Intrapartum Treatment (EXIT) Procedure for Fetal Head and Neck Masses 

Purpose/Goal 

To educate perioperative nurses about the EXIT procedure for delivery of an infant with a head or neck mass and potential airway obstruction.

Behavioral Objectives 

After reading and studying the article on the EXIT procedure, nurses will be able to

Questions 

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Answer Sheet 

The Ex Utero Intrapartum Treatment (EXIT) Procedure for Fetal Head and Neck Masses 

Event #09308

Session #1176

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Learner Evaluation 

The Ex Utero Intrapartum Treatment (EXIT) Procedure for Fetal Head and Neck Masses 

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This evaluation is used to determine the extent to which this continuing education program met your learning needs. Rate these items on a scale of 1 to 5.

Purpose/Goal 

To educate perioperative nurses about the EXIT procedure for delivery of an infant with a head or neck mass and potential airway obstruction.

Objectives 

To what extent were the following objectives of this continuing education program achieved?

Content 

To what extent

Test Questions/Answers 

To what extent

Learner Input 

What other topics would you like to see addressed in a future continuing education article? Would you be interested or do you know someone who would be interested in writing an article on this topic?

Topic(s): _______________________________________________________________________________________

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Acknowledgement 

The authors thank Hamdy El-Hakim, MB, ChB, FRCS(Ed), FRCS(ORL-HNS), pediatric otolaryngologist at Stollery Children's Hospital, Edmonton, Alberta, Canada, for his guidance, knowledge, and encouragement during the writing of this article.

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References 

1. Marwan A , Crombleholme TM . The EXIT procedure: principles, pitfalls, progress . Semin Pediatr Surg. . 2006;15(2):107–115
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3. Chinnappa V , Halpern SH . The ex utero intrapartum treatment (EXIT) procedure: maternal and fetal considerations . Can J Anesthesia. . 2007;54(3):171–175
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4. Skarsard ED , Chitkara U , Krane EJ , Riley ET , Halamek LP , Dedo HH . The OOPS procedure (operation on placental support): in utero airway management of the fetus with prenatally diagnosed tracheal obstruction . J Pediatr Surg. . 1996;31(6):826–828
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5. Leichty KW , Crombleholme TM , Flake AW , et al.   Intrapartum airway management for giant fetal neck masses: the EXIT (ex utero intrapartum treatment) procedure . Am J Obstet Gynecol. . 1997;177(4):870–874
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6. Shih GH , Boyd GL , Vincent RD , Long GW , Hauth JC , Georgeson KE . The EXIT procedure facilitates delivery of an infant with a pretracheal teratoma . Anesthesiology . 1998;89(6):1573–1575
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7. Lymphatic malformations. Lymph Notes.com. http://lymphnotes.com/article.php/id/250. Accessed July 8, 2009.
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8. Lymphatic malformation  . Sick Kids . http://www.sickkids.ca/craniofacial/what-do-we/vascular-anomalies.vascular-malformations/lymphatic%20maleformations/index.html Accessed July 30, 2009.
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9. Giguere CM , Bauman NM , Smith RJ . New treatment options for lymphangioma in infants and children . Ann Otol Rhino Laryngol . 2002;111(12Pt1):1066–1075
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10. Lymphatic malformation  . University of Virginia Health System . http://www.healthsystem.virginia.edu/uvahealth/peds_ent/lymphmalform.cfm Accessed July 30, 2009.
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11. Rahbar R , Vogel A , Myers LB , et al.   Fetal surgery in otolaryngology: a new era in the diagnosis and management of fetal airway obstruction because of advances in prenatal imaging . Arch Otolaryngol Head Neck Surg. . 2005;131(5):393–398
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12. Howell LJ , Burns KM , Lenghetti E , Kerr JC , Harkins LS . Management of fetal airway obstruction: an innovative strategy . MCN Am J Matern Child Nurs. . 2002;27(4):238–243
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13. Rothrock JC . Patient and environmental safety . In:  Rothrock JC editors. Alexander's Care of the Patient in Surgery . 13th ed.. St Louis, MO: Mosby; 2007;p. 23–31
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15. DeLamar LH . Anesthesia . In:  Rothrock JC editors. Alexander's Care of the Patient in Surgery . 13th ed.. St Louis, MO: Mosby; 2007;p. 111–114 125.
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16. Luzzatto C , Midrio P , Tchaprassian Z , Guglielmi M . Sclerosing treatment of lymphangiomas with OK-432 . Arch Dis Child . 2000;82(4):316–318
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17. OK-432 and the treatment of lymphatic malformations. National Organization of Vascular Anomalies . http//www.novanews.org/OK-432.htm Accessed July 26, 2009.
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18. Noah MM , Norton Me , Sandberg P , Esakoff T , Farrell J , Albanese CT . Short-term maternal outcomes that are associated with the EXIT procedure, as compared with cesarean delivery . Am J Obstet Gynecol. . 2002;186(4):773–777
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