Seminars in Pediatric Surgery 23 (2014) 233–237
Contents lists available at ScienceDirect
Seminars in Pediatric Surgery journal homepage: www.elsevier.com/locate/sempedsurg
Perioperative care of the vascular anomaly patient Carol Chute, RN, MS, APRN, PPCNP-BCa,n, Beth Steinb, Mary Beth Sylvia, RN, MS, FNP-BCc, Erin Spera, RN, MS, CPNPc a
Hemangioma and Vascular Malformations Center, Cincinnati Children's Hospital Medical Center, MLC 2023, 3333 Burnet Avenue, Cincinnati, Ohio 45229 Division of Home Care Services, Cincinnati Children's Medical Center, Cincinnati, Ohio c Vascular Anomalies Center, Boston Children's Hospital, Boston, Massachusetts b
a r t i c l e in fo
a b s t r a c t
Keywords: Vascular anomalies Anticoagulation Surgical drains Wound care Compression garment
Patients with vascular anomalies present specific and unique challenges to providers of their postoperative care. Vascular anomalies can range from localized solitary lesions to diffuse lesions with vessel malformations and associated soft tissue, muscle, organ, and bone involvement. Perioperative issues for these patients can be complicated and include coagulopathies requiring anticoagulation, the need for postoperative surgical drains and specialized wound care, and the use of compression garments to maintain the desired postoperative result. This article will address these specific concerns for patients with vascular anomalies in order to create a framework for consistent and appropriate perioperative care. & 2014 Published by Elsevier Inc.
Patients with vascular anomalies can present with unique perioperative needs related to the particular nature and characteristics of their vascular tumor or malformation. Preoperative evaluation may require assessment and management of associated coagulopathy and co-morbidities. Coordination of multiple services including the potential need for preoperative interventional radiology procedures such as caval filter placement or embolization is often essential. Of particular interest during the perioperative period is the management of anticoagulation as well as postoperative surgical drains, wound care, and use of compression garments. These issues will be discussed in order to create a framework for pertinent perioperative care specific to patients with vascular anomalies.
Perioperative anticoagulation Patients with slow-flow vascular malformations are at an increased risk for hematologic complications, which include both bleeding and clotting. Coagulopathy affecting both hemostasis (control of bleeding) and thrombosis (regulation of blood clotting) occurs when blood stagnates in abnormal slow-flow vessels, thereby activating the coagulation system. This results in a process known as localized intravascular coagulopathy (LIC) marked by low fibrinogen, elevated D-dimer, and mild thrombocytopenia, n
Corresponding author. E-mail address: [email protected] (C. Chute).
http://dx.doi.org/10.1053/j.sempedsurg.2014.07.007 1055-8586/& 2014 Published by Elsevier Inc.
which can progress into disseminated intravascular coagulopathy (DIC) with consumption of coagulation factors.1 The etiology of coagulopathy is multifactorial. Abnormal endothelial lining leads to interactions with blood products that initiate the coagulation system. Due to variations in channel size and structural abnormalities, flow abnormalities can occur resulting in local pooling of blood and stasis that may further damage the endothelium and activate the coagulation process.2 The risk of complications secondary to coagulopathy is increased after surgical or invasive radiological procedures involving many vascular malformations. Identification of high-risk patients prior to procedures will help the team to prepare these patients in order to decrease their risks for hematologic complications. Patients at high risk for hematologic complications include those with venous malformations, lymphatic malformations, and combined malformations, such as CLOVES and Klippel–Trenaunay (capillary lymphaticovenous malformation) syndromes. These are slow-flow lesions with abnormal venous anatomy and a lymphatic component that are prone to hemorrhagic as well as thrombotic events. To identify which patients with these diagnoses are at risk for hematologic complications, a complete preoperative hematologic evaluation is necessary prior to considering any invasive procedure. This includes blood work, which may differ by institution but at a minimum should include CBC, PT, PTT, D-dimer, and fibrinogen. Obtaining an antiphospholipid antibody panel, thrombotic profile (protein S, protein C, antithrombin III, plasminogen, and plasminogen activator inhibitor), and thrombotic polymorphism panel (factor V Leiden, prothrombin 20210, PAI-I genotype, and MTHFR genotype) should also be considered.
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The patients identified as being at high risk for complications based on their laboratory results, extent of the lesion, or the presence of large ectatic vessels are prepared by initiation of lowmolecular-weight heparin (LMWH) at prophylactic (0.5 mg/kg/ dose; maximum dose of 60 mg/dose) or treatment dose (1.0 mg/ kg/dose) once or twice daily. The LMWH is usually given for 14 days prior to the invasive procedure and should be stopped 12– 24 h prior to the procedure. LMWH is generally restarted 12–24 h following the procedure depending on the extent of postoperative bleeding and the expected period of inactivity. It should be administered for 14 days following the procedure or until the patient returns to reasonable activity, whichever is longer. This will help to reduce the risk of clotting events even in patients with only mildly elevated D-dimers or with normal fibrinogens.3 Careful planning should be done prior to the procedure, including holding blood products for the procedure and communicating the risks and plan for management with the anesthesia provider. Patients with high D-dimers and/or a low fibrinogen may benefit from cryoprecipitate dripped in slowly throughout the procedure to decrease the risk of bleeding/clotting. For those highest risk patients, it is important to have other blood products on hold or ready, including packed red blood cells, FFP, and platelets. Recombinant factor 7 (NOVO seven) should be available for emergency use if the patient has extensive, uncontrolled bleeding during the procedure. Some patients with extensive lesions and chronic pain can see an improvement in their pain with the use of daily anticoagulation therapy for extended periods of time. Coumadin and vitamin K antagonists do not seem to be as effective with this group of patients, so LMWH is the therapy of choice. Dosing will be at a prophylactic level of 0.5 mg/kg/dose once or twice daily. If the patient is on long-term therapy with LMWH, it is important to check anti-factor Xa levels regularly with a goal of o 0.5 units/ml for prophylaxis. This level needs to be drawn 4–6 h following a dose of LMWH. These patients will also need regular blood work, including CBC and liver function tests. A yearly DEXA scan should be performed for chronic use of LMWH secondary to the potential risk of osteopenia. Novel oral anticoagulants that directly target factor Xa are being used in adults with some evidence that they help with coagulopathy associated with vascular lesions similar to the effect of LMWH. Studies will need to be performed in children to prove the efficacy and elucidate the side-effect profile of oral agents prior to changing these recommendations to use oral agents in place of LMWH.4 Patients identified as high risk may need to be admitted overnight after undergoing sclerotherapy. Sclerosing agents can cause red cell hemolysis resulting in hemoglobinuria, which can damage the kidneys. Intravenous fluids, running at 1.5 times the maintenance rate, are recommended for adequate flushing. If the urine is red post-procedure, sodium bicarbonate is added to the intravenous fluid to alkalinize the urine. Patients are encouraged to drink larger than normal amounts of fluids for several days following the procedure to keep the urine dilute. Patients with large, ectatic vessels may undergo preoperative embolization of the large vessels or may need extra precautions during surgery. They often will receive a single dose of LMWH during the procedure to decrease the risk of clotting or bleeding. They may also have the extremities that are not directly involved in the surgical procedure compressed with wraps throughout the procedure to decrease the risk of thrombus formation. Careful evaluation and planning for patients identified as high risk will help to make invasive procedures safer and decrease the number of hematologic complications. A team of specialists who are experts in the care of individuals with vascular anomalies is best prepared to provide knowledgeable and appropriate care for these
patients. Severe and fatal pulmonary emboli have occurred even at our experienced centers, where we have learned to be very proactive about screening and prevention.
Postoperative surgical drains Patients with vascular anomalies who undergo surgical resection of their lesions may require intraoperative placement of a surgical drain to evacuate dead space and drain blood and serous fluid from the surgical area during the postoperative period. Surgical drain systems can be open or closed and active or passive. Active, closed surgical drains, such as Jackson–Pratts drains and Blakes drains, use negative pressure to remove accumulated blood and serous fluid and reduce dead space to promote healing.5 Active, closed surgical drains are particularly important in both patients with primary lymphatic anomalies and those with a combined vascular anomaly with a significant lymphatic component. Both of these types of patients have a propensity for profound lymphatic flow into the surgical field postoperatively. If not well drained, this can result in fluid collections that contribute to swelling of the surgical site, which may adversely affect the integrity of a closed surgical wound. A single drain may be used if the surgical area is localized, while multiple drains may be necessary when the resection is extensive. Initially, the fluid draining through the surgical drain will be sanguineous, but the fluid will slowly become more serosanguineous over time. The amount of drainage in the immediate postoperative period before the patient becomes more ambulatory may be minimal or reduced, but patients and families are counseled to expect an increase in drainage as the patient becomes more mobile. Drainage can range from minimal amounts in patients with non-lymphatic vascular anomalies to hundreds of milliliters drained in each 24-h period for patients with an associated lymphatic component. The amount of drainage will gradually decrease over time until it slows enough for the body to reabsorb the fluid on its own. Patients with surgically resected vascular anomalies, particularly those with a significant lymphatic component, may need to have the drain in place for weeks to months. Patients and families are advised to empty the drainage bulb as often as it appears nearly full or at least once every 24 h and to measure and record the drainage amount. A small dressing may cover the drain tubing insertion site. This dressing may be changed either once daily or when soiled. The patient and family may opt not to cover the insertion site with a dressing if they are comfortable with this option and there is no drainage around the site. The drain tubing should be milked (stripped) on a regular basis in order to remove any small clots or tissue debris that may prevent the fluid from draining freely. If there is a decrease in fluid draining through the tubing or an increase in leakage of fluid around the tubing at the insertion site, this may be a sign that the tubing is blocked and requires milking. Patients may shower and infants may have a sponge bath with the surgical drain in place. Patients who like to swim may continue to do so in clean, chlorinated swimming pools when they have recovered to the point where the surgical wound is sufficiently healed and they are ready to return to more normal activities. With both bathing and swimming, a dab of antibiotic ointment should be placed around the drain insertion site to prevent water from entering the site. Patients and families are advised to report drainage amounts calculated in 24-h increments every 2 or 3 days so that a decision can be made when the drain(s) can be removed. Generally, if the output drops to 20 cm3 or less every 24 h for a period of 4 or
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5 days, the drain can be removed. The drain can be removed by a local physician, an adult patient, or a parent who is comfortable with the method of removal. If the securing suture is not tight, most families can cut this on their own. They should be reminded to take the drain off suction before removing it. Following removal of the drain, antibiotic ointment should be applied to the opening followed by a small dressing. The opening will close within a few days, no longer require a dressing, and heal by secondary intention. The possible complications of the use of a surgical drain postoperatively in vascular anomalies patients include local discomfort at the insertion site, infection, and accidental removal of the drain. Rarely, patients with surgical drains develop significant bleeding, breakage of the drain tubing, difficulty in removal of the drain, pressure necrosis and visceral perforation caused by chronically indwelling drains, or a cosmetically unpleasing scar after removal of the drain.6 Local discomfort at the insertion site is a common complaint but rarely requires use of pain medication. The risk of postoperative infection can be increased due to retrograde bacterial migration into a collection of serosanguineous fluid that acts as a culture medium for the development of infection.7 While minor erythema is common at the drain insertion site, patients and families are instructed to check for worsening erythema, fever, malaise, and increased pain at the drain insertion site as possible signs of infection. The risk of accidental removal of the drain can be lessened by instructing families to coil the tubing and pinning the plastic loop at the top of the drainage bulb to clothing. While the care of postoperative surgical drains can be challenging to patients and families, most become comfortable with the process with good postoperative teaching and experience. The benefits of surgical drains in maintaining the integrity of the surgical incision as well as in reducing postoperative discomfort associated with swelling and fluid accumulation are well known and enhance the surgical outcome.
Postoperative wound care Postoperative wound management of vascular malformations can be quite challenging. Given that the involved and/or underlying vasculature and soft tissue may be abnormal, it is not uncommon for excisional wound beds to be compromised. The extent to which there may be wound healing difficulties can depend on the location of the malformation, size of the lesion, and the amount of skin that is available to close over the residual defect. Another factor may be the pooling of residual fluids, such as lymphatic fluid and blood, under the skin. If drains are not placed or not placed in the proper location, fluids can push against a possibly already compromised incisional closure and cause the site to open in 1 or more places.8 Another early catalyst for wound compromise is pressure on the healing wound bed. For large surgical wounds, it is imperative in the early postoperative days to position patients off of the affected area as much as possible. If patients are left to lie on the operative site, vascular compromise causing early necrosis can be seen in surgical flaps. Initially, the area of necrosis appears dusky with eventual death of the tissue resulting in an open incisional wound. Duskiness or early necrosis of the overlying skin can also be seen in patients whose large surgical flaps are covered with occlusive dressings. Blisters can form, particularly if there is any fluid collecting on the surface of the wound bed from weak or open areas in the suture line. Swelling of tissues under adhesive dressings creates lateral shear forces on the epithelium that may already have compromised blood supply. After seeing wound breakdown in several patients with occlusive dressings over time,
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Fig. 1. Post surgical wound with superficial open regions. (Color version of figure is available online.)
our team stopped using these to cover large incisional sites. We have seen a far lower incidence of blistering with this change in practice. Once a surgical wound has been compromised, the area is treated with a stepped approach. In the early phases, where there are small, superficial open areas or scabbing, antibiotic ointment and a gauze dressing will suffice (Figure 1). If the wound evolves and becomes deeper, moist to dry gauze dressings may help to clean the wound and promote healing. Other wound care options include petroleum-based dressings, such as Xeroforms (Figure 2). Depending on the size of the wound, it may take several weeks to months for secondary healing and reapproximation of the wound edges.8 Postoperative wounds in patients with vascular anomalies tend to heal slowly. Often the greater the lymphatic involvement, the slower the healing proceeds. For some larger wounds with a significant lymphatic component, healing under traditional dressings will be unsuccessful. Particularly for larger abdominal, pelvic, and lower extremity wounds, vacuum-assisted closure (V.A.C.s) dressings are often utilized to control the overproduction of lymphatic fluid and blood and keep the wound beds clean.9 V.A.C. dressings are utilized in combination with operative debridement techniques to remove eschar and debris in the wound bed and to promote granulation of the wound (Figures 3 and 4). Patients with these larger defects may need to be hospitalized for longer periods of time and are at greater risk for secondary infections and other complications. Surgical debulking and resection of vascular malformations of the genital and perineal regions generally lead to better healing. In the event of an open wound postoperatively, moist to dry loose gauze dressings tend to heal these areas in a few weeks.8 Patients with lymphatic anomalies often develop lymphatic vesicles. This phenomenon is most often seen in areas of capillary staining but is not limited to these areas. The underlying lymphatic malformation forms channels from the lymphatic cysts through the overlying skin surface that allow lymphatic fluid to drain or leak through the skin. The skin responds by forming scabs and/or verrucous skin masses. These manifestations can be problematic for patients who may have to wear pads to control the leakage. In addition, these channels are open avenues for microbes leading to infection. Carbon dioxide laser photoevaporation is used to ablate the epidermis and in some cases the dermis resulting in formation of scar tissue that blocks these lymphatic channels. This treatment results in open superficial wounds that may bleed and leak until healing is complete. The dressings chosen to cover these laser
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Fig. 2. Post surgical wound with scabs, which may benefit from Xeroform dressing. (Color version of figure is available online.)
wounds must not only protect the healing tissues but also provide control of the leaking fluid. First, a thin layer of Bacitracin ointment is applied directly onto the wound bed. This provides antibiotic coverage and ensures that a moist barrier is maintained which is vital to avoiding disruption or unroofing of the healing tissues. The overlying dressings should be non-adherent dressings, such as vaseline gauze and Telfas pads. The outer dressing needs to absorb leaking lymphatic fluid and blood. The dressing can be held in place, when possible, with ace wraps to avoid the use of tape that may further compromise the wound. Dressing changes should be performed daily. The inner layers may need to be soaked in soapy water to aid in removal. Educating patients and families about wound care following carbon dioxide laser photoevaporation might involve hospital admission to ensure proper education for good wound healing and control of bleeding. Healing time can vary depending on the depth of the laser treatment from 5–7 days for superficially treated areas to weeks for those areas lasered more deeply. The resulting scar is usually flat and lighter in color than the surrounding skin. For patients who are preparing to undergo surgical resection, it is important to inform them about the possible wound compromise they may face in the postoperative period. Patients who are
Fig. 4. Wound with healthy granulation tissue bed after use of vacuum-assisted closure dressing. (Color version of figure is available online.)
nearby the treating facility may be seen in office visits to help monitor the wounds and to make recommendations about how to best promote healing. Patients who live further from the treating facility may benefit by assistance of local providers. The exchange of status updates and emailed wound photographs can be an adjunct to the care of patient with surgical wounds. Although the healing process can be challenging for patients and families, thorough preoperative teaching can prepare them and reassure them that it will be a team effort until healing has occurred.
Use of compression garments
Fig. 3. Wounds with eschar prior to initiation of vacuum-assisted closure dressing. (Color version of figure is available online.)
Compression therapy is an effective and highly recommended conservative measure for patients with swelling due to abnormal venous and lymphatic return related to their vascular anomalies. One main goal of compression is to increase lymphatic return and drainage, while reducing edema and pain. Another is to maintain or improve function. Compliance is the key to positive outcomes and patient satisfaction. Choosing a method of compression that suits the individual patient can be challenging. The most common types are bandaging, garments (both ready-to-wear and custom-fitted), and pneumatic compression pumps. Access to multiple manufacturers allows for a choice of fabric selection in an effort to provide therapeutic results and maintain patient compliance and satisfaction. A therapist will discuss the style of the garment and the fabric as well as other options such as zippers, contracture seams, and comfort lining of the garment with the patient and family. Although proper fit is key, comfort is even more crucial. A garment that is not being worn is not helping. There are a multitude of options with custom-fitted garments that are measured for the individual. Circular knit seamless
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stockings are more esthetically pleasing but may be challenging to don. Flat knit fabric garments, which are thicker, are made with a seam that can be constructed in any shape or size and most often used in higher compression classes. Cut and sew garments have a seam up the back of the extremity to apply gradient compression.10 Silver tubular stockinet or garments offer an antimicrobial option for patients with recurrent skin breakdown, cellulitis, or vesicles. Stockinet can be applied under garments to spot treat areas or provide extra padding. The style of the garment is important as well. Thigh-high or chap-style garments usually do not stay up well on children due to their body shape. A waist-high, unilateral leg garment can be worn comfortably using the trunk to anchor the garment. Commercial general consumer garments, such as bike shorts or exercise undergarments, can be useful for swelling in the genital region. When considering upper torso garments for chest compression, a “onesie”-style versus a vest-style garment is best suited for infants. Zippers are of benefit for diaper changes and skin protection from friction or pinching. Many manufacturers offer a wide selection of colors and patterns that may also help to increase compliance, particularly in children. Patients may be prescribed garments with a pressure gradient of 25 mmHg or higher.11 Compression garments are generally worn during waking hours but can be worn to bed for comfort if the patient desires. Some patients prefer the break from the garment at night, while others do not feel that their swelling is well controlled at night when the garments are removed. Some patients with significant malformations even wear their garments to swim. For patients with fibrotic areas of swelling, foam bags and padding can help to soften these areas relatively quickly. For infants, compression bandaging allows a daily fit of the affected extremity and allows for fluctuations in growth and degree of edema. Padding underneath the compression bandages can be used to ensure a better circumferential wrap and skin protection. In a pediatric setting, the majority of garments may need to be custom-fitted, as a ready-to-wear pediatric line has yet to be developed. Compression pumps are an adjunct therapy used to increase venous and lymphatic fluid return and break down lymphatic fibrosis. Pumps are a great tool for those patients who do not have sufficient muscle function for femoral and lymphatic return. Pump manufacturers offer multi-chamber appliances for both upper and lower extremities as well as trunk appliances to facilitate groin and lower abdominal decongestion. Pumps usually require pre-certification and often require a trial of more conservative therapies with documented failure, before they are covered by insurance carriers. Costs can vary from $3000 to more than $10,000, so proper pump choice as well as the length of the appliance (full versus half limb) need to be thoroughly and carefully considered. Pump pressure settings are determined based on the individual patient's needs. Generally, most patients can tolerate a minimum pressure of 40–60 mmHg for use on the lower extremities.12,13
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As the options for compression therapy are as varied as the patients with vascular anomalies, care must be taken when choosing the best compression options for the patient. Providing patients with a solid rationale for what each option may accomplish as well as the possible challenges is important. As part of the team, the patient and/or family should be integrally involved in the decision-making process in order to minimize noncompliance. Open discussion regarding the purpose of the compression garment, the style of the garment, and the degree of compression should result in better patient compliance. If compression therapy is approached in this manner, success is very likely.
Summary Perioperative care of vascular anomalies patients should include consideration of anticoagulation therapy, the need for postoperative surgical drains, potential postoperative wound care challenges, and the use of compression garments to obtain the desired surgical result. When all of these factors are considered and included in perioperative planning and postoperative care, the best surgical result for the patient can more easily be achieved.
Acknowledgment Cindy Kerr, RN, MSN, CPNP, Boston Children's Hospital contributed content regarding healing of laser wounds. References 1. Dompmarti A, Archer A, Thibon P, et al. Association of localized intravascular coagulopathy with venous malformations. Arch Dermatol. 2008;144:873–877. 2. Adams D. Special considerations in vascular anomalies: hematologic management. Clin Plast Surg. 2011;38(1):153–160. 3. Mozoyer E, Enjolras O, Bisdorff A, et al. Coagulation disorders in patients with venous malformation of the limbs and trunk: a case series of 118 patients. Arch Dermatol. 2008;144:861–867. 4. Galanis T, Thomson L, Palladino M, et al. New oral anticoagulants. J Thromb Thrombolysis. 2011;31:310–320. 5. Durai R, Ng PC. Surgical vacuum drains: types, uses, and complications. AORN J. 2010;91(2):266–274. 6. Durai R, Mownah A, Ng PC. Use of drains in surgery: a review. J Perioper Pract. 2009;19(6):180–186. 7. McCarthy CM, Disa JJ, Pusic AL, et al. The effect of closed-suction drains on the incidence of local wound complications following tissue expander/implant reconstruction: a cohort study. Plast Reconstr Surg. 2007;119(7):2018–2022. 8. Mulliken JB, Burrows PE, Fishman SJ, et al. Mulliken & Young's Vascular Anomalies, Hemangiomas and Malformations. 2nd ed., New York, NY: Oxford University Press; 2013. 9. V.A.C.s Therapy for Wounds. Wound V.A.C. Therapy, Negative Pressure Wound Therapy (NPWT). N.p., n.d. Web. 24 Jan, 2014. 10. Graduated Compression Hosiery and Sock. FLA Orthopedics, Miramar, FL; 2004:19–20. 11. Blattler W, Zimmet SE. Compression therapy in venous disease. Phlebology. 2008;23(5):203–205. 〈http://www.ncbi.nlm.nih.gov/pubmed/18806201〉. 12. Ridner SH, McMahon E, Dietrich MS, et al. Home-based lymphedema treatment in patients with and without cancer related lymphedema. Oncol Nurs Forum. 2008;35(4):671–680. 13. Ehrlich A, Harrewijn A, McMahon E Living Well With Lymphedema. Lymph Notes; 2005.
Contents lists available at ScienceDirect
Seminars in Pediatric Surgery journal homepage: www.elsevier.com/locate/sempedsurg
Perioperative care of the vascular anomaly patient Carol Chute, RN, MS, APRN, PPCNP-BCa,n, Beth Steinb, Mary Beth Sylvia, RN, MS, FNP-BCc, Erin Spera, RN, MS, CPNPc a
Hemangioma and Vascular Malformations Center, Cincinnati Children's Hospital Medical Center, MLC 2023, 3333 Burnet Avenue, Cincinnati, Ohio 45229 Division of Home Care Services, Cincinnati Children's Medical Center, Cincinnati, Ohio c Vascular Anomalies Center, Boston Children's Hospital, Boston, Massachusetts b
a r t i c l e in fo
a b s t r a c t
Keywords: Vascular anomalies Anticoagulation Surgical drains Wound care Compression garment
Patients with vascular anomalies present specific and unique challenges to providers of their postoperative care. Vascular anomalies can range from localized solitary lesions to diffuse lesions with vessel malformations and associated soft tissue, muscle, organ, and bone involvement. Perioperative issues for these patients can be complicated and include coagulopathies requiring anticoagulation, the need for postoperative surgical drains and specialized wound care, and the use of compression garments to maintain the desired postoperative result. This article will address these specific concerns for patients with vascular anomalies in order to create a framework for consistent and appropriate perioperative care. & 2014 Published by Elsevier Inc.
Patients with vascular anomalies can present with unique perioperative needs related to the particular nature and characteristics of their vascular tumor or malformation. Preoperative evaluation may require assessment and management of associated coagulopathy and co-morbidities. Coordination of multiple services including the potential need for preoperative interventional radiology procedures such as caval filter placement or embolization is often essential. Of particular interest during the perioperative period is the management of anticoagulation as well as postoperative surgical drains, wound care, and use of compression garments. These issues will be discussed in order to create a framework for pertinent perioperative care specific to patients with vascular anomalies.
Perioperative anticoagulation Patients with slow-flow vascular malformations are at an increased risk for hematologic complications, which include both bleeding and clotting. Coagulopathy affecting both hemostasis (control of bleeding) and thrombosis (regulation of blood clotting) occurs when blood stagnates in abnormal slow-flow vessels, thereby activating the coagulation system. This results in a process known as localized intravascular coagulopathy (LIC) marked by low fibrinogen, elevated D-dimer, and mild thrombocytopenia, n
Corresponding author. E-mail address: [email protected] (C. Chute).
http://dx.doi.org/10.1053/j.sempedsurg.2014.07.007 1055-8586/& 2014 Published by Elsevier Inc.
which can progress into disseminated intravascular coagulopathy (DIC) with consumption of coagulation factors.1 The etiology of coagulopathy is multifactorial. Abnormal endothelial lining leads to interactions with blood products that initiate the coagulation system. Due to variations in channel size and structural abnormalities, flow abnormalities can occur resulting in local pooling of blood and stasis that may further damage the endothelium and activate the coagulation process.2 The risk of complications secondary to coagulopathy is increased after surgical or invasive radiological procedures involving many vascular malformations. Identification of high-risk patients prior to procedures will help the team to prepare these patients in order to decrease their risks for hematologic complications. Patients at high risk for hematologic complications include those with venous malformations, lymphatic malformations, and combined malformations, such as CLOVES and Klippel–Trenaunay (capillary lymphaticovenous malformation) syndromes. These are slow-flow lesions with abnormal venous anatomy and a lymphatic component that are prone to hemorrhagic as well as thrombotic events. To identify which patients with these diagnoses are at risk for hematologic complications, a complete preoperative hematologic evaluation is necessary prior to considering any invasive procedure. This includes blood work, which may differ by institution but at a minimum should include CBC, PT, PTT, D-dimer, and fibrinogen. Obtaining an antiphospholipid antibody panel, thrombotic profile (protein S, protein C, antithrombin III, plasminogen, and plasminogen activator inhibitor), and thrombotic polymorphism panel (factor V Leiden, prothrombin 20210, PAI-I genotype, and MTHFR genotype) should also be considered.
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C. Chute et al. / Seminars in Pediatric Surgery 23 (2014) 233–237
The patients identified as being at high risk for complications based on their laboratory results, extent of the lesion, or the presence of large ectatic vessels are prepared by initiation of lowmolecular-weight heparin (LMWH) at prophylactic (0.5 mg/kg/ dose; maximum dose of 60 mg/dose) or treatment dose (1.0 mg/ kg/dose) once or twice daily. The LMWH is usually given for 14 days prior to the invasive procedure and should be stopped 12– 24 h prior to the procedure. LMWH is generally restarted 12–24 h following the procedure depending on the extent of postoperative bleeding and the expected period of inactivity. It should be administered for 14 days following the procedure or until the patient returns to reasonable activity, whichever is longer. This will help to reduce the risk of clotting events even in patients with only mildly elevated D-dimers or with normal fibrinogens.3 Careful planning should be done prior to the procedure, including holding blood products for the procedure and communicating the risks and plan for management with the anesthesia provider. Patients with high D-dimers and/or a low fibrinogen may benefit from cryoprecipitate dripped in slowly throughout the procedure to decrease the risk of bleeding/clotting. For those highest risk patients, it is important to have other blood products on hold or ready, including packed red blood cells, FFP, and platelets. Recombinant factor 7 (NOVO seven) should be available for emergency use if the patient has extensive, uncontrolled bleeding during the procedure. Some patients with extensive lesions and chronic pain can see an improvement in their pain with the use of daily anticoagulation therapy for extended periods of time. Coumadin and vitamin K antagonists do not seem to be as effective with this group of patients, so LMWH is the therapy of choice. Dosing will be at a prophylactic level of 0.5 mg/kg/dose once or twice daily. If the patient is on long-term therapy with LMWH, it is important to check anti-factor Xa levels regularly with a goal of o 0.5 units/ml for prophylaxis. This level needs to be drawn 4–6 h following a dose of LMWH. These patients will also need regular blood work, including CBC and liver function tests. A yearly DEXA scan should be performed for chronic use of LMWH secondary to the potential risk of osteopenia. Novel oral anticoagulants that directly target factor Xa are being used in adults with some evidence that they help with coagulopathy associated with vascular lesions similar to the effect of LMWH. Studies will need to be performed in children to prove the efficacy and elucidate the side-effect profile of oral agents prior to changing these recommendations to use oral agents in place of LMWH.4 Patients identified as high risk may need to be admitted overnight after undergoing sclerotherapy. Sclerosing agents can cause red cell hemolysis resulting in hemoglobinuria, which can damage the kidneys. Intravenous fluids, running at 1.5 times the maintenance rate, are recommended for adequate flushing. If the urine is red post-procedure, sodium bicarbonate is added to the intravenous fluid to alkalinize the urine. Patients are encouraged to drink larger than normal amounts of fluids for several days following the procedure to keep the urine dilute. Patients with large, ectatic vessels may undergo preoperative embolization of the large vessels or may need extra precautions during surgery. They often will receive a single dose of LMWH during the procedure to decrease the risk of clotting or bleeding. They may also have the extremities that are not directly involved in the surgical procedure compressed with wraps throughout the procedure to decrease the risk of thrombus formation. Careful evaluation and planning for patients identified as high risk will help to make invasive procedures safer and decrease the number of hematologic complications. A team of specialists who are experts in the care of individuals with vascular anomalies is best prepared to provide knowledgeable and appropriate care for these
patients. Severe and fatal pulmonary emboli have occurred even at our experienced centers, where we have learned to be very proactive about screening and prevention.
Postoperative surgical drains Patients with vascular anomalies who undergo surgical resection of their lesions may require intraoperative placement of a surgical drain to evacuate dead space and drain blood and serous fluid from the surgical area during the postoperative period. Surgical drain systems can be open or closed and active or passive. Active, closed surgical drains, such as Jackson–Pratts drains and Blakes drains, use negative pressure to remove accumulated blood and serous fluid and reduce dead space to promote healing.5 Active, closed surgical drains are particularly important in both patients with primary lymphatic anomalies and those with a combined vascular anomaly with a significant lymphatic component. Both of these types of patients have a propensity for profound lymphatic flow into the surgical field postoperatively. If not well drained, this can result in fluid collections that contribute to swelling of the surgical site, which may adversely affect the integrity of a closed surgical wound. A single drain may be used if the surgical area is localized, while multiple drains may be necessary when the resection is extensive. Initially, the fluid draining through the surgical drain will be sanguineous, but the fluid will slowly become more serosanguineous over time. The amount of drainage in the immediate postoperative period before the patient becomes more ambulatory may be minimal or reduced, but patients and families are counseled to expect an increase in drainage as the patient becomes more mobile. Drainage can range from minimal amounts in patients with non-lymphatic vascular anomalies to hundreds of milliliters drained in each 24-h period for patients with an associated lymphatic component. The amount of drainage will gradually decrease over time until it slows enough for the body to reabsorb the fluid on its own. Patients with surgically resected vascular anomalies, particularly those with a significant lymphatic component, may need to have the drain in place for weeks to months. Patients and families are advised to empty the drainage bulb as often as it appears nearly full or at least once every 24 h and to measure and record the drainage amount. A small dressing may cover the drain tubing insertion site. This dressing may be changed either once daily or when soiled. The patient and family may opt not to cover the insertion site with a dressing if they are comfortable with this option and there is no drainage around the site. The drain tubing should be milked (stripped) on a regular basis in order to remove any small clots or tissue debris that may prevent the fluid from draining freely. If there is a decrease in fluid draining through the tubing or an increase in leakage of fluid around the tubing at the insertion site, this may be a sign that the tubing is blocked and requires milking. Patients may shower and infants may have a sponge bath with the surgical drain in place. Patients who like to swim may continue to do so in clean, chlorinated swimming pools when they have recovered to the point where the surgical wound is sufficiently healed and they are ready to return to more normal activities. With both bathing and swimming, a dab of antibiotic ointment should be placed around the drain insertion site to prevent water from entering the site. Patients and families are advised to report drainage amounts calculated in 24-h increments every 2 or 3 days so that a decision can be made when the drain(s) can be removed. Generally, if the output drops to 20 cm3 or less every 24 h for a period of 4 or
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5 days, the drain can be removed. The drain can be removed by a local physician, an adult patient, or a parent who is comfortable with the method of removal. If the securing suture is not tight, most families can cut this on their own. They should be reminded to take the drain off suction before removing it. Following removal of the drain, antibiotic ointment should be applied to the opening followed by a small dressing. The opening will close within a few days, no longer require a dressing, and heal by secondary intention. The possible complications of the use of a surgical drain postoperatively in vascular anomalies patients include local discomfort at the insertion site, infection, and accidental removal of the drain. Rarely, patients with surgical drains develop significant bleeding, breakage of the drain tubing, difficulty in removal of the drain, pressure necrosis and visceral perforation caused by chronically indwelling drains, or a cosmetically unpleasing scar after removal of the drain.6 Local discomfort at the insertion site is a common complaint but rarely requires use of pain medication. The risk of postoperative infection can be increased due to retrograde bacterial migration into a collection of serosanguineous fluid that acts as a culture medium for the development of infection.7 While minor erythema is common at the drain insertion site, patients and families are instructed to check for worsening erythema, fever, malaise, and increased pain at the drain insertion site as possible signs of infection. The risk of accidental removal of the drain can be lessened by instructing families to coil the tubing and pinning the plastic loop at the top of the drainage bulb to clothing. While the care of postoperative surgical drains can be challenging to patients and families, most become comfortable with the process with good postoperative teaching and experience. The benefits of surgical drains in maintaining the integrity of the surgical incision as well as in reducing postoperative discomfort associated with swelling and fluid accumulation are well known and enhance the surgical outcome.
Postoperative wound care Postoperative wound management of vascular malformations can be quite challenging. Given that the involved and/or underlying vasculature and soft tissue may be abnormal, it is not uncommon for excisional wound beds to be compromised. The extent to which there may be wound healing difficulties can depend on the location of the malformation, size of the lesion, and the amount of skin that is available to close over the residual defect. Another factor may be the pooling of residual fluids, such as lymphatic fluid and blood, under the skin. If drains are not placed or not placed in the proper location, fluids can push against a possibly already compromised incisional closure and cause the site to open in 1 or more places.8 Another early catalyst for wound compromise is pressure on the healing wound bed. For large surgical wounds, it is imperative in the early postoperative days to position patients off of the affected area as much as possible. If patients are left to lie on the operative site, vascular compromise causing early necrosis can be seen in surgical flaps. Initially, the area of necrosis appears dusky with eventual death of the tissue resulting in an open incisional wound. Duskiness or early necrosis of the overlying skin can also be seen in patients whose large surgical flaps are covered with occlusive dressings. Blisters can form, particularly if there is any fluid collecting on the surface of the wound bed from weak or open areas in the suture line. Swelling of tissues under adhesive dressings creates lateral shear forces on the epithelium that may already have compromised blood supply. After seeing wound breakdown in several patients with occlusive dressings over time,
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Fig. 1. Post surgical wound with superficial open regions. (Color version of figure is available online.)
our team stopped using these to cover large incisional sites. We have seen a far lower incidence of blistering with this change in practice. Once a surgical wound has been compromised, the area is treated with a stepped approach. In the early phases, where there are small, superficial open areas or scabbing, antibiotic ointment and a gauze dressing will suffice (Figure 1). If the wound evolves and becomes deeper, moist to dry gauze dressings may help to clean the wound and promote healing. Other wound care options include petroleum-based dressings, such as Xeroforms (Figure 2). Depending on the size of the wound, it may take several weeks to months for secondary healing and reapproximation of the wound edges.8 Postoperative wounds in patients with vascular anomalies tend to heal slowly. Often the greater the lymphatic involvement, the slower the healing proceeds. For some larger wounds with a significant lymphatic component, healing under traditional dressings will be unsuccessful. Particularly for larger abdominal, pelvic, and lower extremity wounds, vacuum-assisted closure (V.A.C.s) dressings are often utilized to control the overproduction of lymphatic fluid and blood and keep the wound beds clean.9 V.A.C. dressings are utilized in combination with operative debridement techniques to remove eschar and debris in the wound bed and to promote granulation of the wound (Figures 3 and 4). Patients with these larger defects may need to be hospitalized for longer periods of time and are at greater risk for secondary infections and other complications. Surgical debulking and resection of vascular malformations of the genital and perineal regions generally lead to better healing. In the event of an open wound postoperatively, moist to dry loose gauze dressings tend to heal these areas in a few weeks.8 Patients with lymphatic anomalies often develop lymphatic vesicles. This phenomenon is most often seen in areas of capillary staining but is not limited to these areas. The underlying lymphatic malformation forms channels from the lymphatic cysts through the overlying skin surface that allow lymphatic fluid to drain or leak through the skin. The skin responds by forming scabs and/or verrucous skin masses. These manifestations can be problematic for patients who may have to wear pads to control the leakage. In addition, these channels are open avenues for microbes leading to infection. Carbon dioxide laser photoevaporation is used to ablate the epidermis and in some cases the dermis resulting in formation of scar tissue that blocks these lymphatic channels. This treatment results in open superficial wounds that may bleed and leak until healing is complete. The dressings chosen to cover these laser
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Fig. 2. Post surgical wound with scabs, which may benefit from Xeroform dressing. (Color version of figure is available online.)
wounds must not only protect the healing tissues but also provide control of the leaking fluid. First, a thin layer of Bacitracin ointment is applied directly onto the wound bed. This provides antibiotic coverage and ensures that a moist barrier is maintained which is vital to avoiding disruption or unroofing of the healing tissues. The overlying dressings should be non-adherent dressings, such as vaseline gauze and Telfas pads. The outer dressing needs to absorb leaking lymphatic fluid and blood. The dressing can be held in place, when possible, with ace wraps to avoid the use of tape that may further compromise the wound. Dressing changes should be performed daily. The inner layers may need to be soaked in soapy water to aid in removal. Educating patients and families about wound care following carbon dioxide laser photoevaporation might involve hospital admission to ensure proper education for good wound healing and control of bleeding. Healing time can vary depending on the depth of the laser treatment from 5–7 days for superficially treated areas to weeks for those areas lasered more deeply. The resulting scar is usually flat and lighter in color than the surrounding skin. For patients who are preparing to undergo surgical resection, it is important to inform them about the possible wound compromise they may face in the postoperative period. Patients who are
Fig. 4. Wound with healthy granulation tissue bed after use of vacuum-assisted closure dressing. (Color version of figure is available online.)
nearby the treating facility may be seen in office visits to help monitor the wounds and to make recommendations about how to best promote healing. Patients who live further from the treating facility may benefit by assistance of local providers. The exchange of status updates and emailed wound photographs can be an adjunct to the care of patient with surgical wounds. Although the healing process can be challenging for patients and families, thorough preoperative teaching can prepare them and reassure them that it will be a team effort until healing has occurred.
Use of compression garments
Fig. 3. Wounds with eschar prior to initiation of vacuum-assisted closure dressing. (Color version of figure is available online.)
Compression therapy is an effective and highly recommended conservative measure for patients with swelling due to abnormal venous and lymphatic return related to their vascular anomalies. One main goal of compression is to increase lymphatic return and drainage, while reducing edema and pain. Another is to maintain or improve function. Compliance is the key to positive outcomes and patient satisfaction. Choosing a method of compression that suits the individual patient can be challenging. The most common types are bandaging, garments (both ready-to-wear and custom-fitted), and pneumatic compression pumps. Access to multiple manufacturers allows for a choice of fabric selection in an effort to provide therapeutic results and maintain patient compliance and satisfaction. A therapist will discuss the style of the garment and the fabric as well as other options such as zippers, contracture seams, and comfort lining of the garment with the patient and family. Although proper fit is key, comfort is even more crucial. A garment that is not being worn is not helping. There are a multitude of options with custom-fitted garments that are measured for the individual. Circular knit seamless
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stockings are more esthetically pleasing but may be challenging to don. Flat knit fabric garments, which are thicker, are made with a seam that can be constructed in any shape or size and most often used in higher compression classes. Cut and sew garments have a seam up the back of the extremity to apply gradient compression.10 Silver tubular stockinet or garments offer an antimicrobial option for patients with recurrent skin breakdown, cellulitis, or vesicles. Stockinet can be applied under garments to spot treat areas or provide extra padding. The style of the garment is important as well. Thigh-high or chap-style garments usually do not stay up well on children due to their body shape. A waist-high, unilateral leg garment can be worn comfortably using the trunk to anchor the garment. Commercial general consumer garments, such as bike shorts or exercise undergarments, can be useful for swelling in the genital region. When considering upper torso garments for chest compression, a “onesie”-style versus a vest-style garment is best suited for infants. Zippers are of benefit for diaper changes and skin protection from friction or pinching. Many manufacturers offer a wide selection of colors and patterns that may also help to increase compliance, particularly in children. Patients may be prescribed garments with a pressure gradient of 25 mmHg or higher.11 Compression garments are generally worn during waking hours but can be worn to bed for comfort if the patient desires. Some patients prefer the break from the garment at night, while others do not feel that their swelling is well controlled at night when the garments are removed. Some patients with significant malformations even wear their garments to swim. For patients with fibrotic areas of swelling, foam bags and padding can help to soften these areas relatively quickly. For infants, compression bandaging allows a daily fit of the affected extremity and allows for fluctuations in growth and degree of edema. Padding underneath the compression bandages can be used to ensure a better circumferential wrap and skin protection. In a pediatric setting, the majority of garments may need to be custom-fitted, as a ready-to-wear pediatric line has yet to be developed. Compression pumps are an adjunct therapy used to increase venous and lymphatic fluid return and break down lymphatic fibrosis. Pumps are a great tool for those patients who do not have sufficient muscle function for femoral and lymphatic return. Pump manufacturers offer multi-chamber appliances for both upper and lower extremities as well as trunk appliances to facilitate groin and lower abdominal decongestion. Pumps usually require pre-certification and often require a trial of more conservative therapies with documented failure, before they are covered by insurance carriers. Costs can vary from $3000 to more than $10,000, so proper pump choice as well as the length of the appliance (full versus half limb) need to be thoroughly and carefully considered. Pump pressure settings are determined based on the individual patient's needs. Generally, most patients can tolerate a minimum pressure of 40–60 mmHg for use on the lower extremities.12,13
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As the options for compression therapy are as varied as the patients with vascular anomalies, care must be taken when choosing the best compression options for the patient. Providing patients with a solid rationale for what each option may accomplish as well as the possible challenges is important. As part of the team, the patient and/or family should be integrally involved in the decision-making process in order to minimize noncompliance. Open discussion regarding the purpose of the compression garment, the style of the garment, and the degree of compression should result in better patient compliance. If compression therapy is approached in this manner, success is very likely.
Summary Perioperative care of vascular anomalies patients should include consideration of anticoagulation therapy, the need for postoperative surgical drains, potential postoperative wound care challenges, and the use of compression garments to obtain the desired surgical result. When all of these factors are considered and included in perioperative planning and postoperative care, the best surgical result for the patient can more easily be achieved.
Acknowledgment Cindy Kerr, RN, MSN, CPNP, Boston Children's Hospital contributed content regarding healing of laser wounds. References 1. Dompmarti A, Archer A, Thibon P, et al. Association of localized intravascular coagulopathy with venous malformations. Arch Dermatol. 2008;144:873–877. 2. Adams D. Special considerations in vascular anomalies: hematologic management. Clin Plast Surg. 2011;38(1):153–160. 3. Mozoyer E, Enjolras O, Bisdorff A, et al. Coagulation disorders in patients with venous malformation of the limbs and trunk: a case series of 118 patients. Arch Dermatol. 2008;144:861–867. 4. Galanis T, Thomson L, Palladino M, et al. New oral anticoagulants. J Thromb Thrombolysis. 2011;31:310–320. 5. Durai R, Ng PC. Surgical vacuum drains: types, uses, and complications. AORN J. 2010;91(2):266–274. 6. Durai R, Mownah A, Ng PC. Use of drains in surgery: a review. J Perioper Pract. 2009;19(6):180–186. 7. McCarthy CM, Disa JJ, Pusic AL, et al. The effect of closed-suction drains on the incidence of local wound complications following tissue expander/implant reconstruction: a cohort study. Plast Reconstr Surg. 2007;119(7):2018–2022. 8. Mulliken JB, Burrows PE, Fishman SJ, et al. Mulliken & Young's Vascular Anomalies, Hemangiomas and Malformations. 2nd ed., New York, NY: Oxford University Press; 2013. 9. V.A.C.s Therapy for Wounds. Wound V.A.C. Therapy, Negative Pressure Wound Therapy (NPWT). N.p., n.d. Web. 24 Jan, 2014. 10. Graduated Compression Hosiery and Sock. FLA Orthopedics, Miramar, FL; 2004:19–20. 11. Blattler W, Zimmet SE. Compression therapy in venous disease. Phlebology. 2008;23(5):203–205. 〈http://www.ncbi.nlm.nih.gov/pubmed/18806201〉. 12. Ridner SH, McMahon E, Dietrich MS, et al. Home-based lymphedema treatment in patients with and without cancer related lymphedema. Oncol Nurs Forum. 2008;35(4):671–680. 13. Ehrlich A, Harrewijn A, McMahon E Living Well With Lymphedema. Lymph Notes; 2005.
