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Transfusion-Free Calvarial Vault Reconstruction Using Multimodal Blood Conservation Strategies in Two Pediatric Jehovah’s Witness Patients With Craniosynostosis

Reddy, Srijaya K. MD, MBA*†; Swink, Jonathan M. CAA*†; Rogers, Gary F. MD, JD, MBA, MPH†‡; Myseros, John S. MD*†; Keating, Robert F. MD*†; Santos, Domiciano N. MD*†

doi: 10.1213/XAA.0000000000000334
Case Reports: Case Report
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Transfusion-free surgery for the Jehovah’s Witness patient has been described, but there are few reports in pediatric patients undergoing major elective surgery. We present 2 patients of the Jehovah’s Witness faith affected with craniosynostosis who underwent transfusion-free calvarial vault reconstructions using several blood conservation approaches, including the use of antifibrinolytic ε-aminocaproic acid.

From the *Division of Anesthesiology, Children’s National Health System, Washington, DC; The George Washington University School of Medicine and Health Sciences, Washington, DC; and Division of Plastic and Reconstructive Surgery, Children’s National Health System, Washington, DC.

Accepted for publication February 22, 2016.

Funding: None.

The authors declare no conflicts of interest.

Address correspondence to Srijaya K. Reddy, MD, MBA, Division of Anesthesiology, Children’s National Health System, 111 Michigan Ave. NW, Washington, DC 20010. Address e-mail to sreddy@cnmc.org.

Craniofacial reconstruction surgery in the Jehovah’s Witness patient presents a considerable challenge to the craniofacial team. Traditionally, major elective reconstruction surgery has been avoided in these patients, especially in the pediatric population, because blood loss can be excessive, sometimes exceeding the patient’s circulating blood volume.1 Efforts to minimize blood loss and a multidisciplinary approach to planning and management are crucial to a successful outcome that honors the religious beliefs of Jehovah’s Witness patients.

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Consent for Publication

The institutional review board at Children’s National Health System reviewed this case report and gave permission for the authors to publish the report. In addition, the patients and families were contacted, and written permission to publish this case report was obtained.

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CASE DESCRIPTION

Case 1

An ex-35-week 12-month-old, 7.4-kg Jehovah’s Witness infant with a history of Chiari malformation with hydrocephalus and α-thalassemia trait presented with bilateral coronal and lambdoidal craniosynostosis. The traditional treatment, total calvarial vault reconstruction, was recommended to avoid the development of intracranial hypertension. After extensive consultation with the family and hematologist, the craniofacial team elected to proceed with the shared understanding that transfusion would occur only if necessary. The patient received iron supplementation and erythropoietin for 6 weeks before surgery, resulting in an increase in preoperative hematocrit concentration from 33.3% to 37.7%.

After mask induction of anesthesia with sevoflurane and nitrous oxide and placement of a peripheral IV, endotracheal intubation was performed. Arterial and central venous access was obtained, and acute normovolemic hemodilution (ANH) was performed by removing 100 mL of autologous blood kept in continuous circulation with the patient in 50-mL increments. Intravascular volume was replaced 1:1 with 5% albumin after each 50-mL aliquot of blood was removed, resulting in a posthemodilution hematocrit concentration of 24.6%. Antifibrinolytic therapy with ε-aminocaproic acid (EACA) 100 mg/kg bolus before incision followed by a 40-mg/kg/h infusion administered until the end of surgery was implemented to minimize blood loss. Maintenance of anesthesia was accomplished using 2% sevoflurane, fraction of inhaled oxygen maintained at 100%, and a total of 8 μg/kg fentanyl, and 0.3 mg/kg morphine administered throughout the case.

The surgical site was infiltrated with epinephrine 1:200,000 before incision as a vasoconstrictor. Scalp dissection was performed using unipolar electrocautery and was extended anteriorly in the subgaleal plane. As bone was removed, the bone edges were covered with bone wax to further reduce bleeding. Two-thirds of the cranial vault was remodeled during this procedure. The scope of the procedure did not differ from what would have been done for a non-Jehovah’s Witness patient with similar operative indications and objectives.

Mean arterial blood pressure (MAP) was maintained at >55 mm Hg. Fluid management included 41 mL/kg lactated Ringer’s solution and 41 mL/kg 5% albumin administered throughout the 260-minute operation; his total urine output was 150 mL. The patient was autotransfused the entire 100 mL of blood as needed during the case with an immediate postoperative hematocrit concentration of 16% and lactate concentration of 0.66. He was tracheally extubated and taken to the pediatric intensive care unit (PICU) in stable condition. His postoperative laboratories revealed elevated prothrombin time (PT) 18.8 seconds, partial thromboplastin time (PTT) 41.4 seconds, international normalized ratio (INR) 1.69, platelet counts at 139, and a lactate concentration of 0.85 mmol/L. Intraoperative blood loss was calculated to be 320 mL (43 mL/kg). Despite a hemoglobin level of 5.1 g/dL and hematocrit concentration of 15.4% on postoperative day (POD) 1, he remained hemodynamically stable. The patient was monitored closely in the PICU over the next 4 days in the event that he acutely decompensated or required urgent transfusion and was discharged home on POD 7 from the regular surgical floor. Although there were no complications postoperatively, his hospital length of stay was extended because of his profound anemia and the need for careful observation. His predischarge hemoglobin and hematocrit concentration were 6 g/dL and 20.2%, respectively. Erythropoietin and iron were continued in the postoperative period with hematocrit returning to baseline within 2 months after surgery.

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Case 2

A 3-year-old, 12.9-kg Jehovah’s Witness child with asthma presented with sagittal craniosynostosis. The patient had severe scaphocephaly, and a total calvarial vault reconstruction procedure was recommended to improve cranial shape and reduce the risk of intracranial hypertension. Using the same perioperative strategy as in the previous case, the patient received 2 weeks of erythropoietin and iron supplementation before surgery, resulting in a preoperative increase in hematocrit concentration from 31.9% to 36%.

After mask induction with sevoflurane and nitrous oxide, peripheral venous access was obtained and the patient was tracheally intubated per routine. Invasive lines were placed, and ANH was performed in a similar fashion with 150 mL autologous blood replenished 1:1 with 5% albumin. The post-ANH hematocrit concentration was 30%. Antifibrinolytic therapy with EACA and meticulous surgical hemostasis were again applied as blood conservation methods. Surgical remodeling involved two-thirds of the cranial vault, like in the previous case. Anesthesia was maintained with 2% sevoflurane, 6 μg/kg fentanyl, 0.2 mg/kg morphine, and a remifentanil infusion titrated to maintain MAP between 55 and 60 mm Hg. In addition to 78 mL/kg lactated Ringer’s solution and 19 mL/kg 5% albumin, the entire volume of autologous blood was given back to the patient throughout the 215-minute surgery; he was tracheally extubated and transferred to the PICU. His immediate postoperative hemoglobin and hematocrit concentration were 8.6 g/dL and 26.8%, respectively, and intraoperative blood loss was calculated to be 250 mL (19 mL/kg). His postoperative laboratories included a PT 15.9 seconds, PTT 28.5 seconds, INR 1.27, platelet counts at 442, and a lactate concentration of 0.98 mmol/L. He had an uneventful postoperative course without ever requiring transfusion and was discharged home on POD 3.

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DISCUSSION

Various blood conservation modalities have been described at length for both Jehovah’s Witness patients and in pediatric craniofacial surgery. Although there are several reports of bloodless cardiopulmonary bypass and trauma cases in pediatric Jehovah’s Witness patients, to the authors’ knowledge, these are the first reported cases of transfusion-free calvarial vault reconstruction surgeries in pediatric Jehovah’s Witness patients.

The Jehovah’s Witness religion was first established in 1872, and today, there are >7 million members in >200 countries around the world. In 1945, the Watchtower Society, the governing council of the Jehovah’s Witness religion, prohibited the use of blood products, including allogeneic blood, red blood cell concentrates, white blood cells, plasma, platelets, and autologous blood that has been separated from an individual’s body.2 This decision was made based on the interpretation of several biblical passages stating that blood consumption of any creature is unforgiving and unacceptable by God.2,3 The use of other products such as albumin, recombinant human erythropoietin, immunoglobulins, and factor concentrates can be deemed acceptable by the individual. If a Jehovah’s Witness receives a transfusion against his or her own will, it is legally regarded as battery. However, when it comes to the pediatric population, the principle of parens patriae allows the clinician to make appropriate decisions in the best interest of the child.3 In both of the cases presented, a considerable attempt was made at avoiding allogeneic transfusion to respect the beliefs of the families. The multidisciplinary team agreed on specific triggers for transfusion, which included any sign of hemodynamic instability or decreased perfusion, arterial blood lactate levels higher than 1.5 mEq/L, or a hematocrit concentration <15%.

Erythropoietin, clinically available since 1985, has been shown to be effective in raising hemoglobin levels and decreasing the need for transfusion.4–6 At least 10 to 14 days of targeted erythropoietin therapy is recommended to see an appropriate increase in hematocrit.7 Modifications to dosing regimen or frequency might be necessary to reach an ideal hematocrit level. In the first case, the patient had an excellent initial response to erythropoietin, but unfortunately, the patient leveled out at approximately 37% hematocrit, which was thought to be because of his underlying α-thalassemia trait. The second patient only had 2 weeks for preoperative hematologic intervention, which resulted in a suboptimal increase in hematocrit level. Despite this, the patient tolerated the surgical procedure well and did not require postoperative erythropoietin or iron supplementation.

ANH is a low-risk technique if exercised appropriately and is fairly easy to implement. When performing ANH in a Jehovah’s Witness patient, the blood must be kept in continuity with the patient’s circulatory system at all times. Calculating a predetermined amount of blood to be removed and the exact amount of anticoagulant needed is imperative in pediatric patients because their circulating blood volume is much smaller than that of adults, and there is less room for error. ANH has been shown not only to decrease the need for blood transfusion, but also to improve coagulation status when the autologous blood is transfused back to the patient.7,8 In the cases presented, autologous blood was removed in 50-mL increments into syringes that were prefilled with Citrate Phosphate Dextrose Adenine Solution (CPDA-1) (0.14 mL per 1 mL of blood to be removed) and kept in continuity with the patient. This technique allowed for more control and flexibility in the amount of blood that was removed. In the first case presented, we initially calculated that 150 mL of blood would be the maximum allowable amount of blood to be removed. However, after the first two 50-mL aliquots of blood were removed and replaced 1:1 with 5% albumin, the patient demonstrated mild hypotension (MAP of 55 mm Hg), so we opted not to remove any additional blood. Maintaining normovolemia with intravascular volume replacement after ANH is important to maintain cardiac output and oxygen delivery.9

Lactate levels have been established as a surrogate indicator of cardiac output, oxygen delivery, and cellular perfusion during periods of acute blood loss and anemia, trauma, and during cardiopulmonary bypass.10–12 Although serum lactate as an isolated biomarker for outcome after a major surgery has minimal predictive value, low lactate levels have been shown to be highly predictive of survival for infants after cardiac surgery.13,14 In both of our patients, serum lactate levels remained normal intraoperatively and throughout the postoperative course.

Despite their religious beliefs regarding transfusion, Jehovah’s Witness patients do not have higher mortality rates after traumatic injury or surgery.15 Studies have shown that a hematocrit concentration of 20% is well tolerated in hemodynamically stable children, and mortality only increases with hemoglobin concentrations <5 g/dL.15,16 Although allogeneic blood transfusion is relatively inexpensive and easy to administer, there are notable life-threatening transfusion-related risks; it may lead to immunosuppression and can contribute to prolonged hospitalization.6,10 In light of this, avoiding transfusion and permitting lower hemoglobin levels should be considered in hemodynamically stable patients. However, both our patients were anemic and coagulopathic postoperatively, and it remains to be determined which is ultimately safer for patients: permissible anemia and coagulopathy or transfusion of blood products. Although the optimal transfusion trigger remains elusive, several studies suggest that a hemoglobin transfusion threshold of 7 g/dL is likely to be safe for most clinically stable children when using restrictive transfusion practices.17 It is important to note that the patient in our first case had an extremely low hematocrit concentration of 15.4%, and there is no literature to support that this extreme restrictive transfusion strategy in the pediatric population is safe.

EACA is a synthetic lysine analog that blocks the lysine-binding sites on plasminogen, resulting in antifibrinolytic activity through inhibition of plasmin formation.18 EACA has been shown to be effective in limiting blood loss in infants and children undergoing open heart surgery and spinal fusion.19–21 Several recent retrospective reviews have also suggested a benefit of EACA in children undergoing craniofacial surgery.22,23 EACA is associated with significantly lower calculated blood loss than in those who did not receive EACA (82 vs 106 mL/kg) in pediatric craniofacial surgery.23 Stricker et al.24 identified that an initial loading dose of 100 mg/kg EACA followed by a continuous IV infusion at 40 mg/kg/h maintains the target plasma EACA concentration (130 μg/mL) in children 6 months to 24 months old undergoing craniofacial surgery. However, the efficacy of EACA using this dosing strategy has never been prospectively assessed in the context of pediatric craniofacial reconstructive surgery. Although the calculated blood loss was 43 mL/kg in the first case and only 19 mL/kg in the second case, we believe that the use of EACA was beneficial in reducing intraoperative blood loss and the need for transfusion in both patients. Historically, at our institution, average calculated blood loss for craniofacial patients who do not receive EACA is 63 mL/kg. The difference in calculated blood loss between these 2 cases could be explained by the longer operative time in the first case by 55 minutes along with relatively lower platelets and elevated postoperative PT/PTT and INR, suggesting a more severe coagulopathy.

Although we recognize that our blood conservation strategy might not be appropriate for every patient undergoing total calvarial vault reconstruction, for major elective surgery in a pediatric Jehovah’s Witness patient in which blood loss is expected to be excessive, an attempt at avoiding transfusion and honoring parents’ religious beliefs is a reasonable approach. The most important consideration should always be patient safety by maintaining stable hemodynamics, end-organ perfusion, and oxygenation of vital organs.

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