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Revista Brasileira de Anestesiologia

Print version ISSN 0034-7094

Rev. Bras. Anestesiol. vol.54 no.6 Campinas Nov./Dec. 2004 



Anesthesia for cesarean section in patient with von Willebrand's disease and HIV infection. Case report*


Cesárea en paciente con enfermedad de von Willebrand asociada a la infección por el HIV. Relato de caso



Vanessa Rezende Balle, M.D.I; Sheila Braga Machado, M.D.II; Marcos Emanuel Wortmann Gomes, M.D.III; Florentino Fernandes Mendes, TSA, M.D.IV

IAnestesiologista da Santa Casa de Porto Alegre; Mestranda em Farmacologia pela FFFCMPA, Responsável pelo Núcleo de Analgesia e Anestesia Obstétrica da Santa Casa de Porto Alegre
IIAnestesiologista da Santa Casa de Porto Alegre
IIIME2 em Anestesiologia
IVChefe do Serviço de Anestesiologia da Santa Casa de Porto Alegre; Mestre em Farmacologia pela FFFCMPA





BACKGROUND AND OBJECTIVES: Von Willebrand's disease is the most common hereditary coagulation disorder in young women. The incidence of HIV infection among women has been progressively increasing, and vertical transmission may account for 25% of cases. This report aimed at describing the case of an HIV-positive patient with von Willebrand's disease scheduled for cesarean section.
CASE REPORT: Female HIV-positive patient, 24 years old, with microcytic anemia and von Willebrand's disease, admitted to the emergency room in early labor. She had no pre-natal care. Cesarean section was indicated to lower vertical transmission risks since HIV viral count was unknown. Patient had hematomas on both arms and legs and history of abdominal wall hematoma in previous cesarean section. Coagulation tests were mildly changed. General anesthesia was induced after factor VIII concentrate infusion. Both mother and newborn had satisfactory outcomes.
CONCLUSIONS: Clinical evaluation of patients with coagulopathies is critical for determining the anesthetic technique. Evaluation should be individualized, considering risks and benefits of the technique. Cesarean section for these patients should be avoided whenever possible and replaced by less invasive methods. Factor VIII concentrate therapy is the best treatment option for correcting specific deficiency and lowering viral transmission risks.

Key Words: DISEASES: acquired immunodeficiency syndrome, von Willebrand's; SURGERY, Obstetric: cesarean section


JUSTIFICATIVA Y OBJETIVOS: La enfermedad de von Willebrand es la alteración innata de la coagulación más frecuente en mujeres jóvenes. La infección por HIV ha mostrado incidencia progresivamente mayor en mujeres, constatandose transmisión vertical en hasta 25% de los casos. El objetivo de este relato es mostrar el caso de una paciente con Enfermedad de von Willebrand y HIV positiva sometida a cesárea.
RELATO DEL CASO: Paciente de 24 años, portadora de anemia microcítica, enfermedad de von Willebrand y HIV, llegó a la emergencia obstétrica en inicio de trabajo de alumbramiento. No realizó prenatal. Fue indicada cesárea a fin de disminuir los riesgos de transmisión vertical en paciente con carga vírica de HIV desconocida. Presentaba hematomas por el cuerpo e historia de hematoma de pared abdominal en cesárea anterior. Los tests de coagulación estaban un poco alterados. Después de infusión de concentrado de factor VIII fue realizada anestesia general. Madre y recién nacido presentaron evolución satisfactoria.
CONCLUSIONES: La evaluación de manifestaciones clínicas en pacientes con coagulopatia es fundamental en la decisión del tipo de anestesia que será indicada para cada paciente. La evaluación debe ser individualizada, considerando los riesgos y beneficios de la técnica escogida. En estas pacientes, se debe siempre restringir al máximo la indicación de interrupción de la gestación por vía alta, optándose siempre por los métodos menos invasivos. La terapia con concentrado de factor VIII es actualmente la mejor opción de tratamiento, corrigiendo la deficiencia específica y dismunuyendo los riesgos de transmisión vírica.




Von Willebrand's disease (vWD) is the most common hereditary coagulation disorder in women, affecting up to 1% of the population. So, it is the coagulopathy with higher chances of causing gestation and delivery problems. The severe manifestation of the disease, however, has a lower incidence of approximately 1:10000 1.

HIV infection is becoming increasingly frequent in women and vertical transmission rate may account for 25% of cases. Several studies on serum prevalence in pregnant women have shown rates of 8 to 20 out of 1000 women 2. Currently, surgical gestation interruption is indicated for patients with unknown viral count or with more than 1000 copies/mL, to decrease vertical viral transmission risks 3.

This report aimed at presenting the case of an HIV-positive labor patient with vWD, scheduled for cesarean section, focusing on anesthetic management.



Female, Afro-American term gestation patient, 24 years old, 75 kg, 155 cm, referred to the obstetric emergency in early labor. Patient was classified as physical status ASA III due to microcytic anemia, von Willebrand's disease and positive HIV according to historical information. HIV was confirmed by rapid test at admission. Patient also presented upper airways infection. Her obstetric history included three gestations with one vaginal delivery and one cesarean section 10 months ago, which has evolved with postoperative abdominal wall hematoma. Patient had no pre-natal care during this gestation. At physical evaluation, patient presented perineal condylomatosis, rectum-vaginal fistula and several limb hematomas, without any other signs of bleeding.

Preoperative tests revealed 25.9% hematocrit, 8.7 mg/dL hemoglobin, 142000 platelets, 74% PT, 42.2/36s PTT, 2.71 D-dimers, 523 mg/dL fibrinogen, 1 m 4s bleeding time and 4m 11s clotting time.

Cesarean section was indicated due to HIV infection with unknown viral count.

According to hematologist’s orientation, patient was premedicated with 10 U cryoprecipitate and factor VIII concentrate.

After venoclysis with intravenous 20G and 14G catheters, patient was monitored with cardiac monitor, noninvasive blood pressure and pulse oximetry. Patient was premedicated with intravenous ranitidine (100 mg) and metochlopramide (10 mg). Balanced general anesthesia was induced with rapid sequence technique, with 415 mg thiopental and 80 mg succinylcholine. After oxygenation with 100% oxygen under mask for 3 minutes, tracheal intubation was performed with 7 mm endotracheal tube with cuff, which has been promptly inflated, and mechanically controlled ventilation was started with 100% oxygen.

Anesthesia was maintained with isoflurane. After extraction of live male fetus with 3600 g and Apgar 5 and 6, patient received intravenous 100 µg alfentanil, 1 g cefazolin and 25 Ul ocytocin (total). After beginning of ocytocin infusion, there has been an episode of hypotension, promptly corrected with 10 mg ephedrine. Vital signs were stable. Patient received 2000 mL crystalloids and perioperative bleeding was considered minor. Procedure lasted 55 minutes. Intramuscular ketoprofen (100 mg) was injected for postoperative analgesia.

Patient received continuous intraoperative factor VIII infusion, which was maintained for 8 hours, followed by one dose every 12 hours for 4 days. Postoperative period has evolved without intercurrences. Coagulation was daily monitored with partial thromboplastin time (PTT), being the first postoperative result 51.7s without clinical significance. Patient was discharged in the 4th postoperative day with good evolution with outpatient follow up.



Von Willebrand's disease (vWD) is an autosomal dominant hereditary disease characterized by deficiency or defect of von Willebrand's factor (vWF), which is the protein responsible for one stage of platelet aggregation. In addition, vWF binds to coagulation cascade factor VIII protecting it against degradation. So, vWD, which is primarily a platelet function disorder, may secondarily promote coagulation disorders by coagulating factor VIII deficiency (VIII:C) 1.

vWD may be classified in three types. Two major forms are type 1 with quantitative vWF deficiency, corresponding to 75% of cases; and type 2 with qualitative defect, corresponding to approximately 17% of cases. Bleedings in type 2 are more frequent and severe as compared to type 1. Type 3 is less frequent (1% of patients) and clinically more severe. It is important to determine patient's type because it helps treatment, allowing specialists to act on the specific deficiency 4. In our case, this classification was impossible due to the absence of previous follow up.

In general, there is mucosal bleeding, but there may be GI bleeding. In parturients, bleeding is more frequent after delivery than during gestation and is associated to surgical delivery and perineal injury. If vWD is suspected, history of menorrhagia or other mucosal bleedings and family history of the disease should be investigated 5.

During third trimester gestation, fibrinolytic activity is suppressed and coagulation factors tend to increase (especially fibrinogen), resulting in hypercoagulation, which may promote clinical improvement in vWD patients 5,6. After delivery, patients return to pre-pregnancy state and coagulation problems may be present in the post-delivery period. Patients with moderate disease tend to be more subject to post-delivery as compared to intra-delivery problems 6,7. Although not reporting bleedings, patient presented several hematomas and had history of post-cesarean section abdominal wall hematomas.

Recommended lab monitoring is hematocrit, hemoglobin, prothrombin time, thromboplastin time and bleeding time. In addition, specific factor VIII, vWF antigen and vWF activity dosages are suggested 5.

vWF levels may be measured by factor VIII antigen or by the activity of ristocetin co-factor, which measures functional vWF properties in platelet aggregation. When factor VIII:C levels are below 25%, partial thromboplastin time (PTT) will be prolonged. Low factor VIII levels are major determinants of delivery hemorrhages 5.

These specific dosages were impossible in our case due to lack of time and because they are not routinely used in our service.

Factor VIII complex function may be estimated by bleeding time. In most cases, there are normal platelet morphology and number, and bleeding time is increased. Bleeding time should always be requested if this diagnosis is suspected and is the test correlating the best with bleeding trend 4. In our patient, bleeding time was normal, not suggesting major clinical repercussion.

During cesarean sections or other surgical procedures, factor VIII:C levels should be 80% or more, and bleeding time should be normal 8. Careful surgical hemostasis and effective uterine contraction may compensate increased bleeding time 7.

The risk of post-regional anesthesia hematoma in women with hematological disorders or totally anticoagulated is low 9. Each patient should be individually evaluated. When general anesthesia is indicated, care should be taken to prevent tracheal intubation-induced airway trauma 6.

If coagulation tests are normal with bleeding time below 10 minutes and platelet count above 100000, there is no counterindication for epidural catheter in patients with moderate disease 5,7. Coagulation tests should be normal before catheter is removed 5. Spinal anesthesia may be a safe option for elective surgeries 5,7.

General anesthesia was used in our case because patient had no clinical follow up, with unknown coagulopathy severity, abnormal physical evaluation and history of hematoma in previous surgery. Because patient was admitted already in labor, a deeper investigation of the disease with coagulation factors dosage was impossible.

There are two major therapeutic agents to prepare patients with vWD: desmopressin and blood products containing concentrated factor VIII and vWF 7.

Desmopressin (DDAVP) transiently increases factor VIII and vWF releasing them from storage sites to plasma, being an effective treatment for type 1 disease. In general, high factors concentrations last 8 to 10 hours. Recommended dose is subcutaneous 0.3 µ or nasal 300 µg. Infusions may be repeated every 12 or 24 hours, if needed 7. In our case, DDAVP has been discussed with the hematologist and considered unnecessary, since type of disease was unknown and there was factor VIII concentrate available for intravenous administration. Major risk for fluid retention in patients under this therapy has been observed. In addition, since there is variable response to desmopressin, a test infusion is recommended some weeks before surgery or delivery to measure response and evaluate possible adverse events 5.

Blood transfusion is the treatment of choice when there is bleeding or when it should be prevented in cases were desmopressin is considered insufficient for hemostasis. Large volume fresh frozen plasma may be used 7. Fresh frozen plasma is in general enough to correct coagulation defects, but when there is major fibrinogen depletion (< 0.8 g/L) 10 to 15 units of cryoprecipitate are needed 5. Cryoprecipitate has 5 to 10 times more factor VIII and vWF as compared to fresh plasma 7.

Cryoprecipitate transfusions are recommended (15 to 20 units) when preoperative bleeding time is abnormal or factor VIII:C levels are below 50% 8. Cryoprecipitate every 12 or 24 hours normalizes factor VIII levels and stops or prevents bleeding. Factor VIII and vWF are currently preferred for being free from viral transmission risk.

Recommended dose is 40 to 60 Ul/kg once a day. For surgical procedures, factor VIII should be dosed every 12 hours in surgery day and then every 24 hours. To every 1 Ul/kg concentrate there is 2 Ul/dl factor VIII increase 7. Thrombocytopenia (platelet count below 50000) may require correction with platelet concentrate transfusion 5.

We have initially used cryoprecipitate at hematologist’s indication. After new consultation, factor VIII was made available and therapy was replaced. PTT was used for monitoring because results are faster allowing therapy control. PTT is able to measure intrinsic coagulation pathway activity, which involves factor VIII activity. Although not being the golden standard to evaluate this disorder, it may be an alternative in cases when factor VIII dosage is unavailable or when results cannot be obtained as promptly as needed.

It should also be stressed that risk-benefit ratio should always be taken into consideration when indicating surgical gestation interruption in vWD patients, since there are potential maternal risks 10,11.

vWD patients evaluation should always take into consideration clinical manifestations of the disease, which, together with specific coagulation factors dosages, are the most important elements to determine the severity of the disease. When these tests are unavailable, bleeding time and PTT measures may be useful.

Factor VIII concentrate is currently the most widely indicated therapy for specifically correcting the disorder without viral transmission risk. If this therapy is not available, fresh frozen plasma or cryoprecipitate transfusion is recommended.

Surgical gestation interruption in these patients should be restricted, and vaginal delivery should be the choice, whenever possible, to decrease maternal risks.

The best anesthetic option for coagulopathy patients is still controversial and should be decided in a case-by-case basis.



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Correspondence to
Dra Vanessa Rezende Balle
Address: Rua Felizardo Furtado, 215 Petrópolis
ZIP: 90670-090 City: Porto Alegre, Brazil

Submitted for publication April 28, 2003
Accepted for publication July 20, 2004



* Received from Centro Obstétrico da Maternidade Mário Totta da Santa Casa de Porto Alegre, RS

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