Hemostasis is an important concept in pediatric otolaryngologic surgery. This article details the considerations the otolaryngologist should take when it comes to clinical evaluation and surgical technique. It begins with the preoperative evaluation, and evolves into the use of different mechanical and chemical methods of operative hemostasis. We detail use of different hemostatic techniques in common pediatric procedures, and finally, we discuss indications for intraoperative and postoperative blood transfusion in pediatric patients if the surgeon encounters significant intraoperative hemorrhage. This paper gives a comprehensive look into the hemostatic considerations for the pediatric patient through the preoperative to postoperative period.
Key learning points
At the end of this article, the reader will:
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Be able to make a thorough preoperative assessment of pediatric patients with respect to risk of surgical bleeding.
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Be familiar with common bleeding disorders affecting pediatric patients.
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Be able to discuss hemostatic strategies used in common pediatric surgeries such as adenotonsillectomy, posttonsillectomy hemorrhage, and middle ear surgery.
Preoperative evaluation
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Patient history: prolonged bleeding (spontaneous, epistaxis, dental extractions, surgeries), spontaneous bruising, excessive bruising after surgery, medications affecting platelet function (anticoagulants, nonsteroidal anti-inflammatory drugs, aspirin), diseases (malignancies, hepatic, renal, hematologic).
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Family history: bleeding tendencies, inheritable diseases (von Willebrand, hemophilia), easy bruising.
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Physical examination: petechiae and ecchymoses (thrombocytopenia or functionally deficient platelets), telangiectasias (liver disease or hereditary hemorrhagic telangiectasia), hematomas, evidence of hemarthroses and joint deformities.
If there are any signs of family or personal history of bleeding issues, then preoperative laboratory evaluations are indicated. Preoperative investigations without positive findings in history and physical examination have not been found to be beneficial in either retrospective or prospective studies.
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Prothrombin time (PT): assesses the extrinsic pathway of clotting, and coagulation factors in the common pathway.
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Partial thromboplastin time (PTT): assesses the intrinsic coagulation pathway and final common pathway.
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Platelet count ( Table 1 ).
Table 1
Desired platelet count ranges for prevention of bleeding
Clinical Scenario
Platelet Count
Prevention of mucocutaneous bleeding
>10,000–20,000
Insertion of central venous catheter
>20,000–50,000
Minor surgical procedures
>50,000–80,000
Major surgical procedures
>80,000–100,000
Adapted from Fogarty PF, Minichiello T. Chapter 14. Disorders of hemostasis, thrombosis, & antithrombotic Therapy. In: Papadakis MA, McPhee SJ, Rabow MW, editors. CURRENT Medical diagnosis & treatment 2014. New York: McGraw-Hill; 2014.
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Bleeding time: assess platelet function.
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Hemoglobin: greater than 10 g/dL before surgery for pediatric patients.
PT assesses the extrinsic pathway of clotting, which consists of tissue factor and factor VII, and coagulation factors in the common pathway (factors II [prothrombin], V, and X, and fibrinogen). PTT assesses the intrinsic coagulation pathway (prekallikrein, high molecular weight kininogen, factors XII, XI, IX, and VIII) and final common pathway (factors II, V, and X, and fibrinogen). In general, a platelet count of greater than 80,000 is desirable to minimize bleeding risk during major surgical procedures (see Table 1 ). Bleeding time classically assesses platelet function but it is not a good predictor of intraoperative or postoperative bleeding. A retrospective study found a 5% positive predictive value of the bleeding time and a 95% negative predictive value. Table 2 provides common causes of abnormal PT and/or PTT.
| Test Result | Causes of Test Result Pattern | |
|---|---|---|
| PT | aPTT | |
| Prolonged | Normal | Inherited |
| Factor VII deficiency | ||
| Acquired | ||
| Acquired factor VII deficiency | ||
| Mild vitamin K deficiency | ||
| Liver disease | ||
| Warfarin administration | ||
| Inhibitor of factor VII | ||
| Lupus anticoagulant (rare; may be associated with bleeding rather than thrombosis) | ||
| Normal | Prolonged | Inherited |
| Deficiency of factors VIII, IX, or XI | ||
| Deficiency of factor XII, prekallikrein, or HMW kininogen a | ||
| von Willebrand disease (variable) | ||
| Acquired | ||
| Heparin administration | ||
| Inhibitor of factors VIII, IX, XI, or XII | ||
| Acquired von Willebrand disease | ||
| Lupus anticoagulant b | ||
| Prolonged | Prolonged | Inherited |
| Deficiency of prothrombin, fibrinogen, or factors V or x | ||
| Combined factor deficiencies | ||
| Acquired | ||
| Liver disease | ||
| Disseminated intravascular coagulation | ||
| Supratherapeutic doses of anticoagulants | ||
| Severe vitamin K deficiency | ||
| Combined heparin and warfarin administration | ||
| Angatroban with or without warfarin administration | ||
| Inhibitor of prothrombin, fibrinogen, or factors V or X | ||
| Primary amyloidosis-associated factor X deficiency | ||
a Not associated with a bleeding diathesis.
It is generally suggested that the hemoglobin should be greater than 10 g/dL before surgery for pediatric patients. However, in infants younger than 6 months, the hematologic system is not fully matured and they have physiologic anemia owing to decreased erythropoietin; thus, they are more susceptible to adverse side effects of transfusion. In this age group, it is more important to take into account the severity of the surgery, likelihood of excessive bleeding, and the severity of the anemia before transfusing.
Preoperative evaluation
- •
Patient history: prolonged bleeding (spontaneous, epistaxis, dental extractions, surgeries), spontaneous bruising, excessive bruising after surgery, medications affecting platelet function (anticoagulants, nonsteroidal anti-inflammatory drugs, aspirin), diseases (malignancies, hepatic, renal, hematologic).
- •
Family history: bleeding tendencies, inheritable diseases (von Willebrand, hemophilia), easy bruising.
- •
Physical examination: petechiae and ecchymoses (thrombocytopenia or functionally deficient platelets), telangiectasias (liver disease or hereditary hemorrhagic telangiectasia), hematomas, evidence of hemarthroses and joint deformities.
If there are any signs of family or personal history of bleeding issues, then preoperative laboratory evaluations are indicated. Preoperative investigations without positive findings in history and physical examination have not been found to be beneficial in either retrospective or prospective studies.
- •
Prothrombin time (PT): assesses the extrinsic pathway of clotting, and coagulation factors in the common pathway.
- •
Partial thromboplastin time (PTT): assesses the intrinsic coagulation pathway and final common pathway.
- •
Platelet count ( Table 1 ).
Table 1
Desired platelet count ranges for prevention of bleeding
Clinical Scenario
Platelet Count
Prevention of mucocutaneous bleeding
>10,000–20,000
Insertion of central venous catheter
>20,000–50,000
Minor surgical procedures
>50,000–80,000
Major surgical procedures
>80,000–100,000
Adapted from Fogarty PF, Minichiello T. Chapter 14. Disorders of hemostasis, thrombosis, & antithrombotic Therapy. In: Papadakis MA, McPhee SJ, Rabow MW, editors. CURRENT Medical diagnosis & treatment 2014. New York: McGraw-Hill; 2014.
- •
Bleeding time: assess platelet function.
- •
Hemoglobin: greater than 10 g/dL before surgery for pediatric patients.
PT assesses the extrinsic pathway of clotting, which consists of tissue factor and factor VII, and coagulation factors in the common pathway (factors II [prothrombin], V, and X, and fibrinogen). PTT assesses the intrinsic coagulation pathway (prekallikrein, high molecular weight kininogen, factors XII, XI, IX, and VIII) and final common pathway (factors II, V, and X, and fibrinogen). In general, a platelet count of greater than 80,000 is desirable to minimize bleeding risk during major surgical procedures (see Table 1 ). Bleeding time classically assesses platelet function but it is not a good predictor of intraoperative or postoperative bleeding. A retrospective study found a 5% positive predictive value of the bleeding time and a 95% negative predictive value. Table 2 provides common causes of abnormal PT and/or PTT.
| Test Result | Causes of Test Result Pattern | |
|---|---|---|
| PT | aPTT | |
| Prolonged | Normal | Inherited |
| Factor VII deficiency | ||
| Acquired | ||
| Acquired factor VII deficiency | ||
| Mild vitamin K deficiency | ||
| Liver disease | ||
| Warfarin administration | ||
| Inhibitor of factor VII | ||
| Lupus anticoagulant (rare; may be associated with bleeding rather than thrombosis) | ||
| Normal | Prolonged | Inherited |
| Deficiency of factors VIII, IX, or XI | ||
| Deficiency of factor XII, prekallikrein, or HMW kininogen a | ||
| von Willebrand disease (variable) | ||
| Acquired | ||
| Heparin administration | ||
| Inhibitor of factors VIII, IX, XI, or XII | ||
| Acquired von Willebrand disease | ||
| Lupus anticoagulant b | ||
| Prolonged | Prolonged | Inherited |
| Deficiency of prothrombin, fibrinogen, or factors V or x | ||
| Combined factor deficiencies | ||
| Acquired | ||
| Liver disease | ||
| Disseminated intravascular coagulation | ||
| Supratherapeutic doses of anticoagulants | ||
| Severe vitamin K deficiency | ||
| Combined heparin and warfarin administration | ||
| Angatroban with or without warfarin administration | ||
| Inhibitor of prothrombin, fibrinogen, or factors V or X | ||
| Primary amyloidosis-associated factor X deficiency | ||
a Not associated with a bleeding diathesis.
It is generally suggested that the hemoglobin should be greater than 10 g/dL before surgery for pediatric patients. However, in infants younger than 6 months, the hematologic system is not fully matured and they have physiologic anemia owing to decreased erythropoietin; thus, they are more susceptible to adverse side effects of transfusion. In this age group, it is more important to take into account the severity of the surgery, likelihood of excessive bleeding, and the severity of the anemia before transfusing.
Pediatric bleeding and blood disorders in surgery
von Willebrand disease (vWD) is a family of disorders caused by quantitative or qualitative defects of von Willebrand factor (VWF), a plasma protein that plays a role in both platelet adhesion and fibrin formation. Between 75% and 80% of patients have type 1 vWD. This is a quantitative abnormality of the vWF molecule that results in decreased amounts of VWF protein. The clinical symptoms of type 1 VWD include mucosal bleeding, easy bruising, menorrhagia, and postoperative hemorrhage. Patients with type 1 vWD usually have mild to moderate platelet-type bleeding. Type 2 vWD is seen in 15% to 20% of patients with vWD. There are different subtypes, but all involve a qualitative defect in the VWF protein. Patients with type 2 vWD usually have moderate to severe bleeding that presents in childhood or adolescence ( Table 3 ).
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vWD type 1
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Desmopressin: increases release of vWF, overcoming quantitative defect in type 1 vWD
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vWF concentrate
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vWD type 2
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vWF concentrate is effective
- ○
Desmopressin: not effective owing to qualitative defect in vWF molecule
- ○
| Type 1 | Type 2 |
|---|---|
| 75%–85% of patients with vWD | 15%–20% of patients with vWD |
| Quantitative defect of vWF | Qualitative defect of vWF |
| Mild-moderate platelet bleeding | Moderate-severe platelet bleeding |
| Can present in childhood or adulthood | Usually presents in childhood/adolescence |
| Treat with Desmopressin or vWF concentrate | Treat with only vWF concentrate |
In vWD type 1, desmopressin (DDAVP) can be used effectively. It increases release of vWF, overcoming quantitative defect in type 1 vWD (see Table 3 ). The dose of DDAVP is 0.3 μg/kg intravenously in 50 mL saline over 20 minutes, or nasal spray 300 μg for weight greater than 50 kg or 150 μg for less than 50 kg every 12 to 24 hours, maximum of 3 doses in a 48-hour period. If more than 2 doses are used in a 12- to 24-hour period, free water restriction and/or monitoring for hyponatremia is essential.
vWF concentrate can be given at a dose is 60 to 80 recombinant factor units per kilogram intravenously every 12 hours initially followed by lesser doses at longer intervals once hemostasis has been established.
For vWD type 2, desmopressin is generally not effective owing to qualitative defect in vWF molecule and vWF concentrate is required (see Table 3 ).
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Hemophilia A: 1 per 5000 live male births.
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Hemophilia B: 1 in 25,000 live male births.
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X-linked recessive inheritance.
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Testing is indicated for asymptomatic male infants with a hemophilic pedigree.
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Hemophilia diagnosis: isolated reproducibly low factor VIII or factor IX activity, in the absence of other conditions.
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