Anaesthetic Management of a Child with Factor IX Deficiency Planned for Adenotonsillectomy

Kids with bleeding disorders going for adenotonsillectomy needs meticulous advance planning to avoid any complications. The main complication from adenotonsillectomy for children with bleeding disorders is bleeding. Extra precautions to manage bleeding must be taken before, during and after the surgery.

The risk of haemorrhage post adenotonsillectomy surgery is 0.2 to 2.2 for a primary haemorrhage, and 0.1 to 4.8 for a secondary haemorrhage. These surgeries are done frequently in the paediatric population and bleeding is one of the most common postoperative complications a patient can encounter. This paper describes a case of a five year old male patient with a history of Factor IX deficiency who presented to the hospital for his adenotonsillectomy surgery as planned. In addition, in his preoperative tests it was found that his labs were suggestive of an underlying coagulation disorder. The tests ordered were his prothrombin time (PT) and activated partial thromboplastin time (aPTT) activities in the plasma. However, in this case; it was a good decision to perform these tests as the patient would have suffered from severe haemorrhage during and after the surgery. Preoperative testing should be done in order to ensure that each patient is safe before, during and after the surgery. Depending on the severity of the disease, factor IX concentrate may be given prior to dental extractions and surgery to prevent bleeding. The prognosis is usually good with treatment.

Background

The main feature of haemophilia B, also known as Christmas disease. Haemophilia is a hereditary disorder which results from deficiency of one of the blood's clotting factors. The X chromosome contains both the factor VIII and factor IX genes. Haemophilia B results from factor IX deficiency; it is inherited through an X-linked recessive pattern, predominantly affecting males, but in rare cases could also affect females. Haemophilia B affects 1 in 40,000 live males, or about 15% of haemophilia cases, it is less common than haemophilia A, which affects 1 in 5,000 live males, or about 85% of haemophilia cases, and haemophilia C affects 1 in 100,000 live births, or about 1% of haemophilia cases. All races and ethnic groups are equally affected. Consanguinity is a major factor in increasing frequencies in certain communities, such as Egyptians and Ashkenazi Jews. Individuals with severe haemophilia B are usually diagnosed shortly after birth or within the first 1-2 years of life; those with moderate haemophilia B, are diagnosed at five to six years of age; and those with mild haemophilia B, may find out in adulthood. Some people have mild disease, some moderate, and some more severe. Patients with more serious cases of haemophilia often get regular shots of the factor that they're missing — known as clotting factor replacement therapy — to prevent bleeding episodes. The clotting factors are transfused through an intravenous (IV) line, and can be given in the hospital, at the doctor's office, or at home.

Five year old male patient presented to the ENT clinic with a history of sore throat, breathing difficulties and recurrent tonsil infections, and was planned for an intracapsular tonsillectomy and adenoidectomy done under general anaesthesia. However, prior to the surgery he was diagnosed with moderate haemophilia B as per his preoperative lab assessment and past history of prolonged bleeding after a circumcision surgery at five months of age. The patient is currently not on any chronic medications, no history of any medical illnesses or a similar complaint in the past. In addition, there is no significant family history with regards to any bleeding disorder. Prior to his surgery, coagulation tests were done as per routine assessment, and it was found that his bleeding time was prolonged–also known as partial thromboplastin time (PTT). Therefore, this warranted further evaluation which showed that the patient had some deficiency in one or more of his blood tests. These deficiencies included a low Factor IX activity, a low Factor VIII activity, and a low Von Willbrand activity as well. Therefore, he was diagnosed with a mild haemophilia B defect. The patient was then treated with tranexamic acid 500 mg tablets for two weeks, and was instructed to take his first dose one day prior to the surgery in order to avoid any complications before, during, or after the procedure. Moreover, 25 mg/kg PO, tranexamic acid is indicated in patients with haemophilia for short-term use (i.e, 2-8 days) to reduce/prevent haemorrhage and reduce the need for replacement therapy. In addition, since this is a moderate case of haemophilia B, the patient was not transfused with Factor IX before surgery. The child was planned for general anaesthesia (GA) and had minimal side effects. Prior to the surgery, the child was pre-assessed by an anaesthesiologist for past medical history, any drug history and allergies. Moreover, the child’s airway, heart, and lungs were also examined by the anaesthesiologist to confirm the absence of any airway problem such as wheezing and coughing. After the assessment was completed, the patient was posted for the procedure under GA.  In addition, some blood work up was done, the patient was diagnosed with a Factor 8 deficiency and preparations were done accordingly– such as the availability of Factor 8 in the operation theatre (OT) in case of haemorrhage. Anaesthesia for adenotonsillectomy is a skill test for the anaesthesiologist. Our Aim is to provide safe anaesthesia by avoiding any complications and to bring a successful outcome. Premedication was given which included oral midazolam 6 mg twenty minutes before going for surgery in order to decrease the patient’s anxiety. This helps the child to separate from the parents smoothly and not remember the experience. General anaesthesia for tonsillectomy and adenoidectomy usually begins by having your child breathe aesthetic gas through a mask. Sevoflurane 6% was used for induction, and the child was pre-oxygenated for three minutes with 100% oxygen. Once the child was asleep, an intravenous (IV) line was secured with a 22 G cannula. The following medications were given for GA Intravenously; Propofol 30 mg IV Injection, Fentanyl 20 mg IV injection, Rocuronium 10 mg IV injection. Moreover, oral intubation was done with a number five endotracheal tube (ETT) as the child weighed 20 kgs, and was fixed at 14.5 cm. Finally a ventilator was connected. The child was antagonized with Neostigmine 50 micrograms per kg and Atropine 20 micrograms per kg. Throughout the surgery, the patient was maintained on oxygen nitrous 50.50, and Sevoflurane 2%. As a result, the surgery went smoothly and there were no intraoperative complications like bleeding or fluctuating vitals. The patient was monitored closely, keeping an eye out on the ECG, pulse, temperature, Oxygen and CO2 levels and neuromuscular monitoring. Intra-operatively, paracetamol 15 mg/kg was given, along with Ringer’s Lactate ½ DNS 150 mL IV. In addition, dexamethasone 2 mg IV was also given with Ondansetron 1.5 mg IV. Tranexamic acid 100 mg was also infused via IV for management of postoperative haemorrhage and other complications. Moreover, Factor XII along with coagulation Factor XI were kept on the side in the operation theatre in the case of any bleeding events. There were no complications like bleeding and hypotension during or after the surgery. The endotracheal tube was removed and the child was extubated in the head low and left lateral position (also known as the post tonsillectomy position). The throat was freed of any secretions and suctioned under vision. The patient was then shifted to the recovery room in the lateral position and was closely monitored for bleeding or any deterioration in consciousness. The recovery process was good and the child’s temperature, ECG, pulse, and SpO2 were monitored. Postoperatively, the following analgesia was advised; Pethidine 10 mg IV for effective pain control, Ondansetron 0.1 mg/kg IV for nausea control, and dexamethasone 0.15 mg/kg IV in case the child complains of pain and nausea in the postoperative period. The child was then shifted to the ward after monitoring for 6 hours in the post anaesthesia care unit. After which he stayed in the ward for 24 hours for observation of any postoperative bleeding, fever or related complications because the patient has a history of a bleeding disorder. His parents were advised to continue following up with the paediatric haematologist and ENT doctors [1-13]. 

Table 1: Investigations.