Article Type : Case Report
Authors : Deepali Gupta, Dhruv Gupta, Ashish Aneja, Radha Panchapakesan, Arulmurugan Balasubramanian, Taha M Abdelaal, Mohamed Othman, Samia Al Saddik, Steffi Paulson John and Youmna Ayman Faheem
Keywords : Hemophilia; Adenotonsillectomy; Haemorrhage
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].
Postoperatively, the patient was taken to the
anaesthesia care unit and was sought after for any bleeding, or complications.
Patient followed up in the clinic two weeks later with the ENT doctor, and then
with the paediatric haematologist as well. Finally, there were no signs of
postoperative bleeding, or infection (Tables 1,2).
Table 1: Investigations.
LAB TEST |
DATE |
RESULT |
RANGE |
FACTOR VIII |
7 DECEMBER 2022 |
52 % (LOW) |
57-163 |
FACTOR IX |
7 DECEMBER 2022 |
15 % (LOW) |
52-111 |
FACTOR X |
7 DECEMBER 2022 |
113 % (NORMAL) |
76-183 |
FACTOR XI |
7 DECEMBER 2022 |
74 % (NORMAL) |
60-150 |
FACTOR XII |
7 DECEMBER 2022 |
102 % (NORMAL) |
50-150 |
aPTT |
7 DECEMBER 2022 |
34.9 seconds (borderline) |
26.0-35.0 |
Lupus Anticoagulant |
7 DECEMBER 2022 |
40.5 seconds |
0.0-44.0 |
vWF Activity |
7 DECEMBER 2022 |
41 %(LOW) |
50-200 |
vWF Antigen |
7 DECEMBER 2022 |
61 % (NORMAL) |
50-200 |
Medicine Name |
Strength |
Dosage |
Frequency |
Duration |
Start Date |
End Date |
Quantity |
Instructions |
TRANEXAMIC ACID 500 MG TABLETS |
750 mg |
750 |
TID |
1 week |
26/12/2022 |
1-1-2023 |
21 |
Start one day before operation |
The presence of coagulation disorder in children who
are operated for adenotonsillectomy is a challenge for the Surgeon and
Anaesthetist. Adenotonsillectomy can be done safely in patients with
haemophilia B, but the increased risk of postoperative haemorrhage cannot be
ruled out. Haemophilia B is the most common hereditary coagulopathy and is due
to a quantitative and/or qualitative deficiency of Factor IX. Proper
preoperative history and physical examination with emphasis on past bleeding
and family history can decrease the complications. Children undergo laboratory
screening and Hematologic evaluation to determine the presence of a hereditary
or acquired coagulopathy. This patient was diagnosed with Factor IX deficiency.
Patients are diagnosed to have haemophilia B if they had one or more abnormal
values of factor IX, vWF antigen, or aPTT, along with a family or patient
bleeding history or two or more abnormal laboratory values in the absence of a
family or bleeding history. Our child had low factor VIII, lX, low vWF activity
with prolonged aPTT. Positive history of bleeding was there during his
circumcision surgery.
The management plan included proper
screening, tranexamic acid and extra doses of factor IX surrounding the
surgery.
We conclude that patients with bleeding disorders
undergoing adenotonsillectomy are at a higher risk of bleeding and require
close monitoring with haemostatic support for a prolonged period of time in
post-operative period.
Haemophilia B is a bleeding disorder due to Factor IX
deficiency, and most commonly affects male patients–rarely females. Bleeding is
the main complication from adenotonsillectomy for haemophiliac children.
Therefore, the paediatric haematologist and otolaryngologist (ear, nose and
throat, or ENT specialist) should coordinate a treatment plan in order to
prevent any adverse events in future surgeries.