Anaesthetic management of a rare case of congenital factor VII deficiency – Patient posted for shoulder arthroscopic synovectomy: A case report

INTRODUCTION

Factor VII deficiency (F7D) is an autosomal recessive rare coagulation disorder characterised by reduced plasma factor VII (FVII) activity. FVII is crucial for initiating the coagulation cascade through the extrinsic pathway. With an estimated prevalence of 1 in 500,000, F7D poses a significant risk of bleeding complications during surgical procedures.^[1] This case report presents a unique contribution by showcasing successful anaesthetic management in a young patient with F7D who underwent shoulder arthroplasty.

CASE REPORT

A 20-year-old female with congenital F7D diagnosed in childhood due to heavy menstrual bleeding was managed with hormonal oral contraceptives and tranexamic acid. She had no family history of bleeding disorders, no acquired abnormalities such as vitamin K deficiency, liver disease, or warfarin therapy, and was not on any drugs affecting FVII. Her past medical history included mild bleeding after minor injuries.

Physical examination revealed left shoulder pain and limited range of motion. Airway and other systems examinations were normal. Body mass index was 22.7 kg/m². Laboratory tests confirmed moderate F7D (FVII level: 3%), with haemoglobin at 9.8 g/dl, prolonged prothrombin time (PT) of 64.5 seconds, and international normalised ratio (INR) of 5.6. Liver and renal function tests, activated partial thromboplastin time, and platelet counts were normal. The diagnosis of congenital F7D was established based on the patient’s history, clinical presentation, and laboratory findings. No diagnostic challenges were encountered.

A multidisciplinary team of anaesthetists, orthopaedic surgeons, and haematologists was involved in the patient’s care.

The surgical team, after consulting the haematologist, transfused packed red blood cells on the day before surgery to raise haemoglobin levels above 12 g/dl (corrected haemoglobin—12.3 g/dl). Blood, fresh frozen plasma, and platelets were prepared in advance, fasting status confirmed, and informed consent obtained for anaesthesia, surgery, rFVIIa, and potential blood product transfusions.

Large-bore intravenous (IV) access was established, and recombinant activated FVII (rFVIIa—30 µg/kg) and tranexamic acid (20 mg/kg) were administered [Figure 1a and 1b].

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Figure 1:

(a) Recombinant activated factor VII (rFVIIa), (b) Recombinant activated factor VII (rFVIIa) syringe & vial

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Ten minutes after rFVIIa administration, after preoxygenation, general anaesthesia was provided with controlled ventilation and haemodynamic stability. Premedication included glycopyrrolate (0.005 mg/kg IV), fentanyl (2 µg/kg IV), and midazolam (1 mg IV). Induction involved propofol (2 mg/kg IV) and muscle relaxation with vecuronium (0.1 mg/kg IV), followed by intubation with a cuffed flexo-metallic endotracheal tube (7.5mm internal diameter). Maintenance was achieved with isoflurane (end-tidal concentration: 0.5%–1.5%) in oxygen and air in volume control mode and additional vecuronium (0.02 mg/kg IV/dose × 5 doses).

Immediately after intubation, an ultrasound guided interscalene brachial block was performed with a 22 G × 50 mm needle, using 15 ml of 0.25% bupivacaine and dexamethasone 4 mg (for prolonged analgesia). As this was a superficial nerve block, in the event of a haematoma at the block site, manual compression was planned. Simultaneously, paracetamol (20 mg/kg IV) was administered. The patient was positioned supine with a 15-degree head-up tilt, and body warmers were used.

Monitoring included electrocardiogram, non-invasive blood pressure, percutaneous oxygen saturation, nasopharyngeal temperature (to avoid hypothermia), end-tidal carbon dioxide, neuromuscular function, bispectral index monitoring, blood loss, and coagulation parameters (PT—11.8 seconds and INR—1.0 at 1 hour after rFVIIa administration). Strategies to prevent blood loss, such as tranexamic acid and patient positioning, were employed to reduce blood transfusions (transfusion trigger—7.5 g/dl). Warmed Ringer’s lactate (1000 ml) was administered to maintain haemodynamic stability.

The total surgical time was 2 hours, with estimated blood loss by visual quantification of 300 ml (maximum allowable blood loss—1270 ml). Urine output was 150 ml. After surgery, neuromuscular blockade was reversed with neostigmine (0.05 mg/kg IV) and glycopyrrolate (0.01 mg/kg IV), followed by careful suctioning and extubation after full recovery.

Postoperatively, the patient was closely monitored and treated with rFVIIa (three doses of 30 µg/kg repeated every 6 hours) and paracetamol (20 mg/kg IV every 6 hours). Non-steroidal anti-inflammatory drugs and intramuscular injections were avoided. Haemodynamic stability was maintained, and pain (visual analogue scale) scores at 0 before 18 hours and below 3 thereafter. The patient remained comfortable, with no bleeding complications or unexpected issues. She was discharged 5 days post-surgery with follow-up appointments.

DISCUSSION

CONCLUSION

Our patient was successfully managed with a multidisciplinary team of anaesthesiologists, surgeons and haematologists who created a tailored plan to minimise the bleeding risks. Key strategies included preoperative optimisation with rFVIIa and tranexamic acid, coagulation monitoring, and a combination of general anaesthesia with an ultrasound-guided interscalene brachial block. Continuous monitoring of vital signs, availability of fresh frozen plasma, and meticulous postoperative care facilitated effective management and recovery.