Mary, 24 yo G4P1 has been referred to the obstetric preadmission clinic for an elective LSCS at 38 weeks gestation. She is currently 37 weeks pregnant.
The referral letter includes the following information:
Past Medical History
Chronic pelvic pain with allodynia since appendicectomy 2015
Von Willebrand's Disease
Smoker (quit 2 weeks ago)
Anaesthetic & Surgical History
PONV in 2 previous GAs
Previous emLSCS 2018. Epidural for post-op analgesia for 2 days
Airway: MP 1, broken teeth x 2
Allergy
nil
Medications
Tramadol PRN (before pregnancy)
Panadeine forte
Duloxetine (before pregnancy)
OPENING QUESTION
Candidate has 2 minutes reading time to plan their initial response to the first question below
Click each question to reveal a suggested answer
How do you assess Mary for anaesthesia?
In addition to standard anaesthetic assessment encompassing maternal and foetal well-bing, there should be a focus on the major issues of chronic pain and vWD.
There is a high risk of chronic post-surgical pain after Caesarean Section and increased risk of intra-operative bleeding with a known coagulopathy.
Chronic pain
Site
Characteristics
Relieving factors - what medications work for her?
Aggravating factors - how has pregnancy affected it
Associated symptoms
Known to pain specialist?
Opioid usage / tolerance / dependence
Psychological barrier to neuraxial block
vWD
Age of diagnosis
Type of vWD
Assess risk of bleeding:
- Major bleeding events
- Known to haematologist with regular follow-ups?
- previous peri-procedural management, especially for last LSCS (specifically desmopressin, factor 8, risk mitigation for epidural etc)
Previous anaestheisa /epidural
Any issues from patient perspective
Any excessive bleeding
Investigations
FBC (Hb, platelets), Coags, Fibrinogen, vWB screening tests (vWB factor, ristocetin cofactor, collagen binding assay)
PROGRESS QUESITONS
Mary’s chronic pain is well managed under guidance from a gynaecologist specialising in pain medicine. She practices mindfulness and uses 2 panadeine forte tablets as required and tramadol 100mg for severe pain flares. Previous therapies including nortriptyline and gabapentin have been ineffective.
She has type 1 vWD and has not required any treatment. She has a history of lifelong menorrhagia prior to pregnancy. She has just seen a haematologist. Interpret these blood tests results (click here for expected response):
Hb 106 g/L
PLT 342 x 10^9/L
Iron 9.6 umol/L (ref: 5.0 – 30.4)
Transferrin saturation 9.8% (16 – 51)
Transferrin 3.9g/L (1.8 – 3.5)
Ferritin 11 ug/L (30 – 150)
vWF 33% (ref: 50 – 150%)
vWF:RCo 47% (ref: 50 – 200%)
Factor VIII assay 118% (ref: 50 – 100%)
vWF:RCo / VWF:Ag 0.8 (ref: >/= 0.7)
Iron deficiency anaemia
Von Willebrand studies consistent with Type 1 vWD
- low vWF and concordant decrease in vWF and vWF activity (low vWF:RCo) [ristocetin cofactor]
Specific to Type 1 von Willebrand Deficiency, what unique perioperative medications / products might be required?
- Desmopressin (DDAVP) – specific for Type 1 and 2A vWD
- Tranexamic acid – TPA-inhibitor. Antifibrinolytic.
- Platelet transfusions in severe refractory bleeding
- vWF concentrate in severe refractory bleeding
- Other concentrate products in general
- Recombinant factor 8, fibrinogen concentrate (Riastap), Novo-7 are not considered appropriate. Patients with Type 1 vWD are not deficient in any of these components.
How does desmopressin help in von Willebrand Deficiency?
- ↑release of von Willebrand factor (VWF) from endothelial cells over 30 – 60min with duration of action ~6h
- at least a 3- to 5-fold increase in factor VIII and VWF
- Dose 0.3mcg/kg in 50 – 100mL 0.9% N/S over 30min. Should administer at least 60 – 90min pre-op
- Contraindicated in Type 2B (thrombotic thrombocytopaenia)
How would you assess the safety of neuraxial anaesthesia in this patient?
Expected discussion points:
Consultation with haematology regarding risk of neuraxial haematoma perioperatively especially if plans of leaving in epidural catheter post-op.
- Highly dependent on results of vWD studies and coagulation profile
- low VWF <50% often considered a relative contraindication without correction
- Timing and efficacy of desmopressin if required (vWB Factor levels post-dose)
- Platelet function not usually impaired in Type 1, but need to check recent labs
General risks for difficult neuraxial block and need for multiple attempts which increaeses the risk of trauma and neuraxial haematoma or neuropraxia:
- abnormal spinal anatomy / previous spinal surgery
- severe anxiety and poor positioning
How would you approach analgesia post-operatively, considering her chronic pelvic pain?
Expected discussion points:
The management of post-op pain for this patient is 2-fold:
1. the challenge of optimising surgical pain on top her usual chronic pain
2. avoid side effects to neonate via transfer of drugs through breast-feeding
Patient is at high risk of severe acute on chronic pain and subsequent CPSP.
Essential to have an opioid sparing multimodal analgesic approach.
This would be best acheived with a CSE, including IT morphine 100mcg and using the epidural as her primary modality for post-op analgesia.
Regular paracetamol, NSAID
PRN opioid
Avoid high-dose opioids
- for risk of respiratory depression in breastmilk fed neonate and maternal opioid-induced hyperalgesia)
Referral to APS for daily follow-up
If candidate plans for post-op epidural:
What is your epidural protocol?
Any safe dose / protocol is acceptable.
However, there is now ample evidence that favours a PIEB / PCEA or PIEB + PCEA. 0.2% ropivacaine 2mcg/mL fentanyl. 4 - 8mL/h
If candidate does not choose post-op epidural, they should have an alternate advanced modality e.g. opioid PCA, ketamine infusion.
Given the known history, it would not be appropriate to have a post-op plan of only PO analgesia on Day 1 post-op.
(If not already provided in previous response) Mary asks if the post-op analgesia medications are safe for breastfeeding. She’s read online that she may need to first discard a small volume of expressed milk before feeding her baby. What’s your response?
- The old advice of ‘pumping and dumping’ is out of date and no longer recommended.
- Generally, in terms of anaesthetics and commonly used analgesics, only tiny amounts are transferred into breastmilk.
- Analgesics including opioids are safe provided that are given below a prescribed maximum dose e.g. best to avoid > 40mg oxycodone / day.
- The risk of opioid related respiratory depression and drowsiness in babies becomes less after 6 weeks (corrected gestational age).
- Codeine should be avoided due to unpredictable differences in drug metabolism (CYP2D6 genetic polymorphism)
CHALLENGE QUESTIONS
On day of surgery Mary is premedicated with desmopressin 0.3mg/kg. Repeat vWF testing shows a good response she is deemed safe to proceed with a Combined Spinal Epidural block. The operation begins uneventfully. During uterine closure, the patient develops hypotension 70/40 mmHg, tachycardia HR 130 bpm and looks pale. Estimated blood loss at this point is 1000 mL. What is your differential diagnosis and immediate management?.
- Differentials:
- Uterine atony
- ongoing concealed haemorrhage (broad ligament, retroperitoneal)
- VAE / AFE / PE
- high spinal
- anaphylaxis
- Initial management:
- ABCD approach
- Check reading is accurate
- Call the extra anaesthetist in OT for help
- Left lateral tilt, IV fluid resuscitation
- Activate massive transfusion protocol (MTP) if bleeding suspected
- PRN vasopressors
Mary is conscious and complains of nausea. Her other vital signs are normal. The spinal block to ice is T4 bilaterally. There is no tingling or numbness in her arms. She is not short of breath. There is no rash. Metaraminol infusion (0.5mg/mL) started post CSE is now at 14mL/h (from 6mL/h) and now BP is 75/38 mmHg, HR 140. The second surgical suction cannister is now filling with more blood. What further actions would you take?
The most likely diagnosis is severe PPH from surgical trauma. EBL is likely greater than what is measured
Request surgeons to reinspect / explore for concealed bleeding and control haemorrhage
Temporise:
- Tranexamic acid 1g bolus and 1g over 8h
- bolus 1L CSL
Urgent request for PRBC (e.g. 4 units) and Cryoprecipitate (e.g. 4 - 8 units) or Fibrinogen concentrate. Anticipate massive transfusion protocol may be required.
Tests: ABG/VBG, ROTEM and formal Hb, coagulation panel and repeat vWF and factor 8 levels.
Request desmopressin in room and consider redosing (especially if >6h)
Consult haematology for further specific advice to vWD in this setting
If any of the above is not mentioned, prompt candidate appropriately.
The PRBCs and vials of fibrinogen concentrate arrive. As you start administering the products the surgeon reports he has found bleeding from an aberrant uterine arterial branch and is able to ligate it. Over the next 15 minutes, Mary’s BP and HR start to normalise. The uterus and abdomen are closed without further complications.
In the post-natal ward, ~ 3 hours post-op, the patient develops severe back pain and is screaming for her regular tapentadol. You are called urgently to review. What are your concerns and how would you manage?
Expected discussion points:
Differential Diagnoses
Spinal / Epidural haematoma (aka Vertebral Canal Haematoma) resulting in compressive myleopathy
- High suspicion due to coagulopathy even if vWF topped-up with desmopressin
Arachnoiditis
Rebound acute on chronic pelvic pain post offset of regional block
Epidural abscess
- very unlikely to form so rapidly
Management
Rule out serious complication
Full neurological history and examination - any unreported symptoms during CSE insertion or surgery
Document neurology and timing of symptoms carefully
Urgent CT/MRI spine – time critical
Notify Neurosurgery early - aim for decompression of haematoma within 8 - 12h from symptom onset if proven
Withhold / stop anticoagulants e.g. VTE prophylaxis
Retest vWF levels, FBC and coagulation profile urgently
Temporise pain: IV morphine (any opioid is fine) and reassess response. Chart regular analgesia. Consider ketamine infusion as next escalation.
Candidate must demonstrate understanding of time-critical nature for the MRI as decompression surgery is time-critical for maximising neurological recovery
Mary’s pain improves slightly after analgesia, at least enough to talk full sentences but is still grimacing. She did not have paraesthesia during CSE insertion and was comfortable in her back and legs during surgery. Her back pain is most intense in her lumbar spine and spreads hot electric shocks down her legs. She now feels intense tingling and crawling sensation in her left leg. Motor block is still present.
How does this information change your assessment?
Vertebral Canal Haematoma (VCH) remains likely especially given history of vWD
Arachnoiditis cannot be ruled out
Exacerbation of chronic pelvic pain is less likely
MRI needs to be prioritised.
An urgent MRI is organised in conjunction with Neurosurgical consult. The MRI confirms a compressive haematoma. What are the key factors that influence the patient’s prognosis?
Expected discussion points:
●The time span between hematoma formation and surgical decompression
●The speed with which the hematoma develops
●The severity of the preoperative neurologic deficit
●The size of the hematoma
AFTERMATH QUESITONS
Mary underwent an emergency laminectomy/decompressive surgery. What steps would you have taken to mitigate the risk of this complication?
Expected discussion points:
Even with usual standard of care for central neuraxial block for LSCS the risk of VCH is very low ~ 1:200,000
Steps of mitigation specific for this case:
- Thorough pre-op risk-benefit discussion and documentation with specific regard to VCH
- Consideration of GA if high bleeding risk despite correction
- Consider Use of single-shot spinal over epidural to minimise bleeding risk
- Strict adherence to coagulation thresholds and monitoring
- Pre-op and post-op trends of vWF and vWF activity (raised levels in 3rd trimester decline to below normal baseline levels within 24h post-partum)
- Post-op monitoring for signs of neurological deterioration
How would you counsel the patient regarding this anaesthetic related complication?
Honest, empathetic open disclosure
Clear explanation of what happened, how it was managed, outcomes
Involve senior colleagues as support personnel
Offer psychological support and facilitate ongoing neurology / neurosurgery input
Discuss future anaesthetic planning and implications
END OF VIVA
Source
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