Dysautonomia, uncertain aetiology

29F with cerebral palsy for revision of baclofen pump

Background:

  • Cerebral palsy
    • Non-verbal
    • Mobilises with electric wheelchair, requires a carer to operate
    • Severe spasticity and contractures involving all four limbs
    • Significant scoliosis and restrictive lung disease
    • Gastrostomy tube for feeding
    • Central sleep disordered breathing?
      • Abnormal breathing pattern – crescendo/decrescendo pattern
      • Assumed to have significant restrictive lung disease. No formal spirometry
      • Recent overnight oximetry showed SaO2 maintained >90%

Issues:

  • >1 year of episodic flushing, diaphoresis, tachycardia, tachypnoea 
    • Autonomic dysfunction?
    • Variable severity and duration of symptoms
    • Baseline tachycardia 110bpm (previous HR 100 so may be chronic) 
  • Differentials include: 
    • Central dysautonomia – potential syrinx development  given cerebral pathophysiology
    • Hyperthyroidism 
    • Phaeochromotcytoma
    • Baclofen withdrawal due to malfunctioning pump
    • Pain and Anxiety component?
    • Atropine – PO secretions, episodes don’t appear to correlate with dose
    • Subclinical seizure activity

Discussion:

Perioperative Optimisation

  • Exclude hyperthyroidism and pheochromocytoma preoperatively
  • Co-ordinating investigations while under GA 
    • MRI – can be performed safely with baclofen pump?
    • Discussed with rehabilitation physician  – all baclofen pumps are MRI compatible, however, the magnetic field and associated increased temperature can cause the pump to malfunction. See attached article
  • Consider change atropine to glycopyrrolate

Plan:

  • Pathology testing for TFT’s, plasma, and urine catecholamines
  • Consider MRI under GA 
  • Discuss with treating teams – can we perform any further investigations while under GA/inpatient

Endoscopies, multimorbidity, advanced age

84 yo lady for gastroscopy and colonoscopy

Background

  • CVA – presumed cardioembolic
  • PAF – DOAC
  • Fe deficiency anaemia – chronic
    • AV malformation in small bowel
    • Managed with iron infusions
  • CKD – stage 1
  • Intracerebral aneurysm – under surveillance
  • IHD – stented

Issues

  • Melaena and anaemia – DOAC for CVA. Hb drop from 112 to 53.
  • Previous gas/colon showed healed antral ulcer. 
    • Complicated by hypotension. Treated IVF and vasopressors. 
    • Required ICU for fluid overload.
    • Patient doesn’t want to have procedure – worried about having further cardiac failure, stroke, and ICU admission.
    • Patient concerns reasonable, unclear cause of previous event

Discussion

Proceed to surgery?

  • Reasonable to proceed to gastroscopy –
    • low risk for fluid overload. 
  • Colonoscopy – indication unclear
  • More fluid shifts involved.
  • RFA indication for colonoscopy states haemorrhoids
    • Consider less invasive investigations? Eg. proctoscopy/flexible sigmoidoscopy

Plan

  • Proceed with gastroscopy only at this stage – avoid bowel prep and significant fluid shifts
  • Liaise with surgical team regarding colocoscopy

Cholecystectomy – multimorbidity

70 y/o M with recurrent gallstone pancreatitis. Several other episodes this year, not requiring hospitalisation.

Background:

  • OSA- occasional CPAP compliance
  • NYHA- Class III for breathlessness. TTE – nil significant issues detected.
  • BMI 40
  • ESRF- haemodialysis 3 times a week 
    • 500ml fluid restriction (oliguric)
    • Secondary chronic anaemia, using an ESA + Fe
  • R) nephrectomy 2022 – postoperative ICU admission for vasopressor support and hyperkalemia management
  • 4WW, mobility scooter for longer distances. Reduced exercise tolerance since ICU admission.

Issues:

  • Functional decline
  • Multimorbidity

Discussion:

  • Perioperative risks: NSQUIP: 6.4% serious, 0.8% risk of death, 26 % functional decline 
  • Conservative management carries significant risks with recurrent episodes of gallstone pancreatitis
  • Patient motivated for pre-habilitation 

Plan:

  • Pre-habilitation 6 weeks
  • Dietitian review ongoing (BMI ) through dialysis

Complex lower limb vascular recon

56 y/o M  with critical left leg ischemia. Possible procedure would be a very long, very complex, multi-faceted reconstruction. Conservative management would eventually result in AKA which may be complicated by poor wound healing due to ongoing vascular compromise.

Background

  • CLL- WCC 38
  • Smoker – cigarettes currently 5/d (recently ceased marijuana)
  • Exercise Tolerance- DASI 3.94 METs – Limited by acute limb pain 

Issues:

  • Cardiovascular – Reduced exercise tolerance, ? prior non-transmural infarct in RCA territory on sestamibi (but nil reversible ischaemia). TTE pending.
  • Ongoing smoking – multiple strategies discussed in clinic and NRT provided

Discussion:

  • Optimisation opportunities preoperatively – smoking only
  • Conservative management is a poor option 

Plan:

  • Await TTE Report
  • Smoking Cessation 
  • Likely proceed to surgery given poor conservative mx option

Open AAA – Juxtarenal

77 y/o F with 5.5cm juxtarenal AAA- complex EVAR therefore preference for open procedure, and recent increase in size.

Patient’s sister died from AAA rupture leading to significant patient anxiety and exercise avoidance.

Background

  • Dyslipidaemia
  • Current Smoker- 75 year pack history. Reduced – currently 3/day. Normal spiro.
  • CFS: 4

Issues

  • Elevated risk CPET results – low AT (8.9ml/kg/min), acceptable peak VO2 (16.4). 

Discussion

  • Absence of respiratory issues (beyond residual smoking) to deter from open procedure
  • BP remained within target range for known AAA (<180 systolic) during CPET suggesting prehabilitative exercise will be feasible
  • Patient engaged in planning prehabilitation. Previously active so understands the feel/goals of activity. 

Plan

  • Pre-habilitation 4-6 weeks, no need to repeat CPET 

Proceed to open procedure

Caesarean Section – JW

37 y/o F  for electives LSCS and tubal ligation

Background

  • Previous LSCS with spinal 
  • Current Pregnancy- gestational Hypertension (on Nifedipine) and elevated LFTS and proteinuria (concern for evolving HELP Syndrome) 

Issues

  • Jehovah’s Witness
  • Product discussion with patient and documented would accept: Cell salvage, FFP And cryoprecipitate

Discussion

  • For re-discussion on day of procedure with patient (confidentially) 
  • Patient wishes should be determined in absence of partner or church representative presence

Plan

  • Planned procedure Wed 1/6 (Procedural anaesthetist at meeting and aware)

Thoracotomy – Patient declining op

73y/o F for Left thoracotomy and biopsy for para-aortic left node (PET avid). Not EBUS amenable

Recent Admission with proximal myopathy (improved with steroids) and PET AVID lesion noted – ? paraneoplastic syndrome causing weakness.

Background

  • ? Scleroderma/Dermatomyositis (Recent reviews by Rheumatology- still undergoing investigations)
  • AF – apixaban
  • HTN
  • DASI METS < 4
  • CFS 6
  • COPD
    • FEV1 0.94 , FEV1 1.8, DLCO 39%
    • Exacerbation in Feb 23
    • 96% on RA, hyperinflation of chest on examination 

Issues

  • Patient not keen for procedure due to concern about high risks
  • NSQUIP – Risk of death >5%; 12% complications, Readmission 13%
  • Severe COPD, likely not optimizable

Discussion

  • ? natural history of disease once tissue diagnosis is obtained
  • ? options for treatment of presumed cancer without tissue diagnosis
  • ? risk of anaesthesia and procedure outweighs benefit
  • Respecting patient autonomy 
  • Options for less invasive surgery?

Plan

  • To Discuss with Oncology team re ? option for empirical therapy due to the high risks, ? likely natural history without treatment
  • Discuss with rheum/onc – ? myopathy due to dermatomyositis (not cancer), ? other non-cancerous diagnoses possible for the lymph node
  • To discuss with surgical team – is a thoracotomy required for a tissue diagnosis or is there an alternative option

Obesity and Reverse Total Shoulder Replacement

68 y/o F for Reverse total shoulder replacement

Background:

  • BMI 60
  • Diabetes :
    • HBAIC 7.8% (from 10.6)  -Improved control 
  • CKD : eGFR 52
  • HTN
  • OSA- CPAP compliant
  • Echo- moderate diastolic dysfunction
  • Poor mobility – FASF, weekly physiotherapy and hydrotherapy
  • DASI- Mets:3.63

Issues

  • Exercise Tolerance reduced: SOB on arrival to clinic
  • Poor diabetes control
  • Opioid tolerant (30 BD oxycontin)
  • High BMI

Discussion:

Proceed versus alternative therapy (Steroid injection/ Nerve blocks)

  • Weight loss would assist pain and potentially avoid the need for surgery (note other shoulder becoming more problematic – ? due to FASF)
  • Consideration of non-surgical management options for pain 
  • Surgical team unaware of FASF use – ? will be able to use FASF after surgery, ? patient safe to mobilise without it

Plan

  • Delay for 3 months
  • Weight loss – Dietitian referral to free clinic 
  • Contact GP re. iron replacement and GLP-1 antagonist (weight loss + DM optimisation)
  • Exercise physiologist to optimise exercise tolerance and mobility – ? avoid need for FASF
  • If surgery proceeds, would need ICU level 2 given risk of respiratory compromise with OSA and phrenic nerve block