A case of severe thrombocytopenia during pregnancy:
diagnostic dilemma and multidisciplinary approach
Salota, Vishal; Sharafudeen, Saju; Skelton, Vanessa
King's College Hospital, London
Thrombocytopenia (platelet count of <100×109/L) is the second most common
haematological abnormality during pregnancy1,2 with an incidence of 7-8% of
all pregnancies3. Majority of causes are easily understood. However, various
aetiologies can make it difficult to diagnose in certain cases. Diagnosis carries
importance as this determines outcome based on whether or not a platelet
transfusion can be safely carried out. Management requires a multidisciplinary approach.
Description
A 30 year old healthy primiparous full term woman delivered a healthy baby
by assisted ventouse delivery. She had an epidural for pain relief. Estimated
blood loss was about 1.2 L. Haemodyamic parameters remained stable and
patient was kept in the high dependency unit for further observation. Her
antenatal bloods were all normal, Hb 136 g/L and platelet count (PLT) of
148×109/L. Immediately after delivery, inspite of adequate fluid
resuscitation, she developed acute kidney injury and haemolysis. Hb at the
nadir on day 6 post-partum was 57 g/L with lowest PLT of 16×109/L.
Creatinine increased to a peak of 213 µmol/L. Liver enzymes remained
normal apart from a modest increase in AST to 87 U/L, elevated lactate
dehydrogenase (LDH) levels (peak of 3339 iu/L) with haptoglobin of < 0.1 g/L.
Liver and renal ultrasound remained normal. She had blood 3+ and urine
protein 2+ in urine dipsticks. Blood film showed numerous red cell fragments.
She did not show any neurological symptoms. Following discussion with
haematology team, a diagnosis of microangiopathic haemolytic anaemia
(MAHA), probably atypical HELLP syndrome (haemolysis, elevated liver
enzymes, low platelets) was made. ADAMST-13 test (a disintegrin and
metalloproteinase with a thrombospondin type1 motif, member 13) was
performed to exclude thrombotic thrombocytopenic purpura (TTP). She
received multiple red cell transfusions and a single pool of PLT with little
effect. Therapeutic plasma exchanges were subsequently initiated on day 5
post-partum. She underwent four plasma exchanges with excellent
response and was discharged on day 12 with recommendation to take ferrous
fumarate and folic acid. One week after discharge, her Hb increased to 102
g/L, PLT 338×109/L with normal haptoglobin and LDH levels. She made a
complete recovery.
Figure 1 Trend of haemoglobin and platelet count during pre and post-partum
period
Discussion
The majority of women with HELLP syndrome have hypertension and
proteinuria which may be absent in 10–20% of cases - referred to as atypical
HELLP. These patients may present with gastrointestinal, respiratory, or
hematologic symptoms risking initial misdiagnosis. Management includes:1.
Obstetric management: Treatment is delivery, however, if patient is < 34
weeks gestation corticosteroids can be administered to accelerate fetal lung
maturity. Magnesium sulphate (to prevent seizures) and antihypertensives
may be required. 2. Hematologic management: Corticosteroids may improve
the PLT count.There is no contraindication to PLT transfusion or other blood
products. If patient continuous to worsen 48-72 hours postpartum, therapeutic
plasma exchange should be considered.
References
1. Shehata N, Burrows R, Kelton JG. Gestational thrombocytopenia. Clinical
Obstetrics and Gynecology. 1999 Jun; 42(2):327-34.
2. Sullivan CA, Martin JN Jr. Management of the obstetric patient with
thrombocytopenia. Clinical Obstetrics and Gynecology. 1995 Sep; 38(3):52134.
3. Kadir RA, Mc Lintock C. Thrombocytopenia and disorders of platelet
function in pregnancy. Seminars in Thrombosis and Hemostasis. 2011
Sep;37(6):640-52.
4. Sibai BM. The HELLP Syndrome: much ado about nothing? American
Journal of Obstetrics and Gynecology 1990;162:311- 6.
5. Gernsheimer T, James AH, Stasi R, How I treat thrombocytopenia in
pregnancy. Blood. 2013;121(1):38-47
Anaesthetic Management of a 13-month-old child with medium chain Acyl CoA
dehydrogenase deficiency (MCADD) in a district general hospital.
V Salota1,3, R Salota2 and C Chapman1
1 Queen Elizabeth The Queen Mother Hospital, Margate, Kent; 2 St Helier
Hospital, Surrey; 3 King’s College Hospital, Denmark Hill, London.
Introduction
MCADD is an inherited fatty acid oxidation disorder with incidence of 1:10,000 births
among Caucasians. Fasting periods are poorly tolerated with risk of serious
hypoglycaemia. We report an interesting case of a 13-month-old child with this
condition undergoing major orthopaedic surgery.
Case description
A 13-month-old child with MCADD (weight 11.3 kg) was posted for femoral
osteotomy. Considering the high risk of hypoglycaemia, she was admitted night
before surgery and was started on intravenous drip of 10% dextrose with 2.25 gm
sodium. Oral midazolam was given as premedication. She underwent gas induction
with sevoflurane and airway was secured with LMA. Intra-operatively, caudal
epidural with 0.25% levo-bupivacaine and clonidine was performed. Anaesthesia
was uneventful. Regular blood gas and glucose levels remained within normal limits.
The dextrose sodium infusion was continued for further 9 hours until normal feeding
was commenced. Blood glucose was monitored for 6 hours. She was discharged
home following day, on regular paracetamol and ibuprofen.
.
Discussion
Fatty acid beta-oxidation provides energy after the body has used up its stores
of glucose and glycogen. Defective oxidation of fatty acid in MCADD patients results
in inability to switch to fatty acid metabolism during periods of starvation or increased
requirement hence continued metabolism of glucose ultimately lead to profound
hypoglycaemia.1 Lack of acetyl CoA also affects hepatic ketogenesis. Clinically,
MCADD patients may present with lethargy, seizures, coma and hypoketotic
hypoglycemia, particularly triggered by a minor illness; acute liver disease
and hepatomegaly which can lead to a misdiagnosis of Reye syndrome. In some
individuals, the only manifestation of MCADD is sudden, unexplained death often
preceded by a minor illness that would not usually be fatal.
Goal of anaesthesia therefore, is to minimise ongoing energy requirements by
reducing stress while providing adequate calories in the form of carbohydrate. This
was achieved by using premedication, gas induction rather than repeated
cannulation, avoiding intubation and caudal block with clonidine. Adequate provision
for their particular metabolic needs would allow to safely undertake major procedures
even outside the tertiary centres.
References
1. Ewout JH,Geany D,Robb M,Mitchell L, Bohn H, Bohn D , Paediatrics
2004;113;1279
Brainstem Anaesthesia Presenting as a Fifth and Seventh Cranial
Nerve Palsy Following a Peribulbar Nerve Block for Cataract and
Glaucoma Surgery
Strowbridge S, Milroy S. Department of Anaesthesia, Kings College
Hospital, UK March 2015
Ophthalmic blocks are safe and effective options for eye surgery. The
safety profile of ‘blunt needle’ subtenon blocks is preferable.
However, for complex ophthalmic surgery such as trabeculectomy
and gas injection, the peribulbar block (PB) provides dense and
reliable akinesis and analgesia. These techniques should only be
performed after discussion with the surgical team and patient
assessment. Brainstem anaesthesia is a serious but recognised
complication of these techniques. We present a case of fifth and
seventh cranial nerve blockade after performing a PB for ophthalmic
surgery.
An ASA 2, 61year old female presented for cataract
phacoemulsification, lens implantation and goniosynechialysis.
Preoperative assessment was unremarkable. Optometry revealed
normal axial length but elevated intraocular pressure, treated with
intravenous acetazolamide. A PB was performed with local
anaesthetic (2% lignocaine & 0.5% bupivacaine) and 150i.u
hyaluronidase. 7mls was injected via a transdermal approach inferolaterally. The patient developed immediate, transient agitation lasting
for approximately 10 seconds. She remained haemodynamically
stable. No ptosis or akinesia was noted, and a further 3ml medial
canthus top up was administered.
Post-procedure, the patient developed features consistent with an
ipsilateral motor facial nerve and sensory trigeminal nerve
distribution palsy. Haemodynamic and respiratory parameters
remained stable. Surgery was expedited and completed uneventfully.
3 hours postoperatively, the neurological deficits had resolved fully.
Further review on day 1 postoperatively was unremarkable.
Although rare, brainstem anaesthesia is a recognised complication of
PB. These techniques should be performed by an experienced
practitioner, with intravenous access, monitoring and the facility to
provide ventilatory and haemodynamic support.
References for poster
1. Carneiro H, Oliveira B, Ávila M, Neto O, Brainstem Anesthesia after Extraconal
Retrobulbar block. Can it be avoided? Case report. Rev Bras Anestesiol
2007;57(4):391-400.
2. Loken R, Mervyn Kirker GE, Hamilton RC: Respiratory arrest following peribulbar
anesthesia for cataract surgery: Case report and review of the literature. Can J
Ophthalmol 1998;33:225-226.
3. Roman SJ, Chong Sit DA, Boureau CM, Auclin FX, Ullern MM: Sub-Tenon's
anaesthesia: an efficient and safe technique. Br J Ophthalmol 1997;81:673–676.
4. Singer SB, Preston R, Hodge WG: Respiratory arrest following peribulbar anesthesia
for cataract surgery: Case report and review of literature. Can J Ophthalmol
1997;32:450-454.
Are we achieving recommended targets with labour
analgesia: what happens after 2 hours of epidural
use and how to improve?
Salota, Vishal; Sharafudeen, Saju; Fleming, Ian; Skelton, Vanessa
King's College Hospital, London
PCEA is a widely accepted technique for labour pain relief. We currently use a
bolus only regime with plain bupivacaine (0.1%) and fentanyl (2mcg/L). 8 mL
boluses are delivered every 20 minutes using GemStar™ infusion systems
(GIS). RCoA recommendations include re-siting or alternative form of
analgesia if epidural fails to provide pain relief within 45 minutes. Patient
satisfaction ≥ 98% is considered acceptable1. Our previously presented audit
showed significant motor blockade and less breakthrough pain with a
background only regime in 20102. We audited the quality of analgesia after 2
hours of initiation of our bolus only protocol and compared the results with the
prevoius outcomes.
Methods
King’s College Hospital is a teaching hospital with 7000 deliveries. After
internal governance approval, we included consecutive 52 patients who had
epidural for labour pain relief over a period of 3 weeks. Patients with epidurals
for less than 2 hours were excluded. We looked at incidence and frequency of
breakthrough pain, unilateral motor block, use of other mode of pain relief and
requests for manual top-ups. Patient satisfaction was assessed using a NRS
of 0-10.
Results
All patients experienced good pain relief following initial manual bolus of 15ml
low dose mixture. 26.9% patients experienced breakthrough pain, 23%
requiring 1 or more manual top-ups after 2 hours. 91.6% manual top-ups were
delivered within 30 minutes of the request. 13.4% patients had dense motor
block (modified Bromage scale). Only 92% patients experienced satisfactory
or above pain relief.
Figure 1 Comparison of breakthrough pain & motor blockade with background
infusion & bolus only regime.
Discussion
Bolus only regimes are known for higher incidence of breakthrough pain
compared to those with background infusions. However, background infusions
cause significant motor blockade. Factors which may contribute to the
increased incidence of breakthrough pain include lateral positioning, low
pressure boluses delivered by pumps, or delay in the request for the bolus.
Recently, MHRA has issued a medical device alert related to GIS 3. These
faults may lead to under performances of the pump such as over-infusion,
under infusion or calibration errors3. Our audit showed a significant proportion
of patients experienced breakthrough pain after initial loading dose. This had
an impact on satisfaction scores. However, we still achieve a significantly less
motor blockade with the bolus only regime. Our unit is currently considering
an alternative epidural pump which can deliver bolus with higher pressure
(350-1100 mmHg), which is adjustable. We will re-audit the performance of
new device.
References
1. Royal College of Anaesthetists - Raising the Standard: a compendium of
audit recipes - 3rd Edition 2012.
2. Sharafudeen, S.; Barry,
A.; Nicholson, C.; Skelton, V.; Fleming, I. PCEA labour analgesia: Are we
better with or without background infusion? European Journal of
Anaesthesiology:12 June 2010 - Volume 27 - Issue 47 - p 163-164 Obstetric
Anaesthesia. 3. Medicines & Healthcare products Regulatory Agency:
(Hospira) Due to pump faults. (MDA/2013/016).
Safe combined spinal epidural for a patient with Takayasu’s
arteritis for elective Caesarean section
Esprit S.A1 & Kirby S2.
1. Obstetric anaesthesia fellow, 2. Consultant Anaesthetist
Queen Charlotte’s Hospital, London
Takayasu's arteritis (TA) is a rare chronic progressive
panendarteritis involving the aorta and its main branches (1) often
complicated by severe uncontrolled hypertension, with end-organ
dysfunction, and stenosis of major blood vessels affecting regional
circulation (1). It is vital to control the blood pressure of such
patients because TA is often associated with arterial aneurysms,
which may rupture when exposed to high blood pressure and
cerebral dysfunction (2), so low blood pressure may lead to cerebral
ischaemia. The sympathetic block that occurs with regional
anaesthesia may result in hypotension, however it is easier to
monitor cerebral function in awake patients (2) and regional
anaesthesia is the preferred technique in pregnant women.
Description
A 27year old woman, who had been diagnosed with TA three years
earlier, presented for elective Caesarean section at 38weeks'
gestation in a spontaneous pregnancy. During the pregnancy she
had been experiencing worsening chest pain and claudication in all
four limbs, with left upper limb pulses unpalpable on examination.
Recent CT scans had revealed left subclavian artery stenosis,
coeliac axis stenosis, aortitis and thickening of the aortic arch up to
the left subclavian artery and pulmonary artery inflammation. A
trans-thoracic echocardiogram revealed good biventricular systolic
function and no valvular heart disease. The disease process was
controlled with azathioprine and prednisiolone. A combined spinalepidural was performed at L3/4 space with a spinal dose of 10mg of
heavy bupivicaine and 25mcg of fentanyl. 50mg of hydrocortisone
was given intravenously. A phenylephrine infusion was started and
continued until the blood pressure stabilised. The rate of the
phenylephrine infusion was commenced at 2mg per hour, standard
practice in our department. After five minutes it was necessary to
increase the infusion rate to 3mg per hour, then 4mg per hour in
order to maintain her blood pressure at 110/70. An arterial line was
not inserted due to the risk of compromising arterial blood supply to
the upper limb further. Non-invasive measurement of blood
pressures was adequate throughout the Caesarean section. The
epidural was used to provide analgesia for six hours postoperatively. There were no complications from the regional
anaesthesia.
Discussion
Combined spinal-epidural is a safe technique for regional
anaesthesia in women with TA undergoing Caesarean section.
Maintenance of normotension is crucial to avoid complications from
the vascular abnormalities, in our patient more phenylephrine than
normal was needed.
Acknowledgment
The patient is aware of this case report and has signed a consent
form.
References
1. Kathirvel, S., Chavan, S., Arya, V. K. et al. Anesthetic management of patients with
Takayasu’s arteritis: a case series and review. Anesthesia & Analgesia, (2001) 93(1),
60-65.
2.Beilin, Y, and Berstein H. "Successful epidural anaesthesia for a patient with
Takayasu’s arteritis presenting for caesarean section." Canadian journal of
anaesthesia 40, no. 1 (1993): 64-66.
Patient monitoring during epidural labour
analgesia by midwives: are we doing it
right?
Salota, Vishal; Costantini, Diletta; Sharafudeen, Saju; Dasan, Jay; Skelton, Va
nessa
King's College Hospital, London
While epidural is considered to be the most effective method of labour
analgesia, it is also the most invasive. Therefore, careful monitoring of vital
signs and block level during siting and administration of epidural analgesia is
essential to minimise the potential risks. We use a PCEA bolus only regime
with plain bupivacaine (0.1%) and fentanyl (2 mcg/mL). Current
recommendation by NICE during epidural labour analgesia is to measure
blood pressure every 5 minutes for 15 minutes during establishment of
regional analgesia or further boluses and assess the level of sensory block
hourly1. RCoA recommends monitoring heart rate and blood pressure;
respiratory rate; temperature; sedation & pain score; degree of motor &
sensory block throughout the period of epidural analgesia2.
Methods
King’s College Hospital is a large London teaching hospital with 7000
deliveries annually with a significant proportion being high risk. After internal
governance approval, medical records of 30 patients receiving epidural for
labour analgesia were retrospectively reviewed over a period of 2 weeks.
Patients who used epidurals for less than 2 hours were excluded. We
checked the documented frequency of monitoring of maternal vital parameters
including heart rate, blood pressure, respiratory rate, temperature; sedation &
pain score; degree of motor & sensory block and fetal heart rate throughout
the period of PCEA. We also surveyed 20 midwives to assess their level of
awareness of local epidural guideline.
Results
Compliance to heart rate and blood pressure monitoring after loading dose
was 100%. However, further hourly heart rate monitoring was documented in
only 33.3%, hourly blood pressure was monitored in 16.6% patients. Only
6.6% patients had documented respiratory rate, with no regular time interval.
Regular temperature monitoring was done in 86.6% patients. Sensory block
was documented in 20% patients after loading dose. Subsequently, during the
progress of labour it was documented only in 6.6% patients, with no regular
time interval. None of the patients had documented sedation score, pain score
and degree of motor block. The fetal heart rate was assessed regularly
through CTG and documented every 15 minutes in all the patients.
Figure1 Midwives survey on awareness of epidural guideline (N=20)
Disussion
Above monitoring results show poor awareness of local trust epidural
guidelines among the midwives. This was further confirmed by the survey.
Poor adherence to epidural guidelines could prevent early detection of
potentially serious complications. We aim to re-educate the midwives through
monthly teaching sessions and re-audit the progress in two months time.
References
1. Intrapartum care: care of healthy women and their babies during childbirth.
NICE guideline (CG 190), December 2014
2. Best practice in the management of epidural analgesia in the hospital
setting. RCoA, London, 2010