1. South Thames Retrieval Service STRS Quiz No 2 Welcome 10 Question Quiz www.strs.nhs.uk

2. South Thames Retrieval Service STRS Quiz No 2 Question 1 A 6 week old male infant presented with a 2 days history of cough and fever. Increasing respiratory distress developed over the next 24 hours requiring CPAP support. Prior to intubation the team noted some abnormal movements. Admission bloods had shown Na 134 k 4.1 urea 5 creat 45 Hb 9 wbc 57 pt 521 What is the underlying diagnosis?

3. South Thames Retrieval Service STRS Quiz No 2 Answer: Pertussis Pneumonia A blood film showed a high white count with a predominate lymphocytosis and a PCR from an NPA confirmed Bordetella Pertussis infection. Unimmunized infants are at greatest risk of severe pertussis infection. Both pneumonia and a high lymphocyte count (>100) are associated with risk of death. Bacterial toxin stimulates the production of large numbers of highly activated lymphocytes. The white count can rise extremely quickly and may herald the onset of multiorgan failure. Treatment is supportive although attempts to reduce the white cell count by exchange transfusion or leucopheresis may be employed. Contacts should receive a macrolide antibiotic such as erythromicin to minimize further spread.

4. South Thames Retrieval Service STRS Quiz No 2 Question 2 A 6 month old baby presents unwell to his local emergency department after a 2 day history of mild fever and vomiting. He has a diagnosis of congenitally corrected transposition of the great arteries. He is grey. His respiratory rate is 80 and he is grunting. Central pulses are palpable at a rate of 40-50. He is awake. What does his ECG show? What immediate actions would you take?

5. South Thames Retrieval Service STRS Quiz No 2 Answer: Complete heart block It is not unusual for children with congenital heart disease to present unwell to their local hospital. It is extremely important that resuscitation follows the standard ABC approach, irrespective of diagnosis. This baby is clearly shocked. Initial actions are A/B face mask oxygen + fast bleep anaesthetist C iv access (IO if unsuccessful)/ bolus fluid 20mls/kg Reassess Intubate and ventilate for cardiac support Liaise with STRS and paediatric cardiologist. As is often the case, ventilation improved the child’s clinical condition markedly allowing his safe transfer for further intervention.

6. South Thames Retrieval Service STRS Quiz No 2 Question 3 A baby is noted to be dusky soon after birth. Saturations read 75% on the left foot and 55% on the right hand. There is little response to high flow oxygen and the baby is intubated and commenced on a prostin infusion of 20 ng/kg/min. Saturations in the right hand remain less than 60% irrespective of ventilatory changes and the baby becomes increasingly acidotic. What is the diagnosis?

7. South Thames Retrieval Service STRS Quiz No 2 Answer: Transposition with intact ventricular septum (TGA/IVS) Differential saturation between pre and post-ductal sites is an important clinical sign. Upper limb desaturation, as in this case, can only occur with TGA. TGA requires adequate mixing of systemic and pulmonary venous blood either at atrial or ventricular level for well being. If this does not occur, for example with an intact ventricular septum and an insufficient atrial communication, the baby retains low pre-ductal saturations and can become extremely sick. The only option is urgent transfer (ventilated and on prostin) to a cardiac PICU for balloon septosomy.

8. South Thames Retrieval Service STRS Quiz No 2 Question 4 A 7 year old boy fell off his bicycle at speed earlier today. He is said to have lost consciousness for a couple of minutes and then came round to ‘normal’. He is admitted to your emergency department after a focal seizure and remains drowsy. What does his scan show?

9. South Thames Retrieval Service STRS Quiz No 2 Answer:Extradural bleed with mass effect. This is a neurosurgical emergency and hence the priority is to get this child to a neurosurgical centre as soon as possible for evacuation of the haematoma. The child should be intubated and ventilated (preferably before the scan given the history and the level of consciousness) and transferred by the local team.

10. South Thames Retrieval Service STRS Quiz No 2 Question 5 A 10 year old girl presents with wheeze. Up to 3 weeks ago she has been fit and well with no respiratory symptoms. She is given a salbutamol nebulizer and iv hydrocortisone with little effect. Four hours later she suffers a VF arrest. What does her CXR show and what do you think is the cause of her arrest?

11. South Thames Retrieval Service STRS Quiz No 2 Answer: Widened mediastinum and bilateral hyperinflation / hyperkalaemia secondary to tumour lysis. First presentation of wheeze in a 10 year old should raise suspicions, particularly when unresponsive to salbutamol. The wide mediastinal shadow was caused by fast growing lymphoma which caused tracheobronchial compression and hence her symptoms. This can be very difficult to manage as sedation or anaesthetic (particularly muscle relaxation) can severely exacerbate airway compression. These tumours are highly responsive to steroids and in this case tumour lysis led to an acute rise in potassium and a VF arrest.

12. South Thames Retrieval Service STRS Quiz No 2 Question 6 A 6 week old girl presents after a short apnoeic episode at home. Prior to this she had a mild fever and upper respiratory tract symptoms for 2 days. Mum thought the baby had shown some jerking on the left side earlier in the day that settled spontaneously. In A+E she has a series of short apnoeas and then a generalized seizure. What is the likely cause of the seizures? What is the most likely underlying diagnosis? Results CXR hyperinflated Hb 11 wbc 8 platelets 350 CRP <5 Blood glucose 5mmol/L Na 122 K 4.5 U 2.3

13. South Thames Retrieval Service STRS Quiz No 2 Answer: Hyponatraemia causing seizures secondary to RSV bronchiolitis. Hyponatraemia is the commonest electrolyte abnormality in hospital. It is extremely common in babies with RSV bronchiolitis and may occasionally cause seizures. More often than not the baby will have received hypotonic intravenous fluid but occasionally, as with this child, it occurs in a child receiving normal oral feeds. Acute symptomatic hyponatraemia must be treated urgently. The baby was given 3 mls/kg of hypertonic saline and intubated and ventilated. Thereafter she was restricted to 2 mls/kg/hr of NG feeds and her plasma sodium returned to the normal range within 48 hours. She was later discharged well from PICU.

14. South Thames Retrieval Service STRS Quiz No 2 Question 7 A 2 year old boy develops a reddened area around a chicken pox lesion. He is given flucloxacillin by his GP but attends A+E with vomiting and diarrhoea the next day. He has a fever of 38.5C and is listless. The infected spot is tender to touch and the skin slightly discoloured around it. You perform some baseline blood tests with results as shown. What is the most likely problem? Results Na 129 K 4.5 urea 12 creat 80 Hb 12 wbc 22 platelets 122

15. South Thames Retrieval Service STRS Quiz No 2 Answer: Toxic shock syndrome (TSS) +/- necrotising fasciitis Bacterial complications of varicella infection are not uncommon. Likely pathogens are staph aureus and group A streptococcus (GAS) both of which can cause toxic shock. GAS may also produce a local destructive infection-necrotising fasciitis- which develops extremely quickly and deceptively. The infection spreads underneath the skin in the fascia causing destruction of soft tissues and muscle as it goes. Mild gastrointestinal symptoms commonly precede presentation with TSS and this child’s blood tests show renal impairment, hyponatraemia and low platelets indicating multiorgan dysfunction. Management includes aggressive treatment of shock, antibiotics (including clindamycin) and URGENT review by a plastic surgeon of any localised infection.

16. South Thames Retrieval Service STRS Quiz No 2 Question 8 Results Na 132 urea 10 creat 127 glucose 14.7 mmol/L ALT 930 A 5 day old baby has been off feeds for 12 hours and referred up to your hospital by the GP because he looks pale. He is grey on arrival and grunting. His heart rate is 190 and blood pressure 110/80. Oxygen is given by mask and his saturations are 100%. You notice his liver is enlarged to the umbilicus. Some of the blood tests are shown. What crucial clinical sign should be looked for?

17. South Thames Retrieval Service STRS Quiz No 2 Answer: Femoral pulses / 4 limb blood pressure A baby who presents collapsed in the first few days of life is likely to have sepsis or a duct dependent cardiac condition. Examination of the pulses is essential (although there were many other things you might like to ask as well!). In reality it can be difficult in the very sick neonate to make a diagnosis clinically and therefore all life sustaining therapies should be commenced without hesitation. The priorities for this baby are respiratory support, antibiotics and a prostin infusion (to open the duct) This baby had a severe coarctation of the aorta and multiorgan failure. He improved markedly on prostin and support allowing surgical correction and complete recovery within a few days of presentation.

18. South Thames Retrieval Service STRS Quiz No 2 Question 9 A 3 week old baby presents after a short history of pallor and vomiting to your department. She is grey and sweaty. Her heart rate is well over 200 and you can barely feel peripheral pulses. Her temperature is 36.7C. What does her ECG strip show and what actions should you take?

19. South Thames Retrieval Service STRS Quiz No 2 Answer: Supraventricular tachycardia (SVT) This baby has a narrow complex tachycardia without discernable p waves. She is shocked. Infants do not tolerate SVT well and the priority is urgent cardioversion and organ support. She needs oxygen by mask and urgent iv (or IO access). The anaesthetic team should be contacted urgently but whilst awaiting them adenosine can be given (100mcg/kg, with escalation up to 500mcg/kg in increments of 100mcg/kg). These high doses are often required in neonates. Please run the ECG strip during any attempt at cardioversion. Amiodarone may be required if SVT persists. If shock persists, the baby must be anaesthetised, intubated and ventilated and then cardioverted (synchronised shock starting at 1 j/kg).

20. South Thames Retrieval Service STRS Quiz No 2 Question 10 A 2 year old boy presents with a 3 day history of breathing difficulties and vomiting. He is drowsy and looks dehydrated. What is the diagnosis? What is the most serious complication of this condition? Results Arterial blood gas pH 7.0 pCO2 1.5 SBE – 25 mmol/L Blood sugar 35 mmol/L

21. South Thames Retrieval Service STRS Quiz No 2 Answer: Diabetic ketoacidosis The triad of hyperglycaemia, metabolic acidosis and ketosis is the hall mark of DKA. Severe dehydration results from an osmotic diuresis and often in retrospect the child will have had the classic symptoms of polyuria and polydipsia for sometime. It is not uncommon for the hyperventilation of DKA to be misdiagnosed initially as asthma. Management is aimed at SLOW rehydration (this has not happened overnight!) and initiation of insulin therapy. The most serious complication that may develop over the first 24 hours of therapy is cerebral oedema and close neurological observation is mandatory. Risk factors for cerebral oedema include young age, severe acidosis, excessive fluid therapy in the first 4 hours and raised urea at presentation.

22. South Thames Retrieval Service STRS Quiz No 2 End of quiz