1. South Thames Retrieval Service STRS Quiz No 3 Welcome 10 Question Quiz www.strs.nhs.uk

2. South Thames Retrieval Service STRS Quiz No 3 Question 1 A 3 year old girl presents with severe wheeze. She responds poorly to nebulised salbutamol and atrovent. A salbutamol infusion is commenced at 1 mcg/kg/min. She is intubated for hypoxia and exhaustion. She is known to have episodes of wheeze, usually responsive to salbutamol. Comment on her CXR?

3. South Thames Retrieval Service STRS Quiz No 3 Answer: Complete white out right lung due to collapse and hyperinflation of left lung with diffuse shadowing This child has severe asthma with mucus plugging causing both hyperinflation and collapse. She was extremely hypoxic after intubation (sats 50-70% in 100% oxygen) but responded to urgent physiotherapy (and the mucolytic rhDNase) which partially inflated the right lung. This is a life threatening situation which is recognised by the lack of response to bronchodilators and increasing hypoxia (due to V/Q mismatch.) CXR post physio and rhDNase therapy on PICU

4. South Thames Retrieval Service STRS Quiz No 3 Question 2 A 1 week old baby is referred with cardiovascular collapse. She was born at term after a difficult labour. She responded to resuscitation via bag and mask and was admitted to the NICU. Her condition improved. Four days later she developed respiratory distress initially managed on CPAP. Today she has acutely deteriorated with increasing respiratory distress, tachycardia, poor perfusion and enlarged liver 6 cm. She is intubated and ventilated. On arrival of the team she looks clinically better. Saturations 100% in air, HR 160, good peripheral pulses (including femoral) BP 138/95, liver is 3 cm. Her CXR shows a slightly enlarged heart and plethoric lung fields. What is the most striking clinical finding? What is the most likely cause?

5. South Thames Retrieval Service STRS Quiz No 3 Answer: Severe hypertension (renal artery thrombosis) Neonatal hypertension is a rare cause of cardiovascular collapse in a neonate. Often, it is not until after resuscitation (in this case ventilation) that the high blood pressure is recognised. The most common cause is renovascular disease, most usually due to renal artery or aortic thrombosis. There is a strong association with the use of umbilical catheters (this baby had both UAC and UVC for 24 hours after birth.) A CT angiogram showed clot in her abdominal aorta extending into the ostia of both renal arteries and extensive venous thrombosis. Systemic thrombolysis successfully dissolved the renal clots and renal function improved.

6. South Thames Retrieval Service STRS Quiz No 3 Question 3 A 5 month old boy is admitted with a diagnosis of infective collitis. He has been seen twice in A+E over the last 2 days with vomiting. His mum brought him back when she noticed blood in his nappy (no diarrhoea). He is admitted for iv antibiotics. Comment on his XRAY? What is the most likely diagnosis?

7. South Thames Retrieval Service STRS Quiz No 3 Answer: Dilated loops of small bowel, no distal gas suggesting obstruction. Very likely intussusception This baby required resuscitation and an urgent laparotomy. An ileo-ileal intussusception was found and a small section of bowel resected. Vomiting and abdominal distension without diarrhoea requires careful consideration of a surgical cause, especially in the context of rectal bleeding.

8. South Thames Retrieval Service STRS Quiz No 3 Question 4 An 11 year old girl presents unconscious to her local hospital. She has been unwell for 2 days complaining of heavy, aching legs. On arrival, she has a respiratory arrest and is immediately intubated and ventilated. She had been admitted 2 years previously with a low blood sugar, hyponatraemia and vomiting and improved with fluid. What is the diagnosis? What clinical sign would you look for? Blood results Na 113 K 7.9 Glucose< 1 mmol/l

9. South Thames Retrieval Service STRS Quiz No 3 Answer: Addison’s disease. Hyperpigmentation It was December, yet she had a good tan including palmer creases. Since the episode 2 years ago, she had become increasingly less active and saw the GP 4 weeks. A blood test showed a sodium of 125 mmol/l. Addison’s disease is the commonest cause of primary adrenal failure at this age. Onset is slow and it is often not until adrenal hormone production disappears completely, causing the triad of low sodium, high potassium and low blood sugar that the diagnosis is made. She responded well to fluid resuscitation, dextrose and hydrocortisone acutely. On admission PICU she was found to have evidence of rhabdomyolysis (elevated creatinine kinase with myoglobinuria) which may occur in an acute Addisonian crisis and explains her aching legs.

10. South Thames Retrieval Service STRS Quiz No 3 Question 5 A 10 month old boy is referred to PICU with bronchiolitis. He had been admitted the day before with cough and respiratory distress. He has received atrovent, adrenaline and budesonide nebulisers for wheeze and stridor. At times he looks very distressed with severe recession, inspiratory stridor, wheeze and cyanosis. Other times he looks settled with minimal recession. Comment on the child’s clinical state? What specific questions would you ask his parents?

11. South Thames Retrieval Service STRS Quiz No 3 Answer: Biphasic airway noises suggesting tracheal obstruction. Ask about a history of previous noisy breathing or intubation Bronchiolitis does not cause stridor. Of concern also is the episode of cyanosis. After intubation he was extremely difficult to ventilate. He had never been intubated previously and although had evidence of chronic sternal recession his parents had been reassured this was normal. Investigation revealed the cause to be congenital tracheal stenosis and an anomalous left pulmonary artery (‘ring- sling’ anomaly). CT scan (above) showing a left pulmonary artery sling.

12. South Thames Retrieval Service STRS Quiz No 3 Question 6 A 4 week old baby is referred with a possible chest infection. The baby was born at 35 weeks gestation with an antenatal diagnosis if severe aortic stenosis. This was treated with balloon dilatation on day 2 of life and the baby was discharged well 2 days later. His local team are happy this is not ‘cardiac’. The baby is grunting, recessing and has a heart rate of 180. He is intubated and ventilated. Comment on the CXR?

13. South Thames Retrieval Service STRS Quiz No 3 Answer: Enlarged heart, mild hyperinflation, plethoric lung fields. Although this baby may well have a respiratory infection, the possibility of cardiac failure is high. The baby had a mixed respiratory, metabolic acidosis with a lactate of 7 which, together with the chest x-ray, would implicate cardiac decompensation as part of the picture. For this reason the baby was transferred to a cardiac centre and on review had severe aortic regurgitation and poor left ventricular function.

14. South Thames Retrieval Service STRS Quiz No 3 Question 7 An 18 month old boy presents shocked to A+E. He has been seen by the GP with intermittent bile- stained vomiting over last 4 weeks. He is resuscitated with fluid, inotropes and ventilation. Comment on his CXR and possible diagnosis?

15. South Thames Retrieval Service STRS Quiz No 3 Answer: Diaphragmatic hernia (left hemithorax) The CXR shows diffuse opacification on left with air filled pockets, mediastinal shift to right and abnormal position of the nasogastric tube. This child’s previous history suggests bowel obstruction (bile-stained vomiting). His abdominal x-ray was featureless with a paucity of bowel gas. Unusually, for a child of this age, the chest x-ray changes represent bowel in the chest secondary to a congenital diaphragmatic hernia. After resuscitation the child required removal of a significant amount of dead bowel. Bile stained vomiting is a very important clinical indicator requiring urgent investigation of a surgical cause.

16. South Thames Retrieval Service STRS Quiz No 3 Question 8 A 14 year old girl with cerebral palsy has a sudden respiratory arrest whilst in the shower at home. Her mother performed mouth to mouth ventilation and she was bagged to A+E by the paramedics. Currently, saturations are 100% in face mask oxygen, respiratory rate is 12 with poor effort, heart rate 110, BP 101/95. GCS is 9, pupils not assessed. Arterial pCO 2 is 10. She is blind and has severe scoliosis but a good level of communication. What intervention is required? What important history point must you establish urgently?

17. South Thames Retrieval Service STRS Quiz No 3 Answer: Intubation and ventilation for hypoventilation. Does the child have a VP shunt (this may be blocked)? The cause of the sudden loss of consciousness and hypoventilation is not clear. Ventriculoperitoneal shunts are common in children with cerebral palsy, in whom early signs of complications can be difficult to detect. Differential diagnosis for the acute neurological event in this case would include a seizure, an acute cerebrovascular event or a blocked VP shunt. An urgent CT scan is required after the child has been intubated and ventilated. She did indeed have a VP shunt and CT showed gross hydrocephalous. She was transferred urgently to a neurosurgical centre for intervention. Making the diagnosis of a possible neurosurgical problem as soon as possible is important so the child can be taken to the most appropriate institution as quickly as possible.

18. South Thames Retrieval Service STRS Quiz No 3 Question 9 A 3 year old has a generalized seizure at home which terminated spontaneously on arrival of the paramedic. Temperature and blood sugar were normal at the scene. She is breathing with saturations of 100% in face mask oxygen. You can feel a femoral pulse but BP is unrecordable. GCS is 3 and her pupils are dilated but reactive. What does the ECG strip show and what actions must be taken? What is a possible explanation?

19. South Thames Retrieval Service STRS Quiz No 3 Answer: Broad complex ventricular tachycardia (VT). Requires intubation and ventilation and cardioversion. Exclude possible drug Ingestion as cause. The combination of acute neurological symptoms and arrhythmia immediately raises the possibility of drug ingestion, likely accidental in a toddler. This child is in VT and is shocked which mandates urgent cardioversion (1 J/kg, then 2 J/kg if this fails) She had taken her mother’s amitryptilline. Sodium bicarbonate infusion may be necessary in serious tricyclic overdose, as in this case, aiming to achieve a slightly alkaline pH.

20. South Thames Retrieval Service STRS Quiz No 3 Question 10 A 9 year old boy with sickle cell disease is admitted to A+E with a possible painful crisis. He has been vomiting for 2 days. He is lethargic but talking to you and obviously breathless. His respiratory rate is 30 and there is no recession. Saturations are 100% in air and his chest is clear on auscultation. What is the likely diagnosis and how would you confirm it? Why is the sodium so low? What is the anion gap? An arterial blood gas and chemistry reveal: pH 7.0 pCO2 1.5 pO2 11 HCO3 2 BE -25 Na 125 K 4.5 urea 12 creat 65 Cl 95

21. South Thames Retrieval Service STRS Quiz No 3 Answer: Diabetic ketoacidosis (DKA). Anion gap is 32.5 mmol/l. Hyponatreamia due to osmotic effect of hyperglycaemia Acidotic breathing is characteristic but can be misdiagnosed. Severe metabolic acidosis with a high anion gap is most usually the result of DKA. Treatment requires SLOW rehydration (this has not happened overnight) and insulin therapy (shuts down ketone production). The aim is to maintain plasma osmolality in the first 24 hours when the risk of cerebral oedema is highest. As the plasma glucose falls the plasma sodium should rise (the corrected sodium should stay about the same). As the ketone concentration falls in the blood the anion gap should return to normal, usually in the first 24 hours, although the acidosis will not clear completely as hyperchloraemia is common (resolves with time). Anion gap (normal 8-16) = (Na+K) – (HCO3+Cl) Corrected sodium = measured Na + (0.4 x (glucose-5.5))

22. South Thames Retrieval Service STRS Quiz No 3 End of quiz