Arthrogryposis & Paediatric Difficult Airway


A 4 month old infant with arthropgryposis multiplex congenital was admitted to the paediatric assessment unit. The infant had been acutely unwell over the preceding 12 hours with respiratory compromise and a productive cough with green sputum. He had signs of respiratory distress with a RR of 40, pulse oximetry showed SpO2 of 85% on air and only 90% with a facemask, reservoir bag and high flow oxygen. It was felt that the infant would need to be intubated and ventilated. Two months before the infant had had a respiratory arrest on the neonatal ward and was unable to be intubated. That situation was resolved by mask ventilation and rescue with an LMA. There were obvious concerns that direct laryngoscopy would be unsuccessful and may precipitate a terminal decline in the patient’s condition.

The infant’s breathing was supported by bag/mask ventilation whilst he was transferred to an ENT theatre. Further anaesthetic support and an ENT surgeon were sought. I.v. access was established through a scalp vein. Ventilation was switched to an Ayres T piece with Jackson-Rees modification. Induction of anaesthesia was initiated with sevoflurane and oxygen. Direct laryngoscopy showed a Lehane and Cormack grade 4 view.

A rigid bronchoscope with video camera monitor was used by the ENT surgeon to obtain a view of the glottis. An epidural catheter was placed down the side port of the bronchoscope and was directed through the vocal cords. The bronchoscope was removed and a fine bore suction catheter was railroaded over the epidural catheter to give more stiffness. The positions of the end of the catheters were checked with the bronchoscope. A size 3.0cm uncuffed endotracheal tube was then railroaded over the catheters into trachea. Position and length were confirmed with the bronchoscope and ventilation was continued. The arrangement is shown in Figure 1.

The child was then transferred to the adult ICU where a retrieval team arrived to transfer the patient to a PICU.

What is arthrogryposis? Describe some methods for achieving control of the difficult paediatric airway.Read More »

Management of Inhalational Injury

A 30-year-old man with no significant past medical history was admitted to ED from a house fire started by a piece of faulty electrical equipment. There were superficial skin burns only but some evidence of a possible inhalation injury with singed nasal hairs and a hoarse voice. Coughing resulted in expectoration of carbonaceous sputum with some haemoptysis. Arterial blood gas analysis revealed a PaO2 of 10.4 kPa on 40% oxygen a carboxyhaemoglobin level of 18%.

Semi-elective endotracheal intubation was performed using an uncut orotracheal tube. Ventilatory parameters were adjusted to give a tidal volume of 6-8 ml/kg and plateau pressure of less than 30 cmH20. Recruitment manouveres were performed to give an optimum compliance in the region of 40-50 ml/cmH20 with a positive end-expiratory pressure of 8 H20. The inspired fraction of oxygen was kept high (i.e. greater than 60%) until there was a fall of the carboxyhaemoglobin level to less than 5% at which point downwards titration was performed as guided by a target SpO2 of 94%.

Fibreoptic bronchoscopy was performed approximately six hours after admission to intensive care which demonstrated carbonaceous colonisation of the lower respiratory tract and areas of erythematous and denuded epithelium. Within 12 hours of intubation significant oedema of the face and upper airway had developed. A restrictive fluid regimen was instituted and there was gradual resolution of this swelling over the next 3 days. At this time, gas exchange was satisfactory and the patient was successfully extubated before being discharged to the high-dependency unit.

How is inhalational injury managed on the ICU?Read More »

High Dose Insulin Infusion for Calcium Channel Blocker Overdose

A 24-year-old was admitted following an intentional overdose of 10mg amlodipine tablets following an argument with his family. Approximately 10 tablets were ingested. On self-presentation two hours after the event, he was clinically stable with no haemodynamic compromise. There was no airway or respiratory compromise and a 12 lead electrocardiogram demonstrated sinus rhythm at 98 beats per minute. Both an arterial blood gas and electrolyte analysis were normal. Ionised calcium was 1.14 mol/L.

Over the following two hours he developed hypotension down to a nadir of 58/32 mmHg without change in heart rate or rhythm or the development of metabolic abnormalities. This was initially treated with intravenous fluids without significant response. A bolus of calcium chloride was administered without success; at this time he was referred to the intensive care team for assessment. Careful clinical examination revealed no other abnormality except hypotension. Neurological function remained intact and there appeared to be a vasodilated state with warm peripheries and relative tachycardia at 110 beats per minute in sinus rhythm.

The patient was transferred to the intensive care unit where an infusion of noradrenaline was commenced, rapidly escalating to a rate of 0.92 mcg/kg/min with little improvement in mean arterial pressure beyond 30-40 mmHg and relative oliguria. After consultation with the National Poisons Service, a high dose infusion of actrapid was commenced at rate of 0.5 units/kg/hour, with subsequent improvement of his haemodynamic parameters and a reduction in his noradrenaline requirement. Over the following 4 hours, both this infusion and the noradrenaline infusion were subsequently weaned off. The patient was discharged to the ward after eight hours and after assessment by the psychiatric team, from hospital the following day.

What are the clinical features of calcium channel blocker overdose and what is the role of high dose insulin infusion?Read More »

Management of Variceal Haemorrhage

A 60-year-old alcoholic was admitted with large-volume, frank haematemesis. On presentation he was hypotensive, tachycardic and obtunded with multiple stigmata of chronic liver disease including a moderate volume of ascites and palpable splenomegaly. Initial phlebotomy revealed a haemoglobin of 6.4 g/dL, INR of 4.5 and bilirubin of 54 μmol/L. Arterial blood gas analysis demonstrated a significant metabolic acidosis and lactate of 11 mmol/L. Large bore intravenous access was established and administration of crystalloid initiated, targeting a systolic blood pressure of 90 mmHg. Urgent cross-match of 10 units of packed red blood cells, clotting products and platelets was requested and the patient was transferred to theatre where upper gastrointestinal tract endoscopy was performed under general anaesthesia. This demonstrated three columns of varices involving the gastro-oesophageal junction. Attempts at banding and injection of sclerosant met with variable success. A Senstaken-Blakemore tube was inserted due to incomplete haemostasis and further attempts at endoscopic therapy abandoned.

The patient was transferred to intensive care. Intravenous cefotaxime and terlipressin were commenced. Further transfusion of clotting products continued as guided by thromboelastography with some slowing of transfusion but red cell requirements persisted at a rate of 1-2 units of blood per hour. At 12 hours, repeat endoscopy was performed – further attempts at sclerotherapy were unsuccessful and transjugular intrahepatic porto-systemic shunting was performed by the interventional radiology team. Upon return to intensive care, a significant reduction in bleeding was noted and both haemodynamic indices and coagulopathy improved over the following 12 hours. A repeat endoscopy demonstrated no evidence of active ongoing bleeding. At this point sedation was stopped; some encephalopathy was evident although this improved in the following 24 hours. Extubation occurred on day 3 after admission and he was discharged to the high-dependency unit at day 5 without significant ongoing acute issues.

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Invasive Ventilation of Life-Threatening Asthma

Invasive Ventilation in Life-Threatening Asthma

An 18-year-old known asthmatic presented with a two-day history of increasing shortness of breath on a background of a recent coryzal illness. She had a background of reasonably poor control and had been admitted to the intensive care unit for mechanical ventilation twice as a child. Her current medication included regular inhaled serotide 250, montelukast 10mg and theophylline MR 450mg BD. At presentation she was in extremis; pulse rate was 65 per minute, blood pressure 75/54 mmHg and respiratory rate 14 per minute. Arterial blood gas analysis demonstrated a PaCO2 of 11 kPa and PaO2 of 7.6 kPa with associated respiratory acidosis. Nebulised salbutamol and intravenous magnesium sulphate therapy was administered. along with 200mg of intravenous hydrocortisone. On arrival of the intensive care team, the patient’s respiratory rate deteriorated to a rate of 4 per minute. Assisted ventilation with a self-inflating bag and 100% oxygen was performed; rapid-sequence intubation was performed using ketamine and rocuronium.

Following intubation, immediate difficulties were experienced with mechanical ventilation. High airway pressures in excess of 40 cmH2O with tidal volumes of less than 200 ml were observed. Immediate chest radiography confirmed correct positioning of the endotracheal tube and excluded a pneumothorax. Adequate sedation and neuromuscular blockade were confirmed. Auscultation confirmed severe, widespread wheeze with limited air entry. Further nebulised salbutamol was administered and an aminophylline infusion initiated. The patient was transferred to the intensive care unit where magnesium, ketamine and vecuronium by infusion were added. Various modes of mechanical ventilation were tried including volume and pressure triggered with varying success; this included lengthening the I:E ratio, frequent disconnections to allow deflation and adjustment of PEEP to maximum compliance. Continuous salbutamol was administered via an ultrasonic nebuliser. Airway pressures remained high and there was little improvement in her acidosis. 2 hours after admission the patient suffered a PEA cardiac arrest from which she could not be resuscitated.

What are the difficulties in ventilating severe asthmatics, and what strategies can we use to overcome them?

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Glutamine in Critical Illness

Glutamine in Critical Illness

A forty-year-old motorcyclist was admitted to the ITU following a road traffic accident involving a stationary vehicle. She sustained a fractured right distal radius and multiple left sided rib fractures, involving ribs 2 to 9, with a free floating flail segment. She developed respiratory distress due to underlying lung contusions and a haemopneumothorax and was treated with two left sided intercostal drains, endotracheal intubation and invasive ventilation. She developed ARDS with bilateral infiltrates and PF ratio of <200mmHg, with normal cardiac function on transthoracic echocardiography. She was tracheostomised on day 12, and had a protracted ventilatory wean further complicated by a ventilator associated pneumonia. She was enterally fed during this period but began to develop an ileus and gut dysmotility, resistant to prokinetic treatment, leading to large volume gastric aspirates. She became visibly malnourished and was commenced temporarily on parenteral nutrition and IV glutamine. The ileus resolved over the following week and weaning recommenced, having ceased due to diaphragmatic splinting. She eventually weaned from the ventilator and was discharged from the ITU on day 40. She was profoundly weak due to a critical illness acquired weakness.

What is the role of glutamine supplementation in critical illness?Read More »

Nutrition in the Intensive Care Unit

Nutrition in the Intensive Care Unit

A middle aged man is admitted with abdominal pain and vomiting. He has a history of alcohol excess. A CT scan shows evidence of pancreatic necrosis. Supportive care is initiated and an NGT placed for supplementary enteral nutrition. After 3 days, he is referred to ICU as his oxygen requirements have increased and he is requiring non-invasive ventilation. It is noted that he has had very large gastric aspirates. Parenteral nutrition is commenced at this point.

What is the evidence for enteral versus parenteral feed as a source of nutrition in critically ill patients?Read More »

Tracheostomy in the Intensive Care Unit

Tracheostomy in the Intensive Care Unit

A 47-year-old male was admitted to the intensive care unit (ICU) following a high-speed motorcycle accident. He had a number of injuries including bilateral pneumothoraces, multiple spinal fractures, an open-book pelvis fracture, and a brachial plexus injury. Bilateral chest drains were inserted and external fixation of the pelvis was performed. The patient was extubated eventually at day 15 but required reintubation within 12 hours because of a poor cough and sputum retention 

What are the indications for a tracheostomy and when shout it be considered?

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Heparin Induced Thrombocytopenia

Heparin Induced Thrombocytopenia

A 62 year old lady with a metallic aortic valve was admitted to the cardiac unit for urgent surgical repair of a severely regurgitant mitral valve. He was normally on warfarin for his metal valve. This was stopped and unfractionated heparin commenced on day 4 once his INR level had dropped below the therapeutic range. The patient’s platelet count was 147*10^9/L on admission. By day 4 it had dropped to 85*10^9/L. After heparin was started it dropped further to a nadir of 55*10^9/L on day 8.

Could this be due to heparin induced thrombocytopenia? What investigations are required and how should we treat it?
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Hepatorenal Syndrome

Hepatorenal Syndrome

A 54 year old man with a history of alcohol excess was admitted under the medical team with an upper gastro-intestinal bleed. He had a background of pulmonary fibrosis that limited his exercise tolerance to 30 yards. Antibiotics, terlipressin and fluid resuscitation, including blood, were given. An oesophago-gastro-duodenoscopy demonstrated severe portal gastropathy but no active bleeding or varices. An abdominal ultrasound demonstrated cirrhosis and some moderate ascites. On day two of the patient’s hospital admission he was admitted to the intensive care unit (ICU) with respiratory failure and non-invasive ventilation was started. Over the next few days his condition deteriorated and he required vasopressor support. By day 6 the patient was oliguric, and his creatinine had risen from 102 to 155 µmoles/l.

What is the cause for his acute kidney injury? Could it be hepatorenal syndrome? Read More »