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 »

Tourniquets in Severe Traumatic Limb Haemorrhage

Tourniquets in Severe Traumatic Limb Haemorrhage

A 30 year old male pedestrian was involved in a road traffic collision with a car travelling at speed. On arrival of the paramedics he was found to be unconscious with evidence of severe blood loss. He also had a partial amputation of his right leg below the knee. The paramedics applied a combat application tourniquet to the thigh, above the injury. He then suffered a cardiorespiratory arrest and CPR was commenced. On arrival in the emergency department his trachea was intubated and he underwent bilateral decompressive thoracostomies. Large bore intra-venous access was secured and two units of packed red cells given by a rapid infusion device. He remained haemodynamically unstable requiring a further six units of red cells and associated blood products to maintain a systolic blood pressure of above 80mmHg. Orthopaedic members of the trauma team were persistently keen to remove the tourniquet in order to prevent distal-neurovascular damage. This request was repeatedly denied and he was transferred rapidly to theatre for definitive control of his ongoing haemorrhage with an exploratory laparotomy. No cause for haemorrhage was found on laparotomy so attention shifted to damage control surgery on his leg in order to try and achieve some haemodynamic stability. Unfortunately to achieve this aim the tourniquet was removed. Bleeding was uncontrollable even with reapplication of the tourniquet and the patient exsanguinated and died.

What are the current recommendations for the use of limb tourniquets in trauma, and what is the evidence base for those recommendations?

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