Achondroplasia and deep inferior epigastric artery perforator flap reconstruction

Published Date: 31st October 2018

Publication Authors: Jain N

Abstract

Achondroplasia is the commonest form of dwarfism, with patients presenting significant anaesthetic challenges. Airway management is problematic due to macrocephaly, midface hypoplasia and narrow nasopharynx. Neck manipulation is dangerous owing to potential pre-existing cervicomedullary stenosis. Other problems may include restrictive lung disease, obstructive sleep apnoea, difficult venous access, hypersalivation and gastro-oesophageal reflux. Description A 51-year-old woman with achondroplasia (height 128 cm, weight 56 kg) and a history of breast carcinoma and hypothyroidism presented for deep inferior epigastric artery perforator (DIEP) flap reconstruction. Her anaesthetic history was unremarkable except for difficult venous access (albeit she had no previous documented intubations). Discussion To our knowledge, this is the first documented case of a patient with achondroplasia undergoing DIEP surgery (a known prolonged procedure). Pre-operatively we performed cardio-respiratory investigations (echocardiogram and spirometry), which were normal. Owing to her stature, one of the challenges faced peri-operatively was venous thromboembolism prophylaxis. The correctsized thromboelastic stockings and sequential compression device were unavailable. Therefore, we used low-molecular-weight heparin, passive limb movements and a device that works by neuromuscular electrostimulation technology. Small electrical impulses gently stimulate the common peroneal nerve resulting in foot dorsiflexion, emulating the process normally achieved by walking (up to 60%). We used a paediatric-size blood pressure cuff and obtained venous access on first attempt on the only visible vein. Her airway was uncomplicated and managed with conventional laryngoscopy and size 7.0 tracheal tube. Positioning is key to DIEP surgery as the table is broken at hip le vel to facilitate abdominal closure. In view of our patient's stature it was vital to position her correctly, thus we did this prior to anaesthesia. Finally, other recommended considerations following involvement in this case include ensuring availability of paediatric-sized invasive cannulae, as standard adult devices may be too long, sizing tracheal tube according to patient body weight (do not assume paediatric size) and ensuring that local anaesthesia delivery devices are infused at a suitable rate based on patient body weight. Our case was uneventful.

 

Sagger, A; Jain, N. (2018). Achondroplasia and deep inferior epigastric artery perforator flap reconstruction: Anaesthetic challenges and management . Anaesthesia. 73 (S4), p83

 

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