Hutchinson–Gilford progeria syndrome (HGPS) is a very rare genetic disorder characterized by premature ageing, severe growth failure, and very early onset atherosclerosis. Psychologically and emotionally child-like, these patients suffer from physiological changes of old age. Early and progressive atherosclerosis of intra-cranial vessels in HGPS patients, along with a thin skin and fragile vessels, make these patients susceptible to intra-cranial hematomas following relatively trivial injuries and to severe intra-cranial disease. Anesthetizing HGPS patients for surgery can be challenging due to the presence of a possible difficult airway, multi-system derangements, and associated skin, bone and joint disease. We report here one such child with HGPS who underwent craniotomy and evacuation of an extradural hematoma that developed after minor head trauma. Securing his airway during surgery was difficult.
Eventration of the diaphragm is a rare entity, characterised by abnormal elevation of a dome of diaphragm. In this condition, the diaphragm is composed of fibrous tissue with few or no interspersed muscle fibres. Eventration can be congenital or acquired. Congenital eventration results from inadequate development of muscles or absence of phrenic nerve. The common cause of acquired eventration is injury to the phrenic nerve from traumatic birth injury or surgery for heart disease. The perioperative anaesthetic management of diaphragmatic eventration along with ventricular septal defect with severe pulmonary hypertension makes this case both challenging and unique.
Management of a premature infant with PDA poses a significant challenge in the perioperative period for the anaesthesiologist. The risk is multiplied when it is associated with other congenital respiratory anomalies. In our case a premature child at 5 month of age presented with PDA and tracheomalacia. There was a risk of airway collapse during sedation or induction of anaesthesia along with an anticipated difficult intubation. We have managed the case by inducing and intubating the patient in the lateral position without using muscle relaxants. We have used Sevoflurane as the sole anaesthetic agent for inducing and intubating the patient. Postoperatively patient was extubated in lateral position and succesfully discharged from ICU.
First described in 1908, anomalous origin of left coronary artery from pulmonary artery is a very rare congenital anomaly. Here, the right coronary artery is usually enlarged and has a normal origin from aorta. Numerous collaterals connect the two coronary arteries over right ventricular outflow tract or interventricular septum. It is one of the most common causes of myocardial ischaemia and infarction in children.
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