Anesthesia and perioperative management for a patient with Ullrich syndrome undergoing surgery for scoliosis.

Ullrich syndrome is a rare congenital hypotonic-sclerotic muscular disorder in which affected children develop a slowly progressive scoliosis and contractures and limpness of joints. The disease causes increasingly invalidating contractures and hardening of the muscles of the neck and trunk. While this neuromuscular type of scoliosis is progressive, patients rarely attain the point of surgery due to their compromised general medical condition. This may explain the current lack of outcome data and the paucity of information on perioperative management for patients with Ullrich syndrome undergoing major surgery. The purpose of this report was therefore to describe our first experience with the perioperative and anesthetic management of a 15-year-old boy presenting with Ullrich syndrome and a secondary invalidating scoliosis. The specific challenges of this condition characterized by severe restrictive lung disease and a challenging airway abnormality are discussed.

The peri-operative use of intra-articular local anesthetics: a review.

Acute and chronic pain are of major concern after orthopedic surgery. The increasing trend toward day case surgery induced the development of different techniques in postoperative pain control. One commonly used strategy in pain management after knee and shoulder joint surgery is the intra-articular (IA) use of local anesthetics (LA). Recent attention has been drawn to the possible toxicity on chondrocytes of local anesthetics. The aim of this manuscript is to evaluate and compare through literature review the existing evidence on the clinical use and possible adverse effects of intra-articular injection of local anesthetics peri-operatively.

Augmented serum lactic acid combined with metabolic alkalosis: a case report.

Lactic acidosis is a common problem in the peri-operative period. During extensive surgery we frequently have an augmentation of lactic acid most often on the basis of hypoperfusion. Normally, a rise in serum lactate level causes a fall in blood pH, and this metabolic acidosis is accompanied by a high anion gap. In this case report a perioperative rise in lactic acid and an elevation in serum pH in a patient during meningeal tumour surgery is presented.

Cardiac contractility and the spinal sympathetic neuron.

Changes in cardiac contractility in cats were measured following electrical stimulation of the thoracolumbar spinal cord. Inotropic response sites were highly localized, and inotropic changes were greatest in the region of the intermediolateral nucleus. Cardioaugmentation was elicited from all spinal segments between T1 and L4. Inotropic responses were maximal at a frequency of 25 Hz and, at this frequency, showed strong right-sided preponderance. Similar lateralization was noted for heart rate and blood pressure. The right-sided spinal lateralization of the contractility response appeared to be frequency dependent, with reversal at 10 Hz, a relationship previously demonstrated for zona intermedia pressor responses. Neither adrenalectomy nor change of anesthetic agent affected the magnitude or lateralization of the inotropic response.

Anaesthesia for a patient with Leigh's syndrome undergoing surgery for scoliosis.

Anaesthesia for patients with Leigh's syndrome has rarely been reported. Leigh's syndrome or subacute necrotizing encephalomyelopathy is a neurodegenerative disorder of infancy or childhood. Acute exacerbation with respiratory failure may accompany surgery and general anaesthesia. In this case report we describe the anaesthetic management of a 17 year old patient scheduled for spine surgery.