Tariq Parray

Moyamoya disease: a review of the disease and anesthetic management.

Moyamoya disease is a rare chronic cerebrovascular disease seen both in children and adults. It has a progressive course, but may have a variable clinical presentation. The disease causes ischemic stroke, intracranial hemorrhage, headache, seizures, and transient ischemia attack in children and in adults. Although the pathogenesis of the disease remains unknown, research suggests a genetic predisposition. There are also undefined systemic processes involved in this vasculopathy. Better noninvasive diagnostic techniques for diagnosis of the Moyamoya disease have been developed, but medical treatment can still be challenging. However, various surgical revascularization procedures have shown to provide symptomatic benefit in a majority of these patients. In addition, the anesthetic management of these patients has evolved over the years with an increased understanding of the disease. These have specifically resulted from the identification of risk factors for perioperative complications and outcomes related to the use of anesthetic agents. Finally, research in the last 3 decades has led to the recognition of the importance of pain control, the increased use of regional anesthesia, and better monitoring techniques in providing high quality and safe patient care to patients with Moyamoya disease. This article will provide a comprehensive review of the disease and its anesthetic management.

Cardiac arrest because of unrecognized delayed dilated cardiomyopathy in a child with severe burn injury.

1 Goebel HH, Mole SE, Lake BD. Neuronal Ceroid Lipofuscinoses (Batten disease), Vol. 33. Amesterdam, The Netherlands: IOS Press, 1999. 2 Kohlschütter A. Neuronal Ceroid lipofuscinoses. Orphanet Encyclopedia. Hamburg, Germany. January 2004: 1–6. 3 Defalque RJ. Anesthesia for a patient with Kufs’ disease. Anesthesiology 1990; 73: 1041–1042. 4 Yamada Y, Katsushi D, Shinichi S et al. Anesthetic management for a patient with Jansky-Bielschowsky disease. Can J Anaesth 2002; 49: 81–83. 5 Gopalakrishnan S, Sidduiqui S, Mayhew J. Anesthesia in a child with Batten disease. Pediatr Anaesth 2004; 14: 889–892.

Tension pneumothorax and subcutaneous emphysema during retrieval of an ingested lithium button battery

We present a case of a child with an ingested lithium battery causing esophageal perforation with mediastinal injury extending to the pleural cavity. During the endoscopic retrieval of the battery, the child developed the rare complication of subcutaneous emphysema, tension pneumothorax, and pneumomediastinum from excessive iatrogenic air insufflation. The patient developed mediastinitis and had a complicated postoperative course.

Atypical presentation of an impacted radiolucent esophageal foreign body

Patients with impacted esophageal foreign bodies usually present with gastrointestinal and rarely with respiratory symptoms. Impacted esophageal foreign bodies may be identified by radiologic studies. Ingested radiolucent foreign bodies may be more difficult to diagnose, especially if the patient presents with minimal symptoms. We report a rare case of a child who presented with stridor and obstructive sleep apnea. The cause of respiratory symptoms was thought to be due to enlarged tonsils and adenoids, and the patient was scheduled for tonsillectomy and adenoidectomy. On re-evaluation by the surgeon on the day of surgery, the procedure was changed to diagnostic microlaryngoscopy and bronchoscopy to rule out any other cause. The patient’s respiratory symptoms were resolved when an incidental discovery and retrieval of the radiolucent esophageal foreign body was made. The diagnosis of radiolucent esophageal foreign body can be difficult and can be easily missed without reasonable clinical suspicion.

Stridor due to an innominate artery compression and posterior mediastinal mass in a pediatric patient

There are many causes for stridor in a pediatric patient. We present an interesting case of a pediatric patient who had stridor due to an innominate artery compression and posterior mediastinal mass. We discuss the anesthetic complication and management of patients with stridor.