A&A practice
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We present a case of a child with Aicardi-Goutières Syndrome (AGS) undergoing general anesthesia for placement of a laparoscopic gastrostomy tube. AGS is a rare genetic leukodystrophy that can affect most organ systems with extensive neurologic effects. ⋯ We describe our anesthetic management and discuss these implications. The patient had a prolonged duration of action of rocuronium and an otherwise uneventful anesthetic course.
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This case describes a patient who underwent mitral valve replacement (MVR) surgery with preservation of the subvalvular apparatus who suffered anterolateral papillary muscle rupture (PMR) postseparation from cardiopulmonary bypass. This patient had no history of coronary artery disease (CAD); subsequent pathology of the papillary muscle showed evidence of amyloid deposition. Although most PMRs are caused by ischemia from CAD, cardiac amyloidosis must be considered in the absence of CAD and worked up appropriately as cardiac involvement of amyloidosis, especially the amyloid light-chain (AL) subtype, is prognostic of increased mortality that can be mitigated with therapy.
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Case Reports
A Case Report and Anesthetic Considerations in Subcutaneous Fat Necrosis of the Newborn.
Subcutaneous fat necrosis of the newborn (SCFN) is an uncommon, self-limiting panniculitis generally diagnosed in the first few weeks of extrauterine life in term or postterm infants subjected to perinatal physiologic stress. Hallmarks of the process include dramatic hypercalcemia without identifiable cause that may be associated with renal disease, seizures, and death. Herein, we describe the anesthetic approach in an 11-week-old infant, including perioperative laboratory testing, management of hypercalcemia, and intraoperative considerations.