Anesthesia for sickle cell disease and congenital myopathy in combination

Abstract

We report on the perioperative management of anesthesia and analgesia in a child with sickle cell disease and a congenital myopathy, presenting for corrective orthopedic surgery. The case illustrates two valuable points of interest: the many benefits of regional anesthesia in complex medical cases and the successful use of tourniquets in children with sickle cell disease.

Perioperative care of children with sickle cell disease: A systematic review and clinical recommendations

“…The systematic preoperative assessment of SCD patients varies considerably in its implementation and/or description. As expected, collecting a medical history and performing a physical examination is a standard recommendation . In particular, the documentation of a personal pain management history is part of some assessments, and aims to facilitate postoperative interventions and decision‐making in non‐opioid naïve patients .…”

“…This limit had the tendency to move upwards with time, many authors currently recommending a hemoglobin level ≥9‐10 g/dL, before minor to intermediate risk surgery, whilst some groups still advocate 7 g/dL . A hemoglobin threshold of >10 g/dL is considered by some authors as a target for major surgeries . It is important to note that the definition of risk levels for surgeries remains variable as no consensus exists on this matter.…”

“…It is important to note that the definition of risk levels for surgeries remains variable as no consensus exists on this matter. Some authors advocate for preoperative transfusion without giving a threshold, despite evidence from the abovementioned RCTs. Exchange transfusions (manual and automated) for a leve of HbS <30% is recommended for high risk surgery either preoperatively, or on cardiopulmonary bypass .…”

Kinking of a soft nasal tracheal tube

“…The effect of regional anaesthesia on the progression of the disease in a patient likely to have prolonged immobilisation and muscle atrophy is not known [4]. However, there are reports of the successful use regional anaesthesia and analgesia in patients with various types of myopathy demonstrating the safety and efficacy of regional anaesthesia and analgesia [7,8]. This case illustrates the safe use of regional anaesthesia in a patient with centronuclear myopathy.…”

Regional anaesthesia in a patient with centronuclear (myotubular) myopathy

“…The effect of regional anaesthesia on the progression of the disease in a patient likely to have prolonged immobilisation and muscle atrophy is not known [4]. However, there are reports of the successful use regional anaesthesia and analgesia in patients with various types of myopathy demonstrating the safety and efficacy of regional anaesthesia and analgesia [7, 8]. This case illustrates the safe use of regional anaesthesia in a patient with centronuclear myopathy.…”