Anesthetic management of a pediatric patient with Freeman-Sheldon syndrome undergoing atrial septal defect closure: a case report

Abstract

Background Freeman-Sheldon syndrome (FSS) is a rare disorder characterized by specific deformities of the extremities and face. There have been no reports of open-heart surgery in pediatric patients with FSS. Case presentation We present the case of an 8-year-old girl with FSS who underwent atrial septal defect closure. Tracheal intubation was uncomplicated, although the patient had microstomia. Inhalational anesthetics and dopamine antagonists were avoided intraoperatively and perioperatively. We chose dexmedetomidine as an adjuvant for postoperative pain management contributing to adequate analgesia and early extubation without causing respiratory depression. Conclusions Anesthetic management of FSS requires consideration for airway management and prevention of malignant hyperthermia and respiratory complications. We successfully managed the case avoiding the use of malignant hyperthermia-triggering drugs.

Comment on: anesthetic management of a pediatric patient with Freeman-Sheldon syndrome undergoing atrial septal defect closure: a case report

“…We read with interest a recently published case of general anesthetic management of an 8-year-old girl who was stated to have Freeman-Sheldon syndrome and underwent atrial septal defect closure [ 1 ]. While the authors are to be applauded for their excellent anesthetic management of a patient with a complex presentation, the clinical implications of inaccuracies about the syndrome contained in their report are concerning.…”

“…As suggested by the literature, FBS has a 30–60% false-positive diagnosis rate [ 4 , 5 ]. On closer review, it is not surprising the patient appeared to have anatomic hallmarks of SHS and a history supporting this diagnosis over FBS [ 1 ]. Facial photographs show a triangular face and a small mouth, and the history includes ease of laryngoscopically introduced endotracheal intubation [ 1 , 4 ].…”

“…On closer review, it is not surprising the patient appeared to have anatomic hallmarks of SHS and a history supporting this diagnosis over FBS [ 1 ]. Facial photographs show a triangular face and a small mouth, and the history includes ease of laryngoscopically introduced endotracheal intubation [ 1 , 4 ]. Patients with FBS have elongated faces and true microstomia that prevents the use of a laryngoscope [ 4 ].…”

“…Patients with FBS have elongated faces and true microstomia that prevents the use of a laryngoscope [ 4 ]. Unfortunately, the authors do not clearly specify the diagnostic criteria (microstomia, pursed lips, deep nasolabial folds, and H or V-shaped chin defect and two major arthrogryposes—typically, camptodactyly with ulnar deviation and equinovarus) [ 1 , 4 ].…”

In-reply to the comment by Poling et al

“…We thank Poling et al for their interest in our report [ 1 ]. They raised the importance of a new disease concept and optimal management according to the diagnosis.…”