J Med Cases

Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access

Article copyright, the authors; Journal compilation copyright, J Med Cases and Elmer Press Inc

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Case Report

Volume 7, Number 10, October 2016, pages 420-425

Perioperative Care of a Child With Williams Syndrome

**Table 1.** Previous Reports of Anesthetic Care for Children With Williams Syndrome
Authors and reference	Demographics and surgical procedure	Anesthetic care and outcomes
IM: intramuscular; ROSC: return of spontaneous circulation; SVAS: supravalvular aortic stenosis; LVAD: left-ventricular assist device; CPB: cardiopulmonary bypass; CPR: cardiopulmonary resuscitation; ECMO: extracorporeal membrane oxygenation; PICU: pediatric intensive care unit; NM: neuromuscular.
Horowitz et al [4]	Patient #1: a 6-year-old girl for cardiac catheterization	Sedation with IM meperidine, promethazine, and chlorpromazine. Twenty minutes after the procedure, the patient developed bradycardia, complete heart block, and ventricular fibrillation. There was no ROSC despite immediate resuscitation.
Patient #2: a 3-year-old girl for repair of SVAS	Patient with two cardiac arrests prior to cannulation for CPB. After repair of SVAS, there was ongoing evidence of myocardial ischemia despite coronary artery bypass grafting. LVAD was placed, but there was no recovery of postoperative cardiac function.
Gupta et al [12]	A 5-year-old boy for preoperative CT imaging prior to surgical repair of SVAS	Anesthesia with sevoflurane in oxygen followed by succinylcholine. One minute after Isovue^® contrast was administered, there was loss of pulses, ST depression, and wide complex bradycardia. ACLS resuscitation included 100% oxygen ventilation, chest compressions, atropine, epinephrine, bicarbonate, vasopressin, and isoproterenol. No ROSC.
du Toit-Prinsloo et al [13]	A 2-year-old boy presenting for extraction of teeth under anesthesia	Inhalation induction with sevoflurane. Tracheal intubation facilitated by alfentanil and propofol. Immediately after endotracheal intubation, the patient developed bradycardia and ST-segment elevation with cardiac arrest. CPR was started and defibrillation attempts were made by external defibrillator. There was no ROSC.
Upadhyay et al [14]	A 6-month-old girl presenting for cardiac catheterization	After anesthetic induction and endotracheal intubation, during the process of obtaining vascular access, femoral pulses weaker with a widening QRS complex and marked ST depression. This progressed to cardiac arrest. CPR was started with the administration of epinephrine, sodium bicarbonate, and calcium chloride. Patient was placed on ECMO.
Bird et al [15]	Patient #1: a 6-week-old boy presenting for inguinal hernia repair	During anesthetic induction, bradycardia and hypotension required CPR for 1 min. He was resuscitated and surgery was completed. The patient died suddenly at 6 years of age.
Patient #2: a 9-month-old girl presenting for cardiac catheterization	During the induction of anesthesia, bradycardia developed and proceeded to cardiac arrest. No ROSC.
Patient #3: a 6-month-old girl presenting for cardiac catheterization.	Cardiac catheterization complicated by bradycardia and hypotension when attempting to enter the pulmonary artery. CPR and operative relief of outflow tract obstruction were unsuccessful.
Patient #4: a 10-month-old boy presenting for cardiac catheterization	After left ventriculography, complete heart block and hypotension developed. The boy was successfully resuscitated, but several hours later complete heart block, ventricular fibrillation, and cardiac arrest developed. No ROSC.
Patient #5: a 9-month-old boy presenting for cardiac catheterization	During cardiac catheterization, cardiac arrest occurred with the uninflated balloon in the left pulmonary artery. No ROSC.
Patient #6: a 2-year-old boy presenting for cardiac catheterization.	During catheterization for balloon angioplasty, hypotension, bradycardia, and ventricular fibrillation occurred. Resuscitative efforts were unsuccessful. The patient had a previous cardiac arrest during anesthesia for umbilical hernia repair.
Patient #7: a 21-month-old boy presenting for cardiac catheterization	During balloon dilation of the left pulmonary artery, hypotension and bradycardia occurred which progressed to full cardiac arrest. Resuscitation was unsuccessful.
Medley et al [17]	A 7-month-old presenting for surgical correction of subaortic and supravalvular aortic stenosis	Inhalation induction with sevoflurane and maintenance anesthesia of fentanyl. Cis-atracurium for NM blockade. CPB anesthesia maintained by desflurane. Patient was weaned from CPB with dopamine and epinephrine. Maintenance anesthesia until end of procedure with desflurane in air/oxygen. No complications noted.
Bragg et al [32]	Patient #1: a 3-year-old boy for orchiopexy surgery.	Serial cardiac arrests occurred during induction with ST wave changes, bradycardia, hypotension, and pulseless electrical activity requiring full resuscitation twice. The surgery was cancelled and a heart catheterization was scheduled for the following day. The patient experienced several cardiac arrests during the cardiac catheterization and was placed on ECMO and was transferred for emergent cardiac surgery.
Monfared et al [33]	An 18-month-old boy presenting for adenotonsillectomy	Adenotonsillectomy was successfully performed. The patient was extubated and being transferred from the operating room table, he became cyanotic and bradycardic. CPR was initiated with atropine, epinephrine, volume boluses, and electric defibrillation. Resuscitation was not successful.
Janna [34]	An 8-year-old boy presenting for emergent laparotomy	Patient was premedicated with intranasal dexmedetomidine followed by anesthetic induction with IV thiopentone, fentanyl, and atracurium. Anesthesia maintained with sevoflurane. Intraoperative tachycardia managed with esmolol. Patient was admitted to the PICU postoperatively and was discharged with no complications.
Asegaonkar et al [35]	A 3.5-year-old female presenting for cleft palate repair	Topical local anesthetic cream, antibiotic, and injection of tranexamic acid prior to induction. Anesthetic induction with fentanyl, midazolam, propofol, ketamine, and atracurium. Maintenance anesthesia with nitrous oxide, isoflurane, and atracurium. One episode of tachycardia was treated with fentanyl and deepening of anesthesia with isoflurane. No perioperative complications.
Sahin et al [36]	A 5-month-old male infant presenting for inguinal hernia repair	Anesthetic induction with sevoflurane and nitrous oxide. Maintenance anesthesia with sevoflurane in a nitrous oxide and IV fentanyl. No postoperative complications.
Boncagni et al [37]	A 12-year-old girl presenting for correction thoracic kyphoscoliosis	Anesthetic induction with fentanyl and propofol. Maintenance anesthesia with sevoflurane and remifentanil. Correction of original kyphoscoliosis was impossible due to excessive rigidity of the spine. No other complications noted.
Kawahito et al [38]	A 15-year-old girl presenting for aortoplasty for SVAS	Premedication with atropine and midazolam. Anesthesia induced with fentanyl and thiamyal. Maintenance anesthesia with nitrous oxide, oxygen, sevoflurane, and fentanyl. Neuromuscular blockade with vecuronium. Weaned from CPB with dopamine and prostaglandin E1. Postoperative course was uneventful.

Table 1. Previous Reports of Anesthetic Care for Children With Williams Syndrome

Authors and reference

Demographics and surgical procedure

Anesthetic care and outcomes

IM: intramuscular; ROSC: return of spontaneous circulation; SVAS: supravalvular aortic stenosis; LVAD: left-ventricular assist device; CPB: cardiopulmonary bypass; CPR: cardiopulmonary resuscitation; ECMO: extracorporeal membrane oxygenation; PICU: pediatric intensive care unit; NM: neuromuscular.

Horowitz et al [4]

Patient #1: a 6-year-old girl for cardiac catheterization

Sedation with IM meperidine, promethazine, and chlorpromazine. Twenty minutes after the procedure, the patient developed bradycardia, complete heart block, and ventricular fibrillation. There was no ROSC despite immediate resuscitation.

Patient #2: a 3-year-old girl for repair of SVAS

Patient with two cardiac arrests prior to cannulation for CPB. After repair of SVAS, there was ongoing evidence of myocardial ischemia despite coronary artery bypass grafting. LVAD was placed, but there was no recovery of postoperative cardiac function.

Gupta et al [12]

A 5-year-old boy for preoperative CT imaging prior to surgical repair of SVAS

Anesthesia with sevoflurane in oxygen followed by succinylcholine. One minute after Isovue^® contrast was administered, there was loss of pulses, ST depression, and wide complex bradycardia. ACLS resuscitation included 100% oxygen ventilation, chest compressions, atropine, epinephrine, bicarbonate, vasopressin, and isoproterenol. No ROSC.

du Toit-Prinsloo et al [13]

A 2-year-old boy presenting for extraction of teeth under anesthesia

Inhalation induction with sevoflurane. Tracheal intubation facilitated by alfentanil and propofol. Immediately after endotracheal intubation, the patient developed bradycardia and ST-segment elevation with cardiac arrest. CPR was started and defibrillation attempts were made by external defibrillator. There was no ROSC.

Upadhyay et al [14]

A 6-month-old girl presenting for cardiac catheterization

After anesthetic induction and endotracheal intubation, during the process of obtaining vascular access, femoral pulses weaker with a widening QRS complex and marked ST depression. This progressed to cardiac arrest. CPR was started with the administration of epinephrine, sodium bicarbonate, and calcium chloride. Patient was placed on ECMO.

Bird et al [15]

Patient #1: a 6-week-old boy presenting for inguinal hernia repair

During anesthetic induction, bradycardia and hypotension required CPR for 1 min. He was resuscitated and surgery was completed. The patient died suddenly at 6 years of age.

Patient #2: a 9-month-old girl presenting for cardiac catheterization

During the induction of anesthesia, bradycardia developed and proceeded to cardiac arrest. No ROSC.

Patient #3: a 6-month-old girl presenting for cardiac catheterization.

Cardiac catheterization complicated by bradycardia and hypotension when attempting to enter the pulmonary artery. CPR and operative relief of outflow tract obstruction were unsuccessful.

Patient #4: a 10-month-old boy presenting for cardiac catheterization

After left ventriculography, complete heart block and hypotension developed. The boy was successfully resuscitated, but several hours later complete heart block, ventricular fibrillation, and cardiac arrest developed. No ROSC.

Patient #5: a 9-month-old boy presenting for cardiac catheterization

During cardiac catheterization, cardiac arrest occurred with the uninflated balloon in the left pulmonary artery. No ROSC.

Patient #6: a 2-year-old boy presenting for cardiac catheterization.

During catheterization for balloon angioplasty, hypotension, bradycardia, and ventricular fibrillation occurred. Resuscitative efforts were unsuccessful. The patient had a previous cardiac arrest during anesthesia for umbilical hernia repair.

Patient #7: a 21-month-old boy presenting for cardiac catheterization

During balloon dilation of the left pulmonary artery, hypotension and bradycardia occurred which progressed to full cardiac arrest. Resuscitation was unsuccessful.

Medley et al [17]

A 7-month-old presenting for surgical correction of subaortic and supravalvular aortic stenosis

Inhalation induction with sevoflurane and maintenance anesthesia of fentanyl. Cis-atracurium for NM blockade. CPB anesthesia maintained by desflurane. Patient was weaned from CPB with dopamine and epinephrine. Maintenance anesthesia until end of procedure with desflurane in air/oxygen. No complications noted.

Bragg et al [32]

Patient #1: a 3-year-old boy for orchiopexy surgery.

Serial cardiac arrests occurred during induction with ST wave changes, bradycardia, hypotension, and pulseless electrical activity requiring full resuscitation twice. The surgery was cancelled and a heart catheterization was scheduled for the following day. The patient experienced several cardiac arrests during the cardiac catheterization and was placed on ECMO and was transferred for emergent cardiac surgery.

Monfared et al [33]

An 18-month-old boy presenting for adenotonsillectomy

Adenotonsillectomy was successfully performed. The patient was extubated and being transferred from the operating room table, he became cyanotic and bradycardic. CPR was initiated with atropine, epinephrine, volume boluses, and electric defibrillation. Resuscitation was not successful.

Janna [34]

An 8-year-old boy presenting for emergent laparotomy

Patient was premedicated with intranasal dexmedetomidine followed by anesthetic induction with IV thiopentone, fentanyl, and atracurium. Anesthesia maintained with sevoflurane. Intraoperative tachycardia managed with esmolol. Patient was admitted to the PICU postoperatively and was discharged with no complications.

Asegaonkar et al [35]

A 3.5-year-old female presenting for cleft palate repair

Topical local anesthetic cream, antibiotic, and injection of tranexamic acid prior to induction. Anesthetic induction with fentanyl, midazolam, propofol, ketamine, and atracurium. Maintenance anesthesia with nitrous oxide, isoflurane, and atracurium. One episode of tachycardia was treated with fentanyl and deepening of anesthesia with isoflurane. No perioperative complications.

Sahin et al [36]

A 5-month-old male infant presenting for inguinal hernia repair

Anesthetic induction with sevoflurane and nitrous oxide. Maintenance anesthesia with sevoflurane in a nitrous oxide and IV fentanyl. No postoperative complications.

Boncagni et al [37]

A 12-year-old girl presenting for correction thoracic kyphoscoliosis

Anesthetic induction with fentanyl and propofol. Maintenance anesthesia with sevoflurane and remifentanil. Correction of original kyphoscoliosis was impossible due to excessive rigidity of the spine. No other complications noted.

Kawahito et al [38]

A 15-year-old girl presenting for aortoplasty for SVAS

Premedication with atropine and midazolam. Anesthesia induced with fentanyl and thiamyal. Maintenance anesthesia with nitrous oxide, oxygen, sevoflurane, and fentanyl. Neuromuscular blockade with vecuronium. Weaned from CPB with dopamine and prostaglandin E1. Postoperative course was uneventful.