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 4, April 2016, pages 126-129

Perioperative Care of an Infant With Wolf-Hirschhorn Syndrome: Is There a Risk of Malignant Hyperthermia

**Table 1.** Previous Reports of Anesthetic Care in Wolf-Hirschhorn Syndrome
Authors and reference	Demographics	Anesthetic management and outcome
GA: general inhalational anesthesia; IM: intramuscular; IV: intravenous; MH: malignant hyperthermia; NMB: neuromuscular blockade; TIVA: total intravenous anesthesia; MP: Mallampati.
Ginsburg and Purcell-Jones [11]	21-month-old, 5.2 kg female infant for cleft palate repair	(GA) Frequent respiratory tract infections related to aspiration. Premedication with IM atropine. Inhalation induction with halothane and NMB with succinylcholine. Difficult laryngoscope with poor glottic visualization. Temperature increased to 42.2 °C, 70 min into the case. Volatile agent discontinued, dantrolene administered, and surgery aborted. Surface cooling initiated. Metabolic acidosis treated with sodium bicarbonate. Tracheal extubation the following day and uneventful recovery.
Chen et al [12]	8-month-old, 5 kg infant for cheiloplasty	(GA) Inhalation induction with halothane and NMB with succinylcholine and atracurium. Suspected delayed MH 2 h after surgery. Hyperventilated with 100% O₂, cooled with ice bags, dantrolene 2.5 kg/kg. Recovered uneventfully.
Sammartino et al [13]	8-month-old , 5.2 female infant for goniotomy	(TIVA) Frequent respiratory tract infections related to aspiration. Premedication with IM atropine and ketamine followed by placement of IV cannula. Anesthesia induced with propofol and fentanyl. Difficult airway, requiring three attempts. Maintenance anesthesia with propofol boluses and vecuronium 0.1 mg/kg. Tracheal extubation 2 h later in ICU. Uneventful recovery.
Bosenberg [14]	4-year-old, 12 kg girl for ENT and dental examination	(GA) Prior history of chronic respiratory infections due to aspiration, but alleviated by Nissen at 8 months of age. No premedication. Inhalation induction with sevoflurane. No muscle relaxant. Slightly difficult nasal intubation. Otherwise uneventful anesthetic.
Iacobucci et al [15]	5-year-old, 10 kg boy for circumcision	(GA) MP class 1 examination. Inhalation induction with sevoflurane. Easy endotracheal intubation. Cis-atracurium 0.1 mg/kg, alfentanil 25 µg/kg, and ketorolac 1 mg/kg administered intraoperatively. NMB reversed with neostigmine 0.3 mg. Tracheal extubation in OR. Uneventful anesthetic.
Mohiuddin and Mayhew [16]	Case 1: 9-month-old male for open reduction of left hip	(GA) Previous intraoperative cancellation, at 8 months of age, due to difficult IV access and inability to place Foley catheter. Second surgery at 9 months included inhalation induction with sevoflurane and nitrous oxide. Central venous catheter was placed. Caudal block for postoperative analgesia.
	Case 2: 8-month-old male for bilateral myringotomy tubes	(GA) Inhalation induction with nitrous oxide and sevoflurane. Acetaminophen for postoperative analgesia. No anesthetic complications.
Schmidt et al [17]	4-year-old, 9.9 kg female for atrial septal defect repair	(TIVA) No mention of respiratory infection. Premedicated with IV midazolam. RSI with propofol, fentanyl, and vecuronium (0.2 mg/kg). No airway issues. Maintained with fentanyl boluses and a propofol infusion. Arterial and central lines placed after induction. Uneventful anesthetic.
Choi et al [18]	33-month-old male for bilateral tympanoplasty and myringotomy	(TIVA) IM atropine (0.2 mg). Anesthetic induction with midazolam (0.1 mg/kg) and remifentanil infusion. Once bag-valve-mask-ventilation was established, rocuronium (0.6 mg/kg) was administered. Cormack-Lehane grade IIa view. High fever of 39 °C, 7 h after surgery with seizure activity. Fever persisted despite antipyretic agent. Suspected upper respiratory tract infection rather than delayed-onset MH. No dantrolene given. Eventual normal recovery and uneventful hospital course.

Table 1. Previous Reports of Anesthetic Care in Wolf-Hirschhorn Syndrome

Authors and reference

Demographics

Anesthetic management and outcome

GA: general inhalational anesthesia; IM: intramuscular; IV: intravenous; MH: malignant hyperthermia; NMB: neuromuscular blockade; TIVA: total intravenous anesthesia; MP: Mallampati.

Ginsburg and Purcell-Jones [11]

21-month-old, 5.2 kg female infant for cleft palate repair

(GA) Frequent respiratory tract infections related to aspiration. Premedication with IM atropine. Inhalation induction with halothane and NMB with succinylcholine. Difficult laryngoscope with poor glottic visualization. Temperature increased to 42.2 °C, 70 min into the case. Volatile agent discontinued, dantrolene administered, and surgery aborted. Surface cooling initiated. Metabolic acidosis treated with sodium bicarbonate. Tracheal extubation the following day and uneventful recovery.

Chen et al [12]

8-month-old, 5 kg infant for cheiloplasty

(GA) Inhalation induction with halothane and NMB with succinylcholine and atracurium. Suspected delayed MH 2 h after surgery. Hyperventilated with 100% O₂, cooled with ice bags, dantrolene 2.5 kg/kg. Recovered uneventfully.

Sammartino et al [13]

8-month-old , 5.2 female infant for goniotomy

(TIVA) Frequent respiratory tract infections related to aspiration. Premedication with IM atropine and ketamine followed by placement of IV cannula. Anesthesia induced with propofol and fentanyl. Difficult airway, requiring three attempts. Maintenance anesthesia with propofol boluses and vecuronium 0.1 mg/kg. Tracheal extubation 2 h later in ICU. Uneventful recovery.

Bosenberg [14]

4-year-old, 12 kg girl for ENT and dental examination

(GA) Prior history of chronic respiratory infections due to aspiration, but alleviated by Nissen at 8 months of age. No premedication. Inhalation induction with sevoflurane. No muscle relaxant. Slightly difficult nasal intubation. Otherwise uneventful anesthetic.

Iacobucci et al [15]

5-year-old, 10 kg boy for circumcision

(GA) MP class 1 examination. Inhalation induction with sevoflurane. Easy endotracheal intubation. Cis-atracurium 0.1 mg/kg, alfentanil 25 µg/kg, and ketorolac 1 mg/kg administered intraoperatively. NMB reversed with neostigmine 0.3 mg. Tracheal extubation in OR. Uneventful anesthetic.

Mohiuddin and Mayhew [16]

Case 1: 9-month-old male for open reduction of left hip

(GA) Previous intraoperative cancellation, at 8 months of age, due to difficult IV access and inability to place Foley catheter. Second surgery at 9 months included inhalation induction with sevoflurane and nitrous oxide. Central venous catheter was placed. Caudal block for postoperative analgesia.

Case 2: 8-month-old male for bilateral myringotomy tubes

(GA) Inhalation induction with nitrous oxide and sevoflurane. Acetaminophen for postoperative analgesia. No anesthetic complications.

Schmidt et al [17]

4-year-old, 9.9 kg female for atrial septal defect repair

(TIVA) No mention of respiratory infection. Premedicated with IV midazolam. RSI with propofol, fentanyl, and vecuronium (0.2 mg/kg). No airway issues. Maintained with fentanyl boluses and a propofol infusion. Arterial and central lines placed after induction. Uneventful anesthetic.

Choi et al [18]

33-month-old male for bilateral tympanoplasty and myringotomy

(TIVA) IM atropine (0.2 mg). Anesthetic induction with midazolam (0.1 mg/kg) and remifentanil infusion. Once bag-valve-mask-ventilation was established, rocuronium (0.6 mg/kg) was administered. Cormack-Lehane grade IIa view. High fever of 39 °C, 7 h after surgery with seizure activity. Fever persisted despite antipyretic agent. Suspected upper respiratory tract infection rather than delayed-onset MH. No dantrolene given. Eventual normal recovery and uneventful hospital course.

**Table 2.** Anesthetic Concerns in Patients With Wolf-Hirschhorn Syndrome
1.	Difficult airway management
2.	Difficult vascular access
3.	Associated congenital heart disease
4.	Underlying seizure disorder
5.	Hypotonia and choice of neuromuscular blocking agent
6.	Controversial predisposition to malignant hyperthermia
7.	Perioperative respiratory complications
8.	Chronic aspiration
9.	Non-MH-related febrile episodes (possibly secondary to chronic respiratory infections related to chronic aspiration)

Table 2. Anesthetic Concerns in Patients With Wolf-Hirschhorn Syndrome

Difficult airway management

Difficult vascular access

Associated congenital heart disease

Underlying seizure disorder

Hypotonia and choice of neuromuscular blocking agent

Controversial predisposition to malignant hyperthermia

Perioperative respiratory complications

Chronic aspiration

Non-MH-related febrile episodes (possibly secondary to chronic respiratory infections related to chronic aspiration)