J Dent Anesth Pain Med.  2017 Sep;17(3):231-233. 10.17245/jdapm.2017.17.3.231.

Anesthetic considerations for a pediatric patient with Wolf-Hirschhorn syndrome: a case report

• Tsukamoto
• Yamanaka H
• Yokoyama T

• KMID: 2390809
• DOI: http://doi.org/10.17245/jdapm.2017.17.3.231

Abstract

Wolf-Hirschhorn syndrome is a rare hereditary disease that results from a 4p chromosome deletion. Patients with this syndrome are characterized by craniofacial dysgenesis, seizures, growth delay, intellectual disability, and congenital heart disease. Although several cases have been reported, very little information is available on anesthetic management for patients with Wolf-Hirschhorn syndrome. We encountered a case requiring anesthetic management for a 2-year-old girl with Wolf-Hirschhorn syndrome. The selection of an appropriately sized tracheal tube and maintaining intraoperatively stable hemodynamics might be critical problems for anesthetic management. In patients with short stature, the tracheal tube size may differ from what may be predicted based on age. The appropriate size ( internal diameter ) of tracheal tubes for children has been investigated. Congenital heart disease is frequently associated with Wolf-Hirschhorn syndrome. Depending on the degree and type of heart disease, careful monitoring of hemodynamics is important.

Keyword

Anesthesia, General; Intubation, Intratracheal; Wolf-Hirschhorn Syndrome

MeSH Terms

Anesthesia, General
Child
Child, Preschool
Chromosome Deletion
Female
Genetic Diseases, Inborn
Heart Defects, Congenital
Heart Diseases
Hemodynamics
Humans
Intellectual Disability
Intubation, Intratracheal
Seizures
Wolf-Hirschhorn Syndrome*

Reference

1. Bosenberg AT. Anaesthesia and Wolf-Hirschhorn Syndrome. South Afr J Anaesth Analg. 2007; 13:31–34.

2. Choi JH, Kim JH, Park YC, Kim WY, Lee YS. Anesthetic experience using total intra-venous anesthesia for a patient with Wolf-Hirschhorn syndrome -A case report-. Korean J Anesthesiol. 2011; 60:119–123. PMID: 21390167.

3. Gamble JF, Kurian DJ, Udani AG, Greene NH. Airway management in a patient with Wolf-Hirschhorn syndrome. Case Rep Pediatr. 2016; 2016:7070125. PMID: 27752382.

4. Shott SR. Down syndrome: analysis of airway size and a guide for appropriate intubation. Laryngoscope. 2000; 110:585–592. PMID: 10764002.

5. Takita K, Morimoto Y, Okamura A, Kemmotsu O. Do age-based formulae predict the appropriate endotracheal tube sizes in Japanese children? J Anesth. 2001; 15:145–148. PMID: 14566512.

6. Kohjitani A, Iwase Y, Sugiyama K. Sizes and depths of endotracheal tubes for cleft lip and palate children undergoing primary cheiloplasty and palatoplasty. Paediatr Anaesth. 2008; 18:845–851. PMID: 18768044.

7. Schubert S, Schmitz T, Weiss M, Nagdyman N, Huebler M, Alexi-Meskishvili V, et al. Continuous, non-invasive techniques to determine cardiac output in children after cardiac surgery: Evaluation of transesophageal doppler and electric velocimetry. J Clin Monit Comput. 2008; 22:299–307. PMID: 18665449.