Abstract
We present a case of a 3-month-old female with Wolf-Hirschhorn syndrome (WHS) undergoing general anesthesia for laparoscopic gastrostomy tube placement with a focus on airway management. WHS is a rare 4p microdeletion syndrome resulting in multiple congenital abnormalities, including craniofacial deformities. Microcephaly, micrognathia, and glossoptosis are common features in WHS patients and risk factors for a pediatric airway that is potentially difficult to intubate. We discuss anesthesia strategies for airway preparation and management in a WHS patient requiring general anesthesia with endotracheal intubation.
Highlights
We present the case of a 3-month-old female with Wolf-Hirschhorn syndrome (WHS) undergoing laparoscopic gastrostomy tube placement, with a focus on airway management in a pediatric patient with craniofacial abnormalities
A 3-month-old female born with a prenatal diagnosis of Wolf-Hirschhorn syndrome was taken to the OR for planned laparoscopic gastrostomy tube placement due to poor oral feeding related to her muscular hypotonia
Children with WHS are born with congenital abnormalities requiring special consideration when administering an anesthetic, when it comes to airway management
Summary
Wolf-Hirschhorn syndrome is a genetic disorder resulting from a microdeletion of the short arm of chromosome 4; the majority of these deletions arise as de novo events (85%), and the remainder as unbalanced translocations within the 4p16 chromosome [1]. WHS has a prevalence of 1 : 50,000–20,000 births, with a female to male ratio of 2 : 1 [1, 2]. WHS presents as a constellation of characteristic features, including prenatal growth delay followed by short stature and slow weight gain, generalized hypotonia, variable degrees of intellectual disability, epilepsy, craniofacial dysgenesis, and various congenital midline fusion abnormalities [3, 4]. WHS is associated with a high mortality rate, approximately 30% within the first two years of life, with the most common causes of death being lower respiratory tract infections and congenital heart disease/cardiac failure [1, 5]. We present the case of a 3-month-old female with WHS undergoing laparoscopic gastrostomy tube placement, with a focus on airway management in a pediatric patient with craniofacial abnormalities
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