An infant with Beals- Hecht syndrome: An airway challenge for the anaesthesiologist

Abstract

Beals-Hecht syndrome is a rare connective tissue disorder. Due to multiple contractures, it is associated with difficult airway and other anaesthesia related problems. We hereby present a case of a 2 month old infant who was scheduled for surgical repair of a large inguinal hernia. In view of the anticipated difficult airway, owing to the presence of micrognathia, limited mandibular excursion and cleft palate in this patient, we induced the child with sevoflurane maintaining spontaneous breathing and successfully intubated the child using video laryngoscope (Medicam paediatric video laryngoscope) with Miller blade size 0. Intraoperative course was uneventful, and the child was extubated at the end of surgery.

2022 American Society of Anesthesiologists Practice Guidelines for Management of the Difficult Airway

“…Case reports observed videolaryngoscope intubation successes with a wide range of difficult airway conditions (Category B4-B evidence ). 160,276–297 Adverse outcomes that may occur include sore throat, laryngospasm, lip, dental, or mucosal injuries ( Category B4-H evidence ). 278,298…”

Practical considerations in the anaesthetic management of thoracolumbar scoliosis correction surgery in a child with hecht–beals syndrome

“…Since its first description, the anaesthetic management of HBS or Dutch-Kentucky syndrome mainly revolves around airway management. [1][2][3][4] Preoperative cardiorespiratory assessment is of utmost importance to chalk out the management plan. Peripheral venous access may be difficult due to the presence of multiple contractures.…”

“…In reported cases, inhalation induction was chosen over IV agents to avoid sudden loss of airway patency. [1][2][3][4] However, inhalation agents, may irritate the airway. Hence, we used a combination of both to achieve acceptable intubating conditions.…”

“…Nasreen and Khalid also used videolaryngoscope, stylet, and external laryngeal manipulation to intubate a 2-monthold infant with HBS. 2 Careful placement of limbs during prone positioning is essential due to the presence of fixed flexion deformity. We placed the child's left hand in the gap between two bolsters due to the presence of contracture at the elbow (Figure 1 d).…”

A prospective randomised study to compare the efficacy of Medicam pediatric video Laryngoscope and Macintosh Laryngoscope as an intubating device in pediatric patients: Videolaryngoscopy in pediatric airway

“…In recent years, more sophisticated and advanced airway equipment has led to better outcomes for paediatric patients. Video laryngoscopes (VL) have emerged as a mainstay of the paediatric airway as they give a much better laryngeal view compared to direct laryngoscopy in children with normal or difficult airways (3)(4)(5). Several studies have evaluated the efficacy of video laryngoscopes in the paediatric population, with varying results (6-7).…”