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Difficult intubation in an infant with Hallermann–Streiff syndrome–easy with Airtraq laryngoscope

Hallermann–Streiff syndrome (oculo mandibulo dyscephaly) is a rare congenital disorder with approximately 150 cases reported (1). Cardinal features are dyscephaly with bird facies, frontal/parietal bossing, dehiscence of sutures, hypotrichosis, cutaneous atrophy of scalp and nose, microphthalmia, congenital cataracts, blue sclera, nystagmus, mandibular hypoplasia, forward displacement of temporomandibular joints, high-arched palate, small mouth, multiple dental anomalies, and proportionate small stature (2). We describe the airway management of a 9-month-old infant with Hallermann–Streiff syndrome scheduled for right eye lens aspiration. A 9-month-old female infant weighing 7.3 kg was scheduled for right eye lens aspiration for bilateral developmental cataract. Parents had noticed decreased vision and nystagmus since birth. History of snoring during sleep was present. The motor and social development was normal. Physical examination revealed short stature (65.5 cm-5th percentile), weight of 7.3 kg (10th percentile), microcephaly (head circumference39.5 cm), prominent forehead, bitemporal hollowing, flat occiput, fibrous closure of anterior fontanel, small nose with prominent columella, bilateral cataract, nystagmus, and clinodactyly. Airway examination revealed adequate mouth opening, single prominent upper incisor, retrognathia, double chin, and high-arched palate. Examination of the cardio-respiratory system was normal. Hallermann–Streiff syndrome was diagnosed by the geneticist based on these features. Anticipating difficult airway, we kept the difficult airway cart ready (1–3). Before shifting to the operating room (OR), the infant was fasting adequately and had an intravenous (IV) access secured. Plan of anesthesia was inhalational induction followed by tracheal intubation without the use of muscle relaxants. In the OR, inhalational induction of anesthesia with 1–8% v/v sevoflurane in 100% oxygen was begun, simultaneously applying the standard monitoring. IV glycopyrrolate 0.07 mg was injected. After attaining adequate depth of anesthesia, ventilation was gently assisted to confirm the ease of ventilation. Because there was no evidence of airway obstruction, anesthesia was deepened with IV propofol 10 mg before attempting direct laryngoscopy. Direct laryngoscopy with Macintosh laryngoscope revealed thick stout unliftable epiglottis, with no part of glottis visualized (Cormack and Lehane grade 3b), despite optimal external laryngeal manipulation and attempts to optimize head and neck position. One attempt at blind intubation by maneuvering the styletted tracheal tube behind the epiglottis failed. Anesthesia was deepened with sevoflurane in oxygen and ease of mask ventilation was reconfirmed. To minimize airway trauma that could result from multiple attempts, we decided to use Airtraq laryngoscope (Airtraq optical laryngoscope; Prodol Meditec S.A., Las Arenas, Vizcaya, Spain) for second attempt at intubation. With Airtraq laryngoscope, the view obtained was Cormack and Lehane grade 2a. The thick and stout epiglottis still remained in the field of vision, though unhampering the glottic view. Tracheal intubation with a 3.5 mm internal diameter uncuffed PVC tube was easy. Correct placement of the tracheal tube was confirmed. Subsequent anesthetic management was uneventful. Several factors contribute to ‘difficult airway’ associated with Hallermann–Streiff syndrome. The ascending ramus of the mandible is either underdeveloped or absent resulting in a small mouth cavity. The temporomandibular joint can be easily dislocated. Small nostrils, deviated nasal septum, high-arched palate, and anterior larynx may preclude even blind nasal intubation in these cases (3). In this case, besides several findings associated with Hallermann–Streiff syndrome, the infant had the following predictors of difficult airway: history of snoring, high-arched palate, and receding mandible. As difficult intubation and difficult mask ventilation have been described in two cases reported, we decided to secure the airway under deep inhalational anesthesia preserving the spontaneous ventilation (2,3). We attempted intubation using Macintosh laryngoscope based on our preference. We did not try intubation using a Miller blade or bougie to minimize the attempts at intubation. Our other options of airway management at this point of time were fiberoptic aided intubation under general anesthesia and use of laryngeal mask airway. As review of literature had shown that Airtraq laryngoscope is useful in cases with receding mandible, we used it after failure of the first attempt (4,5). As evident here, intubation with Airtraq laryngoscope was successful and easy when intubation Correspondence