Hallermann-Streiff syndrome is a congenital syndrome characterized by multiple maxillofacial anomalies, including microstomia, mandibular hypoplasia, dental anomalies, hypertichosis, difficult airway, and ophthalmologic abnormalities.1–7 In consideration of the potential airway difficulties, the Intubating Laryngeal Airway by Cookgas®, LLC (ILA™, Mercury Medical, Clearwater, FL, USA) is a new supraglottic airway device with a functionality and insertion technique similar to that of an intubating laryngeal mask airway.8,9 The lighted stylet has been used as an adjunct to guide the passage of a tracheal tube through an intubating laryngeal mask airway.10,11 We describe the successful application of a lighted stylet-guided tracheal tube insertion through an ILA™ in a patient with Hallermann-Streiff syndrome and an anticipated difficult airway. Written consent for publication of the manuscript and the patient image was granted by the patient.

Case report

A 26-year-old male (32.7 kg, 165 cm) with Hallermann-Streiff syndrome was scheduled for a vitrectomy. He had undergone multiple surgical procedures under general anesthesia since childhood, and over the previous five years, he had undergone three eye surgeries performed under general anesthesia with laryngeal mask airways. The patient had a high school education and the mental capacity to provide consent for medical procedures.

He presented with mandibulofacial dystocia involving a bird-like appearance (Fig. 1). He had a 4 cm mouth opening, a receding chin, a normal temporomandibular joint, and a stable cervical spine. The Mallampati examination was class 3. The surgeon stated the critical nature of the retinal surgery and requested assurance of complete paralysis during the procedure. The planned procedure was to induce general anesthesia using short-acting anesthetic drugs and to insert an ILA™ followed by lighted stylet-guided tracheal intubation. If this method was unsuccessful, the back-up plan was fibreoptic bronchoscope-guided tracheal intubation.

Fig. 1
figure 1

Lateral profile view of a patient with typical facial features seen in Hallermann-Streiff syndrome. Note the mandibulofacial dystocia with bird-like facies, parrot-beaked nose, small mouth opening, and receding chin

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Standard monitoring included an electrocardiograph, a non-invasive blood pressure monitor, and a pulse oximeter. A 20G intravenous catheter was inserted. Anesthesia was induced with midazolam 1 mg iv, fentanyl 50 μg iv, and propofol 80 mg iv. A size 3.5 ILA™ (Fig. 2) was inserted in a manner similar to that for standard laryngeal mask airway insertion; then the cuff was inflated with 15 ml of air. Lung ventilation was verified by observation of chest wall movement and the presence of normal end-tidal carbon dioxide waveforms.

Fig. 2
figure 2

(Top) Lateral view of the Intubating Laryngeal Airway (ILA™): (A) removable 15-mm connector; (B) transparent shaft; (C) inflatable silicone ILA™ bowl with a keyhole-shaped airway outlet and mask ridges. (Bottom) Anterior view of the ILA™ showing the inside of the ILA™ bowl: (A) keyhole-shaped airway outlet; (B) mask ridges

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After successful ventilation using the ILA™, the patient received succinylcholine 60 mg iv. The following steps were taken to insert the tracheal tube. First, the rigid stylet was removed from the lighted stylet wand (Trachlight®, Laerdal Medical Corporation, Wappingers Falls, NY, USA; Fig. 3). Second, a conventional tracheal tube (7.5-mm internal diameter, Mallinckrodt Inc, Hazelwood, MO, USA) was mounted and clamped onto the lighted stylet in the usual manner.12 Third, the lighted stylet-tracheal tube assembly was inserted through the ILA™. As the assembly was being advanced, a distinct dime-sized glow was observed in the anterior part of the patient’s neck at the super-thyroid notch, the cricothyroid membrane, the trachea, and, finally, the supra-sternal notch. The endotracheal tube was released from the lighted stylet clamp, and the lighted stylet was removed while the position of the tracheal tube was maintained. The tracheal tube cuff was inflated with 5 ml of air, and the correct tracheal tube position was confirmed by capnography. The ILA™ cuff was deflated and left in situ, and the tracheal tube was then taped and secured. The alternative was to remove the ILA™ while keeping the tracheal tube in position using a stabilizer, a technique similar to that using the intubating laryngeal mask airway. We elected to proceed with the former approach. Anesthesia was maintained with sevoflurane, oxygen, and air and progressed uneventfully.

Fig. 3
figure 3

Lateral view of a tracheal tube mounted on a lighted stylet wand with the rigid stylet removed: (A) conventional 7.5-mm tracheal tube; (B) lighted stylet wand; (C) rigid stylet

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At the end of the surgery, while the patient remained under inhalational anesthesia, both the endotracheal tube and the ILA™ were removed. A Cormack and Lehane grade 4 view was observed under direct laryngoscopy with a # 3 Macintosh blade. The ILA™ was reinserted and sevoflurane was discontinued. The patient awakened and the ILA™ was removed without complications.

Discussion

This case describes a novel application of a lighted stylet-guided tracheal tube insertion through an ILA™ in a patient with Hallermann-Streiff syndrome.

Hallermann-Streiff syndrome, also known as Hallerman-Streiff-Francois syndrome, Francois Dyscephalic syndrome, Oculomandibulofacial syndrome, or Oculo-Mandibulo-Dyscephaly-Hypotrichosis syndrome, was initially described by Aubry in 1893.1 The characteristic features include dyscephaly, mental retardation, bird-like facies, a hypoplastic nose, microstomia, high arched palate, mandibular hypoplasia, anterior displacement of temporomandibular joint, an anterior larynx, and dental abnormalities, including natal malformed brittle teeth.2,3 Multiple ophthalmological abnormalities have also been described.3 Due to the abnormal anatomy of the upper airway, difficult airway management has been described.2–6

To date, several Hallermann-Streiff syndrome studies have suggested that the anesthesiologist use, or have available, alternative airway equipment for tracheal intubation.3–6 The options for tracheal intubation include nasal intubation, oral intubation, and tracheostomy.4–7 However, small nares, a hypoplastic nose, and a deviated nasal septum can make nasal intubation difficult.3,4 In addition, a small mouth, displaced temporomandibular joint, hypoplastic mandible, and an anterior glottis may lead to difficult laryngoscopy and visualization.3,4 If these difficulties are encountered, awake tracheostomy has been recommended; however, a short, thick neck has often been associated with the cricoid cartilage at the level of the suprasternal notch.5,6

Recent practice guidelines from the American Society of Anesthesiologists, the Difficult Airway Society, and the Canadian Airway Focus Group recommend the use of alternative airway devices, for instance, the intubating laryngeal mask airway, in the management of patients with anticipated and unanticipated difficult airways.13–15 The use of a fibreoptic bronchoscope or a lighted stylet, in conjunction with an intubating laryngeal mask airway, has also been described.10,11,16,17 There is a higher success rate and a decreased intubation time with lighted stylet-guided intubation compared to blind intubation through an intubating laryngeal mask airway.10,11 The ILA™, a new supraglottic airway device first introduced for North American clinical use in 2004, has been recommended as an alternative device for tracheal intubation.9 Although the ILA™ and the laryngeal mask airway share functional similarities, there are a number of notable differences. Compared to the laryngeal mask airway, the ILA™ has a removable 15-mm circuit connector, no aperture bars at the ventilatory opening, and shorter shaft distances, thereby allowing insertion of larger diameter tracheal tubes.9 Also, the ILA™ does not have a metal handle or a metal shaft; it is inserted like a standard laryngeal mask airway, and regular polyvinyl chloride tracheal tubes can be utilized.18 This report describes successful lighted stylet-guided tracheal tube insertion through an ILA™ in a patient with Hallermann-Streiff syndrome.

There are several advantages to the presently described technique for tracheal intubation: (1) The ILA™ is relatively inexpensive; (2) there is familiarity with the insertion technique, due to similarities with the intubating laryngeal mask airway; (3) standard tracheal tubes can be used; (4) a flexible fibreoptic bronchoscope is not required; (5) ventilation can be maintained between intubating attempts; and (6) tracheal tube advancement guided by transillumination provides confirmation of intratracheal location, and positioning the tracheal tube tip at the suprasternal notch results in an approximate mid-tracheal location.12 However, disadvantages do exist. (1) Anatomical features of Hallermann-Streiff syndrome, such as a small mouth, high-arched palate, mandibular hypoplasia, anterior displacement of temporomandibular joint, an anterior larynx, and dental abnormalities may prevent the use of supraglottic airways.3,5,7 (2) Although lighted stylet-guided tracheal intubations through supraglottic devices are associated with a higher success rate, they are not universally successful. Thus, alternative techniques, including fibreoptic bronchoscopy, must be available. (3) The ILA™ is only available in sizes 2.5, 3.5, and 4.5; therefore, its use in smaller children and infants is limited. Just recently, a size 1.5 ILA™ became available for children weighing 10–20 kg.

In conclusion, patients with Hallermann-Streiff syndrome have multiple anatomical abnormalities and potentially difficult airways. We describe a novel application of a lighted stylet-guided tracheal tube insertion through an ILA™ in a patient with Hallermann-Streiff syndrome. This intubation technique can be considered as a primary route of intubation in patients with difficult airways or as a secondary rescue strategy if a primary method fails. Further studies are needed to assess the effectiveness of ILA™-guided intubation with fibreoptic bronchoscopy.