Endotracheal intubation in an adult patient of Hallermann-Streiff syndrome-Case report and review of literature.

Abstract

Dear Editor, Hallermann–Streiff syndrome (HSS), a rare genetic polymalformative dysostic syndrome with only 150 reported cases, was first described in 1893 and is characterized by bird-like facies, hypoplasia of nasal bridge, mandibular hypoplasia, hypertrichosis, ocular abnormalities, severe tracheomalacia, and dwarfism.[1] Because of facial features, airway management is considered difficult in them; however, reports are scarce with only two adult patients previously reported.[1] Paucity of literature on airway management in adult HSS patients prompted us to report the present patient as when syndromes are rare, case reports guide an anesthesiologist for management. A 20-year-old female patient of HSS, American Society of Anesthesiologists grade I, 55 kg, and 154 cm height, was posted for septo-rhinoplasty because of progressive nasal deformity since birth. She had history of snoring not associated with daytime somnolescence or night awakenings. Airway examination revealed microstomia, mouth opening of 2 cm, retrognathia, hyomental distance of 21/2 fingers, large tongue, crowding of teeth, high arched palate, small nares, and modified Mallampati Grade 3 [Figures 1 and 2]. Systemic examination and biochemical profile revealed no abnormality. As patient was anxious regarding awake fiberoptic intubation (FOI), plan was to first assess Cormack and Lehane (CL) grade on laryngoscopy in spontaneously breathing patient without muscle relaxants and in case of failure of endotracheal intubation (ETI) oral FOI under anesthesia was planned (nasal FOI was not possible due to small nares). Informed consent for emergency tracheostomy was taken. In the operation theater, routine monitors were attached. Following preoxygenation with 100% oxygen for 3 min, intravenous ketamine 50 mg with glycopyrrolate 0.2 mg was injected and sevoflurane 2–8% was gradually administered in 4–6 L/min 100% O2 preserving spontaneous respiration. Oropharyngeal airway was required for effective ventilation. Under deep anesthesia, check laryngoscopy with C Mac video laryngoscope size 3 blade revealed CL grade 1. Following this, injection atracurium 0.5 mg/kg was given and a cuffed endotracheal tube 6.5 was inserted successfully at first attempt. Intraoperative analgesia was supplemented with injection fentanyl 2 mcg/kg and paracetamol. Rest of the intraoperative period was uneventful. Surgery lasted for 3 h. At the end of surgery, neuromuscular blockade was reversed with neostigmine and glycopyrrolate and trachea extubated with no adverse sequelae. Patient was observed in recovery room for 2 h and was discharged from ward after 2 days.Figure 1: Frontal view of patient showing small and deformed naresFigure 2: Lateral view of patient showing retrognathia and decreased hyomental distanceThere are only eight reported cases of anesthesia management in HSS patients with two described in adults [Table 1][23456789] of which details of two non-English PubMed reported patients were not found.[46]Table 1: Reported cases of HSS patientsIn the reported adult patients, ETI was anticipated to be difficult; thus, awake ETI was performed in one and lighted stylet via the intubating laryngeal airway was used in the other. However in both patients, ETI was easily performed.[89] In the present patient also, airway management was anticipated to be difficult because of associated facial features like microstomia, <2 cm mouth opening, retrognathia, large tongue, crowding of teeth, and high arched palate. Contrarily ETI was easily established without any manipulations or adjuncts. This could be probably due to presence of small teeth, normal BMI, and adequate mouth opening. Through this case report, we want to highlight that even though adult HSS patients are considered to have difficult ETI, previous two reports including the present one have indicated that in an adult HSS patient, ETI might not be difficult; however, mask ventilation might be challenging. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.