Hallermann-Streiff Syndrome (HSS): Anesthetic Considerations
Aynur Mustafayeva, Ruslan Abdullayev, Yavuz Kelleci, Meliha Orhon Ergün, Tümay Umuroğlu,
Marmara Üniversitesi Tıp Fakültesi, Anesteziyoloji Ve Reanimasyon Anabilim DalıMarmara Üniversitesi İstanbul Pendik Eğitim Ve Araştırma Hastanesi, Anesteziyoloji Ve Reanimasyon Bölümü
Introduction: HSS was probably firstly discovered by Aubry in 1893, thereafter was totally described by Hallermann, Streiff and François in 1940th and 50th. As a congenital disorder, HSS includes several abnormalities especially on the face and head, such as bird-like appearance, small mandible, dental and eye anomalies, short stature and low intelligence quotient in some cases. We would like to introduce to your attention a case report of HSS with our anesthetic approach for varicocele repair. Case: A 14-year-old male patient (36 kg weight, 145 cm height) was planned to undergo bilateral varicocelectomy. He had six uneventful eye surgeries previously. Characteristic facial features like bird face, small mandible, hooked nose, ptosis, hypotrichosis, and dental anomalies were present. His mental development was normal. Due to hypophyseal insufficiency the patient was on growth hormone and steroid treatment. Anesthesia induction was performed with propofol 2.5 mg kg-1, remifentanil 1 µg kg-1, rocuronium bromide 0.6 mg kg-1. According to the facial features the patient was considered to have a difficult airway. His sternomental, thyromental and hyomental distances were 11, 5 and 1.5 cm respectively. Mallampati score was 3. Pharyngeal anatomy showed difficult airway on intubation as well. The procedure was done with videolaryngoscopy which took 25 seconds to perform. The patient was extubated uneventfully. Discussion: Difficult airway should always be anticipated in all patients with HSS, and the airway control plan should be established preoperatively using difficult intubation algorithms. Different approaches to establish the airway have been described in the literature, like intubation via Airtraq laryngoscope or lighted stylet-guide. It should be kept in mind that the possibility of difficult intubation may increase with age. No matter what the technique is, proper anesthetic plan should be ready preoperatively for HSS patients.
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