Dear Editor, We report the perioperative management of a 1 year-10 month-old girl child with features of Floating-Harbor syndrome (FHS) such as a triangular face, frontal bossing, flat nasal bridge, long eyelashes, retrognathia, and delayed speech development with bilateral Moya-Moya disease (MMD) for right superficial temporal artery (STA) to middle cerebral artery (MCA) bypass and encephalo-duro-arterio-myosynangiosis surgery Figure 1a. She presented with a history of seizures, left-sided weakness with severe stenosis of the bilateral supra-clinoid internal carotid arteries (ICA), MCA, and anterior cerebral artery (ACA) on magnetic resonance angiography Figure 1b. There was no history of recurrent respiratory infections or feeding difficulties with Mallampati class 3 on examination. Genetic testing revealed SNF2-related CREBBP activator protein (SRCAP) gene (+) in Intron 26.Figure 1: (a) Image of the child showing flat nasal bridge, long eyelashes (partly covered with mask to conceal patient identity), and retrognathia. (b) Magnetic resonance angiography showing severe stenosis of the right supraclinoid internal carotid arteryIntra-operatively after anaesthesia induction with intravenous fentanyl, propofol, and atracurium, mask ventilation was achieved with bilateral jaw thrust and the airway was secured with a 4 mm internal diameter (ID) uncuffed endotracheal tube (ETT) using a CMAC (Karl Storz) video-laryngoscope (Cormack Lehane II A). Anaesthesia was maintained using sevoflurane in air: oxygen (1:1) mixture and fentanyl/atracurium infusion with positive pressure ventilation (PPV). Standard monitors with invasive arterial monitor were used, and normoxia, normocarbia, and euvolaemia were maintained. Following an uneventful surgical procedure and upon assessment for consciousness, respiration, and adequacy of motor power of limbs (after reversal of muscle relaxant), the trachea was extubated in the intensive care unit. The child cried immediately after extubation. However, she developed stridor, which was managed initially with face mask PPV, propofol, and adrenaline nebulisation. However, she again developed stridor which minimally improved on further adrenaline nebulisation. She was reintubated with 4 mm ID ETT using a CMAC video-laryngoscope after administering propofol and atracurium. There were no features of glottic or supraglottic airway oedema. The lowest recorded oxygen saturation during the episode was 95%, with a stable heart rate and blood pressure. She was sedated overnight with intermittent fentanyl boluses, and the trachea was uneventfully extubated the next morning. FHS is a rare, autosomal dominant disorder secondary to mutations in the SRCAP gene.1 Clinical features include short stature/neck, prominent occiput, triangular face, sunken eyes, long eyelashes, wide nose, thin lips, narrow palate, delay in speech/language development, and high pitch voice which can complicate airway management while anaesthetising them (not previously described).1,2 MMD is typically the stenosis of the intracranial ICA and its branches, making them prone to chronic cerebral ischaemia and stroke. Preventing further vasoconstriction of such a compromised brain circulation by maintaining normocarbia, adequate hydration, and avoiding further hypoxia becomes the main anaesthetic goal which can be complicated by FHS.2,3 In view of the difficult airway features, a video-laryngoscope was used in our case. Despite satisfying the extubation criteria, she developed postoperative stridor which was initially treated with PPV, propofol, and adrenaline nebulisation; it however recurred. Since further hypoxia could have potentially worsened cerebral ischaemia in MMD, she was reintubated and ventilated overnight. Prophylactic use of corticosteroid has been suggested to prevent post-extubation stridor (by reducing airway oedema) in paediatric neurosurgical patients.4 Our child was completely awake during extubation without any features suggestive of a hyperreactive airway such as coughing, rhonchi with absence of a history of gastroesophageal reflux, and respiratory infection that would have contributed to stridor. The video-laryngoscope also did not show features of airway oedema, and the same size ETT was used during reintubation. Evidence of induced SRCAP gene dysfunction (animal experiments) causing craniofacial and laryngeal abnormalities (human equivalent of first to fourth pharyngeal arch structural abnormalities) indicate the possibility of laryngeal cartilage abnormality in FHS.5 With this background, we feel that a possible dynamic airway collapse secondary to laryngeal cartilage abnormality following anaesthesia and airway manipulation could have led to post-operative stridor in our case. Also, a report of unmasked laryngomalacia during sevoflurane induction in a child who was completely free of stridor since 1.5 years seemingly supports our hypothesis.6 We conclude that being aware of such a post-operative complication in a FHS child would help in adequate preparedness and timely management. This can prove to be useful in preventing further cerebral ischaemia and stroke, especially in a MMD child with a pre-existing compromised cerebral circulation. Declaration of Patient consent The authors certify that they have obtained all appropriate consent forms from the patient's parents. In the form, the parents consented to the images and other clinical information to be reported in the journal. They understand that the child's name and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Disclosure of use of artificial intelligence (AI)-assistive or generative tools The AI tools or language models (LLM) have not been utilised in the manuscript. Author contributions AA was involved in-concepts, literature search, manuscript preparation, review and approval. SS was involved in-manuscript preparation, literature search, review and approval. MS was involved in-concepts, editing, review and approval. RP was involved in-concepts, literature search, manuscript preparation, editing, review and approval. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.
Ayyappan et al. (Sun,) studied this question.