![]() At the end of the procedure, the child was extubated uneventfully and then shifted to the recovery room for observation and subsequently to wards.įig. All the monitored vitals and temperature were stable throughout the procedure. The surgery lasted for 6 hours with minimal blood loss. The patient developed bronchospasm with peak inspiratory pressure up to 36 cm of H 2O and relieved after steroid and salbutamol puff. For analgesia, a caudal block with 5 mL of 0.25% bupivacaine with 20 μg of clonidine was given. The anesthesia was maintained with oxygen, air, and sevoflurane to maintain MAC 1. The Cormac Lehane was grade I and he was intubated with 4.0 uncuffed ETT. After adequate muscle relaxation, laryngoscopy was done using CMAC videolaryngoscope Miller blade 1. atracurium 5 mg (after confirmation of mask ventilation) were given. ![]() The standard anesthesia monitors were attached and then inj. midazolam 1 mg in the preoperative room and once he was calm and quiet was shifted to the operating room. Since an IV line was in situ, we proceeded with IV induction. He had received nebulization and IV antibiotics in the wards. The child was allowed clear liquids 2 hours prior to surgery. 1: Characteristic features of Freeman–Sheldon syndrome We were anticipating a difficult airway, so the difficult airway cart was kept ready.įig. A pediatric ICU bed was kept booked for this patient. A valid informed consent was taken from the parents and all the risk were explained to them in the language they understood. ![]() The child was started on nebulization and intravenous (IV) antibiotics prior to surgery. The blood investigations and echocardiography were within normal range. Both the anesthesia exposures were uneventful. He had MRI of the brain done few days back under general anesthesia, which revealed brachycephaly. On further history, it was found that he had undergone some surgery for CTEV under general anesthesia, the details of which were not available. Patient’s mother gave history of running nose and cough a day prior to surgery. He had a deviated nasal septum with hypertrophied adenoids and also intermittent snoring episodes at night and also had global developmental delay. Oral examination showed microstomia-mouth opening of 1 cm, poorly formed teeth, and high-arched palate. On clinical examination, he had facial dysmorphism ( Figs 2 and 3)-flat occiput, low-set ears, widely spaced eyes, flat nose, midfacial hypoplasia, congenital left-sided facial palsy, and absent nasolabial folds. Keywords: Anesthesia, Difficult airway, Freeman–Sheldon syndrome, Microstomia.Ī 5-year-old male child, weight 10 kg, height 89.5 cm, presented to a tertiary care center for bilateral posterior release for congenital talipes equinovarus (CTEV) ( Fig. 2 We report the case of a child with Freeman–Sheldon syndrome with microstomia posted for posterior release of clubfoot. The presence of difficult airway, poor venous access, and possible susceptibility to malignant hyperthermia make anesthesia management challenging in these patients. Res Inno in Anesth 2019 4(1):19–20.įreeman–Sheldon syndrome 1 is a rare progressive myopathic disorder. Journal of Research and Innovation in Anesthesia Volume 4 | Issue 1 | Year 2019Ībhilasha D Motghare 1, Nutan D Kharge 2, Shrutee A Meshram 3, Indrani Hemantkumar 4ġ–3Department of Anaesthesiology, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Mumbai, Maharashtra, India 4Department of Anesthesiology, Topiwala National Medical College and BYL Nair Charitable Hospital, Mumbai, Maharashtra, IndiaĬorresponding Author: Abhilasha D Motghare, Department of Anaesthesiology, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Mumbai, Maharashtra, India, Phone: +91 9860819602, e-mail: to cite this article Motghare AD, Kharge ND, Meshram SA, et al.
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