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LETTER TO EDITOR
Year : 2013  |  Volume : 57  |  Issue : 6  |  Page : 632-633  

Anaesthesia challenges in Freeman-Sheldon syndrome


Department of Paediatric Anaesthesiology, B. J. Wadia Hospital, Parel, Mumbai, Maharashtra, India

Date of Web Publication20-Dec-2013

Correspondence Address:
Kiran Patel
6, Amber Palace, Chittaranjan Road, Vile Parle (East), Mumbai - 400 057, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5049.123352

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How to cite this article:
Patel K, Gursale A, Chavan D, Sawant P. Anaesthesia challenges in Freeman-Sheldon syndrome. Indian J Anaesth 2013;57:632-3

How to cite this URL:
Patel K, Gursale A, Chavan D, Sawant P. Anaesthesia challenges in Freeman-Sheldon syndrome. Indian J Anaesth [serial online] 2013 [cited 2019 Dec 14];57:632-3. Available from: http://www.ijaweb.org/text.asp?2013/57/6/632/123352

Sir,

We report a case of a 1-year-old male child, weighing 5.2 kg, who presented with bilateral inguinal hernia. Patient had retrognathia with pouting of lips [Figure 1], microstomia, ulnar deviation of fingers, bilateral congenital talipes equinovarus and knee dislocation. Examination of spine revealed scoliosis involving lower thoracic and lumbar spine. Neck extension was normal. There was no relevant family history. Patient was scheduled for an elective bilateral herniotomy. Pre-operative haematological and biochemical investigations were normal. Pre-operative evaluation revealed harsh breath sounds on auscultation. Hence bronchodilator nebulisation was given a day prior to surgery and also just prior to surgery. Baseline vitals were recorded with non-invasive monitors such as electrocardiogram, pulse oximeter and non-invasive blood pressure. 24 G intravenous cannula was secured on the upper limb. Appropriate size airway, laryngoscope blade, endotracheal tube, emergency tracheostomy tray were kept ready for emergency airway management. We chose a laryngeal mask airway (LMA) after intravenous induction with propofol and fentanyl to preserve spontaneous ventilation. Airway was secured with no. 1.5 LMA. Anaesthesia was maintained on O 2 , N 2 O and propofol infusion at 3 mg/kg/h with spontaneous respiration. Muscle relaxants were avoided. Regional anaesthesia was provided with ultrasonography (USG) guided hernia block using Sonosite M-Turbo ® machine with high frequency linear probe. 0.25% bupivacaine 2 ml was used on each side for nerve block. Intraoperative vitals including end tidal CO 2 (EtCO 2 ) and temperature remained stable and procedure was uneventful. Duration of surgery was 30 min. At the end of surgery, LMA was removed when patient was awake, with return of reflexes. Post-operative analgesia was provided with acetaminophen suppository 80 mg twice daily. Freeman-Sheldon syndrome, a slowly progressive congenital myopathy usually presents with multiple body contractures, skeletal malformations and craniofacial deformities such as microstomia, micrognathia, microglossia, a high arched palate and midfacial hypoplasia characteristically known as whistling mouth syndrome. [1] Facial, limb and respiratory muscles are primarily affected, also known as craniocarpotarsal dystrophy, whistling face-Windmill Vane Hand syndrome and type of arthrogryposis multiplex congenita. [2] Multiple orthopaedic, spine and soft-tissue procedures are performed in these children, for improving the quality-of-life. Systemic nature of this condition often poses a challenge to anaesthetists in airway management, intravenous access and body positioning and regional anaesthesia. The combination of abnormal facial features make direct laryngoscopy and intubation extremely challenging. [3] However, microglossia and high arched palate allows proper placement of LMA. [3] These patients are highly susceptible to muscle rigidity, masseter spasm and malignant hyperthermia. [4] So our technique of securing airway with LMA and maintaining spontaneous ventilation is well-suited for these patients. But at the same time appropriate size airway, laryngoscope blade, endotracheal tube, emergency tracheostomy tray should be kept ready for emergency airway management. Post-operative complications like respiratory insufficiency, [5] upper airway tract obstruction can be decreased by minimal use of systemic sedatives and opiods along with successful regional anaesthesia. Hence peripheral nerve locator and USG guided blocks are useful for regional anaesthesia. [6] Every syndrome complex has a unique cluster of abnormalities. Freeman-Sheldon syndrome also has such manifestations, which are particularly challenging from the anaesthetists perspective. This case highlights the technical modifications, which are necessary to overcome these challenges, for successful management of a patient with this syndrome.
Figure 1: Whistling face deformity with other facial features in Freeman‑Sheldon syndrome (published with permission, from child's parent)

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   References Top

1.Richa FC, Yazbeck PH. Anaesthetic management of a child with Freeman-Sheldon syndrome undergoing spinal surgery. Anaesth Intensive Care 2008;36:249-53.  Back to cited text no. 1
[PUBMED]    
2.Bamshad M, Jorde LB, Carey JC. A revised and extended classification of the distal arthrogryposes. Am J Med Genet 1996;65:277-81.  Back to cited text no. 2
[PUBMED]    
3.Chen A, Lai HY, Lee Y, Yang YL, Ho JS, Shyr MH. Anesthesia for Freeman-Sheldon syndrome using a folded laryngeal mask airway. Anesth Analg 2005;101:614-5.  Back to cited text no. 3
[PUBMED]    
4.Okawa M, Kinouchi K, Kitamura S, Taniguchi A, Sasaoka N, Fukumitsu K. Anesthetic management of an infant with Freeman-Sheldon syndrome. Masui 2002;51:659-62.  Back to cited text no. 4
[PUBMED]    
5.Jones R, Dolcourt JL. Muscle rigidity following halothane anesthesia in two patients with Freeman-Sheldon syndrome. Anesthesiology 1992;77:599-600.  Back to cited text no. 5
[PUBMED]    
6.Vas L, Naregal P. Anaesthetic management of a patient with Freeman Sheldon syndrome. Paediatr Anaesth 1998;8:175-7.  Back to cited text no. 6
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