Abstract
Meckel-Gruber syndrome, characterized by occipital encephalocele, microcephaly, polydactyly, cleft lip or palate, mandibular micrognathism, and anatomical abnormality of the larynx and tongue, along with other associated malformations, is in the list of diseases associated with difficult airway. However, there has been no report on the management of general anesthesia and airway management for such patients. A 2-year-old girl with Meckel-Gruber syndrome was scheduled for cardioplasty and gastrostomy for gastroesophageal reflux under general anesthesia. Preoperative examination revealed obesity, microgenia, dysspondylism, proteinuria, hypoplastic kidneys, and stenosis of the anal canal. Although we anticipated some difficulty with the intubation and prepared several alternative methods for intubation, such as a bronchofiberscope and a laryngeal mask airway, tracheal intubation was completed without difficulty using conventional laryngoscopy after inhalational induction with sevoflurane. Because most patients with this syndrome die before and shortly after delivery, those who survive to some age might have less severe deformities.
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References
J McMillan C Deangelis D Feigin J Warshaw (1999) The nervous system; congenital anomalies of the central nervous system Oski's pediatrics. 3rd edn. Principles and practice. Lippincott Williams & Wilkins Philadelphia 1806
E Motoyama P Davis (1990) Clinical management of special surgical problems; anesthesia for general, urologic, and plastic surgery Smith's anesthesia for infants and children EditionNumber5th edn. Mosby St. Louis 607
T De Chalain R Hudgins F Burstein K Armfield (1994) ArticleTitleCombination of unilateral polydactyly, syndactyly, and clinodactyly with occipitocervical encephalocele and vertebral fusion Ann Plast Surg 33 86–89 Occurrence Handle7944206
N Gazioglu M Vural MS Seckin B Tuysuz E Akpir C Kuday B Ilikkan A Erginel A Cenani (1998) ArticleTitleMeckel-Gruber syndrome Childs Nerv Syst 14 142–145 Occurrence Handle10.1007/s003810050198 Occurrence Handle9579873
T Morishima K Sobue S Tanaka M So H Arima H Ando H Katsuya (2004) ArticleTitleSevoflurane for general anesthetic management in a patient with Larsen syndrome Paediatr Anaesth 14 194–195 Occurrence Handle10.1046/j.1460-9592.2003.01177.x Occurrence Handle14962340
K Goa S Noble C Spencer (1999) ArticleTitleSevoflurane in paediatric anaesthesia: a review Paediatr Drugs 1 127–153 Occurrence Handle10937447
A Infosino (2002) ArticleTitlePediatric upper airway and congenital anomalies Anesthesiol Clin North Am 20 747–766 Occurrence Handle10.1016/S0889-8537(02)00018-4
M Selim H Mowafi A Al-Ghamdi Y Adu-Gyamfi (1999) ArticleTitleIntubation via LMA in pediatric patients with difficult airways Can J Anesth 46 891–893
InstitutionalAuthorNameAmerican Society of Anesthesiologists Task Force on Management of the Difficult Airway (2003) ArticleTitlePractice guidelines for management of the difficult airway: an updated report by the American Society of Anesthesiologists Task Force on Management of the Difficult Airway Anesthesiology 98 1269–1277
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Miyazu, M., Sobue, K., Ito, H. et al. Anesthetic and airway management of general anesthesia in a patient with Meckel-Gruber syndrome. J Anesth 19, 309–310 (2005). https://doi.org/10.1007/s00540-005-0329-x
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DOI: https://doi.org/10.1007/s00540-005-0329-x