Abstract
Larsen syndrome is a rare, inherited, connective tissue disorder which is characterized by facial dysmorphisms, congenital joint dislocations and multi-system involvement including musculoskeletal, respiratory, cardiovascular, and neurological systems. Children with Larsen syndrome are prone to undergo a wide variety of surgical treatments for their orthopedic abnormalities. For this reason these patients may require repeated anesthetic care during surgical procedures. The major concerns about these patients regarding anesthesia include perioperative respiratory dysfunction, cardiovascular problems and difficult airway management. Laryngomalacia, tracheomalacia, bronchomalacia and subglottic stenosis are the main causes for difficult airway management in this syndrome. In this case report we discussed, in light of literature, the anesthetic management of a pediatric patient with Larsen syndrome who underwent a pes equinovarus surgery
References
- Sajnani AK, Yiu CK, King NM. Larsen syndrome: a review of the literature and case report. Spec Care Dentist. 2010;30:255-60.
- Bicknell LS, Morgan T, Bonafé L, et al. Mutations in FLNB cause boomerang dysplasia. J Med Genet 2005;42:3.
- Malik P, Choudhry DK. Larsen syndrome and its anaesthetic considerations. Paediatr Anaesth. 2002;12:632-6.
- Arun O, Oc B, Yildirim S, Simsek M, Oc M, Duman A. Anesthetic management in a newborn infant with Larsen syndrome undergoing closure of patent ductus arteriosus: Case report. Journal-Cardiovascular Surgery 2015:3:48-50.
- Kulkarni M, Nataraj MS, Sarah C. Malignant hyperthermia-like episode in a child with Larsen syndrome. Saudi J Anaesth 2012;6:86-7
- Larsen LJ, Schottstaedt ER, Bost FC. Multiple congenital dislocations associated with characteristic facial abnormality. J Pediatr 1950;37:574-81.
- Latta RJ, Graham CB, Aase J, Scham SM, Smith DW. Larsen’s syndrome: a skeletal dysplasia with multiple joint dislocations and unusual facies. J Pediatr. 1971;78:291-8.
- Stevenson GW, Hall SC, Palmieri J. Anesthetic considerations for patients with Larsen’s syndrome. Anesthesiology 1991;75:142-4.
- Tobias JD. Anesthetic implications of Larsen syndrome. J Clin Anesth. 1996 ;8:255-7.
- Arslan G, Temizel F, Kuzucuoğlu T, Arıkan Z. Larsen Sendromu ve Anestezik Yaklaşım: Olgu Sunumu Bakırköy Tıp Dergisi 2006;2:144-6.
- Swensson RE, Linnebur AC, Paster SB. Striking aortic root dilatation in a patient with the Larsen syndrome. J Pediatr. 1975;86:914-5.
- Kiel EA, Frias JL, Victorica BE. Cardiovascular manifestations in the Larsen syndrome. Pediatrics. 1983;71:942-6.
- Liang CD, Hang CL. Elongation of the aorta and multiple cardiovascular abnormalities associated with larsen syndrome. Pediatr Cardiol. 2001;22:245-6.
- Ghaffaripour S, Ghahramaninejad F, Shahbazi Sh. Malignant hyperthermia in Larsen syndrome. Paediatr Anaesth 2009;19:927-8.
- Morishima T, Sobue K, Tanaka S, So M, Arima H, Ando H et al. Sevoflurane for general anaesthetic management in a patient with Larsen syndrome. Paediatr Anaesth. 2004;14:194-5.
Abstract
Larsen sendromu; yüz dismorfizmi, konjenital eklem dislokasyonları ve kas-iskelet, solunum, kardiyovasküler ve nörolojik sistemleri içeren çoklu sistem tutulumu ile karakterize nadir bir kalıtsal bağ dokusu hastalığıdır. Ortopedik sorunlara bağlı olarak özellikle pediatrik hastalarda, var olan anomalileri nedeniyle, çeşitli cerrahi prosedürler geçirmek zorunda kalırlar. Bu nedenle bu hastalarda tekrarlayan anestezi ihtiyacı gerekebilmektedir. Anestezi yönetimi açısından hastaların majör problemleri genellikle perioperatif solunum fonksiyon bozuklukları, kardiyovasküler problemler ve zor havayolunu içerir. Laringomalazi, trakeomalazi, bronkomalazi ve subglottik stenoz, bu sendromun zor havayolu yönetimi açısından en büyük sorunları arasında yer alırlar. Bu olgu sunumunda Pes ekinovarus cerrahisi planlanan Larsen sendromlu çocuk hastada anestezi yönetimini literatür eşliğinde tartıştık
References
- Sajnani AK, Yiu CK, King NM. Larsen syndrome: a review of the literature and case report. Spec Care Dentist. 2010;30:255-60.
- Bicknell LS, Morgan T, Bonafé L, et al. Mutations in FLNB cause boomerang dysplasia. J Med Genet 2005;42:3.
- Malik P, Choudhry DK. Larsen syndrome and its anaesthetic considerations. Paediatr Anaesth. 2002;12:632-6.
- Arun O, Oc B, Yildirim S, Simsek M, Oc M, Duman A. Anesthetic management in a newborn infant with Larsen syndrome undergoing closure of patent ductus arteriosus: Case report. Journal-Cardiovascular Surgery 2015:3:48-50.
- Kulkarni M, Nataraj MS, Sarah C. Malignant hyperthermia-like episode in a child with Larsen syndrome. Saudi J Anaesth 2012;6:86-7
- Larsen LJ, Schottstaedt ER, Bost FC. Multiple congenital dislocations associated with characteristic facial abnormality. J Pediatr 1950;37:574-81.
- Latta RJ, Graham CB, Aase J, Scham SM, Smith DW. Larsen’s syndrome: a skeletal dysplasia with multiple joint dislocations and unusual facies. J Pediatr. 1971;78:291-8.
- Stevenson GW, Hall SC, Palmieri J. Anesthetic considerations for patients with Larsen’s syndrome. Anesthesiology 1991;75:142-4.
- Tobias JD. Anesthetic implications of Larsen syndrome. J Clin Anesth. 1996 ;8:255-7.
- Arslan G, Temizel F, Kuzucuoğlu T, Arıkan Z. Larsen Sendromu ve Anestezik Yaklaşım: Olgu Sunumu Bakırköy Tıp Dergisi 2006;2:144-6.
- Swensson RE, Linnebur AC, Paster SB. Striking aortic root dilatation in a patient with the Larsen syndrome. J Pediatr. 1975;86:914-5.
- Kiel EA, Frias JL, Victorica BE. Cardiovascular manifestations in the Larsen syndrome. Pediatrics. 1983;71:942-6.
- Liang CD, Hang CL. Elongation of the aorta and multiple cardiovascular abnormalities associated with larsen syndrome. Pediatr Cardiol. 2001;22:245-6.
- Ghaffaripour S, Ghahramaninejad F, Shahbazi Sh. Malignant hyperthermia in Larsen syndrome. Paediatr Anaesth 2009;19:927-8.
- Morishima T, Sobue K, Tanaka S, So M, Arima H, Ando H et al. Sevoflurane for general anaesthetic management in a patient with Larsen syndrome. Paediatr Anaesth. 2004;14:194-5.
Details
| Primary Language | Turkish |
|---|---|
| Journal Section | Case Report |
| Authors | |
| Publication Date | December 1, 2016 |
| Published in Issue | Year 2016Issue: 4 |