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10-2017 A Case of Larsen Syndrome Stanlies D'Souza Baystate Health, [email protected]

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Recommended Citation D'Souza S, D'Souza N. A Case of Larsen Syndrome. ASA Annual Meeting, Oct 2017.

This Presentations, Research is brought to you for free and open access by Scholarly Commons @ Baystate Health. It has been accepted for inclusion in All Scholarly Works by an authorized administrator of Scholarly Commons @ Baystate Health. A Case of Larsen Syndrome

Stanlies D’Souza MD, Nishant D’Souza

Department of Anesthesiology at Baystate Medical Center/University of Massachusetts Medical School

Characteristic Features of Larsen Syndrome1 Causes for airway Difficulties in CASE DESCRIPTION CONCLUSION 4 • th Larsen Syndrome A 37-year-old wheel chair bound female with a Short stature (Height below the 10 percentile in 70% of cases) Careful attention to minimize the neck BMI 41, with Larsen syndrome presented for • Congenital anterior dislocation of large joints ((80% , 80% , Anterior Larynx movement is essential during difficult upper endoscopy and colonoscopy. Features and 65% elbow), airway management due to the consistent with syndrome in this patient were • Cervical (50%) Laryngotracheomalacia presence of cervical kyphosis and short stature, multiple skeletal abnormalities, • Thoracolumbar (84%), subluxation in patients with Larsen Cervical kyphosis syndrome. cervical vertebral anomalies and high arched • Conductive deafness (21%) palate with an anticipated difficult airway with • Cleft palate (15%) Cervical vertebral hypoplasia direct laryngoscopy. Airway should be secured with flexible • Laryngotracheomalacia Cervical subluxation fiberoptic/ rigid laryngoscopy or direct Other associated comorbidities were • Cardiac anomalies similar to Marfan’s syndrome laryngoscopy with manual in line obstructive sleep apnea and non –insulin Cervical cord compression immobilization of the cervical spine. dependent diabetes mellitus. Associated laryngomalacia may lead to The procedure was performed uneventfully with perioperative respiratory compromise. monitored anesthesia care with intermittent Cardiovascular Abnormalities Presence of cardiac anaomalies may bolus of propofol. Anesthetic Implications of Facial Features associated with further complicate the perioperative Larsen Syndrome4 care. of Larsen Syndrome3 Larsen Syndrome 2 ►Airway difficulty Elongation of  Frontal Bossing CASE DISCUSSION Bicuspid aortic valve ►Perioperative respiratory compromise  Mid place hypoplasia • Larsen Syndrome is a rare inherited genetic Subaortic stenosis ►Cervical instability and cord compression  Flattening of nasal disorder of collagen formation with skeletal with mitral ►CHD abnormalities involving large joints and axial REFERENCES bridge regurgitation skeleton. 1. Larsen syndrome, National organization for rare  disorders (NORD) • Most common mode of inheritance is autosomal 2. Larsen syndrome: Genetic Home reference. US dominant involving B Gene (FLMB) and Ventricular septal defect National Library of Medicine less commonly inherited as autosomal recessive Patent ductus arteroiosis 3. Kiel EA, Frias JL, Victorica BE. Cardiovascular mode. manifestations in the Larsen syndrome. Pediatrics Tricuspid valve prolapse 1983; 71:942-6. • Incidence is 1: 100, 000 in general population. 4. Malik P, Choudhry DK. Larsen syndrome and its

Aortic dissection and anesthetic implications. Paediatr Anaesth • Males and females are affected equally. 2002.;12:632-6.