Zusammenfassung
Die Trisomie 21, auch als Down-Syndrom bezeichnet, gehört mit einer Prävalenz von etwa l:800 Schwangerschaften zu den häufigsten angeborenen chromosomalen Veränderungen. Die meisten der resultierenden chronischen Behinderungen gelten als nichtheilbar und betreffen Menschen verschiedenen Alters, Bildungsstands und verschiedener Volkszugehörigkeit. Die Lebenserwartung dieser Patienten steigt – wie auch bei dem Rest der Bevölkerung – mit zunehmendem medizinischem Fortschritt. Aufgabe der Gesundheitsdienstleister ist es, Familien und Betroffenen zu helfen, die Folgen dieser Behinderungen zu mildern sowie mit den Einschränkungen möglichst gut zurechtzukommen. Symptom Variabilität und Expressivität des Down-Syndroms erscheinen äußerst vielfältig, basierend auf dem typischen Phänotyp mit der häufigen Trias aus mentaler Retardierung, Handanomalien und angeborenen Herzfehlern. Auch andere Veränderungen sind mitunter wichtig für die anästhesiologische Versorgung, wie eine atlantoaxiale Instabilität (AAI), Trachealstenose, Neigung zu respiratorischen Infektionen, Hypothyreoidismus, Mikrogenie und relative Makroglossie. Weiterhin haben Patienten bzw. Familien mit Kindern mit Down-Syndrom spezifische Erwartungen an die medizinische und anästhesiologische Versorgung.
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Literature
Yang Q, Rasmussen SA, Friedman JM (2002) Mortality associated with Down’s Syndrome in the USA from 1983 to 1997: a population-based study. Lancet 359:1019–1025
DownJL (1866) Observationsonan ethnic Classification of idiots. Clin Lect Rep Lond Hosp 3:259–262
Meitzner MC, Skurnowicz JA (2005) Anesthetic considerations for patients with Down Syndrome. AANA J 73:103–107
Weijerman ME, Winter JP de (2010) Clinical practice.Thecareofchildren with Down Syndrome. Eur J Pediatr 169:1445–1452
Wilken E (2003) Pränatale Diagnostik und Häufigkeit des Down-Syndroms. Leben Down-Syndr43:6ff.
Schiff JH, Russ N, Ihringer K et al (2012) Pediatric patients with disabilities assessment of satisfaction with anesthesia. Paediatr Anaesth 22:1117–1123
Speicher M, AntonarakisS, Motulsky A (2010) Vogel and Motulsky’s human genetics. Springer, Berlin Heidelberg New York Tokio, S 101 ff., 681 ff.
Rex AP, Preus M (1982) A diagnostic index for Down Syndrome. J Pediatr 100:903–906
Borland LM, Colligan J, Brandom BW (2004) Frequency of anesthesia-related complications in children with Down Syndrome under general anesthesia for noncardiac procedures. Paediatr Anaesth 14:733–738
Pueschei SM, FindleyTW, Furia J et al (1987) Atlantoaxial instability in Down Syndrome: roentgenographic, neurologic, and somatosensory evoked potential studies. J Pediatr 110:515–521
Cohen W (1998) Atlantoaxial instability. What’s next? Arch Pediatr Adolesc Med 152:119–122
Kobel M, Creighton RE, Steward DJ (1982) Anaesthetic considerations in Down’s Syndrome: experience with 100 patients and a review ofthe literature. Can Anaesth SocJ 29:593–599
Morton RE, Khan MA, Murray-Leslie C et al (1995) Atlantoaxial instability in Down’s Syndrome: a five year follow up study. Arch Dis Child 72:115–118
Powell JF, WoodcockT, Luscombe FE (1990) Atlanto-axial Subluxation in Down’s Syndrome. Anaesthesia 45:1049–1051
Smith DS (2001) Health care management of adults with Down Syndrome. Am Fam Physician 64:1031103–8
Asano N, Ishiguro S, Sudo A (2012) Head positioning for reduction and stabilization of the cervical spine during anesthetic induction in a patient with subaxial Subluxation. J Neurosurg Anesthesiol 24:164–165
Maruyama K, Hirabayashi Y, Fujita A et al (2007) Cervical spine movement during laryngoscopy using the Airway Scope compared with the Macintosh laryngoscope. Anaesthesia 62:1050–1055
Prasarn ML, Conrad B, Rubery PT et al (2012) Comparison of4 airway devices on cervical spine alignment in a cadaver model with global ligamentous instability at C5-C6. Spine 37:476–481
Cooley WC, Graham JM Jr (1991) Down Syndrome an update and reviewforthe primary pediatrician. Clin Pediatr 30:233–253
Santamaria LB, Di Paola C, Mafrica F, Fodale V (2007) Preanesthetic evaluation and assessment ofchildren with Down’s Syndrome. Sei World J 7:242–251
Shapiro NL, Huang RY, Sangwan S et al (2000) Tracheal stenosis and congenital heart disease in patients with Down Syndrome: diagnostic approach and surgical options. Int J Pediatr Otorhinolaryngol 54:137–142
Rubens Figueroa J de, Pozzo Magana B del, Pablos Hach JL et al (2003) Heart malformations in children with Down Syndrome. Rev Esp Cardiol 56:894–899
FinesilverC (2002) A newage for childhood diseases. Down Syndrome. RN 65:43–48
<http://www.awmf.org/uploads/tx_>szleitlinien/019-012_S1_Prophyla-xe_der_infektioesen_Endokarditis_11-2007_11-2012.pdf
Bai W, Voepel-Lewis T, Malviya S (2010) Hemodynamic changes in children with Down Syndrome during and following inhalation induction of anesthesia with sevoflurane. J Clin Anesth 22:592–597
Kraemer FW, Stricker PA, Gurnaney HG et al (2010) Bradycardia during induction of anesthesia with sevoflurane in children with Down Syndrome. Anesth Anaig 111:1259–1263
Velik-Salchner C, Margreiter J, Wenzel V et al (2006) Anaesthesia for cardiac catheterization in children. Anaesthesist 55:1291–1298
Shott SR (2000) Down Syndrome: analysis of airway size and a guide for appropriate intubation. Laryngoscope 110:585–592
Bhattarai B, Kulkarni AH, Rao ST et al (2008) Anesthetic consideration in downs Syndrome a review. Nepal Med Coli J 10:199–203
Jong AL de, Sulek M, Nihill M et al (1997) Tenuous airway in children with trisomy 21. Laryngoscope 107:345–350
Guimaraes CV, Donnelly LF, Shott SR et al (2008) Relative rather than absolute macroglossia in patients with Down Syndrome: implications for treatment of obstructive sleep apnea. Pediatr Radiol 38:1062–1067
Asai T, Hirose T, Shingu K (2000) Failed tracheal intubation using a laryngoscope and intubating laryngeal mask. Can J Anaesth 47:325–328
McLean L, MacCormick J, Robb I et al (2003) Cilia ultrastructure in children with Down Syndrome. J Otolaryngol 32:379–383
Piatti G, Allegra L, Ambrosetti U et al (2001) Nasal eiliary funetion and ultrastructure in Down Syndrome. Laryngoscope 111:1227–1230
Cohen W (1996) Health care guidelines for individuals with Down Syndrome. Down Syndr Q 1:1 –11
Donnelly LF, Shott SR, LaRose CR et al (2004) Causes of persistent obstructive sleep apnea despite previous tonsillectomy and adenoidectomy in children with Down Syndrome as depicted on static and dynamic eine MRI. AJR Am J Roentgenol 183:17518–1
Marcus CL, KeensTG, Bautista DB et al (1991) Obstructive sleep apnea in children with Down Syndrome. Pediatrics 88:132–139
Southall DP, Stebbens VA, Mirza R et al (1987) Upper airway obstruetion with hypoxaemia and sleep disruption in Down Syndrome. Dev Med Child Neural 29:734–742
Uong EC, McDonough JM, Tayag-Kier CE et al (2001) Magnetic resonance imaging of the upper airway in children with Down Syndrome. Am J RespirCrit Care Med 163:731–736
Brouilette R, Hanson D, David R et al (1984) A diagnostic approach to suspected obstructive sleep apnea in children. J Pediatr 105:10–14
Li AM, Cheung A, Chan D et al (2006) Validation of a questionnaire Instrument for prediction of obstructive sleep apnea in Hong Kong Chinese children. Pediatr Pulmonol 41:1153116–0
McNamara F, Sullivan CE (2000) Obstructive sleep apnea in infants: relation to family history of sudden infant death Syndrome, apparent life-threatening events, and obstructive sleep apnea. J Pediatr 136:31832–3
Messner AH (2003) Treating pediatric patients with obstructive sleep disorders: an update. Otolaryngol Clin North Am 36:519–530
Whiteford L, Fleming P, Henderson AJ (2004) Who should have a sleep study for sleep related breathing disorders? Arch Dis Child 89:851–855
Arias MA, Garcia-Rio F, Alonso-Fernandez A et al (2006) Pulmonary hypertension in obstructive sleep apnoea: effects of continuous positive airway pressure: a randomized, controlled cross-over study. Eur Heart J 27:1106–1113
Marcus CL, Ward SL, Mallory GB et al (1995) Use of nasal continuous positive airway pressure as treatment of childhood obstructive sleep apnea. J Pediatr 127:88–94
Schwengel DA, Sterni LMJunkel DE et al (2009) Perioperative management of children with obstructive sleep apnea. Anesth Anaig 109:607–5
Rachel Homer J, ElwoodT, Peterson D, Rampersad S (2007) Riskfactors for adverse events in children with colds emerging from anesthesia: a logistic regression. Paediatr Anaesth 17:154–161
Nakazawa K, Ikeda D, Ishikawa S, Makita K (2003) A case of difficult airway due to lingual tonsillar hypertrophy in a patient with Down’s Syndrome. Anesth Anaig 97:704–705
Brown JC (2002) The management of croup. BrMed Bull 61:189–202
Motoyama EK (1992) Anesthesia and the upper airway in infants and children. Int Anesthesiol Clin 30:17–19
Abbag Fl (2006) Congenital heart diseases and other major anomalies in patients with Down Syndrome. Saudi Med J 27:219–222
Freeman SB, Torfs CP, Romitti PA et al (2009) Congenital gastrointestinal defects in Down Syndrome: a report from the Atlanta and National Down Syndrome Projects. Clin Genet 75:180–184
Rohen JW, Lütjen-Drecoll E (2004) Funktionelle Embroyologie die Entwicklung der Funktionssysteme des menschlichen Organismus, 2. Überarb. und erw. Aufl. Schattauer, Stuttgart, S 91 ff.
Arya R, Kabra M, Gulati S (2011) Epilepsy in children with Down Syndrome. Epileptic Disord 13:1–7
Lima FA, Moreira-Filho CA, Ramos PL et al (2011) Decreased AIRE expression and global thymic hypofunetion in Down Syndrome. J Immunol 187:3422–3430
Popova G, Paterson WF, Brown A et al (2008) Hashimoto’s thyroiditis in Down’s Syndrome: dinical presentation and evolution. Horm Res 70:278–284
Murdoch JC, Ratdiffe WA, McLarty DG et al (1977) Thyroid funetion in adults with Down’s Syndrome. J Clin Endocrinol Metab 44:453–458
Malaga S, Pardo R, Malaga I et al (2005) Renal involvement in Down Syndrome. Pediatr Nephral 20:61461–7
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Ihringer, K., Russ, N., Walther, A., Schiff, JH. (2013). Anästhesiologische Besonderheiten der Trisomie 21 (Down-Syndrom). In: Weiterbildung Anästhesiologie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-40715-4_17
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