laryngomalacia

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visited=11%2F23%2F2009; visited=11%2F23%2F2009; visited=11%2F23%2F2009><table cellpadding=0 cellspacing=0 border=0><tr> <td valign="top" colspan=2 height=110 BGCOLOR="#666699" TEXT="#080000" bgproperties="fixed" background="04a00840erlcjm.gif"> <div> <FONT SIZE="5" COLOR="#FFFFFF" FACE="Arial" ><B>pediatryPART I</B></FONT><B>     |     </B><A HREF="index.htm" TARGET="_top" TITLE="pediatryPART I"><U><B>home</B></U></A></div> <div> <A HREF="id31.htm" TARGET="_top" TITLE="PEDIATRIC PEARLS FOR THE USMLE"><U>PEDIATRIC PEARLS FOR THE USMLE</U></A>   |   <A HREF="id48.htm" TARGET="_top" TITLE="pearls pediatry 2"><U>pearls pediatry 2</U></A>   |   <A HREF="id18.htm" TARGET="_top" TITLE="general"><U>general</U></A>   |   <A HREF="id34.htm" TARGET="_top" TITLE="appendiceal colic"><U>appendiceal colic</U></A>   |   <A HREF="id35.htm" TARGET="_top" TITLE="ATOPIC DERMATITIS"><U>ATOPIC DERMATITIS</U></A>   |   <A HREF="id19.htm" TARGET="_top" TITLE="Stuttering"><U>Stuttering</U></A>   |   <A 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TARGET="_top" TITLE="infectious disease"><U><B>infectious disease</B></U></A></div> <div> <A HREF="id32.htm" TARGET="_top" TITLE="HEMORRHAGIC DISEASE IN A NEW BORN"><U><B>HEMORRHAGIC DISEASE IN A NEW BORN</B></U></A></div> <div> <A HREF="id33.htm" TARGET="_top" TITLE="OPTIC NERVE GLIOMA"><U><B>OPTIC NERVE GLIOMA</B></U></A></div> <div> <A HREF="id36.htm" TARGET="_top" TITLE="New Born Assessment "><U><B>New Born Assessment </B></U></A></div> <div> <B>laryngomalacia</B></div> <div> <A HREF="id39.htm" TARGET="_top" TITLE="CRANIOSYNOSTOSIS "><U><B>CRANIOSYNOSTOSIS </B></U></A></div> <div> <A HREF="id40.htm" TARGET="_top" TITLE="DANDYWALKER MALFORMATION "><U><B>DANDY-WALKER MALFORMATION </B></U></A></div> <div> <A HREF="id41.htm" TARGET="_top" TITLE="DEJERINESOTTAS DISEASE "><U><B>DEJERINE-SOTTAS DISEASE </B></U></A></div> <div> <A HREF="id42.htm" TARGET="_top" TITLE="DOWN SYNDROME "><U><B>DOWN SYNDROME </B></U></A></div> <div> <A HREF="id43.htm" TARGET="_top" TITLE="Respiratory Syncytial Virus (RSV)"><U><B>Respiratory Syncytial Virus (RSV)</B></U></A></div> <div> <A HREF="id44.htm" TARGET="_top" TITLE="FABRY DISEASE "><U><B>FABRY DISEASE </B></U></A></div> <div> <A HREF="id45.htm" TARGET="_top" TITLE="PRADERWILLI SYNDROME "><U><B>PRADER-WILLI SYNDROME </B></U></A></div> <div> <A HREF="id37.htm" TARGET="_top" TITLE="new born fun"><U><B>new born fun</B></U></A></div> <div> <A HREF="id46.htm" TARGET="_top" TITLE="RETT SYNDROME "><U><B>RETT SYNDROME </B></U></A></div> <div> <A HREF="id47.htm" TARGET="_top" TITLE="Alexander Disease"><U><B>Alexander Disease</B></U></A></div> <div> <A HREF="id49.htm" TARGET="_top" TITLE="leukemia"><U><B>leukemia</B></U></A></div> <div> <A HREF="id17.htm" TARGET="_top" TITLE="Contact Me"><U><B>Contact Me</B></U></A></div> </td> <td valign="top" height=370 BGCOLOR="#FFFFFF" TEXT="#080000"> <div> laryngomalacia</div> <DIV ALIGN="LEFT"></DIV> <div> LARYNGOMALACIA </div> <div> DEFINITION:</div> <div> A congenital disorder of the larynx characterized by inspiratory stridor and airway obstruction. </div> <div> EPIDEMIOLOGY:</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">incidence: most common congenital laryngeal abnormality </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">age of onset: </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">2 weeks to 2 months (stridor) </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">risk factors: </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">M > F (2:1) </div> <div> PATHOGENESIS:</div> <div> 1. Background</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">due to a congenital weakness of the aryepiglottic folds and epiglottis which are sucked into the airway during inspiration </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">embryologic origin of defect unknown </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">a self-resolving disorder as cartilagenous development will eventually support the affected structures </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">when a similar process involves the: </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">trachea - tracheomalacia </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">bronchus - congenital lobar emphysema </div> <div> DIFFERENTIAL DIAGNOSIS:</div> <div> 1. Larynx</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">malformation of the laryngeal cartilage (vocal cords) </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">juvenile larygneal papillomatosis </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">severe chondromalacia of larynx and trachea </div> <div> 2. Luminal Obstruction</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">laryngeal web </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">laryngeal hemangioma </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">cysts - mucous retention cysts, brachial cleft cysts </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">thyroglossal duct remnants </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">lymphangioma </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">congenital goiters </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">vascular anomalies </div> <div> 3. Others</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">Pierre Robin Syndrome </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">hypoplasia of the mandible </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">macroglossia </div> <div> CLINICAL FEATURES:</div> <div> 1. Congenital Stridor</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">begins within the first few days of life or up to 2 months (but not at birth) </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">louder on inspiration </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">worse with crying, upper respiratory tract infections (URTI), supine position with neck flexed </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">better in prone position with neck hyperextended </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">may be accompanied by: </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">significant inspiratory retractions </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">normal voice -> hoarseness, aphonia, or laryngeal "crow" </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">may worsen over the first few months of life before improving </div> <div> 2. Complications</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">respiratory distress with dyspnea </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">thoracic deformities (if retractions severe) </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">failure to thrive (if difficulty nursing) </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">tracheostomy (0.3%) </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">long-term inspiratory stridor with URTI, exertion, and/or crying throughout childhood </div> <div> INVESTIGATIONS:</div> <div> 1. Direct Laryngoscopy</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">diagnostic </div> <div> MANAGEMENT:</div> <div> 1. Supportive</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">respiratory support with severe or life-threatening upper airway obstruction, i.e., intubation, tracheostomy </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">feeding difficulties </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">slow and careful feedings </div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">may use dropper or gavage feedings </div> <div> 2. Surgery</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">surgical repair in severe cases </div> <div> 3. Prognosis</div> <div> <IMG BORDER="0" SRC="Symb075c00b700f000000000.gif" HEIGHT="16" WIDTH="24" ALIGN="bottom" HSPACE="0" VSPACE="0">natural history is worsening in the first few months of life with improvement at about 12 months with resolution by 18 months </div> <bR> </td> </tr></table></body>