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Choanal Atresia
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Surgery - Otolaryngology and Facial Plastic Surgery

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Last Updated: February 23, 2007

Synonyms and related keywords: complete nasal obstruction in a newborn, congenital c choanae, unilateral atresia, bilateral choanal atresia, posterior nares, definitive choanae, re atresia of choanae, choanal atresia

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AUTHOR INFORMATION

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Author: Ted L Tewfik, MD, FRCSC , Professor, Department of Otolaryngology, McGill Univ Canada; Director, Department of Otolaryngology, Montreal Children's Hospital, Canada Coauthor(s): Abdulrahman A Hagr, MBBS , Staff Physician, Department of Otolaryngolog Canada Ted L Tewfik, MD, FRCSC, is a member of the following medical societies: American Acad Head and Neck Surgery, American Society of Pediatric Otolaryngology, Canadian Medical Society of Otolaryngology-Head & Neck Surgery, Quebec Medical Association, and Royal and Surgeons of Canada Editor(s): Russell A Faust, MD, PhD , Consulting Staff, Department of Otolaryngology, Co Hospital; Francisco Talavera, PharmD, PhD , Senior Pharmacy Editor, eMedicine; Grego Assistant Professor, Department of Otolaryngology-Head and Neck Surgery, University of C Medicine; Christopher L Slack, MD , Otolaryngology-Facial Plastic Surgery, Private Pract ENT; Medical Director, Treasure Coast Sleep Disorders; and Arlen D Meyers, MD, MBA , of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine

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INTRODUCTION

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Complete nasal obstruction in a newborn may cause death from asphyxia. During attempted inspiration, the tongu palate, and obstruction of the oral airway results. Vigorous respiratory efforts produce marked chest retraction. Inc death may occur if appropriate treatments are not available; however, if the infant cries and takes a breath throug airway obstruction is momentarily relieved. Then the crying stops, the mouth closes, and the cycle of obstruction i History of the Procedure: In 1755, Roederer first described congenital choanal atresia; therefore, this condition

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for more than 200 years. In 1854, Emmert reported the first successful surgical procedure for congenital choanal boy using a curved trocar transnasally. Over the years, the necessity of serial dilatations to maintain patency of th clearly recognized. Frequency: The average rate of choanal atresia is 0.82 cases per 10,000 individuals. Unilateral atresia occurs m right side. The ratio of unilateral to bilateral cases is 2:1. A slightly increased risk exists in twins. Maternal age or p increase the frequency of occurrence. Chromosomal anomalies are found in 6% of infants with choanal atresia. F have monogenic syndromes or conditions. Race: Choanal atresia occurs with equal frequency in people of all races. Sex: More studies report significantly more females than males affected. Etiology: The nasal cavities extend posteriorly during development under the influence of the posteriorly directed processes. Thinning of the membrane occurs, which separates the nasal cavities from the oral cavity. By the 38th the 2-layer membrane consisting of nasal and oral epithelia ruptures and forms the choanae (posterior nares). Fa results in choanal atresia. Although these choanae are not in the same location as the definitive choanae, which a more posteriorly, the unexpectedly anterior extent of choanal atresia is explained. Pathophysiology: A number of theories have been proposed to explain the occurrence of choanal atresia, and th summarized as follows: Persistence of the buccopharyngeal membrane Failure of the bucconasal membrane of Hochstetter to rupture Medial outgrowth of vertical and horizontal processes of the palatine bone Abnormal mesodermal adhesions forming in the choanal area Misdirection of mesodermal flow due to local factors Clinical: The clinical evaluation includes a complete physical examination to assess for other congenital anomalie tube could be used to determine the patency of the choana, but a complete nasal and nasopharyngeal examinatio performed using a flexible fiberoptic endoscope to assess the deformity. A simple method using the automatic tym newborns for congenital choanal atresia was recently published. The sensitivity and specificity of the technique in nostril was reported as 100 per cent. However, a high level of suspicion is required to diagnose bilateral choanal a severe airway obstruction and cyclical cyanosis are the classic signs of neonatal bilateral atresia. When crying all distress in an obligate nasal breather, the neonatologist should be alerted to the probability of bilateral choanal at atresia may not be detected for years, and patients may present with unilateral rhinorrhea or congestion. Many patients have an associated narrowed nasopharynx, widened vomer, medialized lateral nasal wall, and/or a Associated malformations occur in 47% of infants without chromosome anomalies. Nonrandom association of ma demonstrated using the CHARGE association, which appears to be overused in clinical practice. The components association are as follows: Coloboma of the iris, choroid, and/or microphthalmia Heart defect such as atrial septal defect (ASD) and/or conotruncal lesion Atresia of choanae

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Retarded growth and development Genitourinary abnormalities such as cryptorchidism, microphallus, and/or hydronephrosis Ear defects with associated deafness (The external, middle, and/or inner ear may be involved. Only a sma with choanal atresia and related components probably represent this entity.) The percentages of the different anomalies in CHARGE association are as follows: Coloboma - 80% Heart defect - 58% Atresia of choanae - 100% Mental retardation - 94% Growth deficiency - 87% Genital hypoplasia in males - 75% Ear anomalies - 88% Differential diagnosis Deviated nasal septum Dislocated nasal septum Septal hematoma Mucosal swelling Turbinate hypertrophy Encephalocele Nasal dermoid Hamartoma Chordoma Teratoma WORKUP Section 3 of 6 Prev Top Next

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Imaging Studies: Rhinography is a procedure that involves the administration of radiopaque dye into the nasal cavity as illus CT scanning is the radiographic procedure of choice in the evaluation of choanal atresia. For good results, performed to clear excess mucus, and a topical decongestant is applied (see Image 2 ). The purpose of CT as follows: Confirm the diagnosis of choanal atresia (unilateral or bilateral). Evaluate choanal atresia (vomer bone width and choanal airspace distance).

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Exclude other possible nasal sites of obstruction. Determine the degree of bony, membranous, or mixed atresia. Delineate abnormalities in the nasal cavity and nasopharynx. Diagnostic Procedures: Failure to pass an 8F catheter through the nasal cavity more than 5.5 cm from the alar rim The lack of movement of a thin wisp of cotton under the nostrils while the mouth is closed The absence of fog on a mirror when it is placed under the nostrils Acoustic rhinometry Listening for breath sounds with either a stethoscope or a Toynbee auscultation tube Gently blowing air into each nasal cavity with a Politzer bag Administering into the nose a colored solution that is visible in the pharynx

TREATMENT

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Surgical therapy: Treatment can be divided into emergent and elective definitive categories. Bilateral choanal at an emergency that is best initially treated by inserting an oral airway to break the seal formed by the tongue again oral airway can be well tolerated for several weeks. The method of repair is controversial, with no technique havin acceptance. Bilateral choanal atresia in the newborn requires prompt diagnosis and airway stabilization. An oral a nipple, and intubation are viable options. The ideal procedure for choanal atresia restores the normal nasal passa to growing structures important in facial development, is technically safe, requires short operative time, and provid hospitalization and convalescence. Procedures Transnasal puncture, with or without a microscope, became unpopular because of the high rate of failure that the This was attributed to the difficulty in visualizing the choanal area that required special surgical attention, such as bridge and bony narrowing of the lateral walls. The transnasal approach becomes more difficult in the presence o turbinate hypertrophy, nasal discharge, and elongation of the depth from the nasal vestibule to the posterior choa The transseptal technique consists of making a window in the septum anterior to the atretic plate. Transpalatal repair is a technique that provides excellent exposure and has a high success rate but requires more increased blood loss, possible occurrence of palatal fistula, palatal dysfunction, and maxillofacial growth disturban of this procedure (see Image 3 ). The endoscopic technique (nasal or retropalatal), with or without powered instrumentation, offers excellent visuali in removing the bony choanae. Microdebriders will continue to advance the field of endoscopic surgery, providing fields and causing less tissue trauma for experienced surgeons. However, the severity of complications, including rapidly aspirating orbital and cerebral contents when laminae are violated, must be appreciated and respected.

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Carbon dioxide and potassium titanyl phosphate (KTP) lasers are easy and quick and create minimal discomfort t time of hospitalization is short, and the operation can be repeated if a good result is not initially achieved. Most im not usually needed. Postoperative details: Infants with documented gastroesophageal reflux disease (GERD) require prolonged sten for choanal restenosis and removal of granulation tissue. Stenting is usually performed using an endotracheal tub The advantages of Foley catheter stenting for choanal atresia are as follows: Well tolerated by the patient Simple to introduce, fix, and remove Minimizes septal or columellar necrosis Minimizes nasal cavity and paranasal sinus infections Adjustable with inflation or deflation of the balloon that controls the pressure on the choanal walls Easy to fix in cases of unilateral atresia The use of stents in the treatment of patients with choanal atresia is a controversial subject. Some surgeons belie useful in stabilizing the nasal airway in the postoperative period to prevent the development of stenosis by mainta However, others believe that stents may act as a nidus for infection and may induce a foreign body reaction. This choanal restenosis, much as an endotracheal tube may cause subglottic stenosis. Therefore, the use of stents fol choanal atresia requires the use of prophylactic antibiotic and antireflux medications.Cedin et al (2006) analyzed t of a new stentless surgical technique for choanal atresia. They reported that, using neither stents or nasal packing allowed fast recovery in a one-step surgery. Follow-up care: Following surgical repair of choanal atresia, patients may require operative debridement or perio Periodic dilations can sometimes be performed as an outpatient procedure with local decongestant and topical an urethral sounds. MULTIMEDIA Section 5 of 6 Prev Top Next

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Caption: Picture 1. Choanal atresia. Rhinogram demonstrating blockage of radiopaque dye at the posterior choanae. From T.L. Tewfik and V.M. Der Kaloustian, with permission.

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Picture Type: X-RAY Caption: Picture 2. Choanal atresia. CT scan showing membranous and bony choanal atresia. From T.L. Tewfik and V.M. Der Kaloustian, with permission.

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Picture Type: CT Caption: Picture 3. Choanal atresia. Diagram illustrating the transpalatal correction of choanal atresia.

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BIBLIOGRAPHY

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Al-Ammar AY: Presentation of choanal atresia in Saudi children. Saudi Med J 2006 Nov; 27(11): 1680-2 [M Brown OE, Burns DK, Smith TH, Rutledge JC: Bilateral posterior choanal atresia: a morphologic and histo computed tomographic correlation. Int J Pediatr Otorhinolaryngol 1987 Aug; 13(2): 125-42 [Medline] . Brown OE, Pownell P, Manning SC: Choanal atresia: a new anatomic classification and clinical manageme Laryngoscope 1996 Jan; 106(1 Pt 1): 97-101 [Medline] . Cedin AC, Fujita R, Cruz OL: Endoscopic transeptal surgery for choanal atresia with a stentless folded-ove Otolaryngol Head Neck Surg 2006 Nov; 135(5): 693-8 [Medline] . Cozzi F, Steiner M, Rosati D, et al: Clinical manifestations of choanal atresia in infancy. J Pediatr Surg 198 [Medline] . Crockett DM, Scamman FL, McCabe BF, et al: Venturi jet ventilation for microlaryngoscopy: technique, co Laryngoscope 1987 Nov; 97(11): 1326-30 [Medline] . Deutsch E, Kaufman M, Eilon A: Transnasal endoscopic management of choanal atresia. Int J Pediatr Oto May 4; 40(1): 19-26 [Medline] . Effat KG: Use of the automatic tympanometer as a screening tool for congenital choanal atresia. .J Laryng 125-8. Friedman NR, Mitchell RB, Bailey CM, et al: Management and outcome of choanal atresia correction. Int J Otorhinolaryngol 2000 Jan 30; 52(1): 45-51 [Medline] . Hackman TG, Ferguson BJ: Powered instrumentation and tissue effects in the nose and paranasal sinuses Otolaryngol Head Neck Surg 2005 Feb; 13(1): 22-6 [Medline] . Josephson GD, Vickery CL, Giles WC, Gross CW: Transnasal endoscopic repair of congenital choanal atr results. Arch Otolaryngol Head Neck Surg 1998 May; 124(5): 537-40 [Medline] . Lazar RH, Younis RT: Transnasal repair of choanal atresia using telescopes. Arch Otolaryngol Head Neck

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(5): 517-20 [Medline] . Panwar SS, Martin FW: Trans-nasal endoscopic holmium: YAG laser correction of choanal atresia. J Laryn 110(5): 429-31 [Medline] . Pasquini E, Sciarretta V, Saggese D, et al: Endoscopic treatment of congenital choanal atresia. Int J Pedia 2003 Mar; 67(3): 271-6 [Medline] . Schoem SR: Transnasal endoscopic repair of choanal atresia: why stent?. Otolaryngol Head Neck Surg 20 [Medline] . Schoem SR.: Transnasal endoscopic repair of choanal atresia: why stent?. Otolaryngol Head Neck Surg. 2 Vickery CL, Gross CW: Advanced drill technology in treatment of congenital choanal atresia. Otolaryngol C Jun; 30(3): 457-65 [Medline] . Wiatrak BJ: Unilateral choanal atresia: initial presentation and endoscopic repair. Int J Pediatr Otorhinolary (1-2): 27-35 [Medline] . Yaniv E, Hadar T, Shvero J: Endoscopic transnasal repair of choanal atresia. Int J Pediatr Otorhinolaryngo 457-62 [Medline] . Zeitouni AG, Shapiro RS: Congenital anomalies of the nose and anterior skull base. In: Tewfik TL, Der Kal Congenital Anomalies of the Ear, Nose, and Throat. New York, NY: Oxford University Press; 1997:189-200 NOTE: Medicine is a constantly changing science and not all therapies are clearly established. New research changes d therapies daily. The authors, editors, and publisher of this journal have used their best efforts to provide informat and accurate and is generally accepted within medical standards at the time of publication. However, as medical changing and human error is always possible, the authors, editors, and publisher or any other party involved with this article do not warrant the information in this article is accurate or complete, nor are they responsible for omis article or for the results of using this information. The reader should confirm the information in this article from oth use. In particular, all drug doses, indications, and contraindications should be confirmed in the package insert. FU

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Medicine is a constantly changing science and not all therapies are clearly established. New research changes drug and treatmen authors, editors, and publisher of this journal have used their best efforts to provide information that is up-to-date and accurate and accepted within medical standards at the time of publication. However, as medical science is constantly changing and human erro possible, the authors, editors, and publisher or any other party involved with the publication of this article do not warrant the inform is accurate or complete, nor are they responsible for omissions or errors in the article or for the results of using this information. Th confirm the information in this article from other sources prior to use. In particular, all drug doses, indications, and contraindications confirmed in the package insert. Full Disclaimer.

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