Presentation is loading. Please wait.

Presentation is loading. Please wait.

Choanal Atresia Saad A. Alsaleh. Questions  What is Choanal Atresia (CA)?  Why does it happen?  Are there risk factors for developing CA?  What is.

Similar presentations


Presentation on theme: "Choanal Atresia Saad A. Alsaleh. Questions  What is Choanal Atresia (CA)?  Why does it happen?  Are there risk factors for developing CA?  What is."— Presentation transcript:

1 Choanal Atresia Saad A. Alsaleh

2 Questions  What is Choanal Atresia (CA)?  Why does it happen?  Are there risk factors for developing CA?  What is CHARGE syndrome?  How do patients with CA present?  How do you evaluate a patient with CA?  What are the treatment options?

3 What is CA?

4  Total obstruction of the posterior nasal choana (the opening between the nose and the nasopharynx).  first described by Roederer In  In 1854, Emmert reported the first successful surgical procedure for congenital choanal atresia in a 7-year-old boy using a curved trocar transnasally.

5 What is CA?  Incidence of 1 in 5000 – 8000 live births.  F : M = 2 : 1  Unilateral : Bilateral = 2 : 1 ( Rt. Side )  J.S. Fraser in 1910 reported that it was 90% bony and 10% membranous atresia. NO pure membranous atresia present.  Brown et al in 1996 reported that it was 29% pure bone, 71% mixed membranous and bone, NO pure membranous atresia present.

6 Why does it happen?

7 Etiology  Failure of the bucconasal membrane of Hochstetter to rupture ( the most widely accepted ).  Misdirection of mesodermal flow due to local factors ( Hengerer, 1982)  Medial outgrowth of vertical and horizontal processes of the palatine bone.

8 Are there risk factors for developing CA?

9 Choanal Atresia Associated With Prenatal Methimazole Exposure: Three New Patients P. Barbero, C. Ricagni, G. Mercado, R. Bronberg, and M. Torrado American Journal of Medical Genetics 129A:83–86 (2004)

10 What is CHARGE syndrome?

11 What is CHARGE Syndrome?  Choanal atresia is associated with other congenital abnormalities 50% of the time. (bilateral > unilateral)  Mutation in CHD7 gene in chromosome 8.  In 1979, CHARGE syndrome or Hall-Hittner syndrome was originally delineated by Bryan Hall in 17 children with multiple congenital anomalies.  In 1981, R Pagon coined the term ‘CHARGE’, an acronym summarizing six cardinal clinical features. Sanlaville D and Verloes A. CHARGE syndrome: an update. European Journal of Human Genetics (2007) 15, 389–399.

12 What is CHARGE Syndrome? C  Ocular C oloboma  H  H eart defects  A  A tresia of choanae  R  R etardation of growth  G  G enito-urinary anomalies  E  E ar anomalies

13 What is CHARGE Syndrome? C  Ocular Coloboma  Chorioretineal coloboma, with (40%) or without microphthalmia, is present in 75–90%.  Other anomalies include hypoplasia of optic nerve, anophthalmia, nystagmus, squint and refractive errors  H  Heart defects  A  Atresia of choanae  R  Retardation of growth  G  Genito-urinary anomalies  E  Ear anomalies

14 What is CHARGE Syndrome? C  Ocular Coloboma  H  Heart defects  Congenital heart defects (CHD) are observed in 50–85% of cases.  Fallot tetralogy (1/3 of cases), atrioventricular canal, VSD, ASD, aortic coarctation and PDA represent 75% of the defects.  A  Atresia of choanae  R  Retardation of growth  G  Genito-urinary anomalies  E  Ear anomalies

15 What is CHARGE Syndrome? C  Ocular Coloboma  H  Heart defects  A  Atresia of choanae  R  Retardation of growth  Most patients have low normal birth weight and length (around 10th centile).  Postnatal growth retardation should be in relation to feeding difficulties and/or surgical problems.  Growth tends to catch up after infancy.  Due to hypothalamo-hypophyseal dysfunction.  G  Genito-urinary anomalies  E  Ear anomalies

16 What is CHARGE Syndrome? C  Ocular Coloboma  H  Heart defects  A  Atresia of choanae  R  Retardation of growth  G  Genito-urinary anomalies  Genital anomalies observed in 50–70% of cases (males: 80– 90%, females: 15–25%).  Hypoplastic external genitalia is the most common anomaly.  Anomalies of the urinary tract in 10–40%, such as renal ectopia, horseshoe kidneys, ureteral anomalies.  E  Ear anomalies

17 What is CHARGE Syndrome? C  Ocular Coloboma  H  Heart defects  A  Atresia of choanae  R  Retardation of growth  G  Genito-urinary anomalies  E  Ear anomalies  In 95–100%, the pinnae are asymmetrically misshaped, low set, anteverted, cup-shaped, wide, but with reduced vertical height.

18

19 What is CHARGE Syndrome? C  Ocular Coloboma  H  Heart defects  A  Atresia of choanae  R  Retardation of growth  G  Genito-urinary anomalies  E  Ear anomalies  Preauricular tags, hypoplastic auditory canal and microtia can be associated.  ME: absence of the stapedius muscle, absence of the oval window, and hypoplastic incus and stapes, with ossicular chain fixation.

20 What is CHARGE Syndrome? C  Ocular Coloboma  H  Heart defects  A  Atresia of choanae  R  Retardation of growth  G  Genito-urinary anomalies  E  Ear anomalies  Inner ear anomalies prevelance could be > 90%.  Malformation of the inner ear (CHARGE type of Mondini dysplasia).  Complete absence of the pars superior (utricle and semicircular canals) with or without Mondini dysplasia of the pars inferior (cochlea and saccule).

21 What is CHARGE Syndrome? C  Ocular Coloboma  H  Heart defects  A  Atresia of choanae  R  Retardation of growth  G  Genito-urinary anomalies  E  Ear anomalies  Deafness affects 60–90% of cases.  The commonest are severe conductive or mixed loss, and usually affects more the high frequencies than the low ones.

22 CHARGE Syndrome Diagnostic Criteria

23

24 What is CHARGE Syndrome?  Verloes major diagnostic criteria concentrated on the 3 C triad:  Ocular C oloboma.  C hoanal atresia.  Abnormal semicircular C anals.

25 Other Syndromes?

26 Choanal and ileal atresia: a new syndrome or association? Adi Yoskovitch, et al. International Journal of Pediatric Otorhinolaryngology 49 (1999) 237–240

27 How do patients with CA present?

28 Presentation ( Bilateral )  A newborn infant is an obligate nasal breather.  Due to the posterior soft palate covering the oropharynx and the neonate's tongue in close proximity with the hard and soft palate.  The hallmark is the cyclic cyanosis of the neonate.  When the infant’s mouth and nasal passages are closed → cyanosis → He cries, air flow occurs through the mouth and the cyanosis disappears.

29 Presentation ( Unilateral )  Unilateral atresia is rarely a cause of neonate respiratory distress.  Often not recognized at birth.  The most common presentation is a 1 to 2 y/o child with unilateral copious mucoid rhinorrhea or sinusitis.

30 Are Infants Really Obligatory Nasal Breathers? Paul S. Bergeson and J. Chris Shaw Clin Pediatr (Phila) 2001; 40; 567

31 Are Infants Really Obligatory Nasal Breathers? can  Rodenstein et al and deAlmieda et al, indicate that most children can initiate oral breathing after nasal occlusion. cannot  Other studies suggest there is a subset of infants who cannot begin mouth breathing after blocking their noses.  Studies claim anywhere between 0% and 50% of very young infants are not able to breathe through their mouths except when crying.

32 Examination

33 Examination  ABCs  V/S and signs of respiratory distress, dysmorphia.  Complete head & neck examination.  The diagnosis of atresia can be made by:  failing to pass no. 8 french catheter at least 32mm pass the anterior nares into the oropharynx.  The lack of movement of a thin wisp of cotton.  Administering methylene blue and assessing passage into nasopharynx.  Flexible fiberoptic endoscopy to assess the deformity.

34

35 Differential Diagnosis

36  Congenital stenosis of the pyriform aperture.  Dacryocystoceles (nasolacrimal duct cysts).  Encephaloceles.  Gliomas.  Dermoid cysts.  Teratomas.

37 How do you evaluate a patient with CA?

38 Evaluation  Genetics evaluation  Cardiology (EKG, Echo)  Ophthalmology evaluation  Hearing assessment  U/S kidneys

39 Imaging  CT scan:  The method of choice in evaluating CA.  Confirm the diagnosis of choanal atresia (unilateral or bilateral).  Evaluate choanal atresia (vomer bone width and choanal airspace distance).  Determine the degree of bony, membranous, or mixed atresia.  Exclude other possible nasal causes of obstruction & Delineates abnormalities in the nasal cavity and nasopharynx for pre-op planning.

40 Imaging  CT scan:  In bony CA: Medial bowing and thickening of the lateral wall of the nasal cavity, composed of the perpendicular plate of the palatine bone and the pterygoid process; enlargement of the vomer and fusion of these bony elements.  In membranous CA: the air passage between the lateral wall of the nasal cavity and the vomer is small when compared with the control group. *Slovis TL et al. Choanal Atresia: Precise CT Evaluation. Radiology. 1985;155:

41

42

43

44 What are the treatment options?

45 Management  Once the diagnosis is made → secure the child's airway and prevent asphyxiation.  Oropharyngeal airway or a McGovern nipple (temporary solution).  Surgical repair is the definitive treatment

46 Management  Bilateral atresia is emergent and usually is repaired within the first week to month of life.  Unilateral atresia can be repaired at a later date when the child's anatomy is larger and the procedure will be technically less difficult.  The method of repair is controversial, with no technique having gained universal acceptance.

47 Management  Main approaches are:  Transpalatal  Transnasal  Transseptal  External Rhinoplasty approach  Laser repair  With the advent of miniaturized endoscopic equipment and powered instrumentation, the most popular and successful method over the past decade has been the transnasal technique* *Schoem S. Transnasal endoscopic repair of choanal atresia: Why stent? Otolaryngology– Head and Neck Surgery 2004;131,4:

48 Management ( Transpalatal )  The approach is through the palate directly to the atretic plate.  Pros:  provides excellent exposure.  high success rate.  Cons:  requires more operative time.  increased blood loss.  longer recovery time.  possible occurrence of palatal fistula, palatal dysfunction, and maxillofacial growth disturbance.

49 Management ( Transpalatal )

50 Management ( Transnasal )  The evolution of optical telescopes, miniaturization of conventional instruments, and development of powered microdebriders with drill attachments have revolutionized the transnasal approach.

51 Management ( Transnasal )  Pros:  Clear vision of the operative field.  Accurate removal of the atresia plate and posterior vomerine bone, thus reducing the rate of restenosis significantly.  It is a short and safe procedure.  Swift recovery and short hospitalization.  Does not impair development of dental arches.  Cons:  The potential for rapidly aspirating orbital and cerebral contents when laminae are violated.

52 Stenting??

53 Transnasal endoscopic repair of choanal atresia: Why stent? Schoem S. Otolaryngology– Head and Neck Surgery 2004;131,4:  Debate still surrounds postoperative stenting. NO stents  13 children ages 2 days to 13 years old (mean 45 months) who presented with unilateral or bilateral CA underwent transnasal endoscopic surgery with NO stents used.  4 who had unilateral CA, underwent office serial examinations alone after surgery and all remained patent (<50% restenosis).

54  9 pts underwent reexamination under GA 3 to 4 weeks after initial surgery followed by serial office nasal endoscopy at 1-,3-, 6-, and 12-month intervals.  7 out of the 9 pts (78%) had varying amounts of emerging granulation tissue or minor synechiae that resolved with microdebrider reexcision. no restenosesNo office dilations no long-term complications  All serial office endoscopies demonstrated no restenoses, No office dilations were performed, and there were no long-term complications. Transnasal endoscopic repair of choanal atresia: Why stent? Schoem S. Otolaryngology– Head and Neck Surgery 2004;131,4:

55 Topical Mitomycin?

56 Topical mitomycin as an adjunct to choanal atresia repair. Prasad M et.al. Arch Otolaryngol Head Neck Surg Apr;128(4):  20 pts. with either unilateral or bilateral congenital choanal atresia underwent repair using the transnasal endoscopic approach, the transpalatal approach, or both with 1 or 2 applications of mitomycin (0.5 mg/ml) for 3 minutes.  17 retained patent airways. 3 pts. experienced improvement from a total atresia to a narrowed, stenotic choana.

57 Effect of use of mitomycin C on the outcome of Choanal atresia repair Al-Ammar AY Saudi Med J Oct;28(10): Al-Ammar AY Al-Ammar AY  Endoscopic repair of CA was performed in 20 cases.  Intra-operative application of topical MMC (0.4 mg/mL for 4 minutes) was carried out in 13 children, and 7 children did not receive MMC.  The follow-up period ranged between 9 months and 6 years.

58  69 % of the cases receiving MMC had a successful outcome compared with 57% of non-MMC cases, The difference was not statistically significant (p=0.230).  In unilateral CA, 70% of those who received MMC had a successful outcome compared with 60% of those who did not; again the difference was not statistically significant (p=0.566)

59 Postoperative Care

60  Stent irrigation and routine suctioning.  The patients are maintained on oral antibiotics (amoxicillin or a cephalosporin) while stented.  The stents are left in place from 2 days – 6 weeks (Debate??)  At the time of stent removal, examination under GA or fiberoptic nasopharyngoscope is performed to assess mucosalization and diameter.

61 Postoperative Care  If granulation tissue is found, it is debrided, cultured and abx are continued.  Systemic or local steroids are often used.  Repeat dilatation with a urethral sound and revision surgery is required for restenosis.

62 Predictive Factors for Success After Transnasal Endoscopic Treatment of Choanal Atresia Teissier N, et al. Arch Otolaryngol Head Neck Surg Jan;134(1):57-61 Teissier N Teissier N  To analyze the different factors affecting the outcome of transnasal endoscopic repair of choanal atresia (CA) in children with a follow-up of 43 months. restenosis  Predictive factors of restenosis are:  The presence of GERD  Age younger than 10 days at the time of surgery  Insufficient postoperative endoscopic revision not  previous surgery and associated malformations are not predictive of a poor surgical outcome.

63 Questions What is Choanal Atresia (CA)? Why does it happen? Are there risk factors for developing CA? What is CHARGE syndrome? How do patients with CA present? How do you evaluate a patient with CA? What are the treatment options?

64 Thank You


Download ppt "Choanal Atresia Saad A. Alsaleh. Questions  What is Choanal Atresia (CA)?  Why does it happen?  Are there risk factors for developing CA?  What is."

Similar presentations


Ads by Google