choanal atresia

Editor's Notes

1. In the name of Allah, The most gracious, the most merciful.
2. Bil choanal atresia is an acute emergency due to its potential to cause acute airway obstruction.Worthy commandant, respected seniors and my fellow colleagues, I Dr Tariq Ahmed, from the dept of ENT and head and neck surgery, will be presenting a case of Bilateral choanal atresia.
3. Sequence of cpc is as flashed
4. First I ll present the case
5. My patient, new born baby boy ,born in cmh rwp on 25th oct,2012 presented with
6. Difficulty in breathing and Cyanotic spells since birth
7. Moic of neonatal intensive care unit called ent splt to attend a newborn baby. who had respiratory distress and cyanotic spells since birth, which were relieved on crying.
8. His mother was a 25yrs old, primigravida, this was her planned pregnancy. She had regular antenal visits
9. Mother went into spontaneous labour at 39wks of pregnancy, Em lscs performed due to failed pol, Liqour was clear
10. Baby cried immediately after birth e APGAR score of 8/10 at 1min and 9/10 at 5 min after birth. Baby was shifted to nicu for observation,
11. Parents had non consanguinous marriage, there was no history of neonatal death, congenital anomalies or chronic illness in family
12. However medical,surgical and drug history is unremarkable and baby belongs to a middle socioeconomic class
13. His heart rate was 150 beats per mint.Respiratory rate was 66 breaths per mint with breathing difficulty having weight of 2.1 kg and occipitofrontal circumference of 31.4 cm
14. Newborn baby had respiratory distress with suprasternal, intercostal,and subcostal recessions,he also had cyanotic spells. which were relieved on opening mouth and crying. There was no misting of metallic tongue plate when placed under nostrils indicating occluded airways, Soft silicone nasal catheter could not be passed through nose on either side
15. Direct laryngoscopy with mackintosch laryngoscope revealed no mass,cyst or laryngeal air way obstruction and Vocal cords were normal and equally mobile
16. The rest of the systemic examinations was unremarkable
17. Based on history and clinical findings, The differential diagnosis included
18. His blood complete picture showed haemoglobin of 17.4g/dL & platelets were 369
19. Blood sugar, was WNL, hepatitis b and c serology were negative, hIS blood group was A positive
20. His coagulation profile was with in normal limit
21. CT scan paranasal sinus with contrast for delineation of Choanal atresia confirmed the diagnosis of bilateral choanal atresia of bony type
22. CT scan paranasal sinus with contrast for delineation of Choanal atresia confirmed the diagnosis of bilateral choanal atresia of bony type
23. CT scan paranasal sinus with contrast for delineation of Choanal atresia confirmed the diagnosis of bilateral choanal atresia of bony type
24. CT scan paranasal sinus with contrast for delineation of Choanal atresia confirmed the diagnosis of bilateral choanal atresia of bony type
25. To summarize, A newborn With breathing difficulty and cyanotic spells since birth, which were relieved by crying, OPENING MOUTH & placement of an oral airway, Soft silicone nasal catheter could not be passed through nose on either side, with confirmation of bilateral choanal atresia on CT scan
26. on basis of history, clinical examination and investigations, final diagnosis of bilateral choanal atresia was made and pediatrician was consulted, to rule out any other associated anomalies.
27. As an Emergency MEASURE,,,,,,,,oropharyngeal Guedel’s airway was placed in mouth and secured, to maintain airway, his dyspnea and cyanosis was relieved, and oxygen saturation became normal.
28. Parents were explained about the condition in detail Surgery was planned through Transnasal approach on Ist day of his birth on 26 oct 2012 Pre-anaesthsia assessment was done Written informed consent was obtained
29. Now the operative steps
30. Patient placed supine
31. General anaesthesia inducted
32. Airway secured with ETT of 2.5
33. Patient draped
34. Nasal cavities cleared of secretions by suction
35. Nasal cavities packed with adrenaline soaked guaze
36. Oral cavity and nasopharynx were examined
37. Nasal cavity examined with nasoendoscope Telescope of o introduced and atresia visualized
38. Nasoendoscopy in progress
39. atretic plate was perforated with the trocar and cannula, in its inferomedial part
40. Here blue arrow is pointing the aperture made in atretic plate1. superiorly - the undersurface of the body of the sphenoid bones, 2. laterally - the medial pterygoid lamina, 3. medially - the vomer, 4. inferiorly - the horizontal portion of the palatal bone
41. Opening made in the atretic plate widened with haegar dilator 3/6
42. Redundent mucosa removed under direct vision
43. Opening was furthur widened with circular cutting forceps under vision
44. Pieces of ETT tube placed on both sides to maintain patency , air flow and prevent stenosis Bilateral nasal stents are fashioned from two ET tubes cut to length,with bevelled end of each siiting in nasopharynx orientated towards septum
45. Both tubes secured with silk Philtrum is protected by a small piece of ET tube. stents are secured by a circumseptal 0 proline suture and are left in situ for six weeks
46. Post operatively patient was nursed in neonatal ICU for 2 days Administered Inj augmentin Inj dexamethasone I/V fluids as advised by Paediatrician And Regular suction of tubes was carried out to prevent blockage
47. Neonate in neonatal ICU after surgery without any distress and cyanosis
48. Regular nasal clearance with suction in progress Post op recovery was smooth and patient was discharged on 3rd post op day
49. Patient was followed up weekly and Endotracheal tubes were removed after 6 weeks
50. Another picture of patient at 8 weeks breathing comfortably through nose
51. Now the literature review
52. Slide 53
53. The choanae or posterior nasal apertures are divided medially by free posterior edge of vomer. Their roof is formed by the body of sphenoid with the overlapping, flared alae of the vomer and the vaginal process of medial pterygoid plate, and the floor by the free posterior edge of the horizontal plate of the palatine bone During embryonic life choanae are closed by bucconasal membrane. This membrane lies slightly more anterior to the eventual position of choanae, which do not become established until the third month of intrauterine life.
54.  Choanal atresia is a rare life threatening congenital anomaly, in which a bony or membranous occlusion blocks the passageway between the nose and pharynx.This condition is believed to be secondary to the persistence of nasobuccal membrane.it can be unilateral or bilateral.
55. It is estimated to affect 1 in 5,000 to 7,000 live births Is twice as common in females as males Atresias are bony,membranous or mixed, 70% are mixed while remaining are purely bony The ratio of unilateral to bilateral cases is 2:1 Unilateral presentation predominantly affects the right nasal aperture
56. exact etiology is not precisely known, both genetic and environmental triggers are suspected,maternal smoking, cofee consumption, excessive zinc and B12intake and prenatal use of antithyroid (methimazole, carbimazole) medications is linked to choanal atresia . 
57. A number of theories have been proposed to explain the occurrence of choanal atresia, they can be summarized as:
58. Associated congenital anomalies occur in association with choanal atresia in about 50% of the patients.which r known as CHARGE, that includes
59. immediate respiratory distress and even potential death due to asphyxia after birth. as the child falls asleep the mouth closes and a progressive obstruction starting with stridor followed by increased respiratory effort and cyanosis.
60. On clinical examination there will be Failure to pass a # 6 to 8 french plastic catheter through the nares. While Placing wisps of cotton in front of the nares, there ll b absence of nasal movement of air. And The absence of fog on a mirror when it is placed under the nostrils
61. An axial high resolution CT scan with thin sections (2-5mm) has become the single radiographic study of choice. The CT scan has proven valuable in the accurate assessment of both the normal and abnormal anatomy of the nasal cavity, posterior nasal choanae and nasopharynx Choanal atresia. Rhinogram demonstrating blockage of radiopaque dye at the posterior choanae. (placing methylene blue in nares & not visualisin g within pharynx.
62. Now coming towards the management of choanal atresia
63. Management of choanal atresia includes emergency or initial management followed by surgical that is the definative management
64. Temporary alleviation can be achieved by inserting an oral airway into the mouth In mild cases of unilateral choanal atresia feeding is main issue. In case of delayed surgical correction due to precarious condition of pt,orogastric tube iz pASSED for feeding
65. only definitive treatment is surgery to correct the defect. This technique should be avoided because of the potentialy serious complications: CSF leaks, midbrain trauma
66. The transnasal endoscopic approach is currently CHOP's procedure of choice for managing choanal atresia
67. Transpalatal approarch is used rarely only when patient has craniofacial anomalies
68. Post op stents are kept in place for 4-8 wks and parents are taught about frequent suction of stents with saline. Antibiotics & decongestants are prescribed.
69. Pts are followed 2 weekly till stents are in place and then weekly for first 2-3 wks after removal of stents to treat any granulation tissue and to prevent stenosis.
70. Lastly, the relevant statistical data
71. , the Total number of patients who presented with CHOANAL ATRESIA were 4, during last 2 yrs , out of them 1 was bilateral.
72. Slide 77