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DR TARIQUE AHMED MAKA

REGISTRAR ENT

MANAGEMENT OF BILATERAL

CHOANAL ATRESIA

MANAGEMENT OF BILATERAL CHOANAL ATRESIA

SEQUENCE OF CPC Case presentation

Literature review

Statistical data

Conclusion

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CASE PRESENTATION

MANAGEMENT OF BILATERAL CHOANAL ATRESIA

PATIENT PROFILE

Name XYZ

Age Newborn

Gender Male

Residence Rawalpindi

Date of admission 25.10.12

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PRESENTING COMPLAINTS

Difficulty in breathing

Cyanotic spells

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since birth

HISTORY OF PRESENT ILLNES

Difficulty in breathing

Cyanotic spells

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ANTENATAL HISTORY

25yrs

PG

Planned pregnancy

Regular antenatal visits

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NATAL HISTORY

39 weeks pregnancy

Spontaneous labour

Vertex presentation

Emergency LSCS

Liquor clear

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POSTNATAL HISTORY

Baby cried immediately

APGAR score 8/10 ,9/10

Admitted in NICU

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FAMILY HISTORY

Non consanguinous

No family history of any

Neonatal death

Congenital anomalies

Chronic illness in family

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Medical History

Surgical History

Drug History

Socio economic history

Unremarkable

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Heart rate 150 beats/min

Respiratory rate 66/min

Weight 2.1 kg

OFC 31.4cm

PHYSICAL EXAMINATION

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Cyanotic spells and breathing

difficulty with suprasternal,

intercostal,and subcostal recessions

Relieved on crying and mouth

opening

No misting of metallic tongue plate

Inability to pass nasal catheter

PHYSICAL EXAMINATION

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No mass/cyst No laryngeal air way obstruction Vocal cords equally mobile

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DIRECT LARYNGOSCOPY

GIT system

Central Nervous system

Cardiovascular System

Unremarkable

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SYSTEMIC EXAMINATION

Bilateral choanal atresia

Laryngomalacia

Vocal cord palsy

Haemangioma

DIFFERENTIAL DIAGNOSIS

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Subglotic stenosis

Test Result Reference range

Haemoglobin 17.4g/dL 16-20g/dL

Total Leucocyte Count 19.8x 10^9 /L 4.0-10.0x10^9 /L

HCT 0.528

MCV 101.3fl

MCH 33.4pg

MCHC 33.0g/l

Platelet Count 369 x 10 9 /L 150-400x10^9 /L

HAEMATOLOGICAL INVESTIGATION

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Test Result Reference range

Blood Sugar Random 89mg/dl

Blood Group A+ve -

Anti HCV Antibody -ve By Elisa Method

Hepatitis B surface antigen

-ve By Elisa Method

HAEMATOLOGICAL INVESTIGATIONS

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TEST PATIENT CONTROL

PT 14 14

PTTK 32 32

HAEMATOLOGICAL INVESTIGATIONS

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CT SCAN

CT scan paranasal sinus with contrast for delineation of Choanal atresia confirmed the diagnosis of bilateral choanal atresia of bony type

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CT SCAN

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CT SCAN

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Case Summary

Newborn

Breathing difficulty and cyanotic spells

Relieved by crying

Inability to pass nasal catheter

Bilateral choanal atresia on CT scan

CASE SUMMARY

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DIAGNOSIS

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BILATERAL CHOANAL ATRESIA

Case Summary

Guedel’s airway placed in mouth and secured to maintain airway

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EMERGENCY MANAGEMENT

Case Summary

Inj vit K 1g i/m stat

Inj clefron 100mg i.v BD

inj amikin15 mg i.v BD

MANAGEMENT

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Parents explained about the nature of condition in detail

Surgery planned through Transnasal approach

Pre-anaesthesia assessment done

Written informed consent obtained

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SURGERY

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OPERATIVE STEPS

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SURGERY

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SURGERY

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SURGERY

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SURGERY

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SURGERY

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SURGERY

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SURGERY

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SURGERY

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SURGERY

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SURGERY

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SURGERY

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SURGERY

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SURGERY

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SURGERY

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SURGERY

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SURGERY

Nursed at neonatal ICU for 2 days Administered

Inj augmentin Inj dexamethasoneI/V fluids as advised by PediatricianRegular suction of tubes to prevent blockage

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POST OP CARE

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POST OP CARE

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POST OP CARE

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FOLLOW UP

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PRESENT STATUS

LITERATURE REVIEW

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PART II

LITERATURE REVIEW

1755, Roederer

18th CenturyFirst recorded case

19th CenturyFirst successful surgery

HISTORY OFCHOANAL ATRESIA

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1854, Emmert

BRIEF ANATOMY OF CHOANAE

• Medially: Free posterior edge of vomer• Laterally: Medial pterygoid lamina• Roof : Body of sphenoid• Floor : Free posterior edge of horizontal plate

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 Choanal atresia is a rare life threatening congenital anomaly.

Persistence of nasobuccal membrane.

Unilateral or bilateral

CHOANAL ATRESIA

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Haddad J Jr. Congenital disorders of the nose. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds.

EPIDEMIOLOGY

1 in 5,000 to 7,000 live births Females > males Atresias are bony,membranous or mixed Unilateral to bilateral cases is 2:1 Unilateral presentation predominantly affects the

right nasal aperture

56 Hengerer AS, Brickman TM, Jeyakumar A. Choanal atresia: embryologic analysis and evolution of treatment, a 30-year experience. Laryngoscope. May 2008;118(5):862-866.

ETIOLOGY

Cause is unknown  Smoking Coffee consumption High zinc and B-12 intake Prenatal use of antithyroid

(methimazole, carbimazole) medications

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Barbero P, Valdez R, Rodríguez H, et al. Choanal atresia associated with maternal hyperthyroidism treated with methimazole: a case-control study. Am J Med Genet A. Sep 15 2008;146A(18):2390-5. 

PATHOGENESIS

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Persistence of the buccopharyngeal membrane

Failure of the bucconasal membrane to rupture

Medial outgrowth of vertical and horizontal processes

of the palatine bone

Abnormal mesodermal adhesions forming in the

choanal area

Assanasen P, Metheetrairut C. Choanal atresia. J Med Assoc Thai. May 2009;92(5):699-706. 

ASSOCIATED CONDITIONS

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Coloboma Heart defects Atresia choanae Retardation of growth Genital anomalies Ear abnormalities

(CHARGE)

Asher BF et al. Airway Complications in CHARGE Association. Arch Otolaryngol Head Neck Surg. May 1990;116:594-595.

CLINICAL PRESENTATION

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Bilateral choanal atresia is a life threatening condition:

Complete nasal obstruction Respiratory obstruction and

cyanosis is cyclic Inability to feed and breath at the

same time.

Sadek SA (January 1998). "Congenital bilateral choanal atresia" Int. J. Pediatr. Otorhinolaryngol. 42 (3): 247–56. doi:10.1016/S0165-5876(97)

CLINICAL EXAMINATION

Pashley NRT. Choanal Atresia,(Chapter33). Clinical Pediatric Otolaryngology. St. Louis, MO: C.V. Mosby Company; 1986 . 61v

Failure to pass a # 6 to 8 french plastic catheter through the nares

Placing wisps of cotton in front of the nares and absence of nasal movement of air.

Absence of fog on a mirror when placed under the nostrils

RECOMMENDED TESTS

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CT scanEndoscopy of the noseCardiac echoRenal ultrasoundRhinogram

DIAGNOSTIC TEST

CT with intranasal contrast that shows narrowing of the posterior nasal cavity at the level of the pterygoid plate is the confirmatory test

Benjamin B. Evaluation of Choanal Atresia. Ann Otol Rhinol Laryngol. 94:429-432.63

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MANAGEMENT OF

CHOANAL ATRESIA

MANAGEMENT

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MANAGEMENT OF CHOANAL ATRESIA

EMERGENCY/ INITIAL

MANAGEMENT

SURGICAL MANAGEMENT

A. Airway oral airway of some sort must be

implemented very early

B. Feeding Delayed surgical correction;

orogastric tube

INITIAL MANAGEMENT

Hengerer AS et al. Choanal Atresia: A New Embryologic Theory and Its Influence on Surgical Management. Laryngoscope. August 1982;92:913-921.

A. Transnasal blind puncture

B. Transnasal endoscopic Approach

C. Transpalatal Approach

SURGICAL MANAGEMENT

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Richardson MA and Osguthorpe JD. Choanal Atresia. Current Therapy in Otolaryngology-Head and Neck Surgery. St. Louis, MO: B.C. Decker Inc.; 1990

TECHNIQUE 120 degree endoscope placed in mouth and positioned

in nasopharynx

Atretic plate perforated

Bilateral nasal stents placed

Philtrum is protected

Stents left in situ for 6 weeks

TRANSNASAL ENDOSCOPIC APPROACH

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Advantages: a. This approach is faster, easier, and less problematic.

b. Blood loss is minimal.

c. Excellent for children of all ages.

d. Allows for earlier repair in properly selected patients and may avoid restenosis.

e. The child can breast feed immediately post op.

f. If no additional problems then the child can be discharged on the 3rd or 4th post op day.

TRANSNASAL ENDOSCOPIC APPROACH

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DISADVANTAGES AND COMPLICATIONS: a. Longer stenting time post op.

b. Can not be done on patients with multiple nasal and nasopharyngeal anomalies.

TRANSNASAL ENDOSCOPIC APPROACH

Josephson GD, Vickery CL, Giles WC, Gross CW. Transnasal endoscopic repair of congenital choanal atresia: long-term results. Arch Otolaryngol Head Neck Surg. May 1998;124(5):537-40.

TRANSPALATAL APPROACH In rare circumstances where the

skull base is abnormally developed (other craniofacial anomalies are present), the surgeon may need to use a transpalatal approach to access the area of choanal atresia through the roof of the mouth.

SURGICAL MANAGEMENT

Zeitouni AG, Shapiro RS. Congenital anomalies of the nose and anterior skull base. In: Tewfik TL, Der Kaloustian VM, eds. Congenital Anomalies of the Ear, Nose, and Throat. New York, NY: Oxford University Press; 1997:189-200

A. STENTING:

1. Secure them in place for a approximate period of 4-8 weeks

B. WOUND CARE:

2. The parents must be taught to maintain the stents with frequent suction and a saline-moistened pipe cleaner or cotton applicator 3 to 6 times per day.

3. Antibiotics and decongestant72

POST OPERATIVE CARE

C. FOLLOW UP:

1. Every 2 weeks or more frequently if needed while the stents are in place.

2. Once the stents are removed, the patients are seen weekly for the first 2-3 weeks.

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POST OPERATIVE CARE

RELEVANT STATISTICAL DATA

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RELEVANT DATA

Total Cases of CHOANAL ATRESIA = 4 Duration 1st JANUARY, 2011 to 1st JANUARY, 2013 Unilateral = 3 Bilateral = 1 75

BilateralUnilateral

RELEVANT DATA

The male female ratio was 1 : 3

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male female0

0.5

1

1.5

2

2.5

3

3.5

Bilateral choanal atresia is a rare life threatening condition which presents with stridor and acute respiratory distress at birth

Only immediate clinical evaluation and efficient management is life saving

CONCLUSION

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Thank You

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