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Choanal atresia
Fahad zakwan
MD5
CONTENTS
1 Introduction
2 Anatomy
3 Aetiology
4 Associated abnormalities
5 Presentation
6 Diagnosis
7 Treatment
INTRODUCTION
• Choana is the posterior nasal aperture.
• The choanae are the posterior openings that
connect the nasal cavities with the nasopharynx.
• They develop between the third and seventh
embryonic weeks, following rupture of the vertical
epithelial fold between the olfactory groove and
the roof of the primary oral cavity (pronasal
membrane)
DEFINITION
• Choanal atresia is a congenital disorder where the
back of the nasal passage (choana) is blocked,
usually by abnormal bony or soft tissue
(membranous) due to failed recanalization of the
nasal fossae during fetal development.
Or simply
• Developmental failure of the nasal cavity to
communicate with the nasopharynx
• Complete nasal obstruction in a newborn may cause
death from asphyxia.
• During attempted inspiration, the tongue is pulled to the
palate, and obstruction of the oral airway results.
• Vigorous respiratory efforts produce marked chest
retraction.
• Increased cyanosis and death may occur if appropriate
treatments are not available; however, if the infant cries
and takes a breath through the mouth, the airway
obstruction is momentarily relieved.
• Then the crying stops, the mouth closes, and the cycle
of obstruction is repeated.
Anatomy of the upper airway
Anatom
Epidemiology
• Occurs in ~1 in 5,000 – 7,000 live births
• More common in girls than boys
• Slightly increased risk exists in twins.
• Approximately 2 out of 3 cases are unilateral
– More commonly right-sided
• Maternal age or parity does not increase the frequency
of occurrence.
• Chromosomal anomalies are found in 6% of infants with
choanal atresia.
• Choanal atresia occurs with equal frequency in people
of all races
Etiology
a)Embryogenesis
• Persistence of the buccopharyngeal membrane
• Failure of the bucconasal membrane of Hochstetler 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
B)Prenatal/maternal
• use of antithyroid (methimazole, carbimazole)
medications was linked to choanal atresia. (In 2008,
Barbero et al )
• Intake in the highest quartile: Vitamin B-12, zinc,
niacin
• Intake in the lowest quartile: Methionine, vitamin D
• Cigarette smoking
• Coffee (≥ 3 cups per day)
TYPES
There are two forms of
choanal atresia,
•unilateral atresia.
•bilateral atresia
Unilateral choanal atresia
• Unilateral choanal atresia is less
threatening to a newborn because the
nasal passage is only blocked on one
side.
• This allows an infant to breathe
somewhat normally at birth, but a mucus
discharge is noticeable in the affected
side of the nose.
Bilateral choanal atresia
• Bilateral choanal atresia is a blockage of both sides
of the nasal passage.
• This is life threatening because the baby cannot
breathe at birth.
• Crying allows the child to breathe until the infant
discovers that it can breathe through its’ mouth.
• A tube is placed in the child’s mouth and taped in
place to allow for an air passage.
Associated abnormalities
•May occur in isolation , or
•May be part of a multiple
congenital anomaly syndrome
Like, CHARGE syndrome and
VACTER association
CHARGE syndrome
• Coloboma of the iris, choroid, and/or microphthalmia
• Heart defect such as atrial septal defect (ASD) and/or
conotruncal lesion
• Atresia of choanae
• Retarded growth and development
• Genitourinary abnormalities such as cryptorchidism,
microphallus, and/or hydronephrosis
• Ear defects with associated deafness
Clinical features
BILATERAL
• Complete nasal obstruction
• Immediate respiratory distress
• Potential death due to asphyxia
• Cyclic respiratory obstruction
• Child cries opens the mouth and obstruction is
relieved
• In some cases, this may present as cyanosis
while the baby is feeding, because the oral
air passages are blocked by the tongue,
further restricting the airway.
• Symptoms of severe airway obstruction and
cyclical cyanosis are the classic signs
• The cyanosis may improve when the baby
cries, as the oral airway is used at this time
• These babies may require airway resuscitation
soon after birth
UNILATERAL
• Sometimes, a unilateral choanal
atresia is not detected until much later
in life because the baby manages to
get along with only one nostril
available for breathing
• Rarely causes respiratory distress
• Mucoid discharge
OTHER MANIFESTATIONS
•Feeding difficulty
•Respiratory collapse
•Failure to thrive
DIAGNOSIS
• Imaging Studies
• Diagnostic Procedures
Imaging Studies
• Rhinography is a
procedure that
involves the
administration of
radiopaque dye into
the nasal cavity as
illustrated in the right
image.
• CT scanning is the radiographic procedure of choice in
the evaluation of choanal atresia.
• For good results, careful suctioning is performed to clear
excess mucus, and a topical decongestant is applied.
• The purpose of CT scanning is outlined as follows:
– Confirm the diagnosis of choanal atresia (unilateral or
bilateral).
– Evaluate choanal atresia
– Exclude other possible nasal sites of obstruction.
– Determine the degree of bony, membranous, or mixed atresia.
– Delineate abnormalities in the nasal cavity and nasopharynx.
• The ENT doctor
can visualize the
choanal closure
with a small,
flexible scope
passed through
the nostril
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
• The Toynbee Test is performed by visual inspection of
the tympanic membrane while the patient swallows
with their nose manually occluded.
• This generates a positive pressure within the
nasopharynx, followed by a negative pressure phase
and is considered positive when there is an alteration
in middle-ear pressure as assessed by pneumatic
otoscopy before and after the maneuver.
• Negative middle-ear pressure or temporary negative
middle ear pressure followed by return to ambient
pressure after the Toynbee test usually is indicative of
normal eustachian tube function
The Politzer test is performed by visual
inspection of the tympanic
membrane while compressing one
naris into which the end of a rubber
tube attached to an air bag has
been inserted while the opposite
naris is compressed with digital
pressure. The patient is asked to
repeat the letter K or to swallow
while air is injected into the nasal
cavity. When positive, the
overpressure that develops in the
nasopharynx is transmitted to the
middle ear, and only indicates an
anatomically patent ET tube
Catheter test
Mirror test
Differential diagnosis
• Deviated nasal septum
• Dislocated nasal septum
• Septal hematoma
• Mucosal swelling
• Turbinate hypertrophy
• Encephalocele
• Nasal dermoid
• Hamartoma
• Chordoma
• Teratoma
Management
• Immediate management of infants with
choanal atresia includes placement of an oral
airway and initiation of gavage feedings
• Definitive repair involves transnasal puncture
and stenting or endoscopic resection of the
posterior nasal septum through a transnasal
approach with or without stenting
• Classic, transpalatal approach is reserved for difficult
or recurrent cases,
• Recurrent stenosis may occur even after successful
surgery, is prevented by inserting a stent and
securing it with a suture (to prevent aspiration).
• The definitive surgical repair of bilateral choanal
atresia is performed during the first weeks or months
of life.
• Surgery for unilateral atresia can be postponed until
school age, when the anatomy of the region is more
similar to that encountered in adults.
Surgical correction
• Surgical procedures to correct choanal atresia can
be broadly classified into transnasal and transpalatal
approaches.
• The decision to use a transnasal versus a transpalatal
approach rests on the surgeon’s assessment of the
choanal anatomy.
• The composition of the atretic plate, the depth and
shape of the nasopharynx, and the presence of
other anomalies are the most important factors.
• The transnasal approach requires less operative time
and causes slightly less morbidity related to the
incision.
• The transpalatal approach provides better exposure
and more accurate bone removal.
–The palatal incision increases operative time and
blood loss.
–The transpalatal approach probably reduces the
risk of major vascular injury, intracranial
complications, and restenosis.
Endoscopic Surgery
• The use of the rigid endoscope in the management of
posterior choanal atresia represents a significant
advancement in choanal surgery.
• It provides an extremely sharp image with a magnified
overview.
• It enables the surgeon to see the tips of his instruments, so that
the bone is removed safely under direct endoscopic vision.
• It allows assessment of the size of the opening, in comparison
to the normal choana.
• It ensures greater precision in flap preservation.
• The technique is short in time and safe, with early recovery
and short hospitalization.
Creation of
fenestra
in
endotracheal
tube
Endotracheal stent
showing vent at
posterior wall of
endotracheal tube
for air to pass
through. The anterior
wall in continuity.
The small vents at
different sides of
tube for nasal
secretion drainage
Red rubber
catheters
passed from
nose to
oropharynx
The red rubber
catheters are
attached to the
stent, so the
stent can be
position in the
choana
when they are
pulled through
nose
The two ends of
stent are
delivered
through nose,
centering the
mid portion at
choana for
respiration
The stent
is sutured
with silk
sutures
Thank yuuuu

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3. choanal atresia

  • 2. CONTENTS 1 Introduction 2 Anatomy 3 Aetiology 4 Associated abnormalities 5 Presentation 6 Diagnosis 7 Treatment
  • 3. INTRODUCTION • Choana is the posterior nasal aperture. • The choanae are the posterior openings that connect the nasal cavities with the nasopharynx. • They develop between the third and seventh embryonic weeks, following rupture of the vertical epithelial fold between the olfactory groove and the roof of the primary oral cavity (pronasal membrane)
  • 4. DEFINITION • Choanal atresia is a congenital disorder where the back of the nasal passage (choana) is blocked, usually by abnormal bony or soft tissue (membranous) due to failed recanalization of the nasal fossae during fetal development. Or simply • Developmental failure of the nasal cavity to communicate with the nasopharynx
  • 5. • Complete nasal obstruction in a newborn may cause death from asphyxia. • During attempted inspiration, the tongue is pulled to the palate, and obstruction of the oral airway results. • Vigorous respiratory efforts produce marked chest retraction. • Increased cyanosis and death may occur if appropriate treatments are not available; however, if the infant cries and takes a breath through the mouth, the airway obstruction is momentarily relieved. • Then the crying stops, the mouth closes, and the cycle of obstruction is repeated.
  • 6. Anatomy of the upper airway
  • 8. Epidemiology • Occurs in ~1 in 5,000 – 7,000 live births • More common in girls than boys • Slightly increased risk exists in twins. • Approximately 2 out of 3 cases are unilateral – More commonly right-sided • Maternal age or parity does not increase the frequency of occurrence. • Chromosomal anomalies are found in 6% of infants with choanal atresia. • Choanal atresia occurs with equal frequency in people of all races
  • 9. Etiology a)Embryogenesis • Persistence of the buccopharyngeal membrane • Failure of the bucconasal membrane of Hochstetler 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
  • 10. B)Prenatal/maternal • use of antithyroid (methimazole, carbimazole) medications was linked to choanal atresia. (In 2008, Barbero et al ) • Intake in the highest quartile: Vitamin B-12, zinc, niacin • Intake in the lowest quartile: Methionine, vitamin D • Cigarette smoking • Coffee (≥ 3 cups per day)
  • 11. TYPES There are two forms of choanal atresia, •unilateral atresia. •bilateral atresia
  • 12. Unilateral choanal atresia • Unilateral choanal atresia is less threatening to a newborn because the nasal passage is only blocked on one side. • This allows an infant to breathe somewhat normally at birth, but a mucus discharge is noticeable in the affected side of the nose.
  • 13.
  • 14. Bilateral choanal atresia • Bilateral choanal atresia is a blockage of both sides of the nasal passage. • This is life threatening because the baby cannot breathe at birth. • Crying allows the child to breathe until the infant discovers that it can breathe through its’ mouth. • A tube is placed in the child’s mouth and taped in place to allow for an air passage.
  • 15. Associated abnormalities •May occur in isolation , or •May be part of a multiple congenital anomaly syndrome Like, CHARGE syndrome and VACTER association
  • 16. CHARGE syndrome • Coloboma of the iris, choroid, and/or microphthalmia • Heart defect such as atrial septal defect (ASD) and/or conotruncal lesion • Atresia of choanae • Retarded growth and development • Genitourinary abnormalities such as cryptorchidism, microphallus, and/or hydronephrosis • Ear defects with associated deafness
  • 17. Clinical features BILATERAL • Complete nasal obstruction • Immediate respiratory distress • Potential death due to asphyxia • Cyclic respiratory obstruction • Child cries opens the mouth and obstruction is relieved
  • 18. • In some cases, this may present as cyanosis while the baby is feeding, because the oral air passages are blocked by the tongue, further restricting the airway. • Symptoms of severe airway obstruction and cyclical cyanosis are the classic signs • The cyanosis may improve when the baby cries, as the oral airway is used at this time • These babies may require airway resuscitation soon after birth
  • 19. UNILATERAL • Sometimes, a unilateral choanal atresia is not detected until much later in life because the baby manages to get along with only one nostril available for breathing • Rarely causes respiratory distress • Mucoid discharge
  • 21. DIAGNOSIS • Imaging Studies • Diagnostic Procedures
  • 22. Imaging Studies • Rhinography is a procedure that involves the administration of radiopaque dye into the nasal cavity as illustrated in the right image.
  • 23. • CT scanning is the radiographic procedure of choice in the evaluation of choanal atresia. • For good results, careful suctioning is performed to clear excess mucus, and a topical decongestant is applied. • The purpose of CT scanning is outlined as follows: – Confirm the diagnosis of choanal atresia (unilateral or bilateral). – Evaluate choanal atresia – Exclude other possible nasal sites of obstruction. – Determine the degree of bony, membranous, or mixed atresia. – Delineate abnormalities in the nasal cavity and nasopharynx.
  • 24.
  • 25.
  • 26. • The ENT doctor can visualize the choanal closure with a small, flexible scope passed through the nostril
  • 27. 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
  • 28. • The Toynbee Test is performed by visual inspection of the tympanic membrane while the patient swallows with their nose manually occluded. • This generates a positive pressure within the nasopharynx, followed by a negative pressure phase and is considered positive when there is an alteration in middle-ear pressure as assessed by pneumatic otoscopy before and after the maneuver. • Negative middle-ear pressure or temporary negative middle ear pressure followed by return to ambient pressure after the Toynbee test usually is indicative of normal eustachian tube function
  • 29. The Politzer test is performed by visual inspection of the tympanic membrane while compressing one naris into which the end of a rubber tube attached to an air bag has been inserted while the opposite naris is compressed with digital pressure. The patient is asked to repeat the letter K or to swallow while air is injected into the nasal cavity. When positive, the overpressure that develops in the nasopharynx is transmitted to the middle ear, and only indicates an anatomically patent ET tube
  • 32. Differential diagnosis • Deviated nasal septum • Dislocated nasal septum • Septal hematoma • Mucosal swelling • Turbinate hypertrophy • Encephalocele • Nasal dermoid • Hamartoma • Chordoma • Teratoma
  • 33. Management • Immediate management of infants with choanal atresia includes placement of an oral airway and initiation of gavage feedings • Definitive repair involves transnasal puncture and stenting or endoscopic resection of the posterior nasal septum through a transnasal approach with or without stenting
  • 34. • Classic, transpalatal approach is reserved for difficult or recurrent cases, • Recurrent stenosis may occur even after successful surgery, is prevented by inserting a stent and securing it with a suture (to prevent aspiration). • The definitive surgical repair of bilateral choanal atresia is performed during the first weeks or months of life. • Surgery for unilateral atresia can be postponed until school age, when the anatomy of the region is more similar to that encountered in adults.
  • 35. Surgical correction • Surgical procedures to correct choanal atresia can be broadly classified into transnasal and transpalatal approaches. • The decision to use a transnasal versus a transpalatal approach rests on the surgeon’s assessment of the choanal anatomy. • The composition of the atretic plate, the depth and shape of the nasopharynx, and the presence of other anomalies are the most important factors.
  • 36. • The transnasal approach requires less operative time and causes slightly less morbidity related to the incision. • The transpalatal approach provides better exposure and more accurate bone removal. –The palatal incision increases operative time and blood loss. –The transpalatal approach probably reduces the risk of major vascular injury, intracranial complications, and restenosis.
  • 37. Endoscopic Surgery • The use of the rigid endoscope in the management of posterior choanal atresia represents a significant advancement in choanal surgery. • It provides an extremely sharp image with a magnified overview. • It enables the surgeon to see the tips of his instruments, so that the bone is removed safely under direct endoscopic vision. • It allows assessment of the size of the opening, in comparison to the normal choana. • It ensures greater precision in flap preservation. • The technique is short in time and safe, with early recovery and short hospitalization.
  • 38.
  • 40. Endotracheal stent showing vent at posterior wall of endotracheal tube for air to pass through. The anterior wall in continuity. The small vents at different sides of tube for nasal secretion drainage
  • 42. The red rubber catheters are attached to the stent, so the stent can be position in the choana when they are pulled through nose
  • 43. The two ends of stent are delivered through nose, centering the mid portion at choana for respiration

Hinweis der Redaktion

  1. Transnasal puncture not favored due to high rate of recurrence