DIAGNOSIS AND TREATMENT ATRESIA KOANA

By NeoDie , 4 February, 2025

DIAGNOSIS AND TREATMENT ATRESIA KOANA

dr. I Made Nudi Arthana

Atresia koana is closed Wrong One or second part posterior from the nasal cavity by an abnormal membrane or bone. This congenital obstruction occurs behind the nose (posterior choana), which connects the nose and nasopharynx. Choanal atresia occurs due to embryological failure of buconal membrane division before birth. This disorder can occur together with other congenital disorders, namely coloboma, heart defects, mental retardation, growth disorders, genital hypoplasia, ear deformities and deafness ( CHARGE syndrome) . In addition, it can also occur together with Crouzon syndrome, Pfeiffer syndrome and Antley-Bixler syndrome . 1,2

LITERATUR REVIEW

Nose

Embryology Nose

Nose formed from nasal placode moment Sunday third And fourth in life intrauterine. Every nasal placode is surface ectoderm thick local and is the lateral area of the head when it becomes an embryo. Then during the fifth week, the nasal placode develops due to ectodermal thickening and can be seen on the frontonasal process. In the sixth week the ectodermal fold at the epicenter of the nasal placode will penetrate the underlying mesenchyme. By the end of the seventh week, there are lateral and inferior walls on the nasal process and the inferior tip forms the anterior roof of the oral cavity. On region superior labial, process intermaxillary develop forming the philtrum . The posterior nasal and oral cavities will be separated by the oronasal membrane, if this oronasal membrane is absorbed it will form a primitive choana at the end. Sunday seventh. So happen formation palate primary, koana This located in side right And left line middle And appropriate in behind palate primary. Then when the mesenchymal cells condense in the ventral region of the secondary palate, the nostrils migrate medially and the soft tissues unite and become circular and become beginning from migration part medial. Part nasal medial will process the formation of the anterior nasal septum, the middle upper lip and the hard part of the hard palate . In the dorsal region of the secondary palate, myogenic mesenchymal cells change into the muscular layer of the soft palate . Then the base of the nasal cavity or primary palate is formed on the intermaxillary process. The eighth and ninth weeks, the secondary palate is formed from the maxillary process. Cell proliferation occurs from the mesoderm and ectoderm of the medial nasal region and the frontonasal process helps form the nasal septum that separates the left and right choana. 3.5

Picture 1. Embryology rice cavity . 3

Picture 1. Embryology rice cavity . 3

Anatomy Nose

The nose from the outside is shaped like a pyramid with parts consisting of osteocartilaginous, muscle, and skin. The nose has an outer and inner part. The parts are the base of the nose (bridge), the bridge of the nose (dorsum nasi), the apex nose ( tips ), rice style, columella and hole nose (nares anterior). Nasal part consists of from part outside And part in. Nose part outside formed by a framework of bones and cartilage covered by skin, connective tissue, and several small muscles that function to widen and narrow the nostrils. The upper third of the outer nose is a bony framework while the upper two thirds underneath is bone vulnerable. Framework bone consists of on ostium nasalis, frontal process, maxillary ostium and nasal process of frontal ostium. The cartilage consists of a pair of superior lateral nasal cartilages, a pair of inferior lateral nasal cartilages and finally the anterior edge of the septal cartilage. 4

The nasal cavity is shaped like a tunnel from front to back, separated by a nasal septum in the middle, which divides the right and left nasal cavity. And left. With door enter Which shared two part that is nares front and the posterior nares which are also called choanae. Each nasal cavity consists of a part lined with skin and mucosa. 4

Picture 2 . Osteocartilaginous on nose.

Picture 2 . Osteocartilaginous on nose.

Picture 3. Nasal wall part lateral. 4

Picture 3. Nasal wall part lateral. 4

The nose receives much of its blood supply from the external and internal carotid arteries. Bleeding nose shared on bleeding part up and down, front and in the septum. At the top, the nasal cavity receives blood from the anterior and posterior ethmoid arteries which are branches of the ophthalmic artery from the internal carotid artery. The bottom receives blood from branches of the internal maxillary artery, including the ends of the greater palatine artery and the sphenopalatine artery. Which go out from foramen sphenopalatine together nerve sphenopalatine and enters the nasal cavity behind the posterior end of the middle concha. The front of the nose is supplied by branches of the facial artery. In the septum there is anastomosis from branches artery sphenopalatine, artery anterior ethmoid, superior labial artery and greater palatine artery called Kiesselbach's plexus ( little's area ). 4

The motor nerves of the nose originate from the facial nerve. The front and upper part of the nose are innervated by sensory from  n.ethmoidalis front Which is branch from n.nasociliary Which originate from n.ophthalmicus. Cavity nose other receives sensory innervation from the maxillary nerve via the sphenopalatine ganglion while the olfactory nerve comes from the olfactory nerve. 4

Atresia Koana

Definition Atresia Koana

The choana is a funnel-shaped cavity or a pair of holes between the nasal cavity and the nasopharynx, also called choanae ossae . Choana atresia is a abnormality congenital Which marked with closed One or both posterior openings of the nasal cavity by abnormal membranes or bones. Choanal atresia occurs due to embryologic failure of the buconal membrane to divide before birth. Atresia koana more often associated with syndrome CHARGE (C= Coloboma , H= Heart Disease , A= atresia choanae , R= retarded growth and development , G= genital hypoplasia , E= ear deformities or deafness ). 1,2,6

Epidemiology Atresia Koana

Choanal atresia is a rare disorder. The incidence of choanal atresia is 1 case per 5,000 - 8,000 births. Unilateral choanal atresia more often happen reach 75% than atresia koana bilateral. The ratio of male to female comparison is 1:2. While the ratio of choanal atresia comparison is 1:2. unilateral And bilateral is 2:1. Risk increase on birth twins. Chromosomal anomalies can also be found in 6% of cases of choanal atresia and 50% of children with CHARGE syndrome almost all suffer from choanal atresia. 1,2,6,7

Etiology Atresia Koana

The cause of choanal atresia is still not known for certain, but there are many assumptions and theories from experts about the occurrence of choanal atresia. Several theories and literature say that choanal atresia occurs due to embryogenic failure. Other suspected causes include genetic disorders. The involvement of chromosome 22q11.2 which can accompany abnormalities congenital other such as facial, nasal and palate deformities, polydactyly, diseases heart congenital, coloboma iris And retina, retardation mentally, external ear malformations, esophageal atresia, craniosynostosis, tracheoesophageal fistula and meningocele. 1,7

Pathogenesis Choanal Atresia

Many recent theories have been proposed regarding the embryogenesis of choanal atresia, such as failure of the nasobuccal membrane to rupture with persistent tissue directed posteriorly and vertically as the face develops. This is thought to be the most likely explanation since 90% of atresias are bony atresias and the formation of the nasobuccal membrane is thought to change before the mesodermal is completely removed from the oral and nasal epithelium. In embryology, the posterior choana will open between the nasal cavity and the nasopharynx, the formation of in the third and seventh weeks after rupture of the vertical epithelial fold between the olfactory groove and the roof of the primary oral cavity (pronasal membrane). If this process is disrupted, choanal atresia occurs. Other causes include persistence of the buccopharyngeal membrane and mesodermal persistence resulting in adhesion to the nasal choana, or abnormalities in secondary mesodermal cell migration due to local factors. 7,8

Picture 4. Process occurrence of atresia koana.5

Picture 4. Process occurrence of atresia koana.5​

Symptom Clinical Choanal Atresia

The clinical symptoms of unilateral choanal atresia are different from bilateral ones. In unilateral atresia, there are usually no symptoms of serious respiratory obstruction in infants at first, unless there is a blockage in the nasal cavity, for example. due to respiratory tract infection above. Because No shows typical symptoms so that unilateral choanal atresia is often not diagnosed in the neonatal period and is only diagnosed as an adult, because the patient complains of a blocked nose on one side, recurrent unilateral rhinitis accompanied by mucoid secretions and hyposmia. 8

In bilateral choanal atresia, the clinical symptoms that appear are very typical and usually appear immediately after birth. The degree of respiratory obstruction and cyanosis even to respiratory distress. Bilateral choanal atresia is found immediately after birth, characterized by "cyclic cyanosis and apnea", namely apnea and cyanosis when resting and will disappear when crying restlessly, because this is when the baby's mouth opens and air can enter. This symptom is very worrying when feeding, because the baby is unable to suckle and breathe. in a way simultaneously. The emergence cyanosis And asphyxia Which can cause death if not treated immediately. 1-3.9

In addition to the above symptoms, other congenital abnormalities that can occur together with choanal atresia should also be considered. Choanal atresia is often associated with CHARGE (C= Coloboma , H= Heart Disease , A= atresia choanae , R= retarded growth and development , G= genital hypoplasia, E= ear deformities or deafness ). 1,2,10

Diagnosis Atresia Koana

The diagnosis of choanal atresia is based on alloanamnesis, physical examination and supporting examination. Complaints in the form of nasal obstruction and cyclic cyanosis and apnea appear when the child is quiet with the mouth closed or while breastfeeding and disappear when the child cries or when the mouth is open. On inspection, it is found that the patient tends to breathe through the mouth due to obstruction in the nose. On anterior rhinoscopy examination, it tends to be within normal limits, but sometimes there is secretion that comes out and persists. 3,9-11

The diagnosis is confirmed by inserting a catheter size no. 5 or 6 F at least. 3 cm through nose to nasopharynx, If catheter No can pass cavity rice so possibility existence atresia koana. Besides That Also by looking at the movement of cotton Which placed in front of the nose with mouth closed, There will be little or no cotton movement. In the mirror placed in front of the nose, there is no seen existence condensation. If methylene blue dripped in the rice cavity and Then seen in the pharynx, so atresia can be ruled out. 3,11,12 Supporting examinations in the form of a flexible endoscope is a method that

better because airway patency can be assessed and anatomy can be evaluated. Radiographic examination by injecting contrast into the nasal cavity will show an image of obstruction. CT scan is an option to assess bone or membrane abnormalities, position, thickness of the obstructed segment, so that it can help in planning reconstruction. CT scan also plays a role in detect other abnormalities that accompany choanal atresia, such as encephalocele , glioma, anterior skull defect. CT scan can also show the extent of the posterior part of the septum and the density of the dense bone that supports it laterally. Use of vasoconstrictor drops, nasal toilet, sedation or general anesthesia in newborns will affect the quality of good CT-scan images, 3,11-14

Picture 5. CT scan Show Atresia Koana Type Membrane And Bones. 2

Picture 5. CT scan Show Atresia Koana Type Membrane And Bones. 2

Treatment Choanal Atresia

Management varies and depends on the age, type of atresia and general condition of the patient. Emergency management of bilateral choanal atresia aims to secure the airway, for example by using McGovern nipple or tool help breathing oropharynx. McGovern nipple is a nipple commonly used in milk bottles that has been modified by making a larger hole. so the baby can breathe through mouth between the swallowing phases when fed, before further handling. 1-3

On atresia koana Which nature unilateral seldom happen condition emergency. Treatment can be delayed for several months, allowing for nasal growth, which improves the ease of surgery and reduces the risk of postoperative complications and restenosis. 1,2 Surgical procedures for choanal atresia is choanoplasty. As for technique the surgery that is with transnasal and transpalatal approaches. 3,15

Choanoplasty with Technique Surgery Approach Transnasal

Technique This can done with or without endoscopy ( blind transnasal ), but using an endoscope will provide better visualization and also satisfactory results. This technique is most suitable for neonates. The principle of this technique is to make a perforation/hole in the thin part of the atresia plate with a curette then widen it as wide as the normal opening and resect the posterior part of the vomer. The part OS plate pterygoid protruding can be reduced by drilling . To maintain the hole that has been made, post operation installed stent during 6 Sunday. Technique transnasal This can done in the following ways: 13-15

  1. Insert the trocar into the atresia wall by tracing the base of the nose. and septum. The hole is widened with Lister's metal catheter in stages. Then a stent is installed from the nose to the nasopharynx and then fixed with sutures on the columella,

  2. Opening the atresia using a drill, an incision is made in the mucosa of the atresia wall, then drilling is carried out on the bone which is part of the atresia, then the flap is rotated posteriorly and a plastic tube is installed.

  1. Opening the atresia with a curette. A hole is made in the wall of the atresia with a mastoid curette, the other index finger is inserted into the nasopharynx to protect the base of the sphenoid.

This method was popularized by Bienfild in 1950. The main advantage of this approach is Transnasal is a minimally invasive procedure with minimal bleeding and does not require a long time during the procedure. 13,16

Choanoplasty with Technique Surgery Approach Transpalatal

The transpalatal approach technique is by incising the mucosa of the hard palate, then elevating the mucoperiosteum, followed by opening the hard palate, then... done drilling wall atresia And installed plastic pipe. U-shaped plastic pipe can be removed and cleaned at any time. Usually the plastic pipe is maintained between 4-6 weeks. 16

Picture 6. Technique surgery with approach transpalatal. 3

Picture 6. Technique surgery with approach transpalatal. 3

The transpalatal procedure has a high success rate, allows better visualization and flap preservation, thus reducing the incidence of postoperative scarring and restenosis, and short-term stent use. The disadvantage of this technique is that it can cause growth disturbances in the palate. hard, arch alveolar, And midface e, abnormality occlusion for example palatal fistula, longer operating time and greater risk of bleeding, risk of damage to the greater palatine neurovascular bundle , and damage to the soft palate which can cause complications of rhinophonia. This transpalatal method is generally used in patients who are older, whose bones are starting to become dense or in cases with recurrent stenosis. 14,16

Topical use of mitomycin C may be an adjunct for improvement surgery atresia koana. Installation stent aiming For maintain choanal patency and prevent postoperative restenosis. The use of stents after choanal atresia surgery is still controversial. Some experts say that stents can stabilize the airway and prevent stenosis, but they can also be a nidus for infection and trigger the body's reaction to foreign body. Therefore, prophylactic antibiotics are recommended after stent installation . Antireflux drugs are also recommended because gastroesophageal reflux can cause granulation and stenosis. 2,9

Complications Atresia Koana

Choanal atresia patients have complications which is different on bilateral choanal atresia And unilateral. On atresia koana bilateral tend own complications of asphyxia that can be life-threatening. Need immediate diagnosis and management. While unilateral choanal atresia, surgery can still be postponed for up to a year. Possible complications are aspiration during feeding or when trying to breathe through the mouth, lack of food intake due to difficulty breathing which has an impact on the growth and development of infants who tend to be inhibited. However, around 50% of choanal atresia can be followed by other congenital abnormalities so that patients usually fall into a state of complications from other diseases. 3,9,11

Prognosis Choanal Atresia

Prognosis on atresia koana determined by a number of matter that is: the number of disorders suffered, the type of surgery performed, and the complications that occurred during And post operation. If done action And maintenance with good, it is also expected to get good results. 11,17

DISCUSSION

Choanal atresia is a congenital abnormality characterized by the closure of one or both posterior parts of the nasal cavity by an abnormal membrane or bone. According to Gupta et al. atresia koana is failure development from the nasal cavity in relation to the nasopharynx, this congenital occurrence is rare happen And appear around 1 from 7,000-8,000 birth life And two time occurs more in women than in men. According to Tewfik et al., the average incidence is 0.82 out of 10,000 individuals. Gawai et al. said that the incidence congenital This very seldom And happen around 1 from 5,000-7,000 live birth . 1,2,9,11

Choanal atresia is associated with other congenital abnormalities up to 50%. Most commonly occurs together with congenital CHARGE abnormalities (C = Coloboma, H = Heart Disease , A = Choanal Atresia, R = Retarded growth and development, G = Genital Hypoplasia, E = ear deformities or deafness). 2 According to Koppen et al., as many as 25% of cases of choanal atresia are associated with CHARGE syndrome. 6 Generally, 65% to 75% of patients with choanal atresia are unilateral, while the rest are bilateral. 5,9 Approximately 30% of choanal atresia occurs due to bone closure, while 70% is a mixture of bone and membrane or abnormal membrane. 1-3 According to Gupta et al., the frequency of unilateral choanal atresia is also more common than bilateral. 11

The exact cause of choanal atresia is still unknown, but many experts theorize that it is an embryogenic failure where the embryological period is... in formation nose, on two layer membrane Which consists of On the nasal and oral epithelium, rupture occurs and changes the shape of the choana which then becomes atresia koana. According to Gupta et al. Also say that reason the theory that most accepted that is failure or rupture membrane oronasal. 11 Other suspected causes include genetic abnormalities, namely the involvement of chromosome 22q11.2, which can accompany other congenital abnormalities. 5,7 Lee et al evaluated the relationship between infant T4 levels and non-syndromic choanal atresia. Research the show role from low level hormone thyroid with the occurrence of choanal atresia. 13

The diagnosis of choanal atresia is based on alloanamnesis, physical examination and supporting examination. Complaints in the form of nasal obstruction and cycles of cyanosis and apnea ( classic sign ) appear when the child is quiet with the mouth closed or breastfeeding and disappear when the child cries or when the mouth is open. 3,7,9 According to Gawai et al., the clinical symptoms of the patient depend on the type of choanal atresia. Bilateral choanal atresia will have symptoms of acute respiratory emergency in newborns. 9 Some literature states that the diagnosis is made by inserting a catheter size no. 5 or 6 F at least 3 cm through the nose to the nasopharynx, If the catheter No can pass through the nasal cavity, then there is a possibility of choanal atresia. Gupta et al. said that the examination was carried out using a French suction tube with a size of 5 inserted from the nose to the nasopharynx. If it does not pass through the nasal cavity, then there is a possibility of choanal atresia. Supporting examination in the form of flexible endoscopy is a better method because respiratory patency can be assessed and anatomy can be evaluated. Radiographic examination by inserting contrast to in cavity rice will show description obstruction. Diagnosis can also be confirmed by CT scan of the nose and paranasal sinuses. 11 CT scan is an option to assess bone or membrane abnormalities, position, thickness of obstructed segments, so that it can help in reconstruction planning. 3,16 According to Gawai et al. CT scan of the paranasal sinuses and endoscopy nasal used For know characteristic obstruction bone or membrane, position, location, so it can help for therapy planning. 9

Management varies and depends on the age, type of atresia and general condition of the patient. Gupta et al. stated that bilateral choanal atresia is an emergency disease because it causes total nasal obstruction so that the patient experiences asphyxia. Management of choanal atresia bilateral Which nature emergency aiming For ensure road breath. 1-3 In atresia koana Which nature unilateral seldom happen condition emergency. Handling can postponed during a number of month, so that allow For nasal growth, which increases the ease of surgery and reduces the risk of postoperative complications and restenosis. 1-3 Gawai et al said that the treatment of choanal atresia, especially bilateral atresia, is Mc Govern or oropharyngeal, which can train patients to breathe through the mouth with the help of indwelling devices such as nipples. 9

Surgical procedures for choanal atresia include transnasal and transpalatal approaches. 3,11 According to Gawai et al., the main advantages of the transnasal approach are minimally invasive procedures, fast time, minimal bleeding. 9 The previous transpalatal approach also had a high success rate, can be used in cases of recurrent stenosis where visualization is possible the good and maintenance of the flap so that reducing the incidence of postoperative scarring and restenosis, and short-term stent use. However, can cause disturbance growth on palate hard, alveolar arch, and midface , occlusion abnormalities such as palatal fistula, length of surgery and blood loss, risk of damage to the greater palatine neurovascular bundle , and damage to the soft palate which can cause future problems in the form of rhinophonia. 9,11

Recent studies using the transnasal approach have shown excellent results. Richardson et al performed both the transnasal and transpalatal approaches. on 37 patient And the result with approach transnasal more effective, minimal bleeding occurs and without abnormal facial growth. 9

Use mitomycin C in a way topical as addition For Surgical repair of choanal atresia may offer improved patency with decreased need for For installation stents, dilation and operation revision. 17 Based on Gawai et al. mitomycin C was not used on visible bone that was already covered by a mucosal flap. 9

Use stent after operation atresia koana Still controversial. Some experts say that stents can stabilize the airway and prevent stenosis, will but can Also become nidus For the occurrence infection And trigger the emergence of reaction body to object foreign. By because That recommended administration of prophylactic antibiotics after stent placement . Administration of antireflux drugs is also recommended because gastroesophageal reflux can cause granulation and stenosis. 1,2,9,11,12

CONCLUSION

Atresia koana is a abnormality congenital Which seldom happen and usually occurs in conjunction with other congenital abnormalities. By definition, atresia koana is closed wrong One or second posterior part cavity rice by abnormal membrane or bone. The diagnosis of this disorder can be established by anamnesis, physical examination and appropriate supporting examinations. Rapid management is needed in bilateral atresia to ensure airway patency. Management can be done using McGovern nipple or oropharyngeal breathing apparatus. While in unilateral choanal atresia, elective management can be done. Management can be done with choanoplasty surgery using transnasal and transpalatal surgical approaches.

REFERENCE

  1. Indrawati LPL, Riski MS, Parjaman R. Bilateral Choanal Atresia. ORLI. 2021;51(2):158-63.

  2. Tewfik TL, Alrajhi YA, Hagr AA. Choanal Atresia. Emedicine. Apr 02, 2021. Available from: http://emedicine.medscape.com/article/872409- overview. Accessed 2021 December 25 .

  3. Zalzal HG, O'Brien DC, Zalzal GH. Pediatric Anatomy: Nose and Sinuses. Operative Techniques in Otolaryngology-Head and Neck Surgery. 2018;29(2):44-50.

  4. Moreddu E, Rossi M, Nichollas R, Triglia J. Prognostic Factors and Management of Patients with Choanal Atresia. The Journal of Pediatrics. 2018;204(1):234-39.

  5. PL Dhingra, Shruti D, Deeksha D. Diseases of Ears, Nose and Throat & Head and Neck Surgery. Elsevier. 2017.

  6. The Coupling T. Bartmann D. Jacob M. Boots F. Muller A. Dresbach T. Send T. Diagnostics and therapy of bilateral choanal atresia in association with CHARGE syndrome. Journal of Neonatal-Perinatal Medicine. 2021;14(1):67-74.

  7. Barrow TA, Saal HM, de Alarcon A, Martin LJ, Cotton RT, Hopkin RJ. Characterization of congenital anomalies in individuals with choanal atresia. Arch Otolaryngol Head Neck Surg 2019;135(6):543-7.

  8. Kars A, Bingol F, Atalay F. A Rare Case Report: Bilateral Choanal Atresia in an Adult Patient. Eur J Rhinol Allergy 2020; 3(1): 26-8.

  9. Gadgets SA, Mail J.J.S. Bilateral Complete Congenital Choanal Atresia in an Adult Managed Endoscopically with Mucosal Flaps without Stenting. Otolaryngology online journal. 2017;6(3):122.

  10. King R. Tunkel DE. Choanal Atresia and Others Neonatal Nasal Anomalies. Clin Perinatol. 2018;45:751–67.

  11. Gupta M, Kour C. Congenital Bilateral Choanal Atresia: A Rare Case. J Rare Disord Diagn Ther. 2017;3(4):9.

  12. Moreddu E, Rizzi M, Adil E, Balakrishnan K, Chan K, Cheng A, et al. International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Diagnosis, pre-operative, operative and post-operative pediatric choanal atresia care. International Journal of Pediatric Otorhinolaryngology. 2019;123:151-5.

  13. Wootten CT, Myer CM. Congenital Aerodigestive Tract Anomalies. Bailey's Head and Neck Surgery Otolaryngology. 5th ed . 2014;1316-18.

  14. Elluru RG, Wootten CT. Congenital malformation of the nose. Cummings Otolaryngology Head and Neck Surgery. Mosby Elsevier. Edition 7th. 2020.

  15. Tinoco P, Pereira JCO, Filho RCL, Brito TSC, Pereira BM, Cararra VL, Godoy SMF. Bilateral Choanal Atresia in 34 Year-old Patients. Intl Arch Otorhinolaryngol. 2010;14(4):481-4.

  16. Brihaye P, Delpierre I, De-Villé A, Johansson A, Biarent D, Mansbach A. Comprehensive management of congenital choanal atresia. International Journal of Pediatric Otorhinolaryngology. 2017;98:9-18.

  17. Moreddu E, Rizzi M, Adil E, Balakrishnan K, Chan K, Cheng A, et al. International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Diagnosis, pre-operative, operative and post-operative pediatric choanal atresia care. International Journal of Pediatric Otorhinolaryngology. 2019;123:151-5.

 

Tags

Comments

BOC

Hosting Indonesia