Congenital of the Nose

3rd-8th week of gestation A funnel shaped dural projection extends inferiorly and anteriorly through a midline opening anterior to the crista galli of the ethmoid bone.This anterior skull base opening is the foramen cecum

The dural diverticulum extends inferior and posterior to the frontal and nasal bones and superior and anterior to the nasal cartilage (prenasal space) and terminates at the skin of the nasal bridge

With normal regression of the dural diverticulum, the prenasal space is obliterated, the foramen cecum closes and fusion of the fronticulus frontalis occurs forming the nasofrontal suture

Jhonson T.J. et al . 2014. Baileys Head and Neck Surgery Otolaryngology.5th ed. Pennsylvania. Lippincot Williams & Wilkins.*

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Abnormal Development

Jhonson T.J. et al . 2014. Baileys Head and Neck Surgery Otolaryngology.5th ed. Pennsylvania. Lippincot Williams & Wilkins.*

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EncephaloceleEncephalocele is an extracranial herniation of cranial contents through a defect in the skull. When an encepaholocele includes meninges only it is termed meningocele. Meningocele presents similarly without herniation of brain tissue

EncephalocelesOccipital (75%) Sincipital (25%)Basal (-1%)Jhonson T.J. et al . 2014. Baileys Head and Neck Surgery Otolaryngology.5th ed. Pennsylvania. Lippincot Williams & Wilkins.*

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EncephaloceleIncidence North America and Europe : 1 in 30,000 Asia : 1 in 5,000 No gender predilection or family tendency Commonly (40%) associated with other congenital anomalies Microcephaly,hydrocephalus,anopthalmia,corpus callosum dysgenesis

*Jhonson T.J. et al . 2014. Baileys Head and Neck Surgery Otolaryngology.5th ed. Pennsylvania. Lippincot Williams & Wilkins.

Leung et al. Congenital Sinanasal Disorders in Rhinology. Kennedy et al.Thieme. New York.2014*

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EncephaloceleLeung et al. Congenital Sinanasal Disorders in Rhinology. Kennedy et al.Thieme. New York.2014*

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Leung et al. Congenital Sinanasal Disorders in Rhinology. Kennedy et al.1st ed. Thieme. New York.2014*

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Leung et al. Congenital Sinanasal Disorders in Rhinology. Kennedy et al.Thieme. New York.2014*

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Treatment of Nasal Gliomas, Encephaloceles and Dermoid Cysts

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Developmental Errors of the Central Midface Nasolacrimal Duct Cyst and CNPAS

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*Leung et al. Congenital Sinanasal Disorders in Rhinology. Kennedy et al.Thieme. New York.2014


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Leung et al. Congenital Sinanasal Disorders in Rhinology. Kennedy et al.Thieme. New York.2014

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Leung et al. Congenital Sinanasal Disorders in Rhinology. Kennedy et al.Thieme. New York.2014

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*Leung et al. Congenital Sinanasal Disorders in Rhinology. Kennedy et al.Thieme. New York.2014Jhonson T.J. et al . 2014. Baileys Head and Neck Surgery Otolaryngology.5th ed. Pennsylvania. Lippincot Williams & Wilkins.

Reeves et al. Nasal cavity dimensions in congenital pyriform aperture stenosis. International Journal od pediatric Otorhinolaryngology. 77(2013) 1830-1832 *

Merea et al. CPAS: Surgical Approach with combined sublabial bone resection and inferior turbinate reduction without stents. The laryngoscope. March 2014*

*Reeves et al. Nasal cavity dimensions in congenital pyriform aperture stenosis. International Journal od pediatric Otorhinolaryngology. 77(2013) 1830-1832


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Congenital Bilateral Choanal AtresiaCongenital choanal atresia (CCA) is the developmental failure of the nasal cavity to communicate with nasopharynxRare malformation that causes airway obstruction in newborns and infantsAn incidence of 1 in 7000 to 8000 birthsIncidency: Females > males (1 : 2 )Frequently unilateral and right-sided than bilateral

*Leclerc JE, Fearon B. Choanal atresia and associated anomalies. Int J Pediatr Otorhinolaryngol 2011 ; 13: 265-72.Saleem A,et al. Congenital Bilateral Choanal Atresia. J Pak Med Associated journal. Vol. 60, No. 10, October 2010 871

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Current theories of Choanal atresia embryogenesis cite either failed oronasal membrane rupture or abnormal migration of neural crest cells into the nasal vault Most cases of CA are isolated malformationsBut association with other congenital deformities is not an exception As in the CHARGE association (a malformative syndrome that includes coloboma, heart disease, CA, retarded development, genital hypoplasia, and ear anomalies, including hypoplasia of the external ear and hearing loss).

*Leclerc JE, Fearon B. Choanal atresia and associated anomalies. Int J Pediatr Otorhinolaryngol 2011 ; 13: 265-72.Saleem A,et al. Congenital Bilateral Choanal Atresia. J Pak Med Associated journal. Vol. 60, No. 10, October 2010 871

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*BonyMixed BonyMembranous / purely membranousAtresia Classified Nature of the obstructing atretic plate has often been described as 90% bony and 10% membranous

Heneger AS, Strom M: Choanal atresia: A new embryonic theory and its influence on surgical management. Laryngoscope 2009; 92: 913-21

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*Bilateral Choanal atresia is a medical emergency maintaining an airway and relieving the obstruction is a priority

Increased cyanosis and death may occur if appropriate treatments are not available

The immediate management of neonates presenting with intermittent cyanosis is the insertion of an oral airway and feeding via an oro-gastric tube

Leclerc JE, Fearon B. Choanal atresia and associated anomalies. Int J Pediatr Otorhinolaryngol 2011 ; 13: 265-72.Saleem A,et al. Congenital Bilateral Choanal Atresia. J Pak Med Associated journal. Vol. 60, No. 10, October 2010 871

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There are numerous methods for correcting the condition Commonly used methods are the trans-palatal approachthe trans-septal approachthe endoscopic transnasal approach.

*Diagnosing and managing this rare neonatal emergency in a symptomatic neonate with bilateral choanal atresia, requiring transnasal repair using a telescope and powered instrumentation with postoperative stenting at a very early postnatal age of 10 days is presented Leclerc JE, Fearon B. Choanal atresia and associated anomalies. Int J Pediatr Otorhinolaryngol 2011 ; 13: 265-72.Saleem A,et al. Congenital Bilateral Choanal Atresia. J Pak Med Associated journal. Vol. 60, No. 10, October 2010 871

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Stankiewicz was one of the first to report the use of Endoscopic instruments for choanal atresia repair Trans - nasal puncture and dilatation followed by stenting with endotracheal portex tubes for 4-6 weeks is the initial surgical procedure of choice. Choanae must be stented for a period of at least 3-4 weekComplications of stenting that include local infection, formation of granuloma, foreign body reaction, and nasal synechia

*Van Den Abbeele T, Francois M, Nancy P. Transnasal endoscopic treatment of choanal atresia without prolonged stenting. Arch Otolaryngol Head Neck Surg 2002; 128: 936-40.Holland BW, McGuirt WF. Surgical management of choanal atresia: improved outcome using mitomycin. Arch Otolaryngol Head Neck Surg 2001; 127: 1375-80.Stankiewicz JA. The Endoscopic repair of choanal atresia. Otolaryngol Head Neck Surg 1999; 103: 931-7

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HighlightsThe human nose has evolved as an organ that maintains a constant, direct contact with the environment Nasal dermoids, gliomas, and encephaloceles share embryogenesis but usually can be differentiated based on clinical features.Potential or actual CNS communications pose the most challenging aspect of managing these three lesions. Once a congenital nasal mass is suspected, MRI should be obtained. cr should be used if MRI leaves any doubts as to bone anatomy or intracranial extent. *

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Approximately 25% of dermoids and 15% of gliomas have intracranial extension, which carries the risk ofmeningitis and therefore warrants promptsurgical treatment. Surgery for lesions without proven intracranial extension may be deferred temporarily. Well-planned surgical treatment with neurosurgical consultation usually leads to successful outcomes. Encephaloceles carry the worst prognosis because of the size ofthe associated skull base defects and associated morbidities.

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Congenital of the Nose