cong nasal malform

7/30/2019 Cong Nasal Malform

1/14

Congenital Sinonasal

Deformities

Jose M. Manaligod MD

Associate Professor

Pediatric Otolaryngology

Nasal Embryology

Nose forms fromprimordial facialstructures Lateral nasal

prominence

Medial nasalprominence

Maxillary prominence

Mandibularprominence


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Nasal Embryology

Developmental Malformations

Choanal atresia/stenosis

Congenital nasal pyriform aperturestenosis

Nasolacrimal Duct Cyst

Midline Nasal Masses

Congenital Tumors (teratomas,gliomas)

Nasal Obstruction in Infancy

Infants are obligate nasal breathers

Bilateral Nasal Obstruction In An InfantIs A Medical Emergency

Acute Treatment- Oral Airway orIntubation


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Bilateral Obstruction

Significant respiratory distress Intermittent cyanosis

Apnea

Feeding difficulties

Relieved by crying

May be life-threatening

Unilateral Obstruction

Asymptomatic

Presents late

Failure of passage of a small suctioncatheter (8F)

Unilateral discharge

Choanal Atresia

Incidence 1 in 7,000 births

Female:Male = 2:1

Unilateral:Bilateral = 2:1

90% bony, 10% membranous

Associated anomalies in 70% (e.g., CHARGE)

Diagnosis

flexible nasal endoscopy

CT scan


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Unilateral Choanal Atresia


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Bilateral Choanal Atresia

Surgical Treatment of ChoanalAtresia

Surgery

Transnasal endoscopic repair

Transpalatal

Endoscopic Transnasal Repair

Favored approach in infants

CO2 LASER

Visualization Otomicroscope

120o transoral telescope

Back-biting forceps

Powered instruments

Mitomycin C

+/- Stents


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Nasal Stents

Need regularirrigation to maintain

patency Remove after 2-3

weeks


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Nasolacrimal Duct Cyst

Failure of canalization of nasolacrimal duct Evagination of lacrimal duct into nose

causes airway obstruction

Can be missed by flexible nasal endoscopy

Anterior rhinoscopy often useful

Treatment: Nasal endoscopy with cystmarsupialization (+/- Crawford tubes)


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Congenital Midline Nasal Masses

Incidence 1 in 20,000 - 40,000 births

Nasal dermoids most common

may be associated with external dimpleor pit

May be asymptomatic or present withrespiratory distress or infection


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Dermoids

Nasal dermoids comprise: 1-3% of all dermoids in the body

3-12% of head and neck dermoids

Benign

Male predominance

Exist as cyst, sinus or fistula

Ectodermal and mesodermal elements

Nasal Dermoid

Rule out intracranial involvement

CT- best for bony detail

MRI- best for soft tissue delineation

High recurrence rate (~50%)

Multiple approaches: Lateral rhinotomy,rhinoplasty, osteoplastic flap, saggitalapproach

Nasal Glioma

Ectopic glial tissue without a patentintracranial connection

3:1 male to female ration

Similar to encephaloceles but withoutCNS and subarachnoid attachment

15% have a fibrous stalk with thesubarachnoid space without CSFconnection


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Nasal Glioma

May present intranasally or extranasally(Extranasal 60%, Intranasal 30%, both 10%)

Intranasal gliomas polypoid mass from lateral nasal wall

Extranasal firm and noncompressible

nasal dorsum

grow slowly

consist of dysplastic brain tissue

Encephaloceles

1:35,000 births in US (1:6000 in Asia)

No familial occurrence or sex predilection

75% are posterior, occipital, or parietal

25% are anterior, sincipital, or basal

Sincipital: around nasal dorsum, orbits andforehead associated with external mass

Basal: in nasal cavity, nasopharynx orposterior orbits without external mass

Faulty closure of foramen cecum at week 3


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Encephaloceles

Soft compressible pulsatile mass Increase with crying

Bluish tinge

Positive Furstenberg test

Extranasal encephaloceles fonticulus frontalis

Intranasal encephaloceles cribriform region

Complications

Cellulitis

Abscess formation

Cosmetic deformities

CSF leak

Recurrent meningitis

Frontal lobe abscess

Treatment

Depends on intracranial extension

Dermoids and nasal gliomas

Extracranial excison External rhinoplasty approach +/- coronal incision

Endoscopic approach

Encephaloceles

combined approach


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Congenital Teratoma

Contains endoderm, mesoderm amdectoderm elements

5% are found in head and neck mainly inthe cervical region and nasopharynx

Pedunculated mass may extend intooropharynx

Treatment: Surgical excision

Follow with CT or MRI +/- alpha fetoprotein levels

Summary

Congenital malformations of the noseand nasopharynx are rare

May be life-threatening

Usually detected at birth

Must rule out intracranialconnection; never biopsy nasalmasses before imaging

cong nasal malform

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