CONGENITAL MALFORMATIONS OF THE NOSE

Mohammadreza OmraniOtorhinolaryngologist,International Board of Facial Plastic and Reconstructive Surgery

OVERVIEW

Rare Manifestations of aero digestive tractFrom subtle cosmetic, to life threatening disorders

CLASSIFICATION

Based on anatomic 1 -anterior Neuropore

2 -central Midface3-nasobuccal membrane

ERRORS OF ANTERIOR NEUROPORE

Anterior neuropore; medial to optic reccesses on 3rd weekBehind nasal bones, in front of septal cartilage; prenasal spacePrenasal space apex: cribriform area, foramen cecum, closed by fonticulus frontalis ( between nasal bone and frontal bones

Dermoid, glioma, meningocele, encephalocele

ENCEPHALOCELE

Meninge only; meningoceleBrain and meninge; meningoencephaloceleAsia; 1/6000No gender, family tendency, 40% other anomalies

ENCEPHALOCELE

OccipitalSincipital; Naso frontal, Naso ehtmoidal, Naso orbital, 25% of all EC.Basal; less common, between SOF and C P. Intra nasal mass, later in life manifest

ENCEPHALOCELE

Both sincipital and basal; Pulsatile, bluish, compressible lesions with transillmination.Expand with crying, straining, compression on jugular veins.HRCT, MRI, ddx of agenesis of CC, hydrocephalusMRI; ddx of meningocele from meningoencephalocele, saggital and contrast; help relation to Intra cranial cavity

ENCEPHALOCELE

Management; Surgery, first few months of life;Lowered risk of meningitis, better cosmetics, more complete repair of dural defect.Small; endoscopic approachLarge; combined approachDefect; peri cranial flap or calvarial boneComplications; CSF, menigitis, hydrocephalus.Reccurance; 4-10%

GLIOMAS

Glial tissue without patent CSF path,5-20% fibrous affiliation,

Rare, non familial, but males Extranasal (60%)

Intra nasal (30%)Combined (10%)

EXTRA NASAL GLIOMAS

Smooth, firm, non compressible, in glabella, sometimes in nose side and naso maxillary lines.

INTRA NASAL GLIOMAS

Polypoid, pale mass, protruding from nostril, no transillumination, no change with cryingMostly, lateral nasal valve near to middle turbinate, but septum.Rarely; oral cavity, naso pharynx, orbitPathology; no ependymal tissue in gliomasDx; CT, MRI, Endoscopy

GLIOMAS

SurgeryExtra; lateral rhinotomy, midline, bicoronal, external rhinoplasty ( may the best)Osteotomy for fibrous stalkIntra; endoscopic, 4-10% reccurance.

DERMOIDS

Fronto nasal inclusion cyst, anterior neuropore.Midline nasal mass; 1/3000-40 000Most common; dermoids, 10% of all dermoidsSporadic, male Accompanying hydrocephalus, mental retard, pinna, aural atresia, hyperthelorism in 5- 41%

DERMOIDS

Mid line pit or mass, ½ a dimple on rhinionFirm, lobulated, non compressible mass with a sinus, with discharge or infection, no enlargement with crying, no transillumination.A hair is in minority, but pathognomicRecurrent meningitis shows a tract to CNS, 4-45% Intra cranial extension

DERMOIDS

Thin slice 1-3mm CT with contrast,Bifid cista galli, but if normal, no IC extension

MRI; ddx nonenhancing dermoid from hemangioma or teratoma, high intensity T1 crista galli; dermoid extension

DERMOIDS

Extra cranial approach; access to midline, skull base, accptable scar, reconstruction of dorsumBest; external RhinoplastyTransglabellar sub cranial approach, midline vertical approach, lateral rhinotomy.IC; bicoronal extended approach

ERRORS OF CENTRAL MIDLINE

Normal nasal development; w 4-12W5; lateral and medial nasal prominencesArhinia, polyrhinia, Proboscis lateralis, robert’s Syndrom ( midline nasal cleft)

ARHINIA

Very rare, 30 casesSporadic, or with other malformationsWith genetic disorders, trisomy 10, 13, 21No external nose, no airway, maxillary hypoplasia, small high arched palate, hypertelorismHypernasality? Hyposmia

ARHINIA

Early; cleft feeder, or gastrostomy tubeProsthsis, vertical distraction osteogenesisSplit thickness skin graft with long term stentingRestenosis; common, dilationTissue expander, grafts, DCR, flaps

POLYRHINIA

Double nose, suplementary nostril ,Only 4 cases, anterior ( duplicate septum and passage) and posterior ( choanal atresia)1st; choana2nd ;lateral wall anastomosisExcision of suplementary nostril with primary closure

PROBOSCIS LATERALIS

A tube to medial canthus and the same side heminasal aplsia2nd to fusion of maxillary processCNS and orbital disorders ,

CT, treament delayed till facial growth completesRestenosis, dilation

CRANIOFACIAL CLEFT

Ocular hypertelorismBroad nasal rootLack of nasal tipTessier classification; 9-14; cranial, 0-8; facial

Median nasal cleft; bifid nose, internasal displasiaAccompanying median cleft lip, airway is acceptable

CLEFT LIP NASAL DEFORMITY

Male, 1/1000 complete cleft lip; nasal floor,Left, right, bilateral; 6, 3,1Most severe; bilateral complete cleft lip; flattened tip, short columella, maxillary hypoplasia, prognathism

CLEFT LIP NASAL DEFORMITY

Unilateral cleft lip; less severe, lateralized ala in the side, flat retracted nostril, caudal septum displaced to cleft side, cleft side maxillary hypoplasia, bifid, asymmetric nasal tipTreatment; primary “and” secondary rhinoplastyFirst attempt; 1st lip surgery ,

2nd; strut or shield graft for projection; stage 1 in 4-6 y, stage 2 ; 8-12y, following orthodontic correctionDefinitive op; 16-18 y

CONGENITAL NASAL PYRIFORM APPERTURESTENOSIS (CNPAS)

Over growth of nasal process of maxillary bone, manifest in 1st few months ,

Central mega incisor, hyper telorism, flat nasal bridge, pituitary disorders, with dental, facial anomaliesSymptoms like choanal atresia, fail to pass NGCT; width of pyriform, cavity and choana all reduced, but height of cavity and choana normal

CNPAS

Width in newborn > 11mm, in CNPAS< 8Central incisor; investigate CNS, chromosomal, pituitary.

CNPAS

Treatmnet; nonsurgical ( drops and tubes)

surgical; sub labial, drilling, 4w stent

NASO LACRIMAL DUCT CYST

Dacrocystocele; obstruction, epiphora30% duct obstruction in neonate, but this

anomaly is rareSusceptible to aspiration, resolves in 9 monthsEndoscopy; cystic mass in inferior meatusCT; confirmation, NLD and sac enlarged, inf meatus cystOp; feeding, infection, obstruction

ERRORS OF NASO BUCCAL MEMBRANE

W3-4; placodes appear.W5-6; naso buccal membrane rupture; fialure; choanal atresiaAnother theory; abnormal migration of neural crest cells ( Treacher Collins)

CHOANAL ATRESIA

1/5000-8000 ,2/3 unilateral, right sided50% of all, and 75% of bilateral;

anomalies; CHRGE, polydactily, Crouzon Syndrom, nasal, palatal, auricular deformitiesProblem; narrow cavity, lateral bony obstruction by pterygoid plates, medial obstruction by thickened vomer, membranous obstructionMixed atresia; 71%, no pure membrane atresia.

CHOANAL ATRESIA

Bilateral; cyanotic event,Fail to pass 6F catheter, in 32mm distanceCT; ddx stenosis and atresia ;

Stenosis; narrowed but patent choana, space<6mm.Unilateral; postpone surgery for several monthsBilateral; OG with orotracheal airway before surgery; try to wait if possible

CHOANAL ATRESIA

Operation; tran nasal; 0 hopkins or 120 fiberoptic naso pharyngoscopeEnter thinnset portion with suction, J currette, using kerrison punch, back bitting (!), laser, deibrider for removal of bone, hold soft tissue as needed, hold upper extension to the posterior tip of middle turbinate to prevent IC trauma.Stenting, mitomycin C, mucosal flaps

CHOANAL ATRESIA

High reccurence, lowest: older, non syndromic, unilateral, minimal mucosal trauma

THANKS