Congenital Malformations of the Nose

1. MohammadrezaOmrani Otorhinolaryngologist, International Board of Facial Plastic and Reconstructive Surgery Congenital Malformations of the Nose

2. Rare Manifestations of aero digestive tract From subtle cosmetic, to life threatening disorders Overview

3. Based on anatomic 1- anterior Neuropore 2- central Midface 3-nasobuccal membrane Classification

4. Anterior neuropore; medial to optic reccesses on 3rd week Behind nasal bones, in front of septal cartilage; prenasal space Prenasal space apex: cribriform area, foramen cecum, closed by fonticulusfrontalis ( between nasal bone and frontal bones Errors of Anterior Neuropore

5. Dermoid, glioma, meningocele, encephalocele

6. Meninge only; meningocele Brain and meninge; meningoencephalocele Asia; 1/6000 No gender, family tendency, 40% other anomalies Encephalocele

7. Occipital Sincipital; Naso frontal, Nasoehtmoidal, Naso orbital, 25% of all EC. Basal; less common, between SOF and C P. Intra nasal mass, later in life manifest Encephalocele

8. 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, hydrocephalus MRI; ddx of meningocele from meningoencephalocele, saggital and contrast; help relation to Intra cranial cavity Encephalocele

9. Management; Surgery, first few months of life; Lowered risk of meningitis, better cosmetics, more complete repair of dural defect. Small; endoscopic approach Large; combined approach Defect; peri cranial flap or calvarial bone Complications; CSF, menigitis, hydrocephalus. Reccurance; 4-10% Encephalocele

10. Glial tissue without patent CSF path, 5-20% fibrous affiliation, Rare, non familial, but males Extranasal (60%) Intra nasal (30%) Combined (10%) Gliomas

11. Smooth, firm, non compressible, in glabella, sometimes in nose side and naso maxillary lines. Extra Nasal Gliomas

12. Polypoid, pale mass, protruding from nostril, no transillumination, no change with crying Mostly, lateral nasal valve near to middle turbinate, but septum. Rarely; oral cavity, naso pharynx, orbit Pathology; no ependymal tissue in gliomas Dx; CT, MRI, Endoscopy Intra Nasal Gliomas

13. Surgery Extra; lateral rhinotomy, midline, bicoronal, external rhinoplasty ( may the best) Osteotomy for fibrous stalk Intra; endoscopic, 4-10% reccurance. Gliomas

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

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

16. Thin slice 1-3mm CT with contrast, Bifid cistagalli, but if normal, no IC extension MRI; ddxnonenhancingdermoid from hemangioma or teratoma, high intensity T1 cristagalli; dermoid extension Dermoids

17. Extra cranial approach; access to midline, skull base, accptable scar, reconstruction of dorsum Best; external Rhinoplasty Transglabellar sub cranial approach, midline vertical approach, lateral rhinotomy. IC; bicoronal extended approach Dermoids

18. Normal nasal development; w 4-12 W5; lateral and medial nasal prominences Arhinia, polyrhinia, Proboscis lateralis, robert’sSyndrom( midline nasal cleft) Errors of Central Midline

19. Very rare, 30 cases Sporadic, or with other malformations With genetic disorders, trisomy 10, 13, 21 No external nose, no airway, maxillary hypoplasia, small high arched palate, hypertelorism Hypernasality? Hyposmia Arhinia

20. Early; cleft feeder, or gastrostomy tube Prosthsis, vertical distraction osteogenesis Split thickness skin graft with long term stenting Restenosis; common, dilation Tissue expander, grafts, DCR, flaps Arhinia

21. Double nose, suplementary nostril, Only 4 cases, anterior ( duplicate septum and passage) and posterior ( choanalatresia) 1st; choana 2nd ;lateral wall anastomosis Excision of suplementary nostril with primary closure Polyrhinia

22. A tube to medial canthus and the same side heminasalaplsia 2nd to fusion of maxillary process CNS and orbital disorders, CT, treament delayed till facial growth completes Restenosis, dilation Proboscis Lateralis

23. Ocular hypertelorism Broad nasal root Lack of nasal tip Tessier classification; 9-14; cranial, 0-8; facial Craniofacial Cleft

24. Median nasal cleft; bifid nose, internasaldisplasia Accompanying median cleft lip, airway is acceptable

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

26. 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 tip Treatment; primary “and” secondary rhinoplasty First attempt; 1st lip surgery, 2nd; strut or shield graft for projection; stage 1 in 4-6 y, stage 2 ; 8-12y, following orthodontic correction Definitive op; 16-18 y Cleft Lip Nasal Deformity

27. 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 anomalies Symptoms like choanalatresia, fail to pass NG CT; width of pyriform, cavity and choana all reduced, but height of cavity and choana normal Congenital Nasal PyriformAppertureStenosis (CNPAS)

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

29. Treatmnet; nonsurgical ( drops and tubes) surgical; sub labial, drilling, 4w stent CNPAS

30. Dacrocystocele; obstruction, epiphora 30% duct obstruction in neonate, but this anomaly is rare Susceptible to aspiration, resolves in 9 months Endoscopy; cystic mass in inferior meatus CT; confirmation, NLD and sac enlarged, infmeatus cyst Op; feeding, infection, obstruction NasoLacrimal Duct Cyst

31. W3-4; placodes appear. W5-6; nasobuccal membrane rupture; fialure; choanalatresia Another theory; abnormal migration of neural crest cells ( Treacher Collins) Errors of NasoBuccal membrane

32. 1/5000-8000, 2/3 unilateral, right sided 50% of all, and 75% of bilateral; anomalies; CHRGE, polydactily, CrouzonSyndrom, nasal, palatal, auricular deformities Problem; narrow cavity, lateral bony obstruction by pterygoidplates, medial obstruction by thickened vomer, membranous obstruction Mixed atresia; 71%, no pure membrane atresia. ChoanalAtresia

33. Bilateral; cyanotic event, Fail to pass 6F catheter, in 32mm distance CT; ddxstenosis and atresia; Stenosis; narrowed but patent choana, space<6mm. Unilateral; postpone surgery for several months Bilateral; OG with orotracheal airway before surgery; try to wait if possible Choanalatresia

34. Operation; tran nasal; 0 hopkins or 120 fiberopticnasopharyngoscope Enter 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 Choanalatresia

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

36. Thanks