assignx_cong diseaseses_nose&pns_mashrab.pptx

1. Alexandria University
Department of Otorhinolaryngology
PRESENTATION ON:
CONGENITAL DISEASES OF THE NOSE AND PNS
PREPARED BY: MASHRAB MUHDHAR OMAR (VISITING ENT RESIDENT)
SUPERVISOR: PROF. DR. AHMED ALY IBRAHIM ELSAYED (PROF. OF ENT)

2. Introduction
The nose is formed by the fusion of 4
embryonic processes at 4th gestation week,
and most congenital anomalies occur due
to failure of the fusion such as:
 Proboscis lateralis, cleft/bifid nose
 Arrhinia, half-nose, polyrhinia
 Supernumerary nostril
 Dermoid cyst, gliomas,
meningoencephaloceles
 Klestadts (naso-labial/-alveolar) cyst)
 May be associate with syndromes e.g.,
CHARGE syndrome

4. Proboscis lateralis Polyrhinia Arrhinia Half nose
Nasal cleft Cong. Midline nasal sinus
Bifid nose (nasal cleft) Supernumerary nostril

6. Congenital Choanal Atresia
 It is the failure of communication of the nasal
cavity with the nasopharynx due to a
persistent, embryonic bucco-/oro-nasal
membrane which was supposed to disappear
at 7th gestation week.
 +Hx/o methimazole/carbimazole use during
pregnancy
 Types: Bony (90%), Membranous or Mixed
 Degree of atresia maybe
• Incomplete unilateral/bilateral
• Complete unilateral/bilateral
 Unilateral:bilateral=2:1

7. Symptoms of Choanal Choanal Atresia
Symptoms for bilateral atresia
 In complete bilateral atresia, a
newborn who is an obligatory
nasal breather develops
asphyxia and may die if
emergency intervention is not
given.
 In incomplete bilateral atresia,
patients present with cyclic or
suckling cyanosis.
 Later symptoms: mouth
breathing (malocclusion),
increased nasal discharge,
failure to develop smell/taste

8.  Unilateral atresia, its usually
diagnosed in late childhood.
The child presents with
unilateral nasal obstruction and
discharge which cannot to be
expelled by blowing the nose.
Symptoms of Congenital
Choanal Atresia

9. Management of
Congenital Choanal
Atresia
 Diagnosis may be by mirror test, catheter test,
Argyrol ephedrine (colored) drops test,
endoscopy or by CT.
 Emergency airway establishment is done by
endotracheal intubation (+mcgovern nipple) or
tracheostomy in cases of acute asphyxia
 Endoscopic/transpalatal repair is then done and
nasal stents placed for 4-6 weeks.
 Unilateral atresia can be repaired after 2-3
years.

10. Dermoid cyst
 Is a midline mature teratoma (benign tumor)
mass consisting of a mixture of ectordemal,
endodermal and mesodermal contents.
 May have a blind-end tract (sinus) or a fistula
opening on the skin
 It accounts for 12% of all H&N dermoids, and
45% case may have intracranial extension.
 It may be complicated with infection, abscess
formation, osteomyelitis, meningitis, brain
abscess
 CT is required to exclude intracranial
extensions, before treatment by excision.

11. Glioma
 Is a mass of glial (brain) tissue.
 Site may be extranasal or intranasal
 It is firm, noncompressible, non-
pulsatile and does NOT enlarge on
crying/straining (negative furstenberg
test) OR transilluminate.
 Histopathology shows glial cell in
connective tissue matrix
 Contrast CT or MRI to evaluate any
intrancranial extension
 Treatment is by surgical incision

12. Meningoencephalocele
 Herniation of brain tissue with or
without meninges, through a skull defect
 It is pulsatile, enlarges with
crying/straining (+furstenberg test) and
transilluminates
 CT/MRI is confirmatory, treatment is by
excision
 Other possible lesions such as
meningoceles, hydroencephalo-
meningoceles can occur

15. Thank you!!!