تشخیص

}  Choanal atresia

}  Most common congenital anomaly of the nose

}  1/7000 live birth

}  Unilateral or bilateral

}  Bony(90%) or membranous(10%) septum between nose and pharynx

}  Most cases :combination of bony and membranous

}  20-50% with other congenital anomaly(more frequently in bilateral form)

}  CHARGE association(Coloboma ,Heart disease ,Atresia choanal ,Retarded growth and development or CNS anomaly ,Genital anomaly or hypogonadism ,Ear anomaly or deafness

}  Clinical manifestation of choanal atresia:

}  Variable ability to breathe in neonates through month

}  Unilateral form: asymptomatic for a long period by a nasal discharge or persistent obstruction

}  Bilateral form: symptomatic in neonatal period

}  Diagnosis of Choanal atresia

}  Inability to pass a firm catheter through each nostril 3-4 cm into the nasopharyinx

}  Fiberoptic rhinoscopy

}  High resolution CTS

}  Differential diagnosis:

}  Congenital defect in nasal septum

}  Stenosis of pyriform apertus

}  Congenital midline nasal mass:dermoid.gliomas,encephalocele,hemangiomas,congenital nasolacrimal duct obstruction.nasal polyp,tumor(rhabdomyosarcoma)

}  Treatment of choanal atresia

}  Prompt placement of oral airway

}  Feeding by gavage

}  Intubation or tracheostomy

}  Trans nasal repair(stent for weeks) in 4-6wk or body weight of 4 kg

}  Unilateral atresia:infancy(4-5yr)

}  Case presentation

}  A 1800 g preterm (36 weeks) male was born to a G3 mother by NVD. The baby cried immediately after birth and there was no perinatal asphyxia. Soon after birth the baby developed respiratory distress with mild cyanosis. Attempts to pass a nasogastric tube through the nose were un-successful. Esophageal atresia was ruled out by passing an oro-gastric tube. A provisional diagnosis of bilateral choanal atresia was made.

}  Routine laboratory investigations were normal. The chest X-ray and X-ray naso-pharyngeal region lateral view did not show any abnormality. Ultrasound cranium was normal. A CT scan of the paranasal sinus region and skull showed a bony defect in the floor of anterior cranial fossa in the region of the cribriform plate with brain parenchyma extend-ing into the nasoethmoidal region (Fig.). These findings suggested the diagnosis of trans-ethmoidal encephalocele as the cause of nasal obstruction.

}  bony defect in cribriform plate and protruding brain tissue (arrow).

}  Risk factors

}  Race

}  Encephaloceles have a multifactorial etiology, and genetic and geographic factors have been implicated. Frontal encephaloceles are far more common in the Far East, particularly in the Chinese population, and are associated with a more favorable prognosis.

}  Sex

}  Encephaloceles occur more commonly in females than in males.

}  Age

}  Currently, most encephaloceles are diagnosed prenatally and present at birth.^,  Some, particularly sphenoidal encephaloceles, may become apparent later in childhood.

}  encephaloceles

}  Occipital in ¾ of cases

}  Frontal (protrusion into the nasal cavity)

}  Rarely :temporal or parietal

}  Anterior encephalocele :infrequent (15–20% of the cranial encephaloceles)

}  1in 35,000 live births

}  the incidence is much higher in Southeast Asian countries, including some parts of India(it is as high as 1:5,000 live births in Thailand.)

}  Transethmoidal encephaloceles (TEE), a subtype of basal anterior encephalo-cele, is the protrusion of a part of brain tissue through a bony defect in the cribriform plate into the anterosuperior nasal cavity.

}  Types of Basal anterior encephaloceles

}  depending on the site of the bony defect :

}  Sphenoethmoidal

}  , trans-ethmoidal,

}   transphenoidal

}  spheno-orbital.

}  Naso-frontal, naso-ethmoidal and naso-orbital or combination of these.

}  Frontoethmoidal encephaloceles are the commonest type,

}  Frontoethmoidal encephaloceles :commonest type, followed by the nasopharyngeal and orbital type.

}  Among the frontoethmoidal encephaloceles, nasoethmoid is the commonest type, (swelling over the bridge of the nose with significant hypertelorism and orbital deformities)

}  The nasopharyngeal type remains occult and presents with nasal obstruction or CSF rhinorrhea. Rarely, the patient may present with meningitis.

}  There was associated hydrocephalus and agenesis of the corpus callosum

}  Clinical manifestation

}  signs and symptoms of nasal obstruction mimicking choanal atresia

}  , hyperteleorism,

}   CSF leakage

}   recurrent meningitis

}   orbital deformities

}  hydrocephalus

}  The aware-ness of this lesion in differential diagnosis of choanal atresia is of practical importance because failure to recognize this lesion can lead to surgical misadventure. The advent of CT scanning and MRI has made the diagnosis simple. These scans help to delineate the extent and characteristics of the anomaly.

}  The aim of surgery is to excise the encephalocele, repair the dural defect and correct the facial and orbital deformity. The ideal age for surgery is around 6-9 months. Early surgery leads to better cosmetic results and prevents CSF leak.