respiratory distress in a neonate is more commonly caused by pulmonary , cardiac , or neurological etiologies .
the obstructive lesions of the upper airway are rare , unsuspected , and potentially treatable causes of neonatal respiratory distress .
an oropharyngeal mass presenting as a cause of neonatal respiratory distress is rare and may include bronchogenic cyst , epidermoid cyst , congenital cysts of third and fourth pharyngeal pouches , and the least common basal meningoencephalocele .
they originate from a congenital hiatus in the midline region of the skull base , which permits meninges , neural tissue , or both to herniate from the intracranial space .
basal encephaloceles occur with an estimated incidence of 1 in 35,000 live births and have been further subdivided , depending on the location of the bone defect , into transethmoidal , sphenomaxillary , sphenoorbital , and transsphenoidal .
our patient had presented with respiratory distress and an oropharyngeal mass in the neonatal period and was eventually diagnosed to have transalar transsphenoidal meningoencephalocele .
a 30-day - old male infant weighing 2.6 kg presented with difficulty in breathing and feeding noticed since 4 day of life .
the infant was observed to have tachypnea with a respiratory rate of 70 breaths per minute along with marked suprasternal retractions and stridor , though cyanosis was absent .
arterial blood gas analysis revealed normal oxygenation , and chest x - ray did not show any significant abnormality .
the patency of all natural orifices was checked , and choanal atresia , tracheo - esophageal fistula , and anorectal malformation were ruled out .
examination of the oropharyngeal cavity showed presence of a cystic swelling protruding through the soft palate .
aspiration of the cyst with a 26-gauze needle produced a small quantity of clear fluid with no cells and a protein content of 80 mg% which was compatible with a transudate .
subsequently , after 48 hours of aspiration , the child had recurrence of symptoms and reappearance of the swelling .
the computed tomographic ( ct ) scan with bone windows revealed a large defect in the sphenoidal base with herniating meningoencephalocele [ figure 1 ] .
a transsphenoidal encephalocele was diagnosed on the magnetic resonance imaging ( mri ) [ figure 2 ] .
the patient was successfully operated on 40 day of life using the transpalatal approach involving a multidisciplinary team comprising neurosurgeons and otolaryngologists .
ct scan shows defect in sphenoid with herniating meningoencephalocele mri showing trans - sphenoidal encephalocele during follow - up on 70 day of life , the child is stable , accepting breast feeds , and neurologically normal .
obstructive lesions of the newborn airway include choanal atresia , macroglossia , pierre - robin syndrome , lymphangioma , teratoma or other mediastinal masses , cysts , subglottic stenosis , and laryngotracheomalacia .
an oropharyngeal mass presenting as a cause of neonatal respiratory distress is rare and may include bronchogenic cyst , epidermoid cyst , congenital cysts of third and fourth pharyngeal pouches , and the least common basal meningoencephalocele .
there are only two case reports in the literature of a newborn presenting with respiratory distress caused by basal transsphenoidal meningoencephalocele .
symptoms as a result of a basal encephalocele vary according to the site and size of the lesion .
consequently , respiratory distress , episodes of apnea , difficulty with feeding , and failure to thrive can be seen .
severe respiratory distress and feeding difficulty as seen in our patient were attributable to pharyngeal airway obstruction .
only 11 pediatric cases of transalar transsphenoidal encephaloceles have been reported in the literature , with two of them presenting in neonatal period with respiratory distress [ table 1 ] .
he was observed to have a cystic mass protruding through the soft palate which was diagnosed on neuroimaging as transalar transsphenoidal meningoencephalocele .
pediatric case series of transalar transsphenoidal encephaloceles the majority of transsphenoidal meningoencephaloceles are diagnosed during the first year of life due to manifestations such as respiratory distress caused by epipharyngeal obstruction , feeding difficulties , cranial midline defects with cleft lip or cleft palate , hypertelorism , optic malformations with anophthalmia , retinal abnormalities , optic nerve hypoplasia , unexplained bouts of recurrent meningitis , or endocrine abnormalities .
associated congenital anomalies have been noted in one third of the cases of sphenoidal encephalocele .
however , if there are no considerable difficulties and no distinctive facial anomalies during childhood , the diagnosis of the disease may be delayed up to adulthood , when distinctive symptoms such as rhinorrhea , visual defect , or endocrine dysfunction occur .
advanced imaging studies are necessary to confirm the diagnosis of transsphenoidal encephalocele and to define any neural or vascular elements that may be included in the herniation .
ct scan and mri are the most useful modalities for diagnosing meningoencephalocele . in the present case , ct scan including 3d reconstruction allowed visualization of bone defects in the skull base and a well - circumscribed expansile mass lesion in the extracranial area communicating with the intracranial space .
mri with gadolinium enhancement evaluated the content of the encephalocele and eliminated other brain anomalies .
the contents of the sac need to be preserved as the sac invariably contains vital structures .
transsphenoidal encephalocele is frequently accompanied by a split palate , and these conditions can be operated upon at the same sitting via the transpalatal approach . there is also less risk of damaging the functioning tissues within the wall of the encephalocele in transpalatal surgical approach . in conclusion , this case is unique because it concerns an infant with severe respiratory problems from the time of birth as a result of a transsphenoidal encephalocele presenting as an oropharyngeal cystic swelling .
obstructive causes of respiratory distress in neonatal period are rare and pose a diagnostic challenge .
a detailed oropharyngeal examination should be considered in a neonate with respiratory distress with no apparent cardiac or respiratory cause .
it is important to consider early neuroimaging for diagnosing these conditions and plan definitive treatment for successful outcome .
 respiratory distress in an infant is a common cause of admission in neonatal intensive care facility . 
 obstructive lesions of the airway constitute a minority of problems in the new born but present a diagnostic challenge . 
 we present a 30-day - old male infant admitted with respiratory distress who was diagnosed to have an oropharyngeal cystic mass which on further evaluation by computed tomography and magnetic resonance imaging revealed a transalar transsphenoidal meningoencephalocele herniating into the oral cavity through a congenital split palate . 
 the patient was operated successfully using a transpalatal approach leading to complete resolution of respiratory distress . 
Introduction
Case Report
Discussion