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SYNCHRONOUS CONGENITAL NASAL PIRIFORM APERTURE STENOSIS AND ATRESIA : A RARE ACCOMPANIMENT OF MID-FACIAL DYSTOSIS AND CAUSE OF NEONATAL UPPER AIRWAY OBSTRUCTION. A CASE REPORT John EN1,Mbam TT2, Nimkur LT3, Fasunla AJ4 Correspondence: [email protected] 1. Senior registrar, department of ORL, Jos University Teaching Hospital, Jos, Nigeria 2. Senior registrar, department of ORL, University college Hospital, Ibadan, Nigeria 3. Consultant ORL/HN Surgeon, Jos University Teaching Hospital, Jos, Nigeria 4. Consultant ORL/HN Surgeon, University college Hospital, Ibadan, Nigeria ABSTRACT Congenital nasal piriform aperture stenosis (CNPAS) is a recently distinguished clinical entity that causes airway compromise in neonate as a result of a narrowing of the nasal piriform aperture. It may be bilateral or unilateral. Often considered as a form of holoprosencaphaly. Being the narrowest part of the nasal airway, any slight reduction in its cross sectional area will profoundly increase the nasal airway resistance with the attendant clinical challenges. Readily making an accurate clinical diagnosis may be very challenging when CNPAS occurs in the setting of a craniofacial dysostosis. This communiqué is aimed at increasing awareness among clinicians involved in paediatric care of the clinical entity congenital nasal piriform aperture stenosis and atresia presenting atypically as synchronous disorder with midfacial dysostosis. Hence, it should be kept in mind as a rare mimicker of bilateral choanal atresia and requires mandatory high resolution computerized tomography to confirm the diagnosis. Keywords: Congenital nasal piriform aperture stenosis, atresia, craniofacial dysostosis, neonate INTRODUCTION The first radiological report of congenital nasal episodic or cyclical cyanotic spells, feeding piriform aperture (CNPAS) was in 1988 by difficulty which may subsequently result in poor 6 Chinwuba and Wallman1,while, the first clinical weight gain and failure to thrive . Presentation may description of CNPAS was 19892. be soon after delivery or many weeks after birth. Respiratory distress is often relieved on crying, Neonatal nasal obstruction due to CNPAS is very thereby, making this conditions a true mimicker of rare and uncommon2,3,4,. Estimated to occur at a choanal atresia5. The breathing problem may be frequency of between one fifth to one third that of triggered by episodes of recurrent upper respiratory choanal atresia5. It is a potentially lethal form of tract infections5. Provisional diagnosis is suggested airway obstruction in newborns6. Since, newborns by history and physical examination; however, are obligatory nasal breathers, immediate imaging is essential to confirm CNPAS2. It has been recognition and appropriate therapy is mandatory suggested that a total nasal piriform aperture width to avoid untoward consequences7. This anomaly less than 8mm in a term infant is diagnostic of may present as an isolated malformation or may be CNPAS2. associated with other craniofacial anomalies including central nervous system and endocrine Surgery is indicated in cases of severe respiratory abnormalities5. CNPAS has been considered by distress, feeding difficulties, and when there is no 3 some authors as a form of holoprosencephaly8. The benefit with conservative treatment . Surgical piriform aperture (bony inlet) is the narrowest correction done via sub-labial approach followed by portion of the normal nasal passage, therefore, any nasal stenting has proven to be most effective further reduction in the cross sectional area results surgical treatment with very low risk of re-stenosis 6 in an overt increase in nasal airway resistance9. during the long term follow-up period . Clinically, CNPAS shows non-specific symptoms The midfacial dysostosis encountered in the index of nasal airway obstruction such as apnoeic crisis, case report is Crouzon's syndrome. Crouzon Jos Journal of Medicine, Volume 6 No. 3 37 Synchronous Congenital Nasal Piriform Aperture Stenosis and Atresia : A Rare Accompaniment of Mid-facial Dystosis.............. syndrome is a hereditary syndrome of craniofacial revealed an obviously mouth breathing male child dysostosis and it is an autosomal dorminant with dysmorphic craniofacial features i.e. bilateral disorder with complete penetrance and variable buphthalmos , marked hypertelorism, beaked nose, expressivity10. Characteristically patients have hypoplastic maxilla and almost completely closed premature closure of the calvaria and cranial base anterior and posterior fontanelles. Patient was also sutures as well as bones of the orbit and maxillary having intermintent cyanotic spells. Attempts at complex (craniosynostosis). Other clinical features passing a well lubricated size 5 G feeding tube via include hypertelorism, exorbitism, strabismus, both nasal cavities was not successful. However, beaked nose, short upper lip, hypoplastic maxilla scanty nasal air current was demonstrable from the and relative mandibular prognathism10. Prevalence left nasal cavity after suctioning of mucoid of this condition is 1 case per 60,000 live births and secretion. No other obvious structural defect responsible for approximately 4.8% of all cases of detected in the ear, external genitally or limbs. craniosynostosis11. There is no racial or sex Admission weight and PCV were 3kg and 30% predilection. The unique anatomy of the skull respectively.Patient was transferred to the special base,facial skeleton and nasopharynx in these babby care unit for proper monitoring and nursing newborns with craniofacial dysostosis predispose care. them to having severely narrowed nasal and nasopharyngeal tract hence making them high risk Breast feeding and all oral feeds were stopped and group for development of obstructive sleep apnoea patient commenced on intravenous fluid regimen. and nasal airway obstruction .The nasal symptoms Patient was nursed in propped up position and of craniofacial dysostosis may therefore mask an connected to humidified oxygen delivered via otherwise obvious choanal atresia or CNPAS appropriate size oropharyngeal airway anchored to during assessment by the unsuspecting physician. the lips and buccal commissures. This case report is aimed at increase the awareness Given the severe financial constraint of the parents, of clinicians involved in paediatric care on the the hospital indigent fund was access to do an clinical entity congenital nasal piriform aperture urgent computerized tomography (CT) scan of the stenosis and atresia which is though rare but may paranasal sinuses was done. An urgent abdominal occur concurrently and misdiagnosed as choanal ultrasound scan, echocardiography and otoacoustic atresia. emission were also done with no significant Case Report abnormal findings. However, axial projections of Patient is a 5 weeks old male, first child of a non- CT done revealed hyperdense shadow in the region sanguinous parents. Patient was delivered at term of the both right and left maxillary nasal processes via spontaneous vaginal route by traditional birth worse on the right. Complete Atresia of the right attendant (TBA). Index pregnancy was neither nasal piriform aperture also noted. There is also booked nor supervised in the hospital. However, severe narrowing of the left nasal piriform aperture, pregnancy was said to be uneventful. No history of width measures about 1mm at the level of inferior ingestion of any identifiable teratogenic drugs and turbinate. Overall, features are in keeping with no family history of congenital anomaly. Apgar synchronous congenital nasal piriform aperture score and birth weight were not known, however, stenosis and atresia. baby cried immediately after birth. Soon after birth, Parents of the child were educated on the disease he continued to have noisy breathing, recurrent condition and re-assured. Non-operative copious mucoid left nasal discharge, apnoeic management and patient followed up closely at the spells, mouth breathing and refusal to suck from the outpatient clinic with consideration of surgical breast. Childhood immunization was delayed due correction later if symptoms persist. Please see to the unremitting symptoms mentioned above. figure 1,2,3 and 4 below for pictures and images Upon the advice of the TBA, mother presented with Discussion patient at the emergency paediatric unit of the Neonatal nasal obstruction can present at birth as University college hospital, Ibadan. Patient was severe birth asphyxia. It is a potentially life assessed by both the paediatricians on duty and threatening condition unless it is recognized early otorhinolaryngological team. Examination and treated, it may lead to profound respiratory distress in neonates because they are obligate nasal Jos Journal of Medicine, Volume 6 No. 3 38 Synchronous Congenital Nasal Piriform Aperture Stenosis and Atresia : A Rare Accompaniment of Mid-facial Dystosis.............. breathers. When complete bilateral nasal and atresia in the other. obstruction is missed , it can lead to apnoeic crisis and repeated cyanotic episodes. The nasal Phenotypically, there are 2 forms of CNPAS, pathology in the index case was missed at birth by namely, an isolated form and another variant that is the TBA, hence, the similar clinical presentation. associated with other another anomalies including a The differential diagnosis of neonatal nasal midface dystostosis, endocrine and CNS 17 obstruction includes skull base defects abnormalities . CNPAS with single central (meningoencephalocoele and encephalocoele), maxillary mega-incissor

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