A new bom child presented with CHD has cyanosis, become prominanat on breathing and improved in crying. The Diagnosis is
i.e. (Bilateral choanal atresia) : (336-Ghai 7th) (178-458-Dhingra 5th)* Affected baby cycles between spells of cyanosis and crying. Attempts at suckling immediately precipitates cyanosis in CHOANAL ATRESIACHOANAL ATRESIA* More often unilateral. More common in female (2:1), more often on the right side and more often bony than membranous (9:1)* Due to persistance of bucconasal membrane* Bilateral choanal atresia usually presents immediately after birth with respiratory distress which is due to the fact that neonates are obligate nose-breathersSometimes babies bom with choanal atresia also have other abnormalities:ColobomaHeart defectsMental retardationGrowth impairmentOthers (see also CHARGE syndrome)Also any condition that causes significant depression of the nasal bridge or midface retraction can be associated with choanal atresia. Examples include the craniosynostosis syndrome such as Crouzon syndrome, and Antley- Bixler syndrome.* Diagnosis(i) Presence of mucoid discharge in the nose(ii) Absence of air bubbles in the nasal discharge(iii) Failure to pass a catheter from nose to pharynx(iv) Putting a few drops of a dye (methylene blue) in to the nose and seeing its passage into the pharynx(v) Installing - radio - opaque dye into the nose and taking a lateral film* Bilateral choanal atresia requires - Urgent management by inserting a finger in the baby's mouth: this can be replaced by with a plastic oropharyngeal airway or a Me Govern nipple - Failure - Endotrachial tube or tracheostomy* Bilateral atresia can be associated with other syndrome head and neck abnormalities eg CHARGE association (Coloboma, hearing impairment, choanal atresia, mental retardation, genital abnormality, endocardial cushion defects) meningo-myelocoeles, craniosynostosis etc.* Most patient with CHARGE syndrome have mutation in CHD7 gene. Mitomycin C has been used to present the development of granulation tissue & stenosis (1743-Nelson 18th)Diaphragmatic hernia - occurs because offailure of closure of the pleuroperitoneal membrane - at birth presented with respiratory distress and a scaphoid abdomen and mediastinal shift
Bilateral choanal atresia
Diaphragmatic hernia
Genitourinary defects
Coloboma
C.V.S.