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WHAT IS LARYNGEAL ATRESIA?

        WHAT IS LARYNGEAL ATRESIA?                    
 Laryngeal  Atresia is a rare birth defect, result from recanalization of the larynx during the 10th week of development, which produce obstruction(blocking) of the upper fetal airway
This disease is also known as congenital high airway obstruction syndrome.
Distal to the region of atresia or stenosis , the fetal airways become dilated , and the lungs are enlarged and filled with fluid.
The diaphragm is either flattened or inverted, and there is an accumulation of serous fluid in the peritoneal cavity in the intracellular spaces, causing severe edema.
High Risk
CHAOS syndrome is very often fatal due to fetal heart failure due to blockage of airway obstruction.
In some severe cases , postpartum airway intervention (tracheostomy) may lead to survival.
WHAT ARE THE TYPES OF ESOPHAGEAL ATRESIA?
Esophageal atresia with a tracheoesophageal fistula(IS AN ABNORMAL CONNECTION BETWEEN TWO ORGANS) at the distal one-third end of the trachea .
Trachea is an air passageway
occurrence:
 This is the most common type, occurring in 82% of cases. The AP radiograph of this malformation shows an enteric tube (arrow) coiled in the upper esophageal pouch. The air in the bowel indicates a distal tracheoesophageal fistula.
H-TYPE TRACHEOESOPHAGEAL FISTULA
H-type tracheoesophageal fistula only . This malformation occurs in 6% of cases. The barium swallow radiograph  shows a normal esophagus (E), but dye has spilled into the trachea (T) through the fistula and outlines the upper trachea and larynx.
DETAILED DISCUSSION
Postnatal management of affected infants is difficult. In many cases, even when an EXIT procedure has been preformed, the prognosis, in terms of survival and ventilation ability, may be poor, particularly if laryngeal reconstruction is not possible3,8,9. The low rate of long-term survival and severe mental and growth retardation often prevent surgery,1,9,11 as the patient fails to reach an age or appropriate clinical condition at which surgical correction is possible; even anatomic characteristics of the airway defect may be such that surgical correction is not feasible. Due to adequate neonatal management in our case, the infant was discharged with no need for ventilatory support, and with normal neurodevelopment. Surgical correction could therefore be performed at the age of 2 years. Several methods of tracheal replacement have been proposed, including the use of allografts, prosthetic materials, autologous tissue or a combination of these materials8. In our case, autologous costal cartilaginous tissue was used successfully9. The proper timing of laryngeal reconstruction is still debated and long-term prognosis in terms of ventilatory, feeding and speech functions is unclear. A rapid postoperative complete recovery of ventilatory and feeding functions was observed in our case. However, the baby still shows moderate impairment in language functions. Despite the positive outcome in the case described, further studies on larger groups of infants affected by CHAOS are needed to better define proper management and long-term outcome of these infants.
INCOMPLETE ATRESIA OR LARYNGEAL WEB
Laryngeal Web, is a defect in which the connective between the vocal folds is covered with a mucous membrane: this causes airway obstruction and a hoarse cry in the neonate.
Treatment
This defect is treated by endoscopic dilation of the laryngeal web.
DEVELOPMENT OF LARYNX
The opening of the respiratory diverticulum into the foregut becomes the laryngeal orifice. The laryngeal epithelium and glands are derived from endoderm. The laryngeal muscles are derived from somitomeric mesoderm of pharyngeal arches 4 and 6 and therefore are innervated by branches of the vagus nerve CN X; i.e., the superior laryngeal nerve and recurrent laryngeal nerve, respectively. The laryngeal cartilages (thyroid, cricoid, arytenoid, corniculate, and cuneiform) are derived from somitomeric mesoderm of pharyngeal arches 4 and 6.
Reference book
BRS EMBRYOLOGY
CLINICALLY ORIENTED EMBRYOLOGY BY KEITH L MOORE

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