Journal List > Perinatology > v.29(3) > 1102888

Do, Oh, and Park: Successful Treatment of an Unexpected Neonatal Case of Tracheal Agenesis

Abstract

Tracheal agenesis is an extremely rare and typically fatal congenital anomaly, with only scattered case reports attesting to of its successful management. This condition usually presents with severe respiratory distress and aphonia after birth. Failed attempts at intubation make neo natal resuscitation difficult. This condition appears to be under-recognized, and there is a lack of con sensus regarding the optimum approach for managing this lethal condition. We report herein a rare case of tracheal agenesis and describe our experience, following its successful treatment through surgical management.

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Fig. 1
Initial infantogram (A) and infantogram after tracheostomy (B). (A) Infantogram shows quite good air content in both lungs, but with fine granular appearance. (B) The lungs have increased air content after tracheostomy.
pn-29-142f1.tif
Fig. 2
Pharyngeal non-contrast computed tomography showed no visualization of trachea. (A) At level of C3 vertebral body, (B) at level of C5 vertebral body, (C) at level of C7 vertebral body, tra cheostomy tube insertion site. White arrow indicates esophagus and black arrow indicates tra cheostomy tube.
pn-29-142f2.tif
Fig. 3
(A) Pharynx 3 dimension computed tomography showed no visualization of proximal trachea: white arrow indicates esophagus and black arrow indicates distal trachea. (B) Pharyngeal non-contrast computed tomography showed no visualization of trachea at level of C5 vertebral body: thin black arrow indicates C5 vertebral body and thin white arrow indicates esophagus and no visualization proximal trachea.
pn-29-142f3.tif
Fig. 4
Floyd's classification of tracheal agenesis by Floyd et al.16 Three types of tracheal agenesis. Type I: atresia of the proximal trachea with presence of the distal trachea and carina and two completely formed bronchi. Type II: the bronchi join in the midline and communicate with the esophagus as a common fistula. Type III: independent communication between the bronchi and esophagus.
pn-29-142f4.tif
Table 1.
Comparison: Five Cases of Tracheal Agenesis Reported in Korea
Case 1 2 3 4 5
Gestational age (weeks) 34+3 38+0 32+0 34+0 38+5
Birth weight (g) 2,030 2,250 1,560 2,000 2,600
Apgar score (at 1 minute/5 minutes) 2/2 2/4 5/2 1/2 5/3
Polyhydramnios + Unknown + Unknown +
Accompanied malformation VSD, PDA, Rt. lung lobulation defect Dextrocardia VSD, Rt. lung lobulation defect, Meckel's diverticulum Rt. lung lobulation defect, SUA Lung lobulation defect, IA, Rectovesical fistula
Esophageal intubation + + + + +
Type of tracheal agenesis II II II I II
Age of death 4 days 1 day 4 hours 3 hours 6 hours

Abbreviations: VSD, ventricular septum defect; PDA, patent ductus arteriosus; Rt., right; SUA, single umbilical artery; IA, imperforate anus.

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