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J Thorac Cardiovasc Surg 2004;127:283-285
© 2004 The American Association for Thoracic Surgery

Brief communication

Complex double-outlet right ventricle repair in a neonate with complete tracheal agenesis

Guido Oppido, MDa,*, Carlo Pace Napoleone, MDa, Antonino Loforte, MDa, Simonetta Baroncini, MDb, Mario Lima, MDc, Gaetano Gargiulo, MDa

a Department of Pediatric Cardiac Surgery,, S. Orsola-Malpighi Hospital, University of Bologna Medical School, Bologna, Italy
b Department of Pediatric Anesthesiology,, S. Orsola-Malpighi Hospital, University of Bologna Medical School, Bologna, Italy
c Department of Pediatric Surgery, S. Orsola-Malpighi Hospital, University of Bologna Medical School, Bologna, Italy

Received for publication May 21, 2003; accepted for publication June 18, 2003.

* Address for reprints: Guido Oppido, MD, Department of Pediatric Cardiac Surgery, Ospedale S. Orsola-Malpighi, Via Massarenti, n.9, 40138 Bologna, Italy
guidooppido{at}yahoo.com


Dr Oppido


Tracheal agenesis is a rare malformation with fatal consequences in very early life. We report the case of a baby girl with tracheal agenesis who during early infancy underwent complete repair of her associated {S,D,D} double-outlet right ventricle with supracardiac total anomalous pulmonary venous connection and is still alive at the age of 10 months.

Clinical summary

The patient was born spontaneously at a gestational age of 35 weeks. Birth weight was 2 kg, and Apgar score at 1 minute was 2, increasing to 6 after 2 minutes of mask ventilation. Critical clinical conditions required intubation, but the tube failed to pass the larynx under direct laryngoscopy. A blind end of the larynx was visualized with the aid of a flexible endoscope, whereas the tracheal bifurcation could be visualized from the distal esophagus through a tracheoesophageal fistula. A Portex 3 tube (Portex, Inc, Keene, NH) was positioned through the esophagus into the fistula, achieving a satisfactory bilateral ventilation.

Helical TC scan showed complete agenesis of the trachea (Floyd type II) with a distal tracheoesophageal fistula (Figure 1). Echocardiography showed {S,D,D} double-outlet right ventricle (with subaortic ventricular septal defect), total anomalous pulmonary venous connection to the right superior vena cava, and unrestricted atrial septal defect.



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  		Figure 1. Helical computed tomographic scan showing complete congenital absence of trachea from larynx (white arrow) to bifurcation arising from tracheoesophageal fistula (black arrow). Asterisk indicates esophagus.

 
After 30 days, the patient underwent a complex palliative operation to separate the airway from the alimentary tract, consisting of (1) division of the esophagus below the fistula and gastrostomy (for alimentation), (2) gastroenteroanastomosis (to resolve pylorus and proximal duodenal atresia), (3) Merkel diverticulum resection, and (4) double cervical esophagostomy. The proximal esophagostomy was created to divert salivary secretion protecting the airway; the distal esophagostomy was used as the entrance of the newly created airway, after positioning a 10 by 50-mm Rusch Polyflex esophageal stent (Rusch Inc, Duluth, Ga) to keep the esophagus air patent. A tracheostomy 5.5-mm (external diameter) Shiley cannula (Shiley, Inc, Irvine, Calif) was positioned through the lower esophagostomy into the stented esophagus, connecting the infant to the ventilator (Figure 2).



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  		Figure 2. Three-dimentional reconstruction of helical computed tomographic scan after palliative operation accomplished to separate airway from alimentary tract. Stent keeps esophagus air patent, guaranteeing adequate ventilation. Tracheostomy cannula (black arrow) has been inserted through distal esophagostomy into stented esophagus (asterisk). Esophagus has been divided just below level of tracheoesophageal fistula (white arrow).

 
Because of failure to thrive and severe cyanosis, the infant underwent catheterization, which confirmed the cardiac diagnosis. Complete surgical repair was carried out, consisting of (1) intracardiac left ventricle–aorta pericardial baffling, (2) right superior vena cava division (above the drainage of the four pulmonary veins); (3) intra-atrial pericardial patch baffling of the right superior vena cava orifice (draining all four pulmonary veins) to left atrium through the atrial septal defect, and (4) anastomosis of the proximal right superior vena cava to the right atrial appendage. Cardiopulmonary bypass and cardioplegic cardiac arrest times were 166 and 94 minutes, respectively, and body temperature was lowered to 26°C. The child was taken to the postoperative cardiac intensive care unit and could be weaned from mechanical ventilation at 7 days.

Currently the patient is 10 months old and is waiting for airway and alimentary tract surgical reconstruction. Her hemodynamic status is unremarkable, and she has gained weight, being now in the 50th percentile.

Discussion

Tracheal agenesis, or congenital absence of the trachea, is a very rare malformation accounting for less than 1:50000 live births.1 It was first described in 1900 by Payne.2 Since that time, fewer than 100 cases have been reported in the literature.

According to Floyd and coauthors3 tracheal agenesis is classifiable into three anatomic subtypes: type I, agenesis of the proximal trachea, with a normal distal trachea connected to the esophagus through a tracheoesophageal fistula; type II, complete agenesis of the trachea, with the two main stem bronchi fused in the midline at the carina, which arises from a tracheoesophageal fistula; and type III, complete agenesis of the trachea, with the two main stem bronchi arising separately from the esophagus. Associated congenital malformations and congenital heart disease account for 90% and 70%, respectively, of the cases reported in the literature.4 Some authors refer to tracheal agenesis as part of multimalformation syndromes, such as VA(C)TER(L) (vertebral anomalies, anal atresia, cardiac abnormalities, tracheoesophageal fistula or esophageal atresia, renal agenesis and dysplasia, limb defect) and TACRD (tracheal agenesis or atresia, complex congenital cardiac malformations, radial ray defects, duodenal atresia) syndromes.4,5

Clinical presentation is characterized by rapid onset of severe respiratory distress at birth, absence of audible crying, and impossibility of endotracheal intubation. Survival is due to either ‘incidental’ endoesophageal intubation or prompt surgical tracheostomy (Floyd type I).

Surgical repair or palliation of associated congenital cardiac lesions has been never described in the literature, because most affected patients usually die before any attempt at surgical intervention. This is the first patient to our knowledge to survive palliation of the respiratory tract malformation as well as definitive intracardiac repair. She is now thriving and is currently being evaluated for definitive surgical reconstruction of the airway and alimentary tract.

References

  1. Manschot HJ, van den Anker JN, Tibboel D. Tracheal agenesis. Anaesthesia. 1994;49:788–790[Medline]
  2. Payne WA. Congenital absence of the trachea. Brooklyn Med J. 1900;14:568
  3. Floyd J, Campbell DC, Dominy DE. Agenesis of the trachea. Am Rev Respir Dis. 1962;86:557–560[Medline]
  4. van Veenendaal MB, Liem KD, Marres HA. Congenital absence of the trachea. Eur J Pediatr. 2000;159:8–13[Medline]
  5. Evans JA, Reggin J, Greenberg C. Tracheal agenesis and associated malformations: a comparison with tracheoesophageal fistula and the VACTERL association. Am J Med Genet. 1985;21:21–34[Medline]




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