Tracheal Agenesis Reconstruction with External Esophageal Stenting: Postoperative Results and Complications

Tracheal agenesis is an extremely rare and typically lethal congenital disorder. Approximately 150 cases have been described since 1900, and very few cases of survival have been reported. We describe tracheal reconstruction with external esophageal stenting in a patient with Floyd's type II tracheal agenesis. Neither long-term survival nor survival without mechanical ventilation for even a single day has previously been reported in patients with Floyd's type II tracheal agenesis. The infant in the present case survived for almost a year and breathed without a ventilator for approximately 50 days after airway reconstruction using external supportive stents.

Agenesia Traqueal. Anomalía congénita rara con alta mortalidad / Tracheal Agenesis. a Rare Congenital Anomaly with High-Mortality

Introducción: La agenesia traqueal es una malformación congénita muy rara, incompatible con la vida, el defecto consiste en una ausencia parcial o total de la tráquea debajo de la laringe permitiendo que el tracto respiratorio inferior se conecte con el tracto gastrointestinal por medio de una fístula traqueó-esofágica distal. Caso Clínico: Se presenta el caso clínico de un recién nacido, con antecedente materno de polihidramnios, nace con dificultad respiratoria, ausencia de llanto y dificultad para la intubación orotraqueal, Apgar 4/8, pasa a Unidad de Cuidados Intensivo neonatales (UCIN) y es conectado a Asistencia Respiratoria Mecánica (ARM) a las 6hs de vida se extuba en forma accidental, con imposibilidad para la reintubación, se realizan Tomografía de cuello y tórax donde se constata agenesia traqueal con fistula traqueoesofágica distal, sin otras malformaciones congénitas asociadas, se realizan varias cirugías paliativas. A pesar de todos los esfuerzos médicos, el paciente fallece a los 6 días de vida, ante colapso de vías aéreas. Discusión: Se presenta este caso por ser la agenesia traqueal una malformación extremadamente rara, y con muy pocos casos descriptos en la literatura. Es el primer caso en nuestro hospital, se presenta como un caso clínico complejo, de manejo multidisciplinario, sometido a varias cirugías paliativas, el paciente fallece, la evolución es generalmente fatal y de mal pronóstico debido a que aún no se han desarrollado técnicas adecuadas de reemplazo traqueal .

Introduction: Tracheal a genesis is a very rare congenital malformation that is incompatible with life.The anomaly consists of the partial or total absence of the trache a below the larynx that allows connection of the lower respiratory...

Agenesia traqueal: revisão de literatura e relato de caso / Tracheal agenesis: literature review and case report

Agenesia traqueal (AT) é uma anomalia congênita muito rara e quase sempre fatal, que tem associação com prematuridade e polidrâmnio. Pode ser classificada, de acordo com Floydeem, em três categorias, sendo esta a mais usada e a qual é universalmente aceita. Relatamos o caso de um recém-nascido (RN) do sexo feminino, pré-termo, que apresentava diagnóstico pré-natal, através de ultrassonografia, de malformação cardíaca, hemivértebra, atresia de esôfago e polidramnia. O mesmo nasceu deprimido e evoluiu para o óbito. No laudo da patologia foram evidenciados anormalidades na anatomia traqueal. Realizamos revisão bibliográfica a respeito da agenesia traqueal, suas complicações e possíveis tratamentos...

Tracheal agenesis with broncho-esophageal fistula in VACTERL / TACRD association.

Tracheal agenesis (TA) is an extremely rare malformation. We report here autopsy findings in a case of TA with bronchoesophageal fistula of Floyd type III. The other malformations present included laryngeal atresia, Right lung hypolobulation, ventricular septal defect in membranous portion, bilateral cystic renal dysplasia, spleninculus, Meckel's diverticulum, and imperforate anus. The constellations of malformations present in our case have overlapping features with Vertebral anomalies, Anal atresia, Cardiovascular anomalies, Tracheo-esophageal fistula, Esophageal atresia, Renal anomalies, Limb anomalies and Tracheal atresia or laryngo tracheal atresia, Cardiac anomalies, Renal anomalies, Duodenal atresia association described previously in the literature.

A Case of Tracheal Agenesis with Tracheoesphageal Fistula / 대한주산의학회잡지

Tracheal agenesis with bronchoesophageal fistula should be suspected in any newborn who is cyanotic at birth, has an absent cry, and cannot be intubated but shows some improvement when ventilated by bag and mask. It is important to make the diagnosis as soon as possible because esophageal intubation or a distal tracheostomy may be the only method to provide an airway while other measures and/or diagnostic confirmation are being sought. We experienced a case of complete tracheal agenesis with carinoesophageal fistula, cloacal anomalies in a male neonate.

Tracheal Agenesis: A case report / 대한마취과학회지

Agenesis of trachea is a rare congenital anomaly. The main signs are respiratory distress, cyanosis, inability to vocalise and impossible tracheal intubation. There is no long-term surgical solution at present, therefore the condition is ultimately fatal. We report a case of tracheal agenesis. We anesthetized a 2.25 kg neonate for endoscopic examination, who was intubated successfully. Beyond 2.5 cm from the vocal cord, there was no trachea and we can only see esophageal lumen with fistula opening. After the endoscopic examination, exploratory thoracotomy was done, but surgical correction was impossible. After the surgery, she died from progressive ventilatory failure. Autopsy revealed a Floyd's type II tracheal agenesis with tracheo-esophageal fistula.

An Autopsy Case of Tracheal Agenesis Type 2

Tracheal agenesis is the rare and uniformly lethal anomaly that presents with severe respiratory distress and aphonia after birth. In this anomaly, the trachea is usually absent and air is reaching the bronchi through a communication with the esophagus. The diagnosis should be suspected in a nowborn infant with respiratory distress whose intubation is difficult. We report an autopy case of tracheal agenesis, type 2 in a female newborn infant: Complete tracheal agenesis with the fistula between the esophagus and the carina. Associated anomalies were bilobed right lung, Meckel's divericulum and ventricular septal defect.

Tracheal Agenesis: Report of an autopsy case

Tracheal gaenesis is an extremely rare congenital anomaly and is characterized by totally or partially undeveloped trachea. In many instances, the lesion has been associated with malformations in other organ systems. This anomaly is classified into 3 types based on developmental period of the respiratory tract. We report a case of tracheal agenesis with tracheoesophageal fistula in a 2-hour-old male. Postmortem examination revealed that the upper and mid portion of the trachea was absent but 1 cm segment of the trachea above the carina was present. The proximal end to this trachea opened to the esophagus by a fistula, 1 cm in diameter. The well-formed larynx ended blindly in a fibrous sac at which thyroids and cartilage islands are noted. Associated anomalies were bilobed right lung and single umbilical artery.