Cases reported "Esophageal Atresia"

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1/10. Tracheoesophageal malformation: pathogenetic evidence provided by two cases.

    BACKGROUND: The aim of this observational study is to examine cases of upper respiratory atresia, in an effort to seek pathogenetic evidence suggestive of either a primary defect in embryogenesis or of a secondary etiology. methods: Archival material of 412 fetal and perinatal autopsies with congenital malformations was reviewed in an effort to identify infants with complete upper airway obstruction without tracheoesophageal (TE) communication. Histological sections of the upper and lower respiratory tract were examined, seeking evidence of previous amniotic fluid or meconium aspiration, indicated by the presence of amniotic squamous epithelial cells, lanugo hair, or bile-stained meconium in the airways. Immunohistochemical stain for epithelial membrane antigen (EMA) and high-molecular-weight cytokeratin (CK1) were used to identify amniotic fluid and keratinizing squamous epithelial cells. RESULTS: Eight infants with upper airway obstruction were identified, three of them without a TE communication. Two of the latter, one infant with multiple atresia born at 34 weeks gestation and another with tracheal atresia born at 32 weeks, presented evidence of amniotic fluid aspiration in their lungs. CONCLUSIONS: Evidence of amniotic fluid aspiration in the above two cases indicates that there had been a previous patency of the upper respiratory pathway, since the absence of a TE fistula excludes any communication with the oronasal cavity. This implicates a secondary pathogenesis for at least some cases of upper airway obstruction.
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2/10. [Operated esophageal atresia of late diagnosis, with postoperative survival]

    The authors present two cases of oesophageal atresia with oeso-tracheal communication that, although they have been diagnosed in the 2-nd and the 4-th day of life, have been operated successfully. Both children, investigated at the age of one and one and a half years respectively, are in perfect health.
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3/10. Communicating bronchopulmonary foregut malformations: classification and embryogenesis.

    Communicating bronchopulmonary foregut malformations (CBPFMs) are characterized by a fistula between an isolated portion of respiratory tissue (ie, a lung, a lung lobe, or a segment) and esophagus or stomach. We combine our 30-year (1959 to 1989) experience of 6 cases with 51 reported patients to propose a CBPFM classification supported by a proposed embryogenesis theory. Group I (16%): anomaly is associated with esophageal atresia and tracheoesophageal fistula. Group II (33%): one lung originates from the lower esophagus. Group III (46%): an isolated anatomic lung lobe or segment communicates with the esophagus or stomach. Group IV (5%): A portion of the normal bronchial system communicates with the esophagus. The portion of the lung served by the communicating bronchus receives systemic blood supply. The right and left lung sacs curve dorsally to embrace the lower esophagus during normal lung development. At this stage a part of the lung bud joins the esophagus. This segment then breaks away from the main pulmonary anlage to form a CBPFM. CBPFMs should be considered in the workup of infants with respiratory distress and/or recurrent pneumonias. patients with suspected pulmonary sequestration should undergo contrast studies to exclude a gastrointestinal communication.
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4/10. Successful thoracoscopic repair of esophageal atresia with tracheoesophageal fistula in a newborn with single ventricle physiology.

    A neonate with VACTERL association including tricuspid atresia was scheduled for thoracoscopic esophageal atresia with tracheoesophageal fistula (EA/TEF) repair and laparoscopic gastrostomy tube placement. In addition to standard noninvasive monitoring, arterial blood pressure, central venous pressure, and cerebral oxygen saturation were monitored. Gastric distension resulting from positive pressure ventilation prevented laparoscopic gastrostomy tube placement. thoracoscopy with a CO2 insufflation pressure of 6 mm Hg at low flow (1 L/min) was well tolerated hemodynamically despite hypercarbia and cerebral oxygen saturation was maintained. Careful monitoring and good communication were critical to the safe management of this single ventricle patient during thoracoscopic EA/TEF repair. IMPLICATIONS: Esophageal and tracheoesophageal fistula in conjunction with single ventricle physiology carries a significant risk of mortality. We present the anesthetic management of a neonate with unpalliated tricuspid atresia who underwent thoracoscopic tracheoesophageal fistula repair.
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5/10. Sirenomelia (Mermaid baby).

    Sirenomelia is a rare anomaly that rarely occurs as an isolated lesion. Several theories have been proposed regarding the etiopathogenesis. In this communication, we report a case of sirenomelia. Our patient was referred to hospital at the age of four hours. On examination, fusion of both lower limbs with hook shaped appendage, attached distally, absent genitalia and absent anal orifice was found. spine was deficient in sacral region. Upper torso looked normal. Baby also had frothing from mouth. abdomen was non-distended. Feeding tube no.10 was tried to pass through mouth, which got obstructed at the level of upper esophagus that suggested oesophageal atresia. The skeletogram revealed absence of pelvic bones, sacral agenesis, absent fibulae and fracture of both femora. The patient died at the age of 12 hours.
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6/10. The birth of a child with a congenital anomaly. Part I. Some difficulties experienced by parents in the maternity home.

    An investigation study of 90 cases in which a baby had been born with cystic fibrosis, oesophageal atresia, Hirschsprung's disease or an anorectal malformation was undertaken to establish the duration of the prediagnostic phase and the difficulties experienced by parents before discharge from the maternity home. The congenital anomalies requiring immediate surgical intervention were diagnosed very soon after birth. A wider diversity of problems were encountered in the case of those conditions with a longer prediagnostic phase. The problems encountered by parents, viz. lack of communication with staff, fantasies of gross anomalies with fear for the baby's life, and the lack of viewing and mothering of the baby are discussed, and some long-term effects are highlighted.
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7/10. Tracheo-oesophageal anomalies in the Goldenhar anomalad.

    A case of the Goldenhar anomalad is presented with a previously undescribed association with oesophageal atresia and tracheo-oesophageal fistula. This is the second instance of a tracheobronchial-oesophageal communication being found in association with the anomalad. awareness of this combination may facilitate future diagnosis and treatment of the anomaly.
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8/10. Distally placed transanastomotic drainage tube in the management of the severely leaking esophageal anastomosis.

    Although anastomotic leaks following retropleural esophagoesophagostomies for esophageal atresia are usually followed by spontaneous closure, disruptions may be life-threatening because of inability to control oral secretions. This communication describes the use of a distally placed transanastomotic tube to control severe leakage in three patients.
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9/10. The speech pathologist and management of children with tracheostomies.

    Troublesome airway problems necessitating the presence of a tracheostomy for extended periods of time are now not uncommon in children. To date, little attention has been devoted to the communication problems these young patients encounter. Experience with 44 children with tracheostomies, some of whom are ventilator-dependent, at the Children's Hospital of philadelphia has enabled us to develop a therapy program that we feel would be useful to other speech professionals treating such patients. The speech pathologist must take an active role in the evaluation and management of these patients and the education of the family and hospital staff. The ultimate goal is to provide communicative and feeding skills to these handicapped children.
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10/10. Endoscopic esophageal anastomosis.

    This paper describes four cases of esophageal atresia without a lower fistula in which a perlon thread was inserted through the two esophageal segments by an endoscopic method. With the aid of the thread and two metal olives the segments were drawn closer together and finally a communication was established without an operation. The method of introducing the thread and the olive technique are demonstrated in a series of drawings, and the results achieved in the case of four children are also presented.
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