Cases reported "Esophageal Atresia"

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1/95. Esophageal lung with multiple congenital anomalies: conundrums in diagnosis and management.

    BACKGROUND/PURPOSE: Communicating bronchopulmonary foregut malformations (CBPFM) are a diverse group of potentially devastating congenital anomalies with anatomy that may be difficult to delineate. The authors present a case that illustrates conundrums in the diagnosis and management of these complex disorders. methods: A term baby had esophageal atresia (EA), tracheoesophageal fistula (TEF), and tetralogy of fallot. Initially, a gastrostomy was performed, and a balloon catheter was inserted through the endotracheal tube to occlude the fistula until the patient was hemodynamically stable. Subsequently, the fistula was ligated. Postoperatively, the left lung collapsed, and bronchoscopy showed an atretic left mainstem bronchus. Repeat thoracotomy showed that the fistula ligation was intact. air was introduced through the gastrostomy tube, and, surprisingly, the left lung inflated, indicating the left mainstem bronchus arose from the esophagus distal to the ligated TEF. RESULTS: Despite reopening this fistula, ventilation remained poor, and support was withdrawn. autopsy findings confirmed a unilobed left lung arising from the esophagus, EA, TEF, an atretic left mainstem bronchus, tetralogy of fallot, and digeorge syndrome. CONCLUSIONS: This is the first report of a combination of EA and distal TEF with a second CBPFM involving the esophagus and the entire left lung. Successful correction of these anomalies will require extensive delineation of the anatomy to plan an operative strategy.
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2/95. A successful one-stage reconstruction of the esophagus in a newborn with a long gap esophageal atresia and tracheo-esophageal fistula.

    A method of one-stage surgery of long gap esophageal atresia was applied in a newborn child. An anterior muco-muscular flap was moulded from the upper pouch and transposed to the lower pouch of the esophagus. The technique allowed the tension in the anastomosis between the two parts of the esophagus to be reduced. The child had no feeding abnormalities during the follow-up till the age of 5 years.
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3/95. Prognostic significance of the pouch sign in fetuses with prenatally diagnosed esophageal atresia.

    OBJECTIVE: esophageal atresia may be diagnosed prenatally by ultrasonographic visualization of the blind-ending esophagus during fetal swallowing, which is referred to as the pouch sign. Our purpose was to determine whether this sign can be used to predict outcomes of affected fetuses. STUDY DESIGN: Four cases of esophageal atresia diagnosed in our center during the past 2 years were analyzed, in conjunction with 3 cases from published series. Ultrasonographic features of the pouch sign were categorized according to localization. RESULTS: In the neck pouch group (n = 3) 1 fetus died in utero and 1 died before a corrective operation could be undertaken. In the only fetus of this group to survive a staged repair was necessary because of a long atretic gap. Conversely, 3 of the 4 fetuses with a mediastinal pouch survived after a successful corrective operation, and primary repair was possible in all cases. CONCLUSIONS: The base of the proximal blind-ending esophagus can be clearly localized by means of ultrasonography. Our data suggest that a neck pouch may be associated with an adverse outcome. This information may be useful in counseling parents when esophageal atresia is diagnosed prenatally.
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4/95. Use of "Gore-Tex surgical membrane" to minimize surgical adhesions in multistaged extrathoracic esophageal elongation for esophageal atresia.

    The procedure of choice in the surgical correction of "long gap" esophageal atresia should, when possible, preserve the native esophagus. We present a modification of "the multistaged extrathoracic esophageal elongation method," designed to facilitate esophageal elongation and use of a Gore-Tex (W.L. Gore and Associates, Flagstaff, AZ) surgical membrane to minimize surgical adhesions. We used this technique to successfully treat a 1-kg infant, with type A esophageal atresia, associated aortic coartation, and severe necrotizing enterocolitis with multiple perforations. Multistaged extrathoracic esophageal elongation was begun at the age of 9 months and concluded at 17 months.
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5/95. Hydrostatic balloon dilation of congenital esophageal stenoses associated with esophageal atresia.

    It has been stated that congenital cartilage rings in the esophagus do not respond to dilation and should be resected. The authors report on 3 infants with congenital esophageal stenoses who were treated successfully with hydrostatic balloon dilation. Based on the appearance during dilation the authors believe that these stenoses were cartilage rings. The technique is described in detail. Balloon dilation is the treatment of choice for these patients. Resection should be reserved for those who do not respond to this form of therapy.
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6/95. Lateral esophagostomy: an alternative in the initial management of long gap esophageal atresia without fistula.

    The authors report an alternative method of cervical esophagostomy that was used in a child with type A esophageal atresia. This method involved performing a lateral esophagostomy in the proximal pouch, preserving its distal end, allowing the child to swallow normally, without choking, while stimulating the spontaneous growth of the proximal esophagus. As a result, the infant could be discharged home on G-tube feedings while waiting for spontaneous growth of the proximal pouch to occur. There were no episodes of aspiration during this period, and definitive reconstruction through end-to-end esophageal anastomosis was accomplished successfully at the age of 18 months. The authors consider that this alternative might increase the possibility of a definitive correction through delayed primary anastomosis of the infant's own esophagus in children with this type of malformation.
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7/95. Early primary repair of long gap esophageal atresia: the VATER operation.

    Despite the numerous approaches described for the management of neonates with "long gap" esophageal atresia, controversy still exists as to the preferred method. Delayed primary anastomosis is probably the most frequently adopted practice, but often the native esophagus is abandoned, and the long gap is bridged by some form of esophageal replacement. A case is reported of a 1.38-kg newborn with pure esophageal atresia and a long gap undergoing early primary repair. The technique used in this patient and the various options available for management of long-gap esophageal atresia are discussed.
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8/95. Oesophageal perforation presenting as oesophageal atresia in a premature neonate following difficult intubation.

    Iatrogenic oesophageal perforation in neonates is well recognized in the medical and surgical literature with intubation injury listed as a possible contributing mechanism besides nasogastric tube placement and suctioning. Diagnosis can be difficult and sometimes confused with other entities. With early diagnosis, nonsurgical management often leads to complete resolution in neonates. We report the case of a 1-day-old premature neonate who was brought to the operating room with the preliminary diagnosis of proximal oesophageal atresia with stump perforation and distal tracheo-esophageal fistula. His intubation for respiratory distress at birth had been difficult due to Pierre-Robin sequence with micrognathia. Oesophagoscopy in the operating room revealed a patent oesophagus but perforations in the pharynx and in the proximal oesophagus with the nasogastric tube entering the pharyngeal perforation. Oesophageal perforation and the limitations of the difficult airway algorithm in small neonates are discussed.
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9/95. leiomyoma at the site of esophageal atresia repair.

    Esophageal leiomyomas are rare in the pediatric population. They frequently occur in association with other anomalies, such as Alport's syndrome, osteoarthropathy, and leiomyomas elsewhere in the body. The authors describe the case of a focal esophageal leiomyoma in a 12-month-old girl with a history of long-gap esophageal atresia. The patient initially underwent bouginage of the proximal pouch while awaiting definitive repair. After esophageal repair, the patient required multiple dilatations for anastomotic strictures. A segmental resection of the esophagus was performed because of recurrent strictures. A leiomyoma, arising from the site of the previous esophageal atresia repair, was noted on histologic evaluation. Esophageal leiomyomas have not been reported previously in a child with esophageal atresia. J Pediatr Surg 36:1832-1833.
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10/95. A new variant of esophageal atresia associated with esophageal heterotopic pancreas.

    An infant with esophageal atresia (EA) and absence of gas on abdominal radiographs was found to have an obliterated distal tracheoesophageal fistula (TEF). Preoperative bronchoscopy and surgical exploration found that the reason for gasless abdomen was the atresia of the distal portion of lower esophagus, which also contained heterotopic pancreatic tissue. The type of trachea/esophageal anomaly found in this patient, with the association of esophageal heterotopic pancreas (EHP), has not yet been reported in the literature.
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