1/21. 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 RESULTS: Despite reopening this fistula, ventilation remained poor, and support was withdrawn.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.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
2/21. Prefabrication of jejunum for challenging reconstruction of cervical esophagus.A significant benefit exists for a jejunal replacement of the cervical esophagus, if indicated. The absence of available recipient vessels may impede free tissue transfer. If vascular induction between a vascular carrier and the selected jejunal segment is done as a kind of flap prefabrication, the jejunal interposition flap can be used without the need for complex microsurgery.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
3/21. Unilateral bronchiectasis and esophageal dysmotility in congenital adult tracheoesophageal fistula.Tracheoesophageal fistulas (TEF) in adults are most commonly neoplastic, and very rarely congenital in nature. We report a 45-year-old Hispanic male with TEF and initial presentation of minimal hemoptysis. The patient had radiographic evidence of unilateral upper lobe (RUL) bronchiectasis, massive esophageal dilatation, and dysmotility. However, there was no evidence of esophageal malignancy, achalasia, or Chagas' disease. bronchoscopy revealed a large TEF in the posterior wall of trachea, which was not visualized on esophagram or esophagoscopy. bronchoalveolar lavage (BAL) cultures grew mycobacterium avium complex (MAC). Our report illustrates that idiopathic, or congenital, TEF can be associated with esophageal dysmotility, adulthood bronchiectasis, and atypical mycobacterial superinfection.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
4/21. Tracheal agenesis with anomalies found in both VACTERL and TACRD associations.Tracheal agenesis (TA) was diagnosed by endoscopy after esophageal intubation in a 34-week newborn. diagnosis, work-up, and management approach are discussed. Similar to previous reports, this case of tracheal agenesis presented with multiple associated defects found at autopsy. Tracheal agenesis had previously been reported as a part of the VATER (vertebral defects, anal atresia, tracheoesophageal fistula and/or esophageal atresia, radial dysplasia, renal defects) and VACTERL (VATER plus cardiovascular and limb defects) associations/syndromes. More recently, cases of TA have been reported with associated anomalies described as TACRD (tracheal agenesis/atresia, complex congenital cardiac abnormalities, radial ray defects, and duodenal atresia) association/syndrome. We present a case of TA with anomalies found in both VACTERL and TACRD associations, which support the speculation that both are different manifestations of a spectrum of mesodermal dysplasia.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
5/21. Urogenital sinus, rectovaginal fistula, and an anterior stenosed anus--another cloacal variant.The persistent cloaca is one of the most complex and challenging developmental malformations. It is a rare anomaly occurring only in the female newborn and is represented by the association of urogenital sinus with an anorectal malformation (arm). Each case is probably unique. We report here one such case of cloaca with the VATER association-tracheoesophageal fistula (TOF) with a urogenital sinus, rectovaginal fistula, and an anteposed stenosed anus, along with preaxial syndactyly of the right hand. The spine, renal, and cardiac systems were normal. Interim management was directed towards repair of the TOF and a right transverse defunctioning colostomy. Despite thorough radioendoscopic preoperative investigations, the complexity of the cloacal anomaly was not delineated until surgery. This case is a rather rare combination of an intermediate form of the cloacal-arm spectrum. Such patients present with many diagnostic and therapeutic problems. Interval surgery should be directed towards decompression of the affected organ systems, and definitive surgery must be carefully planned and, whenever possible, done in a single stage with simultaneous multisystem repair to correct all significant malformations related to the cloacal complex.- - - - - - - - - - ranking = 3keywords = complex (Clic here for more details about this article) |
6/21. Tracheal agenesis: management of the first 10 months of life.Tracheal agenesis is a potentially lethal congenital anomaly, appearing only at birth. We describe a newborn preterm infant who presented with immediate respiratory distress and no audible cry. There was almost complete tracheal agenesis with a very short segment of distal trachea (only two tracheal rings) arising from the anterior wall of the esophagus, before dividing into the mainstem bronchi. The anomaly was unsuspected prenatally, as the scan showed pyloric atresia and complex congenital cardiac disease. Despite the patient's difficult course, with correction of the rare-associated malformations (cardiac and gastrointestinal tract anomalies), the fact that the child is lively and neurologically normal for her age, requires that we now consider the patency of the airway and the possibility of surgical correction, in accordance with a good quality of life.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
7/21. The TACRD association is distinct from VACTERL association--a case report.Tracheal agenesis is a rare malformation. A case of tracheal agenesis with associated ventricular septal defect, gastric hypoplasia, hiatus hernia, duodenal atresia, isomerism of the lungs, hypoplastic spleen with polysplenia, Meckel's diverticulum and fused crossed ectopia of the kidneys is presented. This case fits into a newly proposed TACRD association, an extremely rare condition composed of tracheal agenesis, cardiac, renal and duodenal malformations. It is distinct from the more common VACTERL association of which tracheo-esophageal fistula and not tracheal agenesis is a part. TACRD association has complex internal malformations and lacks the external skeletal manifestations of VACTERL association. awareness amongst pathologists conducting neonatal autopsies is necessary for correct identification of this condition.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
8/21. Multidetector-row computed tomography three-dimensional volume reformatted 'transparency' images to define an upper pouch fistula in oesophageal atresia.Oesophageal atresia (OA) is an important congenital malformation in which prompt diagnosis and appropriate management can significantly improve outcome. The surgical approach to repair of OA and associated tracheo-oesophageal fistulae (TOF) depends upon correct evaluation of the tracheobronchial tree. Three-dimensional imaging of the tracheobronchial tree using CT data to produce shaded surface displays and virtual bronchoscopy has been reported in paediatric and neonatal patients with OA and TOF and is described as accurate and helpful, non-invasively facilitating the appreciation of complex anatomy prior to surgery. We describe the technique of reconstructing 3D volume-reformatted 'transparency' images using insufflated air as a negative contrast medium. This technique is fast, accurate and produces high-quality images that are easy to reproduce.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
9/21. Agenesis of tibia with bifid femur, congenital heart disease, and cleft lip with cleft palate or tracheoesophageal fistula: possible variants of Gollop-Wolfgang complex.Two patients with bifurcation of one femur, ipsilateral absence of tibia and one partially formed tibia, or bilateral absent tibiae, club feet with normal or nearly normal digits, congenital heart disease and, in one case, tracheoesophageal fistula; in the second, cleft lip and cleft palate and enlarged head with increased fluid and lissencephaly, are described. These appear to be unique combinations of defects but overlap with the Gollop-Wolfgang complex is present, particularly with the case of possible Gollop-Wolfgang described by Raas-Rothschild et al.- - - - - - - - - - ranking = 5keywords = complex (Clic here for more details about this article) |
10/21. balloon valvuloplasty for critical aortic stenosis in a low birth weight baby with tracheoesophageal fistula.The use of valvuloplasty in low birth weight infants with valvular aortic stenosis has not been widely reported. We report a 1556 g 3-day-old female infant with complex heart disease including critical valvular aortic stenosis, bicuspid aortic valve, mitral stenosis, patent ductus arteriosus, and secundum type atrial septal defect. She also had esophageal atresia with a distal tracheoesophageal fistula and trisomy 18. balloon valvuloplasty was considered to be more dangerous in this patient than in infants with simple critical aortic stenosis without other associated anomalies or low birth weight. Retrograde aortic balloon valvuloplasty was done carefully with the patient intubated and mechanically ventilated. We successfully dilated the stenotic aortic valve, which resulted in a dramatic decrease of the left ventricular pressure from 139/12 mm Hg to 81/11 mm Hg. Despite the high operative risk and poor prognosis, balloon valvuloplasty may be the first feasible step towards the survival of low birth weight infants with valvular aortic stenosis.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
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