Cases reported "Constriction, Pathologic"

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1/42. Rapid two-stage repair of S,L,L, ventricular septal defect, pulmonary atresia, ebstein anomaly of the tricuspid valve, and stenotic pulmonary arteries.

    The repair of a corrected transposition of the great arteries, ventricular septal defect, and pulmonary atresia is presented. An ebstein anomaly of the tricuspid valve, dextrocardia, and severe distortion of the pulmonary arteries complicated the surgical procedure, which was performed in two stages. Reconstruction of the pulmonary arteries and a bidirectional cavopulmonary anastomosis were performed first; Rastelli and hemi-Mustard procedures completed the correction. The rationale and the possible indications of this "one and a half ventricle" repair are discussed.
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ranking = 1
keywords = atresia
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2/42. Stenting of stenosed aortopulmonary collaterals and shunts for palliation of pulmonary atresia/ventricular septal defect.

    patients with unrepaired pulmonary artery atresia and ventricular septal defect (PA/VSD) depend on aortoplumonary collaterals and surgically created shunts for pulmonary blood flow. These vessels frequently develop stenoses with time, leading to hypoperfusion of lung segments and systemic hypoxemia. The purpose of this article is to describe catheter palliation of hypoxemic patients with PA/VSD who were not candidates for surgical repair. We present our experience with stent implantation for stenosis of aortopulmonary collaterals and shunts in these patients. Three patients with hypoplastic pulmonary arteries underwent stent placement in aortopulmonary collateral arteries (APCAs) or their shunts. Technical aspects of the interventional catheterization procedure are discussed in detail. Case 1 underwent placement of five stents in collateral vessels and one stent in the Blalock-Taussig shunt (BT) with dramatic increase in vessel size and improvement in saturations from 70% to 89%. Case 2 underwent placement of two overlapping stents in a collateral vessel with an increase in diameter of the collateral vessel from 2.3 to 6 mm and an improvement in saturation from 68% to 88%. Case 3 underwent placement of three overlapping stents in a BT shunt with an increase in diameter of the shunt from 2.2 to 6.6 mm and an improvement in saturation from 71% to 89%. All three patients had excellent clinical improvement and stable saturation at follow-up. Stent placement for maintaining patency of APCAs and aortopulmonary shunts is feasible and safe.
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ranking = 1
keywords = atresia
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3/42. Placement of a colonic stent by percutaneous colostomy in a case of malignant stenosis.

    We present a patient with disseminated stomach cancer who presented with symptoms of acute obstruction of the splenic flexure of the colon caused by tumor spread. During a first attempt to insert a colon stent through the anus under endoscopic guidance as final palliative therapy, it was not possible to reach the region of the stricture, and iatrogenic perforation of the descending colon occurred, which resolved favorably under conservative management. A second attempt to insert a stent was made via percutaneous puncture of the transverse colon, approaching the region of the stricture by a descending route. The procedure was completed without complications and the patient's symptoms improved. Stent placement via percutaneous puncture of the colon has not previously been described in the literature. It may be an alternate route in cases of proximal strictures in which access through the anus has been unsuccessful even with the aid of endoscopic guidance.
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ranking = 0.0060339060314768
keywords = anus
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4/42. Anomalous origin of the pulmonary artery from the right coronary artery.

    A 5-year-old boy who had an anomalous origin of a stenotic pulmonary artery from the right coronary artery with ventricular septal defect and pulmonary atresia is reported. The diagnosis was made at operation. Successful repair was achieved for the complex anomaly by using a pulmonary artery homograft as a conduit and closing the ventricular septal defect.
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ranking = 0.2
keywords = atresia
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5/42. lichen planus involving the esophagus.

    lichen planus is a common mucocutaneus disorder that rarely involves the esophagus. We report two challenging cases presenting with recurrent dysphagia originally suspected due to gastroesophageal reflux. Subsequent evaluation revealed peculiar endoscopic findings of desquamative esophagitis leading to the diagnosis of lichen planus of the esophagus. This disorder should be considered in middle age or elderly women presenting with unexplained dysphagia or odynophagia. In this paper we review the available literature on the subject and summarize every case reported to date.
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ranking = 0.01810171809443
keywords = anus
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6/42. pallister-hall syndrome with stenosis of the cricoid cartilage and microphallus without hypopituitarism.

    The pallister-hall syndrome is characterised by a spectrum of anomalies including congenital hypothalamic "hamartoblastoma" hypopituitarism, imperforate anus, polydactyly and various visceral anomalies. Rare familial cases with an autosomal dominant inheritance pattern with variable expressivity have been reported. Cases of more mildly affected individuals with pallister-hall syndrome have been described, including cases of asymptomatic individuals. We report a case of pallister-hall syndrome with microphallus and without growth hormone deficiency that has been followed successfully for two years. The patient presented postaxial polydactyly of hands, dysplasic nails, imperforate anus, small penis, scrotum bifidum with very thin urethra, bifid epiglottis and a bilateral simian crease. There was vesico-ureteral-reflux, insertional hexadactyly of the left hand and two Y shaped metacarpal with six fingers at the right hand. brain MR imaging revealed a large sellar and suprasellar mass. A perineal anorectoplasty and a vesicostomy were performed. Laryngeal dyspnea appeared when he was 13 months old. bronchoscopy revealed anterior synechia of vocal cords with cricoidian stenosis. A tracheostomy was performed. Mental development was normal. No mutation of the zinc finger transcription factor gene, GLI 3 was detected.
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ranking = 0.0060339060314768
keywords = anus
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7/42. choanal atresia repair. The use of reinforced silicone tube to prevent restenosis.

    There are different approaches for the repair of congenital choanal atresia. A stent is usually inserted in the nostrils to prevent restenosis. We describe a case of an infant who was presented with recurrent choanal stenosis and was managed by a stent made of reinforced silicone rubber tracheal tube. The tube has several advantages over other known stents.
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ranking = 1
keywords = atresia
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8/42. choanal atresia: an unusual serious complication of complementary and alternative medical treatment.

    The use of complementary and alternative medicine (CAM) is common among patients with chronic, long-standing nasal disease. Complications resulting from inappropriate CAM practices are sometimes reported, but serious complications such as choanal atresia and stenosis are rare. We report a case of choanal atresia and stenosis due to herbal drug (ie, chemical) cauterization of the nasal cavity in a 39-year-old man. We reviewed the literature on why patients previously given conventional treatment seek alternative therapies. The patient underwent surgical intervention to relieve choanal atresia and stenosis. The postoperative course was unremarkable, and follow-up for 1 year revealed no evidence of restenosis. choanal atresia and stenosis resulting from CAM treatment are rare. Otolaryngologists should be aware of possible complications occurring in patients receiving such treatments.
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ranking = 1.6
keywords = atresia
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9/42. Valuable use of computer-aided surgery in congenital bony aural atresia.

    Congenital aural atresia repair is difficult owing to unpredictable anatomy. Benefits may be gained from computer-aided surgery (CAS), but its exact role has yet to be clearly defined. This is a retrospective study of 18 patients with bony type C (Schuknecht classification) congenital atresia. In the first group (n = 9), repair was performed with CAS while in the second group (n = 9), similar intervention was applied without CAS. Intra- and post-operative clinical and audiological findings were compared. CAS computed tomography (CT) images correlated well with intra-operative findings giving the surgeon more security and reducing operative time by 25 minutes. In our estimation, CAS is valuable for type C congenital aural atresia repair. It serves as an educational tool and as a guide for the experienced surgeon in critical situations where anatomical landmarks are distorted and where access is limited.
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ranking = 1.4
keywords = atresia
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10/42. Technique for fabrication of splint preventing postsurgical restenosis in choanal atresia.

    choanal atresia, a congenital narrowing or obstruction of the nasal airway caused by significant respiratory distress in neonates, may require emergency intervention. Although atresia can be repaired surgically, restenosis is a common complication with this procedure. To prevent this, tubes are inserted into the nasal cavity immediately after surgery. This article describes a technique for preparing individual surgical splints designed to prevent postsurgical obstruction of the nasal cavity.
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ranking = 1.2
keywords = atresia
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