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1/7. Congenital high airway obstruction syndrome and airway reconstruction: an evolving paradigm.

    OBJECTIVES: To refine the classic definition of, and provide a working definition for, congenital high airway obstruction syndrome (CHAOS) and to discuss the various aspects of long-term airway reconstruction, including the range of laryngeal anomalies and the various techniques for reconstruction. DESIGN: Retrospective chart review. patients: Four children (age range, 2-8 years) with CHAOS who presented to a single tertiary care children's hospital for pediatric airway reconstruction between 1995 and 2000. CONCLUSIONS: To date, CHAOS remains poorly described in the otolaryngologic literature. We propose the following working definition for pediatric cases of CHAOS: any neonate who needs a surgical airway within 1 hour of birth owing to high upper airway (ie, glottic, subglottic, or upper tracheal) obstruction and who cannot be tracheally intubated other than through a persistent tracheoesophageal fistula. Therefore, CHAOS has 3 possible presentations: (1) complete laryngeal atresia without an esophageal fistula, (2) complete laryngeal atresia with a tracheoesophageal fistula, and (3) near-complete high upper airway obstruction. Management of the airway, particularly in regard to long-term reconstruction, in children with CHAOS is complex and challenging.
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ranking = 1
keywords = airway obstruction, airway, obstruction
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2/7. The successful management of congenital laryngeal web with endoscopic lysis and topical mitomycin-C.

    Laryngeal web is a rare congenital anomaly. The primary goals of management for congenital laryngeal web are to provide a patent airway and to achieve a good voice quality. However, vocal cords have a tendency for fibrosis and granulation tissue formation after surgical interventions. Traditionally, the treatment of choice for laryngeal web is laryngofissure and placement of a stent or keel. This report presents the successful management of a congenital laryngeal web in a 10-month-old boy with endoscopic lysis and topical mitomycin-C application.
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ranking = 0.033278002923202
keywords = airway
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3/7. 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.
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ranking = 0.033278002923202
keywords = airway
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4/7. Congenital arhinia with de novo reciprocal translocation, t(3;12)(q13.2;p11.2).

    A female newborn suffering from congenital arhinia with complete airway obstruction is reported. In addition, she had hypertelorism, microphthalmia, high-arched palate, and hypoplasia of the auditory canal and mastoid and facial bones, along with the absence of olfactory bulbs and tracts. She had a de novo reciprocal translocation between chromosomes 3q13.2 and 12p11.2. Certain gene(s) located at either of the breakpoints, 3q13.2 and 12p11.2, may be involved in the pathogenesis of her arhinia.
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ranking = 0.11654422930438
keywords = airway obstruction, airway, obstruction
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5/7. Tracheal cartilaginous sleeve with cricoid cartilage involvement in Pfeiffer syndrome.

    Pfeiffer syndrome is one of a group of craniosynostosis syndromes in which rare tracheal anomalies have been described. This group of patients have a poor prognosis, and mortality can be related to airway complications and respiratory distress. We report a case of type II Pfeiffer syndrome with tracheal cartilaginous sleeve and cricoid cartilage involvement. We discuss our strategy for the management of the airway of this patient.
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ranking = 0.066556005846404
keywords = airway
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6/7. Airway anomalies in the oculoauriculofrontonasal syndrome.

    Oculoauriculofrontonasal syndrome was the subset of patients with oculo-auriculo-vertebral spectrum and frontonasal malformation. Radiographic evidence of tracheal duplication was documented in a male infant with oculoauriculofrontonasal syndrome. Although previously unreported in oculoauriculofrontonasal syndrome, airway anomalies in our case can be attributed to the oculo-auriculo-vertebral component of the oculoauriculofrontonasal syndrome.
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ranking = 0.033278002923202
keywords = airway
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7/7. Anaesthetic considerations in a patient with fraser syndrome.

    A 16-year-old girl with fraser syndrome underwent abdominal hysterectomy under general anaesthesia. She had multiple congenital anomalies which included a cardiac defect and airway abnormalities. intubation difficulties were circumvented by the retrograde technique of placement of the tracheal tube.
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ranking = 0.033278002923202
keywords = airway
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