Kaposiform hemangioendothelioma involving whole of a leg in a neonate with Kasabach-Merritt phenomenon causing limb and life-threatening situation has not been reported. One such case and its successful management is presented in this case report. Literature review is made.
Background: Complex injury to the esophagus, trachea, and tracheoesophageal fistula in a case of esophageal atresia is rarely reported that requires prompt identification and management.
Case Presentation: A very-low-birth-weight preterm with a tracheoesophageal fistula on ventilatory support for respiratory distress syndrome was transferred to our hospital. The baby developed air-leak through the tracheoesophageal fistula into the stomach causing gross distension of the stomach and reduction in oxygen saturation. Repositioning and reintubation failed to reduce the abdominal distension and to improve the saturation. Subsequent intubation with large volume, low pressure cuffed tube, and replacement of Replogle tube resulted in pneumothorax and further deterioration requiring emergency thoracotomy. Complex tracheoesophageal injury to the esophagus, trachea, and fistulous tract was repaired.
Conclusion: The baby survived the early repair of the tracheoesophageal fistula and the iatrogenic complex tracheoesophageal injury. Pre-operative tracheo-esophagoscopy with stenting of the fistula with Fogarty catheter helped to carry out a successful repair technically.
Bifid epiglottis is a rare laryngeal anomaly that can be an isolated occurrence or as part of malformation syndromes, commonly the Pallister Hall syndrome (PHS). PHS presents with hypothalamic hamartomas, polysyndactyly, bifid epiglottis, imperforate anus and genitourinary abnormalities. We report a case of a 22 months old male child with features of PHS and Hirschsprung's disease. The presentations are with a hypothalamic mass, a bifid epiglottis and a midline mass in the larynx with symptoms of respiratory distress. The laryngeal lesion was endoscopically excised and its histology confirmed as hamartoma (laryngeal). The importance of an early diagnosis of PHS, genetic counselling and prompt management of a compromised airway and endocrine abnormalities is emphasized to have a better outcome.
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