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A female infant was born at 32 weeks gestation. Growth restriction and oligohydramnios were previously noted. She was intubated and ventilated at birth because of poor respiratory effort. After self extubation at 24 h, she was given a trial of nasal continuous positive airway pressure (CPAP). This failed so she was re-intubated and ventilated, maintaining oxygen saturation of >95% in 30% FiO2, with pressures of 18/4. Attempts to increase nasogastric feeds resulted in large aspirates and an increase in respiratory distress. At end of week 1 she had developed radiological changes consistent with aspiration pneumonia. A stomach bubble was clearly seen on x ray but showed the nasogastric tube in the left main bronchus with a prominent oesophageal shadow leading to the stomach (fig 1). The patient was discussed with the local perinatal centre on day 10 with a view to further investigation and management. Intubation was difficult and vocal cords were not visualised. At resuscitation it was noted that chest movement was seen only with external abdominal compressions and not with attempted mask inflation. Ear, nose and throat (ENT) review after transfer confirmed type 4 laryngotracheo-oesophageal cleft (LTEC). She died on day 13. Post mortem examination confirmed the cleft (fig 2).
Type 4 LTEC is the most severe of a spectrum of clefts, extending from the larynx to the carina. Mortality of 93% has been reported. Many have upper gastrointestinal anomalies including oesophageal atresia and cleft lip and palate. Fistula formation, gastro-oesophageal reflux, microgastria and tracheomalacia are documented surgical complications.1 Important prognostic factors are the magnitude of the LTEC and coexistence of other congenital anomalies.2 Although rare, it requires prompt surgical intervention to optimise outcome.3
Acknowledgments
Dr Ezam Mat-Ali (Conultant Neonatologist), Northwick Park Hospital. Dr Wyatt-Ashmead (Consultant Pathologist), Queen Charlotte’s & Chelsea Hospital.
Footnotes
Competing interests: none.
Patient consent: Patient/guardian consent was obtained for publication