In September, 2007, a 9-day-old baby girl presented to the emergency department with increased respiratory eff ort and weight loss. She was delivered by elective caesarean section for breech presentation. At birth, she was fl oppy, cyanosed, and bradycardic. Her Apgar score was 5 and 9 at 1 and 5 min, respectively. On examination she had a grade II systolic murmur. Blood pressure and oxygen saturation were normal. On day 3, she was noted to have some weight loss due to poor feeding. Following successful supervised feeding and some weight gain, she was discharged the following day, in good condition, with an echocardiogram planned for day 9. On day 6, however, the mother raised concerns about her baby’s rapid breathing and continuing feeding diffi culties but was reassured by the community midwife. When we saw the baby in the emergency department she was cyanosed (SaO2 88% on room air), with a respiratory rate of 30–40/min, intercostal recession, and grunting. She had noisy breathing with intermittent episodes of desaturation and bradycardia. Her nasal passages were patent. The rest of the clinical examination was unremarkable. Capillary blood gas showed respiratory acidosis (ph 7·28, PO2 6·5 kPa, PCO2 9·1 kPa, HCO3 31 mmol/L, and base excess +1·6 mmol/L). Septic screen was negative. Chest radiography showed a boot shaped heart, suggestive of congenital cyanotic heart disease. She was transferred immediately to a specialist unit for a cardiology opinion. Echocardiography showed a small posteromuscular ventricular septal defect which did not account for the clinical presentation. Her noisy breathing was attributed to inspiratory stridor, and subsequent microlaryngoscopy and bronchoscopy (MLB) showed a large cyst arising from the vallecula (fi gure A). Aspiration of the cyst failed because of the viscosity of the contents; the cyst was incised and de-roofed successfully (fi gure B). Histological examination showed a squamous epi thelium lined cyst with a smooth muscle-containing wall. She was discharged home on the 10th post-operative day with intermittent soft stridor, and on demand feeds. When the baby was reviewed 6 weeks later in clinic, she had gained weight and was well, with no stridor or breathing diffi culty, and feeding well. Congenital tumours of the oropharynx are rare. Laryngeal cysts are broadly classifi ed as ductal (75%) or saccular (25%). Ductal cysts arise because of obstruction in the collecting ducts; vallecular cysts are a form of ductal cyst. The vallecula is the groove between the epiglottis and the base of the tongue; during swallowing food enters the vallecula and passes around the epiglottis either side into the pyriform aperture and then into the oesophagus. Although vallecular cysts are benign, a mortality rate of 40% has been reported because of major airway obstruction. They arise because of obstruction in mucous glands at the base of the tongue. Vallecular cysts can be detected in pregnancy as early as 25 weeks’ gestation. They can present antenatally as polyhydramnios, tracheal compression, or compression of cervical vessels or hypoglossal nerve. Postnatally, they can present as respiratory distress. Other clinical features are intercostal recession, failure to thrive, cough, dysphonia, dysphagia, and cyanosis. MLB and rigid endoscopy are the mainstay of diagnosis. A fi bre-optic review of the upper airway and bronchus is mandatory to rule out laryngo/bronchomalacia. The defi nitive treatment of these cysts is marsupialisation with CO2 laser or electrocautery. The prognosis is generally good with no risk of recurrence. Cyanosis in neonates is often caused by congential heart problems or lung pathology; our case shows that investigating non-cardiac causes of cyanosis in neonates can be a life-saving measure.
Read full abstract