Anaesthetic Management for Surgical Relief of Neonatal Airway Obstruction Caused by a Cystic Hygroma

Background: Cystic hygroma is a rare congenital malformation of the lymphatic system which may cause neonatal airway obstruction. Surgical excision of this tumour is associated with high morbidity and mortality. Aim: To report the anaesthetic management for a successful excision of a cystic hygroma in a neonate. Case Report: A two-day-old female presented with history of an anterior neck swelling; tachypnoea and dyspnoea ; noticed at birth. There was associated stridor and subcostal recession. A diagnosis of cystic hygroma with upper airway obstruction was made. Tumour excision was done under endotracheal general anaesthesia. An intraoperative tracheostomy was carried out. Cyanosis occurred frequently within first 24 hours postoperatively while in the special care baby unit (SCBU) and was relieved by intermittent suction of the tracheostomy tube. Patient was weaned off the tracheostomy tube on the 30th post-operative day and discharged home two days later. She was subsequently followed up at the paediatric surgery outpatient clinic. Conclusion: Anaesthetic management for excision of a cystic hygroma in a neonate is by no means easy. It can be successful if the airway remains patent; ventilation is adequate; normothermia and optimal replacement of body fluid losses are ensured

Neurofibromatosis in Children as Seen in the University of Port Harcourt Teaching Hospital

Background: Neurofibromatosis (NF) is transmitted as an autosomal dominant trait but 50of new cases appear because of mutation. The disease can be confounded by a broad spectrum of complications; such as various kinds of osseous lesions; stenosis of the renal arteries; optic glioma; and learning disabilities and disfigurement. Aim : To determine the prevalence; pattern of presentation and outcome of neurofibromatosis in children in the University of Port Harcourt Teaching Hospital. Method: This was a prospective study done from 1st June 2004 to 30th May 2009. All the children who presented with neurofibromatosis over the 5- year period were studied. Data collected included age; sex; and reasons for presentation. Definitive diagnosis was made using the National Institute of Health Diagnostic Criteria. Data was entered into excel and analyzed using SPSS. Simple statistics; frequency and percentages were used. Results: The total number of children seen over the period of study was 12;443 children. Out of these; 18(0.19) had neurofibromatosis. All (100) had the type 1 variety. None had type 2 variety. The patients' ages ranged between 5 and 16 years. There was a positive family history in 13 (72.2) of them. All the patients had cafe au- lait spots. Clinically the predominant forms of the neurofibromas were cutaneous 15(83.3); subcutaneous 10(55.6); and plexiform 5(27.8). None of the patients died. Five cases with huge masses causing pain and disfigurement had surgical intervention. Conclusion: Neurofibromatosis is not rare in this environment. This neurocutaneous inherited genetic disorder is a common cause of morbidity. Multidisciplinary management reduces the disease burden borne by affected individuals