Intracranial hemorrhage in patients with hemophilia is associated with high mortality and morbidity. We report a case of 15 years old boy with haemophilia A, who presented with a spontaneous acute subdural hematoma and underwent craniotomy for clot evacuation. The patient also received Factor VIII infusions peri-operatively along with other measures, to decrease blood loss. The patient presented with signs of raised intracranial pressure and received mannitol intra-operatively and postoperatively to prevent brain edema. Hypertonic saline (3 ml/kg of 3% solution) was also given over 30 minutes for brain relaxation. Recommendations for peri-operative preparation and management of haemophilia, especially in the setting of emergency major surgery were also reviewed.
Introduction: Giant encephalocele are encephalocele more than the size of head from which it arises. They pose a management challenge in view of risk of associated anomaly, challenges in anaesthesia and surgical management. There is no literature available from peripheral tertiary care centres from India apart from few case reports. Methods: This case series comprises of seven cases operated at our institute from 2014 to 2021. After routine clinical examination, patients subjected to recommended radiological investigations. Patients were anaesthetised with standard protocol with intubation in lateral or supine position with head hanging at edge of table and supported at table. Surgery carried out in lateral position with complete excision of sac with CSF diversion if pre existing hydrocephalus was present. Results: Out of seven cases, 3 were males and four female with age range from 12 days to 14 Mo. All patients were anaesthetised with standard protocol and had excision of sac with CSF diversion if required with satisfactory outcome in post operative phase and follow up. No death was recorded. Conclusion: With careful assessment and all recommended investigation with standard practice of anaesthesia and surgery, acceptable outcomes can be expected. Emphasis should be laid for good antenatal folate supplementation and possible antenatal diagnosis of meningoencephaloceles.
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