Intracranial hemorrhage in patients with hemophilia is associated with high mortality and sequelae. We report the case of 50-year-old man with Hemophilia A, who presented with spontaneous acute subdural hematoma and underwent craniotomy for clot evacuation. The patient received Factor VIII infusions perioperatively along with other measures to decrease blood loss. The patient presented with signs of high intracranial tension and received 3% saline intraoperatively and postoperatively to prevent brain edema. Recommendations for perioperative preparation and management of hemophilia, especially in the setting of emergency major surgery are reviewed.
Acromesomelic dysplasias are autosomal recessive osteochondrodysplasias. Acromesomelic dysplasia Maroteaux-type (AMDM), also known as St Helena dysplasia, is of two types: The classical and the mild variety. About 50 cases of AMDM have been reported till date, most of them being the classical variety. There is scarcity of literature on anesthesia for such patients. We are reporting a case of general anesthetic management of AMDM, associated with hydrocephalus, Arnold Chiari malformation type-1 and syringomyelia. The patient was a 10-year-old short-statured boy who presented with symptomatic thoracic kyphoscoliosis, gibbus deformity and back pain. On examination, there was no neurological deficit. Radiology revealed thoracic kyphoscoliosis, mild ventriculomegaly and upper cervical syringomyelia. The patient underwent posterior fossa decompression in the prone position under general anesthesia. We will discuss the anesthetic considerations for such patients and review the pertinent literature.
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