Gorham's disease of skull base and cervical spine--confusing picture in a two year old.
The unusual presentation of Gorham's disease of skull base and cervical spine in a two-year-old female child with radiological signs mimicking those of raised intracranial pressure is discussed. The differential diagnosis consists of skull base tumours, meningitis, osteomyelitis of the base of skull, congenital hydrocephalaus and congenital syndromes involving the skull base. Pathologically it can be very difficult to differentiate it from lymphangioma of the bone. Difficulty in establishing the diagnosis is discussed along with failure of radiotherapy and palmidronate therapy to cause arrest of the disease process and failure of surgery to provide stabilisation. We describe the course of the disease in this child over the period of last eight years. To the best of our knowledge this is the youngest case of Gorham's described so far.
Girn
H R S
HR
Department of Neurosurgery, Leeds General Infirmary, Great George Street, Leeds. hrsgrin@aol.com
Towns
G
G
Chumas
P
P
Holland
P
P
Chakrabarty
A
A
D050071:Bone Density Conservation Agents / Q000627:therapeutic use; D001842:Bone and Bones / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D002574:Cervical Vertebrae / Q000000981:diagnostic imaging* / Q000473:pathology* / Q000503:physiopathology; D002648:Child; D003638:Deafness / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D003937:Diagnosis, Differential; D016267:External Fixators; D017809:Fatal Outcome; D005260:Female; D005269:Femur / Q000000981:diagnostic imaging / Q000473:pathology; D006390:Hemangioendothelioma / Q000175:diagnosis; D006801:Humans; D019586:Intracranial Hypertension / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D010015:Osteolysis, Essential / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy; D010388:Pelvis / Q000000981:diagnostic imaging / Q000473:pathology; D011878:Radiotherapy; D019291:Skull Base / Q000000981:diagnostic imaging* / Q000473:pathology* / Q000503:physiopathology; D013117:Spinal Cord Compression / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D014057:Tomography, X-Ray Computed; D017211:Treatment Failure
D050071:Bone Density Conservation Agents