J Bone Metab.  2014 Aug;21(3):227-232. 10.11005/jbm.2014.21.3.227.

A Case of Cavernous Sinus Thrombophlebitis and Meningitis as a Complication in Osteopetrosis

• Chung HC
• Park SH
• Kim ES
• Kim YI
• Lee SH
• Nam-Goong

• KMID: 2286294
• DOI: http://doi.org/10.11005/jbm.2014.21.3.227

Abstract

Osteopetrosis is a rare genetic bone disease characterized by increased bone density but prone to breakage due to defective osteoclastic function. Among two primary types of autosomal dominant osteopetrosis (ADO), osteopetrosis type II is characterized by sclerosis of bones, predominantly involving the spine, the pelvis, and the skull base. Fragility of bones and dental abscess are leading complications. This report presents a case of osteopetrosis in a 52-years-old female, which was complicated by the development of cavernous sinus thrombophlebitis and meningitis. She was suffered from multiple fractures since one year ago. Laboratory data revealed elevated serum levels of tartrate resistant acid phosphatase (TRAP) without carbonic anhydrase II DNA mutation. A thoracolumbar spine X-ray showed, typical findings of ADO type II (ADO II; Albers-Schonberg disease), prominent vertebral endplates so called the 'rugger jersey spine'. Her older sister also showed same typical spine appearance. We report a case of ADO II with cavernous sinus thrombophlebitis and meningitis that was successfully treated with long-term antibiotics with right sphenoidotomy.

Keyword

Cavernous sinus thrombosis; Meningitis; Osteopetrosis

MeSH Terms

Abscess
Acid Phosphatase
Anti-Bacterial Agents
Bone Density
Bone Diseases
Carbonic Anhydrase II
Cavernous Sinus Thrombosis*
DNA
Female
Humans
Meningitis*
Osteoclasts
Osteopetrosis*
Pelvis
Sclerosis
Siblings
Skull Base
Spine
Acid Phosphatase
Anti-Bacterial Agents
Carbonic Anhydrase II
DNA

Figure

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  Fig. 1 Radiograph showed that internal fixation state of both femurs due to shaft fracture of both femurs, a common complication of autosomal dominant osteopetrosis type II.

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  Fig. 2 Radiographs of chest (A) and peripheral nervous system (PNS) (B) showed diffuse sclerotic change in whole bony structures. Both ethmoid and maxillary sinusitis with postoperative state of right maxillary sinus was seen.

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  Fig. 3 Brain magnetic resonance imaging (MRI) showed that abnormal leptomeningeal enhancement along both hemisphere, more pronounced right side than left side (A) and abscess formation with abnormal enhancement along right Meckel's cave (blank arrow), right cavernous sinus wall, right side prepontine and mesencephalic cistern, and choroid fissure (B). At hospital day 10, follow up brain MRI showed that aggravation of meningeal enhancement (C) and epidural empyema along right frontotemporal convexity and brain abscess with peripheral edema (filled arrow) in right anterior temporal lobe (D).

• 

  Fig. 4 Plain radiographs of the spine showed end-plate thickening and sclerosis producing the classic "sandwich vertebra" appearance in our patient (A) and in patient's older sister (B).

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