A Large Skull Defect Due to Gorham-Stout Disease: Case Report and Literature Review on Pathogenesis, Diagnosis, and Treatment

A 24-year old man was referred to the Erasmus MC Bone Center because of an asymptomatic increasing skull defect of the left parietal bone. The defect was first noticed at the age of six, and gradually increased over the years. His medical history was unremarkable, without any known trauma and a negative family history for bone diseases. Laboratory tests showed a low vitamin D level without other abnormalities. Particularly, there was no increase in markers of inflammation or bone turnover. CT-scans of the skull showed an osteolytic region of the parietal skull bone, with a two-centimeter increase in diameter over 9 years. Contrast enhanced MRI showed lymphangiogenic invasion, which was compatible with our suspicion of Gorham-Stout disease. The patient was referred to the neurosurgeon for treatment with a bone graft while considering additional drug treatment. Gorham-Stout or vanishing bone disease is a rare entity characterized by progressive osteolysis with lymphangiogenic bone invasion. Although already reported in 1838, currently the diagnosis and treatment of Gorham-Stout disease is still challenging. The underlying pathophysiology is not clarified yet and several theories exist. The disease usually affects persons younger than 40 years and the majority present with bone disease of the maxillofacial region, the upper extremities or the torso. The clinical presentation includes most frequently pain, swelling, and functional impairment of the affected region, but the diseas...
Source: Frontiers in Endocrinology - Category: Endocrinology Source Type: research

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Authors: Zhao CG, Qin J, Wang X, Xu G, Jia Y, Guan YC, Mou X, Yuan H Abstract The aim of the present study was to compare the clinical outcomes of cage-shaped demineralized bone plus local bone grafts (CDBLG) with those of autogenous iliac crest bone grafts (ICBG) implanted for the treatment of single-level lumbar intervertebral disc degenerative diseases. A total of 69 cases of degenerative spinal disorder treated between January 2011 and December 2013 were retrospectively analyzed. Of these, 44 were treated with CDBLG and 25 with autogenous ICBG. All fusions were instrumented single level. Fusion was assessed aft...
Source: Experimental and Therapeutic Medicine - Category: General Medicine Tags: Exp Ther Med Source Type: research
Conclusions: EH of multiple bones responded favorably to intravenous Zometa with improvement of bone metabolic markers. After 1 year on follow-up, the patient was doing well with no significant pain. We suggest that bisphosphonates should be considered in the treatment of multifocal osteolytic EH of bone.
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
CONCLUSION: The treatment of an os calcis lipoma should be individualized, depending on the symptoms, the location and size of the lesion. Surgeons, electing to proceed with bone grafting, should consider the probability of bone graft resorption. PMID: 31360644 [PubMed]
Source: World Journal of Orthopaedics - Category: Orthopaedics Tags: World J Orthop Source Type: research
CONCLUSIONS: Posterior internal fixation for unstable C2 fractures in children can be effective and relatively safe. PMID: 31301388 [PubMed - as supplied by publisher]
Source: Neuro-Chirurgie - Category: Neurosurgery Authors: Tags: Neurochirurgie Source Type: research
ConclusionsTo our knowledge, this is the first report of spondylodiscitis caused byAspergillus terreus after an abdominal penetrating injury. The histological finding of chronic suppurative osteomyelitis and the radiological findings strongly suggested direct inoculation ofAspergillus terreus.
Source: Journal of Medical Case Reports - Category: General Medicine Source Type: research
Rationale: Secondary hyperparathyroidism (SHPT) is often complicated with chronic renal failure. Though the total parathyroidectomy (TPTX) with forearm autotransplantation (FAT) has been commonly used to treatment refractory renal SHPT, the recurrence of SHPT is not infrequent, resulting from hyperplastic autograft, remnant parathyroid tissues, and supernumerary parathyroid gland (SPG). Patient concerns: A 67-year-old man undergoing TPTX+FAT 4 years previously for renal SHPT, who received regular hemodialysis with active vitamin D supplements of Rocaltrol treatment postoperatively, was admitted to our hospital with pr...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
CONCLUSION: Displacement of broken allograft interbody spacer following TLIF procedures can result in neurological sequelae that require revision. To avoid such an occurrence, the authors recommend allowing sufficient time for the reconstitution of the graft in saline prior to use to decrease brittleness, to use an impactor size that is as close as possible to the spacer size and meticulous inspection of the cortical allograft spacer for any visible imperfection prior to insertion. PMID: 31041162 [PubMed]
Source: World Journal of Orthopaedics - Category: Orthopaedics Tags: World J Orthop Source Type: research
We report the first surgical patient who received a semispinalis capitis muscle pedicled, occipital bone graft for supplementation of a revision atlantoaxial arthrodesis. A 72-year-old female had a failed atlantoaxial fusion and developed neck pain from continued instability and fractured hardware. The fixation and fusion were revised and supplemented with a novel, pedicled occipital bone graft. A craniectomy was performed in the occipital bone while still attached to the semispinalis capitis muscle to provide graft vascularity. This graft was rotated inferiorly from the skull base to the C1 arch and C2 spinous process in ...
Source: Journal of Clinical Neuroscience - Category: Neuroscience Source Type: research
ConclusionsEnhanced strut porosity SiCaP EP provided high (month 12: 86.3%) spinal fusion success rates in PLF surgery. Fusion success was associated with improved clinical outcomes in patients within 12  months, relative to baseline.ClinicalTrials.gov identifierNCT01452022Graphical abstractThese slides can be retrieved under Electronic Supplementary Material.
Source: European Spine Journal - Category: Orthopaedics Source Type: research
In this retrospective study, we report the preliminary results of a novel technique for volar vascularized strut grafting to treat avascular scaphoid nonunion by using the 1,2 intercompartmental supraretinacular artery through a single incision. Forty-three of 45 patients with avascular scaphoid nonunion healed at a mean of 13 weeks (range, 3 to 10 mo). Complications consisted of 1 pin tract infection that resolved with oral antibiotics and 4 cases of transient dysesthesia of the radial sensory nerve. In 4 patients with equivocal radiographs, computed tomography scans confirmed bony union. The 2 patients who remaine...
Source: Techniques in Hand and Upper Extremity Surgery - Category: Surgery Tags: Techniques Source Type: research
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