Background: Ollier disease (OD) is a rare nonhereditary type of dyschondroplasia characterized by multiple enchondromas, with typical onset in the first decade of life. Surgery is the only curative treatment for primary disease and its complications. Patients with OD are at risk of malignant transformation of enchondromas and of occurrence of other neoplasms. Methods: A wide literature review disclosed thirty cases of glioma associated with OD, most of them belonging to the pre-molecular era. Our own case was also included. Demographic, clinical, pathologic, molecular, management, and outcome data were analyzed and compared to those of sporadic gliomas. Results: Gliomas associated with OD more frequently occur at younger age, present higher rates of multicentric lesions (49%), brainstem localizations (29%), and significantly lower rates of glioblastomas (7%) histotype. The IDH1 R132H mutation was detected in 80% of gliomas of OD patients and simultaneously in enchondromas and gliomas in 100% of cases. Conclusions: The molecular data suggest a higher risk of occurrence of glioma in patients with enchondromas harboring the IDH1 R132H mutation than those with the IDH1 R132C mutation. Thus, we suggest considering the IDH1 R132H mutation in enchondromas of patients with OD as a predictive risk factor of occurrence of glioma.
Spinal cervical extradural and intra-extradural hemangioblastomas are exceptional, with only nine reported cases. This study reviews the diagnostic and surgical problems of this rare entity. Two female patients, aged 80 years and 25 years, respectively, one with Von Hippel–Lindau disease (VHLD), experienced brachial pain and weakness. On magnetic resonance imaging, a dumbbell intra-extraspinal hemangioblastoma was evidenced. The surgical resection through posterior laminectomy resulted in clinical remission of brachial pain and weakness. The magnetic resonance aspect of a dumbbell lesion suggests a neurogenic tumor; the correct preoperative diagnosis is possible in individuals with VHLD. The surgical problems include high tumor vascularity, vertebral artery control, and nerve root preservation. However, the surgical excision results in clinical remission.
Background: The anterior approach to the cervical spine is safe and effective, but not without risks. The pharyngoesophageal perforation (PEP) is a rare but potentially life-threatening complication of this surgical route. A prompt diagnosis and adequate treatment are crucial for the prognosis; nevertheless, there is no unique consent about the best management. Case Description: A 47-year-old woman was referred to our neurosurgical unit for clinical and neuroradiological signs of multilevel cervical spine spondylodiscitis, which was conservatively treated with long-term antibiotic therapy and cervical immobilization after computed tomography-guided biopsy. Nine months later, when the infection was resolved, the patient underwent C3–C6 spinal fusion with anterior plate and screws through anterior approach to the cervical spine for degenerative vertebral changes causing severe myelopathy, and C5– C6 retrolisthesis with instability. Five days after surgical procedure, the patient developed a pharyngoesophageal-cutaneous fistula, detected through wound drainage, and confirmed by swallowing contrast study, without systemic signs of infection. The PEP was conservatively treated, with antibiotic therapy and parenteral nutrition, and it was monitored through seriate swallowing contrast and magnetic resonance studies up to the complete resolution. Conclusion: The PEP is a potentially fatal complication of the anterior cervical spine surgery. We suggest an accurate intraoperative control of the pharyngoesophageal’s tract integrity at the end of the surgical procedure and a longtime follow-up, because the risk of occurrence is up to several years after surgery.
Background: Thoracic disc herniations are rare and occur at the rate of 1/1,000,000/year. Surgical approach must be individually tailored to the size, location, and consistency of the herniated disc. Notably, here, we report the unusual recurrence of a thoracic herniated disc. Case Description: In 2014, a 53-year-old female presented with thoracic back pain, and paraparesis, attributed to an magnetic resonance imaging/computed tomography (CT)-documented left paramedian T8–T9 calcific disc herniation. She underwent a left hemilaminectomy/costotrasversectomy following which she experienced complete regression of her symptoms. Notably, the postoperative radiological studies at that time demonstrated some residual although asymptomatic calcific disc herniation. Eight years later, she again presented, but now with the chief complaint of difficulty breathing. The new CT scan showed a new calcified herniated disc fragment superimposed on the previously documented residual disc. Through a posterolateral transfacet approach, she underwent resection of the disc complex. An intraoperative CT scan confirmed complete removal of the recurrent calcified disc herniation. Following the second surgery, the patient fully recovered and remains asymptomatic. Conclusion: A 53-year-old female first presented with a left-sided T8/T9 thoracic calcified disc herniation that was initially partially resected). When another larger fragment appeared 8 years later, superimposed on the previously documented residual disc, it was successfully removed through a posterolateral transfacet approach completed with CT guidance and neuronavigation.
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